I relatively generalized syndromes

525

I

RELATIVELY GENERALIZED SYNDROMES

Peripheral Neuropathy (I-l)

Definition

Constant or intermittent burning, aching or lancinating generalized or focal diseases of peripheral nerves.

Site

Usually distal (especially the feet) with burning pain, but often more proximai and deep with aching. Focal with mononeuropathies, in the territory of the affected nerve (e.g. meralgia paraesthetica).

System

Peripheral nervous system.

Main Features

Prevalence - common in neuropathies of diabetes, amyloid, alcoholism, polyarteritis, Guillain Barre Syndrome, neuralgic amyotrophy, Fabry’s disease. Age of Onset - variable, usually after second decade. Quality - (a) burning, superficial distal pain often with dysaesthesia, constant. May be in the territory of a single affected nerve; (b) deep aching, especially nocturnal, constant, and (c) sharp lancinating “tabetic” pains, especially in legs, intermittent.

Associated

limb pains due to

Symptoms Sensory loss, especially to pin prick and temperature; sometimes weakness and muscle atrophy (especially in neuralgic amyotrophy); sometimes reflex loss; sometimes signs of loss of sympathetic function, smooth, fine skin, hair loss.

Laboratory

Findings (a) features of the primary disease, e.g. diabetes; and (b) features of neuropathy: reduced or absent sensory potentials, slowing of motor and sensory conduction velocities, EMG evidence of muscle denervation.

Usual Course

Distal burning and deep aching pains are often long-lasting and the disease processes are relatively unresponsive to therapy. Pain resolves spontaneously in weeks or months in self-limited conditions such as GuillainBarre Syndrome or neuralgic amyotrophy.

Complications

Drug abuse, depression.

Social and Physical Disabilities Decreased mobility. Pathology

Summary

Nerve fibre damage, usually axonal degeneration. Pain especially occurs with small fibre damage (sensory fibres). Nerve biopsy may reveal the above, plus features of the specific disease process, e.g. amyloid. of Essential Features and Diagnostic Criteria Chronic distal burning or deep aching pain with signs of sensory loss with or without muscle weakness, atrophy and reflex toss.

Differential Diagnosis Spinal cord disease, muscle disease.

S26 Code

Arms: 203.X2a 203X% 203X5a 203.X8a

(infective) (inflammatory or immune reactions) (toxic, metabolic, &c.i (unknown or otherj

Legs: 603.X2a 603.X3a 603.X5a 603.X8a References

Thomas, P.K. Pain in peripheral neuropathy: Clinical and morphological aspects. In: Ochoa, J. and Culp, W. (eds.) Abnormal Nerues and Muscles as Impulse Generators. New York: Oxford Univ. Press, 1982. Asburn, A.K. and Fields, H.L. Pain due to peripheral hypothesis. Neurology. 34 (1984) 1587-1590.

nerve damage:

An

Stump Pain (I-2) Definition

Pain at the site of an extremity amputation.

Site

Upper or lower extremity at the region of amputation. Pain is not referred to the absent bcdy part, but is perceived in the stump itself, usually in region of transected nerve(s).

System

Peripheral nervous system; perhaps central nervous system.

Main Features

Sharp, often jabbing pain in stump, usually aggravated by pressure on, or infection in, the stump. Pain often elicited by tapping over neuroma in transected nerve(s).

Assaciated Symptoms Refusal to utilize prosthesis Pain elicited by percussion

SiiS

over stump neuromata.

Laboratov Finding None

Usual

course

Develops several weeks to months untreated.

after amputation;

persists indefinitely if

Relief

(a) Alter prosthesis to avoid pressure on neuromata; (b) resect neuromata so that they no longer lie in pressure areas; and(c) utilize neurosurgical procedures such as rhizotomy and ganglionectomy or spinal cord or peripheral nerve stimulation in properly selected patients.

Complications

Refusal to use prosthesis

Social and Physical DbabUes Severe pain can preclude normal daily activities; failure to utilize prosthesis can add to functional limitations.

S27 Pathology

Neuroma at site of nerve transection.

EssentialFeatures Pain in stump. Di~erenti~ Diagnosis Phamtom limb pain, radiculopathy. Code

203.Xla 603.Xla

Phantom Pain (I-3) Definition

Pain referred to a surgically removed limb or portion thereof.

Site

In the absent body part.

System

Central nervous system.

Main Features

Follows amputation, may commence at time of amputation or months to years later. Varies greatly in severity from person to person. Reports of’prevalence vary from < 1% to > 50% of amputees. Believed to be more common if loss of limb occurs later in life, in limbs than in breast amputation, in the breast before the menopause rather than after it, and particularly if pain was present before the part was lost. Pain may be continuous, often with intermittent exacerbations. Usually cramping, aching, burning; may have superimposed shock-like compo nents. Seems to be less likely if the initial amputation is treated actively and a prosthesis is promptly utilized. Phantom limb pain is almost always associated with distorted image of lost part.

Associated Symptoms Aggravated by stress, systemic disease, poor stump health. Signs

Loss of body part.

Usual Course

Complaints persist inde~nitely; ~equently with gradual ameIioration over years.

Relief

No therapeutic regimen has more than a 30% long-term efficacy. TENS, anticonvulsants, antidepressants or phenothiazines may be helpful. Sympathectomy or surgical procedures upon spinal cord and brain, including stimulation are sometimes helpful.

Social and Physical Disabilities May preclude gainful employment Pathology

Related to deafferentation hyperexcitability.

EssentialFeatures Pain in an absent body part.

or normal daily activities.

of neurons

and their spontaneous

and evoked

S28

Differential Diagnosis Stump

pain

203X7a (armsj 693.X7a (legs)

Code

Causalgia (I-4) Definition

Burning pain, allodynia, and hyperpathia, usually in the hand or foot, after partial injury of a nerve or one of its major branches.

Site

In the region of the limb innervated

Main Features

Onset usually immediately after partial nerve injury or, may be delayed for months. Causalgia of the radial nerve is very rare. The nerves most commonly involved are the median, the sciatic and tibial, and ulnar. Spontaneous pain. Pain described as constant, burning; exacerbated by light touch, stress, temperature change or movement of involved limb, visual and auditory stimuli, e.g.. a sudden sound or bright light, emotional disturbances.

Associated

by the damaged nerve.

Symptoms Atrophy of skin appendages, secondary atrophic changes in bones, joints and muscles. Cool, reddish, clammy skin with excessive sweating. Sensory and motor loss in structure innervated by damaged portion of nerve.

Signs

Cool, reddish, clammy, sweaty skin with atrophy of skin appendages structures in painful area.

Laboratory

and deep

Findings Galvanic skin responses and plethysmography reveal signs of sympathetic nervous system hyperactivity. Roentgenograms may show atrophy of bone.

usual

course

Relief

If untreated, the majority of patients will have symptoms indefinitely; spontaneous remission occurs.

which persist

In early stages of causalgia (first few months) sympathetic blockade plus vigorous physical therapy usually provides transient relief; repeated blocks usuaIIy lead to long-term relief. When a serii of sympathetic blocks does not provide long-term relief, sympathectomy is indicated. Long-term persistence of symptoms reduces the likelihood of successful therapy.

Social and Physical Dkabilities Disuse atrophy of involved limb; complete disruption of normal daily activities by severe pain. Risk of suicide, drug abuse if untreated. Pathology

Partial injury to major peripheral nerve; actual cause of pain is unknown. Peripheral, central and sympathetic mechanisms involved in an unexplained way.

Essential Features Burning pain and cutaneous hypersensitivity with signs of sympathetic activity in portion of limb innervated by partially injured nerve.

hyper-

DifferentialDiagnosis

None Code

207.91 (arm) 607.91 (leg)

Reflex Sympathetic

Dystrophy

(I-5)

Definition

Continuous pain in a portion of an extremity after trauma which may include fracture but does not involve a major nerve, associated with sympathetic hyperactivity.

Site

Usuallythe distral extremity adjacent to a traumatized area.

System

Peripheral nervous system; possibly the central nervous system.

Main Features

The pain follows trauma (usually mild), not associated with significant nerve injury; the pain is described as burning, continuous, exacerbated by movement, cutaneous stimulation, or stress. Onset usually weeks after injury.

Associated Symptoms

Initiallythere is vaso-dilatation with increasing temperature, hyperhidrosis and oedema. Hyperhidrosis and reduced sympathetic activity also occur. Atrophy of skin appendages, cool, red, clammy skin are variably present. Disuse atrophy of deep structures may progress to Sudeck’s atrophy of bone. Aggravated by use of body part, relieved by immobilization. Sometimes foilows a herniated inte~e~ebr~ disc, spinal anaesthesia, ~liomyelitis, severe iliofemoral thrombosis or cardiac infarction. This may appear as the shoulder-hand syndrome. Later vasospastic symptoms become prominent with persistent coldness of the affected extremity, pallor and cyanosis, Raynaud’s phenomenon, atrophy of the skin and nails and loss of hair, atrophy of soft tissues and stiffness of joints. Without therapy these symptoms may persist. It is not necessary for one patient to exhibit all symptoms together. An additional limb or limbs may be affected as well. Signs

Variable; there may be florid sympathetic hyperactivity.

Laboratory Findings

In advanced cases, roentgenograms may show atrophy of bone. Usual Course

Persists indefinitely if untreated; smali incidence of spontaneous remission.

Relief

Sympathetic block and physical therapy; sympathectomy iflong-term results not achieved with repeated blocks; may respond in early phases to high doses of corticosteroids, e.g. Prednisone 50 mg. daily.

Complications

Disuse atrophy of involved limb; suicide and drug abuse if untreated; sometimes spreads to contralateral limb.

Social and Physical Disability

Depression, inability to perform daily activities.

S30

Pathology

Unknowtl

Essential Features Burning pain in distal extremity usually after minor injury without I wve damage Differential Diagnosis Unrecognized local pathology (fracture, strain, sprain). Causalgta, post-trau matic vasospasm or thrombosis. Code

203.91b (arms) 603.91b (legs)

Central Pain (Including Thaimnic and Pseudo-Thalmnic Pain) (I-6) Definition

Diffuse unilateral pain, often burning with allodynia, hypoaesthesia, hypoalgesia. hyperpathi, dysaesthesiae and neurological signs of damage to structures which supply the affected region.

Site

Usually one-half of the whole body, contralateral to a cerebral iesion; rarely the face andior the head alone (more often one lower limb alone); sometimes the upper extremity, with or without the head; most often by far the entire contralateral half of the body or the upper and lower extremities together. In some cases the face on one side and limb(s) on the opposite side (as with bulbar lesions).

System

Central nervous system.

Main Features

Onset is most often after age of 40 years in groups subject to cardiovascular disease. Pain most often appears within a few weeks to 2 years later. The pain is spontaneous, continuous, burning, stabbing and often aching. It varies from mild to intolerable in different cases and is localized to the skin, muscle or bone in the majority of patients. Usually constant and persistent by day, the pain is subject to exacerbations which may occur for no apparent reason or be evoked by normally non-noxious stimuli such as light touch, heat, cold, movements, TENS. It can be made worse also by auditory or visual stimuli (e.g., sudden sound or light) and by visceral activity, e.g., micturition, and is often exacerbated by anxiety and emotional arousal.

Associated

Symptoms

and Sii

There may be various neurological symptoms and signs, especially those typical of hemiparesis. Most often there are motor impairments and sensory deficits in the affected areas. Light touch is impaired. Allodynia and hyperpathia are almost always found and dysaesthesii often occur either spontaneously or otherwise. Vasomotor and sudomotor atrophic changes are often present. Anxiety and depression often occur with the syndrome. Laboratory

Findings CT scan may indicate a relevant lesion in the thalamus or elsewhere, especially the cerebral peduncies.

$531 usual course

Persists indefinitely in most cases. In some, may show signs of ~minution.

Relief

May be relieved by centrally-active medications such as phenothiazines and tricyclic antidepressants. Stabbing element often appears to be helped by carbamazepine or diphenyl hydantoin.

Complications

Depression, suicide.

Social and Physical Disabilities

Work impairment. Pathology

Thalarnic lesions due to any pathol~ical process that damages the ventral postero-lateral nucleus. More than 80% of cases are due to cerebra-vascular haemorrhage or occlusion. May be complication of surgery, neoplasm, trauma, multiple sclerosis. Lesions in cerebral peduncles, pons and medulla and juxtathalamic areas may produce similar syndrome due to comparable causes, but pain is ipsilateral in the face and contralateral to the lesion elsewhere. Within these areas the followingtypes of cause are particularly likely to produce pain: occlusion of posterior inferior cerebellar artery; occlusion of posterior cerebral artery or of its branches to the brain-stem; haematobulbia or syringobulbia; neoplasm; multiple sclerosis; trauma; and stereotactic surgical lesions. Facial pain may aiso be due to bulbar lesions. Occasional cases of localized cerebral lesions producing central pain have afso been reported.

Diagnostic Criteria

Spontaneous pain with allodynia, hypoaesthesia, hypoalgesia and hyperpathia with evidence of damage to the thafamus or structures or the central nervous system which supply the affected region. DifferentialDiagnosis

If pain involves all of one-half of body, differentiation from hysteria may be required. Pain localized to the head alone, or one limb alone, may require differentiation from other neuro1~~~ disorders. Pain from spinal lesions is not included. Code

xo3.x5c

003.X5c when affecting head and face; code additional entries for other areas.

;;ro3.Xlc xo3X2c xo3.x4c XO3.X8c

Syndrome of Syringomyelia (I-7) Definition

Aching or burning pain usually in a limb, commonly with muscle wasting due to tubular cavitation gradually developing in the spinal cord.

Site

Pain in shoulder-arm or leg, rareIy in the face, occasionally bilateral.

System

Central nervous system.

s32

Main Features

Associated

Pain is usually unilateral and continuous in an area that corresponds to the site 01 cavitation of spinal cord or brainstem, most frequently in the shoulder-girdle and arm. It may be a periodic diffuse dull ache but sometimes, and particularly when the pain is situated in forearm and hand, may have an intense burning quality. The pain may be severe and referred to deep structures irl the limb. no! responding to rest or minor sedation.

Symptoms Muscular weakness in affected region. There is commonly muscle wasting beginning in small muscles of the hand and ascending to the forearm and shoulder-girdle with fasciculation and an early loss of tendon reflexes. Scoliosis and kyphosis may occur. Characteristically, pain and temperature sensations are impaired but other sensations are intact. The area of sensory impairment typically has a shawl distribution over the front and back of the upper thorax. A Homer’s syndrome may appear.

usual

CourEie

The disease usually begins in the second or third decade and slowly progresses.

sociaiandFJhy&alDisabiii The disease may be present for 15 or 20 years, progressing slowly, but still compatible with an active self-supporting life. After 15 or 20 years the problems of pain, weakness and general infirmity usually result in increasing invalidism, eventually leading to total dependency. Pathdosy

A tubular cavitation develops slowly in the spinal cord extending over many segments. The most common location is in the lower cervical cord near the central canal. There is loss of anterior horn cells and interruption of spinothalamic fibers. The cavity may be lined by a thick layer of gliil tissue. Cavities may be bilateral and asymmetric and may communicate with an enlarged central canal. Ascent of the cavity into the brain stem produces syringobulbia. The canal may extend the entire length of the cord. Associated findings may be ectopic cerebellar tonsils, hydrocephalus, cerebellar hypopIasii, and astrocytoma or ependyrnoma of the spinal cord.

Essential Features Pain in the relevant distribution of slowly progressing muscle weakness and wasting and impairment of sensation to pin prick and temperature, whilst other sensory modal&s remain intact. Differential Diagnosis Other conditions which have to be considered are: (1) amyotrophic lateral sclerosis; (2) multiple sclerasis; (3) tumor of the spinal cord; (4) skeletal anomalies of the cervical spine; (5) platybasii; and (6) cervical spondylosis. Code

007.X0 (face) 207.X0 (arm) 607.x0 (leg)

s33

Polymyalgia Rheumatica (I-8) Definition

Diffuse aching, and usually stiffness, in neck, hip girdle or shoulder girdle, usually associated with a markedly raised sedimentation rate, sometimes associated with giant cell vasculitis, and promptly responsive to steroids.

System

Muscul~kelet~.

Main Features

Incidence about 53.7 per fOO,OOO in those over 30 years of age. Deep muscular aching pain usually begins in the neck, shoulder girdle and upper arms, but may only involve the pelvis and proximal parts of the thighs. Morning stiffness and stiffness after inactivity are protiinent features.

Associated Symptoms

Malaise, fatigue, depression, low grade fever, weight loss, and giant cell arteritis. Aggravating Factors

Movement. Signs

No muscle tenderness or weakness.

Laboratory Findings

Anaemia of chronic disease, raised ~~mentatjon ~m~hour Westergren).

rate (usu~~y greater than

Relief

Dramatic response to oral corticosteroids, usually in low doses, e.g. 520mg Prednisone daily.

Complications

Blindness from giant cell arteritis.

PathoIogy

Giant ceil vasculitis.

EssentialFeatures

Diffuse pain with malaise, elevated sedimentation rate, response to steroids. DifferentialDiagnosis

Polymyositis, fibrositis, hyperthyroidism. Code

X32X3a

Fibrositis or Diise

Myofascial Pain Syndrome (I-9)

(Non-articular rheumatism, fibromyalgia) Definition

Diffuse aching musculoskeletal pain associated with multiplediscrete predictable tender points and stiffness.

Site

Multiple anatomical areas.

System

Musculoskeletal (ligaments and tendons).

Main Features

Primary fibrositis, without important associated disease 1s uncommon compared to secondary diffuse fibrositis. It usually occurs in the3rd to 4th decade. The sex ratio is 5 female to one male (5:l). Pain Widespread aching, of more than 3 months duration, poorly circumscribed and perceived as deep, usually referred to muscle or bony prominences. Although pain in the trunk and proximal girdle is aching, distal limb pain is often perceived as associated with swelling, numbness or stiff feeling. Day to day fluctuation in pawn intensity and shifting from one area to another is characteristic although the pain is usually continuous. Stiffness - is perceived as an increased resistance to joint movement particularly towards the end of the range of movement. It is worse in the mornings. Both the pain and the stiffness are maximal within the broad sclerotomic and myotomic areas of reference of the lower segments ot the cervical and lumbar spine. Chronic exhaustion is often marked in the morntng and associated with feeling unrefreshed after rest or sleep. Tender Pninfs --synonym: trigger points (TP). Discrete local areas of deep tenderness involving a variety of otherwise normal tissues are a pathognomonic feature provided that at least 12 are present. The tender points usually lie within an area of taut muscle fibres (“taut bands”) or over origins and insertions (entheses). The patient is usually unaware of the existence of these points. Palpation of them may be associated with a “jump sign” in which the patients move or t:ry out with minimal pressure at these points. The pressure may cause pain at a different site (area of reference) usually reproducing the pain complained of by the patient. The predictable location of these tender points and their multiplicity are essentiai features of the syndrome. Epidemiology

Associated Symptoms Tenderness and Reactive Hyperaemia - rolling oi the skin and subcutaneous tissue of the upper scapula region between the examiner’s thumb and index finger characteristically elicits tenderness and reactive hyperaemia, particulariy in the general regions of deep tenderness. The distal limbs and lumbar region usually are devoid of this phenomenon of reactive hyperaemia. Autonomic Phenomena -. although a variety of autonomic phenomena are described as associated features, only reactive hyperaemia or erythema is common. Skinfold

Aggravating and Rekving Factors Cold, damp weather, interrupted sleep, fatigue, mental stress and under or over-exercise accentuate the widespread aching and stiffness. Local heat, massage, warm dry weather, moderate activity and exercise, and relief horn stress such as vacations, give temporary symptomatic relief. Chronic muscle overloads may occur in settings that demand repetitive or sustained contractions, poor posture or work habits, and stress that is manifested by increased muscle tension. Chronic overload is also related to skeletal asymmetry or disproportion, dental malocclusion, ergonomicaily inappropriate furniture, and prolonged constrictive pressure or immobiity. Trigger point sensitivity is positively correlated with a sedentary lifestyle; and myofascial pain with associated headache, neck pain, or low back pain is common among persons reporting many hours of TV-viewing. There may also be indirect routes of TP activation, including visceral disease (myocardii infarctions, peptic ulcer, ch&Iithiiis, renalcokf, nerve compression, disc disease, arthritic joint involvement, and neuropathy.

s35 Signs

See alsotender points. Widespread tenderness over the trapezius muscles at the

mid-point of the upper fold and also at the second costochondral junctions is found. Further tender points occur l-2cm distal to the lateral epicondyle; at the origin of the supraspinatus tendons, the interspinous ligaments of L4-Sl, the upper outer quadrants of buttocks and the medial fat pads of the knees. Relief

Relief may be provided by reassurance and explanation about the nature of the illness and possible mechanism of pain, by symptomatic treatment with heat or massage and by encouraging moderate activity. Low dose amitriptyline may help.

Pathology

No consistent specific or nonspecific pathological feature has been demonstrated in the tissues. Thermography may demonstrate patterns of skin thermal radiation correlated with tender points. Some authors imply an association between chronic cervical and lumbar strain and the frequent occurrence of tender points and areas of aching that are found in the broad sclerotomal and myotomal reference areas of the lower cervical and lumbar segments. It is in these motion segments that degenerative changes maxi~ly occur.

Aetiology

Unknown. One view holds that physical or mental stress or fatigue exacerbate a pathophysiological dysfunction. Another view suggests that this syndrome represents epiphenomena and that the basic problem is altered behaviour of the lower cervical and lumbar motion segments where postural stress and mobility requirements are high. Experimental reproduction of the diffuse fibrositis syndrome was obtained in some healthy university students by means of deprivation of slow wave nonREM sleep.

Diagnostic Criteria Primary Diffuse Fibrosifis Syndrome -

(1) Widespread aching of more than 3 months duration; (2) skinroll tenderness over the upper scapular region; (3) disturbed sleep, with morning fatigue and stiffness; (4) demonstrated bilateral tender points in at least 6 areas, mid-point of upper fold of trapezius; superiorlateral aspect of 2nd costochondral junction; anterior aspect of interspinous spaces of C4C6; lateral epicondyle within proximal muscle belly of the long finger extensor; interspinous ligament region of L4Sl; upper outer buttock over gluteus medius; medial knee fat pad, overlying medial collateral ligament, proximal to joint line; and (5) absence of laboratory evidence of infl~mation or muscle damage. NB: Criteria 1,4 and 5 are essential to establish a diagnosis of Primary Diffuse Fibrositis Syndrome. Criteria 1 and 4 only are needed for a diagnosis of Secondary Diffuse Fibrositis Syndrome. Code

X33.XSa

References

Cyriax, J. Textbook of Orthopaedic Medicine, Vol. 1. Fam, A.G. and Smythe, H.A. Musculoskeletal chest wallpain. Con. Med. Assoc.

J., 133 (1985) 379-389.

Hier, D.B. and Spector, R.H. Neuropsychiatric disorders. In: M.A. Samuets (Ed.), Manual of Neurologic Therapeutics, 3rd edition, Little. Brown & Co.. Boston, 1985, in press. Kellgren, J.H. Observations on referred pain from muscle. C/in. SC-I..3 (1938) 175.190. Ochoa, J. and Torebjork, H.E. Pain from skin and muscle. Pain (Suppl.) I ( 1081) 08.

Reynolds, M.D. The development of the concept of fibrositis.J. Hisr. Med. Allied sci., 38 (1983) 535.

Simons, D.G. Myofascial pain syndrome. In: J. Basmajian and R.L. Kirby (Eds. 1. Medical Re~b~~i~u~on,pp. 209214.

Note: Specifx Myofascial

PainSyndromes

Synonyms: fibrositis (syndrome), myalgia, muscular rheumatism, nonarticular rheumatism. Specific myofascial syndromes may occur in any voluntary muscle with referred pain, local and referred tendemess, and a tense shortened muscle. The pain has the same qualities as that of the diffuse syndromes. Passive stretch or strong voluntary contraction in the shortened position of the muscle is painful. Satellite tender points may develop within the area of pain reference of the initial trigger point. Other phenomena resemble those of the diffuse syndromes. Diagnosis depends upon the demonstration of a trigger point (tender point) and reproduction of the pain by manoeuvres which place stress upon proximal structures or nerve roots. This suggests that the syndrome is an ~~~o~non secondary to proximal pathology such as nerve root irritatiin. Relii may be obtained by stretch and spray techniques, tender point compression or tender point injection including the use of “dry” needling. Some individualsyndromes are described here, e.g., stemocleidomastoid and trapezius. Others may be coded as required according to individual muscles which are identified as being a site of trouble.

Rheumatoid Arth&is

(I-10)

Definition

Aching, burning joint pain due to systemic inflammatory dii affecting all synovial joints, muscle, ligaments and tendons in accordance with diagnostic criteria below.

We

Symmetrical involvement of small and large joints.

SYStetll

Musculoskeletal system and connective tissue.

Main Features

Diffuse aching, burning pain in joints, usually moderately severe; usually intermittent with exacerbations and remissions. The condition af%cts about I% of the population and is more common in women. Diagnostic criteria of the American Rheumatism Association describe and further define the illness.They are as follows: (1) morning stiffness, (2) pain on motion or tenderness at 1joint or more; (3) swelling of one joint; (4) swelling of at least one other joint; and (5) symmetrical joint swelling. Allof the above have to be of at least 6 weeks duration. Further criteria include: (6) subcutaneous nodules; (7) typical radiographic changes; (8) positive test for rheumatoid factor in the serum; (9) a poor response in the mucin clot test in the synovial fluid, (10) syrx%al histopathology consistent with rheumatoid arthritis; and (11) characteristic nodule pathology.

S37 Classical rheumatoid arthritis requires 7 criteria to be diagnosed. Definite rheumatoid arthritis may be diagnosed on 5’criteria and probable rheumatoid arthritis on 3 criteria. _ Associated Symptoms Morning stiffness usually greater than half an hour’s duration; chronic fatigue. Inflammation may affect eyes, heart, lungs. Signs

Tenderness, swelling, loss of range of motion of joints, ligaments, tendons. Chronic destruction and joint deformity are common.

Laboratory Findings Anaemia, raised ESR (sedimentation majority of cases.

rate), rheumatoid factor in the serum in the

Relief

Usually good relief of pain and stiffness can be obtained with nonsteroidal anti-inflammatory drugs, but some patients require therapy with gold or other agents.

Pathology

Chronic inflammatory process of synovium, ligaments or tendons. There may be systemic vasculitis.

EssentialFeatures Aching, burning joint pain with characteristic

pathology.

DifferentialDiagnosis Systemic lupus erythematosus, palindromic rheumatism, mixed connective tissue disease, psoriatic arthropathy, calcium pyrophosphate deposition disease, seronegative spondyloarthropathies, haemochromatosis (rarely). Code

X34.X3a

Osteoarthritis (I-11) Definition

Deep, aching pain due to a “degenerative” process in a single joint or multiple joints, either as a primary phenomenon or secondary to other disease.

Site

Joints most commonly involved are distal and proximal interphalangeal joints of the hands, the carpo-metacarpal thumb joint, the knees, the hips and cervical and lumbar spines. Many joints or only a few joints may be affected, e.g. at C5 or L5, the hip or knee; proximal joints may be involved alone or only distal interphalangeal joints.

System

Musculoskeletal.

Main Features

There is deep, aching pain which may be severe as the disease progresses. The pain is felt at the joint or joints involved but may be referred to adjacent muscle groups. Usually the pain increases in proportion to the amount of use of the joint. As the disease progresses there is pain at rest and later nocturnal pain. The pain tends to become more continuous as the severity of the process increases. Stiffness occurs after protracted periods of inactivity and in the morning but lasts less than half an hour as a rule.

There is a discrepancy between radiological prevalence and cllnlc
Features Use, fatigue.

SigtlS

Clinically, joint line tenderness may be found and crepitus on active or passive joint motion; non-inflammatory efisions are common. Later stage disease is accompanied by gross deformity, bony-hypertrophy, contracturc. X-ray evidence of joint space narrowing, sclerosis, cysts, and osteophytes may occur

Ldoratoxy

Findings None specific.

usual

Initially there is pain with use and minimal x-ray and clinical findings. Later pain becomes more prolonged as the disease progresses and nocturnal pain occurs. The course is one of gradually progressive pain and deformity.

course

Relief

Some have relief with nonsteroidal anti-inflammatory agents or with non-narcotic analgesics. Joint rest in the early stages relieves the pain. Occasional relief in the early phases may appear from intra-articular steroids.

Physical Disability Progressive limitation of ambulation occurs in large weight bearing joints. Pathology

This is loosely described as a “degenerative”

disease of articular cartilage.

Essential Features Deep, aching pain associated with the characteristic joints.

“degenerative”

changes in

Llifferentiai Llbgmsii Calcium pyrophosphate deposition disease; presence of congenital traumatic, inflammatory, endocrinologkal or metabolic disease to which the osteoarthritis may be secondary. Code

X38.X6 a

Calcium PVrophosphate Depodhm

IIBiwam(CPPD) (i-12)

DefGtion

Attacks of aching, sharp and throbbing pain with acute or chronic recurrent inflammation of a joint caused by calcium pyrophosphate crystals.

Site

Usually one joint, sometimes more, often alternating. metacarpckphalangeal joiits are most frequent sites.

Knees,

wrists, and

s39 System

Musculoskeletal.

Main Features

The disorder occurs clinically in about 1 in 1000 adults, more often in the elderly, but radiology shows the presence of the disease in 5% of adults at the time of death. There are 4 major clinical presentations: (1) pseudogout - acute redness, heat, swelling and severe pain which is aching, sharp or throbbing in one or a few joints. The attacks last from 2 days to several weeks with freedom from pain between attacks. (2) pseudorheumatoidarthritis - marked by deep aching and swelling in multiple joints with attacks lasting weeks to months; (3) pseudoosteoarthritis - see the description of osteoarthritic features; and (4) pseudoosteoarthritis with acute attacks - the pain being the same as in osteoarthritis but with superimposed acute painful swollen joints.

Signs

Aspiration of calcium pyrophosphate crystals from the joint is diagnostic. X-rays show calcification in the cartilage of the wrists, knees and symphysis pubis.

Relief

Acute attacks respond well to nonsteroidal without local corticosteroid injections.

Complications

Chronic disabling arthritis.

anti-inflammatory

drugs, with or

Associated Disorders Hyperparathyroidism, metabolic causes. Pathology

hemochromatosis.

There may be hereditary, sporadic, or

Acute and chronic inflammation or degeneration.

DifferentialDiagnosis Gout, infection, palindromic rheumatism, Code or

osteoarthritis.

x38.X0 X38.X5a

Gout (1-13) Definition

Paroxysmal attacks of aching, sharp or throbbing pain, usually severe and due to inflammation of a joint caused by monosodium urate crystals.

Site

First metatarso-phalangeal or elbows.

Main Features

Commoner in men in the 4th to 6th decades of life and in post menopausal women. Acute severe paroxysmal attacks of pain occur with redness, heat, swelling, and tenderness, usually in one joint. The pain is aching, sharp and throbbing. The patient is often unable to accept the weight of bedclothes on the joint and unable to bear weight on the affected joint. Attacks last 2 days to several weeks in duration.

joints, mid-tarsal joints, ankles, knees, wrists, fingers

Associated Symptoms In the acute phase, patients may be febrile and have leukocytosis.

Awavating

Factors Trauma, alcohol ingestion, surgery, starvatron.

Signs

Redness, heat and tender swelling of the joint which may be extremely painful ro move. Intracellular urate crystals, aspirated from the joint, are diagnostic.

Laboratory

Findings Serum urate may vary during the acute attack. sedimentation rate are seen during the attack.

usual

course

Leukocytosis

and raised

Initially the disorder is monoarticular; in 50% of patients the first metatarso phafangeal joint is involved in the great toe. Acute attacks are separated by variable symptom-free intervals. Attacks may become polyarticular and recur at shorter intervals and may eventually resolve incompletely leaving chronic. progressive crippling arthritis.

Relief

Responds well to nonsteroidal and local steroid injections.

Complications

Renal calculi, tophaceous

Pathokqy

Acute inflammatory response induced by uric acid crystals.

anti-inflammatory

agents, intravenous

colchicine

deposits, and chronic arthritis with joint damage.

Essential Features Paroxysmal joint pains with sodium monourate

deposition.

Differential Diagnosis Calcium pyrophosphate Code

deposition disease, infection, palindromic rheumatism.

X38.X5b

Haemophibc Arthropathy(I-14) Dehition

Routs of acute, constant, nagging, burning, bursting and incapacitating pain or chronic, aching, nagging, gnawing and grating pain occurring in patients with congenital blood coagulation factor deficiencies and secondary to haemarthrosis.

Site

The most common joints affected initially are the knees, ankles and elbows. Shoulders, hi and wrist joints are affected next most often. As the first joints become progressively affected, other remaining articular and muscle areas are involved with changes of disuse atrophy or progressive haemorrhagic episodes.

System

Musculoskeletal.

Main Features

Prevalence - haernophiliac joint haemorrhages occur in severely and moderately affected male haemophiliacs. They only rarefy occur in female Factor VIII and Factor IX can-& and in hornozygous severely affected patients with von Willebrand’s disease. Acute haematthroeis occurs most commonly in the juvenile in association with minor trauma. In the adult spontaneous haemorrhages and pain occur in association also with minor or severe trauma. Characteristically the acute pain is associated with such haemarthrosis whiih is

!541 relieved by replacement therapy and rest of the affected limb. A reactive synovitis results from repeated haemarthroses which may be simply spontaneous small recurrent haemorrhages. The pain associated with them is extremely difficult to treat because of the underlying inflammatory reaction. Time Course - The acute pain is marked by fullness and stiffness and constant nagging, burning or bursting qualities. It is incapacitating and will cause severe pain for at least a week depending upon the degree of intracapsular swelling and pressure. It will recur episodically from the causes indicated. Chronic pain is otten a dull ache, worse with movement, but can be debilitating, gnawing and grating. At the stage of destructive joint changes the chronic pain is unremitting and relieved mainly by rest and analgesics. These syndromes are exacerbated by accompanying joint and muscle degeneration due to lack of mobility rather than repeated haemorrhages. Associated Symptoms Depressive or passive/aggressive symptoms often accompany haemorrhages and are secondary to the extent of pain or to the realization of vulnerability to haemorrhage, which is beyond the control of the haemophiliac. If bleeding occurs into a muscle or potential space, e.g., retroperitoneal space, and iliopsoas muscle, this can mimic joint haemorrhage and also cause severe nerve compression syndromes, e.g., of the femoral nerve. Numerous psychosomatic complaints are associated with the chronic and acute pain of chronic synovitis, arthritis and haemarthrosis. Signs

Reactive Synouitis - There is a chronic swelling of the joint with a “boggy” consistency to the swelling which is tender to palpation. Marked limitation of joint movement with, often, signs of adjacent involvement of muscle groups due to disuse atrophy. Chronic Joint Degeneration - Severe bony remodelling with decrease in joint movement, adjacent muscular atrophy with subsequent fixation of the joint and loss of effective use.

Laboratory Findings X-rays with the large haemarthrosis show little except for soft tissue swelling. In reactive synovitis there is often evidence of osteoporosis accompanied by overgrowth of the epiphyses but no evidence of joint destruction. In chronic arthropathy there is cartilage destruction and narrowing of the joint space. Gross misalignment of the joint surfaces progresses. Cysts, rarefactions, sub-condylar cysts, and an overgrowth of the epiphysis are noted. This progresses through to fibrous joint contracture, loss of joint space, extensive enlargement of the epiphysis and substantial disorganization of the joint structures. The articular cartilage shows extensive degeneration with fibrillation and ebumated bone ends. Usual Course

Until the availability of therapy with blood clotting factor concentrate there was an inexorable deterioration of the affected joints, following the initial repeated spontaneous haemarthroses in the severely affected individual. Thii joint deterioration was associated with pain as described in the section regarding time course. The introduction of concentrated clotting factor transfusions has avoided the consequence of repeated acute severe haematthroses. However, it is by no means certain whether the pain pattern of chronic synovitis and arthritis can be avoided or merely delayed using such therapy. Therapy with blood clotting factor concentrate is only available on a regular basis in North America and Europe at this time.

s42 Relief

replacement wrth qqxopnatt, coagulation factors with subsequent graded exercise and physiotherapy will provide good relief. Aspiration of the joint till be necessary under coagulation factor cover if there is excessive intracapsular pressure. Analgesics ‘Ire required for acute pain management. Reactive and Chronic ~aernarthr~s~~ Prophy lactic factor replacement is required in association with analgesics and careful19 selectedanti-inflammatory agents, e.g., steroids or Ibuprofen. Pain control using analgesics and transcutaneous nerve stimulation is also useful and physiotherapy is of considerable assistance in managing both symptoms and signs. Synovrctomy may be of use for the control of pain secondary to the recurrent bleeding. Chronic Destructiue Arthropathy - Replacement therapy is of iittie assistance in relieving pain and disability. Carefully selected anti-inflammato~, agents and rest are the major therapies of use. Physiotherapy after control 01 SC-W symptoms is useful. Joint replacement is a final choice for ~.:YOIW pair; management.

Complications

Analgesic abuse is a common problem in haemophilia due to the
Sod

Acute Haemarthrosk -.- Adequate intravenous

and Physical Disability Severe crippling and physical disability, with prolonged schooi and work absences, have traditionally been associated with this form of arthropathy. Consequently, affected individuals have not been able to achieve satisfactory school and job schedules. It is considered that the higher suicide rate is not only related to the family and psychosocial aspects of the disease but also the chronic pain syndromes which these indi~duals experience.

Pathology

This depends upon the phase of the disorder. Generally two pathologic phases are associated with the haemophiliic joint. Phase one involves an early synovial soft tissue reaction caused by intraarticular bleeding. Synovial hypertrophy with haemosiderin deposition and miM perivascular inflammation are present. Cartilage degeneration and joint degeneration, similar to that seen in osteoarthritis and rheumatoid arthritis is seen in the second phase joint. Associated with this type of phase two change is synovial thickening and hyperplasia which falls into numerous folds and clusters of villi. The amount of haemosiderin deposited is increased compared to phase one.

!Summary of Eksential Features and Diagnostic Criteria Acute and chronc pain, as the result of acute haemarthrosis with chronic synovial, cartilaginous and bony degeneration is exacerbated by spontaneous and trauma-related haemorrhage.

In the presence of a severe (less than 0.01 units/ml) haemophilic factor deficiency no other diagnosis is possible. In the mildly affected individual (greater than 0.05 units/ml) all other causes of degenerative arthritis, particularly in the older affected individual, must be considered. X34.XOa

References

Arnold, W.D., Hilgartner, M.W. Haemophilia arthropathy - current concepts of pathogenesis and management. J. Bone Joint Surg., 59A (1977) 287-305. Duthie, R.B., Matthews, J.M., Rizza, C.R., and Steele, W.M. The Management of Musculoskeletol Problems in the Haemophiliac, 1st edition. Blackwell, Oxford, 1972. Hilgartner, M.W. Haemophiliac arthropathy.

Adu. Paediatr., 21(1975) 139-165.

Hoskinson, J., Duthie, R.B. Management of musculoskeletal problems in the haemophiliac. Orthop. Clinics North Amer., W.B. Saunders, 1978, pp. 455-480.

Bums (I-15) Definition

Acute and severe pain at first, following bums, later continuous tions, gradually declining.

Site

Anywhere on the body surface and deep to it.

System

Usually only epidermis and/or dermis, but any system may be involved.

Main Features

The prevalence is approximately

with exacerba-

3 per thousand of the population. Ten per cent of these will require hospital admission. Any age can be affected but the highest incidence (18%)is between 20 and 29 years. Children are the next largest group with 30% of these being in the 1-2 year age group. Sex ratio: approximately l:l, but 3:2 males to females in children. Initially the pain is acute and intense. It is frequently described as throbbing, smarting and stinging and marked exacerbations of stabbing pain occur with any movement or procedure. Thus, it is particularly intense where there are skin creases, flexures or where pressure is applied, such as palms, soles, genitalia, ears or resting surfaces. This applies especially to partial thickness burns. Despite the destruction of all cutaneous nerve endings, full thickness bums are often painful with a quality described as deep, dull and aching. The pain tends to diminish in intensity as healing takes place. In addition the quality of the pain changes and at one to two weeks after the bum, is usually described as sore, aching, tender, tiring and tight. After three or four weeks it is described as itchy or tingling. These descriptions also apply to pain at donor sites. Pain is exacerbated by procedures such as “tanking” for the removal of eschar, and physiotherapy. In addition, frequent surgery is often necessary with an accompanying increase in pain. Relief may be promoted by the use of opioid premeditation prior to procedures, time-contingent analgesics, inhalational analgesia during procedures, ensuring that the burnt areas never dry out, protecting the burn with creams and achieving skin cover by some means as soon as possible.

Associated Symptoms

Dyspnoea may occur as a result of smoke inhalation. causalgia-like symptoms. Usual Course

Disuse may lead to

Tends to settle with skin healing. Burnt areas may be tender and sore for up to a year. Itch and irritation may continue for two or three weeks.

s44 Complications

If healing occurs it is unusual to have persistent pain unless deep structures (muscle, bones, major nerves) are involved. Cell&is in burnt areas or donor sites may lead to a marked increase in the severity of pain.

This is most frequent where the burn is extensive and such cases often require sustained treatment and prolonged hospitalization. Psychological treatment is also needed where scars affect the patients’ ability to function socially or physically, for example, as a result of scars of the hands, face or genitalia. Pathdogy

Summary

Loss of skinintegrity with consequent loss of fluid and thermoregulation and an increased likelihood of infection. Burns are classified in three degrees of severity based on bum depth. A superficial bum involves the epidermis only. A partial thickness bum involves epidermis and dermis at varying depths and a full thickness bum involves epidermis, dermis and at times deeper tissues. Electrical bums may cause considerabIe damage to deeper tissues by direct effect and by occlusion of blood vessels. The severity of damage is related to the temperature to which the area was exposed, the duration of exposure and the thickness of the skin involved. The agents responsible may be thermal, electrical or chemical. of Essential Feature.sand Diagnostic Criteria Pain with the appropriate

time course following burns.

DifferentialDiagnosis Possibly hysterical conversion pain or pain of psychological origin may prolong or exacerbate the original effects of the injury. This may be more important in work-related injuries or where there is litigation. Code or

x42x1 X82X1

Pain of Psychological Originz MuscleTension

Pain (I-16)

Dehition

Virtually continuous pain in any part of the My due to sustained muscle contraction and provoked by emotional causes or by persistent overuse of particular muscles.

Site

Any region with pain reference from voluntary muscle.

svstm

Central nervous system (psychological and social)

Main Features

Prewlence: often diagnosed. Even approximate prevalence is unknown. Onset: from age 8 onwards, usually before age 30. Females more than males. Ratio 4: 1 in those who consult doctors. Start: gradual emergence intermittent at first, as mild diise ache or unpleasant feeling, increasing to a definite pain part of the time. Fluctuation during the day is typical. These exacerbations seem to emerge after several years of lesser headache. Occurrence and Lhratim: most days per week, usually every day for most of the day. Occasionally in longstanding severe cases pain may wake the patient from sleep. Qua&y: dull ache, us& cbes not throb; severe dur5ng exacerbations often or almost always with thmbbi~ Some describe tight bands or gripping headache. They may be a minority. Others describe pressure sensations. Precjpitants andEmcerbd~Firctors: emotional stress, anxiety and depression, physical exercise, alcohol.

