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Orthopedic Problems of the Shoulder and Neck
Common Orthopedic Problems
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Orthopedic Problems of the Shoulder and Neck
Robert N. Hensinger, M.D. *
If one excludes trauma, problems of the neck and shoulder in children are relatively uncommon. Still, the pediatrician or family practitioner should have sufficient familiarity to provide the family with appropriate counseling and referral. This article emphasizes the common problems but also notes less common conditions which, if unrecognized, may have serious impact on the health and well-being of the child. TORTICOLLIS Torticollis, or wryneck, is a common clinical sign found in a wide variety of childhood illnesses. When torticollis is recognized at or near the time of birth, the usual cause is congenital muscular torticollis. However, one must exclude other less common congenital conditions such as a fixed or bony torticollis due to the Klippel-Feil syndrome; anomalies of the atlantoaxial articulation; soft tissue problems, abnormal skin webs or folds (pterygium colli); and tumors in the region of the sternocleidomastoid (cystic hygroma, branchial cleft cysts, and thyroid teratomas). Congenital Muscular Torticollis (Congenital Wryneck) Congenital muscular torticollis usually is discovered in the first 6 to 8 weeks of life (Fig. 1A). If the infant is examined within the first month of life, a mass or "tumor" is usually palpable in the neck. 16 It is generally a nontender, soft enlargement that is mobile beneath the skin and attached to, or located within, the body of the sternocleidomastoid muscle (Fig. 1B). The mass attains maximal size within the first month and then gradually regresses. If the child is examined at 4 to 6 months of age, the tumor is usually absent and the contracture of the sternocleidomastoid muscle and torticollis posture are the only clinical findings. Recent pathologic studies *Professor, Department of Surgery, Section of Orthopedic Surgery, University of Michigan Medical Center, Ann Arbor, Michigan
Pediatric Clinics of North America-Vol. 33, No.6, December 1986
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Figure 1. A, One-year-old child with congenital muscular torticollis due to contracture of the sternocleidomastoid. The head is tilted toward the involved side and the chain rotated toward the contralateral shoulder. B, One-month-old infant with congenital muscular torticollis with the typical mass in the neck.
suggest that, with time, the fibrosis of the sternal head of the muscle may entrap and compromise the branch of the accessory nerve to the clavicular head, further increasing the deformity. 26 If the condition is progressive, deformities of the face and skull can result and are usually apparent within the first year. Flattening of the face on the side of the contracted sternocleidomastoid muscle may be impressive, and is due to the position of the head while the child sleeps. If the child sleeps prone, it is more comfortable to have the affected side down. As a consequence, the face remodels to conform to the bed. In children who sleep supine, reverse modeling of the contralateral aspect of the skull (plagiocephaly) will be evident. If the condition remains untreated during the growth years, the level of the eyes and ears are distorted and may result in considerable cosmetic deformity. 30 Congenital muscular torticollis is not accompanied by bone abnormalities or neurologic deficit and is believed to be caused by ischemia of the sternocleidomastoid muscle, particularly the sternal head, because of intrauterine positioning or increased pressure during passage through the birth canal. 16, 19 A common misconception is that the neck is contused during delivery and the resultant hematoma leads to fibrosis and contracture. However, experimental work suggests that the fibrosis within the muscle is due to venous occlusion from pressure on the neck in the birth canal. 2 The clinical deformity is probably related to the ratio of the extent of fibrosis to the amount of remaining functional muscle after the initial insult. 7 If sufficient normal muscle is present, it will stretch with growth and the child will not develop a torticollis posture, whereas the fibrotic area has little elastic potential. There are two additional factors that as yet are unexplained. In three of four children, the lesion is on the right side, 16, 19 and 20 per cent of the children have congenital dysplasia of the hip. 14 Excellent results can be obtained in the majority of patients with
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Figure 2. Five-year-old child with untreated torticollis. Thick fibrotic tendon-like bands have replaced the sternocleidomastoid as if the head is tethered to the clavicle. Note the heads of the sternocleidomastoid, one inserting on the clavicle and the other on the sternum.
stretching exercises. 16. 19 Standard maneuvers include positioning of the ear that is opposite the contracted muscle to the shoulder and the chin to the shoulder on the affected side. When adequate stretching has been obtained in the neutral position, these maneuvers should be repeated with the head hyperextended. Other treatment measures include positioning of crib toys so that the neck will be stretched when trying to reach and grasp. If conservative measures are unsuccessful by 18 to 24 months of age, surgical resection is required, usually of a portion of the tendon at its distal attachment? (Fig. 2). The surgery should be performed prior to school age; however, a good (but not perfect) cosmetic result can be obtained as late as 12 years of age. 7.30 Asymmetry of the skull and face will correct as long as adequate growth potential remains after the contracture of the sternocleidomastoid is released. 13 The results of surgery have been uniformly good, with a low incidence of complications or recurrence, and nearly all patients are pleased with the end result. 5. 7. 16. 19. 30
Atlantoaxial Rotatory Subluxation The torticollis posture will occur if the atlas locks in rotation on the axis. In children, this occasionally develops following an upper respiratory infection or surgery in the upper pharynx, such as tonsillectomy, and has been referred to as spontaneous hyperemic subluxation of the atlas. It is assumed that the inflammation of the posterior pharynx leads to softening and laxity of the capsular structures about the joints, permitting excessive rotation of Cion C2, and locking of the facets. This is seldom accompanied
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Figure 3. Intervertebral disc calcification of childhood. Sevenyear-old girl with spontaneous onset of torticollis. Cervical roentgenogram demonstrates calcification between C3 and C4.