Associated Symptoms

Many patients have anxiety, depression, combined.

irritability or more than one of these

Signs

Muscle tenderness normal individuals.

Relief

Resolution or treatment of emotional problems, anxiety or depression often diminishes symptoms. Relaxation treatment helps. Anxiolytics may help but should be avoided since some patients become depressed and others develop dependence. Tricyclic antidepressants are frequently very useful. Analgesics only help a little.

Complications

Analgesic, narcotic and other drug abuse.

occurs but may also be found in other conditions

and in

Social and Physical Disability

Reduction of activities and of work. Pathology

Unsettled.

Differential Diagnosis

From delusional and conversion pains; from muscle spasm provoked by local disease; and from other causes of dysfunction in particular regions, e.g., migraine, post-traumatic headache, cervical spine disorders, depression, hallucinatory headache and conversion hysteria.

Code

X33.X7b Note: (b) coding used to allow the (a) coding to be employed syndrome needs to be specified.

if an acute

Pain of Psychological Origin: Delusional or Hallucinatory Pain (I-16) Definition

Pain of psychological origin and attributed by the patient to a specific delusional cause.

Site

Any part of the body. May be symmetrical, e.g., in a fronto-temporal-occipital ring distribution or in one place, e.g., at vertex, pre-cordial, genital.

Main Features

Rare; estimated to be present in less than 2% of patients with chronic pain without lesions; not apparently reported in children; onset in late adolescence or at any time in adult life; quality may be sensory or affective or both, not necessarily bizzare; essential characteristic is attribution of the pain by the patient to a specific delusional cause, e.g., to a crown of thorns in a patient who had messianic delusions; time pattern in accordance with the delusion; intensity from mild to severe; duration in accordance with the causal psychological illness.

Associated Symptoms and Modifying Factors

May be exacerbated remission of illness.

by psychological

No physical signs or laboratory findings.

stress, relieved by treatment

causing

S46 Complications

In accordance depressive or in established without any schizophrenic

with causal condition; usually lasts for a few weeks in manicschizo-affective psychoses, may be sustained for months or years schizophrenia if resistant to treatment. Occasionally chronic pain formal delusions remits to be succeeded by C>l?aranoid or psychosis.

Social and Physical Disabilities In accordance reported. Aetiology

with the mental state and its consequences.

Manic-depressive,

schizophrenic

Drug addiction not

or possibly other psychoses.

Essential Features Those required for diagnosis are pain, without a lesion or overt physical mechanism and founded upon a delusional or hallucinatory state. Differential Diagnosis From undisclosed or missed lesions in psychotic patients, or migraine, giving rise to delusional misinterpretations; from tension headaches; from hystericai, hy~hondriacal or conversion states. Code

XlX.XYa X = to be completed individually according to circumstances

Pain of Psychobgkal

In e&

case.

Origin: Hysterical or Hypochondriacal (l-16)

Definition

Pain specifically attributable to the thought processes, emotional state or personality of the patient in the absence of an organic or delusional cause or tension mechanism.

Site

May be symmetrical: if lateraiized, possibly more often on the left; precordium, genitals, may be at any single point over the cranium or face, can involve tongue or oral cavity or any other body region. Usually diffuse in fronto-temporo occipital region or in maxillary area.

Main Features

True population prevalence unknown. Frequency Increases from general practice populations to specialized headache or pain clinics or psychiatric departments. Estimates of 11% and 43% have been found in psychiatric departments depending on the sample. Onset may be at any time from childhood onwards but most often in late adolescence. Estimated female to male ratio 2:l or greater .-. particularly if multiple complaints occur. Pain described mostly in simple sensory terms but complex or affective descriptions occur in some cases. Pain is usually continue throughout most of the waking hours but fluctuates somewhat in intensity, does not wake the patient from sleep and usually lasts for more than 6 months.

Associed

Symptoms Loss of function without a physical basis (anesthesia, paralyses, etc.) may be present. Pain is often present in other areas. There may be frequent visits to

s47 physicians to obtain relief despite medical reassurance, excessive use of analgesics, as well as other psychotropic drugs for complaints of depression, neither type of remedy proving effective. The pain may have a symbolic significance e.g., identifying the patient with someone who died of brain tumor. Psychological interpretations are frequently not acceptable to the patient although emotional conflict may have provoked the condition. These patients tend to marry but have poor marital relationships. La Belle Indifference can occur but is not common. Depressive complaints and resentment are more frequent. The personality is often of a de~ndent-hist~oniclabile type. (“Hysterical personality” or “passive dependent personality”). A history of past conversion symptoms is helpful in diagnosis. There are three overlapping types in this category. The first is largely monosymptomatic, - is relatively rare and consists of patients who have pain in one or two regions only, who have only recently developed pain and who have clear evidence of emotional conflicts, perhaps with an associated paralysis or anesthesia, and a relatively good prognosis. Some patients who primarily have a depressive illness also present with pain as the main somatic symptom. Their pain may be interpreted delusionally or may be based on a tension pain, etc., or may be hysterical. The second type is of patients with more numerous or multiple complaints, often of many and varied types without a physical basis. In the history these often number more than 10 including classical conversion or pseudoneurological symptoms (paralyses, weakness, impairment of special senses, difficulty in swallowing, etc.), gastrointestinal, cardiovascular (palpita~ons, shortness of breath), disturbances in sexual function (impaired libido, reduced potency), etc. as well as pains in different parts. In the third or hypochondriacai sub-type the patient presents excessive concern or fear of the symptoms and a conviction that disease is present despite thorough physical examination, appropriate investigation and careful reassurance. There may also be other signs of preoccupation with somatic health, e.g., great anxiety over constipation, the colour of the urine, etc. As emphasized, the sub-types overlap. The the second one described. A hy~hondriac~ alone or with the first or the second sub-type types, physical treatments (manipulation, produce brief improvements which are not types a disorder of emotional development

commonest pattern in pain clinics is pattern may be observed either - more often with the second. In all physiotherapy, surgery) tend to maintained. In the second and third is often present.

Note - Depressive pain has been distributed between the above three types and also into the delusional and tension pain groups. This is done because there does not seem to be a single mechanism for pain associated with depression even though such pain is frequent. The words “depressive pain” as indicating a particular type or mechanism should be avoided. Aggravating Factors Emotional stress may be a predisposing factor and is almost always important in the mono-symptomatic type. Experience of physical illness or pain due to it in person or in a family member or close associate may be a predisposing factor. usual

course

Usually chronic in the first subtype. In relatively acute mon~~ptomatic conditions environmental change and sometimes individu~ psychothera~ may promote recovery.

Complications

Dependence on minor tranquilizers, salicylate addiction. narcotic dctd~c~~on,drug induced confusional states, excessive investigations, unsuccessfui surgery sometimes repeatedly.

Social and Physical Disability Often associated with marital disharmony. Inability to sustain regular employ ment. sometimes loss of function or limbs due to surgery!. Essential Features Pain without adequate organic or pathophysiological explanation. Separate evidence other than the prime complaint to support the view that psychiatric illness is present. Proof of the presence of psychological factors in addition by virtue of at least one of the following: (1) an appropriate relationship in time between the onset or exacerbation of the pain and an emotional conflict or need; (2) the pain enables the individual to avoid some activity that is unwelcome to him or her; and (3) the pain enables the individual to obtain support from the environment that otherwise might not be forthcoming. ‘The condition must not be attributable to any other psychiatnc disorder than the following and it should conform also to the requirements for the diagnoses of hysteria (300.11) or hypochondriasis (300.7) in the International Classification of Diseases, 9th edition (CD.9) or to those for somatization disorder (300.81). or conversion disorder (300.11), psychogenic pain disorder (307.80). or hypochondria& (300.70) in the American Psychiatric Association Diagnostic and Statistical Manual. 3rd edition (DSM-III). Differential Diagnosis (1) From physical causes of pain e.g., tumor, acromegaly, Paget’s disease ot bone, etc; (2) From physical illnesses that may present with multiple. often diffuse symptoms, e.g., hypothyroidism, hyperparathyroidism, disseminated lupus erythematosis, multiple sclerosis, porphyria; (3) From schizophrenia, endogenous depression, reactive depression or major depressive disorder according to DSM-III; and (4) From tension pain particularly headache. The differential diagnosis from tension headache usually will be based on one or more of the following: (a) the level of observed anxiety is not sufficient to account for tension which might produce the symptom. (b) the personality conforms to a hysterical or hypochondriacal pattern and the complaint to an acute conflict situation or to a pattern of multiple symptoms. (e) relaxation exercises and sedation do not provide relief. Code

XlX.X9b

References

International Classification of Diseases, 9th edition, World Health Organization, Geneva, 1977; Diagnostic and Statistical Manual, 3rd edition, American Psychiatric Association, Washington. 1980.

A Note On Factitious IHnessand Mdingering (I-17) Factitious illness is of concern to psychiatrists because both it and malingering are frequently associated with personality disorder. Physicians in any discipline may encounter the problem in differential diagnosis. No coding is given for pain in these circumstances because it will either be induced by physical change or counterfeit. In the first instance it can be coded under the appropriate physical heading. In the second case, the complaint of pain does not represent the presence of pain. The code for factitious illness and malingering in the International Classification of Diseases may be used, viz: -V6!5.2

!349

RELATIVELY LOCALIZED SYNDROMES

GROUP II: NEURALGIAS OF THE HEAD AND FACE

TrigeminaI Neuralgia (Tic Douloureux) (II-l) Definition

Sudden severe brief stabbing recurrent pains in the distribution of one or more branches of the fifth cranial nerve.

Site

Strictly limited to the distribution of the fifth nerve. Usually involves one branch; may involve two. More frequent on the right side.

System

Nervous

Main Features

Prevalence - relatively rare - incidence: men 2.7, women 5.0/100,OOO/annum in U.S.A. Age of onset - after fourth decade, with peak onset fifth and sixth decades; earlier onset does occur, but is extremely uncommon before age 30. Sex Ratio - women affected more commonly than men in ratio of approximately 3:2. Quality - sharp, agonizing electric shock-like stabs of pain felt superficially in the skin or buccal mucosae, triggered by light mechanical contact from a more or less restricted site (trigger point or trigger zone) usually of brief duration - seconds - with repetition in bursts for several seconds to a minute or two, followed by a refractory period of 30 seconds or so up to a few minutes. Time Pattern - episodes may occur at intervals of several or many times daily or in rare instances, succeed one another almost continuously. Periodicity is characteristic, with episodes occurring as described for a few weeks to a month or two, followed by pain free interval of months or years and then recurrence of another bout. Intensity - extremely severe, probably one of the most intense of all acute pains. Usual duration - as described above.

system.

Associated Symptoms

Occasionally a mild flush may be noted during episodes. Precipitated by mechanical stimuli, as noted, but contrary to earlier reports no seasonal predominance. No particular aggravating factors; some relief obtained by firm pressure as with the hands around but not touching the trigger point. Signs and Laboratory Findings

In true trigeminal neuralgia, apart from the trigger point, neurological examination is negative. In symptomatic trigeminal neuralgia areas of hypoaesthesia on the face or absence of the comeal reflex may occur; if sensory deficit is detected a lesion is probably responsible. Usual Course

Recurrent bouts over period of years interspersed asymptomatic phases.

Complications

None

Social and Physical Disability

Only as related to the pain episodes.

with more or less prolonged

Pathology

Summary

When present always involves the peripheral trigeminal (primary afferent) neuron. Impingement on root by vascular loops, etc. appears, to be most common cause. Demyelination and hypermyelination on electron inic-roscopy of Essential Features

and Diagnostic

Criteria

Sudden, transient, intense bouts of superficially located pain, stnctiv confined to the distribution of one or more divisions of the trigeminal nerve usually precipitated by light mechanical activation of a trigger point or area. No sensory or reflex deficit detectable by routine neurologic testing. Differential Diagnosis Must be differentiated from symptomatic tngeminal neuralgia, due to a small tumor such as an epidermoid or small meningioma involving either the root or the ganglion. Sensory and/or reflex deficit in the face may be detected in a significant proportion of such cases. Differential diagnosis between trigeminal neuralgia of mandibular division and glossopharyngeal neuralgia may, in rare instances, be difficult. 006.X8d

Code

Secondary Neuralgia (Trigeminal) From Central Nervous *stem

Lesions (II-2)

Definition

Sudden severe brief stabbing recurrent pains in the distribution of one or more branches of the fifth cranial nerve, attributable to a recognized lesion such as tumour or aneurysm.

Site

Usually limited to distribution of trigeminal nerve

System

Nervous.

Main Features

Prevalence - rare; probably less than 2”?>of cases of tic douloureux. Age of Onset - corresponds to that of appearance of tumours. Sex Ratio - not remarkable. Quality -. paroxysmal pain may be indistinguishable from “true” tic douloureux. Non-paroxysmal pain of dull or more constant type may occur. 7ime Pattern - may mimic tic douloureux; progression and constancy of non-paroxysmal pain. Intensity --. may be as severe as in tic douloureux. Usual Duration ..- indeterminate.

Si

and Laboratory

Findings

Sensory changes (hypoaesthesia) or loss of cornea1 reflex may be noted. Motor deficit is difficult to detect until late phase. X-ray and CAT scan may reveal mass lesion in Meckel’s cave or in pontine cistern. usual

course

Complications

Progression,

usually very gradual

Related to location of tumour.

s51 Social and Physical Disability Related to painful episodes and neurologic deficit when present. Pathology

Summary

Meningioma of Meckel’s cave, epidermoid cyst and less frequently vascular malformation (arterio-venous aneurysm or tortuous basilar artery) of cerebellopontine angle, are the most frequently cited causes of this rare condition. of Essential Features and Diagnostic Criteria Paroxysmal neuralgia in trigeminal distribution with one or more atypical features such as hypoaesthesia or depression of cornea1 reflex.

Differential Diagnosis With “essential” trigeminal neuralgia. Code

Secondary

006.X4 (tumour) 006.X0 (aneurysm)

Trigeminal

Neuralgia From Facial Trauma (II-3)

Definition

Chronic throbbing or burning pain with paroxysmal exacerbations distribution of a peripheral trigeminal nerve subsequent to injury.

Site

Maxillofacial region.

System

Nervous system.

Main Features

Prevalence - 5-10% following facial fractures; common after reconstructive orthognathic surgery; l-5% after removal of impacted teeth. Quality - biphasic with sharp, triggered paroxysms and dull throbbing or burning background pain. Occurrence constant with triggered episodes. Intensity - moderate. Duration - constant.

Signs

Tender palpable nodules over peripheral nerves; neurotrophic

Usual Course

Progressive for 6 months then stable until treated with microsurgery, graft-repair reanastomosis; transcutaneous stimulation and anti-convulsant pharmacotherapy.

in the

effects.

Social and Physical Disabilities Impaired mastication and speech. Pathology

Neuromata;

deafferentation,

hypersensitivity.

Differential Diagnosis Idiopathic trigeminal neuralgia, secondary trigeminal neuralgia from intracranial lesions, post-herpetic neuralgia, odontalgia, musculoskeletal pain. Code

006.x1

Acute Herpes Zoster (Trigemind) (H-4) Definition

Pain associated with acute herpetic lestons in the distribution branches of the 5th cranial nerve.

Site

Face. Pain limited to distribution of trigeminal nerve (1st divIslon1

System

I‘rigeminal nerve.

Main Features

Prevalence -- in~equent. Age 01 Onset -. adults, more common 111mIddIe and old age. Sex Ratio --- not remarkable. Quality - burning, tiering pain with occasional lancinating components felt in the skin. fime Pattern ...- pain usually precedes the onset of herpetic eruption by one or two days (pre-herpetic severe. neuralgia); may develop coincident with or after eruption. Intensitv Usual Duration -- one to several weeks.

Associated

OI ri branch or

Symptoms May be general malaise, low fever, headaches.

S@LS and Laboratory

Findings

Clusters of smafl cutaneous vesicles, almost invariably in the ~st~bu~n of the ophthalmic distribution of the trigeminal. Frequently associated with lymphoma in treatment. Uevated protein and pieiocytosis in spinal fluid. usual

Course

Complications So&l

Spontaneous and permanent remission. In the older age group, progression chronic (postherpetic) neuralgia is not uncommon.

to

Corwal ulceration due to vesicles has been reported.

and Physical Disabiii Related to cosmetic aspects and to pain.

Pathology Summary

Small cell infiltrates in affected skin and builous cutaneous infiltrates in ganglion and root entry zone. of Essential Features

and Diagnostic

changes.

Similar

Criteria

Herpetic vesicular eruption in distribution of first division of trigeminai nerve. History of burning pain in the peri-eruptive period. Differential Diagnosis Syndrome is usually unmistakable. Often related to impaired resistance, e.g., in the elderly or in the presence of carcinomatous metastases. Code

002.xza

s53

Post-Herpetic Neuralgia (Trigeminal) (11-5) Definition

Chronic pain with skin changes in the distribution of one or more roots of the fifth cranial nerve subsequent to acute Herpes Zoster.

Site

Face. Usually distribution of first (ophthalmic) division.

System

Trigeminal nerve.

Main Features

Prevalence - relatively infrequent. Age of Onset - 6th and later decades. Sex Ratio - more common in males. Quality - burning, tearing, itching dysaesthesia and crawling dysaesthesiae in skin of affected area. Exacerbated by mechanical contact. Time Pattern - Constantly present with exacerbations. May last for years but spontaneous subsidence is not uncommon. Intensity usually moderate, but constancy and intractability in many instances, contribute to intolerable nature of complaint. Usual Duration - months to years.

Associated Symptoms Depression,

irritability

Signs and Laboratory Findings Cutaneous scarring, loss of normal pigmentation in area of earlier herpetic eruption. Hypoaesthesia to touch, hypoafgesia, hyperaesthesia to touch and hyperpathia may occur. Usual Course

Chronic, intractable, may last for years. Some cases “burn out” spontaneously.

Complications

None.

Social and Physical Disability Severe impairment of most or all social activities due to constant pain. Suicide occasionally. Pathology

Loss of many large fibres in affected sensory nerve. Chronic inflammatory changes in trigeminal ganglion and demyelination in root entry zone.

Summary of EssentialFeatures and Diagnostic Criteria Chronic burning, dysaesthesias, paraesthesias and intractable cutaneous pain in distribution of the ophthalmic division of the trigeminal associated with cutaneous scarring and history of herpetic eruption in an elderly patient. DifferentialDiagnosis The syndrome is usually characteristic. Other conditions, carcinoma under treatment may promote its occurrence. Code

003.X2b

e.g., metastatic

~kiculate

Neuralgia

(VIIth Cranial

Nerve): Ramsay

Hunt Syndrome

Definition

Severe lancinating pains felt deeply in external auditory canal subsequent to an attack of acute herpes zoster.

Site

External auditory

System

The sensory fibres of the facial nerve.

Main Features

Prevalence - extremely rare; few cases in world literature. Sex ratio - no data. Qualify - sharp, lancinating, shock-like pains felt deeply in external auditory canal. Intensity - severe.

Signs and Laboratory

meatus with retroauricular

radiation.

Findings

Usually follows an eruption of herpetic vesicles which appear in the concha and over the mastoid.

Complications

None.

Social and Physical Dibilii Only as related to the pain episodes.

Patholosy Summary

No reported case with pathological examination. of Essential Features

and Diagnostic

Criteria

Onset of lancinating pain in external meatus several days to a week or so after herpetic eruption on concha. Differential

Diagnosis Differentiate from otic variety of glossopharyngeal herpetic prodromata.

Code

006x2

neuralgia which does not have

~lo~opha~n~e~

Neuralgia (IXth Cranial Nerve) (II-7)

Definition

Sudden severe brief stabbing glossopharyngeal nerve.

Site

Tonsiltar fossa and adjacent area of fauces. Radiation to external auditory canal (otic variety) or to neck (cervical variety).

System

Peripheral and central mechanisms

Main Features

incidence - 0.5/l~,~/annum in U.S.A. Sharp, stabbing bouts of severe pain, often triggered by mechanical contact with faucial area on one side, also by swallowing and by ingestion of cold or acid fluids. Prevalence - very rare. Quality - sharp, stabbing bursts of high intensity pain, felt deep in throat or ear. Time Pattern - episodic bouts occurring spontaneously several times daily or triggered by any of above mentioned stimuli. Intensiiy - very severe, interferes with eating. Usual Duration - episodes last fcr weeks to a month or two and subside spontaneously. Tendency to recurrence is common.

Associated

recurrent

pains

in the distribution

involving glossopharyngeal

of the

nerve fibres.

Symptoms Cardiac a~h~hmia

Signs and Laboratory

and syncope may occur during paroxysms in some cases.

Findings

The important and only sign is the presence of a trigger point, usually on fauces or tonsil; may be absent. Usual Course

Fluctuating; bouts of pain interspersed

Complications

Transitory cardiac arrythmias, as noted.

by prolonged asymptomatic

periods.

Social and Physical Disability Only as related to pain episodes. Pathology Summary

Unknown. Vascular loops impinging on roots may be a cause. of Essential Features

and Diagnostic Criteria

Paroxysmal bursts of sharp, lancinating pain, spontaneous or evoked by mechanical stimulation of tonsillar area, often with radiation to external ear or to angle of jaw and adjacent neck. Application of local anaesthetic to tonsil (or trigger point) relieves pain. Differential Diagnosis Usually characteristic syndrome. limited to mandibular division. Code

006.X8b

May be confused with trigeminal neuralgia

Neuralgia of the Superior Laryngeal Nerve (Vagus Nerve Neuralgia) (11-8) ~finition

Paroxysms of unilateral lancinating pain radiating from the side 01 the thyrcnd cartilage or pyriform sinus, to the angle of the jaw and occasionally to the ear.

Site

Unilateral, possibly more on the left in the neck from the side of the thyroid cartilage or pyriform sinus to the angle of the jaw and occasionally to the ear.

System

Nervous system.

Main Features

Rare. May be a variant of glossopharyngeal neuralgia which has ah been called vagoglossopharyngeal neuralgia. Combined ratio of vagoglossopharyngeal neuralgia to trigeminal neuralgia is about 130. Sex ratio about equal. Quality usually severe, lancinating pain often precipitated by talking, swallowing, coughing, yawning or stimulation of the nerve at its point of entrance into the larynx. Mild forms do occur.

Associated

Symptoms Local tenderness. Possibly autonomic possibly tinnitus and vertigo.

Signs Laboratory

phenomena,

e.g., salivation. flushing;

Presence of a trigger zone. Findings None.

Relief

Relief from: analgesic

Compliitions

Loss of weight.

nerve block, alcohol nerve block or nerve section.

Social and Physical Disability As related to pain episodes. Pathokqy

There may be a history of local infection. A large styloid process or calcified stylohyoid ligament may be contributory (cf. Eagle’s Syndrome).

Essential Features Sudden attacks of unilateral lancinating pain in the area of the thyroid cartilage radiating to the angle of the jaw and occasionally to the ear. Differential l&gnosii Glossopharyngeal Code

006x8~

neuralgia; carotidynia; local lesions, e.g., carcinoma.

Occipital Neuralgia (11-9) Definition

Pain, usually deep and aching, in the distribution of the IInd cervical dorsal root.

Site

Suboccipital area, unilateral in the IInd cervical root distribution from occiput to vertex. May radiate stilt further forward, see below.

System

Nervous.

Main Features

Prevalence - Quite common; no epidemiological data; most often follows acceleration/deceleration injuries. Age of Onset - from second decade to old age. More common in 3rd to 5th decades. Sex Ratio - women more frequently affected, but statistical data lacking. Quality - deep, aching, pressure pain in suboccipital area, sometimes stabbing also. Unilateral usually; may radiate toward vertex or to onto-orbital area and/or face. Time Pattern - Irregular, usually worse later in day. ~nfe~i~ - from moderate to severe.

Associated

Symptoms Hyperaesthesia of scalp. A variety of symptoms such as vertigo, tinnitus, tears, etc., have been described in some cases, but probably these are transitional forms to cluster headache. Nerve block may give effective relief.

Signs and Laboratory

Findings

Diminished sensation to pin prick in area of C2 and tenderness nerve may be found. Usual Course

Chronic, recurrent episodes. May cease spontaneously

Complications

None.

of great occipital

on occasion.

Social and Physical Disabiiity Only as related to pain episodes. Pathology Summary

Unknown. Perhaps reIated to increased muscle activity in cervical muscles. May be secondary to trauma, including flexion-extension (whiplash) injury. of Essential Features

and Diagnostic Criteria

Intermittent episodes of deep, aching, and sometimes stabbing pain in suboccipital area on one side. Marked tendency to chronicity. Often associated with tender posterior cervical muscles. Can be bilateral. Differential Diagnosis Cluster headaches, posterior fossa and high cervical tumour, herniated cervical disc, uncomplicated flexion-extension injury. Metastatic neoplasm at the base of the skull. Code or References

004.X8 004.X1 if subsequent

to trauma

Behrman, S. h-2. med. J, 286 (1983) 1312-1313.

!358

GROUP IIk CRANIOFACIAL PAIN OF MUSCULOSKELETAL ORIGIN

Acute Tension Headache (III-l) Definition

Acute, continuous unilateral or diffuse head pain related to anxiety, depression or emotional tension.

Main Features

As for presumed chronic tension headache except as follows: Very frequent. Affects the majority of the population. Sex ratio probably equal. Acute, selflimiting, repeated separate attacks, relatively short-lasting (for a few hours or less). Dull with gradual onset, steady rise, plateau and then falling in intensity and less throbbing. Sometimes severe. No nausea, vomiting or photophobia or, usually, any complications.

Code

034.X7a

Tension Headache: Chronic Form (Scalp Muscle Contraction Headache) (III-2) Definition

Virtually continuous head pain, usually symmetricat, associated with muscle tension, anxiety and “depression”. Exacerbations with increased pounding headache, nausea and vomiting are typical; usually responds to measures for treatment of anxiety and depression, including particularly antidepressant medication.

Site

Frontal, fronto-occipital, occipital, orbital, or whole scalparea, nuchal. Diffuse or band-like, usually bilateral, sometimes more on one side during exacerbations.

System

Apparently musculoskeletal. some opinions.

Main Features

Prevalence - often diagnosed: even approximate prevalence is unknown. Onset - from age 8 onwards, usually before age 30. Females more than males. Ratio 4:l in those who consult doctors. Start - gradual emergence intermittent at first, as mild diffuse ache or unpleasant feelii, increasing to a definite pain part of the time. Fluctuation during the day is typical. These exacerbations seem to emerge after several years of lesser headache. Occurrence and ~urafion ---most days per week, usually every day for most of the day. Occasionally in longstanding severe cases pain may wake the patient from sleep. Quality - dull ache, usually does not throb, severe during exacerbations often or almost always with throbbing. Some describe tight bands or gripping headache. They may be a minority. Others desc&e pressure sensations. PrecWunts and Exacerbating Factors - emotional stress, anxiety and depression, physical exercise, alcohol.

Vessels or central nervous system according to

s59 Associated

Symptoms Many patients have anxiety, depression or both. Nausea, vomiting and photophobia and phonophobia during exacerbations. Vomiting is however unusual. Irritability.

Signs

Muscle tenderness normals.

Relief

Resoiution or treatment of emotional problems, anxiety or depression often diminishes symptoms. Relaxation treatment helps. Anxiolytics may help, but should be avoided since some patients become depressed and others develop dependence. Tricyclic antidepressants are frequently very useful. Analgesics only help a little.

Complications

Analgesic, narcotic and other drug abuse.

occurs but may also be found in other conditions

and in

Social and Physical Disability Reduction of activities and of work. Pathology

Unsettled. Good evidence of chronic muscle tension is often only found on E.M.G. in patients who also have migraine.

Differential Diagnosis Multiple attacks of acute tension headache, common migraine, “mixed vascular tension headache”, refractive erros and heterophoria (“eye strain”), posttraumatic headache, cervical spine disorders, depression, conversion hysteria and hallucinatory headache. Code

033.X7b

Temporomandibular Pain and Dysfunction (alsocalled Myofascial Pain Dysfunction Syndrome)

Syndrome

(III-3)

Definition

Aching in the muscles of mastication plus, in some cases, an occasional brief severe pain on chewing, possibly leading to restricted jaw movement.

Site

Preauricular,

System

Musculoskelet~.

Main Features

Prevalence - young adult, middle-upper class; there may be a history of mild trauma to face or jaws; history of recent psychosocial stress common. Age of Onset - most often 15-35 years; range 5-55 years. Sex Ratio - strong female predilection. Start - evoked by mandibular opening, teeth clenching or occurs spontaneously during sleep or with stress. Qua&y - cramping, locking, penetrating, aching. Occurrence - episodic, with mandibular function, neck flexion andspontaneously. &en&y -variable from dull ache to severe distress. Duration - minutes to hours, sometimes persisting for years.

temporal, zygomatic, occipital, tem~rorn~~bu1~.

Associated

Symptoms Clicking or popping of temporomandibular joint, tinnitus, rarely Iaad auricuiar paraesthesias, occasional depression or anxiety; relief by lid anaesthetic injection of trigger zones. May also be associated with other facial pains, e.g. atypical odontalgia, pain of psychological origin.

Signs

Varying malocclusion, restricted mandibular opening, temporomandibular crepitus, subluxation. tenderness on palpation of muscles of mastication.

Laboratory

Joint

Findings Elevated electromyograph voltage in masticator-y muscles with trigger zones. increased “silent pericd” of the masseteric chin tap reflex.

Radiographic Findings Normal temporomandibular joint radiographic structure, variable meniscus displacement with arthrography. Occasional pre-senile osteo-arthrosis. usual

course

Complications

Socii

Variable, dependent on resolution of complex multiple factors of trauma, myofascial trigger points, and psychosocial stress response. Often responds to benign physical measures including bite guards and reassurance, and to counselling. Possible degenerative joint disease with long duration iatrogenic medical-surgical disease.

of trauma; secondary

and Physical Disabiii Interference with mastication, social, vocational and sexual activity, development of secondary psychological changes.

Pathology Summary

Unknown. of Essential Features

and Diagnostic

Criteria

Muscle tenderness; temporomandibular joint clicking; mandibular dysfunction; high psychophysiologic stress response. Dull ache with severe exacerbations. Frequently long standing. Associated with ttismus, clicking, lo&ii of the joint and bruxism. Related to adverse life events. Association with malocclusion unproven. Differential fliagnwis Degenerative joint disease, rheumatoid arthritis, traumatic arthralgia, temporal arteritis, otitis media, parotitis, mandibular osteomyelitis, stylohyoid process syndrome, dea&rentation pains, pain of psychological origin. Code

c!34.x8a

References

Feinmann, C., Harris, M., Cawley, R.M. Psychogenic and treatment. BnI. Med. J., 288 (1984) 436438.

facial pain. Presentation

Greene, C.S., Laskin, D.M. Split therapy for the myofascial w-dysfunction (MPD) syndrome: a comparative study. J. Am. Dent. Assoc., 84 (1972) 624-628. Griffiths, R.H. Report of the president’s conference on the examination diagnosis and management of temporomandibular disorders. J. Am. Denf. Assoc. 166 (1983) 7577.

!361

Osteoarthritis of the Temporomandibular Joint (111-4) Definition

Degenerative change, possibly leading to the masticatory dysfunction syndrome and associated pain.

Site

Preauricular area, jaws and face.

System

Musculoskeletal.

Main Features

Preualence - sometimes in patients with histories of acute jaw trauma or chronic traumatic jaw habits; Age of Onset - 40-50 years; Sex - strong female predilection. Start - pain with sustained mastication, speaking-singing, cold weather, palpation condylar head and lateral pterygoid muscle; Quality aching, deep; Occurrence - cyclical with remissions of months’ duration; Intensity - mild A.M., moderate-severe P.M.; Duration - days to years.

Signs

Crepitus, trismus, malocclusion, tenderness

of condyles.

Laboratory and RadiologicalFindings Early sign is flattening of condyle head. Intraarticular lipping, subarticular cavitations and radiolucencies.

osteophytes,

condylar

Usual Course

Progressive, episodic pain and condylar deformation of years’ duration, partially responsive to mechanical-surgical therapies with recurrence likely. Self-limiting.

Relief

Immobilizing joint, anesthetic injections joint capsule, acetylsalicylicacid.

Complications

None.

Social and Physical Diibility Masticatory and speech-singing Pathology

restrictions.

Primary articular surface erosion with secondary calcifications; associated articular disc perforations.

joint space

dystrophic

Diagnostic Criteria Radiographic condylar lucencies, tipping, osteophytes, arthrographic perforations, clinical evidence of chronic masticatory trauma. DifferentialDiagnosis Rheumatoid arthritis, traumatic arthritis, myofacial pain dysfunction. Code

033.X6

meniscus

Rheumatoid

Arthritis of the Temporo~

Joint (H-5)

Definition

Part of the systemic disorder of rheumatoid proliferating onto the articular surface.

Site

Temporomandbular

System

Musculoskeletal.

MainFeatures

Prevalence - Caucasian, approximately 50% occurrence with general rheumatoid arthritis; Age ofonset- juvenile or pubertal; adult onset 40-60 years; Sex -. female predfiection; Start - spontaneous onset; Quo/& - boring, penetrating, aching; Ocamence - constant with diurnal variation; Intensity -moderate A.M., mild P.M.; Duration - minutes to hours.

arthritis with granulation

tissue

joint, external acoustic meatus.

Preauricular etythema, crepitus, tenderness external acoustic meatus, restriction and deformation of other joints, open bite eventually. Laboratory and Radbbgd

Findings

Positive latex fixation; radiographic joint space narrowing. usual

course

5-9 months acute painful course follaved by joint restriction responsive to condyloplasty without recurrence.

Relief

Heat, joint physiotherapy,

Complications

Fibrous or bony ankylosis.

anti-inflammatory

and ankylosis;

agents.

Social and Physical Disabiii Mastication impairment; Pathology

D&no!&

associated orthopedic restrictions.

Synovitis, foam cell degeneration (“Pannus Cell” formation), secondary resorp tion of the attic&r surfaces, adhesiins to the articular disc, fibrous adhesiins narrowing and loss of joint space. criteria Multiple joint involvement, tion, positive lab findings.

radiographic joint space loss, and condylar deforrna-

DifferentialDbgnosii Includes degenerative joint disease, traumatic myofascial pain dysfunction. Code

@32.X3b

arthritis, inflammatory

arthritis,

S63

GROUP IV: LESIONS OF THE EAR, NOSE AND ORAL CAVITY Maxillary Sinusitis (IV-l) Definition

Constant burning pain with zygomatic and dental tenderness of the maxillary sinus.

Site

Upper cheek and sometimes teeth.

System

Respiratory.

Main Features

Prevalence -

from inflammation

common; Age of onset - usually adults; Sex - no difference; Or-set - spontaneous; Quality - dull ache, unilaterally or bilaterally, sense of fullness in face. Occurrence - usually associated with nasal cold. Other nasal disease, dental disease (20% of cases); Intensity - mild to severe; Duration days. Zygomatic area of face may be slightly flushed and slightly swollen (“puffy”). Pain exacerbated by bending. Tenderness of upper molar and pre-molar teeth.

Laboratory

Findings Radiography may show fluid level.

Usual course

Subsides in several days to a week.

Relief

Analgesics, sometimes with drainage by lying down on the opposite side.

Pathology

Inflammation of the lining of the maxillary sinus(

Differential Diagnosis Periapical disease of the associated teeth, malignant disease. Code

031.X2a

Odontalgkx Definition

Toothache

1. Due To Dentino-Enamel

Defects

(W-2)

Short lasting diffuse ore-facial pain due to dentinoenamel defects and evoked by local stimuli.

Site

Orofacial pain.

System

Musculoskeletal

Main Features

Preualence

SP=

Dental caries, fracture, crack or lost restoration.

Laboratoly

-. extremely common; Age of onset - 2 years of age to any age; Sex --.- no difference; Start -.- stimulus evoked, not spontaneous. heat, cold, mechanical, osmotic; Quality -- bright to dull; Occurrence ---- intermittent; Intensity - mild to moderate; Duration - less than a second to minutes.

and Rarlidogical

Fiings

Radiographic evidence of caries. usual course

If neglected, there may be mineralisation within the dentine resulting in less frequent pain, or no pain; or pulpal involvement.

Relief

By protecting

Complications

Laceration of soft tissues by sharp edge of tooth.

Patho&

Dental caries, trauma, operative procedures.

Diiic

defective area with a dressing or restoration.

Criteria Visually observed defects, or defects palpated with a probe, plus radiographic

examination. Differential D&n&s Pulp disease, p&apical disease Code

034Xb

s65

Odontalgia: Toothache 2. Pulpitis

(IV-3)

Definition

Orofacial pain due to pulpal inflammation, often evoked by local stimuli.

Site

Face, jaw, mouth.

System

Musculoskeletal.

Main Features

Preucrlence - very common; Age of onset - after eruption of teeth; Sex - no difference; Start - milder cases may be started by hot or cold stimuli. In severe cases may be spontaneous (no external st~ulus needed) but is exacerbated by heat and cold stimuli; Qua&y - sharp or dull ache, may throb; Occurrence with food intake in milder cases. Daily until treated in severe cases; intensity can be moderate, usually severe; Duration -bouts lasting minutes or hours; may continue for days.

Signs

Deep dental caries, seen both directly and on radiography.

Laboratory

and Radiological Findings Radiologic evidence of caries usually extending to pulp chamber.

Usual Course

If untreated the pulp dies and infection spreads to the periapical tissues, producing acute or chronic periapical periodontitis which is likely to be acute but might be chronic. Death of the pulp ends pain from this source, but by then pain may already have started from the acute periapical periodontitis.

Relief

By analgesics, sometimes by cold fluids, extirpation of the dental pulp; extraction of the tooth.

Complications

Spread of infection to the periodontal tissues, jaws, lymph glands.

Pathology

Histopathol~~

Diagnostic

ex~ination

of the pulp reveals acute infl~mation.

Criteria Spontaneous pain confirms. Tooth not tender to percussion unless periodontitis has supervened.

Differential Diagnosis Other forms of dental disease. Rarely can mimic trigeminal neuralgia. Sinusitis, vascular facial pain syndromes. Code

031.xzc

Odontalgia:

Toothache

3. Periapical Periodontitis

and Abscess (IV-4)

Definition

Orofacial pain due to the causes named and having a graduated response to local stimulation.

Site

Face, jaw. mouth.

System

Musculoskeletal.

Main Features

Prevalence - common; Sex - no difference; Start - biting on the tooth makes the pain worse. in milder cases biting can start the pain. In severe cases pain occurs spontaneously; Qua~i~ - sharp, or dull ache, may be throb~ng; Occurrence - with meals in milder cases; da& in severe cases; intensity --moderate to severe; Duration - hours to days. Ten days from onset radiography may show resorption of bone in the periapical area.

Laboratory Findings Various rnicr~r~n~~ usual

course

from the exudate.

If untreated, pain may cease because of drainage but there are, in many cases, recurrences with further attacks of pain. Chronic abscess may atso occur.

Relief

By analgesics, drainage by pulp canal therapy, extraction of the tooth. Abscess may track to mucosal surface.

Compkations

Ceilulitis, facial sinus, iymp~denit~, abscess.

Pathology

Rarefying osteitis about apex of the tooth, abscess formation.

Ditic

criteria Tenderness

DiierenW

of tooth on palpation. Periapical resorption of bone on radiography.

t&nosii Other dental disease.

Code

sinusitis, spread further, including cerebral

031Xd

s67

Odontalgia: Toothache 4. Tooth Pain Not Associated With Lesions (Atypical Odontalgia) (IV-5) Definition

Severe throbbing pain in the tooth without major pathology.

Site

Teeth and gingivae.

System

Musculoskeletal.

Main Features

Female preponderance; adults; teeth hypersensitive to stimuli. Severe throbbing pain in teeth and gingivae usually continuous, may vary from aching mild pain to intense pain, especially with hot or cold stimuli to the teeth. Duration may be from a few minutes to several hours. May be widespread or well localized, frequently precipitated by a dental procedure. May move from tooth to tooth.

Associated Symptoms Emotional problems. May be associated with hypotensive therapy. Also complaints of temporomandibular pain and dysfunction syndrome, oral dysaesthe&a, and pains of psychological origin. May be a symptom of depressive or monosymptomatic hypochondriacal psychosis. Often excessive concern with oral hygiene. Signs and Laboratory Findings Teeth hypersensitive

to heat and cold.

Relief

Antidepressants. Small doses of phenothiazines. unnecessary pulp extirpations and extractions.

Pathology

Possibly hyperalgesia of pulp and periodontal pain receptors due to persistent vasodilation.

Counselling;

avoidance

of

Summary of EssentialFeatures and Diagnostic Criteria Continuous throbbing pain in the tooth, hypersensitive pressure. No organic pathology.

to temperature

and

Diagnostic Criteria Patient with history of tooth pain associated with endodontic therapy and/or extractions. Remaining teeth clinically sound and vital are tender to thermal stimuli and to percussion. Code

034.X8b

References

Harris, M., Rees, R.T. Atypical odontalgia. &it. J. OralSurg., 16 (1979) 212-218. Brooke, R.I. Atypical odontalgia. Oral Surg., 49 (1980) 196199. Marbach, J.J. Phantom tooth pain. J. Endodontics, 4 (1978) 362-372.

Gkwodynb and Sore Mouth (also known as Burning Tongue or Oral Dysaesthesia) (IV-6) Definition

Burning pain in the tongue from any cause.

Site

Most often tip and lateral borders of glossal mucosa. Palate and lips are often involved and sometimes other buccal mucosa; sometimes even the latter alone.

System

Cutaneous

Main Feature-s

Preualence - common; Age of onset - mainly over 50 years 01 age; Sex -women predominate; Quality - burning, superficial pain; may be throbbing; Time Pattern recurrent and variable, persisting for 5-10 days at a time or Intensity

continuously; Associated

-

usually

mild, sometimes very severe.

Symptoms Taste is often subjectively altered, some patients are uncomfortable with their bite. Aggravated by local surface irritants or sometimes hot foods. Some patients are overtly depressed. Topical anaesthetic applied to the site of pain arrests it. Temporary relief by food and drink is almost pathognomonic. Denture intolerance and cancer phobia are common features.

Signs and Laboratory

Fii

Some patients are anaemic or have low serum iron, or Bl2 or fofate deficiency. Some have impairments of taste on threshold testing. usual

course

Months or years, and may be intractable. May respond to antidepressant or calcium antagonists.

Complications

Secondary

emotional changes

Pathology

Unknown, events.

but clinical presentation

Summary

of Essential Features

and Diagnostic

drugs

bears a strong relationship to adverse life

Criteria

Burning tongue, odd taste, dry mouth, uncomfortable

bite, denture intolerance.

Differential Diagnosis Pain referred to the tongue. Neuritic pains in the tongue. Phantom bte. Erosive lichen planus. Undiagnosed malignancy. Code References

041.X5 (if known) 041.X8 (alternative) Feinmann, C., Harris, M. Psychogenic facial pain: The clinical presentation.

&if.

Dent. J., 156 (1984) 165185. Marbach, J.J. “Phantom Bite” classification and treatment. J. Pro&ret. Dent., 49 ( 1984) 556559.

S69

Cracked Tooth Syndrome (IV-7) Definition

Brief, sharp pain in a tooth, often not understood tooth.

Site

Mouth.

System

Musculoskeletal.

Main Features

Prevalence - fairly common; Age of onset - third decade onwards; Sex - no difference; Start - brief pain on biting or chewing; Qua&y - sharp; Intensity -moderate; Duration - few seconds.

Signs

It may be a visible crack. Percussion of this cusp provokes the pain. The cusp might move away from the tooth when manipulated.

usual course

The pain recurs completely.

Relief

It is relieved when the cracked portion of the tooth finally fractures off, or if the crack is detected by the dentist and the defective portion is restored.

Complications

None.

until a piece fractures off the

with biting and chewing until the cusp finally separates

Social and Physical Disability Eating is more difficult. Pathology

A crack in the tooth allows chemicals and microorganisms to enter and make the dentine at the pulpal side of the crack hypersensitive, possibly by a mild underlying pulpitis.

Diagnostic Criteria A sharp brief pain on biting or chewing. There is pain on percussing the affected cusp but not the other cusps. The piece finally fractures off. DifferentialDiagnosis Other forms of toothache mainly from the dentine and the pulp. Code

034.x1

s70 Dry

Socket (W-8)

Definition

Unilateral pain in the jaw, usually lower, usually associated with additional tenderness due to submandibular lymphadenitis following dental extraction and due to a localized osteitis.

Site

Face, jaw, mouth, upper neck.

System

Musculoskeletal.

Main Features

Prewrlence - fairly common; Age ofonset- any age from when the teeth can be extracted; Sex - no difference; Star-f - - 2 days after a dental extraction, the pain starts without stimulation. The submandibular lymph &nds soon become involved with added tenderness; Quo&y - constant, dull ache, may throb, associated with severe halitosis; Intensity -. moderate, exacerbated by mechanical stimulation; Duration -- hours to days. A recent extraction socket with no clot (and therefore dry), with food debris.

Laboratory and Radiok&al

Find&s

Recent empty tooth socket. usual

course

Continuous

unless treated. Gingiva tends to grow over the socket.

Relief

It is relieved by washing out the socket and packing it with ribbon gauze covered with Whitehead’s varnish (an iodoform resinous material).

Complications

Submandibular

lymphadenitis.

Social and Physical Disabiii Severe halitosis.

Pathology Diagnostic

Localized osteitis. criteria Continuous ache which starts Zdays after tooth extraction. Socket not closed by blood clot. Food debris within. Halitosis. Pain from mechanical stimuli. Submandibular lymphadenitis.

Differential ~nosis Osteomyelitis. Code

031.x1

Retained tooth root.

s71

GROUP V: PRIMARY HEADACHE SYNDROMES Classical Migraine (V-l) Definition

Unilateral, throbbing head pain often with a prodromal state and usually preceded by an aura which is usually visual, nausea, vomiting and photophobia often accompany the pain.

Site

Typically unilateral. Frontal, temporal, occipital, or whole hemicranium. typically changes in different attacks and even during single attacks.

System

Unknown; vascular disturbances have been emphasized; system changes may be fundamental.

Main Features

Prevalence - unsettled: females more than males; Onset -from childhood to about 35. Most by late puberty. Frequent positive family history of headache. Onset of attacks may be associated with emotional stress, relaxation, “anxiety” dietary causes (chocolate, cheese, citrus fruits, etc.), flashing lights, atmospheric changes, etc; Prodromal phase - may last for one to two days; often mood changes, weight gain; Aura - in approximate order of frequency the following occur: blurring of vision, flickering changes in visual field, effects like a curtain or mist in parts of the field, fortification figure-s, scotomata and a variety of other visual changes, paresthesiae, mostly in hand and mouth regions, mild paresis, dysarthria and aphasic disturbances, ailoesthesia, micropsia and macropsia, distortions of perspective. If paresis, hemianopias and sensory loss are prominent, they may be part of other migraine variants (V-3); Duration -usually lo-25 minutes. Aura may occur without subsequent pain. Pain - May overlap with aura or succeed it with or without a symptom free interval, or recur without a preceding aura. Most often pain follows the aura. The pain is throbbing or pulsating, ranges from mild to severe in intensity, reaches a plateau and usually lasts from 4-10 hours if unmodified by drugs but may last up to 24 hours. Frequency - Varies from one or two attacks in a lifetime to several every week for months or years at a time. Most usual pattern is l-4 a month. Exacerbations often occur during episodes of anxiety, depressive illness or personal conflict. There may be fewer attacks in pregnancy. Nausea and vomiting, and photophobia.

central

Side

nervous

Precipitating Factors

Numerous: may include stress, mood changes, relaxation, ingestion of caffeine, tyramine, etc. Signs

None definite except with hemiplegic migraine variant.

Laboratory Findings

Fall in platelet serotonin during attack. Changes in cerebral blood flow. Visual Course

In time inter-paroxysmal headaches attacks after the menopause.

may develop. Some patients have fewer

Social and Physical Disabiii

Interruption Pathology

in work in severe cases. Reduced efficiency for many.

No definite confirmed findings.

S72 Diagnostic

Criteria Focal neurological prodromal symptoms from the aura group, at !east on some occasions, and usually unilateral headache as well as usually anorexia, nausea and vomiting.

Differential Diiosis Common migraine, migraine variants, cerebral angioma, tension headache and headaches of psychological origin. Code

004.X7a

Common Migrak

Note: See note on Cluster Headache

(V-2)

Common migraine has the same characteristics

as classical migraine with the following differences.

Definition

Unilateral, throbbing head pain often with a prodromal state but without a distinct aura, usually accompanied by nausea, vomiting and photophobia.

Main Features

Prevalence - is perhaps higher with the ratio of common migraine to classical migraine 2:l; Aura - is absent. If a patient has had several attacks with an aura but also gets attacks without an aura the latter are still grouped under classical migraine.

Other Features

Common migraine attacks usually last l-3 days but may last longer. The headache may be less frequently unilateral than in classiial migraine.

Code

004.X7b

Note: See note on Cluster Headache.

Migraine Variants (V-3) Hemiplegic Migraine, Migraine Accompagnee, Migraine.

Basilar Migraine. Ophthalmoplegic

Migraine, Retinal

These variants are not described because the neurological signs are still more pronounced than in migraine and the problem of differential diagno& with other headache syndromes is secondary to the question of the nature of the neurological disturbance. Miiaine “Cervicale” is not grouped as a migraine variant because it may not be a true migraine variant but rather headache associated with cervical spine disorders. It is uncertain also if some of the above are separate entities, e.g., retinal migtaine. Differential

Diagnosis For the above syndromes this includes classical and common migraine, angioma, Tolosa-Hunt syndrome and Raeder’s syndrome.

Code

004.X7c For all except migraine cervicale Note: See note on cluster headache

s73

Carotidynia (V-4) Definition

A form of migraine in which the pain occurs in the distribution of the external carotid artery, thus being experienced in the head, face and neck.

Site

Pain in the neck, face and head.

System

As for migraine.

Main Features

Prevalence - occurrence

Associated

unknown. Eleven cases were seen in 9 years in one series (Murray). Another study indicates 8 patients seen over 6 years (Raskin). Quality - The pain is a constant aching or throbbing. Time Pattern - The attacks have a usual duration of 7 to 10 days but may last for weeks, or just a few hours. There is a tendency to recur after a symptom-free interval of weeks or months. Some patients seem to experience only one episode.

Symptoms The usual associated features of migraine with incapacity, nausea, vomiting, photophobia. The carotid artery may be enlarged and tender and pressure over the common carotid artery may reproduce pain in the neck and face. Regional muscles also may be tender.

Relief

Treatment of carotidynia is the same as that for migraine, that is prophylactic drugs such as propranolol, amitriptyline, ergotamine, methysergide, etc. and symptomatic treatment with aspirin/caffeine/codeine preparations or narcotics.

Pathology

Unknown.

Differential Diagnosis Sinusitis, cluster headache. Code

004.X7d

References

Murray, T.J. Carotidynia: a cause of neck and face pain. Can. med. Ass. J., 120, 4 (1979) 441443. Raskin, N.H., Prusiner, S. “Carotidynia”. Neurology (Minneapolis) 27, 1 (1977) 43-46.

Mixed Headache (V-5) Mixed headache probably refers in most cases either to migraine with inter-paroxysmal headache, or to chronic tension headache as described above. The term should therefore be avoided when possible. Code

003.X7b

s74

Cluster Headache (V-6) Definition

Unilateral pain principally in the ocular, frontal and temporal areas recurring in separate bouts with daily attacks for several months, usually with rhinorrhoea or la&nation.

Site

Ocular, frontal, temporal areas; considerably less frequently in infraorbital areas, ipsilateral upper teeth, back of the head, entire hemicranium, neck or shoulder. Unilateral pain is characteristic. The side may change even within a cluster period, but a change of side is rare.

!3ystem

Uncertain.

Main Features

-- approximately 4 per loo0 population. 8590% male. Most frequently headaches start between 18 and 40. Pain --- the pain is constant stabbing, burning or throbbing. Attacks - are grouped in bouts of several weeks to months duration with more or less attack free intervals of some months duration in between. Routs most often last 4-8 weeks. Range 1 week to 12 months. Usually one cluster period per 618 months.

Possibly vascular or autonomic

nervous system.

Occurrence

Usually l-3 attacks lasting from half an hour to 2 hours each per 24 hours in the cluster, frequently at night. Maximum 8 attacksper24 hours. Attacks may skip a day or two or more during the cluster. At maximum the intensity is excruciating. Abortive or mild attacks may nevertheless occur. Patients character&&@ pace the floor, bang their heads against the walls, etc. during attacks and are usually unable to lie down. Associitd

Symptoms

Usually no nausea but some may occur, probably with the more severe attacks or at the peak of attacks. Vomiting is still less frequent than nausea. lpsilateral ptosis/miosis are associated with some attacks; occasionally they persist after attacks and sometimes permanently. IpsiIateral conjunctival injection and la&-nation occur in most patients. Ipsilateral nausea stuffiness and/or rhinorrhea occur in most patients. Hyper-acusis and photophobia occur in many patients. A reduction in heart rate is a feature in some patients, especially during severe attacks. SiS

Ptosis/miosis,

Relief

From ergot preparations,

Patholosy

Unknown

tearing, rhinorrhea and blocked nose, and changes in heart rate. oxygen, prednisone,

methysergide,

etc

Differentid llbgmsii

Code

Sinusitis, chronic paroxysmal syndrome and migraine.

hemicrania, chronic cluster headache, cluster tic

004.X8a

Note: Although cluster headache is grouped with migraine and sin&r disturbances, it is doubtful if vascular disturbances are the source of pain and the second code digit refers to alternative possi bilities for the origin of the pain.

s75

Chronic Paroxysmal Hemicrania (Chronic Stage) (V-7) Definition

Multiple daily attacks of pain usually in females and principally in ocular, frontal and temporal areas by day and night, usually with lacrimation and nasal stuffiness and/or rhinorrhea, with absolute relief from indomethacin.

Site

Ocuiar, frontal, and temporal areas. Occasionally the occipital, ~~~rbit~, aural, mastoid and nuchal areas. Pain may also be felt in the neck, arm and upper part of the chest. Invariably unilateral and the side does not change.

System

Uncertain. Possibly vascular or autonomic nervous system. Central nervous system changes may be fundamental.

Main Features

Rare, 80-90%females, age of onset probably above 20 but pre-CPH stage may appear about time of puberty. Daily attacks at maximum 12 or more per 24 hours, usually 15-30in 24 hours Range l-30 attacks per 24 hours. Patients have attacks every day. Characteristically there is marked fluctuation in the severity of attacks and their frequency. l-2 attacks per day which are barely noticeable may alternate with frequent severe attacks every day thus providing a modified cluster pattern. Attacks last 5-45minutes, maximum 60 minutes, the most usual duration being 1@30minutes. At maximum they are excruciating. Some patients walk around during attacks, other sit quietly, stillothers curl up in bed. The pain is pressing, knifelike, boring, occasionally throbbing. Attacks occur at regutar intervals all through day and night. The patients are awakened by the nocturnal attack.

Associated Symptoms

Nausea is rare and vomiting very rare. Slight ipsilateral ptosis/m~~ may occur during attacks, sometimes also edema of the upper lid. Ipsilateral conjunctival injection and lacrimation probably occurs in most patients and so do ipsilateral nasal stuffiness and/or rhinorrhea. Bradycardia and extrasystoles occur in some patients during severe attacks. Attacks may be precipitated in some cases by bending or rotating the head, p~icul~ly when at the peak of the attack curve. Attacks disappear partly or even completely during the greater part of pregnancy, to reappear immediately postpartum. Signs

Ptosis/miosis, tearing, rhinorrhea and blocked nose.

Relief

Immediate absolute and permanent effect of continuous indomethacin treatment.

Pathology

Unknown.

Diiostic

Criteria

Absolute response to indomethacin. DifferentialDiagnosis

Sinusitis, chronic cluster headache, cluster headache, cluster tic syndrome, milyaine, psychiatric illnesses. Code

006.X&

See note on Cluster Headache

S76

Chronic Cluster Headache (V-8) The features of chronic cluster headache following differences.

are the same as those for cluster headache

with the

Definition

Bouts of unilateral pain usually in males, principally in the ocular, frontal and temporal areas occurring at least twice a week for more than one year.

MainFeatures

Chronic cluster headache is much more rare than cluster headache; the diagnosis requires at least two or more attacks per week over a period of more than one year. The pattern may arise from the beginning as regular attacks of more than two per week or a chronic pattern may supersede an ordinary intermittent cluster headache pattern.

Relief

The same measures are effective as for cluster headache but lithium carbonate tends to work relatively better for chronic cluster headache.

Diierential Diagnosis Sinusitis, chronic paroxysmal drome, migraine. Code

hemicrania,

cluster headache,

cluster tic syn-

Note: See note on cluster headache

004.X8b

Cluster-Tic Syndrome (V-9) Definition

The association of the features of cluster headache and tic-douloureux (trigeminal neuralgia), whether the two entities occur concurrently or separated in time in the same individual.

Site

Pain limited to the head and face. The most common site of pain is the second division of the trigeminal nerve.

System

Nervous

Main Features

Preualence -

system.

rare, there being a total now of only 14 reported cases; Sex Ratio - 7 males, 6 females, in 13 of 14 cases; Age of Onset - the median age in this

series was 44 years, with a range of 28 to 76years; Quo& - Thecombination of cluster headache, either concurrently or separated in time, with sharp, agonizing electric shock-like stabs of pain felt superficially in the skin or buccal mucosa, triggered by light tactile stimuli from a restricted trigger point (the features of trigerninal neuralgia). The features of cluster headache include agoniziigly severe burning or throbbiig pain; Time P&tern - Episodes of briefpains occur many times a day with periods of freedom from pain. The attack is often precipitated by speaking, swallowing, washing the face or shaving. This happens concurrently with, or temporally separated from, the features of cluster headache. The latter comprises severe episodes of steady pain lasting from 10120 minutes frequently occurring at night, chactW occurring in clusters lasting 4-8 weeks, once or twice a year, but at times entering a chronic phase and occurring daily for months, Intensity - extremely severe, that is, both facets of the pain are among the most severe pain experienced.

s77 Associated

Symptoms Prominent autonomic features with the cluster-type pain, that is ipsilateraf nasal obstruction and/or discharge, ipsilateraf lacrimation and conjunctive injection, facial flushing, facial diaphoresis and a behavioural state of agitation.

Signs and Laboratory

Findings

Occasionally the presence of a Horner’s syndrome is noted, presumably as a residuum from the attacks of cluster headache, which are frequently accompanied by the development of a Horner’s syndrome. No sensory deficit present over the face. Usual Course

The attacks of cluster headache and tic-douloureux may occur concurrently and this is most common (8 out of 14 cases), or the attacks of ticdouloureux may precede cluster headache by up to 3 years or as little as 2 months. In only 1 case did attacks of cluster headache clearly precede the later development of ticdouloureux, in that instance by 6 years.

complications

None.

Social and Physical Disabilities Usually profound during the attacks. Pat hology

Unknown.

Treatment

The most successful treatment appears to be the use of carbamazepine and/or baclofen rather than the conventional drugs used for cluster headache.

Differential Diagnosis Sinusitis, chronic paroxysmal hemicrania, migraine. A careful neurological examination and appropriate tests such as CT scans may be necessary to rule out tumours in the cerebella-pontine region. Code

O&.X&l

References

Watson, C.P.N., Evans, R.J.E. Cluster-tic 123-126.

syndrome.

f-lea&r&e,

25 (1985)

Post-Traumatic Headache (V-10) Definition

Continuous or nearly continuous diffusely distributed head pain associated with personality changes involving irritability, loss of concentration ability, dizziness, visual accommodation problems, change in tolerance to ethyl alcohol, loss of libido and depression, and with or without post-traumatic stress disorder and with or without post-traumatic migraine, folfowing head injury.

site

Head.

System

Nervous system.

S78 Main Features

Prevalence- unknown; Onset --- difficult to recognize in children, particularly during rebellious age; Sex ratio - males more than females; Quality nonspecific, generalized, non-throbbing, without aura and without autonomic. dysfunction such as nausea, vomiting or diarrhea; Time pc7fterr: nearly constant; Intensity ~~-mild (relative to migraine) but can be severe.

Associated Symptoms Personality change involving irritability, inability to concentrate on relatively trivial matters such as balancing a chequebook, light-headedness or vertigo, intermittent visual accommodation error, change in tolerance, usually intolerance of ethyl alcohol, loss of libido with or without depression and with or without post-traumatic stress disorder. Signs and L&oratory Findings Any objective abnormality including MMPI changes, EEG abnormalities, clinical convulsions, focal neuroiogic findings and organic brain syndrome usually absent and if present markedly limits the prognosis. Usual Course

Without treatment, weeks to months and, in the presence of focal neurologic abnormalities, convulsions or organic brain syndrome, indefinite.

Complications

Loss of victim’s will to combat the illness

Social and Physical Disabilities At worst, left untreated, loss of gainful employment to the point of complete destitution. Pathology

and family and social status

Disruption of central axons and boutons due to angular positive or negative acceleration of the brain (unproven hypothesis). Damage to labyrinth is often postulated as well, and soft-tissue lesions from cervical sprain syndrome.

DifferentialDiagnosis The word concussion is to be avoided because of lack of agreement in definition of term. Confusion with possibly accompanying depression, post-traumatic stress disorder and other accompanying or complicating “psychiatric” organic brain dysfunction disorders is to be avoided. In the presence of focal neurologic findings, convulsions or organic brain syndrome, it is necessary to rule out subdural haematoma and other space-occupying lesions. It is difficult or impossible to distinguish from tension headache. The spouse or family is much more likely to be aware of the irritability than is the victim. Code

002.x1

References

Brenner, C., Friedman, A.P., Merritt, H.H. and Denny-Brown, traumatic headache. J. Neural., 1 (1944) 379. Merskey, H. Psychiatry and the cervical sprain syndrome. 130 (1984) 11191121 (Editorial).

D.E. Post-

Can. Med. Assoc. J.

Tyler, G.S., NcNeely, H.E. and Dick, M.L. Treatment headache with Amitriptyline. Headache. 20, 4 (1980) 213.

of post-traumatic

Trimble, M.R. Post-traumatic

Chichester,

Neurosis. John Wiley &Sons,

1981.

s79

GROUP VI: PAIN OF PSYCHOLOGICAL IN THE HEAD AND FACE

ORIGIN

As for I-16 with local distribution.

Delusional or Hallucinatory Pain (VI-l) Differential diagnosis from local and general conditions. Code

OlX.X9a

Hysterical or Hypochondriacal Pain (VI-2) Distribution possibly more often on the left, except in cases with lesions or compensation claims. Differential diagnosis from local conditions (see above) and general conditions, e.g., hypothyroidism, polyarthralgia, etc., which cause diffuse symptoms. Code

OlX.X9b

GROUP VII: SUBOCCIPITAL AND CERVICAL MUSCULOSKELETAL DISORDERS

Myofasd Syndrome: Cervical Sprain or Cervical Hyperextension Injury (VII- 1) (‘Whiplash”) Definition

Dull, aching pain, in back of head or neck or both, in an area of reference from one or more muscles of the occiput and neck and with a trigger point usually outside the painful area from hyperextension injury.

Site

Rack of head or neck or both, with spread in area of the affected muscle, e.g.. trapezius. Particularly common just medial to the right or left inferior scapula pole.

System

Musculoskeletal.

Associated

Symptoms The pain is aggravated by motion of the cervical spine, tension, sitting, reading; relieved by mild analgesics, cervical collar, heat; more prolonged symptoms helped by cervical traction, biofeedback. Sometimes there is dizziness and tinnitus. Pain may shift to the axilla, arm, or anterior upper chest, or inferior scapula pole.

Sis

and Laboratory

Findings

Limited motion of cervical spine, especially toward flexion and rotation. Tenderness to palpation in trapezius and posterior cervical spine (diffuse). Muscle spasm and trigger points in one or more muscles of occiput or neck. Prolonged or repetitive use of the shoulder girdle muscles, e.g., carrying dishes or washing them may induce radiation of pain into the upper extremity. Push/pull activities e.g., vacuum cleaning may aggravate pain also. Laboratory findings normal. X-Rays: Normal, although loss of usual cervical lordosis common; lateral flexion/extension views may reveal motion at one or two segments as opposed to all. usual

course

Complications

Gradual complete recovery over a l-3 month period of time. The emphasis is on regaining a normal range of motion concurrently with symptomatic treatment. If the initial symptoms are severe, or premorbid personality disorder exists, the symptoms may persist or even increase. In a group of patients they persist longer than 3 years, even independently of litigation. Reactive depression,

unemployment.

Role changes may occur

Social and Physical Disabiiii Significant stress may be experienced by the patient or family if endurance work is decreased and social and financial problems follow. Pathology

Unknown,

may represent

at

injury to ligamentous tissue and/or skeletal muscle.

S81 Summary

of Essential Features

and Diagnostic Criteria

Posterior/c~ic~/upper trapezius - mild/m~erate pain precipitated by a traumatic event. Differential diagnosis primarily one of excluding disc herniation or cervical fracture. Different~

Diagnosis Degenerative disc; brachial neuritis; cervical disc herniation; pancoast tumour; cervical fracture; infection.

codes

133.x1 233.X1

References

MacNab, I. The whiplash syndrome. C/in Neurosurg,

20 (1973) 232-241.

Mendelson, G. Not “cured by a verdict”. Effect of legal settlement compensation claimants. Med J A&, 2 (1982) 132-134.

on

Merskey, H. (1984) Psychiatry and the cervical sprain syndrome. Can Med Ass

J. 130 (1984) 1119-1121.

Myofascial Syndrome: Sternocleidomastoid Muscle (VII-2) Definition

Continuous aching pain sometimes with autonomic phenomena which may arise from hyperactive trigger points (TP) in either the sternal or clavicular division of the mu&e. Each will produce a different pattern of referred phenomena.

System

Musculoskeletal.

Site

Ste~~leidomastoid

Main Features

Pain is usually described as dull and aching. The associated symptoms vary in intensity from mild to disabling, but generally persist over a period of weeks to months, more intense at times of stress or seeming to recur without a known precipitating cause. Stern~leidom~toid TPs are the most commonly overlooked source of multiple symptoms.

muscle.

Sternal Division: (1) Trigger points (TP) in the lower third may refer continuous dull aching pain to the upper portion of the sternum so that a paroxysmal dry cough will be produced; (2) Trigger points in the middIe level refer pain across the cheek into the maxilla, over the supraorbital ridge, deep into the orbit, sometimes into the external auditory canal. Along the inner margin TP will refer pain to the pharynx and back of tongue (during the act of swallowing) creatingasore throat, and to the tip of the chin; and (3) Trigger points in the upper third refer pain to the occipital ridge behind but not close to the ear, and the vertex of the head along with surrounding scalp tenderness. Chuicuhr Division: (1) Trigger points located in the middle level refer pain to the frontal forehead area which when severe, crosses the midline and becomes bilateral; and (2) Trigger points located in the upper level refer pain ipsilaterally deep into the ear and posterior auricular zone. Occasionally there may be poorly localized pain ipsilaterally in the cheek and molars.

S82 Associated

Symptoms Sternal Division: Autonomic phenomena are experienced in t tw tpsilated eye and nose. Eye symptoms may include: increased lacrimation: conjunctival reddening secondary to vascular engorgement; apparent ptosis due to spasm of the orbicularis oculi muscle such that to look upward the head must be tilted backward; visual disturbances such as blurred vision and dimming of perceived light intensity. Nasal symptoms consist of coryzaand maxillary sinus congestion. Decreased hearing may also occur on the ipsilateral side of the affected muscle. Cluuicubr Division: Autonomic phenomena are experience4 sweating and vasoconstriction in the frontal forehead area.

;ti localized

Proprioceptive disturbances may be numerous and can be more disablrng themselves than the referred pain. Possible symptoms include amongst others: postural dizziness; motion sickness; disequilibrium (sudden falling when bending/stooping and ataxia); occasional vertigo (upon either sudden rotations of the head or following sustained periods wherein the head has been tilted to one side); and in severe cases syncope. Nausea and vomiting may also sometimes occur. These changes create a state wherein there are exaggerated postural responses, e.g., while looking downward the patient falls folutard and while looking up the patient fails backward. Alone or together these disturbances may interfere with daily functions. Satellite adder Fojnfs: Satellite TP are usually found in such nearby muscles as scaleni, tevator scapulae, trapezius and splenius cervicis. In the zone of referred pain, satellite TP may occur in the foflouing muscles: masse&r; temporalis; orbicularis ocuii; and frontalis. Physical Examination: Examination should reveal palpable band(sj. area(s) oi deep tenderness and a local twitch response. Marked limitation of rotation and flexion may occur Pathology

See myofascial pain syndrome,

AetioloSy

SCM trigger points may be activated through mechanical overload in situations where there are protracted periods of neck extension, e.g., overhead work, sports overuse and accidental injury (whipl~h). Other potential acute precipitants include: sleeping on two pillows whereby the neck is flexed, reading in bed with a light placed to one side, cocking the head to avoid glare or improve hearing, compression from a tight collar or necktie.

1-9.

Chronic stress can result from the following: postural stress due to deformity 01 an injury that acts to restrict upper extremity movement. The former may arise from either a short leg or small hemipelvis. The latter would result in awkward compensatory neck positioning. Other sources of chronic overload may arise through paradoxical breathing or chronic cough which would then strain the accessory muscles of respiration. Differential Diagnosis Vascular headache, trigeminal neuralgia, Homer’s syndrome, congenital torticollis, and other neurological disease; Meni&e’s disease, other vestibular disease; non-vestibular sources of dizziness, e.g., internal carotid artery stenosis, hypertension, intracranial tumour/aneurysm, drug side effects, excess ear wax impinging on the temporomandibular joint; and conversion hysteria. Code

132.X7a 232.X7a

933

Myofascial Syndrome: Trapezius Muscle (VII-3) Definition

Dull aching continuous pain arising from hypersensitive trigger points (TP), in the upper, middle or lower portions of the trapezius muscle.

Site

Trapezius muscle.

System

Musculoskeletal.

Main Features

Continuous, dull aching or burning pain with myofascial trigger points in the affected area, often bilateral but more on one side. There are seven potential sites for their development throughout the length of the muscle. There are potentially two TP in each of the upper, middle and lower portions of the muscle. A seventh is also possible though rarely present in the middle portion of the muscle. Upper Trapezius: Trigger points (TP) refer pain unilaterally upward along the posterolateral aspect of the neck to the mastoid process and the postero-inferior area behind the ear. When intense, the pain can extend across to the other side of the head where it concentrates in the temple area. From there, it may travel to the back of the orbit, the angle of the jaw, the lower molar teeth, and the pinna of the ear. Complaints may include any of the following: posterolateral neck pain, temporal headache, a stiff neck, and intolerance to heavy clothing. Middle Trapezia TP here cause local pain or referred pain in the posterior cervical region and the top of the acromion process. Lower Trapezius: TP here cause local pain and refer pain to the following areas: the high cervical region of the paraspinal muscles, adjacent mastoid area, the acromion, and the suprascapular region. In addition, the pain may be referred to the medial border of the scapula.

Associated

Symptoms

Entrapments. (1) Greater OccipitalNerve: Tautness of the trapezius muscle can create a shearing stress that may contribute, but alone be insufficient, to entrap the greater occipital nerve; and (2) Spinal Accessory Nerve: If this is entrapped by sternocleidomastoid muscle fibres, the trapezius muscle will appear weakened upon examination.

Sntelhte Trigger Points: These may develop in temporalis

and occipitalis

muscles. Signs

Standing Posture: The patient may develop an abnormal stance wherein there is bilateral shoulder elevation with a slight tilting of the head towards the more affected side. SeatedPosture: The patient will usually sit with arms crossed and chin cradled. Behavior: The patient may rub the muscle in an attempt to reduce the pain as he may also move his head about in an attempt to stretch out the muscle and thereby reduce the pain.

Pathology

See myofascial pain syndome (I-9).

Aetiology

The antigravity function may be overloaded by any activity that requires the trapezius to carry the weight of the homolateral arm for an extended period of time. A few such examples would be: telephoning, sitting without adequate armrest support, holding the forearms up to reach a high typewriter. Chronic overloads may appear as a cumulative stress from any of the following: clothing and accessories (narrow brassiere straps, shoulder bags, backpacks); activities that involve prolonged periods of shoulder elevation (long telephone calls, playing of the violin); and holding the head far to one side in a fixed position for an

extended period of time. Cervical radiculopathies can, as part of thcrr sequelae. result in the development of TP in the upper trapezius muscle. ~i~~fe Trapezius: This part of the muscle may become overloaded when the arm is held up and forward for extended periods of time. An example of this would be the driving of a car with both hands held gripping the top of the steering wheel creating a round-shouldered posture

Lower Trapezius: ‘This can be overloaded through long periods of nending and reaching forward. An example of such an activity would be sitting with the chin supported by the hand with the elbow resting on the chest because of inadequate armrest support. 132X7b 232.X7b

Code

Stylohyoid Process Syndrome (Eagle’s Syndrome) (W-4) Definition

Pain following trauma in the region of a calcified stylohyoid ligament.

Site

Mandible, floor of mouth, lateral pharynx.

*stem

Musculoskeletai.

Main Features

Prevalence ..- among patients with calcified stylohyoid ligament and history of trauma to mandible and/or neck; Age on onset -- 4050 years; Sex - no predilection; Start evoked by swallowing, opening mandible, turning head toward pain and down, with palpation of stylohyoid ligament; Kanji ---throbbing, deep; Occurrence -- with function; ~nfens~~ -- mild to moderate; Duration -- seconds to minutes.

Associated

Symptoms Dizziness, tenderness

Signs

on palpation of the carotid trunk and branches.

Carotid bruit. transient ischemic episodes

Radiolo&

Findings Calcified stylohyoid process.

usual

course

Compliitions !Gcii

Benign, intractable if styloid process not excised or fractured, partial relief from stellate ganglion local anaesthetic infiltration, and acetyl-salicylic-acid. Secondaty

carotid arteritis and cerebral ischemia.

and Physical Dkabiiity interference

Pathology

with speech and mastication.

Calcified stylohyoid ligament, carotid - external carotid branch arteritis

s85 Summary of EssentialFeatures and Diagnostic Criteria Presence of calcified stylohyoid ligament, tenderness history of trauma.

of superficial vessels,

DifferentialDiagnosis Myofascial pain dysfunction, carotid arteritis, tonsillitis, parotitis, mandibular osteomyelitis. Code

gtossophatyngeal

neuralgia,

036.X6

GROUP VIIr:VISCERAL PAIN IN THE NECK

Carcinoma of Thyroid (VIII-l) Definition

Pain in the thyroid gland, aggravated adherent neoplastic mass.

Site

Throat and anterior neck area, spreading to the ear.

System

Endocrine.

Main Features

Localized sharp or dull, aching or burning, occasionally laryngeal nerve involved.

by palpation

and associated

with an

stabbing if superiir

Associated Symptoms Mass in neck, dysphagia, deposits. Signs

dyspnoea

or stridor, symptoms

Neck swelling; fixation of thyroid; stridor.

Laboratory Findings Cold nodule on scan. Complications

Local -

Code

172.X4

Dysphagia; stridor.

from secondary

Carcinoma of Larynx (VIH-2) Definition

An aching soreness in the throat, aggravated by swallowing with hoarseness and dysphagia.

Site

Larynx and adjoining portions of neck.

System

Respiratory system.

Main Features

Present on swallowing; spreads to ear (otalgia); is moderately severe; late, dull aching, burning in character, occasionally sharp, stabbing or lancinating if superior laryngeal nerve involved.

Associated

Symptoms Hoarseness;

dysphagia, when local spread has occurred.

Signs

Tumour on inspection of larynx.

Complications

Stridor progressing to respiratory obstruction; occurred.

Social and Physical Disability Loss of voice following surgical treatment. Essential Features Persistent hoarseness. Code

122.X4

dysphagia, when local spread has

sa7

Tuberculosis of Larynx (VIII-3) Definition

A painful irritation in the throat on air flow during breathing, swallowing due to tuberculous lesions.

Site

Larynx and adjoining regions of neck.

System

Respiratory system.

Main Features

Now rare. Local in larynx; spreads

Symptoms

Hoarseness;

coughing and

to ear (otalgia); continuous, dull, aching burning; stabbing or lancinating if superior laryngeal nerve involved. Worse on swallowing. MB. In early stage is pain free. cough.

Associated Symptoms

Sputum -

purulent; night sweats and fever; weight loss.

Signs

Inflammation of larynx; ulceration of larynx; chest signs.

Pathology

Infection with mycobacteria

tuberculosis.

Summary of Essential Features and Diagnostic Criteria

Hoarseness in someone with tuberculosis nightsweats and weight loss. Differential Diagnosis

Cancer of larynx. Code

123.X2

of chest,

i.e., cough, sputum,

GROUP D(: PAIN OF NEUROLOGICAL ORIGIN IN NECK, SHOULDER AND UPPER EXTREMiTY Prolapsed Disc (IX-l) Definition

Pain in the distribution of a dermatome

Site

9@h of cases affect C6 or C7 nerve roots. Lesions of C2-C3 affect occipital region; lesions of C3 to C4 - neck and shoulder; lesions C5 to C8 - neck and shoulder and upper limb; with C5 lesions the pain is on the lateral aspect of the shoulder approximately over the deltoid muscle; with C6 lesions the dermatome on the radii side of the arm and forearm downwards to include the thumb is affected; with C7 lesions --. the dermatome on the postero-lateral aspect of the arm from the C6 dermatome to the second and third fingers is affected; with lesions of C8 - the dermatome joins C7 dermatome posteriorly and includes the fifth finger. The commonest sites are from the C5i6 and C7!8 discs. The pain often starts in the neck and irradiation to the upper extremity follows.

System

Peripheral nervous system

Main Features

Prevalence .- common in middle and older age groups, males more than females; often related to injury including repeated minor trauma or to sudden bending or twisting movement. In cases from repeated trauma the onset of pain is gradual and progressive with aching and burning. With acute, usually moderate or worse trauma, the onset is sudden with intense pain radiating in the territory of the affected root. After onset exacerbation follows from some movements of the head or arm and from straining, coughing, etc. Lancinating pains may occur. Time Course - diminution and later recurrence of the intense symptoms; indefinite persistence of pain can occur.

Associated

Laboratow

disc.

Symptoms Headache; feelings of numbness

Signs

due to a prolapsed intervertebral

in affected areas.

Diminution of normal cervical lordotic curve. Neck rigidity, limitation ot extension and of rotation to the defected side. Muscle weakness depending on root(s) affected; hypoaesthesia to touch and noxious stimulation in affected dermatome. Rarely long tract signs, from anterior central protrusion. Biceps jerk diminished or absent (C5, 6). Triceps jerk diminished or absent (C7, 8). Frequently, regional muscle tenderness. Findings On x-ray examination: marked straightening of cervical spine or possible tilt to affected side. Distinct narrowing of intervertebral space. Sliiht or moderate arthritic changes. CSF protein may be increased. Defect may be shown by myelography and the protrusion may be shaun by discography. Thermography - abnormal “thermatome”. Electromyographic evidence of denervation may be present.

s89 Relief

Relief from neck-rest; anti-spasm~ic decompression.

Pathology

Protrusion of portion of annulus fibrosus or rupture and herniation of nucleus pulposus or both. Often there are associated changes of cervical spondylosis.

and anti-inflammato~

drugs; and surgical

Essential Features Segmentai pain aggravated by coughing, sneezing and neck movements and relieved by neck traction. Sensory deficit, and diminished deep tendon reflexes at appropriate levels. Differential Diagnosis Osteo-arthritic changes, fracture, abscess, intraspinal, vertebral and superior pulmonary sulcus tumours, traumatic avulsion of nerve root and brachial plexus lesions, syringomyelia, herpes zoster, tuberculosis, syphilis, and transverse myelitis. Code

203.X6a (arm)

Note Because of similar codings with other syndromes, a, b, or c is used in the sixth digit place to distinguish them from each other.

SW

Osteophyte:

Cervical

Spondylosis

(R-2)

Definition

Pain in the distribution spur.

Site

Occipital region. Neck and one or both upper extremities. Pain of C6 lesions is more anterolateral in the arms and that of C7 lesions more postero-lateral. Frequently associated with regional tenderness and muscle spasm.

System

Peripheral nervous system.

Main Features

Prevalence - common in middle-aged and older men and women, more frequent than cervical disc lesions; Onset - frequently sudden following trauma or slow with sudden exacerbations. Usually stiffness and continuous aching pain in the neck and pain similarly in the arms in the distribution of the dermatome. Exacerbations occur with neck movement and can be accompanied by sharp radicular pain; Course - fluctuating or progressive.

of a dermatome

due to protrusion

of a vertebral bony

Associated Symptoms Numbness, tingling and occasionally weakness of appropriate muscle group(s). Tingling in thumb (C6), middle 3 fingers (C7). Sims

Hypoaesthesia to touch and noxious stimulation. Paravertebral tenderness in region of osteophyte. Muscle spasm, tenderness and occasional relief changes (diminution).

Laboratory Findings Lateral x-ray shows reduction of lordotic curve. Straight x-ray demonstrates osteophyte. Myelogram abnormal Thermogram - abnormal thermatome. Relief

Rest and prolonged traction.

Pathology

Protrusion

of bony spurs into vertebral foramen.

DifferentialDiinosis Ruptured cervical disc, fracture, abscess, intraspinal vertebral and superior pulmonary sulcus tumours, traumatic avulsion of nerve root, trauma, other features of osteoarthritis, herpes zoster, tuberculosis, syphilis, etc. Code

203.X6b

s91

Intra-Spinal Tumour

(1X-3)

Definition

Neck pain or dermatomal pain or both, associated with intra-spinal tumour, aggravated by traction, coughing or sneezing.

Site

Initially in the neck with extension to the upper thoracic region, scapular area, shoulder and upper limb or may begin with pain in the more distal areas and involve the neck later.

System

Nervous system.

Main Features

Prevalence -

Associated

relatively rare; and Pain - dull, local neck pain with or without aching, burning pain along the distribution of the affected nerve roots. Intense root pain may occur with meningiomas or neurinomas. These latter account for more than 50% of tumours at this level. Intensity varies from mild to very severe, usually sustained with exacerbations of jabbing or shooting pain due to sudden increases in CSF pressure, e.g., coughing, sneezing.

Symptoms With intra-spinal extension long tract symptoms appear (especially proprioceptive disturbance, pareses). Loss of feeling and weakness occur in the root distribution.

Signs Laboratory

Long tract signs and reflex changes in the distribution of the affected nerve. Findings X-ray abno~~iti~;

Pathology

myelogram abnormal.

Benign or malignant neoplasm.

Differential Diagnosis Prolapsed intervertebral disc, osteoarthritic changes, fracture, abscess, vertebral and superior pulmonary sulcus tumours, traumatic avulsion of nerve root, herpes zoster, tuberculosis, syphilis, etc. Code

202.X4a

s92

Fracture or Collapse of Cervical Vertebrae (1X-4) Definition

Neck pain and dermatomal

Site

Fractures with mild compression Those which produce severe distribution (radiculalgiaj.

System

Nervous system

Main Features

Onset - slow, progressive, continous, local, dull, aching pain from mild compression. Severe compression produces sudden intense lancinating pain which continues as sharp, burning pain exacerbated by movement.

Associited

pain, or both, associated with fracture of nerve roots produce locallsed cervlcai pawn. compression cause pain also in the root

Symptoms Sensory and/or motor symptoms and/or reflex changes in affected distribution.

Laboratory

Findings X-ray evidence of h-acture and possible dislocation.

Relief

Neck traction.

Pathology

Fracture from injury, neoplasm or decalcification.

Diagnostic

Criteria Pain and signs as described. X-ray evidence of fracture

Differential Diagnosis Prolapsed intervertebral disc, other osteoarthritic changes, abscess, intra-spinal vertebral and superior pulmonary sulcus tumours, traumatic avulsion of ne-rve root, herpes zoster, tuberculosis, syphilis, etc. Code

203.Xlb

s93

Epidural Abscess (1x-5) Definition

Pain at site of infection, exacerbated by movement, with consequent spasm of erector spinae muscles and root pains after 2 to 3 days due to abscess.

Site

Neck and affected dermatome(s).

System

Nervous system.

Main Features

Preualence - rare; Pain - gradual onset, increasing from dull aching to severe burning pain in root distribution (radiculalgia).

Associated

Symptoms Loss of feeling and weakness in affected distribution, pins and needles.

Signs

Laboratoty

Depending on the size and the extent of the abscess, long tract symptoms and signs may appear with proprioceptive disturbance, pareses. Hyposensitivity to pin prick and noxious stimulation, muscle weakness and reflex impairments will occur in the affected distribution. Findings Findings indicating inflammation and straight x-ray evidence of encroachment the spinal canal, abnormal myelogram; abnormal thermogram.

Relief

Radiculalgia may be relieved by traction.

Pathology

Inflammation and abscess formation.

of

Essential Features Pain as described with clinical and laboratory evidence of abscess formation. Differential Diagnosis Ruptured intervertebral disc. Other osteoarthritic changes, fractures, intraspinal, vertebral and superior pulmonary sulcus tumours traumatic avulsion of nerve root, herpes zoster, tuberculosis, syphilis, meningitis, etc. Code

202Xa

s94

Vertebral Tumour (K-6) Definition

Neck pain and tenderness tumour.

Site

Neck and dermatome(s)

system

Nervous system.

Main Features

Prevalence than those -- gradual distribution

Associited

followed by radiculalgia due to primary or metastatic

of shoulder and upper limb.

- both primary and metastatic cervical tumours are less common in thoracic or lumbar region. Usual&~ affects older age groups; Onset onset, increasing from dull aching to severe burning pain in root (radiculalgia).

Symptoms Loss of feeling and weakness in affected distribution, pins and needles. Depending on the size and the extent of the tumour, long tract symptoms and signs may appear with proprioceptive disturbance, pareses. Hyposensitivity to pin prick and noxious stimulation, muscle weakness and reflex impairments will occur in the affected distribution.

Laboratoly

Findings Radiological evidence, usually significant on straight x-ray after onset of pain.

Relief Diignostic

Radiculalgia may be relied

by traction.

Criteria Local neck pain with or without root pain and evidence of neoplastic disease.

Differential

Diagnosis Prolapsed intervertebral dii, other osteoarthritic changes, fracture, abscess, intra-spinal, vertebral and superior pulmonary sulcus tumour, herpes zoster, tuberculosis, syphilis, etc.