by neurologic symptoms or signs, and pain is mild unless one tries forcibly to correct the deformity. Roentgenographic documentation is difficult, owing to the unusual position of the head and neck, and open-mouth views, computerized tomography, and cineradiography may be necessary.lO Simple conservative measures to relieve symptoms, such as a soft cervical collar, are usually sufficient. Those who fail to respond within a few days will require bed rest and light cervical traction. If the condition is allowed to persist, the child may develop a fixed deformity requiring surgical correction. lO Intervertebral Disc Calcification in Childhood Spontaneous calcification of a disc is an unusual phenomenon of unknown etiology and has been found in all vertebral levels. The cervical spine is the most common region reported, as 90 per cent of these are symptomatic. 29 Clinical symptoms are usually muscle spasm, torticollis, localized pain, and nonspecific low-grade fever. Symptoms are acute in onset and may be related to trauma (30 per cent) or upper respiratory infection (15 per cent). The symptoms may last only a few days or persist for 1 to 2 months. Laboratory studies are usually within normal limits. The calcification typically disappears within 2 to 3 months, but may be permanent. On the roentgenogram, there is a fluffy area of calcification within the nucleus pulposis but not involving the annulus (Fig. 3). Herniation of the disc is an infrequent complication. When symptomatic, bed rest, mild analgesics, and cervical collar appear to be adequate therapy, usually
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resulting in rapid disappearance of symptoms, two thirds in 3 weeks and 95 per cent within 6 months. 30 Inflammatory Conditions Local irritation from cervical lymphadenitis may lead to the appearance of a wryneck or tilt of the head. Another less frequent cause is a retropharyngeal abscess following inflammation of the posterior pharynx or tonsillitis. Children with polyarticular juvenile rheumatoid arthritis frequently develop involvement of the cervical joints. 3 Torticollis and limitation of cervical motion may be the only clinical signs. The child should be followed closely, as the condition may result in spontaneous fusion of the entire cervial spine. 13 Other Causes of Torticollis Intermittent torticollis can occur in the young child. A seizure-like disorder called benign paroxysmal torticollis of infancy is due to many neurologic causes, including drug intoxication. 25 Similarly, Sandifer's syndrome, involving gastroesophageal reflux with sudden posturing of the trunk and torticollis, is being recognized more often, particularly in the neurologically handicapped child, such as in cerebral palsy. 23 Traumatic causes should be considered and carefully excluded in the evaluation. If unrecognized, they may have serious neurologic consequences. Torticollis most commonly follows an injury to the CI-C2 articulation. Because fractures and dislocations of the odontoid may not be apparent on initial radiographic views, a high degree of suspicion with careful follow-up is required. Children with bone dysplasias, Morquio's syndrome, spondyloepiphyseal dysplasia, and Down's syndrome have a high incidence of CI-C2 instability with accompanying torticollis. 13. 18 Neurologic disorders, particularly space-occupying lesions of the central nervous system (for example, posterior fossa tumors, tumors of the spinal column, cordoma, and syringomyelia), are often accompanied by torticollis. Uncommon neurologic causes include dystonia musculorum deformans and problems of hearing and vision that can result in head tilt. Though uncommon, hysterical and psychogenic causes exist but should be diagnosed only after carefully excluding other causes. CONGENITAL ANOMALIES OF THE OCCIPITOCERVICAL JUNCTION Occipitalization This condition is characterized by partial or complete fusion of the bony ring of the atlas to the base of the occiput. Clinically, the patient has torticollis, short neck, a low hairline, and restricted neck motions, not unlike the Klippel-Feil syndrome. Fusion of the occipitocervical joint may lead to increased strain on the CI-C2 articulation and subsequent instability, particularly for those who in addition have a C2-C3 fusion 13 (Fig. 4). The odontoid may gradually encroach anteriorly into the spinal cord or medulla,
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Figure 4. A, Os odontoideum in a seven-year-old boy. B, With forward flexion, the ring of Cl and os odontoideum move forward, demonstrating marked instability of the CI-C2 articulation.
or the posterior ring of Cl may be pulled forward into the spinal cord. If the condition is found, roentgenograms, particularly in flexion-extension, and/or laminagraphy should demonstrate the abnormal motion of the odontoid and ring of CIY Congenital Anomalies of the Odontoid There are several variations recognized: (1) agenesis, the complete absence of the dens; (2) hypoplasia, a short, stubby projection seldom extending beyond the inferior facets; and (3) os odontoideum (Fig. 4A and B), where the body of the odontoid is represented by a free bony ossicle separated from the axis by a wide gap, suggesting a nonunion. 13 In the infant, the odontoid is normally formed and can be seen on routine roentgenograms, but is separated from the body of the axis by a broad cartilaginous band. This corresponds to a rudimentary intervertebral disc and is present in all children under 2 years of age; complete fusion can be expected after age 5 years. Regardless of the type of odontoid anomalies, they all share the same signs and symptoms, and treatment is identical. Minor trauma is commonly associated with the onset of symptoms. Symptoms may be due to local irritation of the atlantoaxial articulation or neurologic, due to CI-C2 instability and spinal cord compression. A significant proportion present with an insidious onset and slowly progressive neurologic impairment of both posterior and anterior spinal structures. In children, the presenting symptoms may be quite subtle and nonspecific, such as generalized weakness, frequent falling, or asking to be carried. 18 Certain of the bone dysplasias that lead to dwarfism, such as the mucopolysaccharidoses and spondyloepiphyseal dysplasia, have a high incidence of odontoid anomalies and should be searched for on a routine basis. 18 If CI-C2 instability is suspected, it should be confirmed with lateral roentgenograms in flexion-extension. Lateral laminagrams and/or cineradiography may be required. 13 If neurologic problems can be documented
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Figure 5. Pseudosubluxation ofC2 on C3. Note the straight-line relationship of the posterior elements in flexion, known as the posterior cervical line, a helpful guide in determining pathologic from physiologic displacement.