Code

202.X4b

595

Herpes Zoster: Acute (IX-T) Definition

Pain associated with acute herpetic lesions in a dermatomal

Site

Dermatome(s)

System

Nervous system; dorsal nerve root ganglion.

Main Features

Prevalence - commoner in the elderly; Onset - mild pins and needles sensation, burning, gradually increasing over 3 to 4 days; moderate to severe continuous burning pain. Pain continues over 2 to 3 weeks and sometimes longer, gradually declining in most cases.

Associated

of neck and limb.

Symptoms Impaired sleep in severe disturbances, pruritis.

Signs Laboratory

distribution.

cases,

chills, fever, ma&se

e.g. ~trointesti~l

Erythema followed by vesicles in affected area, and encrustations white and brown mottled areas.

succeeded by

Findings Thermogram: lesion.

frequently abnormal

heat emission in distribution

of radicuiar

Relief

Avoidance of contact on affected skin.

Pathology

Acute viral inflammation of dorsal root ganglia and dorsal horn cells.

Diagnostic Criteria Pain associated with characteristic Code

203.X2a

eruptions and dermatomai distribution.

S%

Post-Hetpetic

Neuralgia (IX-g)

Definition

Chronic pain with skin changes in a dermatomal herpes zoster.

Site

Dermatome(s)

System

Nervous system.

Main Features

Preualence -- commoner in the elderly and in association with neoplasms; sustained mild to severe pain with burning, sharp, and brief, intense, shooting pains in some cases; other descriptions include twisting, boring, formication, jabbing, buzzing, etc.

Asso&ted

distribution

following acute

of neck and limb.

Symptoms

Dysaesthesiie,

and exacerbation

of pain on contact with clothes.

Scarred, depigmented and mottled white and brown skin in a dermatomal distribution. Hypoaesthesia to touch, hypoalgesia, hyperaesthesia to touch and hyperpathia. Laborato~

Findings Thermogram:

Pathol~

Diagnostic

abnormal thermatome

in dermatomes

affected.

Post inflammatory scarring of affected sensory ganglia. Tne process can extend inward toward the meninges and into the root entry zone. The peripheral nerves are progressively scarred and fibrotic. The larger A fibres suffer more than the small C fibres. Secondary degeneration may occur within the dorsal columns.

criteria Burning pain and hyperaesthesii

COd4?

- heat emissiin

203.X2b

with evidence of skin changes as described.

s97

Syphilis: Tabes Dorsalis and Hypertrophic

Pachymeningitis

(IX-9)

Site

Upper limbs.

System

Nervous system.

Main Features

Very rare. Ordinarily occurs in middle life or later. Tabes classically produces acute severe lancinating pains for a few moments on many days, appearing in clusters. Terms such as crushing, burning, cramping or electric as well as lightning are used. With an associated pachymeningitis the pains may have a radicular distribution with a sustained pressing or cramping quality.

Associated Symptoms Sensory changes as if “walking on cotton-wool”, girdle paraesthesiae dysaesthesiae, hyperaesthesiae of feet or body and ataxia. Signs

or

Impairment of peripheral sensory modalities, ataxia and diminished or absent tendon jerks. Sometimes joint changes. Pupillary changes. Muscle weakness if pachymeningitis occurs.

Laboratory Fiidings kh;b;;+s;ic

colloidal gold test curve (Lange Test). X-ray and myelogram may

Relief

Nil.

Pathology

Extensive and numerous

spinal cord changes related to syphilis.

Diagnostic Criteria Lightning pains plus neurological diagnosis of Tabes Dorsalii. DifferentialDiagnosis Other syphilitic illness, osteoarthritic changes, prolapsed intervertebral disc, intra-spinal, vertebral and superior pulmonary sulcus tumours, tuberculosis, traumatic avulsion of nerve root, etc. Code or

206X2 207X2 if associated with root pain.

Other Syphilitic Changes

Including Gumma

These are not described because extremely rare. They may be associated with Tabes. They may also mimic compressive lesions.

S%

and Arachnoiditis (Ix-lo)

Meningitis NOTE

Acute infectious meningitis will, rarely, produce radicular pain. Occasionally it will progress to arachnoiditis involving one or more spinal segments, causing root pain in the upper limb. More frequently the arachnoiditis is relatively diffuse with widespread and bilateral symptoms. Dehition

Continuous increasing pain, usually associated with a band-like constricting sensation and due to local infection and scar tissue formation.

Site

Neck, shoulder and arm, usually bilateral.

System

Nervous system.

Main Features

Prevalence - very rare in localised form, in which case, it is seen after tuberculous meningitis, viral, purulent or syphilitic meningitis; Pain - initially local, continuous, dull, aching pain with progression to a painful burning sensation of increasing intensity.

Associated

!3ymptoms There may be cord compression with various sensory and motor impairments. Neck stiffness, numbness, tingfii hot and cold sensations. Band-like sensation often described. Aggravated by movement, coughing and sneezing. Worse with bed rest and in the morning. Moderately frequent, sensory impairment.

Signs Laboratory

Neck stiffness. Motor and sensory impairments. Fiidings Blood and CSF changes appropriate to causal infection. Myelography may show partial or complete obstruction or polymorphous fragmentation of dye into multiple areas. Local or generaiised inflammation with loose proliferation of pia-arachnoid followed by formation of dense, fibrous adhesii between dura-arachnoid and spinal cord.

Pathdosv

. I)lagnostic

criteria History of pain as above and myelographic changes as described

Differentialt%gnosii From causes of cervical compression especially ruptured intervertebral disc, osteoarthritic changes, fracture, abscess, intra-spinal, vertebral and superior pulmonary sulcus tumour; also from traumatic avulsion of nerve root, herpes zoster, tuberculosis and syphilis. Code

203.X2C 203.X4 - if due to neoplasm

S99

Traumatic Ad&on

of Nerve Roots (K-11)

Definition

Pain in one or more root distributions from traumatic avulsion.

Site

Neck and limb, especially the limb.

System

Nervous system.

Main Features

Onset - after direct or indirect trauma. Initiallysharp pain and dysaesthesiae

over site of injury followed in a few minutes by severe pain and dysaesthesiae in the dermatomal distribution; Pain - may decline but in the more severe cases, continues, becomes burning, and increases sometimes to distressing and incapacitating levels with lancinating bouts in late stages. Associated

Symptoms

Anaesthesia or hypoaesthesia to touch and noxious st~muiationin the painful area, motor weakness. Signs Laboratory

Hypoaesthesia and anaesthesia, weakness of lower motorneurone type, impairment or loss of tendon reflexes. Findings

Abnormal myelosram; dye can be seen extravasating from avulsed nerve root. Relief

Nil.

Pathology

Partial or complete avulsion, sometimes with late scar formation.

Diagnostic Criteria

History of injury with severe pain and loss of sensation and impairment as described. Differential Diagnosis

Brachial plexus lesions. Code

203.Xlb

Sloe

Superior Pulmonary Sukus Sydrome

(Pancoast Tumour) (R-12)

Lhdhith

Progressively intense pain in the shoulder and ulnar side of the arm associated with sensory and motor deficits and Homer’s Syndrome due to neoplasm.

Site

Shoulder and upper limb.

*tern

Nervous.

Main Features

The age on onset is usually in the decades corresponding with the Occurrence of carcinoma of the lung; males more than females. The pain is continuous, involving the root of the neck and ulnar side of the upper lib. It is usually progressive requiring narcotics for relief and becomes excruciating unless properly managed. The lesion is involvement of the 8th cervical and 1st thoracic roots. The pain is a severe aching and burning associated with sharp, lancinating exacerbations. There is paralysis and atrophy of the small muscles of the hand and a sensory loss corresponding to the pain distribution.

Associited

symptoms The cervical sympathetic

si

and Laboratoty

is involved with a Homer’s syndrome.

Fi

Atrophy of the small muscles of the hand, ulnar sensory loss, ulnar paraesthesias and pain, and Homer’s syndrome. The diagnosis is made on chest x-my by the appearance of a tumour in the superior sulcus. EIectromyography will demonstrate denervation in the appropriate distribution. usual

COul?X?

Complications

The course is generally relentless and the prognosis poor.

Occasionalinfiltration of spinal cord with compression. from infiltration of the w nerves.

Occasional hoarseness

!3cx5alandPlyskallXmbilii Those related to the neurological loss, unemployment Paw

or Other

and family stress.

Contributory Factors

Virtually ahways carcinoma of the lung, though any tumour metastatic to the area may give identical findings. Summary

of Ehential Features and Diagnwh Criteria The essential features are unremitting pain, the presence

of the Homer’s syndrome associated with Tl, C8 and occasionally neumlogkal losq and the diagnosis is made by chest x-ray and appropriate biopsy of the tumour. 102.x6a V., and Pagni, C.A. (Eds). Management of Superior (Panmast Syndrome) (Advances in Pain Research and Therapy, Vol. 4). New York, Raven Press, 1982. Bonica, J.J., Ventafndda,

Pulmonary Sulcus S&m

SlOl

Thoracic Outlet Syndrome (K-13) (Includes Scalenus Anticus Syndrome, Cervical Rib Syndrome) Definition

Pain in the root of the neck or shoulder radiating to the arm most often down the ulnar aspect. Due to compression of the bra&al plexus by an hypertrophied or abnormal scalenus anticus muscle, or a cervical rib or malformed first thoracic rib (cf. IX-14).

Site

Cervical and cervical spine.

System

Musculoskeletal system.

Main Features

Scalenus anticus syndrome is rare without an associated cervical rib. It is a disease of young to middle-aged adults. There is no sexual predilection. The pain characteristically begins in the root of the neck or shoulder and radiates down the arm. The ulnar aspect is the most commonly involved, but the pain may reach the entire arm. Paraesthesias in the same distribution are common. The pain occurs irregularly usually with activity. The pain is not intense and is described more as annoying. When the pain occurs, it usually relents promptly with rest.

Associated Symptoms

Raynaud’s phenomenon involving the same extremity is common. Hemiplegia from stroke secondary to vascular thrombosis and propagation of the clot may occur. The pain is generally aggravated by exercise and relieved by rest. A dystrophic sympathetic change may also occur. Signs and Laboratory Findings

Postural abnormalities are common. Tingling in the hand or hands, and colour change, if not present already, may follow abduction and elevation of the upper limb; brachial plexus tenderness in the neck or axilla may occur and colour change may also appear with other manoeuvres, e.g., bracing back the shoulders. The classic sign is Adson’s manoeuvre. This is performed by maximal extension of the chin and deep inspiration with the shoulders relaxed forward and the head turned towards the suspected side of abnormality. Obliteration of the pulse should occur. The most common laboratory abnormality is the presence of a cervical rib seen on x-ray. Electromy~~hy may demonstrate evidence of nerve root compression across the thoracic outlet and denervation distally in the arm. Angiography can demonstrate vascular compression, either arterial or venous. uslial

course

Complications

Unless there is evidence of vascular compression, the usual course is one of continued pain without significantneurological loss or vascular compromise. The patient must be carefully examined on a regular basis to determine ifneurological loss has occurred. Arterial compression with ischemia of the upper extremity is possible. Stroke, secondary to carotid or vertebral occlusion from a propagated clot in the axillary artery is also possible.

s102 Pathology

A variety of anatomical anomaiii that compress the neurovascular bundle at the thoracic outlet. The syndrome may be precipitated in predisposed individualsby flexion extension injuries of the cervical spine with consequent postural or other change.

social and Physical Disability The physical disabilities relate entirely to dysfunction of the involved extremity and depend upon vascular compression, neural compression, and pain. Summary of Essential Features and Diagnchc Criteria Patients with scalenus anticus syndrome suffer from compression of the brachial plexus by an hypertrophied or abnormal scalenus anticus muscle. They c~acte~stic~~ dafelop pain and paraesthesias in the upper extremitieswith activity and are relieved by rest. Adson’s sign is positive. Electromyographic studies indicate delayed conduction time across the thoracic outlet. Differential Dkqnosii Cervicalrib, Pancoast’s tumor, aneurysm of the subciavian artery, tumour of the brachial plexus, adenopathy or tumour of other supraclavicular structures, cervical disc, and metastatic cancer to the cervical spine. Code

133.X6

233.X6

Cervical Rib or Malfrornrd First Thwack Rib W-14) It is impossible to differentiate the scaknus anticus syndrome from cervical or malformed first thoracic rib, except by x-ray. The presentations are identical. The diagnosis anddifferentiald&noses are the same. The only variation from the scalenus anticus syndrome is the findingof the abnormal or deformed rib on x-ray. The code is the same and the reference for this syndrome is the same.

s103

Pain of Skeletal Metastatic Disease of the Arm or Shoulder Girdle (IX-E) Definition

Dull aching pain in the shoulder girdle or upper extremity due to tumour infiltration of bone.

Site

Clavicle, scapula, humerus.

System

Skeletal system.

Main Features

The age of onset is usually in the 5th, 6th and 7th decades - corresponding to the occurrence of carcinoma of the lung, breast and prostrate. The pain is usually described as a continuous dull ache or a constant throb. It may radiate up into the neck or down into the anterior chest wall. An expanding lesion in the humerus may radiate into the forearm. The cardinal feature is acute exacerbation of the pain by any movement of the shoulder girdle.

Associated

Symptoms

Pain at rest usually responds to non-steroidal anti-inflammatory drugs and narcotic analgesics. Pain secondary to movement is sometimes relieved by internal fixation. Both types of pain may respond to radiation therapy. Signs and Laboratory

Findings

The active range of movement of the shoulder girdle is usually much more limited than the passive range of movement. Well localized bony tenderness is common. Neurological signs are unusual. A radio-isotope bone scan is usually positive before a plain x-ray. However, both of these tests may be normal in the setting of severe pain. Complications

The tendency to keep the upper extremity immobilized may result in a ‘frozen shoulder’with secondary pain on that basis. A pathological fracture in the shaft of the humerus severely exacerbates pain on movement and this usually requires treatment with internal fixation.

Social and Physical Disability

There may be loss of use of the involved upper extremity. Summary

of Essential Features and Diapostic

Criteria

The essential features are continuous aching pain, exacerbation of the pain by

movement and localized bony tenderness from a metastatic deposit. ~~eren~

Diagnosis It is important to rule out referred pain to the shoulder girdle and upper extremity

due to tumour infiltration of the cervical roots and bra&al plexus. Code

233.X4

SlO4

GROUP x: LESfONS OF THE BRACHAL PLEXUS Tumours of the Brad&d Plexus (X-l) D&nit&

Progressive aching, burning pain with paraesthesias and sensory and motor impairment in the distribution of a branch or branches of the bra&ii plexus due to tumour.

Site

Shoulder and upper limb.

System

Nervous.

TumOUrS

Benign turnours - ~hwan~~, ~urofibr~; ~a~~ tumours -- malignant schwannoma and fibrosarcoma, metastatic neoplasm or direct invasion from other lesion, neuroblastoma, ganglioneuroma (secondary neoplasia of peripheral nerves occurs frequently in lymphoma, leukemia, multiple my&ma). Breast, lung and thyroid neoplasia frequently involve the brachial plexus.

Main Features

The specific tumours of peripheral nerVe are extremeiy rare. There is no sex predilection and the age of onset is young adulthood. They are more common with Von Recklinghausen’s disease. The pain tends to be constant, gradual in onset, aching and burning, associated with paraesthesii in the distriiution of the pain, and progressive wasting of muscles depending upon what groups are invohd, and sensory loss. The intensity is severe.

Associated

symptoms

The pain is generally not affected by activity. There is associated sensory loss and muscle wasting depending upon the area of the brachial plexus invoIved. Pain relief even with sign&ant narcotics is often not adequate. Si

and Laboratory Findings The laboratory findings are those of the underlying disease. Signs are loss of reflexes, sensation, and mu&e strength in the auburn of the involved portion of the plexus. There may be a local mass. The diagn&i is usually made promptly by x-ray or by CT scan. Ekzctromyographii studies validate the location of the lesion and there may be a palpable mass in the supracbvicular space.

usuaJcour8e

These turnout-s are relentkAy progressive unless treated.

SociiandPhysicADia!ability Those related to the loss of function. Summaxy of I!ZssentialFeatures ad f3&nostk Criteria The tumours are associated with sbwiy progressive pain and paraesthesii, and subsequently severe sensory loss and motor loss. The diagnostic criteria are the increasing aching, burning pain, its distribution in the brachial pkxu~, the associated paraesthesias, motor and sensory loss, and the presence of a mass by palpation and on x-ray or CT scan.

Slck5 DifferentialDiagnosis Includes all those lesions above, the scalenus anticus syndrome malities of the 1st thoracic rib or the presence of a cervical rib. 102.X6b 202.X6b

Code

Chemical

and abnor-

Irritation of the Brachial Plexus (X-2)

Definition

Continuous burning pain occasionally accompanied by severe paroxysms, in the distribution of the brachial plexus or one of its branches, with sensory-motion deficits due to effects of local injection of chemical irritants.

Site

Upper limb.

System

Nervous system.

Main Features

Injections in the shoulder area with any noxious agent are extremely rare. However, the pain syndromes from these injections are quite well defined. The pain begins almost immediately with the injection and is continuous. It usually is burning in character, superficial and unaffected by activity. Occasional paroxysms may occur. It frequently persists even after neurological loss has resolved and is not necessarily associated with paraesthesias or sensory loss. There are no differences between noxious agents as to time pattern, occurrence, character, intensity or duration.

Signs and Laboratory Findings The signs are of brachial plexus injury. Atrophy, sensory loss, and paraesthesias in the appropriate area depending upon the portion of the plexus injured. There are no specific laboratory findings. UsuaI course

Pain is generally acute with the injection and gradually improves. Most disappear within a few weeks. Those that persist continue unabated permanently.

Complications

The complications

Pathology

The pathology is a combination of intraneural and extraneural scarring with focal demyelinization.

are those of brachial plexus injury.

Summary of EssentialFeatures and Diagnostic Criteria These are those of brachial plexus injury. The diagnosis can only be made by history of injection. DifferentialDiagnosis This includes all of the muscular tumours previously described. Code

102X5 202X5

and bony compressions,

anomalies

and

s106

Traumatic Awl&on of the Bra&ii

Plexus (X-3)

Definition

Pain, most often burning or crushing with super-added avulsion lesions of the brachial plexus.

Site

Felt almost invariably in the forearm and hand irrespective of the roots avulsed. Occasionally, in avulsion of C5 root only, pain may be felt in shoulder.

!5ystem

Nerve roots tom from the spinal cord.

Main Features

Prevalence - some 90% of the patients with avulsion of one or more nerve roots

paroxysms,

following

suffer pain at some time. Virtually ail patients with avulsion of all five roots suffer severe pain for some months at least; Age ofonset - vast majority of patients with this lesion are young men between the ages of 18 and 2.5 suffering from motorcycle accidents. The older the patient the more likely he is to suffer pain from the avulsion lesions. The pain is characteristically described as burning or crushing as if the hand was being crushed in a vice or was on fire. The pain is constant, is a permanent background to their life, and in a high proportion of patients (in one series 90%) there were periodic paroxysms or shots of pain lasting for a few seconds and of agonising intensity. These paroxysms stopped the patient in his tracks and may cause him to cry out and grip his arm and turn away. Their frequency varies between a few an hour, a few a day, or a few a week. In a few patients they can be very frequent with as many as 20 or 30 times in an hour. There is no set pattern to the paroxysms and the patient has no warning of their arrival. The constant pain may also be described as severe pins and needles and electric shocks, but it is most often burning or crushing. In some patients there is a gradual increase in the intensity of the pain over a period of days building up to a very hiih level of pain lasting a day or more and then gradually subsiding over the next few days. In these patients the pain is particularly unpleasant and interferes seriously with their lives. Associited

!symptoms Aggravating factors: cold weather, extremes of temperature, emotional stress, intercurrent illness, all aggravate the pain. The pain is almost invariably relieved by distraction involving absorbing work or hobbies. The pain is at its worst when the patient has nothing with which to occupy his mind. Patients often grip the anaesthetic and paralyzed arm or hit the shoulder to try and relieve the pain. Drugs are singularly unhelpful and a full range of analgesics is usually tried but very few patients respond significantly. Alcohol helps, probably by relaxing the patient and promoting sleep. A number of patients have found that smoking cannabis can markedly reduce the pain, but if so it interferes with their concentration and very few indeed are regular cannabis smokers.

Signs

Paralysis and anasthetic loss in the territory of the avulsed nerve root, i.e., avulsion C5-T1- a totally paralysed and insensitive arm. Most patients ask their doctors about amputation as a means of reliving the pain and it has to be made clear to them the pain is central and amputation has no effect at all. In fact, there is a good likelihood of adding stump pain to their existing pain. Avulsion of Tl is associated with a Homer’s sign, drooping of the eyelid and constriction of the pupil. Myelography often shows evidence of meningoceles or root avulsion. Electrophysiological tests may well show the presence of sensory action potentiais in anaesthetic areas indicating that the lesiin must be proximal to the posterior root

ganglion. A flare response to intra-dermal histamine is occasionally particularly in C5 lesions, again indicating preganglionic lesions. usual

course

Complications

s107 useful,

Two-thirds of patients come to terms with their pain or say the pain is improved, within three years of onset. If the pain is still severe at three years after onset, it is likely to last for the rest of their lives, and in these patients the pain steadily gets worse as they get older. Rarely, trophic lesions of the anaesthetic arm, e.g., bums, infections, severe depression may occasionally follow prolonged pain but it is remarkable how these young men manage to come to terms with their disability.

Social and Physical Disability The major disability is the paralysis of the arm and the effect this has on work, hobbies and sport. Pain itself can interfere with their ability to work and can cut them off from normal social life. Pathology

Summary

Avulsion is associated with spontaneous firing of deafferentated nerve cells in the spinal cord at the level of the injury and may in time cause abnormal firing at higher levels of the central nervous system. of Essential Features and Diagnostic Criteria The pain in avulsion lesions of the brachial plexus is almost invariably described as severe burning and crushing pain, constant, and very often with paroxysms of sharp, shooting pains that last seconds and vary in frequency from several times an hour to several times a week. So characteristic is the pain of an avulsion lesion that it is virtually diagnostic of an avulsion of one or more roots. Traction lesions of the brachial plexus that involve the nerve roots distal to the posterior root ganglion are seldom if ever associated with pain. Sometimes in regeneration spontaneously, or after nerve grafts for rupture of nerve roots distal to the intervertebral foramen, a causalgic type of pain develops but this is highly characteristic of causalgia and cannot be confused with avulsion or deafferentation pain.

Code

203.Xlc

Reference

Wynn Parry, C.B. Pain in avulsion lesions of the brachial plexus, Pain, 9 (1980) 41-53.

Painful Arms and Moving Fingers (X-5) See XXX-7. Reference

Verhagen, W.I.M., Horstink, M.W.I.M. and Notermans, S.L.H. Painful arm and moving fingers. J. Neural. Neurosurg. and Psychiatry, 48 (1985) 384-389.

s108

GROUPXkPAlNINTHESHOULDER,~ANDHAND Tendonitis (XI-l)

Bii Dehition

Severe pain with acute onset due to inflammation of the longhead biceps tendon.

Site

Anterior shoulder.

System

Musculoskeletal.

Main Features

Severe pain usually with acute onset in the anterior shoulder, following trauma or excessive exertion. It may radiate down the entire arm and is usually self bited but there may be recurrent episodes.

Aggramting Factors Movement of shoulder and elbow. Tendon palpation in the shoulder biiipital groove is painful. Pain is reproduced by resisted supination of the flexed forearm Wergason’s sign). usual

course

Occurs primarily after repeated use or heavy strain on tendon. It may become chronic.

Relief

Nonsteroidal

Complications

Frozen shoulder (adhesiie capsulitis).

fidrdosv

Inflammation of the tendon sheath.

anti-inflammatory

agents; local steroid injection.

Essential Features Acute pain in the anterior shotier, forearm.

aggravated by forced supination of the flexed

Diffelwltia.lDiagn&s Subacromial Code

bursitis, cakific tendon&,

rotator cuff tear.

231.x3

Aching pain in the shoukkr Site

Shoulder and upper arm.

svst-

Musculoskeletal.

due to it&mm&on

of subacromial

bursa.

s109 Main Features

Common over 30 years of age. The cond~t~n presents with aching pain in the deltoid muscle and upper arm above the elbow aggravated by using the arm above the horizontal level (painful abduction). The pain is aggravated by sleeping on the affected shoulder. It is usually precipitated by repeated or minor trauma.

Signs

Tenderness over the insertion of supraspinatus and internal rotation.

tendon. Painful arc of abduction,

Radiologic Findings High riding humeral head on x-ray when chronic attenuation usual

course

of bursa occurs.

Recurrent acute episodes may produce chronic pain.

Relief

Nonsteroidal anti-inflammatory heat, physiotherapy.

Complications

Frozen shoulder (adhesive capsulitis).

Pathology

Chronic inflammation of bursa; tendon.

agents, local steroid injection, ultrasound, deep

Ekential Features Aching pain in shoulder with in~ammation of the subacromi~ bursa and exacerbation on movement as well as tenderness over the insertion of the supraspinatus tendon. DifferetialDiagnosis Calcific tendonitis, rotator cuff tear. Code

238.X3

Rotator Cuff Tear -

Partial or Complete (XI-3)

Definition

Acute severe aching pain due to traumatic rupture of supraspinatus

Site

Shoulder and upper arm.

System

Musculoskeletal.

Main Features

Acute severe aching pain in the shoulder foII~ng trauma, usually a fall on the outstretched arm. Abduction is extremely painful or impossible. The patient is unable to sleep on the affected side.

Signs

A partial tear is distinguished from a complete tear by subacromial injection of local anaesthetic, partial tears will resume normal passive range of motion. The arm may drop to the side if passively abducted to 90” (“drop arm sign”) if there is a complete tear.

Radiologic Findings High riding humeral head on x-ray.

tendon.

s110

Complications

Frozen shoulder

Essential Features Acute severe pain due lo trauma at the supraspinatus tendon

Differential Diagnosis Calcific tendonitis. subacromial bursitis

Code

231.x1

Lateral Epicondylitis

-

(Tennis Elbow) (XI-4)

Definition

Pain in the lateral epicondylar region of the elbow due to strain or partial tear of the extensor tendon of the wrist.

Site

Musculoskeletal.

Main Features

Acute, subacute or chronic pain of the elbow during grasping and supination of the wrist. Most common between 40 and 6Oyears of age. Pain radiates down the lateral forearm or to the upper arm.

Aggravating

Factors Repeated trauma.

Relief

Relieved by local steroid injectlon and physiotherapy.

Signs

Tenderness of the wrist extensor tendon about 5cm distal to the epicondyle Resisted wrist dorsiflexion reproduces pain.

usual course

Usually self limiting; several months duration

Laboratory

and Radiologic Findings Negative.

Pathology

Strain or partial tear of tendon at tendoperiosteal junction

Essential Features Pain at the lateral epicondyle, worse on movement, aggravated by overuse

Differential Diagnosis Nerve entrapment, cervical root impingement, carpal tunnel syndrome. Code

235.Xla

Sill

Medial Epicondylitis“Golfer’s Elbow” (XI-5) Definition

Pain in the medial epicondylar region of the elbow

Main Features

As for “tennis elbow” (XI-4) but much less common.

Aggravating Factors

As for “tennis elbow”. Signs

Tenderness

over the tendon insertion of the medial epicondyle.

Laboratory and Radiologic Findings

Negative. Usual Course

As for “tennis elbow”. Pathology

As for “tennis elbow”.

Differential Diagnosis

As for “tennis elbow”. Code

235.Xlb

DeQuervain’s Tenosynovitis (X1-6) Definition

Severe aching and shooting pain due to stenosing pollicis longus or extensor pollicis brevis.

Site

Wrist.

System

Musculoskeletal.

Main Features

Sudden onset of severe aching or shooting pains. There may be localized swelling and/or redness.

tenosynovitis

of abductor

Aggravating Factors

Aggravated by pinch, grasping or repetitive thumb and wrist movements. Signs

Occasional tendon swelling; tenderness over the tendon in the anatomical snuff box area. Finkelstein’s sign reproduces the pain; the patient’s thumb is folded into a fist and then the wrist is deviated to the ulnar side.

s112

usual course

May be single self-limited episode or recurrent

Relief

Relief from local splinting or local steroid injection

Pathology

Inflammatory lesion of tendon sheath usually secondary direct trauma.

and chronic

to repetttive motion or

Essential Features Severe aching and shooting pain in the radial portion of the wrist related to movement Differential Diagnosis Arthritis of the wrist, scaphoid injury. Code

Ostearthritis

233.X3

of the Hands (XI-7)

Definition

Chronic aching pain in the fingers with degenerative proximal phalangeal joints of the hands.

System

Musculoskeletal

Main Features

The illness occurs mainly in women over 45 years of age. The pain is chronic and aching in the fingers and aggravated by use and relieved by rest. There may be mild morning stiffness for less than half an hour and subjective reduction of grip strength, worse with trauma to nodes.

Signs

Bony enlargements of the distal interphalangeal joints are called Heberden’s nodes and the proximal interphalangeal joints are called Bouchard’s nodes. The fingers may be stiff and lose some degree of full flexion. Grip strength is usually normal when measured.

changes of distal and

Radiologic Findings Narrowing of joint spaces, sclerosis and bony osteophytosis. Relief

Analgesics, soaking in hot tluids.

Code

238.X6b

55113

Carpal Tunnel Syndrome (XI-@ Definition

Stinging, burning, or aching pain in the hand, often nocturnal, due to entrapment of the median nerve in the carpal tunnel.

Site

One hand (sometimes bilateral), in the fingers, often including the fifth digit, often spreading into the forearm and occasionally higher; not usually well localized.

System

Peripheral nervous system.

Main Features

Preualence - very common; Age of Onset - usually 4th~5th decades; Sex Ratio - female:male = 5:l; Quaky - pins and needles, stinging, often aching, occasionally burning; Time pattern -usually nocturnal, typically awakening the patient several times and then subsiding in a few minutes; aching pain is often more constant; Intensity - may be severe briefly.

Associated Symptoms

Aggravated by handwork such as knitting. Signs and Laboratory Findings

Clinical examination often normal but one may find decreased pin prick sensation on the tips of digits I-III, a positive Tinel’s or Phalen’s sign or rarely, weakness and/or atrophy of the thenar muscles (abductor pollicis brevis); nerve conduction studies showing delayed sensory and motor conduction across the carpal tunnel are diagnostic. Usual Course

Very slow progression for years.

Social and Physical Disability

May impair ability to do handwork. Pathology

Compression of median nerve in wrist between the carpal bones and the transverse carpal ligament (flexor retinaculum); focal demyelination of nerve fibres, axonal shrinkage and axonal degeneration.

Summary of Essential Features and Diagnostic Criteria

Episodic paraesthetic nocturnal pain in the hand with electrophysiological evidence of delayed conduction in the median nerve across the wrist. 204.X6

Pain of Psychological Origin in the Shoulder and Arm (XI-g) Code

233.X7b(tension: arm)

21X.X9a (delusional: arms) 21X.X9b (conversion: arms)

s114

GROUP XII: VASCULAR DISEASE OF THE LJMBS

Raynaud’s Disease (XII-l) Definition

Episodic attacks of aching, burning pain associated with vasoconstnction arteries of the extremities in response to cold or emotional stimuli.

Site

Predominantly in the hands, unilateral initially. later bilateral. Rarely lower limbs and exposed areas of face.

System

Cardiovascular.

Main Features

Prevalence -- Raynaud’s phenomena can occur in 5’b of normal females as secondary to connective tissue disease. Raynaud’s disease is uncommon, with a female-male ratio of 51, onset most common between puberty and age forty. Exacerbations during emotional stress and possibly at time of menses; Start -evoked by cold, nervousness and other stimuli which vary among patients. A typical attack occurs in three phases. Initially the digits become ashen white, then they turn blue as the capillaries dilate and @Iwith slowly flowing deoxygenated blood. Finally the arterioles relax and the attack comes to an end with a flushing of the diseased parts; Quality - initially the pain is deep and aching and varies from mild to severe, changing to severe burning dysaesthesiae in the phase of reactive hyperaemia; Occurrence - recurring irregularly with changes in environmental temperature and emotional status; intensify -- variabie from mild to severe depending upon the temperature and other stimuli; Duration... minutes to hours. Sometimes may last days if painful ischaemic skin ulcers develop.

Associited

Symptoms

of the

and Sins

Numbness or hypoaesthesia are present. Progressive spasm of the vessels leads to atrophy of the tip, giving the finger a tapered appearance. The nail becomes brittle and paronychia is common. Advanced cases may develop focal areas of necrosis at the finger tip, occasionally preceded by cutaneous calcification. These areas are extremely painful and tender to palpation. Anxiety and other signs of sympathetic over-activity such as increased sweating in the limbs and piloerection develop. Relief

Temporary relief from sympathetic block occurs and occasional prolonged relief from s~~thectomy in the early phases. Calcium channel blocking agents may help.

Pathology

The cause of “cold sensitivity” is unknown. Abnormalities in sympathetic activity have not been proven. However, local application of cold is necessary to elicit the response of Raynaud’s syndrome and the threshold for triggering the response is lowered by any factor which increases sympathetic outflow or circulating catecholamines.

s115 Essential Features

Colour changes of digits, excited by cold or emotions, extremities and absence of specific organic disease.

involving both upper

Differential Diagnosis

Raynaud’s disease, which has no other known cause, and Raynaud’s phenomenon, which is a response occurring in other illnesses, should be distinguished. The following other diseases should be recognized; collagen-vascular diseases: scleroderma, rheumatoid arteritis, systemic lupus erythematosis, dermatomyositis, periarteritis nodosa; other vascular diseases: thromboangiitis obliterans, thrombotic or embolic occlusion, arteriosclerosis obliterans, syphilitic arteritis; trauma: vibration (air-hammer disease, etc.), percussion (digital pianist, typist, etc.), palmar (hypothenar hammer syndrome); neurovascular syndromes: thoracic outlet syndromes, spondylitis, causalgia; central and peripheral nervous disorders (rarely): syringomyelia, poliomyelitis, ruptured cervical disc, progressive muscular atrophy; cold injury: frostbite, nonfreezing cold injury, (pernio, immersion foot), cold sensitivity syndrome; lack of suspension stability of blood: cold agglutinins, cryoglobulinaemia, cryofibrinogenaemia, polycythaemia Vera; intoxication: ergot, arsenic, heavy metals (lead), nicotine and tobacco. Code

Raynaud’s

224.X7a 224XXb 624.X7a 624.XXb 024.X7a 024XXb

Raynaud’s Disease Raynaud’s Phenomenon (legs) (legs) (face) (face)

Phenomenon

Note: When the legs are affected codes are altered to provide a ‘6’ in the first digit. When the face is affected codes are altered to provide a ‘0’ in the first digit.

(X11-2)

Definition

Attacks like those of Raynaud’s disease but related to one or more other disease processes.

Usual Course

In accordance

Pathology

Systemic and vascular diseases such as collagen disease, arteriosclerosis obliterans, nerve injuries, occupational trauma for example in chain saw operators, pianists and pneumatic hammer operators, may all contribute to the development of Raynaud’s phenomenon.

Code

224XXb 624XXb 024XXb

with the underlying disease.

Raynaud’s (legs) (face)

Phenomenon

Note: When the legs are affected codes are altered to provide a ‘6’ in the first digit. When the face is affected codes are altered to provide a ‘0’ in the first digit.

S116

Fro&bite and Cold Injury (XII-3) Definition

Severe burning pain 111digits or exposed areas of face due to cold injury

Site

Periphery of limbs (digits) and exposed areas of face.

System

Cardiovascular.

Main Features

Preualence

-- increased incidence in elderly patients with arterial disease and in

young men with hazardous exposure to cold environment, e.g., soldiers, mountaineers; Start --frostbite commences with an initial vasospastic phase with pallor and numbness, followed by cyanosis. Rubor only returns on rewarming. Signs and severity vary steadily with degree of cold exposure, see below; Quality - at time of exposure numbness and tingling of digits and severe

aching pain occur. After a few days severe burning or stinging pain, particularly after exposure to warmth. Then pain becomes a deep aching or throbbing which may persist for many weeks; Occurrence .- single episode after cold exposure or recurring episodes if there is a predisposition to cold injury; Intensity .- mild initially, then severe after a few days if limb warmed; Duration usually 2-3 weeks to eight weeks, but pain can become chronic. Associated

Symptoms In chronic stages: sometimes hyperaesthesia and increased sweating, increased sensitivity to cold, numbness, aching, paraesthesias and dysaesthesias.

Si

and Usual Course 1“ frostbite - oedema, erythema and hypoaesthesia lasting 2-3 weeks followed by superficial desquamation; 2”frostbite - vesicles and blisters in superficial skin layers. In 2-3 weeks vesicles dry and leave thickened epithelium (in absence of infection); 3” frostbite - involves full skin thickness. Hard black scar develops and separates in about 8 weeks; and 4”frostbite - results in deep tissue necrosis down to bone and requires amputation of the affected area.

Complications

Infection leading to cellulitis, contamination occurs after gangrenous extremities after paraesthesias; hyperhidrosis relieved by sympathetic block

tetanus and gas gangrene are unlikely unless rewarming; amputation may be required for fourth degree injury; persistent cold sensitivity; and burning pain which may be prevented or or denetvation.

Social and Physical Disabilii Restriction of use of limbs due to cold sensitivity, hyperhidrosis and pain. Pathology

On initial exposure to cold, intense vasoconsttiction occurs in extremity areas and results in reduced microcirculation flow with sludgingof red cells; eventually flow ceases at the onset of freezing. Frozen tissue is bloodless, hard, cold and pale. As tissues thaw vasodilation occurs and flow is resumed, however interstitial oedema restricts flow and white emboli dislodge from injured vessel walls and mix with platelets to form thrombi at venular bifurcations and this obstructive process extends through to precapillaty arterioles so that within 1 hour most of these microvascular channels are occluded. Tissue necrosis is attributed to mechanical effects of microvascular occlusion, to extracellular ice crystals and cellular dehydration.

s117 Essential Features

Exposure to cold below 0” C followed by tissue injury a variable period after exposure. Differential Diagnosis

Erythema Pernio (chilblains), trench foot, immersion foot, cold sensitivity, cold agglutinin syndrome, cryoproteinemia. Persistent pain and hyperhidrosis following frostbite may simulate causalgia. Code

222.Xla (arms) 622.Xla (legs)

Erythema Pernio (Chilblains) (X11-4) Definition

Pain and itching in areas of extremities following exposure to cold and wet environment above 0” C and associated with pigmented or purpuric skin lesions.

Site

Digits and limbs, especially lower limbs.

Main Features

Similar to 1” frostbite except that women are more susceptible (especially those with “sensitivity to cold”). At time of exposure numbness and tingling of digits may occur. Redness and itching of the skin is a feature together with excessive sweating. The pain is often mild but may be associated with intense itching and with burning sensations. Pernio tends to be seasonal in occurrence, associated with cold exposure. The pain is always aggravated by warmth.

Associated Symptoms

Blebs filled with clear or bloody fluid may form and pigmented or purpuric lesions may develop. Differential Diagnosis

Erythema nodosum, erythema induratum, Raynaud’s disease and acrocyanosis. N.B. The latter is painless. Code

225.X1 (arms) 625.X1 (legs)

S118

Acrocyanosis (XII-5) Definition

Persistent blueness and coldness of hands and feet, sometimes wit t?xhmg pct~n.

Site

Hands and feet, expecially digits.

System

Vascular

Main Features

Blueness and coldness, commoner

in women, sometimes aching pain. often noi.

Asscxiited Features Often chilblains. Trophic changes. ulceration or gangrene do not occur. Symptoms are more marked in cold periods. Commences in late childhood and seems to have an hereditary background. Code

222.Xlb (arms) 622.Xlb (legs)

References

Juergens, J.t. et. al., Pe~p~eral Vascular Diseases, 5th edition, W.B. Saunders Co., Philadelphia, 1980.

Livedo Reticularis (XIM) Definition

Common, possibly, vasospastic disorder in women under the age of 40; associated with persistent aching in the skin of the arms, itching of c-ircular and reticular lesions which have a mottled cyanotic appearance.

Site

Upper or lower limbs.

Main Features

Occurring in women, the more severe form (cutis marmorata) isassociated with persistent aching in the skin of the arm. Itching, circular and reticular lesions with a mottled cyanotic appearance are evident.

DifferentialDiagnosis Pernio, other skin changes. Code

222.68a (arms) 622.6&a (legs)

5119

GROUP XIII: COLLAGEN DISEASE OF THE LIMBS Scleroderma (XIII-1) De~nition

Intermittent vasospasm often with soreness, stiffness or swelling of peripheral joints of the fingers and toes due to collagen disease of the skin, particularly affecting the limbs.

Main Features

Onset - from young adult life onwards; Incidence - 3-5 new cases per million per annum; Male/female ratio - 1:3; Pain is not a great problem in most cases. However, pain can occur intermittently with soreness and pain of Raynaud’s phenomenon, especially aching pain in episodes ranging from mild to severe and changing to burning dysaesthesiae in the phase of reactive hyperemia. Numbness or h~oa~th~iae are present also.

Associated

Symptoms

and Signs

Stiffness and swelling of peripheral joints of the fingers and toes. A tight skin which may or may not be thickened. Skin temperature is often lowered and the underlying tissues seem firm. Fingers although swollen and stiff can be moved with difficulty. The skin appears pale and waxen, skin temperature is lowered in the affected parts and although pulses are palpabie at the wrist, there is usually complete arterial obstruction in the digits. Microstomia and multiple telangectasia may be observed over the face and hands. Essential Features Evidence of scleroderma

with Raynaud’s phenomenon.

Differential Diagnosis See Raynaud’s disease and Raynaud’s phenomenon. Code

226.X5 (arms) 626.X5 (legs)

Ergotism (XIII-Z) Definition

Burning pain in the extremities, identical to Raynaud’s phenomenon, associated also with systemic symptoms attributable to excessive ergot intake.

Site

Fingers and toes especially; viscera are occasionally involved also.

s120 Main Features

Occurs in patients taking excess ergotamine tartrate or others (rarely) who have eaten rye or wheat contaminated by ergot. Uncommon in general. Presents with burning pain in the extremities identical to Raynaud’s phenomenon Three stages can be seen in the changes in the circulation: (1) a stage of cyanosis or pallor from which recovery is rapid; (2) a stage of deep purple colouration in which blanching cannot be effected by pressure and from which rer-over-ymay be slow or may not occur: and (3) a stage of necrosis. Severe cases of ergot intoxication are however sporadic. Symptoms can consist of dizziness, frontal headache, angina pectoris, Raynaud’s phenomenon. coldness of the extremities and pain. Roth neurologic and vascuiar symptoms may produce the feeling of intense heat and cold. burning pains. known in the past as St. Anthony’s fire.

Associated

Symptoms Headaches, dizziness, nausea and vomiting, visual disturbances, angtna pectons, mono- or hemiplegia. May result in gangrene.

usual

course

On discontinuation of ergot administration pulses and signs of ischaemia return to normal in 1 to 3 days. In stages (2) and (3) more vigorous therapy is needed with anticoagulant and vascular dilatation agents.

compiicationsGangrene.

In some cases residual anaesthesia of the skin or paralysis of the extremities may persist.

Pathokqy

Summary

Ergot intoxication results in constriction of the arteries. Due to the vasoconstriction the endothelium of the vessels suffers, stasis occurs in the capillaries and thrombosis follows. After thrombosis gangrene is inevitable. Actual intoxication is not necessary for diminution of arterial pulses. The chronic use of therapeutic doses leads to lowered foot systolic blood pressure. The degree of tolerance to the vasoconstrictive effects varies widely.

of Essential Features

and Diagnostic

Criteria

Colour changes of digits, burning pain as described, evidence of excessive ingestion of ergotamine.

Differential Diignosis See Raynaud’s disease. Code

281.X5 (upper limbs) 681.X5 (lower limbs)

References

Juergens, J.L., et al., Peripheral Vascular Diseases, 5th edition, W.B. Saunders Co., Phifdelphia, 1980. Dukes, M.N.G. (Ed.), Meyler’s Side Effects of Drugs, 9th edition, Excerpta Medica, Amsterdam, 1980. Goodman, L.S. and Gilman, A., The Pharmacological Basis of I%erapeutics, 7th edition, Macmillan Publishing Co., New York, 19%.

s121

GROUP XIV: VASODILATING FUNCTIONAL DISEASE OF THE LIMBS Erythromelalgia (XIV-l) Definition

Episodic burning pain in the extremities accompanied by bright red discoiouration in response to increased environmental temperature.

Site

Extremities of the limbs but almost always the feet rather than the hands.

System

Vascular.

Main Features

Primary form rare and more often bilateral than the secondary type which is related to the frequency of the conditions. Men in the middle-age group are more often involved but women and children may also be affected. Characterized by severe, burning pain and red discolouration. The skin temperature is often raised, flushed with venous engorgement, and the surface hyperaesthetic. Attacks of severe burning pain last from a few minutes to many hours.

Associated

Symptoms Arteriosclerosis, hypertension, peripheral neuritis, cold injury, ~iycythaem~, disseminated sclerosis, infections, hemipie~, gout or chronic heavy metal poisoning may be present. Uicers or gangrene rare in primary type.

Signs and Laboratory

Findings

Diagnosed by reproducing symptoms after raising skin temperature to 31-36” C. Relief from cooling. Skin cyanotic or deeply red in response to rise of temperature. PathoIogy

Cause of most cases unknown. disorders listed above.

Secondary

e~thromel~~a

may result from

Differential Diagnosis Burning pain which comes in attacks and affects the foot-sole or palm of the hand, closely related to objective increased local skin temperature. Reduction of pain by elevating or cooling the affected extremity. Code

224.X8b (hands) 624.X8b (legs)

References

Juergens, J.L., et al., Peripheral Vascular Diseases, 5th edition, W.B. Saunders Co., Philadelphia, 1980.

Note: Add code for secondary cases according to aetiology.

Kappert, A., Lehrbuch u&Atlas Bern, 1981.

der Angologie, 1st edition, Verlag Hans Huber,

Fitzpatric, T.B., et al., Dermatology in GeneralMedicine: 2nd edition, McGraw-Hill, New York, 1977.

Textbook and Attlas,

s122

Thromboangiitis

Obliterans

(XIV-2)

Definition

Pain in the fingers or hands or small digits ot the feet, usually in males who smoke. associated with ulceration of fingertips and margins of nails; related initially to segmental inflammation of walls of medium and small arteries and veins.

Site

Fingers and hands, more often toes and feet. rarely the cdf

System

Cardiovascular.

Main Features

Prevalence a rare disease with a possible preponderance III Jews. Close association with smoking; SexRatio -- males more than females: ratio above 9: 1: Age of Onset usual age of onset between 30 and 40 years; Quality, Time. Intensity .- usual onset is sharp pain in fingers or hands or more often in the foot or calf. There may be episodes of intermittent claudication in the hands or legs or constant burning in the tips of the digits (rest pain); Pain -. may he unbearable. often aggravated by elevation.

Associated Symptoms

Tenderness in superficial arteries, veins or nerves in affecteti area. Cold extremities (upper and lower), dysaesthesiae and muscle weakness. Signs

Coldness and sensitivity to cold, sensations of numbness. paraesthesiae. sometimes superficial thrombophlebitis. Ulceration of finger tips and margins of nails, gangrene of digits which may be wet gangrene if there is venous obstruction; oedema present if there is venous obstruction. Absent ulnar or tibia1 artery pulsation and positive Allen test in cases affecting the arms (see scalenus anticus and thoracic outlet syndrome for Allen test). Abnormal colour of skin of digits: pale if elevated, red when first dependent, then blue. Cyclic co/our changes may occur in response to cold (Raynaud’s phenomenon).

Laboratoty Findings

Thermogram shows differences in temperature of digits. Skin plethysmography shows reduced blood flow in one or more digits, indicating local arterial disease, Tobacco sensitivity may be demonstrated. Vigorous muscle contraction of the digit may result in sufficient pressure to overcome intravascular pressure with cessation of blood flow as measured by plethysmogram. Doppler test may br helpful. Usual Course

Gradual progression smoking ceases.

Complications

Gangrene

Pathology

Ulnar, palmar and digital arteries affected early with segmental Inflammation initially. Arteries are contracted and hard and lumens occluded by adherent mass. Arteries and veins bound together by infammatoxy tissue. Acute stage: granulation tissue in all layers of affected arteries (pan-arteritis) and usually a thrombus in vessel lumen. Subacute state: thrombus recanaliied. Chronic stage: thrombus sclerotic, dense fibrous tissue encloses arteries, veins and nerves.

from age 30 for many decades. Favourably

and infection of digits. Osteoporosis

Influenced if

of bones of extremities.

S123 Summary of EssentialFeatures and Diagnostic Criteria Organic arterial disease of one or more digits almost afways in a male under 40 with a history of migrating superficial throm~phlebitis. DifferentialDiagnosis Arteriosclerosis, (larger vessels and more widespread) periarteritis nodosa, (veins not involved), giant cell arteritis (mainly branches of carotid), thoracic outlet syndrome. Code

224X3b (arms) 624.X3b (legs}

References

Juergens, J.L., et al., Pe~p~eral Vascular Diseases, 5th edition, W.B. Saunders Co., Philadelphia, 1980. Rutherford, R.B., Vascular Surgery, W.B. Saunders,

Co., Philadelphia, 1977.

Haimovici, M., Vascular Surgery: Principles and Techniques, McGraw-Hill, New York. 1976.

Chronic Venous

Insufficiency

(XIV-3)

Definition

Dull, aching pain in limbs, especially legs, characterized tortuous veins.

Site

Limbs, usually the legs; especialiy the distal portions. Found equally on right or left sides. Bilateral in seven percent.

Main Features

Preuafence - Females more than males about 15% of adult population; severe

by abnormally dilated or

only in 1%. More common in women, prevalence increases with age, and there may be an hereditary predisposition; Pain - Dull, aching pain, usually associated with varicosities. Additional pain often due to thrombosis and/or thrombophlebitis acutely. Associated Symptoms Feelings of heaviness, numbness of the skin. Previous throm~phlebitis in a vein of the extremity, orthostasis with oedema, developing during the day and disappearing during the night when the patient lies flat. After oedema has been present for some time, areas of brown pigmentation (hemosiderin and melanin) may appear. Eczema is a common feature. After longer periods of time there is a tendency towards the development of subcutaneous fibrosis with induration and swelling. Signs and Laboratory Findings Oedema, dilated superficial veins, varicosities, corona phlebectatica, hyper- and de-pigmentation, induration, open or healed ulcus cruris. In obscure or borderline cases phlebography is required. Usual Course

Chronic, but dependent on stage of insufficience and reaction on causal therapy.

s124 Relief

Relief, even of ulcer pains, occurs gradually as a result of recumbency and more quickly if the extremity is elevated. (Relief after 5-30 minutes). Causal therapy for ulcers and dermatitis is indicated.

Complications

Ulceration. Neuritis of II. saphena longus is occasionally seen as a compiration Chronic Venous Insufficiency.

ot

Social and Physical Disabiiy Dependent

on degree of insufficiency.

Pathology

Chronic Venous Insufficiency is the late consequence of extensive deep veins by thrombosis, in a given case. thrombophlebitis. The associated with oedema largely of the subcutaneous tissues. The pain of ulcers and indurative cell&is is usually due to secondary rather than congestion.

Aetiolosy

Hereditary factors. blockage by thrombosis or other disease (rarely carcinoma).

damage of the aching pain is more epicritic inflammation

EssentialFeatures Signs of venous insufficiency. Deep venous thrombosis in history Code

222.X4 222.X6 622.X4 622.X6

References

Juergens, J.L. et al, Peripheral Co., Phi~delphia, 1380.

(neoplasm) (arms) (legs. neoplasm) (legs)

Widmer, I-K., Peripheral 1978.

Vascular Diseases, 5th edition, W.B. Saunders

Venous Disorders,

Haimovici, M., Vascuk;r Surgery; Principles New York, 1976.

Hans Huber Publishers, and Techniques,

Rem.

McGraw-Hill,

Rutherford, R.B., Vascular Surgery, W.B. Saunders Co., Philadelp~~,

1977.

S125

GROUP XV: ARTERIAL INSUFFICIENCY

IN THE LIME%!+

Intermittent Claudication (XV-l) and Rest Pain (XV-Z) Definition

Intermittent cramping pain in a muscular area produced by exercise and relived by rest (XV-l) or constant pain in an extremity associated with hypoaesthesia and/or dysaesthesia and areas of skin ulceration or gangrene (XV-2).

Site

Intermittent claudication (pain after exercise) is almost always confined to the lower limbs. Pain from arterial insufficiency arising at rest may occur in lower limbs or upper limbs and may be related to gravity.

System

Cardiovascular.

Main Features

Age of onset- over 30, increasing in later middle age and decreasing in the aged; Sex Ratio - males affected earlier than females. The inte~ittent pain is cramping and severe and arises, usually, after fixed and consistent amounts of exercise. Severe ischaemia will result in rest pain and night pain. The pain is relieved by the dependent position which initially causes the limb to flush red and then become cyanotic. Elevation of the limb causes blanching and increased pain.

Associated Symptoms Hypothyroidism or myxedema, diabetes mellitus, hypercholesteremia, hyperlipidaemia, xanthomatosis and long-standing heavy smoking may be found. Occlusion leads to ulceration ~n~ene, etc. Associated hypertensive ischaemic ulceration - In patients with hypertension of long duration, ulceration of skin results from insufficiency of small ‘arteries or arterioles; more common in women of age 45-70 years; lesions usually in skin of legs but sometimes in the upper limbs. Signs

A systolic murmur may be heard over the abdominal aorta or iliac arteries. Pain is relieved by the dependent position which initially causes the limb to flush red and then become cyanotic. Elevation of the limb causes blanching and increased pain. Absent or diminished pulses, reduced skin temperature, and coldness of the limb are characteristic.

Laboratory Findings Arteriography

demonstrates

the level of arterial obstruction

or obstructions.

S126 Usual course

May gradually progress so that the patient can walk less far. Sudden progression indicates acute occlusion of main or collateral arteries.

Relief

Relief may be provided by sympathectomy relieved by this technique.

Complications

Ulceration, gangrene.

Pathdm

Claudication intermittens is a symptom that always indicates an inadequate supply of arterial blood to contracting muscle. Atherosclerosis is usually the underlying condition.

Aetiology

May be due to (a) arteriosclerosis, characterized by local depositton of fat under and within the intima of arteries, most commonly the aorta, coronary, cerebral arteries; (b) arteriosclerosis obliterans, an obstructive late stage of athero sclerosis characterized by partial or complete occlusion of arteries by atheromata, often with superimposed thrombosis: more common in men, involves large vessels such as aorta and arteries of lower limb; or(c) Monckeberg’s medial calcification and sclerosis, much more common in men: patients usually over 50 years of age. Changes confined to muscular media of medium-sized arteries, e.g., radial or ulnar, leading to nodularities around the artery. Only rarely leads to obstruction of arteries. Intermittent claudication and rest pain are more benign with this type of arterial disease than with others.

for rest pain; claudicatron is less often

Essential Features Exercise-induced pain which passes off very quickly by rest. Signs of arterial or arteriolar insufficiency. Differential Diagnosis Neurogenic claudication inter-mittens. Arterial or arteriolar vascular insufficiency by other conditions like entrapment syndromes, arteriospastic or arteritic conditions. Code

224.X& (arms) Intermittent 624.x& (legs)

claudication

222X8b (arms) Rest pain 622.X8b (legs) References

Silva, D.A. and Widmer, Publishers, Bern. 1978.

L.K.. Peripheral

Venous

Disorders,

Hans Huber

Juergens, J.L., et al., Peripheral Vascular Diseases, 5th edition. W.B. Saunders Co., Philadelphia, 1980.

s127

GROUP XVI: PAIN IN THE LIMBS OF PSYC~OLOGIC~ (XVI-l)

ORIGIN

General Descriptions See section I-16 Pain in the limbs of psychological origin is rarely considered to be due to muscle tension. Delusional or conversion pain in these (and other) locations may be more common on the left. Recurrent or chronic limb pain due to inapprop~ate use of muscle groups whether or not for psyc~i~c~ reasons may be quite common. Code

233.X7b 633.X7b 21X.X9a 61X.X9a 21X.X9b 61X.X9b

References

Merskey, H. and Watson, G.D., The Iaterakzation of pain, Pain, 7 (1979) 271-280.

(tension: upper limb) (tension: lower limb) (delusional: upper limb) (delusional: lower limb) (conversion: upper limb) (conversion: lower limb)

Hall, W. and Clarke, LM.C., Pain and laterality in a British pain clinic sample, Pain, 14 (1982) 6366.

s128

GROUP XVII: CHEST PAIN

Post-Infectious and Segmental Peripheral Neuralgia (XVII-3) Definition

Paroxysmal pain in the distribution of an intercostal nerve commonly associated with cutaneous tenderness in the affected dermatome.

Site

In the distribution of spinal nerve roots or trunks (if segmental neuralgia); in the distribution of the intercostal nerves; or in the distribution of the posterior primary division of the nerve trunk (if peripheral neuralgia).

System

Peripheral nervous system.

Main Features

Quality --- sharp or burning pain, usually intermittent; often precipitated by lateral movements of trunk or vertebral column. Associated with tenderness at points of exit of the nerve from a deep to a more superficial plane, e.g., at mid-ax&r-y or parasternal lines. Post-traumatic intercostal neuralgia often has continuous pain with exacerbation.

Aetiology

Neuralgic pains may be due to post-infectious radiculitis, osteoarthritic spurs, other spinal lesions, trauma, toxic and metabolic lesions, etc. In acute cases thgi are most often precipitated by upper respiratoy infection or trauma. In chronic cases bad body mechanics, lordosis or scoliosis, trauma and arthritis are the most common causes. Primary neuralgia occurs rarely.

Differential Diagnosis From neuralgias attributable to specific causes, or described above Code

306X2 post-infecttous 306.X8 unknown 306.X1 or 303.Xla (post-traumatic)

Angina Pectoris (XVII-4) Definition

Pain, usually constricting, and a heavy feeling in the chest, related to ischaemia of the myocardium without myocardial necrosis.

Site

Pain classically is in the precordium, although radiation to the arms and hands is common, particularly to the medial aspect of the left arm. Pain may also radiate up into the sides of the neck or jaw, or into the back, or epigastrium.

System

Cardiovascular.

Main Features

Prevalence - common in middle and older age groups, males more than females; Qualify - the pain tends to be dull, crushing or constricting and heavy. It is frequently precipitated by stress, either physical or psychofo&al. It usually lasts a few minutes but can be prolonged or intermittent, lasting hours or occasionally longer.

Sl29 Associated

Symptoms As noted, pain is aggravated by stress and relieved promptly by rest or nitroglycerin. Frequently patients also experience breathlessness, sweating, nausea, and belching.

Signs and Laboratory

Findings

Frequently there are no objective findings but patients may at the time demonstrate a tachycardia, a mitral regurgitant mu~ur of papillary muscle dysfunction, an S3 or 54, and reversed splitting of the second heart sound. Laboratory testing may show characteristic electrocardiographic changes, most commonly ST segment depression. Between pains, a stress electrocardiogram may show ST depression with exercise. Coronary angiography may show typical atherosclerotic narrowing of the coronary arteries. UsuaI Course

Anginal pain typically is brief and intermittent, brought on by exertion or stress and relieved by rest and nitr~ycerin. It may remain stable over many years, or may become “atypical” or accelerate to “preinfarction (or “unstable”) angina”.

Complications

Arrhythmia and myocardial infarction may occur.

Social and Physical Disability If angina is brought on by little extra stress, there is serious reduction in the work capacity. If the patient is particularly fearful, angina can cause interruption of normal psychological function as well. The big concern is usually fear of progression to sudden death or myocardial infarction, though limitation of activity level may also be a serious threat. Patholw

Summary

A list of risk factors predisposing individuals to atherosclerotic heart disease continues to develop but includes age, sex, hypertension, smoking, family history, hyperlipidaemia, obesity, sedentary life style, diabetes, etc. Superimposed on atherosclerotic coronary artery narrowing, such factors as increased cardiac oxygen demand, decreased flow related to coronary artery spasm or arrhythmias may be cont~buto~. of Essential Features

and Diagnostic Criteria

Crushingretrosternal chest pain brought on by stress (physical or psychological) and relieved by rest and nitroglycerin, with ST depression on E.C.G. but no evidence of infarction on sequential E.C.G.s and cardiac enzymes. Code

324.X6 224.X6 if mostly in the arms.

Myocardial Infarction (XVII-5) Definition

Pain, usually crushing, from myocardial necrosis secondary to ischaemia.

Site

Retrosternal

System

Cardiovascular.

area with radiation to arms, neck, jaw, epigastrium.

s130

Main Features

Associated

freuaience ..- the pain severe and slowly over

-..common in middle and older age groups, especially maies; Quality is dull, central, retrostemal, and heavy or crushing. Usually it is very lasts several hours or until relieved by morphine. It may diminish a day or two.

Symptoms Breathlessness, sweating, nausea, and vomiting, apprehension ness are common.

Si

and Laboratory

and lightheaded-

Findings

Physical examination may be normal but may show hypertensron, S3 or S4 gallop rhythm, and papillary muscle dysfunction with a mitral regurgitant murmur, as well as signs of forward or backward cardiac failure. Laboratory abnormalities include elevation of cardiac enzymes such as C.P.K., L.D.H. and S.G.O.T., classical sequential E.C.G. changes (ST elevation and the development of Q waves), and abnormal ra~onucljde heart scan.

usual

course

Complications

In patients surviving myocardial infarction the severe pain tends to diminish and disappear over several hours to a day or two. Often the patient is then pain free, although recurrent pain may represent angina or reinfarction. Sudden cardiac post-myocardial rupture, mural thrombosis and

death, arrhythmias, congestive heart failure, cardrogenic shock, infarction syndrome, pericarditis, septal perforation, valve cusp thrombus and embolism, myocardial aneurysm, deep vein pulmonary emboliim.

!Sociai and Physical Disability Myocardial infarction is a major cause of death and disability. Recovery frequently takes several months and physical and psychological complications may prolong recovery and affect not only the patient but family members, friends, and employers. The significance of the heart as the source of life makes inte~retation of this type of pain particularly threatening. Pathokgy

The main pathogenic process is atherosclerosis of the coronary arteries. Other factors such as coronary artery spasm or arrhythmias, or decreased blood volume, or decreased total peripheral resistance may also be significant as “last straws”. The risk factors mentioned with angina -.- sex, age, hypertension, smoking, Type A personality, etc., are important predisposing factors.

Differential Diagnosis Angina pectons, Dissecting aneurysm, pulmonay hiatus hernia, and pericarditis. Summary

of Essential Features

and Diagnostic

embolism, esophageal spasm,

Criteria

Crushing retrostemal chest pain with myocardial E.C.G. and enzyme changes. Code

321.X6 221.X6 if in the arms

necrosis as evidenced

b=y

s131

Pericarditis (XVII-6) Definition

Pain, often sharp, arising from inhalation

Site

The pain is classically in the precordium but may radiate through to the midthorax posteriorly or follow the pattern of angina, or to the superior border of the trapezius muscles.

System

Cardiovascular.

Main Features

Most cases are acute and this is particularly true of pericarditis causing pain. Pain may be severe and pleuritic. It may be aggravated by swallowing. It may be steady, crushing sub-sternal pain. It may occasionally be synchronous with the heart beat.

Associated

of the pericardium.

Symptoms Weight loss, fatigue and fever are common especially in chronic cases.

Signs and Laboratory

Findings

Leaning forward may reduce the pain. The hallmark sign is a triphasic pericardial friction rub. If there is a significant effusion, heart sounds may be decreased and a paradoxical pulse may be elicited. Laboratory signs include a “water bottle” confi~ration on chest x-ray if there is an effusion, as well as changes in fluoroscopy, echocardiography or angiography. Even without an effusion the electrocardiogram may show typical changes, ST elevation, or inverted T waves.

usual

course

Complications

The course varies depending on the aetiology and may range from being acute to chronic. May interfere with cardiac output.

Social and Physical Disabilities. Probably only significant in chronic cases where weight loss and generalized debility are part of the syndrome. Aetiology

Summary

A wide range of aetiologies can cause pericarditis and its subsequent pain. The most treatable causes are infections, collagen, vascular, and reinduced effects. Post-myocardial injury is also an important aetiology. of Essential Features

and Diagnostic Criteria

Sharp retrosternal pain aggravated by breathing and relieved by leaning forward, with auscultation revealing a friction rub, E.C.G. showing ST elevation or T wave inversion and echocardiogram showing an echo-free pericardial space. Differential Diagnosis Angina, myocardial infarction, pulmonary phageal spasm, etc. Code

323.X2 323.X3 323.X1 323.X4 323.X5

Known infection Unknown infective cause Trauma Neoplasm Toxic

embofism, hiatus hernia, and eso-

SK32

Aneurysm of the Aorta (XVII-7) Definition

Pain from an abnormal widening of the aorta

Site

Central chest pain which may radiate to the mid-scapular regon

System

Vascular.

Main Features

Deep, diffuse, aching central chest pain is associated with large aneurysms. dissection occurs, sudden and severe pain occurs, maximal at onset.

Associated

if

Symptoms Acute congestive heart failure may develop.

Sii

and Laboratory

Findings

A discrepancy may develop between pulses or blood pressures in the two arms. A new aortic regurgitant murmur may develop. A neurological impairment may develop. Chest x-ray may show widening of the superior mediastinum. Aortography may demonstrate a fake lumen. usual

course

Complications

If there is a large aortic aneurysm, there can be chronic dull, central chest aching. If dissection occurs, an acute medical and surgical emergency has developed. Chronic complications include pain and emboli. Acute complications include acute aortic valvular incompetence, occlusion of major vessels, hypotension and death.

Social and Physical Diibiky The main problems with aortic aneurysms Pathology

are life and death considerations.

“True” aneurysms involve aft three layers --. intima, media and adventitia. “Fake” aneurysms involve disruption of the inner and medial segments so that the wall of the aneurysm consists only of adventitia and/or perivascular clot. In the past, syphilis was the main cause. Cystic medial necrosis is a major cause of dissection. Arteriosclerosis is a major cause. Occasionally Marfan’s syndrome is the cause. Hypertension is important and so is trauma.

Summay

of Essential Features

and Diagnostic

Criteria

A rare cause of chronic chest pain with a wide superior media&urn on chest x-ray. A dramatic cause of excruciating acute pain with importance because of medical and surgical therapies available. Differential Diagnok Angina, pulmonary Code

diseases, and thoracic disc disease.

322.X6 (chronic aneurysm)

s133

Diseases

of the Diaphragm

(XVII-8)

Definition

Pain from the diaphragm related to irritation of the diaphragmatic nerves by a disease process above the diaphragm, in the diaphragm (rare) or below the diaphragm.

Site

Diaphragmatic pain is deep and difficult to localize. Noxious stimulation may affect phrenic nerve sensory fibers C3, C4 and C5 and therefore is often felt at the shoulder tips and along the upper border of the trapezius muscle, or it may affect the intercostal nerves T6, T7, T8 and T9 with radiation of pain into the anterior chest, the upper abdomen and the corresponding region of the back.

System

The system is musculoskeletal, the disease.

Main Features

The pain is deep, dull, poorly localized, and nonspecific, if it involves only the central chest and upper abdomen and upper back, but becomes better identified if there is shoulder tip radiation as well.

Associated

cardiac, pulmonary, or intestinal depending upon

Symptoms These symptoms depend most on the underlying pathology, so that if there is pulmonary pathology, respiratory symptoms may be prominent. Likewise, if the basic disease is gastrointestinal or subphrenic, gastrointestinal complaints are most likely associated. Hiccoughs may be present.

Signs and Laboratory

Findings

Frequently there are no physical findings but if there are, the most classic would be elevation of a hemidiaphragm. Laboratory testing might show elevation of the diaphragm on chest x-ray, abnormal movement of the diaphragm on fluoroscopy, a pleural effusion on chest x-ray, an echo-free space on abdominal ultrasound, a space between liver and heart on radio-nuclide imaging, or a space beneath the diaphragm on CT scan or nuclear magnetic resonance scanning. Usual Course

There is usually a specific therapy once the aetiology is determined, considerable time may elapse before a conclusive diagnosis is reached.

Complications

Depend on the underlying cause.

but a

Social and Physical Disability These relate partly to the underlying disease process and partly to the vagueness of understanding of the cause of pain. This latter can be extremely frustrating to doctor and patient alike. Aetiology Summary

Although a wide range of causes can cause disease affecting the diaphragm, the most important are infections and neoplasms. of Essential Features and Diagnostic Criteria Abdominal pain in epigastrium with radiation to central chest, posterior midthorax and shoulder tip(s), with evidence of space-occupying lesions above or below the diaphragm.

S134 DifferentialD&n&s Involves a wide range of cardiac, intestinal uses. Code

423.X2 423.X4 433.X2 453.X2 453.X4a 453.X6

pulmonary,

musculoskeietal.

and gastro

Infection: chest or pulmonary source Neoplasm: chest or pulmonary source Musculoskeletal Infection: gastrointestinal source Neoplasm: gastrointestinal source Cholelithiasis

Fracture or Coilapse of Thoracic Vertebrae (XVII-9) Definition

Thoracic pain or dermatomal

Site

Fractures with mild compression that produce severe compression regions.

Other Features

(as in IX-4 and XXVII.7 and 8)

Code

303.Xlb

pain or both associated with fracture of a vertebra.

of nerve roots produce localized pain. Those cause pain also in the root distribution of these

Sipping Ftibsymtome (XVII-10) (alsoknown as Clicki Rib !Sy&rome and Rib Tip Syndrome) Definition

Chronic pain at the costal margin which may mimic visceral parn

Site

8th, 9th or 10th rib cartilages, one or more rib cartilages being involved. The condition may be bilateral.

Main Features

Freucdence - Fairly common; Age of Onset -. 1560 years. More common in females (3F:lM); Qua/i& - A constant dull ache or a sharp stabbing pain which may itself be followed by a dull ache; Time Pattern - The pain may last from several hours to many weeks and some patients have constant pain.

Awavating

Factors Movement, especially lateral flexion and rotation of the trunk. Rising from a sitting positiin in an arm chair is often a particularly painful stimulus. Manipulation of the affected rib and its costal cartilage will exactly reproduce the presenting pain.

SigllS

usual

course

Some cases may resolve spontaneously permanently.

but most patients

have symptoms

5135 Relief

Resection

Complications

Depression and anxiety. Patients may be misdiagnosed and undergo unnecessary investigations and even inappropriate surgical procedures.

of movement

may give relief.

Social and Physical Disability Physical activities exacerbation.

by pain or fear of provoking

No specific histological changes identified. Cause of pain is presumed i~tation of intercostal nerve by adjacent h~~obiie rib cartilage.

Pathology Summary

are often restricted

of Essential Features

an

to be

and Diagnostic Criteria

A fairly common condition which should be considered in any patient complaining of upper abdominal pain. The diagnosis is clinical and should be made only when the patient’s symptoms are exactly reproduced by manipulation of the appropriate rib or ribs. An intercostal nerve block with local anaesthetic may produce confirmatory evidence where the clinical findings are equivocal. Treatment

Reassure patient - this may be sufficient for some patients who do not have severe pain. If severe pain persists, then the offending costal cattiIage should be excised.

Differential Diagnosis Biliary tract pathology, duodenal and gastric ulceration. Code

333.X6

References

Copeland, G.P., Machin, D.G., and Shennan, J.M., Surgical treatment slipping rib syndrome, &it. J. Surg., 71 (1984) 522-523.

of the

Holmes, J.F., Slipping rib cartilage, Amer. J. Surg., 54 (1941) 326338.

Post-Mastectomy Pain: Acute and Sub-Acute (XVII-11) Definition

Acute and semi-acute pain following extensive mastectomy or modified radical mastectomy.

Site

Axilia, lateral upper chest wall, upper, inner arm.

System

Skin and peripheral nervous system.

Main Features

Burning pain in the axilla and upper lateral chest and medial aspect of the arm, due to complete transection or neurapraxia of the intercosto-brachial nerve. Sometimes worse on light touch to the skin. Intercosto-brachial neruepain with paraesthesiae or anaesthesia occurs after this type of surgery and lasts up to three months but seldom persists and is not usually a problem. ~ncisiona~~in is minimal following breast surgery because of the hypoaesthesia that develops after the flaps are mobilized.

breast surgery, either radical

s136

Associated

Symptoms Dysaesthesia in the distributron of the intercosto-brachial allodynia or other evidence of partial innervation is found.

Pathology

rww

$tnetimes

Nerve trauma or transection

Diagnostic

Criteria Burning pain and hyperaesthesia in the area of the intercosto-brachial following extensive breast surgery.

nerve.

303.XlC

Code

Post-Mastectomy Pain: Chronic Non--t

(XVII-12)

Definition

Chronic pain commencing immediately or soon after mastectomy anatomical area on the anterior thorax.

Site

Anterior thorax, usually one side only.

System

Central nervous system (psychological and social).

Main Features

Prevalence - infrequent; Age -..- 40 _60 years; Sex Ratio - more common in females; Quality --. often burning, intensified by touch/clothing, no response to stethoscope pressure; gently picking up the skin produces an increased response; Time Pattern ..- constant/unremitting to analgesics; Intensity -years. moderate to severe; Duration

Associated

in a non

Symptoms The patient may be unable to tolerate a prosthesis, clothing or touch. Emotional lability or depression or episodes of crying may occur and sexual encounters are avoided.

Signs

Increased response to touch; hyperaesthesia to pin prick, or patchy anaesthesia, may occur. Non-anatomical distribution; absence of sternal/rib tenderness; and trigger point tenderness present.

UsUal course

May remain intractable to physical measures. triptyline and psychotherapy.

Complications

Can be com~unded

Commonly

by emotional stress, recurrence

responds

to Ami-

of disease.

Social and Physical Disability Impairment of social&accupation~sexual Pathology Summary

activities.

None known.

of Essentkd findings

and Diagnastic

Criteria

Intractable pain, onset postoperatively, in anterior hemithorax, present more than one month, with superficiil local sensitivity to touch, and non-anatomical neurological findings. Poor motivation to flexion of the arms against resistance. More common on the non-dominant side.

s137 Differential Diagnosis

Herpes zoster, local infection, radiation necrosis in ribs, incisional neuralgia (intercosto-brachial nerve syndrome of acute and sub-acute post-mastectomy pain), and neuromata.

Late Post-Mastectomy Pain or Regional Carcinoma (XVII-13) Definition

Shooting, jabbing or burning pain commencing more than three years after the initial treatment for cancer of the breast and due to local metastases.

Site

Spine, thorax at site of cancer, arms.

Main Features

Prevalence - Fairly common; Age - 35 and upwards; Sex Ratio - most common in females; Quality - varies according to aetiology. With skeletal secondary deposits, pain is increased with movement. Shooting or jabbing pain occurs with brachial plexus lesion, usually spontaneously, sometimes with paraesthesiae. Burning, shooting and numb feelings are found with brachial plexus damage from radiation; h-density - moderate to severe; Duration - less than 12 months, due to short life expectancy.

Associated Symptoms

Weakness

and reduced range of movement of the ipsilateral limb.

Signs

Ipsilateral Horner’s syndrome, lower brachial plexus signs, e.g., anaesthesia, muscle weakness and wasting, chest wall or axillary or supraclavicular recurrent disease. Bony tenderness to percussion.

Usual Course

With skeletal secondaries and brachial plexus damage, the course is usually progressive deterioration. However, with radiation damage to the brachial plexus, the course is more protracted, with onset more than five years after treatment and long survival.

Complications

Patients with skeletal, visceral, and brachial plexus damage have a short survival of less than one year. Radiation damage is a progressive disorder with disability and long survival.

Social and Physical Disability

Moderate impairment of social and occupational activity, with depression related to chronic illness. Pathology

Local skin, subcutaneous, skeletal or visceral metastatic disease; with recurrent disease there is local lymphatic spread, and extradurai and brachial plexus involvement. Radiation damage to the brachial plexus is more common in patients who have received repeated or excessive doses of radiation, and in such patients, telangiectasia may be present in the skin with pigmentation and signs of radiation arthritis.

S138

Summary

of Essential Findings and Diagnostic Criteria Chronic pain of cutaneous, visceral, skeletat or neurogenic pattern ansing more than three years after the initial diagnosis of cancer of the breast. associated with classical signs of lymphadenopathy, neurological findings. weight loss and anaemia. Neurogenic pain of radiation or brachial plexus infiltratio:l :S c-ommonly refractory to narcotics.

Differential Diagnosis Iierpes zoster, pleurisy related to infection, and second tumour, e.g.. Pancoast’s tumour with brachial plexus involvement, Code

307.x4

Post-Thoracotomy

Pain Syndrome (XVII-14)

Definition

Pain that recurs or persists along a thoracotomy following the surgical procedure.

Site

Chest wall.

Systems

Skeletal and nervous system.

Main Features

Pain following thoracotomy is characterized by an aching sensation in the ~~tibution of the incision. It usually resolves in the hue months following the surgery. Pain that persists beyond this time or recurs may have a burning dysesthetic component. There may also be a pleuritic component to the pain. Movements of the ipsilateral shoulder make the pain worse.

Associated

scar at least two months

Symptoms If the thoracotomy was done for tumour resection and there was evidence of pleural or chest wall involvement at the time of surgery, it is likely that the pain is due to tumour recurrence in the thoractomy scar.

S&s

and Laboratory

Findings

There is usually tenderness, sensory loss and absence of sweating along the thoracotomy scar. Auscultation of the chest may reveal decreased breath sounds due to underlying lung consolidation or a maliant pleural effusion. A specific trigger point with dramatic pain relief folloGng local anaesthetic injection suggests that the pain is benign in nature and due to the formation of a traumatic neuroma. A CT scan through the chest is the diagnostic procedure of choice to establish the presence or absence of recurrent tumour. usual

course

Complications

If the pain is due to traumatic neuromata, it usually declines in months to years and can be relieved by anti-depressant-type medications and anticonvulsants. If the pain is due to tumour recurrence, some relief may be obtained by an intercoastal nerve block or radiation therapy. Immobility of the upper extremity because of exacerbation of the pain may result in a frozen shoulder. Aggressive physiotherapy is necessary to prevent this complication.

Pathology

Summary

s139 For benign disease, the pathology is that of neuroma formation. If there is an underlying malignancy, there is tumour infiltration of the intercostal neurovascular bundle. of Essential Features and Diagnostic Criteria Persistent or recurrent pain in the distribution of the thoracotomy scar in patients with lung cancer is commonly associated with tumour recurrence. CT scan of the chest is the diagnostic procedure of choice to demonstrate this recurrence.

Differential Diagnosis Epidural disease and tumour in the perivertebral region can also produce intercostal pain if there is recurrent disease following thoracotomy. Code

303.Xld (neuroma) 333.X4 (metastasis)

s140

GROUP XVIII: CHEST PAIN OF PSYCHOLOGICAL

ORIGIN

Muscle Tension Pain (XVIII-l) Definition

Virtually continuous pain in the thorax, due to sustained muscle contraction and related to emotional causes.

Site

Either symmetrical. more often in the posterior thoracic region or precordial.

System

Central nervous system (psychological and social).

Main Features

Tension pain is rare in the posterior thoracic region compared with tension headache (perhaps one-tenth or less of the frequency of the latter). Precordial pain is more common, often associated with tachycardia or a fear of heart disease. The exact prevalence is unknown. The other features of these pains are the same as for muscle tension pain in general (III-l. 2). See also I-16.

Code

333.X7b

Delusional Pain (XVIII-2) See the general description of delusional pain (I-16). Perhaps more frequent in the precordial region. Code

31X.X9a

Conversion Pain (XVIII-3) See the description of conversion pain in general (I-16). Most frequent in precordium; may be associated with tachycardia and fear or conviction of heart disease being present. Often mimics angina, but without adequate evidence of organic disease. Differential Diagnosis As for conversion pain in general and angina pectoris.

Code

31X.X9b

s141

GROUP XIX: CHEST PAIN: REFERREZDFROM ABDOMEN OR GASTROINTESTINAL TRACT

Subphrenic Abscess (X1X-l) Defmitian

Pain, often referred diaphragm.

Site

Upper abdominal pain and tenderness may be present.

System

Gastrointestinal.

Main Features

Deep, dull and often poorly localized pain in epigastrium with tenderness beneath the rib margin. Shoulder tip pain often occurs also. Often follows intra-abdominal surgery, especially with perforated viscus. May follow closed blunt trauma.

Associated

to the shoulder,

from a collection

of pus under

the

along the costal margins. Shoulder pain

Symptoms Fever, malaise, weight loss, hiccoughs.

Signs and Laboratory

Findings

These may be vague. The patient may be febrile and cachectic. There may be a pleural effusion or lack of diaphragrnatic movement. There may be tenderness to percussion or to palpation of the upper abdomen. White blood cell count and erythrocyte sedimentation rate may be elevated. Chest x-ray may show pleural effusion or elevated hemi-diaphragm. Abdominal ultrasound or CT scan may reveal the collection of pus. Usual Course

Treatment with antibiotics with or without surgery usually leads to resolution. There may be a prolonged phase of pre-diagnosis.

Complications

Prolonged fever and weight loss. May lead to death. Chronic pain. Septic shock.

Social and Physical Disability May lead to usual effects both of chronic sepsis and chronic pain. Aetiology

Most common organisms are E. co& non-soup ~ebsiella-entero~cter and anaerobes.

A streptococci,

staphylococci,

Differential Diagnosis A wide range of upper gastrointestinal Summary

of Essential Features and Diisnostic

Criteria

Chronic illness often after abdominal often with shoulder tip radiation. Code

353.X2 (thorax) 453.x2 (abdomen)

diseases.

surgery with fever and abdominal pain,

5142

Herniated Abdominal Organs (XIX-Z) Definition

Pain related to the protrusion of an abdominal containing walls of the abdomen.

Site

Pain can be related either to the organ herniating or the wails of the orifice. For hiatus hernias the main pain is epigastric.

System

Gastrointestinal

Main Features

Burning epigastric pain (or retrosternal lying recumbent.

Associated

organ throuyh

IIW norm4

pain, or both), often followtng eating or

Symptoms The patient may also complain of chest pain similar to angina, right upper quadrant abdominal pajin similar to that in cholelithiasis, epigastric pain like that in peptic ulcer disease, abdominal bloating and air swallowing.

Signs and Laboratory

Findings

There are usually no physical findings. Radiographic techniques will show evidence of abdominal viscera in places they are not supposed to be, such as gastric mucosa above the diaphragm or colon above the diaphragm. Usual Course

Pain typically is intermittent and aggravated by certain foods, aspirin, alcohoi, bending over or straining, abdominal pressure or tight clothing, and carbonated beverages. Likewise, more esophageal reflux may occur with caffeine or nicotine which relax the lower esophageal sphincter.

Complications

Pain and Qastrointestinai upset

Social and Physicai Disabiii May lead to chronic complaint, usually not too severe. Aetiolosy Summary

Traumatic and congenital or degenerative weaknesses in the diaphragm are of key aetiologic significance although the exact cause is often obscure. of Essential Features and Diagnostic

Criteria

Epigastric discomfort and esophageal reflux are key symptoms with radiographic or endoscopic evidence of extra-abdominal organs. Differential Diagnosis Angina, cholelithiasis, acid-pepsin disease without hernias, and pancreatitis, etc. Code

355.X6 Thoracic pain 455.X6 Abdominal pain

s143

Oesophageal Motility Disorders (X1X-3) Definition

Attacks of severe pain, usually retrosternal and midline, due to a diffuse disorder of the oesophageal musculature with severe attacks of spasm and/or failure of relaxation of the cardiac sphincter.

Site

Pain is usually well localized to the midline behind the sternum, epigastrium and the suprasternal notch.

System

Gastrointestinal.

Main Features

Uncommon. Occurs in young adults and middle aged. Males and females equally affected. Sudden onset of pain usually sharp and stabbing, spasmodic and severe, at times excruciating, lasting from 30 seconds to a few minutes, and leaving a residue of retrosternal soreness. The bouts are usually infrequent. Air swallowing and belching are common and the pain is aggravated by swallowing.

between the

Associated Symptoms

Dysphagia occurs in patients with achalasia of the lower oesophageal sphincter. There is a sensation of the food sticking in the lower part of the oesophagus. With the aid of gravity the weight of the food causes the sphincter to open when the patient rises from the chair and the sticking sensation disappears. Relieving factors include smooth muscle dilatation agents such as glyceryl trinitrate or amyl nitrite, which may relieve the pain. Signs and Laboratory Findings

Patients usually point out their pain with one finger. Gastroscopy, barium swallow, tine-oesophagoscopy or oesophageal manometry may show evidence of increased or asynchronous oesophageal motility. A barium swallow may show disordered oesophageal contractions with or without ‘spasm’ or oesophageal dilatation. The cardiac sphincter may remain closed until a large amount of barium fills the oesophagus, when it will suddenly open. In patients with prolonged achalasia the oesophagus may contain foreign material which is undigested food. Oesophageal manometry will show disordered motility with a lack of normal peristalsis and occasional high pressure contractions or ‘spasm’. In patients with achalasia the cardiac sphincter will fail to relax normally following swallowing, although sphincter pressure is normal. Special pressure devices in the oesophagus for 24 to 48 hours may pick up very high pressure contractions, which may be related to the pain. Usual Course

Pain tends to be severe and episodic. It may vary from very occasional to cyclic or be continuous throughout the day. Anxiety and eating may aggravate it. Most patients with motility disorders run a benign course with occasional attacks of pain. Occasionally the symptoms progress to the point where the patient has to undergo active therapy. In contrast, patients with achalasia usually progress to the point where they require definitive treatment.

s144

Complications

If the pain is severe, it may lead to anorexia and weight loss. Vomiting may be a problem. Patients with achalasia can develop aspiration pneumonia from retained oesophageal contents. The incidence of oesophageal cancer in those patients is slightly increased.

Social and Physical Disability Severe pain may restrict normal activities and be socially disahluncl

Pathology Summary

This is mainly a physiologic rather than a pathologic problem. Stress may be an important contributing factor. There is frequently a positive family history.

of Essential Features

and Diagnostic Criteria

This syndrome consists of short attacks of acute severe retrosternal pain which may be relieved by nitrites, with or without dysphagia. The diagnosis is made with a combination of barium swallow appearances and disordered oesophageal motility and normal mucosal appearances on oesophagoscopy. Differential

Diagnosis Pericarditis, pulmonary embolism, angina pectotis, dissecting aneurysm, tertiary oesophageal contractions in the elderly, and carcinoma of the oesophagus.