or if there is excessive mobility of the CI-C2 articulation (Fig. 4B), then surgical fusion of the CI-C2 articulation should be considered. 1 Congenital Laxity of the Transverse Atlantal Ligament This may occur from acute trauma or repetitive trauma, such as was described in occipitalization of Cl, or the attachment may be weakened due to inflammation from infection or rheumatoid arthritis. Laxity of the transverse atlantal ligament is common in Down's syndrome, with a reported incidence of 20 per cent. 13 The laxity may be due to either rupture or attenuation of the transverse atlantal ligament and may lead to severe instability of the CI-C2 articulation. Lateral flexion-extension radiographs should be routine in children with Down's syndrome who have neurologic problems 13 or those who will be participating in sports that may cause trauma to the head and neck. Pseudosubluxation of C2 on C3 Interpretation of roentgenographs of the immature cervical spine poses several problems-unique vertebral configuration, incomplete ossification, the presence of epiphysis, singly or in combination, and hypenpobility.6, 31 Children can be expected to have more flexibility in the cervical spine than the adult. A commonly recognized finding is pseudosubluxation of C2 on C3 (Fig. 5), the anterior displacement of the second cervical vertebra on the third in flexion, This is due to the normal laxity of the intervertebral ligaments, and nearly half of the children under 8 years of age may have 3 mm or more of anteroposterior movement. 6 However, specific measurements of the normfll flexion-extension movement of the bodies of C2 on C3 have proved unreliable. The straight-line relationship of the posterior elements in flexion (the posterior cervical line) is helpful in differentiating physiologic from pathologic anterior displacement of C2 on C3 (see Fig. 5).32 Another common finding is overriding of the atlas on the odontoid with extension, which Cattell found in 20 per cent of normal children. 6
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KLIPPEL-FElL SYNDROME The term Klippel-Feil syndrome includes all persons with congenital fusion of the cervical vertebrae, whether it be two segments (congenital block vertebrae) or the entire cervical spine. Congenital cervical fusion is the result of failure of the normal segmentation of the cervical somite. The etiology is as yet undetermined. It is important to remember that the embryologic abnormality is not limited to the cervical spine. Patients with Klippel-Feil syndrome, even those with minor cervical lesions, may be at risk of having other less apparent but serious defects in the genitourinary, nervous, and cardiopulmonary systems and hearing disorders. 12.20.22 Many of these "hidden" abnormalities may be far more detrimental to the patient's general well-being than the obvious deformity of the neck. 21. 22
Clinical Appearance The classic clinical description of the syndrome is the triad of low posterior hairline, short neck, and limitation of head and neck motion. Many of the mildly involved patients do not have the classic triad. 12 Conversely, patients with extensive cervical involvement often are able to maintain a deceptively good range of motion (Fig. 6A-D). Sprengel's deformity is a frequent finding (25 to 35 per cent). 12 Approximately 20 per cent of patients with the Klippel-Feil syndrome have facial asymmetry, torticollis, or webbing of the neck. 12
Radiologic Features Fixed bony deformities frequently prevent proper positioning, and overlapping shadows from the mandible, occiput, or foramen magnum may obscure the upper vertebrae. Lateral flexion-extension and laminographic views are quite helpful in evaluating the deformity. If vertebral instability is suspected, the study should be augmented by cineradiography. In the young child, narrowing of the cervical disc space cannot always be found because the ossification of the vertebral body is incomplete and the unossified end-plates may give the false impression of a normal disc space. However, with continued growth the ossification of the vertebral bodies is completed, and the fusion becomes obvious. 12 Juvenile rheumatoid arthritis, rheumatoid spondylitis, and infection can lead to similar radiologic findings, but usually the history and physical examination will indicate the correct diagnosis. .
Associated Conditions Scoliosis and/or kyphosis is the most frequent anomaly (60 per cent) found in association with this syndrome. 12 Most of these patients require treatment and should be followed closely through the growth years with roentgenographic examination of the entire spine, both anteroposterior and lateral views. A high incidence of renal abnormalities has been reported in patients with congenital scoliosis. 1z . 20 Similarly, in the Klippel-Feil syndrome, one third of the children can be expected to have a significant urinary tract anomaly.12 Initially, these conditions are asymptomatic, and renal ultrasonic evaluation should be a routine procedure for children with
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Figure 6. A and C, Clinical appearance of a patient with the Klippel-Feil syndrome with apparently normal range of cervical flexion-extension. Band D, Flexion-extension roentgenograms of the same patient. Note the marked degree of cervical fusion, with the majority of flexion-extension occurring at one open disc space.
spine anomalies. The most common abnormality is unilateral absence of the kidney and/or hydronephrosis from ureteropelvic obstruction. 12 Hearing loss is common (30 per cent),12 and may include absence of the auditory canal and microtia. Less frequently, the Klippel-Feil syndrome may be associated with congenital heart disease (4.2 to 14 per cent). 12 Approximately 20 per cent demonstrate synkinesia or mirror motions, consisting of involuntary paired movements of the hands and occasionally the arms. The patient is unable to move one hand without similar reciprocal motion of the opposite hand. 12
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Treatment Except for anomalies involving the atlantoaxial joint, there are no symptoms that can be directly attributed to the lower fused cervical vertebrae. All symptoms commonly associated with Klippel-Feil syndrome originate at the open segments adjacent to the area of synostosis. These remaining free articulations may become hypermobile due to increased stress or in response to trauma (Fig. 6). This hypermobility can lead to frank instability and/or early degenerative arthritis. Very few children are symptomatic, and most patients who develop symptoms are in the second or third decade of life. The minimally involved patient with the KlippelFeil syndrome can be expected to lead a normal, active life with only minor restrictions or symptoms. Many of the severely involved children can enjoy the same favorable prognosis if early and appropriate treatment is instituted. 12 This is particularly applicable in the area of associated scoliosis and renal abnormalities. Prevention of further deformity or complications can be of great benefit to the patient both in longevity and quality of life.
SHOULDER PROBLEMS Brachial Plexus Injury in the Newborn Brachial plexus injury is due to traction on the brachial plexus during delivery. The usual mechanism is distraction of the upper extremity away from the head and neck and stretching of the nerves as they exit from the cervical spine. The delivery is usually difficult, and the infants are large with cephalopelvic disproportion. The infant holds the arm loosely at the side of the thorax, and the entire upper extremity is internally rotated at the shoulder with extension of the elbow, pronation of the forearm, and flexion at the wrist Cheadwaiter's tip" position) (Fig. 7). There is swelling in the region of the shoulder and supraclavicular fossa. Initially, there will be a full passive range of joint motion and no active motion in the denervated areas. Pain and tenderness are common, as sensation is usually intact except in total plexus involvement. The differential diagnosis includes a fracture of the clavicle or proximal humerus and infection in the region of the shoulder. Roentgenograms of the upper limb, clavicle, and cervical spine should be routine. In the severely involved child, a chest roentgenogram should be obtained to rule out a concomitant injury to the phrenic nerve and unilateral paralysis of the diaphragm. The appearance of the upper extremity is similar to infants with arthrogryposis, but passive motion of the joints will be limited. The injury is divided into three types: (1) Erb's palsy, the C5-C6lesion (Erb's point) with denervation of the deltoid, supraspinatus, biceps, and brachioradialis; (2) Klumpke C8-Tl lesion, denervation of the intrinsics of the hand, flexors of the wrist, and finger sympathetics (Horner's syndrome) and diaphragmatic paralysis fi'om phrenic nerve injury; and (3) ErbDuchenne-Klumpke paralysis, or combined lesion involving the whole arm. In the newborn it is unnecessary to localize the anatomic lesion precisely. Rather, one should make a careful joint-by-joint examination, recording the
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Figure 7. Brachial plexus injury in the newborn. Three-month-old infant exhibiting the typical posture of Erb's palsy (headwaiter's tip position).