Code

356.X7

0esQphagitis

(xIX-4)

Definition

Pain due to inflammation of the oesophageal mucosa.

Site

Retrosternal or epigastric pain, depending on the aetiology. e.g., upper oesophagus --- moniliai, lower oesophagus --- acid reflux.

Main Features

Common, especially in middle aged and obese. More common in women. Burning retrostemal pain, especially at night if lying flat, or on bending over. May last minutes or hours.

Associated

Symptoms Aggravated by very hot or cold drinks, acidic drinks, alcohol or strong coffee. Relieved by antacids or food.

Sii

and Laboratory

Findings

No physical findings. There may be iron-deficiency anaemla and positive occult blood tests.

Usual Course

Chronic, intermittent, rarely constant.

Compliitions

Chronic occult G.I. bleeding, stricture of lower oesophagus

Social and Physical Disability Unable to tolerate certain foods, unable to sleep flat in bed.

s145 Pathology

Summary

Peptic - Dysfunction of cardiac sphincter results in intermittent regurgitationof gastric acid contents into lower oesophagus when intragastric or intra-abdominal pressure is increased and aided by gravity. Monilial - Commonly secondary to immun~uppression and corticosteroids. of Essential Features

and Diagnostic Criteria

Burning retrosternal

pain from oesophageal inflammation.

355.X2 Monilial 355.X3a Peptic

Code

Reflux Oesophagitis

with Peptic Ulceration

(XIX-5)

Definition

Retrosternal ulceration.

Site

Typically retrosternal far as the neck.

System

Gastrointestinal

Main Features

Common in young adults and middle age group, starting in third decade. Commoner in females especially in the obese or during pregnancy. Bouts of pain occur often after postural changes such as bending over or lying down. They also may be associated with a sour taste or waterbrash. Attacks are usually mild except with ulceration where they are very severe and last minutes to hours. With ulceration, pain may be continuous.

Associated

burning chest pain due to acid reflux causing inflammation

and

midline pain radiating from behind the xiphisterum up as

(oesophageal mucosa).

Symptoms Sour taste, waterbrash.

Ag~avating

Factors Certain postures such as bending over, sitting in a slumped position or lying down; very hot or cold drinks; acidic drinks. Relieved by antacids.

Signs and Laboratory

Findings

The only abnormal findings are appearances of oesophagitis (reddening or haemorrhagic mucosa) or of actual ulceration on oesophagoscopy. Oesophageai motility studies may show a decrease in cardiac sphincter pressure, a pH probe may detect acid reflux, and the pain may be reproduced by the infusion of .l N hydrochloric acid proximal to the cardiac sphincter. Usual Course

In the majority of patients the symptoms persist intermittently for years. In pregnant women they usually disappear after childbirth except in the obese patients.

Pathology

Changes in the lower oesophageal mucosa may vary from the mildest changes with blunting of the rete papillae to severe haemorrhagic inflammation with ulceration and loss of mucosa.

S146 Complications

Patients with ulceration may develop a stricture in the region ot the ulcer which can cause dysphagia. Rarely a malignancy may develop in the area of chronic oesophagitis.

Summary of Essential Features and Diagnostic Criteria Oesophagitis with nonmalignant ulceration presents with retrosternal pain especially on bending or lying down, or on drinking very hot or <,old fluids or acidic foods. The diagnosis is made on the history, oesophagoscopy and oesophageal motility studies. Differential Diagnosis Monilial oesophagitis, herpetic oesophagitis. foreign body in wall of oesophagus. Crohn’s disease.

Code

355.X3b

Carcinoma of Oesophagus (XIX-6) Definition

Pain due to malignant disease of the oesophagus resulting from malignant transformation of either the squamous epithelium of the upper oesophagus or the mucosa of the lower oesophagus.

Site

Retrosternal pain, extending sometimes to the back

System

Gastrointestinal.

Main Features

This is a relatively uncommon tumour in the Western World but has localised areas of high incidence especially in Iraq and Iran amongst the Kurds. Pain is not usually a prominent feature. The presenting symptom is usually dysphagia without pain which usually occurs only when the cancer extends beyond the oesophagus. At that point dysphagia and retrosternal pain may become continuous and radiate through the back.

Associated Symptoms Dysphagia is the major symptom; others include regurgitation and recurrent pneumonia. Signs and Laboratory Findings Evidence of weight loss and cervical lymphadenopathy, particularly deep to the sternomastoid. Chest x-ray may show a dilated oesophagus; barium swallow a narrowing of the oesophageal lumen; iron-deficiency anaemia. usual course

Unless the tumour is removed, the patient will become obstructed

Complications

Oesophageal obstruction, erosion into a bronchus, bronchoesophageal stricture, erosion into aorta with catastrophic haemorrhage.

Social and Physical Disabiiii If the tumour is inoperable and the patient cannot eat, a plastic tube can be passed through the tumour or a feeding jejunastomy performed.

s147 Pathology

Summary

Chronic ingestion of carcinogens in certain areas of the world, e.g., Kurdistan and Lake Victoria, East Africa. Smoking - chronic disorders of oesophagus, e.g., achalasia, Barrett’s oesophagus. of Essential Features and Diagnostic Criteria Presents with dysphagia with pain as a late feature. Diagnosed by barium swallow and oesophagoscopy with biopsy or cytology.

Differential Diagnosis Benign stricture, achalasia.

s148

GROUP XX: ABDOMINAL PAIN OF NEUROLOGICAL ORIGIN

Segmental or Intercostal Neuralgia (xX-3) See description of these conditions in thoracic section. Characteristics aetiology.

as for thoracic parn of similar

Differential Diasnosis Nso includes entrapment in rectus sheath or operative scars. Post-traumatrc pain often has continuous ache with paroxysmal exacerbations. Code

406X2 Post-infectious 406X8 Unkn~n 406X1 or 403.X1 Post-traumatic

TweIfth Rib Syndrome (xX-4) Definition

Chronic pain in the toin, sometimes with acute exacerbations groin.

site

Eleventh or twelfth rib. or both.

Systems

Skeletal and nervous systems.

Main Features

A fairly common condition that seems to occur more often in women than men (4:l). Patients usually develop the problem between the ages of 20 and 40years. There is not usually any history of trauma, but it may start during a pregnancy. The pain may take the form of a sharp pain or a dull ache, or a combi~tion of the two, (the initial lancinating pain being followed by a prolonged period of aching pain). Patients are rarely free from pain although the intensity vanes from time to time. An attack of severe pain can be bad enough to mimic ureteric colic. The sharp pains usually last for several hours and the subsequent dull ache subsides over a couple of days.

Aggravating

and radiation to the

Factors Certain movements, involving alternating flexion and extension of the spine, e.g.. using a vacuum cleaner.

Relief

Flexion of the spine (i.e., sitting forwards).

signs

Tenderness of the affected ribs. M~ipulation reproduce the patient’s syndrome.

of the ribs should exacrly

s149 Laboratory

Findings None diagnostic but a chest x-ray, intravenous help to exclude other causes of loin pain.

Usual Course

Pain continues indefinitely.

Complications

Depression.

urogram and spinal x-rays will

Social and Physical Disability Quality of life moderately or severely impaired. Patients usually limit physical activities lest they provoke an acute attack. Pathology Summary

No histological abnormality identified in ribs. It is assumed irritation of an intercostal nerve by the offending rib. of Essential Features

that the cause is

and Diagnostic Criteria

Loin pain, either intermittent or continuous and sometimes with radiation to the groin. Frequently misdiagnosed as pain of renal origin. Diagnosis is clinical and depends upon exactly reproducing the patient’s pain by palpation of the rib. Confirmatory evidence can often be obtained by using local anaesthetic to block the appropriate intercostal nerve, but a negative test would not necessarily exclude the syndrome. Treatment

Reassure the patient of the benign nature of the condition. Excise as much of the shaft of the rib as possible.

Differential Diagnosis Renal or ureteric pathology, spinal problems, pulmonary pathology. Code

433.x6a

Reference

Machin, D.G. and Shennan, J.M., Twelfth Rib Syndrome; a differential diagnosis of loin pain, Bit. Med. J., 287 (1983) 586.

Sk50

Abdominal Cutaneous Nerve Entrapment Syndrome (xX-5) Definition

Segmental pain in the abdominal wall due to cutaneous nerve entrapment in its muscular layers, commonly at the outer border of the rectus sheath or by involvement in postoperative scar tissue.

Site

Unilateral in the abdomen usually confined to a single dermatonw

System

Peripheral nervous system.

Main Features

Abdominal wall pain occurs which is sharp and burning in character, initially intermittent but often continuous later on. Characteristically, it is reproduced by localized pressure over the tender area with a finger tip. pencil head or similar object. With nerve entrapment in the rectus sheath, the pain occurs only when the abdominal wall is tensed, for example by asking the patient to raise head and neck off the examining couch. The diagnosis is frequently missed when the abdomen is relaxed, as it is for conventional examination. The onset is oAen unremarkable, but pain may be initiated by any sharp, twisting movement, repeated coughing or increased intra-abdominal pressure.

Relief

Differential

Relief is obtained immediately by injection of local anesthetlc into the trigger zone.

Diagnosis Serious intra-abdominal pathology, such as acute appendicitis, is normally not so prolonged over weeks or months. The pain of appendicitis is present even when the abdomen is relaxed and usually is associated with other well-known physical signs. Entrapment neuropathy may require distinction from other causes of segmental pain (see intercostal neuralgia). Pain of psychological origin, especially in young women, is another diagnostic alternative.

Code

433.X6b

s151

GROUP XXI: ~DO~NAL

PAIN OF VISCERALORIGIN

Cardiac Failure @XI-l) Definition

Dull aching pain from congestive failure.

Site

Pain from congestive abdominal quadrant.

System

Gastrointestinal. Pain is thought to be related to distension capsule. Bowel ischemia may also be a factor.

Main Features

Dull aching pain in association congestive heart failure.

heart failure is usually epigastric or in the right upper

of the hepatic

with a tender enlarged liver and other signs of

Associated Symptoms Dyspnea, increased abdominal girth, ankle oedema, decreased ance.

exercise toler-

Signs and Laboratory Findings Physical findings of congestive heart failure may include crackles on auscultation, elevated jugular venous pressure, hepatomegaly and occasionally a pulsatile liver, ascites, and oedema. An S3 and S4 gallop may be heard. Chest x-ray may show cardiomegaly and pulmonary edema. L.D.H., bilirubin, and S.G.O.T. may be elevated secondary to hepatic congestion.

Usual Course

This is variable depending on the treatability of the congestive failure. The pain may settle promptly with good medical management.

Complications

Long term this may result in “cardiac cirrhosis”.

Social and Physical Disability If prolonged, it may be part of a disability secondary to heart failure. Pathology

Passive congestion of the liver is the pathol~cal is usually coronary artery disease.

finding. The primary pathology

EssentialFactors Dull aching right upper quadrant and epigastric pain with a large tender liver and elevated liver enzymes in association with other findings of heart failure. DifferentialDiagnosis Hepatitis and diseases of the gallbladder. Code

452.X6

Sk52

Gallbladder Disease

(xX1-2)

Definition

Pain due to an inflammatory gallstones.

Site

Right upper quadrant,

System

Gastrointestinal;

Main Features

Common, especially in middle age except in ethnic minorities with high prevalence when younger age groups are also often affected (e.g.* some North American Indians). Much more common in women. Pain associated with passage of stone into the cystic duct is a severe colic, short lived with associated sweating.

disorder of the gallbladder usually ‘issoctated with

but also epigastrium and other parts ot the abdomen

gallbladder and bile duct

Associated Symptoms Anorexia, nausea and vomiting, jaundice, dark urine, pale stool. Relieved with antispasmodics or opiates. Dyspepsia with fatty foods. Signs and Laboratory Findings Tenderness in right upper quadrant. Neutrophil leucocytosis; hyperbilirubinaemia; elevation in serum transaminases and alkaline phosphatase. Evidence also from ultrasound and cholecystograms. usual

course

Resolves within 2 or 3 days unless stone impacts in common bile duct, causing obstructive jaundice.

Complications

Obstructive jaundice, mucocoele of the gallbladder, empyema of gallbladder with or without rupture.

Pathology

Gallstones may be cholesterol from lithogenic bile, pigment secondary to chronic haemolysis, or mixed.

Summary of Essential Features and Diagnostic Criteria Acute right upper quadrant ultrasound or cholecystogram.

pain, dyspepsia

DifferentialDiagnosis Hepatitis, renal colic, hepatic flexure syndrome. Code

456.X6

to fatty foods

Diagnosis

by

SK3

Post-Cholecystectomy

Syndrome

(XXI-3)

Definition

Right upper quadrant pain in patients following cholecystectomy.

Site

Right upper quadrant.

System

Gastrointestinal

Main Features

This pain is a common occurrence soon after the gallbladder has been removed, often with a short initial pain-free period. It is commoner in females. The pain is similar to ‘gallbladder’ pain, may be colicky in nature, daily, but not at night, may be dull or very intense lasting all day and may continue for months or years.

(external biliary tree).

Associated Symptoms

Nausea, occasionally vomiting. Aggravated by eating. Signs and Laboratory Findings

Tenderness in right upper quadrant laboratory tests.

in ‘region of the scar’. No abnormal

Usual Course

Chronic, unrelenting.

Complications

Risk of analgesic addiction or further unnecessary

surgery.

Social and Physical Disability Those of chronic pain and addiction. Summary of Essential Features and Diagnostic Criteria

Right upper quadrant pain in a patient following cholecystectomy with no obvious cause. Endoscopic retrograde cholangiography often reproduces the pain. Differential Diagnosis

Retained bile duct stone, hepatic flexure syndrome. Code

457.x1

s154

Chronic Gastric Ulcer (xX1-4) Definition

Attacks of periodic upper abdominal mucosa.

Site

Pain is generally rather diffuse over the central upper abdomen. any direction and occasionally through to the back.

System

Gastrointestinal.

Main Features

Can occur at any age but most common in the middle-aged and the elderly. Males and females are about equally affected although in some areas it is more common in females, e.g., Australia. Sudden onset of pain after meals from within one-half to two hours. Pain may be aggravated by eating, relieved by fasting or antacids. At first may be periodic and infrequent, every two to three months lasting for a few days.

Associited

pain due to ulceration

or the gastric

It may radiate in

Symptoms Anorexia and mild weight loss, often nausea, but vomiting is rare and associated with a prepylotic ulcer.

Signs and Laboratory

Findings

May be anaemic. Patient shows site of pain by pointing to diffuse area of upper abdomen with hand. Tender on palpation in that area. The diagnosis is made on endoscopy or barium meal (upper gastrointestinal series). Mikl iron-deficiency anaemia, or elevation of ESR or both may be found on blood examination. usual

course

Complications

Periodic pain becomes more frequent and perhaps severe and for longer duration until pain-free periods may disappear. Pain commonly responds to regular antacid and anticholinergic therapy and particularly to H2 receptor antagonists, but there is a high incidence of relapse. Gastric ulcers may bleed, usually chronically, presenting with iron-deficiency anaemia but occasionally acutely presenting with haematemesis and melaena; chronic ulceration leads to scarring so that prepyloric ulcers may cause obstruction with vomiting. Peptic ulcers may perforate, though usually insidiously, resulting in erosion into adjacent structures such as the pancreas. This causes localized but rarely generalized pancreatitis, or acute perforation with resulting acute peritonitis.

Social and Physical Disability Recurrent or chronic pain will restrict normal activities and reduce productivity at work. Pathology

Summary

Chronic ulceration with transmural inflammation results in localized fibrosis and cicatrization. of Essential Features and Diagnostic

Criteria

Chronic gastric ulcer is a syndrome of periodic diffuse post-prandial, upper abdominal pain relieved by antacids. The diagnosis is made by endoscopy or barium contrast radiology. Code

455.X3a

s155

Chronic Duodenal Ulcer (XXI-5) Definition

Attacks of periodic epigastric pain due to ulceration duodenal mucosa.

Site

Pain is classically localized to a spot high in the epigastrium, either central or under the right costal margin, and commonly radiates through to the back.

System

Gastrointestinal.

Main Features

Occurs at any age but commonly in young and middle-aged adults and is still more common in men. However, the incidence is less than 2:1, males to females. Commonly occurs when the patient is fasting, especially at night, and is relieved by eating or antacids. Periodic pain, which commonly lasts from a few days to two or three weeks, with pain-free periods lasting for months,

of the first part of the

Associated Symptoms Weight loss uncommon; patients may actually gain weight. Dyspepsia and often nausea occur but vomiting is uncommon. Signs and Laboratory Findings Patient often points to site of pain, which is also tender, with one finger. The diagnosis is made on endoscopy or barium meal (upper gastrointestinal series). Mild iron-deficiency anaemia and elevated ESR may occur. Rarely hypercalcaemia is discovered in association with hyperparathyroidism. Usual Course

Attacks of periodic pain may become more frequent Pain commonly responds to appropriate doses of promoted by H2 receptor antagonists. But there is a which can be considerably prevented by maintenance

Complications

Duodenal ulcers may acutely bleed or perforate.

and for longer duration. antacids and healing is high incidence of relapse doses.

Social and Physical Disability Restriction of normal activities and reduction of productivity at work. Pathology

Chronic ulceration with transmural and cicatrization.

inflammation resulting in localized fibrosis

Summary of EssentialFeatures and Diagnostic Criteria Chronic duodenal ulcer is a syndrome of periodic, highly localized, upper epigastric pain relieved by antacids The diagnosis is made by endoscopy or barium contrast radiology. Code

455.X3b

Carcinoma of the Stomach (XXI-6) Definition

Constant

upper abdominal pain due to neoplasm of the stomach.

s156 Site

Anywhere in the upper abdomen.

System

Gastrointestinal

Main Features

Uncommon, occuring predominantly in middle-aged and elderly patients but can occur in the third decade of life. There may be a past history of a gastric ulcer or partial gastrectomy 15 years or more previously. Pain varies from a dull discomfort to an ulcer-like pain, which is not relieved by antacids. to a constant dull pain

Associated

.

Symptoms Anorexia and weight loss early in the disease together with fatigue. The patient may present with acute gastrointestinal bleeding, haematemesis and or melaena. or signs of anaemia, e.g., fatigue, shortness of breath on exertion and even angina and swelling of the ankles. Later, symptoms of obstruction either at the pyiorus, with gastric distension and forceful vomiting, or at the cardia with dysphagia and regurgitation, may occur.

Sii

and Laboratory

Findings

Physical findings include those of obvious weight loss of cachexia, a palpable mass in the epigastrium and an enlarged liver. Laboratory findings are mainly of anaemia which may be microcytic due to chronic blood loss, normocytic due to chronic disease or macrocytic due to achlorhydria and even to underlying pernicious anaemia. Occult blood is commonly present in the stool. Hypoproteinemia is found, at times associated with a protein-losing enteropathy. Liver chemistry tests, especially alkaline phosphatase, will be abnormal in patients with hepatic metastases.

usual

course

Complications

If the patient presents early in the course of the disease the tumour may be resectable, although the chance of recurrence in the local lymph glands is high. There may be obstruction at the cardia or pylorus, or metastases In the liver or in more distant organs such as the lungs or bone, resulting in bone pain.

Social and Physical Disability Inoperable patients continue with anorexia and weight loss, become cache& and totally incapacitated.

Pathology Summary

The tumour is usually an adenocarcinoma. It may present as an ulcerating lesion or with diffuse infiltration of the stomach wall (linitus plastica)

of Essential Features and Diigno&ic

Criteria

Indefinite onset of anorexia, weight loss and fatigue in an elderly patient with vague upper abdominal discomfort developing into constant upper abdominal pain associated with anaemia. The overall prognosis depends on the stage of the tumour at the time of diagnosis, early resectable tumours having an excellent prognosis.

Differential Diagnosis Gastric ulcer.

Code

45XX4b

s157

Carcinoma of Pancreas (xX1-7) Definition

Chronic constant abdominal within the pancreatic gland.

Site

Central or paraumbilical or upper abdominal over the surface markings of the pancreas.

System

Gastrointestinal.

Main Features

Uncommon, occurring predominantly in older patients, i.e., average age 65 years but can occur in third decade of life. Pain can vary from a dull discomfort to, in the later stages, an excruciating severe pain boring through to the back, which is difficult to relieve with analgesics.

Associated

pain or discomfort due to neoplasia anywhere

Symptoms Generalized symptoms of fatigue, anorexia, weight loss, fever and depression occur early in the course of the disease. The patient may present with a sudden onset of diabetes mellitus late in life, without a family history, or with recurrent venous thromboses. Later symptoms include jaundice with pale stools and dark urine, pruritus, nausea and vomiting.

Signs and Laboratory

Findings

Evidence of recent weight loss and eventually cachexia are common. Jaundice and a central or lower epigastric hard mass are late findings and a palpable spleen tip is uncommon. Laboratory findings usually show normochromic normocytic anaemia with or without thrombocytosis, elevated fasting or two-hour postprandial blood glucose. Later, an elevated alkaline phosphatase and serum conjugated bilirubin may occur and the serum amylase may be slightly elevated. Usual Course

Only a minority of patients are operable at the time of diagnosis, i.e., from 20 to 40%. Only about 20% of those (i.e., 5 to 10% overal1) have a potentially curative resection with a four-year survival of about 40%, or 4% of the whole.

Complications

These include diabetes mellitus, obstructive jaundice, portal vein thrombosis, and small or large intestinal obstruction.

Social and Physical Disability The symptom complex with weight loss and generalized weakness is eventually totally incapacitating. Pathology

The tumour is usually adenocarcinoma.

Summary of Essential Features and Diignostic

Criteria

Indefinite onset of anorexia, weight loss and fatigue in an elderly patient with vague central abdominal discomfort eventually turning to severe constant pain with or without obstructive jaundice. The overall prognosis even with modern imaging techniques is poor. Differential Diagnosis Malignancy in other organs, stricture or impacted stone in the common bile duct. Code

453.x4c

SE8

Chronic Mesenteric Ischaemia (XXI-S) Definition

Intermittent central abdominal pain or discomfort related to Ischaemia of the large or small intestine.

Site

Central. peri-umbilical, occasionally radiating to the back.

System

Gastrointestinal

Main Features

Progressively severe abdominal pain precipitated by ingestion may progress to almost constant pain and fear of eating.

Associated

ois large

meal. It

Symptoms There may be symptoms suggestive of gastric or duodenal intermittent incomplete small bowel obstruction.

Signs and Laboratory

ulceration

01

Findings

There may be evidence of generalized atherosclerosis as shown by absent femoral popliteal or pedal pulses, or the presence of an epigastric bruit. No specific laboratory findings are diagnostic. Weight loss is associated with a severe form of this disease. Arter~~raphic evaluation indicates severe stenosis or occlusion ofall three mesenteric vessels, including the inferior mesenteric artery, the superior mesenteric artery and the coeliac axis. A meandering artery. indicating collateral blood flow to the colon, is a common finding. Usual Course

Progressive weight loss and abdominal pain if untreated. infarction of the small bowel may occur.

Sudden and complete

Social and physical Diibility This unusual problem may be part of a picture of generalized atherosclerosis in which case the patient may suffer from angina, cerebral vascular disease, or inte~ittent claudication. Pat hology

Summary

Patients with true mesenteric ischaemia show severe narrowing of all three mesenteric vessels by atherosclerosis, which leads to inadequate supply of blood flow to the gastrointestinal system. Atherosclerosis is usually isolated at the origin of these three vessels. of Essential Features

and Diagnostic Criteria

Mesenteric ischaemia may result in central abdominal pain, associated with ingestion of meals. When this becomes severe, weight loss results and sudden small bowel infarction may occur. Differential Diagnosis This rare disease is usually diagnosed abdominal pathology. Code

455.x5

by exclusion of other causes of intra.

s159

Crohn’s Disease (XXI-g) Definition

Pain due to chronic granulomatous

Site

Principally distal ileum and colon; less commonly anus.

System

Gastrointestinal: sometimes including liver. Other systemic involvement is principally skeletal muscle.

Main Features

Becoming increasingly common in young adults but can occur at any age; males and females affected equally; pain usually due to obstruction in the distal ileum with colicky central abdominal pain in bouts; or localized inflammation (abscess formation) may cause a constant severe pain. Both pains will persist until treated.

disease of the gastrointestinal

tract.

Associated Symptoms

Intestinal obstruction associated with distention, nausea and vomiting, alteration in bowel habit, constipation or diarrhoea or both, aggravated by eating, relieved by “bowel rest”. Localized inflammation associated with fever, anorexia and malaise. Signs and Laboratory Findings

Mass in right lower quadrant; sounds.

central abdominal

distension;

increased bowel

Usual Course

The symptoms in patients with Crohn’s disease will often settle with bowel rest (parenteral nutrition), with anti-inflammatory therapy, Salazopyrine, or Metronidazole with or without corticosteroids.

Complications

Strictures, fistulae.

Social and Physical Disability

A high proportion of patients with Crohn’s disease require surgery. Aetiology

Unknown.

Essential Features

Pain due to a chronic inflammatory granulomatous condition of the G.I. tract resulting in narrowing of the ileum and inflammatory ‘skip’ lesions of the colon. Differential Diagnosis

Small intestine Code

benign strictures; large intestine -

ulcerative colitis.

456.X3 (colicky pain) 452.X3 (sustained pain)

Chronic Constipation (XXI-10) Definition

Abdominal pain, usually dull, due to chronic alteration in bowel habit resulting in fewer bowel movements and diminished mean daily faecal output.

s160 Site

Left lower quadrant and upper abdomen.

System

Gastrointestlnnl.

Main Features

Common in any aye group but becoming increasingly common !:I rhe elderly. More common in women during menstruation, pregnancy and menopause. The pain is located over the cutaneous markings of the colon most commonly in the left lower quadrant and upper abdomen over the transverse colon The pain may vary from being constant and dull to sharp or very severe but it tlc’vpr prevenfs sleep. It may last all day, every day, with exacerbations associattsd hith eating; defaecation may bring partial temporary relief.

Associated Symptoms The pain may be aggravated by eating and relieved by detaecatlon. However certain high fibre foods such as vegetables and bulk laxatives failing to cause defaecation increase the pain, as do bowel irritants. Stool softeners can relieve the pain. Signs and Laboratory Findings The abdomen may be distended due to chronic distension: colonic faecal contents are palpable as well as the colon itself, especially the descending and transverse colon, which can be tender. The rectum may be full of hard faeces (rectal constipation) or empty, but with faeces palpable in the sigmoid colon on bimanual examination (sigmoid constipation). Usual Course

Unless the constipation is due to some correctable abnormality, such as carcinoma or a particularly poor diet, the course is usually chronic, i.e., continuous for years.

Complications

There is a suggestion on epidemiol~ic~ and experimental grounds that chronic constipation predisposes to diverticular disease and carcinoma. Neither is proven in humans. Faecal impaction, particularly in the elderly, can lead to large bowel obstruction or spurious diarrhoea.

Socii and Physical Disability Severe constipation, particularly in the elderly, can cause spurious diarrhoea resulting in faecal incontinence. PathoIogy

Chronic constipation is most closely related to diet. The We-stern World? highly refined low fibre diet predisposes to small stool weights and constipation, which is little known in Third World countries. Rarer causes include disorders of colonic muscle such as congenital megacolon, Hirschprung’s disease.

Summary of EssentialFeatures and Diagnostic Criteria Abdominal pain, usually dull, sometimes exacerbated by eatzng due to chronic constj~tion, which is largely a disorder of Western civilization and increases with age. The diagnosis is made from the history and physical examination. DifferentialDiagnosis Diverticular disease, carcinoma of colon Code

453.X7a

5361

Irritable Bowel Syndrome (XXI-11) Definition

Chronic abdominal pain of no apparent cause associated with alteration of bowel habit.

Site

Anywhere over the cutaneous markings of the colon but maximal on the left lower quadrant over the descending colon.

System

Gastrointestinal.

Main Features

Very common, maximum in second, third and fourth decades but onset at any age from first to eighth decade. Commoner in females with ratio varying from 2 to 5: 1. The pain varies from dull to very severe, often throughout the day with some fluctuations but never wakes the patient at night. It occurs daily throughout the year and ‘never misses a day’ often for many years. The pain is out of keeping with the patient’s physical condition.

Associated

Symptoms Nausea and vomiting but not anorexia or weight loss. There is always an alteration in bowel habit, either morning diarrhoea with 5 to 6 bowel actions followed by normal bowel action later in the day or chronic constipation. Pain aggravated by eating, occasionally by milk and smoking. It may be relieved by defaecation, lactose-free diet, and stopping smoking.

Signs and Laboratory

Findings

Tenderness over part of colon. Extremely tender on rectal examination and on sigmoidoscopy at rectosigmoid junction. Scars of previous surgery, e.g., appendectomy, cholecystectomy and hysterectomy common. Majority of investigations negative. Barium enema shows colonic spasm; a small percentage is lactose intolerant.

Usual Course

Chronic, often lasts for many years.

Complications

May predispose to diverticular disease, secondary neuropsychiatric ities.

abnormal-

Social and Physical Disability The pain can be incapacitating and result in deterioration in performance, relationships, etc. Pathology

social

Disturbance of motility throughout G.I. tract from oesophagus to anus. May be associated with increased sensitivity to G.I. hormones, e.g., cholecystokinin and to prostagiandins (in the chronic diarrhoeal patients).

Essential Features Usually there is a long history of constant abdominal pain and tenderness in young women; associated with alteration in bowel habit and no abnormal investigations. Differential Diagnosis Diverticular disease of colon. Code

453.X7b

S162

DiverticularDisease of the Colon (XXI-12) Definition

Pain, usually dull, arising in relation to multiple small sac-like proJectIons from the lumen of the colon through the muscular wall and beyond the serosal surface.

Site

The pain is most commonly in the left lower abdominal quadrant, related to the sigmoid colon, spreading more widely if the disease involves the whole colon.

System

Gastrointestinal

Main Features

The pain is not a common symptom in this very common condition. which rarely presents before age 40, but becomes increasingly common with age. Males and females are equally affected. Pain may be dull and chronic. recurrent in nature, associated with constipation or acute severe pain in the left lower quadrant, associated with acute inflammation (acute diverticulosis), and lasting one to two weeks.

Associated

Symptoms Chronic constipation, acute or chronic abdominal distension, rectal bleeding (in diverticulitis). Aggravated by chronic constipation. Relieved by high cereal fibre diet (e.g., bran).

Signs and Laboratory

Findings

Abdominal (colonic) distension. Palpable descending and sigrnoid colons with or without tenderness. Barium enema shows multiple diverticuli. usual

course

Complications

Chronic disorder with constipation as the main problem. Acute attacks of diverticulitis occur infrequently. Patients rarely require operative intervenfion for subacute obstruction. Acute diverticulitis, obstruction peridiverticular abscess.

with or without spurious diarrhoea, bleeding,

Social and Physical Diibitity Chronic constipation and spurious diarrhoea may lead to rectal incontinence. surgery is needed. a permanent colostomy may be required. Pathology

Summary

Hypertrophy of circular colonic muscle with penetration mucosa, connective tissue and the serosal surface.

ofEssential

Features

and D&no&k

If

by sacs consisting of

Criteria

A common chronic condition of the elderly resulting in constipation, colonic distension and sometimes abdominal pain. The diagnosis is made by identification of diverticuli on barium enema. DifferentialDiagmsii Chronic constipation, Code

454.X6

carcinoma of colon

s163

Carcinoma of the Colon (XXI-13) Definition

Pain due to malignant neoplasm of the large bowel.

Site

Most commonly lower abdominal or perineal pain from a lesion of the rectosigmoid area, Then any part of the abdomen from involvement of the colon, including the caecum.

System

Gastrointestin~.

Main Features

One of the commonest cancers in the developed countries, in contrast to developing countries. It is common in the middle aged and elderly. However it can occur rarely in young adults and children. The sex ratio is equal. The iiiness presents commonly with an alteration in bowel habit or with iron-deficiency anaemia. There are several possible mechanisms of pain: the commonest is due to obstruction with colon distension. Rarely pain is due to erosion through the colonic wall with peritoneal involvement. Pain is persistent and progressive until treatment.

Signs and Laboratory

Findings

A palpable abdominal mass or colonic distension or a palpable rectal mass. Positive faecal occult blood. Iron-de~ciency anaemia. ~suali~tion by barium enema or endoscopy. Usual Course

The pain is short lived once the diagnosis is made and it disappears with surgical removal of tumour, but pain may result later from metastases.

Complications

Acute or chronic rectal bleeding. There may be obstruction with a change in bowel habit, rarely colonic perforation or fistula formation into another viscus such as the bladder.

Social and Physical Disability Surgical treatment Pathology

Summary

may involve a permanent

colostomy.

The pathology is that of adenocarcinoma, beginning in the mucosa or in an adenomatous polyp, and spreading through the muscular wall to the serosa and via the lymphatic system and later the mesenteric blood supply, to metastases to the liver, lung, etc. of Essential Features and Diagnostic Criteria One of the commonest cancers in the Western World manifesting either as iron deficiency anaemia, rectal bleeding, or an alteration in bowel habit, sometimes with abdominal or perineal pain. Diagnosed by endoscopy or barium enema.

Differential Diagnosis Benign polyps and strictures, diver-tic&r disease, ischaemic colitis. Code

452.x4

s164

GROUP XXII: ABDOMINAL PAIN SYNDROMES OF GENERALIZED DISEASES

Familial Mediterranean Fever (F.M.F.) (xX11-1) Definition

Disease of unknown cause predominant 111those of Mediterranean stock, notably Sephardic Jews, Armenians and Arabs. Classic features are periodic acute self-limiting febrile episodes with peritonitis, pleuritis, synovitis an&or erythema resembling erysipelas.

Site

Abdomen or chest.

System

Peritoneal and pleural cavity.

Main Features

Prevalence .--- unknown;

Associited

affectseither sex; attacks usually appear before age 20. Hereditary; transmitted as a single genetic characteristic with autosomalrecessive inheritance; Onset - abdominal pain (peritoneal) most frequent presenting feature, vanes in seventy from mild abdominal discomfort with mild pyrexia to board-like rigidity, absent peristalsis and vomiting; Time - attacks settle within 48 hours leaving no residual signs. Pleural attacks resemble peritoneal ones but are less common and usually precede or follow abdominal pain. Chest wall tenderness may be marked during attack and transient pleural effusion may occur. Attacks occur with varying frequency.

symptoms Erysipelas-like erythema over the cutaneous aspects of thighs, legs or dorsa of feet. Arthralgias or acute arthritis involving mainly large joints such as knees or ankles. Attacks typically accompanied by fever and sometimes myalgia. Precipitants such as exercise, emotional stress, menstruation, fatty food, cold exposure have been implicated. Relief obtained only from strong analgesics, though colchicine may diminish frequency of attacks.

Laboratory

Findings Haemocytosis

Complications

!%&I

may occur. Pleural fluid contains polymorphs but is sterile

Amyloidosis is the commonest cause of death and is chiefly nephropathic. Its occurrence is highly variable depending on race, geography. When it does occur death is usually before age 40.

and Physical Disability Interruption supervene.

Pathology Diagmdc

of work when severe. No physical disability if amyloidosis does not

Unknown. Criteria Periodic attacks of peritonitis (rarely pleuritis) occurring in people chiefly of Mediterranean stock. Self-limiting and associated with fever, leucocytosis, and occasional rash. Arthralgic amyioiiosis may supervene and lead to death in renal failure. Sporadic cases in people of other races have been described.

Differential Diagnosis

Other causes of peritonitis, peptic ulcer, porphyria. Code

or

434.XOb 334.XOb

Abdominal Migraine (XXII-2) Definition

Characterised by recurrent attacks of abdominal pain, and/or vomiting occurring in association with typical migraine or as a replacement or migraine equivalent.

System

Unknown; lated.

Site

Abdomen.

Main Features

Prevalence - unknown; but uncommon in contrast to common or classical migraine, mates more than females, most common in children between 2 and 11; occurs in young adults; Aura - prodromal symptoms may occur such as listlessness, mood disturbance, yawning or, rarely, typical aura of common migraine; Pain - may be anywhere in abdomen but usually epigastric or periumbilical; a diffuse burning or aching increasing in severity lasting several hours but terminated by sleep; frequently associated with nausea and vomiting and is commonly replaced by vomiting alone; Frequency - more common in childhood as “bilious” attacks. Attacks occur often during episodes of stress frustration or personal conflict.

Signs

Skin may show vasodilation; non-specific fever has been recorded.

vasospasm

in the autonomic

diencephafic centres has been postu-

Laboratory Findings

E.E.G. during attack may show mild generalised dysrhythmia, with high voltage slow waves, thought to indicate cerebral hypoxia; transient leucocytosis may occur at height of attack. Course

Tends to become less frequent with age and usually disappears when personal conflicts resolved.

Complications

None.

Social and Physical Disabilities

Reduced work performance Pathology

in some.

Unknown.

Summary of Essential Features and Diagnostic Criteria

Recurrent attacks of vomiting and/or abdominal pain occurring either as a migraine equivalent or associated with a migraine attack; more frequent in childhood and often associated with stress or personal conflict.

s166 Differential Diagnosis

Gallstones; peptic ulcer, porphyrra, irritable gut syndrome, etc 404.x7

Code

Porphyria -

Hepatic Porphyrias

Group of disorders characterized by increased formation of porphyrins and/or prophyrin precursors in the liver. Principal clinicalmanifestations are photosensitivity and neurological lesions which result in abdominal pain, peripheral neuropathy, and mental disturbance. Within this group three diseases are recognized: (1) intermittent acute porphyria (I.A.P.); (2) hereditary coproporphyria (H.C.P.); and (3) variegate porphyria (V.P.)

IntermittentAcute Porphyria (I.A.P.) (XXll-3) Detinition

Inherited disturbance of porphyrin metabolism nof associated with photosensitivity, with attacks of abdominal pain as a constant feature, and sometimes variable hypertension, peripheral and central neuropathy (mainly motor) or psychosis

Site

Abdomen, either general&d or locafised.

System

Autonomic peripheral or central nervous system.

Main Features

Prevdence - females/maIes 32; exact incidence unk~n; Onset - after puberty; Inherifunce - transmitted by a sir& autosomal-dominant gene with variable penetrance; positive family history commonly obtained; Manifestutions ..- colicky abdominal pain, moderate or severe, generalised or local&d is usually the first and most prominent syndrome. Constipation, abdominal distension and profuse vomiting common; attacks are intermittent lasting several days to several months with periods of remission during which symptoms are slight or absent. Attacks may be precipitated by (a) wide variety of drugs, hormones; (b) metabolic and nutritional factors (dieting, low carbohydrate intake).

Associited

Symptoms

Neurological symptoms and signs are variable but may include peripheral neuritis (motor), autonomic, brain stem, cranial nerve and cerebral dysfunction. Hypertension is frequent. sins LabratoIy

The abdomen is soft, tenderness is marked and rebound tenderness is absent. Abdominal distension, slit fever, and leucocytosis may occur. FindiIqs

X-rays often show areas of intestinal distension proximal to areas of spasm. Hyponatremia may be severe. Po~obi~~n and A.L.A. (deita-aminolaevufinicacid) in urine.

S167 Usual Course

Severe cases may terminate in death from respiratory failure or from azotemia. Many however are clinically mild or latent and may exhibit only minor or vague complaints.

Social and Physical Disabilities

Pain often results in frequent admissions to hospital. Great caution needed when administering any drug. Pathogenesis

The primary

genetic defect is a generalised deficiency of enzyme uroporphyrinogen I synthetase acting in the pathway of haem synthesis predominantly in the liver. This leads to depression of &amino-laevulinic acid (A.L.A.) synthetase activity and overproduction of A.L.A. and porphobilinogen.

Essential Features

Acute intermittent abdominal colic without photosensitivity, with or without neuro-psychiatric associated symptoms and hypertension, and typical urinary findings (q.v.). Diagnostic Criteria

Intermittent abdominal pain with excess porphobilinogen

and/or A.L.A. in urine.

Differential Diagnosis

Peptic ulcer, gallstones, appendicitis, diverticulitis, irritable colon, lead poisoning, etc. Code

404.X5a

Hereditary Coproporphyria (H.C.P.) (xX11-4) Definition

An inherited disturbance of porphyrin metabolism characterized by attacks of abdominal pain, occasional photosensitivity, neurological and mental disturbance (see Intermittent Acute Porphyria (I.A.P.) XXII-3).

Site

Abdomen

System

Autonomic nervous system.

Main Features

Very rare; only a few families described; autosomal dominant; both sexes affected; often clinically silent (see I.A.P.). Similar but milder disturbance; acute attacks often precipitated by drugs.

(see I.A.P.)

Associated Features As in I.A.P. but photosensitivity

occurs, though uncommonly.

Usual Course

As in I.A.P. but milder.

Pathology

Due to probable partial block in conversion of coproporphyrin III to protoporphyrinogen IX. Coproporphyrinogen oxidase activity decreased, probably mainly in liver.

Code

404.X5b

s168

Variegate Porphyria (V.P.) (xX11-5) (South African genetic porphyria or protocoproporphyria

hereditaria)

Definition

A rare hereditary disorder of porphyria metabolism charactensed by acute attacks of abdominal pain, neuro-psychiatric manifestations. and photocutaneous lesions.

Site

Abdomen, diffuse or localised.

System

Autonomic

Main Features

Prevalence --.- unknown. Sporadic families reported throughout world. First reported in Dutch descendants in South Africa where incidence is 3 in 1000 Afrikaners. Autosomal dominant in either sex; Onset - usually in 3rd decade with cutaneous photosensitivity being initial feature. Attacks of abdominal pain, identical to those described in I.A.P. (q.v.); Frequency - variable. Provoked by a variety of drugs, particularly barbiturates and sulphonamide, hormones, anaesthetics, ethanol.

Signs

See I.A.P.

Laboratoty

nervous system.

Findings Excretion of large amounts of protoporphyrin and coproporphyrin In faeces. Urinary porphyrin precursors only modestly increased or normal, except during acute attack. Dehydration may lead to azotaemia and hyponatraemia is common.

UsuaI course

Variable; not as severe as I.A.P. Permanent

Pathology

Partial enzyme block between protoporphyrinogen major anatomic abnormalities at autopsy.

and haem is postulated. No

Intermittent acute abdominal pain with prominent and often neuro-psychiatric manifestations.

cutaneous

DiagMX&

change can occur.

criteria

Differential Diagnosis See I.A.P. Code

neuropathic

404.X5C

photosensitivity

S169

GROUP XXIII: ABDOMINAL

PAIN OF PSYCHOLOGICAL

ORIGIN

Muscle Tension Pain (XXIII-l) Rare. See General description (I-16-1 and III-l, -2). Code

433.X7b

Delusional or Hallucinatory Pain (xX111-2) See general description (I-16-2). Code

41X.X9a

Conversion Pain (xX111-3) See general description (I-16-3). Abdominal pain of psychological origin occurs as the Couvade syndrome in men during their wives’ pregnancies. This may be manifest as pains of discomfort, or at the time of labour, very rarely in developed societies, as an episode of pains resembling contractions. Code

41X.X9b

References

Trethowan, W.H. and Conlon, M.F., The Couvade syndrome,Btit. 111,470 (1965) 57-66.

Abdominal Pain Pericarditis -

Visceral Pain Referred to the Abdomen

See XVII-4.