remaining active musculature and range of motion. This will proVide an excellent record for later assessment of the patient's progress. Treatment The primary goal is to avoid contractures of the involved joints by preserving a full range of passive motion. The importance of maintaining normal joint motion must be emphasized to the parents. 8 If there is return of neurologic function, the reinnervated musculature will require supple joints; and if there is no return, prevention of contractures will allow a greater latitude in choice of reconstructive procedures. Indiscriminate use of rigid bracing is to be discouraged, as harmful contractures may develop. Similarly, pinning the arm to the bed can lead to additional injury by the infant's tugging and pulling on the relatively insensitive extremity. Judicious use of bracing in concert with physical therapy is helpful, such as splinting the fingers in extension or splinting the wrist to avoid a volar contracture. Most neurologic recovery is within the first 6 months but may extend
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Figure 8. A and B, Sprengel's deformity in a six-year-old child.
to 18 months. 1 Less than 20 per cent obtain full neurologic return. l Reconstructive procedures are considered when the child is older and can cooperate with preoperative evaluation of remaining muscle function. The development of newer microsurgical techniques has stimulated interest in direct surgical repair of the lesion, but results are preliminary. 28 Sprengel's Deformity (Congenital Elevation of the Scapula) The scapula is elevated and hypoplastic. The affected side of the neck is fuller and shorter, with a decrease in the cervicoscapular line and the appearance of torticollis (Fig. 8). Abduction of the affected shoulder is limited due to a decrease in the scapulocostal motion. Scapulohumeral joint motion is usually within normal limits. There is no right or left preponderance and one third are bilateral. Associated congenital scoliosis is common and a third of those affected will have renal anomalies. l2 Approximately one third have an omovertebral bone, an osseous and cartilaginous structure originating in the upper part of the scapula and attaching to the spinous process of the cervical vertebrae. This abnormal bar, occasionally in combination with a contracture of levator scapulae muscles, may further limit scapular motion. Approximately one third of the children have sufficient deformity to warrant surgical intervention. 4 Treatment of Sprengel's deformity in properly selected patients can provide considerable cosmetic benefit.4 It can restore a more natural contour of the shoulders and neck as well as apparent increase in neck length. If an omovertebral bone is present,. its removal may permit an increase in neck and shoulder motion. 4 Surgery is usually
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recommended between 3 and 5 years of age. In the older child there is an increased risk of injury to the brachial plexus from stretching or compression from the clavicle. Cleidocranial Dysostosis This is a hereditary condition that results in incomplete formation of the clavicles and skull but also may involve other skeletal structures. There may be complete absence of the clavicle or loss of a small segment in the mid or outer portion. 9 The defect is usually bilateral (82 per cent).21 There is delayed closure of the cranial sutures and fontanels and incomplete development of the pubis. Severely affected children may have scoliosis and anomalies of the mandible, teeth, and small bones of the hands and feet. The defect in the pubis may be quite alarming and has been mistaken for erosion from a tumor. Clinically, the typical patient has a large head, small face, long neck, drooping shoulders, narrow chest, and short stature. For most it is not a disabling condition, and many have a long and productive life. 21 Congenital Pseudarthrosis of the Clavicle This is a rare lesion, usually within the middle third of the clavicle and present at birth, and spontaneous healing does not occur. The deformity may become larger and more conspicuous as the child grows, with a false joint developing between the enlarged ends of the clavicle. 24 The shoulder on the affected side tends to droop lower and forward, nearer the midline than the normal side. There is a variable degree of painless motion between the ends of the clavicle but seldom any functional problems. 24, 33 The condition may be confused with a simple fracture, cleidocranial dysostosis, or neurofibromatosis. Recently, Lloyd-Roberts and coworkers have noted that the condition is predominantly right sided, and they suggest that the lesion may be due to pressure on the developing clavicle by the subclavian artery, which is normally at a higher level on the right side. 17 Surgery is recommended only for those who have an unsightly lump, pain, or shoulder weakness. Congenital Absence of the Pectoralis Musculature This is often incorrectly referred to as Poland's syndrome, which includes syndactyly and hypoplasia of the ipsilateral upper extremity. The deformity at times can be minimal and the patient and family completely unaware of the absent muscle. A well-developed, full, curving anterior axillary fold is absent. The affected side of the chest is flat and the nipple is high. Male children are more frequently affected than are female children. In addition, there may be associated hypoplastic or absent breast and nipple. Less frequently reported are hypoplasia of a rib, herniation of the lung, elevated scapula, and scoliosis. 15 All have absence of the costal head of the pectoralis major and, in most, the pectoralis minor is also absent. Only 13 per cent of patients have the true Poland's syndrome. Few have any functional impairment. 15 Occasionally, a fibrous remnant of the pectoralis muscle may require resection if it limits abduction or extension.
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REFERENCES 1. Adler, J. B., and Patterson, R L.: Erb's palsy: Long-term results of treatment in 88 cases. J. Bone Joint Surg., 49A:I052-1064, 1967. 2. Brooks, B.: Pathologic changes in muscle as a result of disturbances of circulation. Arch. Surg., 5:188-216, 1922. 3. Calabro, J. J., Katz, R. M., and Maltz, B. A.: A critical reappraisal of juvenile rheumatoid arthritis. Clin. Orthop., 74:101-119, 1971. 4. Carson, W. G., Lovell, W. W., and Whitesides, T. E., Jr.: Congenital elevation of the scapula. Surgical correction by the Woodward procedure. J. Bone Joint Surg., 63A:11991207, 1982. 5. Canale, S. T., Griffin, D. W., and Hubbard, C. N.: Congenital muscular torticollis. A long-term follow-up. J. Bone Joint Surg., 64A:81O-816, 1982. 6. Cattell, H. S., and Filtzer, D. L.: Pseudo subluxation and other normal variations in the cervical spine in children. J. Bone Joint Surg., 47A:1295-1309, 1965. 7. Coventry, M. B., and Harris, L. E.: Congenital muscular torticollis in infancy; some observations regarding treatment. J. Bone Joint Surg., 41A:815-822, 1959. 8. Eng., G. D., Koch, B., and Smokvina, M.: Brachial plexus palsy in neonates and children. Arch. Phys. Med. Rehab., 59:458-464, 1978. 9. Fairbanks, T. A. H.: Cranio-cleido-dysostosis. J. Bone Joint Surg., 31B:608-617, 1949. 10. Fielding, J. W., and Hawkins, R. J.: Fixed rotatory subluxation of the atlanto-axial joint. J. Bone Joint Surg., 59A:37-44, 1977. 11. Gibson, E. A., and Carroll, N.: Congenital pseudarthrosis of the clavicle. J. Bone Joint Surg., 52B:629-643, 1970. 12. Hensinger, R. N., Lange, J. R, and MacEwen, G. D.: The Klippel-Feil syndrome: A constellation of related anomalies. J. Bone Joint Surg., 56A:1246-1253, 1974. 13. Hensinger, R. N., and MacEwen, G. D.: Congenital anomalies of the spine. In Rothman, R. N., and Simeone, F. A. (eds.): The Spine. Vol. 2. Philadelphia, W. B. Saunders Company, 1982, pp. 188-316. 14. Hummer, C. D., Jr., and MacEwen, G. D.: The coexistence of torticollis and congenital dysplasia of the hip. J. Bone Joint Surg., 54A:1255-1256, 1972. 15. Ireland, D. C. R., Takayama, N., and Flatt, A. E.: Poland's syndrome. A review of 43 cases. J. Bone Joint Surg., 58A:52-58, 1976. 16. Ling, C. M., and Low, Y. S.: Sternomastoid tumor and muscular torticollis. Clin. Orthop., 86:144-150, 1972. 17. Lloyd-Roberts, G. D., Apley, A. G., and Owen, R: Reflections upon the aetiology of congenital pseudarthrosis of the clavicle with a note on cranio-cleido-dysostosis. J. Bone Joint Surg., 57B:24-29, 1975. 18. Kopits, S. E., Perovic, M. N., McKusick, J. A., et al.: Congenital atlanto axial dislocations in various forms of dwarfism. J. Bone Joint Surg., 54A:1349-1350, 1972. 19. MacDoneld, c.: Sternomastoid tumor and muscular torticollis. J. Bone Joint Surg., 51B:432-443, 1969. 20. MacEwen, G. D., Winter, R B., and Hardy, J. H.: Evaluation of kidney anomalies in congenital scoliosis. J. Bone Joint Surg., 54A:1451-1454, 1972. 21. Outland, T., and Sherk, H.: Cleidocranial dysostosis: The hereditary aspects. Am. J. Dis. Child., 123:218-221, 1972. 22. Quam, L., and Smith, D. W.: The VATER association-vertebral defects, anal atresia, IE fistula, esophageal atresia, radial and renal dysplasia-a spectrum of associated defects. J. Pediatr., 82:104-107,1973. 23. Ramenofsky, M. L., Buyse, M., Goldberg, M. J., et al.: Gastroesophageal reflux and torticollis. J. Bone Joint Surg., 60A:1140-1141, 1978. 24. Quinlan, W. R., Brady, P. G., and Regan, B. F.: Congenital pseudarthrosis of the clavicle. Acta Orthop. Scand., 51 :489-492, 1980. 25. Sanner, G., and Bergstrom, B.: Benign paroxysmal torticollis in infancy. Acta Paediatr. Scand., 68:219-223, 1979. 26. Sarant, J. B., and Morrissy, R T.: Idiopathic torticollis: Sternocleidomastoid myopathy and accessory neuropathy. Muscle Nerve, 4:374, 380, 1981. 27. Scheckter, L. S., Smith, A., and Pearl, M.: Intervertebral disc calcification in childhood. Am. J. Dis. Child., 123:608-611, 1972.
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28. Solonen, K. A., Telaranta, T., and Ryoppy, S.: Early reconstructions of birth injuries of the brachial plexus. J. Pediatr. Orthop., 1:367-370, 1981. 29. Sonnabend, D. H., Taylor, T. K. F., and Chapman, C. K.: Intervertebral disc calcification syndromes in children. J. Bone Joint Surg., 64B:25-31, 1982. 30. Staheli, L. T.: Muscular torticollis: Late results of operative treatment. Surgery, 69:469473, 1971. 31. Sullivan, R. C., Bruwer, A. J., and Harris, L.: Hypermobility of the cervical spine in children: A pitfall in the diagnosis of cervical dislocation. Am. J. Surg., 95:636-640, 1958. 32. Swischuck, L. E.: Anterior displacement of C2 in children: Physiologic or pathologic? A helpful differentiating line. Radiology, 122:759-763, 1977. 33. Wall, J. J.: Congenital pseudarthrosis of the clavicle. J. Bone Joint Surg., 52A:1003-1009, 1970. C4554 Orthopedics University of Michigan 1405 East Ann Ann Arbor, Michigan 48109
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Orthopedic Problems of the Shoulder and Neck
Robert N. Hensinger, M.D. *
If one excludes trauma, problems of the neck and shoulder in children are relatively uncommon. Still, the pediatrician or family practitioner should have sufficient familiarity to provide the family with appropriate counseling and referral. This article emphasizes the common problems but also notes less common conditions which, if unrecognized, may have serious impact on the health and well-being of the child. TORTICOLLIS Torticollis, or wryneck, is a common clinical sign found in a wide variety of childhood illnesses. When torticollis is recognized at or near the time of birth, the usual cause is congenital muscular torticollis. However, one must exclude other less common congenital conditions such as a fixed or bony torticollis due to the Klippel-Feil syndrome; anomalies of the atlantoaxial articulation; soft tissue problems, abnormal skin webs or folds (pterygium colli); and tumors in the region of the sternocleidomastoid (cystic hygroma, branchial cleft cysts, and thyroid teratomas). Congenital Muscular Torticollis (Congenital Wryneck) Congenital muscular torticollis usually is discovered in the first 6 to 8 weeks of life (Fig. 1A). If the infant is examined within the first month of life, a mass or "tumor" is usually palpable in the neck. 16 It is generally a nontender, soft enlargement that is mobile beneath the skin and attached to, or located within, the body of the sternocleidomastoid muscle (Fig. 1B). The mass attains maximal size within the first month and then gradually regresses. If the child is examined at 4 to 6 months of age, the tumor is usually absent and the contracture of the sternocleidomastoid muscle and torticollis posture are the only clinical findings. Recent pathologic studies *Professor, Department of Surgery, Section of Orthopedic Surgery, University of Michigan Medical Center, Ann Arbor, Michigan
Pediatric Clinics of North America-Vol. 33, No.6, December 1986
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Figure 1. A, One-year-old child with congenital muscular torticollis due to contracture of the sternocleidomastoid. The head is tilted toward the involved side and the chain rotated toward the contralateral shoulder. B, One-month-old infant with congenital muscular torticollis with the typical mass in the neck.