Diaphragmatic Hernia -

See XIX-Z.

J. Psychiat.,

s170

GROUP XXIV DISEASES OF UTERUS, OVARIES AND ADNEXA

Mittelschmerz (XXIV-l) Definition

Mittelschmerz, also called midcycle pain, occurs as recurrent pain episodes at the time of ovulation.

System

Female internal genital organs; either in an ovary or in a tube, or in the uterus.

Site

Either unilateral or bilateral in the lower abdomen. May be felt always in the same iliac fossa, or alternately on one side or the other, or in the whole lower abdomen.

Main Features

Predence

Associited

- Mittelschmerz is the complaint of 1 to 3% of patients in a gynaecological outpatient clinic. It is mostly found in young women. between 20 and 30 years old; Symptoms - Mittelschmerz usually presents as a recurrent pain around the time of ovulation. It may last from a few hours to 1 or 2 days, sometimes up to 4 days. The severe form is infrequent. It presents, around the date of ovulation as a severe pain in an iliac fossa, lasting some 20 to 30 minutes and then gradually fading away. It may be accompanied by symptoms and signs of intra-peritoneal bleeding: anaemia, abdominal meteor&m, diaphragmatic and/or shoulder pain and fainting; Time Course - the severe form recurs only rarely; it may be followed by the recurrent less severe form. The less severe form may last for several years.

Symptoms Increase of cervical mucorrhea; bleeding.

sometimes

accompanied

by mld~ns~ual

Signs

In the less severe form, there are no signs, or only tenderness on bimanual palpation, especially in the corresponding iliac fossa. When the severe form is accompanied by intra-peritoneal bleeding, there are signs of acute anaemia, or rebound tenderness on palpation of the abdomen.

Complications

None in the less severe forms. In the severe forms there may be massive intra~ritoneal haemorrhage; as in these cases an operation is necessary, this may be followed by post-operative adhesions around the ovary or the adnexa.

PathoIogy

No definite pathology known; pathophysiology remains to be elucidated. Possible causes include maturation of the follicle or ovulation itself or contractions of the tubal wall in a case of hydrosalpinx, or an increase in the basal tone of the myometrial contractions around the time of ovulation. It may also be due, although rarely, to a focus of endometriosis.

Treatment

If the pain is mild in the less severe form: analgesics. The pain episode may be prevented by cyclic oestropmgestogens. In more severe forms with intraperitoneal bleeding, a laparotomy may be necessary.

Diagnostic Criteria ad Differential Diagnoh The essential feature is recurrence at the time of ovulation; it may be useful to confirm the coincidence with the pet-i-ovulatory period by means of the basal body temperature which will show a shift towards a premenstrual plateau. Severe cases with right-sided location may erroneously be taken for appendicitis.

s171

Appendicitis frequently starts with a pain in the peri-umbilical region, and it gives rise to nausea or vomiting, muscle guarding and a slight fever. When accompanied by intraperitoneal haemorrhage, the time of occurrence will differentiate severe Mittelschmerz from ectopic pregnancy or rupture of a corpus luteum cyst, but blood transfusion and laparotomy will be indicated in both cases.

Reference

M. Renaer, Midcycle pain. In: M. Renaer (Ed.), Chronic Pelvic Pain, Springer Verlag, Berlin, New York, 1981, pp. 65-68.

Secondary Dysmenorrhoea (XXIV-2) Definition

Dysmenorrhoea is called secondary if a structural anomaly is found that is probably responsible for the pain or when the pain seems to have a psychological origin.

System

Genital system.

Site

Pelvis. The pain is more often unilateral than in the primary variety, especially when the causal condition is unilateral, as for example in some cases of endometriosis.

Main Features

These resemble primary dysmenorrhoea in endometriosis.

Main Causes

The main causes of secondary dysmenorrhoea are: endometriosis; adenomyosis; submucous fibroids; and various causes of obstructive dysmenorrhoea, as described below.

but the pain often lasts longer, e.g., as

ENDOMETRIoslS is a condition caused by foci of ectopic endometrium, located either in the pouch of Douglas, on the ovaries, or on the broad ligament, which undergo to a variable extent the influence of the sexual steroids secreted during the menstrual cycle. Main Features. Up to 40% of the endometriotic lesions remain symptomless. The most frequent symptom is pain, which may present as dysmenorrhoea or as premenstrual pain with menstrual exacerbation, or continuous pain with or with menstrual exacerbation. The menstrual pain may last the whole duration of the menstrual period and sometimes even one day after its end. For the other clinical features of endometriosis, refer to the section on Endometriosis (XXIV-4). ADHVOMYOSIS. The presence of islands of endometrium deep in the uterine muscle wall is called adenomyosis or endometriosis interna. Main Features. As clinical diagnosis is difficult, diagnosis has generally to await microscopic examination of a hysterectomy specimen. The prevalence varies greatly, depending on the depth of penetration of endometrial tissue into the myometrium required by the pathologist in order to consider a case as adenomyosis. The prevalence is of the order of 3 to 8% of hysterectomies. Fifty percent of patients are in the 5th decade. The most common symptoms are menorrhagia or metrorhagia and dysmenorrhoea; but both symptoms, abnormal bleeding and

$372 menstrual pain, coincide in only 20% of cases. The dysrn~norr~~~ea 1s usualiy severe and may be incapacitating.

Associated Symptoms. Adenomyosis

frequently causes infertiiit5. Signs. The uterus is either symmetrically or asymmetrically enlarged and firm, and there are generally no well-circumscribed nodules as in a potyfibromatous :tterus. &MI/ Course. The uterine volume enlarges progressively over the years but rarely grows larger than a 1Qweek gestation. The pain and the abnormal bleeding disappear at menopause but, owing to the severity of symptoms, most patients have to undergo a hysterectomy before menopause. Complications. None. Pathology. Adenomyosis is diagnosed only when endometrial glands are found at least one low-power microscopical field below the myoendometrial junction. The nests of endometrial tissue are generally surrounded by a proliferation of fibrous tissue. Diagnostic Criteria. Differentiation from uterine fibroids may be difficult. In adenomyosis no nodules are found; the uterus varies in consistency. size and tenderness on palpation during the menstrual cycle, size and tenderness increasing premenstruaily. Hysterography performed with a rvater-soluble contrast medium may suggest adenomyosis if, in a patient with dysmenorrhoea and menorrhagia, the uterine cavity has an irregular shape and if small diverticula are found either in the fundus or along the lateral borders.

FIBROIDS. Fibroids are rarely responsible for pain or dysmenorrhoea.

They may cause colicky pains generally accompanied by menorrhagia when they are extruded out of the myometrium into the uterine cavity or pushed against or through the cervical isthmus. Diagnostic Criteria. If the uterine size is only slightly enlarged, hysterography may detect a submucous fibroid or a fibroid polyp. A circular or polycyclic filling defect is then found that generally deforms the uterine cavity, whereas a mucous polyp does not.

O~TRUCT~VE

DYS~O~~O~. Secondary dysmenorrhoea is called obstructive when obstruction of the menstrual flow is due to an organic cause; this cause may be either congenital or acquired. Main Features. The prevalence is difficult to evaluate. In congenital forms the pain mostly begins a few months after menarche, as it starts only when enough blood has been retained to distend the vagina or the uterus. When there is an atresia of the hymen. there is dysmenorrhoea with cryptomenorrhoea as the menstrual blood is retained in the vagina. If there is retention of fluid in one half of a double uterus, the menstrual pain will be unilateral. AssociatedSymptoms, The asymmetrical varieties of double uteri are frequently accompanied by absence or hypotrophy of one kidney. The retained blood may distend the vagina and the uterus and give rise to a retrograde menstruation. which, after a few months, may cause implantation of menstrual debris, i.e.. endometriosis. Signs. If there is atresia of the hymen, retention of blood in the vagina wilI manifest itself by distention of the vagina with the hymen bulging at the introitus and the posterior wall of the vagina bulging into the rectum. Retention of blood in one half of a double uterus will cause an asymmetrical enlargement ot' the uterus. The distended blind half of a double vagina till bulge into the other half of the vagina, Pathology. Various congenital anomalies may cause secondary dysmenorrhoea, e.g., atresia of the hymen, a rudimentary uterine horn, a double uterus one half of which does not communicate with the vagina, or a uterus duplex bicollis, one half of which opens into a blind half of a double vagina. Acquired forms may be due to adhesions in the cervical canal after amputation of the cervix or conisation or electro-coagulation. Adhesions may also be situated in the tower part of the uterine cavity, for example, in an Asherman syndrome.

s173 Diagnostic Cr$eria. Diagnosis must rely on history and clinical examination. An early unilateral dysmenorrhoea, combined with the presence of an asymmetrical mass in the lower abdomen or in the vagina is suggestive of an asymmetric malfusion deformity. Vaginography after injection of an opaque medium into the blind vagina or hysterography through the accessible cervix may be used. Radiological exploration of the reno-uretera! tract is also indicated.

ACQUIRED FORMS. Main Features. The acquired forms are usually easy to diagnose. If dysmenorrhoea or cryptomenorrhoea appear after an amputation of the cervix or an electro-coagulation or a conisation of the cervix, or after a currettage performed for retained products of conception, the diagnosis is easy and the condition may be cured with a dilatation of the cervix or with a curettage by means of a smooth curette in order to destroy the adhesions. A laparotomy will rarely be required to divide the adhesions under visual control.

DYSMENORRHOEA

OF PSYCHOLOGICAL ORIGIN. The frequency such dysmenorrhoea has been exaggerated. The diagnosis of dysmenorrhoea psychological origin should be accepted only where no organic cause can found and when psycho~thol~c evaluation reveals neurotic behaviour other psychopathological problems sufficient to account for the complaint.

Code

765.X6a 765X4 765.X0 765.X6b 765.X9a 765X9b 765.X9c

of of be or

(endometriosis) (adenomyosis or fibrosis) (con~nita! obstruction) (acquired obstruction) (psychological, tension) (psychological, delusional) (psychological, conversion)

Primary Dysmenorrhoea (XXIV-3) Note: Endometritis does not cause pain, neither acute nor chronic. Definition

Dysmenorrhoea, or painful menstruation, refers to episodes of pelvic pain whose duration is limited to the period of menstrual blood flow, or which start one, or at the earliest, two days before and stop one or at the latest, two days after the blood flow. In primary dysmenorrhoea there is no structural lesion.

System

Female internal adnexum.

Site

The pain is localized either in the whole lower abdomen nearly always symmetrically or in an iliac fossa. It may radiate towards the sacro-glutea! zone in the tower back, i.e., to the superior half of the sacrum and, laterally, to one or both g!utea! regions. It sometimes radiates into the anterior and superior aspect of one or both thighs.

Main Features

There are two varieties of dysmenorrhoea; primary or essential and secondary or symptomatic. If the pain has a lower abdominal location, which is usually symmetrical, and if no structural anomaly is found on clinica! examination, the

genital organs;

either the uterus

or both adnexa

or one

S174 dysmenorrhoea is termed primary. Cases with structural organic anomalies are classified as secondary. Prevalence - between 5 and 10% of all girls in their late teens and early 20s suffer from severe, mostly primary, dysmenorrhoea during the first hours of their periods. In one study 72% of women aged 19 years had some dysmenorrhoea; Age of Onset -- primary dysmenorrhoea mostly starts a few months after menarche and lasts for several years; Quality _-- the pain is generally colicky; in about one-fourth of all cases the pain is continuous. Intensity --the pain may be mild. It is called severe (or second degree) if it seriously interferes with the patient’s work. Third degree or incapacitating dysmenorrhoea has an intensity that compels the patient to stay in bed; Duration - in most cases the pain starts a few hours or half a day before the beginning of the blood flow, and usually lasts less than one day. Associated Features With third degree primary dysmenorrhoea and/or diarrhea. usual

course

Complications

there may be nausea,

vomiting

Primary dysmenorrhoea may disappear spontaneously after a few years, but it mostly disappears in 8 cases out of 10, after the birth of the first baby. None.

Social and Physical Disability Third degree dysmenorrhoea is the cause of periodic absence school in many teenagers and young women. Pathology

from work or

No definite pathology known.

Pathophysiology Primary dysmenorrhoea is found at the end of an ovulatory cycle; it has also been reported in women taking oral contraceptives. In some patients uterine contractions during dysmenorrhoeic episodes show well-coordinated conttactions with extremely high intrauterine pressures; in others “dysrhythmic” contractions with high or low pressures; and in others an elevated intra-uterine pressure between contractions. Several authors have found elevated prostaglandin concentrations in endometrium and menstrual fluid of patients with primary dysmenorrhcea. Although the exact mechanism of primary dysmenorrhoea is unknown, it is probable that in most cases the pain is due to hypertony of the uterine isthmus, i.e., failure of the normal menstrual relaxation with temporary obstruction of the flow of menstrual blood, this is combined with an increased production (or increased retention?) of prostaglandins, which leads to increased, or dysrhythmic, myometrial contractions, sensitization of nerve terminals to prostaglandins and ischaemia of the uterine wall. Treatment

Mild and moderate cases are best treated by analgesics. In severe cases the pain can be prevented by cyclic oestroprogestogens, or the pain may, when it appears, be alleviated by prostaglandin inhibitors.

DifferentialDiagnosis From conditions causing seconday dysmenorrhoea, viz: endometriosii, etc. Primary dysmenorrhoea is characterized by the absence of any structural abnormality of the internal female genital organs. Recent observations have shown that in about 10% of cases with a negative clinical examination, Iaparoscopic visualization of the internal genitalia may detect endometriotic

5175 lesions, so that the diagnosis of primary dysmenorrhoea previously thought.

is not as simple as

Code

765.X7b

Reference

Andersch, B. and Milsom, I., An epidemiologic study of young women with dysmenorrhoea, Am. J. Obst. Gynecol, 144 (1982) 655660.

Endometriosis (XXIV-4) Definition

Lower abdominal pain due to foci of ectopic endometrium uterus (endometriosis externa of endometriosis).

System

Genital.

Site

The pain may be located in one or in both iliac fossae or over the whole lower abdomen. It frequently radiates toward the sacro-gluteal region in the lower back.

Main Features

Prevalence - The frequency with which endometriosis is found depends on the circumstances in which it is sought. It was found in 15 and 20% of two different series of laparoscopies, but, on the other hand, it was found in 50% of a large series of laparotomies. As many endometriotic lesions remain symptomless, the true incidence is difficult to determine. The ectopic foci are located either in the pouch of Douglas or on the ovaries or on the posterior leaf of the broad ligament and, less frequently, on the wall of rectum or sigmoid colon; rather seldom they infiltrate the bladder wall or the wall of the ureter. Age ofOnset - It used to be thought that endometriosis usually develops in the late twenties or in the thirties, but since more laparoscopies have been performed on younger patients it has been found rather frequently in teenagers, especially those complaining of secondary dysmenorrhoea and/or infertility. Symptoms - In some 30 to 40% of patients with endometriosis there are no complaints except perhaps infertility. The main symptom of endometriosis is pain; it may manifest itself either as dysmenorrhoea or as premenstrual pain with menstrual exacerbation or as chronic pain. Lesions located in the pouch of Douglas may provoke firm adhesions between the anterior wall of the rectum and the posterior vaginal wall; this location may cause pain on defaecation during menstruation. Foci located in the pouch of Douglas or a fixed uterine retroversion due to endometriotic adhesions frequently cause deep dyspareunia. Endometriotic foci that penetrate into or through the bladder wall may cause painful micturition with or without hematuria during menstruation.

Signs

On pelvic examination a fixed painful retroversion may be found, or tender, enlarged, adherent adnexa on one or both sides. Small, tender nodular lesions, which are frequently palpated either in a sacro-uterine ligament or on the posterior surface of the uterus, are almost pathognomonic of endometriosis.

Usual Course

The ectopic foci remain receptive in a variable degree to the ovarian steroids, and they will undergo the same histologicat changes as the “‘eutopic” endometrium. The ectopic tissue may grow on the surface of the peritoneum or it may become

located outside the

S176 buried in a fibrous capsule. The encapsulated lesions are thost: lnost Ilk& :(. become painful, whereas the superficial ones are usually painlesL Pw pain WI& start as secondary dysmenorrhoea, it may later become prerner~‘,! rlial ds well iI:; menstrual; it may also become continuous. The pain due to end
Infertility is a frequent complication. Subocclusion or occlusion of iiik!srrrali ot ciw large intestine is possible but infrequent. Rupture of an endometriotic ~1st located in an ovary may cause an acute abdominal emergency due to irritation of the peritoneum by the old blood flowing from the ruptured cyst

Pathogenesis

Retrograde menstruation, i.e., passage ot menstrual blood ill ~:i endometnal debris through the tubes towards the pelvic cavity often occurs. T3is seems to be the pathogenetic mechanism in most cases of endometriosis. However, it does not explain all the possible locations of the foci. Tiny fragments of menstruai endometrium may be carried away by lymphatics and, more rarely, by veins of the endometrium.

Diagnostic

Criteria The history and the findings on clinical examination will frequel1tly lead to the diagnosis. When any doubt remains. a therapeutic trial with c!:c-lit oestropro gestogens will alleviate the pain in 8 cases out of 10. Laparoscoplc- inspection of the pelvic cavity has been used rather frequently in recent years to verify the diagnosis and to evaluate the extent of the lesions. Acute pain episodes in the right iliac fossa due to endometriosis may be mistaken for appendicitis. Recurrent episodes of lower abdominal pain, tenderness and a slight fever may erroneously be taken for recurrent petvic inflammatory disease

Treatment

Treatment of endometriosis will be either hormonal or surgical Ior combined. I! will vary, depending on age of the patient, stage of the disease and the main presenting problem: pain or infertility or both. Hormonal treatment consists of cyclic oestroprogestogens or in the continuous daily administration of oral progestogens as e.g., Lynestrenol or norethisterone acetate. Thr two last drugs produce a hypoestrogenic amenorrhoea. During recent years excellent results have been obtained by the continuous oral administration of Danazol. a strong antigonadotropin and mild androgenic drug. Surgical treatment will, depending on the indication and the stage of the disease, consist of conservative surgery preferably by microsurgical techniques, or semi-radical or radical surgery. i.e.. total hysterectomy and bilateral salpingo-oophorectomy.

Code

764.X6

References

Varangot, J., Giraud, J.R., Bignon-Schnizer, J., Etiologie, clinique et pathologie de I’endom&iose @nitale, Bull. Fed. Sm. Gynec. O&et. Fr. 17 (1%5) 239292. Chalmers. J.A.. Endometriosis, Butteworth.

London, Boston. 1975

s177

Posterior Parametritis

(XXIV-5)

Definition

Pain with low grade infection of parametrial tissues, especially the posterior parametrium. Synonyms: pelvic lymphangitis, chronic parametrial cellulitis.

System

Uterine cervix and parametrial tissues.

Main Features

Site - Lower abdomen,

Signs

A more or less severely torn cervix is found and either an acute or a chronic cervicitis. One or both utero-sacral ligaments are tender on palpation.

Pathology

Posterior parametritis on chronic cervicitis is believed to be due to extension of a cervical infection along the lymphatics of the parametrium. Chronic cervicitis is not painful by itself.

sometimes the back also; Prevalence - Because histological proof of the diagnosis is usually missing, the prevalence is unknown, but the condition is seen infrequently. It may be found soon after a delivery, especially if the cervix has been torn and infected. Symptoms - The patient complains of lower abdominal pain with or without low backache, and deep dyspareunia. There is usually no fever. The pain may occur during the premenstrual period and disappear during menstruation, or it may be continuous, with premenstrual exacerbation.

Diagnostic Criteria and Treatment

Diagnosis of cervicitis depends on finding agglutinated leucocytes in the cervical mucus during the peri-ovulatory period. The presence of an infected cervical canal and of a tender posterior parametrium and the absence of a history and of clinical findings suggestive of endometriosis make the diagnosis of posterior parametritis plausible. In these circumstances treatment with broad-spectrum antibiotics and local heat is indicated. If the pain disappears, this confirms the diagnosis. If the pain and the parametrial tenderness persist, another cause of the pain should be looked for by laparoscopy. Code

733.x2

Reference

Renaer, M., Chronic Pelvic Pain in Women, Springer, Berlin New York, 1981.

Heidelberg -

S178

Tuberculous Salpingitis

(XXIV-6)

Definition

Pelvic pain due to tuberculosis

Main Features

Prevalence

Signs

On pelvic examination a fixed retroversion with palpable tubo-ovdnan masses may be found. Spontaneous pain and dysmenorrhoea may be explained by a pyo- or hydrosalpinx or by a tuberculous pelviperitonitis. Dyspareunia may be due to a fixed retroversion or to adherent adnexal masses.

usual

course

salpingitis.

-- Genital tuberculosis has become quite uncommon in most developed countries thanks to the gradual disappearance of pulmonary tuberculosis. It remains a problem in many less developed countries where pulmonary tuberculosis is still widely prevalent. Symptoms - The most frequent symptoms are sterility, pelvic pain, poor general condition and menstrual disturbances. Genital tuberculosis presents under two forms, either the silent or the active form. In the silent forms there are no particular symptoms; there is no pain and no fever. In the active or advanced forms there are general symptoms and signs of the tuberculous process, meno- or metrorrhagias. sometimes amenorrhoea. Pelvic pain may be present. In the active cases there is usually pyrexia, weight loss and night sweats.

The tuberculous process may become latent or may heal spontaneously. It may, on the other hand, evolve towards a pyosalpinx or an ovarian abscess or to a tuberculous pelviperitonitis or a general peritonitis.

Diagnostic Criteria In advanced cases general symptoms and signs of the tuberculous process, abdominal pain or discomfort, signs of a pelvic infection, together with a positive tuberculin test and bacteriological evidence of tuberculosis constitute the basis of the diagnosis. Tubercle bacilli may be cultured either from menstrual blood or from an endometrial biopsy, taken preferably in the premenstrual phase. Silent cases are usually diagnosed by the presence of tubercular lesions in an endometrial biopsy taken during the evaluation of infertility cases. Treatment

Treatment is essentially medical by means of a combined drug regimen with Rifamycin, isoniazid and ethambutol. It should last for a minimum of 18 months to two years. Surgery will be resorted to only if pelvic masses persist or increase under medical treatment, if endometrial lesions persist and if pain or other pelvic symptoms are not alleviated by drug therapy.

Code

763.x2

Reference

Schaefer, 223-239.

G.. Female genital, tuberculosis,

C/in. Obsret. Gynecol.

19 (1976)

s179

Retroversion of the Uterus (XXIV-7) Definition

Lower abdominal pain due to a retroverted

Main Features

Retroversion of the uterus is found in 15 to 20% of adult women, but only a small number of mobile retroversions cause symptoms. In a few cases it may give rise to intermittent pain with or without deep dyspareunia. The pain will be located either in the lower abdomen or in the sacro-gluteal region or in both sites. The pain usually is worse during the premenstrual period and mostly disappears or decreases after the first or second day of the period. It also decreases with horizontal rest. On pelvic examination the retroverted uterus is tender and frequently slightly enlarged and softer than normal.

Pathology

It has repeatedly been observed that the size of a painful retroverted uterus diminishes and that it becomes firmer after anterior reposition. If the pain disappears after correction of the retroversion and insertion of a pessary it does so gradually during the two to three days following the reposition. These circumstances seem to indicate that circulatory disturbances, probably passive pelvic congestion, cause the pain.

uterus.

Diagnostic Criteria The uterus is said to be retroverted when the axis of the cervix is directed towards the symphysis pubis and the axis of the uterine corpus towards the excavation of the sacrum. A retroversion is either mobile or fixed. A retroversion is said to be fixed when adhesions bind the uterine corpus down in the pouch of Douglas. A mobile retroversion should be considered the cause of the pain only if no other causes of pain are found, such as endometriosis or posterior parametritis on a chronic cervicitis, and if the pain disappears after anterior reposition of the uterus. It is then maintained in its corrected position by inserting a vaginal pessary. If a patient with a fixed retroversion complains of some symptoms, it is usually impossible to prove which symptoms are due to the retroversion and which are not. Treatment must therefore be directed against the causal disorder, which may be either endometriosis or sequelae of acute Pelvic Inflammatory Disease or of a pelvi-peritonitis, or a tuberculous salpingitis. Treatment

A mobile retroversion that causes no symptoms does not require any treatment. If the patient complains of pain, reposition of the uterus will be tried and a pessary inserted. If the pain disappears the pessary may be left in situ for 6 or 8 weeks. It is then removed and nothing more is necessary if the pain does not recur. If it does, operative correction of the retroversion may be undertaken. If the retroversion is fixed, treatment must be directed against the causal condition and a suspension operation should be performed only when the retroversion itself is probably the cause of the complaint, as in some cases of dyspareunia, or when there are other reasons for surgical intervention, e.g., tubal surgery for infertility.

Reference

Renaer, M., Pain in gynecologic practice. III. The symptomatology of uterine retroversion and, in particular, pain in uterine retroversion (Dutch), Verhand. Koninkl. Acad. uoor Gen. van Be&! 17 (1955) 433-457.

S180

Ovarian Pain (XXIV-S) Definition

Lower abdominal pain due to an ovarian lesion

RECURRENTPAINFUL FUNCTIONAL OVARlAN CYSTS. Mm Features. Lower abdominal pain due to recurrent painful functional cysts is sometimes, although rarely, seen in young women. It is called by some “painful ovarian dystrophy”. Diagnostic Criteria. If the condition is very painful laparoscopy may be indicated in order to ascertain the cause of the pain; the cystic fluid may then be aspirated and submitted to cytological examination. If the result of this examination is compatible with a functional cyst, it is recommended to treat It conservatively by means of oral contraceptives. There is a good chance that the cyst and the pain will disappear, whereas surgical exploration with wedge resection of theovary is likely to be followed by a recurrence of the cyst and of the painful episode. Code

764.X7a

OVAR~N R~NA~SYN~RO~E. Pain due to ovarian remnants following operation. lain Features. When a bilateral ~phorectomy has been performed in conditions that make it difficult to be sure that all ovarian tissue is removed. e.g., when the ovaries were embeded in endometriotic scar tissue or were surrounded by dense adhesions, active rests of ovarian tissue may cause a painful condition called the “ovarian remnant syndrome.” Diagnostic Criteriu. An ovarian remnant will be suspected when the patient presents evidence of oestrogen secretion that persists after a short course of corticoids prescribed to suppress adrenal androstenedione secretion and its peripheral conversion to oestrone. Treatment will consist of meticlous excision of the residual ovarian tissue. Code

764.X7b

References

Stone, S.C. and Schwartz, W.J., A syndrome characterized by recurrent symptomatic functional ovarian cysts in young women, Am. J. Obstet. Gynecol.. 134 (1979) 310314. Symmonds,

R.E. and Pettit, P.D.N., Ovarian

remnant

syndrome,

O&et.

Gynecol., 54 (1979) 174177.

Chronic Pdvic Pain Without Obvious Pathdogy (C.P.P.W.O.P.) (xxiv-9) Definition

Chronic or recurrent pelvic pain that has apparently a gynaecoiogical origin but for which no definite lesion or cause is found.

System

Genital.

Site

Lowr abdomen,

S181 Main Features

Chronic pelvic pain without obvious pathology is the name given recently to a syndrome that has been known and described for more than a century under many different names, some of them being: parametropathia spastica, pelvic congestion and fibrosis, pelipathia vegetativa, pelvic sympathetic syndrome. Prevalence - This syndrome is rather uncommon. Until 20-30 years ago, it was considered rather common, but the diagnosis should be considered only under the following conditions: (1) if the patient’s symptoms are not due to a gynaecological cause; (2) if the pain has characteristics of a gynaecological pain; and (3) if the syndrome is not due to one of the acknowledged causes of gynaecological pain which supposes that the patient underwent a laparoscopy. It has become clear that formerly many chronic painful conditions erroneously been classified under the above heading.

have

Associated Symptoms

The most important symptom is lower abdominal pain and, less frequently, low back pain. The lower abdominal pain may be felt either in the whole lower abdomen or in both iliac fossae, or in one fossa only. The low back pain may be felt over the whole width of the sacro-gluteal zone or over a part of this zone. The pain is usually more severe for several days before menstruation and its intensity decreases on the first or second day of the period. Deep dyspareunia is a frequent complaint. Medical treatment is not well-defined and is therefore not usually successful in chronic cases. When a chronic pelvic pain syndrome has lasted for several months and has not been cured by medical treatment, it is useful to perform a laparoscopy in order to look for non-palpable lesions, such as endometriosis or sequelae of chronic P.I.D., which might explain the pain. By definition, those lesions are not found in C.P.P.W.O.P. Signs

On abdominal examination tenderness at ovarian points may occur. Uterine tenderness may be found. On gynaecological examination the uterus and adnexa may be tender; there is frequently tenderness of the posterior parametrium and sometimes it is shortened. The vagina usually appears congested.

Pathology

Besides lower abdominal pain with or without sacro-gluteal pain and the frequent complaint of deep dyspareunia, many patients have several complaints including one or more that are usually considered functional; these patients may therefore be called polysymptomatic. Most oligo-symptomatic patients complain merely of spontaneous pelvic pain and deep dyspareunia. During the last decades various conditions have been suspected as possible causes. It has been thought that in a precentage of cases the syndrome is due to traumatic laceration of a sacrouterine ligament or of a posterior leaf of one or both broad ligaments. It seems however, that the role of those tears is negligible. There is good indirect evidence that circulatory factors may give rise to chronic or intermittent lower abdominal pain. There is uterine and pelvic phlebographic evidence for the presence of passive pelvic congestion in a percentage of cases with C.P.P.W.O.P., but this passive pelvic congestion does not seem to be the sole factor that causes the pain. Pelvic varicosities are likely to be the major cause of the pain. Tenderness with or without shortening of the posterior parametrium is found in the majority of cases of C.P.P.W.O.P.; as pressure or traction exerted on the posterior parametrium usually reproduces the pain the patient feels either spontaneously or during intercourse, this condition seems to intervene in the pain mechanism in many cases. However, the morphological or functional basis

s182 of this tenderness remains to be elucidated. All those whn studled the psychological characteristics of these patients found definite psychopathological anomalies or stress situations in most. although not all. of the patients rxaminc>d. It is probable that patients with C.P.P.W.O.P. constitute a heterogenous group made up of a spectrum of mi~el~neous conditions. At one er;d. there are patients with very little peripheral noxious stimulation whose compl&tts will. to ‘1 large extent, have a psychological explanation. The other extreme :s made up of persons with rather intense peripheral noxious stimulation 1.‘It her pelvic. circulatory disturbances or tenderness of the posterior paramerrlum and, less often, uterine cramps or a real tear in a sacro-uterine ligament, drld little or no psychological factors. In between these extremes there are apparetltly a rtumbet of mixed cases with less pronounced peripheral noxious stimulation ‘lnd one ot more of the psycho-physiological mechanisms that may induce (‘()mplalnts and care-seeking behaviour. Varicosities may be a major contrib\\rory physical factor. Treatment

Even if one does not find a satisfactory explanation, the patient’: c~ompiaints should be taken seriously. The doctor should try to obtain Information concerning the patient’s family and personal history, her marital life, and heI general behaviour. As said earlier, the diagnosis of C.P.P.W.O.P. cannot be made without the aid of laparoscopy; this may be useful in lessening the anxiety of the patient. C.P.P.W.O.P. is frequently associated with psychological problems or with a neurotic disorder, in which case treatment should attend to the conditions. Some patients have been helped with cyclic oestroprogestogens; others have had hype-oestrogenic amenorrhoea induced by continuous administration of oral progestogens e.g., 5 mg. of Lynestrenol daily or 5 mg. of Norethisterone acetate daily during several months, but we think this of little value. Many gynaecologists have performed, or still perform, a total hysterectomy for C.P.P.W.O.P. The low percentage of long-term results has shown that a total hysterectomy is not an effective treatment in this syndrome and that it should be performed only if the patient has been treated conservatively for several months or years, if she is oligosymptomatic and does not present psychopatho logic disturbances.

Code

763.X8 See also I-16

References

Beard, R.W., Highman, J.H., Pearce, I., Reginald, P.W., Diagnosis of pelvic variocosities in women with chronic pelvic pain. Lance& ii (1984) 946949. Renaer, M., Jijs, P., Van Assche, A., Ve~ommen, H., Chronic pelvic pain without obvious pathology: personal observations and a review of the problem. European J. Obstet. Gynaecol. and Repro&&e Biol., 10 (1980) 415-453. Taylor, H.C., Vascular congestion and hyperemia: 1. Physiologc basis and history of the concept, Am. J. Obstet. Gynecol., 57 (1949af 211-230. Taylor, H.C., Vascular congestion and hyperemia: II. The clinical aspects of the congestion-fibrosis syndrome, Am. J. Obstet. Gynecol., 57 (1949b) 637-653. Taylor, H.C., Vascular congestion and hyperemia: III. Etiology and therapy, Am.

J. Obstet. Gynecol., 57 (1949~) (354-m.

s183

GROUP XXV: PAIN IN THE RECTUM, PERINEUM AND EXTERNAL GENITALIA

Neuralgia of Iliohypogastric, Ilio-Inguinal or Genito-Femoral Nerves (XXV-l) Testicular Pain Definition

Burning or lancinating or other pain syndrome due to injury of the respective nerve, usually followingsurgical intervention in the hypogastric or inguinal region.

Site

Inguinal area and external genitalia.

System

Peripheral nervous system.

Main Features

The pain can occur immediately after the operation but not infrequently occurs

after months or years. Sometimes there is no history of operation or trauma. The pain is burning or lancinating and radiates to the area supplied by the sensory nerve. For the iliohypogastric nerve the pain radiates to the midline above the pubis but also laterally to the hip region. For the ilio-inguinal and the genitofemoral nerve the pain radiates from the groin into the anterior part of the labia major (or the scrotum and the root of the penis) and on the inside or the anterior surfaces of the thigh, sometimes down to the knee. Usually the pain is continuously present but it can be intensified by forcible stretching of the hip joint, by coughing, sneezing, sexual intercourse or general tension in the abdominal muscles. The patient frequently adopts a posture that eases discomfort, with a slight flexure of the hip and a slight forward inclination of the trunk. Signs

On examination the pain can be triggered in a narrowly circumscribed area of the operative scar. Usually, there is a tenderness along the course of the nerve from near the anterior superior iliac spine to the external genitalia; when the genitofemoral nerve is involved, the internal ring of the inguinal canal can be very painful. As a rule, cutaneous sensibility is more or less impaired in the region innervated by the affected nerve. Usually, there is an increased threshold for touch and prick sensation in combination with hyperalgesia; the hypoesthesia is sometimes best demonstrated with cold stimuli. In some cases scratching the skin induces less reddening or an absence of it on the affected side as compared to the intact side, indicating the degeneration of afferent C fibres. Although motor impairment of abdominal muscles can be present, this is hard to evaluate because the motor tests usually exacerbate the pain. If the iliohypogastric nerve is damaged, the lower abdominal skin reflex may be absent. Typically, with involvement of the genital branch of the genito-femoral nerve in man, the cremaster reflex is absent on the affected side.

Usual Course

Without treatment the pain may persist for several years without tendency to improvement. Surgical repair is the most effective treatment.

Pathology

If the nerve was sectioned during surgical intervention, histological examination may show a neuroma. If the nerve was ligated or entrapped by a tear, there is endoneural fibrosis.

s184 Diagnostic Criteria l”ypical pain radiation with sensory impairment and pain relief by locC~l anesthetic Treatment

The pain can be relieved by injection of a local anesthetic proxtmally from the injury side; for the iiiohy~~t~c and ilio-inning nerve the injection is done two

fingerwidths medially from the anterior superior iliac spine, where they leave the internal oblique muscle. Differential Diagnosis lnguinal and femoral hernia; lymphadenopathy;

Code

periostitis of pubic. rubercle

407.x71, 407.X1 Testicular pain

Tumour Infiitratbn of the !i!Sacrumand Sacral Nexws (XXV-Z) Definition

Dull aching sacral pain accompanied and perineum.

Site

Sacrum, rectum and perineum.

!&stems

Skeletal and nervous systems.

Main Features

Pain in a sacral distribution usually occurs in the 5th, 6th and 7th decades as a result of the spread of bladder, gynaecological and colonic cancers. There is dull aching mid-line pain and usually burning or throbbing pain in the soft tissues of the rectal and perineal region. The pain is usually made worse by sitting and lying. The rectal and perineal component of the pain may respond poorly to analgesic agents.

Associated

by burning or throbbing pain in the rectum

Symptoms With bilateral involvement,

!3igns and laboratory

sphincter incontinence

and impotence are common.

Findings

There may be tenderness over the sacrum and in the region ot the sciatic notches. Sometimes there is limitation of both direct and reverse straight leg raising. Involvement of S 1 and SZ roots will produce weakness of ankle plantar flexion and the ankle jerks may be absent. There is usually sensory loss in the p&anal region and in the genitalia and thii may be accompanied by hyperpathia. CT scan of the pelvis usually shows sacral erosion with a pre-sacral mass. The pain and sensory loss may be unilateral initially with progression to bilateral sacral in~lv~t and sphincter disturbance.

usual

GNrse

Socii

and Physical Disability The major disabilities are a result of intractable pain and loss of sphincter function. An indwelling urinary catheter may be required.

s18.5 Summary of Essential Features and Diagnostic Criteria:

The essential features are dull aching sacral pain with burning or throbbing perineal pain. There is usually sacral sensory loss and sphincter incontinence. A CT scan of the pelvis may show sacral erosion and a presacral soft tissue mass. Differential Diagnosis The differential diagnosis

includes post-traumatic neuromas in patients with previous pelvic surgery, pelvic abscess, radiation fibrosis and tension myalgias of the pelvic floor. Code

702.X4 nerve infiltration 732.X4 musculoskeletal deposits

Rectal, Perineal and Genital Pain of Psychological Origin (XXV-3) About 10% of psychiatric patients with pain have rectal, perineal or genital pain. This is usually mentioned as a secondary site of pain. Only about 2% report pain in these parts as the primary site. When that happens the rectal pain is usually associated with severe depressive or schizophrenic illness but may also be associated with conversion symptoms. It occurs in a few patients. For the general description, see I-16. Conversion Code

pain in these patients is usually accompanied 81X.9a delusional 81X.9b conversion

by pains elsewhere. See also I-16.

s186

GROUP XXVI: BACKACHE AND PAIN OF NEUROLOGKXL ORIGIN IN TRUNK AND BACK

Prolapsed Intervertebral

Disc (XXVI- 1) See (XXVII-91

502.X 1~.

Acute Herpes Zoster (XXVI-2)

303.X& 403 X2a 503X% 603.X2a

chest abdomen tow back leg

Post-Herpetic

303X2b 403X2b 503.X2b 603X2b

chest abdomen low back leg

Neuralgia (XXVI-3)

302.X4a thorax 402.X45 abdomen 502.X4a low back

Intraspinal Tumour (XXVI-4)

Fracture of Lumbar Vertebrae (XXVI-5) (See Section XXVII) Collapse of Lumbar Vertebrae (XXVI-6)

503.Xla trauma 503.X4a neoplasm 503.X5a metabolic

Epidural Abscess (XXVI-7)

302X2a thorax 402.XZa abdomen 502.X2a low back

Vertebral Tumour (XXVI-8) Definition

Pain in the mid or low back region, often with a radicular component, tumour infiltration of the vertebral body and the epidural space.

Site

Lumbosacral

Systems

Skeletal and nervous systems.

Main Features

The usual age of onset is the 5th, 6th and 7th decades .- corresponding to the occurrence of carcinoma of the breast, lung and prostate. Lumbar metastases produce dull aching mid or low back pain exacerbated by lying or sitting and relieved by standing. Occasionally patients with an Ll lesion will have pain referred to the sacro-iliac region. Sacral metastases produce low back or coccygeal pain which is also amavated by lying or sitting and relieved by waiking. By the time of presentation, most patients will also have radicular pain which is worsened by coughing, sneezing and straining.

due to

spine.

S187 Associated Symptoms

About three-quarters of the patients will present with leg weakness and some of these will be paraplegic. There may also be bowel and bladder dysfunction and impotence. Signs and Laboratory Findings

Spinal tenderness at the level of the involved vertebral bodies is almost always present. Leg weakness can be either unilateral or bilateral. The weakness can be segmental or it can involve all the roots below the level of the lesion if there is obliteration of the spinal canal by tumour. Sensory loss usually corresponds to the site of the lesion. Plain films may show collapse of one or more vertebral bodies and the bone scan may be positive in these areas. Myelography is the definitive study - if there is significant epidural disease, there will be extrinsic compression of the spinal canal or even a complete block to the flow of contrast material. Usual Course

Without intervention, the usual course is progression from local back pain to radicular pain and weakness and then to paraplegia with loss of bowel and bladder function.

Social and Physical Disability

Patients who develop paraplegia and sphincter loss are wheelchair-bound, require assistance in transferring from chair to bed and require urinary catheterization. Pathology

In 80 to 90% of cases tumour seeds the vertebral body and then invades the epidural space to compress the spinal cord and nerve roots.

Summary of Essential Features and Diagnostic Criteria

Metastases to the lumbosacral spine produce local and radicular pain, which can progress to paraplegia, sensory loss and sphincter disturbance if untreated. The pain is usually worsened by lying and sitting and alleviated by standing or walking. One or more of the following studies may show the level of involvement - plain films, bone scan and myelography. Differential Diagnosis

The differential diagnosis includes benign disease such as disc herniation and other malignant conditions such as meningeal carcinomatosis and tumour infiltration of the lumbosacral plexus. Code

303.X4 333.X4

nerve involvement: thorax musculoskeletal involvement: thorax

403.X4 nerve involvement: abdomen 433.X4 musculoskeletal involvement: abdomen 503.X4b nerve involvement: low back 533.X4 musculoskeletal metastasis: low back

s188

(XXVI- 11)

Meningeal Carcinomatosis Definition

Dull aching pain in the lower back or buttocks with radiation Into the legs on d radicular basis. There are usually more signs than symptoms indicating involvement of multiple levels of the neuraxis.

Site

Back and lower extremities.

System

Nervous system (meninges and nerve roots).

Main Features

The age of onset is usually the 5th, 6th and 7th decades for carcinoma of the lung and breast and the younger age groups for lymphoma and leukaemia. The pain is usually severe and may be accompanied by numbness and tingling. The back and buttock pain is usually dull and boring, but the radiular pain may have a burning or lancinating quality. About two-thirds of patients with meningeal carcinomatosis will have back andior leg pain.

Symptoms

Associated

About half the patients with meningeal carcinomatosis will have headache or mental status changes. Two-thirds will have visual blurring or double vision. Signs and Laboratory

Findings

There may be tenderness to percussion over the lumbosacral spine and impairment of straight leg raising. Weakness and numbness occur in radicular distribution and may be multifocal. There may also be papilloedema and cranial nerve abnormalities. Almost all patients will have CSF abnormalities including elevated white count and protein and positive cytology. The myelogram may show multiple nodular defects on nerve roots. usual

course

Pathology Summary

The course is usually inexorably progressive, although breast carcinoma. lymphoma and leukaemia may show a transient response to intrathecal chemotherapy and radiation treatment. Infiltration of nerve roots and meninges with fibrosis and intlammatory changes.

of Essential Features

and Diagnostic

Criteria

The essential features are low back pain with a radicular component and evidence of involvement of multiple levels of the neuraxis. The CSF is almost always abnormal and CSF cytology may be positive. Differential

Diagnosis It is important to exclude a chronic meningeal infection such as fungal meningitis. Metastatic epidural disease can be ruled out by performing a myelogram.