suggest that, with time, the fibrosis of the sternal head of the muscle may entrap and compromise the branch of the accessory nerve to the clavicular head, further increasing the deformity. 26 If the condition is progressive, deformities of the face and skull can result and are usually apparent within the first year. Flattening of the face on the side of the contracted sternocleidomastoid muscle may be impressive, and is due to the position of the head while the child sleeps. If the child sleeps prone, it is more comfortable to have the affected side down. As a consequence, the face remodels to conform to the bed. In children who sleep supine, reverse modeling of the contralateral aspect of the skull (plagiocephaly) will be evident. If the condition remains untreated during the growth years, the level of the eyes and ears are distorted and may result in considerable cosmetic deformity. 30 Congenital muscular torticollis is not accompanied by bone abnormalities or neurologic deficit and is believed to be caused by ischemia of the sternocleidomastoid muscle, particularly the sternal head, because of intrauterine positioning or increased pressure during passage through the birth canal. 16, 19 A common misconception is that the neck is contused during delivery and the resultant hematoma leads to fibrosis and contracture. However, experimental work suggests that the fibrosis within the muscle is due to venous occlusion from pressure on the neck in the birth canal. 2 The clinical deformity is probably related to the ratio of the extent of fibrosis to the amount of remaining functional muscle after the initial insult. 7 If sufficient normal muscle is present, it will stretch with growth and the child will not develop a torticollis posture, whereas the fibrotic area has little elastic potential. There are two additional factors that as yet are unexplained. In three of four children, the lesion is on the right side, 16, 19 and 20 per cent of the children have congenital dysplasia of the hip. 14 Excellent results can be obtained in the majority of patients with
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Figure 2. Five-year-old child with untreated torticollis. Thick fibrotic tendon-like bands have replaced the sternocleidomastoid as if the head is tethered to the clavicle. Note the heads of the sternocleidomastoid, one inserting on the clavicle and the other on the sternum.
stretching exercises. 16. 19 Standard maneuvers include positioning of the ear that is opposite the contracted muscle to the shoulder and the chin to the shoulder on the affected side. When adequate stretching has been obtained in the neutral position, these maneuvers should be repeated with the head hyperextended. Other treatment measures include positioning of crib toys so that the neck will be stretched when trying to reach and grasp. If conservative measures are unsuccessful by 18 to 24 months of age, surgical resection is required, usually of a portion of the tendon at its distal attachment? (Fig. 2). The surgery should be performed prior to school age; however, a good (but not perfect) cosmetic result can be obtained as late as 12 years of age. 7.30 Asymmetry of the skull and face will correct as long as adequate growth potential remains after the contracture of the sternocleidomastoid is released. 13 The results of surgery have been uniformly good, with a low incidence of complications or recurrence, and nearly all patients are pleased with the end result. 5. 7. 16. 19. 30
Atlantoaxial Rotatory Subluxation The torticollis posture will occur if the atlas locks in rotation on the axis. In children, this occasionally develops following an upper respiratory infection or surgery in the upper pharynx, such as tonsillectomy, and has been referred to as spontaneous hyperemic subluxation of the atlas. It is assumed that the inflammation of the posterior pharynx leads to softening and laxity of the capsular structures about the joints, permitting excessive rotation of Cion C2, and locking of the facets. This is seldom accompanied
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Figure 3. Intervertebral disc calcification of childhood. Sevenyear-old girl with spontaneous onset of torticollis. Cervical roentgenogram demonstrates calcification between C3 and C4.
by neurologic symptoms or signs, and pain is mild unless one tries forcibly to correct the deformity. Roentgenographic documentation is difficult, owing to the unusual position of the head and neck, and open-mouth views, computerized tomography, and cineradiography may be necessary.lO Simple conservative measures to relieve symptoms, such as a soft cervical collar, are usually sufficient. Those who fail to respond within a few days will require bed rest and light cervical traction. If the condition is allowed to persist, the child may develop a fixed deformity requiring surgical correction. lO Intervertebral Disc Calcification in Childhood Spontaneous calcification of a disc is an unusual phenomenon of unknown etiology and has been found in all vertebral levels. The cervical spine is the most common region reported, as 90 per cent of these are symptomatic. 29 Clinical symptoms are usually muscle spasm, torticollis, localized pain, and nonspecific low-grade fever. Symptoms are acute in onset and may be related to trauma (30 per cent) or upper respiratory infection (15 per cent). The symptoms may last only a few days or persist for 1 to 2 months. Laboratory studies are usually within normal limits. The calcification typically disappears within 2 to 3 months, but may be permanent. On the roentgenogram, there is a fluffy area of calcification within the nucleus pulposis but not involving the annulus (Fig. 3). Herniation of the disc is an infrequent complication. When symptomatic, bed rest, mild analgesics, and cervical collar appear to be adequate therapy, usually
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resulting in rapid disappearance of symptoms, two thirds in 3 weeks and 95 per cent within 6 months. 30 Inflammatory Conditions Local irritation from cervical lymphadenitis may lead to the appearance of a wryneck or tilt of the head. Another less frequent cause is a retropharyngeal abscess following inflammation of the posterior pharynx or tonsillitis. Children with polyarticular juvenile rheumatoid arthritis frequently develop involvement of the cervical joints. 3 Torticollis and limitation of cervical motion may be the only clinical signs. The child should be followed closely, as the condition may result in spontaneous fusion of the entire cervial spine. 13 Other Causes of Torticollis Intermittent torticollis can occur in the young child. A seizure-like disorder called benign paroxysmal torticollis of infancy is due to many neurologic causes, including drug intoxication. 25 Similarly, Sandifer's syndrome, involving gastroesophageal reflux with sudden posturing of the trunk and torticollis, is being recognized more often, particularly in the neurologically handicapped child, such as in cerebral palsy. 23 Traumatic causes should be considered and carefully excluded in the evaluation. If unrecognized, they may have serious neurologic consequences. Torticollis most commonly follows an injury to the CI-C2 articulation. Because fractures and dislocations of the odontoid may not be apparent on initial radiographic views, a high degree of suspicion with careful follow-up is required. Children with bone dysplasias, Morquio's syndrome, spondyloepiphyseal dysplasia, and Down's syndrome have a high incidence of CI-C2 instability with accompanying torticollis. 13. 18 Neurologic disorders, particularly space-occupying lesions of the central nervous system (for example, posterior fossa tumors, tumors of the spinal column, cordoma, and syringomyelia), are often accompanied by torticollis. Uncommon neurologic causes include dystonia musculorum deformans and problems of hearing and vision that can result in head tilt. Though uncommon, hysterical and psychogenic causes exist but should be diagnosed only after carefully excluding other causes. CONGENITAL ANOMALIES OF THE OCCIPITOCERVICAL JUNCTION Occipitalization This condition is characterized by partial or complete fusion of the bony ring of the atlas to the base of the occiput. Clinically, the patient has torticollis, short neck, a low hairline, and restricted neck motions, not unlike the Klippel-Feil syndrome. Fusion of the occipitocervical joint may lead to increased strain on the CI-C2 articulation and subsequent instability, particularly for those who in addition have a C2-C3 fusion 13 (Fig. 4). The odontoid may gradually encroach anteriorly into the spinal cord or medulla,
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Figure 4. A, Os odontoideum in a seven-year-old boy. B, With forward flexion, the ring of Cl and os odontoideum move forward, demonstrating marked instability of the CI-C2 articulation.
or the posterior ring of Cl may be pulled forward into the spinal cord. If the condition is found, roentgenograms, particularly in flexion-extension, and/or laminagraphy should demonstrate the abnormal motion of the odontoid and ring of CIY Congenital Anomalies of the Odontoid There are several variations recognized: (1) agenesis, the complete absence of the dens; (2) hypoplasia, a short, stubby projection seldom extending beyond the inferior facets; and (3) os odontoideum (Fig. 4A and B), where the body of the odontoid is represented by a free bony ossicle separated from the axis by a wide gap, suggesting a nonunion. 13 In the infant, the odontoid is normally formed and can be seen on routine roentgenograms, but is separated from the body of the axis by a broad cartilaginous band. This corresponds to a rudimentary intervertebral disc and is present in all children under 2 years of age; complete fusion can be expected after age 5 years. Regardless of the type of odontoid anomalies, they all share the same signs and symptoms, and treatment is identical. Minor trauma is commonly associated with the onset of symptoms. Symptoms may be due to local irritation of the atlantoaxial articulation or neurologic, due to CI-C2 instability and spinal cord compression. A significant proportion present with an insidious onset and slowly progressive neurologic impairment of both posterior and anterior spinal structures. In children, the presenting symptoms may be quite subtle and nonspecific, such as generalized weakness, frequent falling, or asking to be carried. 18 Certain of the bone dysplasias that lead to dwarfism, such as the mucopolysaccharidoses and spondyloepiphyseal dysplasia, have a high incidence of odontoid anomalies and should be searched for on a routine basis. 18 If CI-C2 instability is suspected, it should be confirmed with lateral roentgenograms in flexion-extension. Lateral laminagrams and/or cineradiography may be required. 13 If neurologic problems can be documented
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Figure 5. Pseudosubluxation ofC2 on C3. Note the straight-line relationship of the posterior elements in flexion, known as the posterior cervical line, a helpful guide in determining pathologic from physiologic displacement.