Code

502.X4c (low back) 302.X4c (thorax) 402.X4c (abdomen)

S189

Tumour Infiltration of the Lumbosacral Plexus (XXVI-12) Definition

Progressively extremity.

Site

Lower back and leg.

System

Nervoussystem (lumbosacral plexus).

Main Features

Lumbosacral plexopathy occurs most commonly in patients with genitourinary, gynaecological and colonic cancers as a result of local tumour extension. The local pain is pressure-like or aching in quality. The referred pain varies with the site of plexus involvement and can be burning, crampy or lancinating. Upper lumbar plexus involvement produces pain in the anterior thigh and groin, whereas pain in the L5Sl distribution radiates down the posterior aspect of the leg to the heel. The pain is often worse at night and is usually aggravated by movement of the hip joint.

intense pain in the low back or hip with radiation into the lower

Associated Symptoms Typically, leg weakness and numbness occur 3 to 5 months after the onset of pain. Sphincter disturbance is uncommon. Signs and Laboratory Findings There may be tenderness in the region of the sciatic notch. There is usually limitation of both direct and reverse straight leg raising. Focal weakness and sensory loss with depressed deep tendon reflexes may be evident. The cardinal feature is progressive weakness in a pattern involving more than one nerve root. There may be pedal oedema due to lymphatic obstruction. An IVP may show hydronephrosis. A CT scan through the abdomen and pelvis is the definitive study. It may show a paralumbar or pelvic soft tissue mass and there may be bony erosion of the pelvic side wall. Myelography may be positive if there is epidurai extension of disease. Usual Course

The course is inexorably progressive and leads to a wheelchair or bed-ridden existence.

Summary of EssentialFeatures and Diagnostic Criteria Low back and hip pain radiating into the leg is followed in weeks to months by progressive numbness, paraesthesias, weakness and leg edema. The physical findings indicate that more than one nerve root is involved. CT scan of the abdomen and pelvis is the study of choice. DifferentialDiagnosis Myelography and CSF analysis should rule out epidural and meningeai metastatic disease, respectively. Other entities to consider are radiation fibrosis, lumbosacral neuritis, and disc disease. Code

502.X@

GROUP XXVII: BACK PAIN OF MU!3CULOSKELETAL ORIGIN Osteophyte (XXVII-l) Definition

Pain in the distribution of a dermatome bony spur.

due to encroachment

Site

Rack and lower limb in the dist~bution

of a nerve root.

System

Musculoskeletal.

Main Features

Premlence - common in middle-aged and older men and women. Less frequent than lumbar disc lesions; Onset --. may be sudden or slow, with sudden exacerbation. Usually stiffness and continuous aching pain in the back and in the leg in the distribution of the dermatome. Exacerbations occur with movement and can be accompanied by sharp radicular pain; Course fluctuating or progressive.

Associated

on
Features The L5 and Sl roots are those most often involved. Numbness and tingling affect the Iateral rim of the foot in Sl lesions and the dorsum of the foot and great toe in L5 lesions; occasionally weakness of the appropriate muscle groups occurs, especially the dorsiflexors of the great toe and of the foot in L.5lesions and pbntar flexion of the foot in Sl lesions; quadriceps paralysis (rising from a chair) may be found in L4 and L3 lesions.

Sins

Laboratory

Hypoaesthesia to touch and noxious stimulation; paravertebrat tenderness in the region of the osteophyte; Las&ue sign (“reversed” I_as&ue sign in high lumbar lesions); pain on pressure on the trunk of the sciatic nerve and its divisions down to the Achilles tendon (Valleixs pressure points); muscle spasms, tenderness and occasional diminished reflexes. Findings X-ray may show the ~teoph~e and shows the degenerative CT scan centred on the foramen will show the narrowing foramen. Myelography may be negative. E.M.G. may compression signs. Thermograms may show the abnormal

signs of spondylosis. of the intervertebral show the radicular dermatome.

Relief

Rest, surgical decompression

Pathology

Osteophyte is one of the four major groups of lumbar root entrapment (disc herniation, bony nerve root entrapment, ligamentum compression and adhesive radiculitus. Bony entrapment may mimic the symptoms and signs of disc hemiation.

of the root.

Diff erentiai Diagnosis Ruptured lumbar disc, fracture, abscess, intraspinal tumour causes of nonradicular paralysis involving the lumbar plexus (e.g., diabetic neuropathy), vascular lesions of the iliac arteries and their branches and stenosis of the lumbar canal. Code

533.X&l

s191

Lumbar Spondylolysis (XXVII-2) Definition

Lumbar spondyiolysis is an acquired bony defect in the pars interarticularis and is not necessarily painful.

Site

The most common site for lumbar spondylolysis is the L5 vertebra. Other sites may also be involved including the cervical spine. The defects are typically bilateral but unilateral spondylolysis is also observed.

System

The systems involved are primarily the musculoskeletal system with occasional indirect involvement of the peripheral nervous system.

Main Features

Lumbar spondylolysis seems to occur in the second decade of life and appears to be more common in young active individuals. This defect is more common in males. This may not, however, represent a discrete genetic selection but may be more related to activities. The pain occurs at the time of the injury and results in acute pain which gradually recedes over time. The pain is usually a deep ache, poorly localized in the lumbosacral region.

Associated

Symptoms Initially, symptoms seem to diminish with decreased activity. Most patients do not develop significant progressive symptoms as a result of isolated spondylolysis.

Signs and Laboratory

Findings

The defect is recognized most clearly on oblique radiographs of the lumbar spine. A defect may be present in spite of initially normal roentgenograms. A technetium monodiphosphonate bone scan should be obtained with the use of pin hole columnation. Increased activity in the pars interarticularis suggests the presence of a defect prior to actual radiographic changes. Physical signs include limited motion of the lumbosacral spine with no specific findings. In m&t individuals symptoms resolve over two to three weeks. After rehabilitation, patients gradually resume full, normal, unrestricted activity. Certain individuals develop progressive pain that does not respond to non-operative management. In a few selected individuals, surgical stabilization may be indicated. Some authors have reported primary bone grafting of the defects, but this at present has not achieved widespread acceptance. Complications

Bilateral spondylolysis may lead to lumbar spondylolisthesis skeleton. This does not usually occur in adults.

in the adolescent

Social and Physical Disability Occasional patients develop discomfort with heavy activity and therefore are better off in jobs that require less exertion. Pathology

Summary

The most likely cause for the defect in the pars interarticularis is cumulative or repetitive loading. The defect is therefore a stress or fatigue of bone and is rarely, if ever, developmental. Wiltse has demonstrated healing of the pars interarticularis with immobilization of the spine. of Essential Features and Diagnostic Criteria Lumbar spondylolysis occurs most commonly in the second decade of life and is associated with low back discomfort. Occasional patients have radiculopathy as

SW2 a result of neural compression secondary to exuberant bone formation. The diagnosis is generally made from roentgenograms of the Iumbosacrat spine. A bone scan is also helpful in the assessment and diagnosis of patients. Differential Diagnosis Lumbar spondylolysis as a cause of significant back pain should be a diagnosis of exclusion, since many patients do not have long-term symptoms. Spondylolisthesis, the dislocation of one vertebral body on its neighbour, occurs in a small fraction of the patients with bilateral spondylolysis. Spondyiolisthesis can lead to deep aching lumbosacral pain and radicular pain if nerve roots are compressed. Code

531.Xla

Spinal Stenosis (XXVII-3) Definition

Chronic pain usually experienced in the buttocks and legs, at times extremely severe, usually deep and aching with “heaviness” and “numbness” in the leg, from buttock to foot secondary to compression of the cauda equina, of multiple aetiology, common but often missed.

Site

Low back, buttocks and lower extremities.

System

Musculoskeletal.

Main Features

Patients usually have a long history of gradually increasing back pain, worse with physical activity, and notice a gradual onset of aching in the legs with walking, along with a sense of “heaviness” or “clumsiness”. The pain may also be produced by protracted periods of standing. Usually the distance walked diminishes progressively. The symptoms are exacerbated by extension of the spine and reduced by flexion. Thus, the patient develops a markedly flexed posture of the trunk as walking continues through the day from morning to evening.

Associated

Symptoms There may be dermatomal impotence.

Aggravating

paraesthesiae

and bowel or bladder disturbance

or

Factors Walking, standing.

Signs and Laboratory

Findings

X-rays usuaUy demonstrate diffuse severe degenerative disease with facet hypertrophy and a shallow ant-posterior diameter of the lumbar canal. Cystometrogram may be helpful in diagnosis. Myelography and CT scanning are helpful in showing narrowing of the spinal canal. Magnetic resonance imaging and electrodiagnostic studies can also be helpful in demonstrating the areas involved, The dilemma posed by thii condition is the discrepancy between physical signs, which are usuaily not great, and the subjective complaints.

5193 Aetiology

Congenital factors, e.g. lumbar spondylosis, lumbar spondylolisthesis; tive disease, osteoarthritis.

Treatment

Surgical decompression.

degenera-

DifferentialDiagnosis Peripheral vascular claudication, sciatic nerve compression, osteoarthritis of hip or knee, retroperitoneal tumours, other tumour or abscess, prolapsed lumbar disc. Code

533.X6b (back) 633.X6 (legs)

Sacralization or Lumbarization (Transitional Vertebra) (XXVII-4) Definition

Approximately 5 to 6% of patients have radiographic evidence for transitional lumbar vertebra representing either lumbarization of Sl or sacralization of L.5. This condition may be either unilateral or bilateral. The syndrome usually represents a failure of segmentation involving the development of the lumbosacral junction region.

Site

This condition occurs at the lumbosacral junction.

System

Musculoskeletal.

Main Features

This abnorm~ity, although common, is rarely symptomatic. Many patients have radiographic findings but do not suffer from symptoms. As postulated by Farfan and others, the abnormality may impose stresses on the disc level above. Thus, patients have a slightly higher propensity to develop a disc herniation at the level above a transitional vertebra. Pain in a patient with transitional vertebrae is due to pathology elsewhere, often an adjacent disc or facet. A pseudo-arthrosis associated with a transitional vertebra can lead to diffuse aching pain.

Associated Symptoms As indicated, patients may develop a herniated developmental anomaly.

disc at a level above the

Signs and Laboratory Findings Diagnosis is made from r~nt~n~ams in the antero-poste~or direction. No specific clinical findings have been observed. Usual Course

Since the majority of patients are asymptomatic, developmental anomaly is unknown.

Complications

An occasional patient develops a disc herniation above the anomaly.

and lateral

the usual course of this

Social and Physical DisabiIity Probably none of significance other than the propensity for disc problems at the level above.

s194 Pathology Summary

This anomaly results from a failure of segmentation mental period, usually within the first thirty days. of Essential Features

and Diagnostic

in the early letai develop-

Criteria

This anomaly can be recognized on roentgenograms. have been observed. No neurologic deficits occur.

No specific clrnrcal features

Abnormal Articular Facets (Facet Tropism) (XXVII-5) Definition

Facet tropism is a developmental or acquired condition in which the lumbar articular facets are in different planes. Specifically, one facet joint is in a coronal plane while the contralateral facet joint is in the sag&al plane. Thus, in L&Qstress and strain patterns are altered by this developmental anomaly.

Site

The most common site for facet tropism is the lumbar spine.

Systems

Musculoskeletal

Main Features

The concept of pain in facet tropism is somewhat controversial. Farfan has shown biomechanical evidence that the stresses on the adjacent disc may in fact be increased in this condition and, thus, acceleration in the normal aging process can possibly result. Other authors feel that this condition is similar to spina b&da occulta, in that it is an observed radiographic variation that is nof in any way correlated with symptoms. When pain is associated with abnormal articular facets, it is usually deep and poorly localized but may radiate to the buttocks.

Associated

system is involved.

Symptoms Facet tropism can of course be accompanied by degenerative changes in the intervertebral dii. In addition, the usual symptoms resulting from aging changes of the spine can also be confusing. Spinal stenosis can be due to facet tropism and lead to radicular pain or claudication.

Signs and Laboratory

Findings

The anterior, posterior, lateral, and oblique x-rays of the lumbosacral reveal the lumbar facet joints are in different places.

spine

Usual course

Presumably, the majority of patients who exhibit this radiographic alteration in their anatomy do not have disabling symptoms. It is reasonable, however, to suggest that symptoms may be forthcoming due to the alterations in the usual in u&o stress and strain patterns.

Pat hology

The defect is a developmental defect; if acquired it may be related to abnormal stresses and strains at the joints themselves. The pain may be related to the foregoing or to cauda equina compression or to radiculopathy.

s195 Summary

of Essential Features

and Diagnostic Criteria

Facet tropism represents a developmental alteration in the anatomy of the lumbar facet joints typified by one joint being in the sagittal plane while the other is in a coronal plane. Patients have minimal symptoms, and disability as a direct result of this variation is uncommon. Code

530.X6

Acute Low Back Strain (XXVII-6) (Acute mechanical backache).

backache,

Definition

Acute dull aching, cramping or knife-like back pain due to minimal trauma or to precipitate movement with sudden loading of a motion segment.

Site

Pain in the lumbar region, radiating into buttocks or posterior thighs. Occasionally the maximum pain is located in the groin, testes or shin.

System

Musculoskeletal.

Main Features

Acute minor trauma or strain is the most common type of low back pain and usually occurs in individuals under 40 years of age. It is most often the sequel of lifting, twisting or falling and results from brief unexpected high magnitude forces or loads applied suddenly. A high proportion of the injuries occur at work. The pathophysiology is unknown; diagnoses range from sprain, strain, myofascial syndrome and muscle spasm to ruptured disc. There is dull, aching, nagging, gnawing or cramping pain in the low back with fluctuating intensity, made worse by physical activities. The pain may radiate to the buttocks or occasionally to the thoracic region and is not associated with neurological findings. Patients often have focal lumbar paravertebral muscle spasm and tenderness.

Associated

Lumbosacral

strain, Acute ligamentous

strain, Acute discogenic

Symptoms Decreased range of motion in lumbar spine, stiffness of spine.

Signs and Laboratory

Findings

Muscle spasm, local tenderness in paravertebral muscles and impaired straight leg raising may occur. Loss of normal lordosis may be seen and also shown on x-ray. There is usually a normal CT scan, myelogram and EMG. Usual Course

This is often a self-limited condition which may resolve more quickly if the patient initially avoids physical activity and makes use of physical therapies such as heat, massage and non-narcotic analgesics. However, some cases persist indefinitely, albeit with diminished physical signs. The therapeutic priority initially is symptom relief, then flexibility and exercises for general fitness such as increased standing and walking. Specific muscle strengthening exercises are best left to the last.

Sl%

Complications

This type of injury can, on rare occasions, lead to long-term disability; owertreatment with medication and premature surgery may be factors. Pre-existing personality and environmental factors may also complicate r(‘t-every. Root irritation or deficits may occur.

Pat hology

The exact pathology of acute low back pain following rntnor trauma hcis never been elucidated.

Summary

of Essential Features and Diagnostic

Criteria

Onset of symptoms immediately following tninor trauma or brief unexpected high magnitude forces; local muscle spasm and tenderness; symptoms usually resolve. Recurrences likely. Differential Diagnosis Herniated nucleus pulposus (especially “midline” at L3-4, L4-5. or L5-Sl). degenerative arthritis of lumbar spine, congenital abnormalities of spine. retroperitoneal pathology, disc-space infection. Code or

531.X8 531.Xlb

Recurrent Low Back Strain (XXVII-7) (Postural backache,

Recurrent mechanical backache)

Definition

Mild or moderately severe dull aching or burning back pain correlated with prolonged posture of sitting or standing or repetitive awkward movements or postures.

Site

Lower back, buttocks, posterior thighs; rarely pain radiates to the thoracic region. Uncommonly pain presents primarily in the groin, testes or shin.

Systems

Musculoskeletal.

Main Features

Recurrent pain usually of mild to moderate intensity, aching fluctuating or continuous in nature; often worse with activity and particularly with prolonged or repetitive awkward postures of movements. Pain is maximal at the end of the day or of the working shift. No significant neurological abnormalities usually occur but particular activities either at home or at work will promote recurrences.

Signs and Laboratory

Findings

Sometimes spasm, loss of lordosis, limitation of movement.

usual

course

Pathology

Becomes chronic with the persistence

of the provocative activities.

Unknown. Current thinking attributes pain to mechanical instability of a motion segment as distinct from any specific structure.

s197 Essential Features

Recurring pain exacerbated by specific activities; particularly those activities associated with repetitive loading or prolonged loading with hyperextension of a motion segment. Differential Diagnosis

Herniated nucleus pulposus, retroperitoneal facet hyperextension sprain. Code

pathology, myofascial syndrome,

532.X7b

Trauma, Acute (XXVII-8) Definition

Pain in low back, usually severe, associated with fractures or dislocation of spine and disruption of joints, ligaments, tendons, from major trauma (usually motorvehicle accident or fall of greater than 6 feet (1.85 m)).

Site

Pain in low back, may radiate into flanks or legs depending upon level of injury.

Systems

Musculoskeletal.

Main Features

There is a clear-cut history of severe trauma with immediate pain, usually severe, and perhaps with loss of neurological function. The patient may have a visible and palpable deformity of the spine with a local haematoma. There will often be associated muscle spasm.

Associated Symptoms

If neurological damage: paresis of legs, bowel and bladder sphincters. Hypovolemic shock if major retroperitoneal haemorrhage or visceral damage. Signs and Laboratory Findings

X-rays show fracture or dislocation; nuclear studies may demonstrate early compression. CT scan may show disruption of neural canal. Physical examination shows neurological deficits. Usual Course

The injury requires reduction

and fixation of fractures to prevent progressive deformity and neurological deficit; pain abates in a few days if the back is properly stabilized. A small percentage will develop chronic pain because of a complication of the original injury.

Complications

Progressive kyphosis, pain, increased neurological deficit.

Social and Physical Disability The initial injury may require up to three months to heal prior to resumption

of weight-bearing. Neurological deficits (which can include paraplegia) may require extensive social and physical rehabilitation.

S198 Pathology

Fracture of lumbar spine, disruption of associated ligaments and tendons and muscles, damage to nerve roots, local haematoma.

Essential Features Major trauma; disruptjon of lumbar spine; sometimes associated neurological deficits; severe pain which usually abates over a week or so. Differential Diagnosis Injury to retroperitoneal Code

structures.

531.Xlc

Chronic Mechanical Low Back Pain (XXVII-9) Definition

Continuous dull aching or burning pain in the low back associated with motion segment failure and made worse by specific activities. Duration beyond 6 months and associated with significant impairment of activities of daily livinganr$or work activities.

Site

Low back; may radiate to flanks or into buttocks, legs, abdomen. Occasionally the primary presentation is pain in the groin, testes, perineum or shin.

Systems

Muscuioskeletal.

Main Features

Continuous, dull, nagging, aching or burning low back pain, sometimes with extension to the abdomen, perineum or legs. The pain due to mechanical instability is usually associated with m~ement or specific positions (sitting, standing and especially extension from the flexed posture). It is usually a deep, aching or burning sensation. There is often a prior history of recurrent postural strain, disc disease, or hyperextension strain. It interferes with activities of daily living, work and leisure as well. There is markedly reduced endurance for sitting, standing or walking.

Associited

Features Often depression and insomnia.

Signs and Laboratory

Findings

Weak abdominal musculature. Loss of normal lordosis. Impairment of movement of the low back (flexion or extension or rotation), limitation of straight leg raising. Local muscle spasm or tenderness may be present. Neurological deficits may be confirmed by E&IG. usual

course

Complications

Chronic. Continued pain of this type can interfere with rehabilitation depression, Disability may be disproportionate to the known organic pathology.

and add to mechanical!

s199 Social and Physical Disabilities Chronic pain can limit both physical and social rehabilitation after injury. Pathology

Not known. Some associated degenerative spinal disease (osteoarthritis) is very likely to be present but its contribution is uncertain. Less often other evidence of bony change, compression of nerve roots by disc protrusion, etc. will complicate the condition.

Essential Features Chronic low back pain related to activity, and multiple possible contributory factors. Differential Diagnosis Herniated nucleus pulposus, disc space infection, inadequate decompression nerve roots following fracture of spine. Code

of

533.X1 mechanical instability

Ankylosing Spondylitis (XXVII-12) Definition

Aching low back pain and stiffness of gradual development inflammatory change of unknown origin.

Site

Low back.

System

Musculoskeletal.

Main Features

Prevalence in 1 to 2% of the population. Males 9 times more common than females. Peak onset in the 3rd or 4th decades. More common in Caucasian populations, but some other ethnic groups, e.g., Haida Indians, have unusually high prevalence rates. Chronic, persistent low back pain of insidious onset, aching discomfort, and stiffness while sleeping that forces the patient to get up and move around; morning stiffness is usually greater than half an hour in duration and stiffness occurs also after periods of inactivity (“gelling phenomenon”).

Associated

due to chronic

Symptoms Peripheral joint disease in 20% of patients, conjunctivitis and iritis in 25% of patients, chronic pulmonary fibrosis and cardiovascular disease.

Aggravating

Features Inactivity.

Signs Laboratory

Depressed spinal mobility and chest expansion with chest involvement. Findings None specific; 90% of patients are HLA-B27 positive, but 8 to 10% of normal populations also are HLA-B27 positive.

Radiographic Findings Bilateral symmetric sacroiiiitis; s~d~mophytes

usual

course

Chronic lumbar pain often with acute diminishes as spine fuses.

of lumbar thoractc spines

exacerbations

intermittently.

pain

Relief

Some severe morning stiffness of pain abates with activity. A good response to nonsteroidal anti-inflammatory drugs.

Complications

Spinal immobility, fracture of fused spine. Hiier spinal disease may cause vertical odontoid subluxation or penetration with brain-stem compression.

Aetiology

Unknown; may be imrnunol~jc~. with possible environmenral with apparent genetic susceptibility.

tactors. along

Essential Features Chronic aching lumbar pain and stiffness with “gelling” and with characteristic x-ray changes as described. Differential

Diagnosis Psoriatic spondyiitis; Reiter’s spondylitis; mechanical back pain; discogenic back pain.

Code

533.X8b

Quadratus Lumborum Syndrome (XXVII-13) Definition

Dull aching pain arising from either the deep or superficial fibres of the quadratus lumborum muscle.

Site

Quadratus

System

Musculoskeletal.

Main Features

UsuaIly there is dull, aching pain that varies with movement or posture. It may also be expressed as a “stitch in the side”. Patients with such trigger points (TPs) may have pain on walking, when twisting, while stooping, when turning in bed, upon rising from a chair, when coughing or sneezing, and while climbing stairs; also pain on deep inspiration due to attachment of the muscle to the 12th rib. Pain during rest times may be particularly severe.

lumborum muscle.

The effects from different parts are as follows: (1) Superficicz~ Fibres -.- the superficial tibres refer pain to the buttocks, the lateral side of the hip region, over the iliac crest and downward to the greater trochanter, and occasionally anteriorly to the lower abdomen and into the groin; and (‘2)Deep Fibres - - the deep fibres refer pain downward posteriorly to the sacro%ac joint and deep into the middle buttock region. Bilateral involvement of the quadratus lumborum may lead to pain on both sides of the back at the Levelof the lumbosacral junction. Pain may be extended downward into the lower extremity when satellite TPs have developed in the gluteus minimus muscle.

s201 Aggravating Factors

A discrepancy in leg length while sitting or standing, or a smallhemipelvis; a tilting of a normal pelvis because of a wallet in the back pocket; short upper arms in relation to the torso height - this requires the patient to assume awkward positions to obtain elbow support which chronically strains the muscle. Signs

The patient may use guarded movement while walking, rising from a chair, or while on a couch. Listing of the pelvis to one side may occur while standing. A distinct area of tenderness may be located near the fifth lumbar transverse process. In addition, there is pain and limited flexion and extension of the spine as well as localized tenderness overlying the muscle on at least one side.

Pathology

See myofascial pain syndromes.

Aetiology

Trigger points in the quadratus lumborum may be activated by any of the following events: a quick stooping movement when the torso was twisted more to one side, a fall, low back strain following sustained or repeated overloads (gardening, scrubbing a floor), and lastly by a trigger point in the contralateral muscle. An alternative interpretation of the phenomena is that they represent referred pain from instability of the lumbar motion segment resulting in posterior facet hyperextension strain, i.e., recurrent mechanical low back strain.

Differential Diagnosis

Trochanteric Code

bursitis, sacroiliac joint dysfunction.

532.X7c

Gluteal Syndromes (XXVII-14) Definition

Aching myofascial pain arising from trigger points located in one of the three gluteal muscles.

Site

Gluteus maximus, medius or minimus muscles.

System

Musculoskeletal.

Main Features

Aching pain related to the gluteal muscles according to the following patterns: Gluteus Maximus: Trigger points in this muscle may refer pain to any part of the buttock or coccyx areas. GIuteus Medius: Trigger points in thii muscle refer pain medially over the sacrum, laterally along the iliac crest, and occasionally downward to the mid-buttock level and upper portion of the posterior thigh. Sometimes it travels far down into the calf. When this occurs it mimics the pain pattern of “sciatica”. Gluteus Minimus: Trigger points may arise in either the posterior or superior aspects of this muscle. Those in the posterior portion refer pain downward into the lower part of the buttock, the posterior part of the thigh and rarely to the posterior part of the calf. The knee joint is spared in this distribution. Again, this pattern is similar to that of sciatica and also of other low back pain conditions involving the gluteal musculature. Trigger points located in the anterior portion refer pain similarly except that it is distributed along the lateral rather than posterior aspect of the thigh and calf.

s202 Aggravating Factors A foot with a long second and short first metatarsal bone. It can act as a perpetuating factor for all the &teal muscles, especially the gluteus medius. Signs

Pressure on the responsible TP will reproduce the referred pain pattern. Straight leg raising is usually restricted due to tightness in the hamstring and gluteus maximum muscles.

Pathology

See myofascial pain syndromes.

Aetiology

Trigger points of the gluteal musculature very often function as sateilite TPs of those located in the quadratus lumborum muscle.

DifferentialDiagnosis Sacroiliac joint dysfunction, Code

533.x7c

sciatic neuritis, pyriformis syndrome.

s203

GROUP XXVIII: BACK PAIN OF VISCERAL ORIGIN Carcinoma of the Rectum (XXVIII-I) Code

753.X4 (pelvic pain) 853.X4 (perineal pain)

See also: XVII, XXI, XXIV, and XXV

GROUP XXIX: LOW BACK PAIN OF PSYCHOLOGICAL

ORIGIN

The frequency of low back pain due to solely psychological causes is unknown, but probably relatively low compared with low back pain overall, which of course is very common. Psychological causes may play a very important part in protracted low back pain in a large number of patients. I-iowever they will rarely be seen to be the sole cause of the pain, nor will the diagnosis emphasize them in the first instance. See I-16. Code

533.X7b (tension) 51X.X% (delusional) 51X.X9b (conversion)

S204

GROUP XXXz LOCAL SYNDRObES IN THE LEG OR FOOT PAIN OF NEUROLOGICAL ORIGIN LateraI Femoral Cutaneous Neuropathy (MeraIgia Paraesthetica)

(xXx-1) Definition

Hypoaesthesia and painful dysaesthesia cutaneous nerve.

Site

Upper anterolateral

System

Peripheral nervous system.

Main Features

Prevalence

S&s

Hypoaesthesia and paraesthesia in upper anterolateral thigh; occasionaily tenderness over lateral femoral cutaneous nerve as it passes through iliacus fascia under inguinal ligament.

Relief

Reassurance is the first step. if pelvic tilt is a factor, it should becompensatedwith heel lift. Diabetes or any other systemic disease will be treated appropriately. In obese patients the pain wilf benefit from lossof weight. Surgical decompression of the lateral femoral cutaneous nerve as it passes under the inguinal fiiment is, on rare occasions, helpful in the patient who has failed conservative therapy.

in the distribution of the lateral femorai

thigh.

-.. more common in middle age, males slightly more often than females. Associated with obesity, pregnancy, trauma to inguinal region, diabetes mellitis and, possibly other factors; Time Course - usually gradually abates over months to years without specific therapy. All complaints are of pain or related sensations in the upper anterolateral thiih region; patients may describe burning, tingling, aching, numbness, h~r~nsiti~~ to touch or just vague discomfort.

Essential Features Hypoaesthesia

and paraesthesia

in upper anterolateral

thigh.

Differential Diagnosis ~diculo~thy of L2 or 13; upper lumbosacrai plexus lesion due to infection or tumor; entrapment of superior gfuteal nerve (pyriformis syndrome); arthropathy of hip, or rarely the knee. Code

602.Xla

s205

Obturator

Neuralgia (xXx-2)

Definition

Pain in the distribution of the obturator nerve.

Site

Groin and medial thigh as far distal as the knee; usually unilateral.

System

Peripheral nervous system.

Main Features

Constant pain in the groin and medial thigh; there may be sensory loss in medial thigh and weakness in thigh adductor muscles. Indefinite persistence if cause not treated.

Associated Symptoms

If secondary to obturator hernia, pain is increased by an increase in intraabdominal pressure. If secondary to osteitis pubis, pain is increased by walking or hip motions. May be tender in region of obturator canal. Signs

Hypoaesthesia muscles.

of medial thigh region, weakness

and atrophy

in adductor

Laboratory Findings

If the neuropathy is severe, there may be EMG evidence of denervation adductor muscles of the thigh. UsuaI course

Constant

Complications

Progressive loss of sensory and motor functions in obturator nerve.

in the

aching pain which persists unless the cause is treated successfully.

Social and Physical Disability

When severe, may impede ambulation and physical activity involving hip. Pathology

Obturator hernia; osteitis pubis, often secondary to lower urinary tract infection or surgery; lateral pelvic neoplasm encroaching on nerve.

Essential Features

Pain in groin and medial thigh; with time the development changes in obturator nerve distribution.

of sensory and motor

Differential Diagnosis

Tumor or inflammation involving I2-L4 roots, psoas muscle, pelvic side wall. Hip arthropathy. Code or

602.X6a 602.Xlb 602X2a 602.X4a

(obturator hernia) (surgery) (inflammation) (neoplasm)

s206

Femoral Neuralgia (xXx-3) Definition

Pain in the distribution of the femoral nerve.

Site

Anterior surface of thigh, anteromedial surface of leg, medial aspect of foot to base of first toe.

System

Peripheral nervous system.

Main Features

Constant aching pain in anterior thigh, knee, medial leg and foot. The pain may involve only a portion of the sensory field due to pathology in only one branch of the nerve. There may be sensory loss in similar areas and weakness of the quadriceps femoris, sartorius and associated hip flexor muscles.

Associited

Symptoms If the disorder is secondary to femoral hernia, pain is increased by increase tn intra-abdominal pressure. Trauma to the saphenous nerve may result in an isolated sensory deficit in the knee or leg with local pain.

Sians

Hypoaesthesia in anterior thigh, medial leg and foot or portion thereof; weakness and atrophy in sartorius or quadriceps femoris muscles if lesion proximal to upper thigh. There may be local tenderness at the site of nerve iniuy.

Laboratory Findings If the neuropathy is severe, there may be EMG evidence of denervation in sartorius and quadriceps femoris muscles. Usual Course

Constant aching pain which persists unless cause is successfully treated.

Complications

Progressive sensory and motor loss in femoral nerve or its branches depending upon site of lesion.

Social and Physical Disability Major gait disturbance if quadriceps femoris is paretic Pathology

Trauma to femoral nerve or its branches; femoral hernia.

Essential Features Pain, weakness and sensory loss in the distribution of the femoral nerve or its branches. Differential Diagnosis Neoplasm or infection impinging upon femoral nerve, L2-L4 roots, psoas muscle or pelvic sidewall. Hip or knee arthropathy.

Code

602X2b (inflammation) 602.X4b (neoplasm) 602.X6b (arthropathy)

9207

Sciatica Neuralgia (xXx-4) Definition

Pain in the distribution of the sciatic nerve due to pathology of the nerve itself.

Site

Lower extremity; may vary from gluteal crease to toes depending upon level of nerve injury.

System

Peripheral nervous system.

Main Features

Continuous or lancinating pain or both, referred to the region innervated by the damaged portion of the nerve; exacerbated by manipulation or palpation of the involved segment of the sciatic nerve.

Associated Symptoms

Weakness and sensory loss in muscles and other tissues innervated by the damaged portion of the nerve; secondary changes due to denervation if there is major injury to the nerve. Signs

Sensory loss; weakness, atrophy and reduced reflexes in denervated

muscles.

Laboratory Findings

Electromyographic and nerve conduction studies document nerve damage; roentgenograms or CT scans may reveal lesion causing nerve damage. Usual Course

If a progressing lesion is the cause of the pain, the patient will have an increasing neurological deficit and pain may decrease. If a static intraneural lesion is the cause of the pain, the neurological deficit is fixed and pain is likely to persist indefinitely.

Relief

Remove offending lesion impinging upon nerve.

Complications

Progressive neurological deficit in the territory of the involved nerve.

Social and Physical Disability

Severe pain can preclude normal daily activities; a variable loss of function occurs due to nerve damage. Pathology

Varying degrees of myelin and axonal damage within nerve. Compression of the sciatic nerve by the pyriformis muscle can be a cause. The actual cause of the pain is unknown.

Essential Features

Pain in the distribution of the damaged nerve. Differential Diagnosis

Myelopathy, radiculopathy, lumbosacral plexus lesion involving L&S1 segments. Code

602.Xlc

St208

InterdigitalNeuralgia of the Foot (Morton’s Metatarsalgb) (xXx-5) Definition

Pain in the metatarsal region

Site

Usually in the area of the third and fourth metatarsal heads

System Involved

Peripheral nervous sysrem.

Main Features

Constant aching pain, often lancinating; ohen worse at night or during exercise; perceived in the region of the metatarsal head. Most commonly involves third and fourth metatarsals.

Signs

Hypoaesthesia of opposing surface of adjacent toes; focal tenderness between metatarsal heads when palpated.

Laboratory

Tests None

usual course

Pain initially when walking, relieved by rest. Progressively severe and frequent lancinating pain in the toes associated with constant metatarsal ache. May follow local trauma. Some cases spontaneously remit. Often associated with abnormal postures (narrow shoes or high heels) or deformities of the foot and alleviated by treatment of causative condition.

Relief

Orthotic devices to force plantar flexion, i.e., metatarsal bars or pads; local infiltration of steroids with local anaesthetic; when conservative therapy fails, incision of tranverse metatarsal ligament and excision of interdiital neuroma.

Pathology

Compression of interdigital nerve by metatarsal heads and transverse metatarsai ligament; development of interdigital neuroma.

Essential Features Pain in region of metatarsal heads exacerbated by weight-bearing Differential

Diagnosis Sciatic or peroneal neuropathy, plantar fasciitis, metatarsal pathology, such as inflammation, march fracture, or osteoporosis of metatarsal head “Friebergs infraction”.

Code

603.Xlb

S209

Painful Legs and Moving Toes (xXx-7) Definition

Deep pain, often gnawing, twisting or aching in an extremity, with involuntary movements of the extremity, especially the digits.

Site

Pain in lower leg and foot, and sometimes toe. It may be unilateral or bilateral, or start uniiater~ly and spread to the other limb.

System

Nervous system. In some cases peripheral causes have been described; the spinal cord is probably also involved.

Main Features

Pain is usually severe, deep, poorly localized. It is often described as gnawing, twisting, aching. It is more severe in the leg than in the periphery. Sometimes the pain is relieved by activity though it may be worse following exercise. It occurs in the second half of fife. The movements may be florid or almost im~rceptible, and in the latter case, the patient may never have noticed them. They consist of irregular, involuntary, and sometimes writhing movement of the toes, and they cannot be imitated voluntarily. They can be suppressed for a minute or two by voluntary effort and then return when the patient no longer attends to them. There can also be movements of the feet. There is not usually a relation between the pain and the movements.

Usual Course

It continues indefinitely.

Relief

No consistently effective measures have been found.

Pathology

Precise pathology unknown, spinal cord damage.

Code

602.X8 (legs) 202.X8 (arms) (See X-5 for reference)

References

Spillane, J.D., Nathan, P.W., Kelly, R.E. and Marsden, CD., Painful legs and moving toes, Brain, 94 (1971) 541556.

but nerve root lesions have been described, and

Nathan, P.W. Painful legs and moving toes: Evidence on the site of the lesion. J. Neural. Neurosurg. Psychiatry, 41(1978) 934939. Montagna, P., Cirignotta, F. Sacquegna, T., Martinelli, P., Ambrosetta, G. and Lugaresi, E., Painful legs and moving toes: Associated with polyneuropathy, J. Neural. Neurosurg. Psychiatry, 46 (1983) 399403.

s210

Metastatic Disease (XXX-S) Definition

Pain in the hip joint and thigh region due to tumour infiltration oi bone

Site

Acetabulum,

System

Skeletal system.

Main Features

Metastases to the hip joint region produce contrnuous aching or throbhing pain in the groin with radiation through to the buttock and down the medial thigh to the knee. The pain is made worse by movements of the hip joint and is especially severe on weight-bearing. A metastatic deposit to the femoral shaft produces local pain which is also aggravated by weight-bearing.

Associated

head of the femur, femoral neck and femoral shaft.

Symptoms Pain at rest due to tumour infiltration of bone usually responds reasvnabiy well to nonsteroidal anti-in~ammato~ drugs and narcotic analgesics. Pain due to hip movement or weight-bearing responds poorly to analgesic agents.

Signs and Laboratory

Findings

There may be tenderness in the groin and in the region of the greater trochanter. Internal and external rotation of the hip are especially painfut. There is usually no deformity unless a pathol~c~ fracture has occurred. Plain films and bone scan may be positive. Complications

Summary

The major complication is a pathological fracture of the femoral neck or the femoral shaft. This of course puts the patient to bed. Hip replacement or internal fixation of the femur produces dramatic pain relief.

of Essential Features

and Diagnostic

Criteria

The essential features for disease in the hip joint are severe pain in the groin with radiation into the buttock and down the medial thigh. There is usually tenderness in the groin and increased pain on internal and external rotation. Plain films and bone scan may be positive. Differential Diinosis The differential diagnosis includes upper lumbar pkaxopathy, avascular necrosis of the femoral head, and septic arthritis and radiation fibrosis of the hip joint. Code

633.x4

s211

GROUP XXXI: PAIN S~DRO~ES PKJSCULOSKELETAL ORIGIN

OF HIP AND THIGH OF

IschiaI Bursitis @XXI-l) Definition

Severe, sharp or aching pain syndrome arising from inflammatory lesion of ischial bursa.

Site

Buttock.

System

Musculoskeletal.

Main Features

Uncommon. There is often severe sharp or aching pain while sitting or lying. With coexistent sciatic irritation, the pain may be acute, radiating in the sciatic distribution. Attacks may last weeks or months without treatment. Cases are often secondary to systemic inflammatory disease, such as ankylosing spondylitis, rheumatoid arthritis or Reiter’s syndrome.

Signs

Tenderness deep in buttock patient’s symptoms.

Reiief

Injection into the ischial bursa with local anaesthetic cushion as used for treatment of haemorrhoids.

Complications

None.

Pathology

Inflammatory process of ischial bursa usually occurring with repeated trauma.

over ischial tuberosity,

which reproduces

the

and steroid; “doughnut”

EssentialFeatures Recurring injection.

pain in ischial region aggravated

by sitting or lying, relieved by

DifferentialDiagnosis Acute sciatica, spondylo-arthropathies, Code

prostatitis.

533.x3

Trochanteric Bursitis (XXXI-2) Definition

Aching or burning pain in the high lateral part of the thigh and in the buttock caused by inflammation of the trochanteric bursa.

Site

Thigh and buttock.

System

Musculoskeletal.

s212 Main Features

Aggravating

Very common condition especially over 40 years of age, marked by severe aching or burning pain usually perceived by the patient to be “in the hip” but the pain is localized to the high lateral thigh, and low buttock, often radiating to the knee. It is characteristically impossible to sleep on the affected side. The acute episode may last weeks to months and may recur. Often associated with mild “hip” stiffness, somewhat relieved by activity.

Factors Aggravated by climbing stairs, extension of the back from flexion with knees straight.

Signs

Tenderness usually one inch (2.54 cm) posterior and superior 10 the greater trochanter, which reproduces patient’s symptoms.

usual course

Usually of sudden onset. The pain tends to be severe and persistent. If untreated it may last for several months. Repeated attacks occur at variable intervals.

Relief

Local infiltration of local anaesthetic and steroid into the area ot the greatest tenderness produces excellent pain relief.

Compliitions

None.

Pathology

Inflammatory process of bursa caused by repeated trauma or generalized inflammation such as rheumatoid arthritis.

Essential Features Local pain aggravated by climbing stairs, extension of the back from flexion with knees straight.

Differential Diagnosis Disorders of the hip joint. Referred pain from diseases of lumbosacral spine. Code

634.X3b

Osteoarthritisof the Hip (xXx1-3) Definition

Pain due to primary or secondary degenerative process involving the hip joint.

Main Features

As for osteoarthritis. Often felt deep in the groin, sometimes buttock or thigh. reproduced on passive or active movement of hip joint through a range of motion. As disease progresses, range of motion declines. Other features as for osteoarthritis (I-11).

Code

638.X6b

S213

GROUP xXx11: MUSCULOSKELETAL

SYNDROMES OF THE LEG

Osteoarthritis of the Knee (xXx11-2) Definition

Pain due to a degenerative the knee joint.

System

Musculoskeletal.

Main Features

As for osteoarthritis but localized to the knee. Epidemiology, aggravating and relieving features, signs, usual course, physical disability, pathology, and differential diagnosis as for osteoarthritis (I-11).

Code

638.X6c

process of one or more of the three compartments

of

Night Cramps (XXX&S) Definition

Painful nocturnal cramps in the calves.

Site

Lower limbs.

System

Musculoskeletal.

Main Features

Severe aching cramps in the calves of the legs often preventing the patient from sleep or waking him from sleep. Nightly pain for variable intervals which recur frequently in clusters. Experienced especially by children and the elderly, but can occur at any age.

Aggravating Factors

Aggravated by prolonged walking or standing on concrete provoked by sudden dorsiflexion of ankle or knee joint. Relief

Walking, moving the legs, elevation of the legs or calf stretching provide occasional relief. Treatment with quinine calcium supplements, diphenhydramine, diphenyl hydantoin, or vitamin E (alphatocopherol) may be helpful.

Differential Diagnosis

Electrolyte disorder, hypothyroidism. Code

floor. May be

634.X8

S214

Plantar Fasciitis (XXX&4) Definition

Pain in the foot caused by inflammation of the plantar aponeurost!-

Site

Foot

System

Musculoskeletal.

Main Features

Pain with insidious onset in the plantar region of the foot, especially worse when initiating walking. Worse with prolonged activity. The patient may describe shooting or burning in the heel with each step.

Signs

Tenderness along the plantar fascia when ankle is dorsiflexed.

Radiographic Findings Often associated with calcaneal spur when chronic.

Relief

Arch supports, local injection of corticosteroid, oral nonsteroidal anti-inflammatory agents. Surgery as a last resort.

Pathology

15% have some form of systemic rheumatic disease, usually a sero-negative form of spondylo-arthritis.

Differential Diiosis Keiter’s syndrome. arthritis.

Code

633.X3

ankylosing spondylitis, rheumatoid

arthritis. psoriatic