or if there is excessive mobility of the CI-C2 articulation (Fig. 4B), then surgical fusion of the CI-C2 articulation should be considered. 1 Congenital Laxity of the Transverse Atlantal Ligament This may occur from acute trauma or repetitive trauma, such as was described in occipitalization of Cl, or the attachment may be weakened due to inflammation from infection or rheumatoid arthritis. Laxity of the transverse atlantal ligament is common in Down's syndrome, with a reported incidence of 20 per cent. 13 The laxity may be due to either rupture or attenuation of the transverse atlantal ligament and may lead to severe instability of the CI-C2 articulation. Lateral flexion-extension radiographs should be routine in children with Down's syndrome who have neurologic problems 13 or those who will be participating in sports that may cause trauma to the head and neck. Pseudosubluxation of C2 on C3 Interpretation of roentgenographs of the immature cervical spine poses several problems-unique vertebral configuration, incomplete ossification, the presence of epiphysis, singly or in combination, and hypenpobility.6, 31 Children can be expected to have more flexibility in the cervical spine than the adult. A commonly recognized finding is pseudosubluxation of C2 on C3 (Fig. 5), the anterior displacement of the second cervical vertebra on the third in flexion, This is due to the normal laxity of the intervertebral ligaments, and nearly half of the children under 8 years of age may have 3 mm or more of anteroposterior movement. 6 However, specific measurements of the normfll flexion-extension movement of the bodies of C2 on C3 have proved unreliable. The straight-line relationship of the posterior elements in flexion (the posterior cervical line) is helpful in differentiating physiologic from pathologic anterior displacement of C2 on C3 (see Fig. 5).32 Another common finding is overriding of the atlas on the odontoid with extension, which Cattell found in 20 per cent of normal children. 6
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KLIPPEL-FElL SYNDROME The term Klippel-Feil syndrome includes all persons with congenital fusion of the cervical vertebrae, whether it be two segments (congenital block vertebrae) or the entire cervical spine. Congenital cervical fusion is the result of failure of the normal segmentation of the cervical somite. The etiology is as yet undetermined. It is important to remember that the embryologic abnormality is not limited to the cervical spine. Patients with Klippel-Feil syndrome, even those with minor cervical lesions, may be at risk of having other less apparent but serious defects in the genitourinary, nervous, and cardiopulmonary systems and hearing disorders. 12.20.22 Many of these "hidden" abnormalities may be far more detrimental to the patient's general well-being than the obvious deformity of the neck. 21. 22
Clinical Appearance The classic clinical description of the syndrome is the triad of low posterior hairline, short neck, and limitation of head and neck motion. Many of the mildly involved patients do not have the classic triad. 12 Conversely, patients with extensive cervical involvement often are able to maintain a deceptively good range of motion (Fig. 6A-D). Sprengel's deformity is a frequent finding (25 to 35 per cent). 12 Approximately 20 per cent of patients with the Klippel-Feil syndrome have facial asymmetry, torticollis, or webbing of the neck. 12
Radiologic Features Fixed bony deformities frequently prevent proper positioning, and overlapping shadows from the mandible, occiput, or foramen magnum may obscure the upper vertebrae. Lateral flexion-extension and laminographic views are quite helpful in evaluating the deformity. If vertebral instability is suspected, the study should be augmented by cineradiography. In the young child, narrowing of the cervical disc space cannot always be found because the ossification of the vertebral body is incomplete and the unossified end-plates may give the false impression of a normal disc space. However, with continued growth the ossification of the vertebral bodies is completed, and the fusion becomes obvious. 12 Juvenile rheumatoid arthritis, rheumatoid spondylitis, and infection can lead to similar radiologic findings, but usually the history and physical examination will indicate the correct diagnosis. .
Associated Conditions Scoliosis and/or kyphosis is the most frequent anomaly (60 per cent) found in association with this syndrome. 12 Most of these patients require treatment and should be followed closely through the growth years with roentgenographic examination of the entire spine, both anteroposterior and lateral views. A high incidence of renal abnormalities has been reported in patients with congenital scoliosis. 1z . 20 Similarly, in the Klippel-Feil syndrome, one third of the children can be expected to have a significant urinary tract anomaly.12 Initially, these conditions are asymptomatic, and renal ultrasonic evaluation should be a routine procedure for children with
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Figure 6. A and C, Clinical appearance of a patient with the Klippel-Feil syndrome with apparently normal range of cervical flexion-extension. Band D, Flexion-extension roentgenograms of the same patient. Note the marked degree of cervical fusion, with the majority of flexion-extension occurring at one open disc space.
spine anomalies. The most common abnormality is unilateral absence of the kidney and/or hydronephrosis from ureteropelvic obstruction. 12 Hearing loss is common (30 per cent),12 and may include absence of the auditory canal and microtia. Less frequently, the Klippel-Feil syndrome may be associated with congenital heart disease (4.2 to 14 per cent). 12 Approximately 20 per cent demonstrate synkinesia or mirror motions, consisting of involuntary paired movements of the hands and occasionally the arms. The patient is unable to move one hand without similar reciprocal motion of the opposite hand. 12
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Treatment Except for anomalies involving the atlantoaxial joint, there are no symptoms that can be directly attributed to the lower fused cervical vertebrae. All symptoms commonly associated with Klippel-Feil syndrome originate at the open segments adjacent to the area of synostosis. These remaining free articulations may become hypermobile due to increased stress or in response to trauma (Fig. 6). This hypermobility can lead to frank instability and/or early degenerative arthritis. Very few children are symptomatic, and most patients who develop symptoms are in the second or third decade of life. The minimally involved patient with the KlippelFeil syndrome can be expected to lead a normal, active life with only minor restrictions or symptoms. Many of the severely involved children can enjoy the same favorable prognosis if early and appropriate treatment is instituted. 12 This is particularly applicable in the area of associated scoliosis and renal abnormalities. Prevention of further deformity or complications can be of great benefit to the patient both in longevity and quality of life.
SHOULDER PROBLEMS Brachial Plexus Injury in the Newborn Brachial plexus injury is due to traction on the brachial plexus during delivery. The usual mechanism is distraction of the upper extremity away from the head and neck and stretching of the nerves as they exit from the cervical spine. The delivery is usually difficult, and the infants are large with cephalopelvic disproportion. The infant holds the arm loosely at the side of the thorax, and the entire upper extremity is internally rotated at the shoulder with extension of the elbow, pronation of the forearm, and flexion at the wrist Cheadwaiter's tip" position) (Fig. 7). There is swelling in the region of the shoulder and supraclavicular fossa. Initially, there will be a full passive range of joint motion and no active motion in the denervated areas. Pain and tenderness are common, as sensation is usually intact except in total plexus involvement. The differential diagnosis includes a fracture of the clavicle or proximal humerus and infection in the region of the shoulder. Roentgenograms of the upper limb, clavicle, and cervical spine should be routine. In the severely involved child, a chest roentgenogram should be obtained to rule out a concomitant injury to the phrenic nerve and unilateral paralysis of the diaphragm. The appearance of the upper extremity is similar to infants with arthrogryposis, but passive motion of the joints will be limited. The injury is divided into three types: (1) Erb's palsy, the C5-C6lesion (Erb's point) with denervation of the deltoid, supraspinatus, biceps, and brachioradialis; (2) Klumpke C8-Tl lesion, denervation of the intrinsics of the hand, flexors of the wrist, and finger sympathetics (Horner's syndrome) and diaphragmatic paralysis fi'om phrenic nerve injury; and (3) ErbDuchenne-Klumpke paralysis, or combined lesion involving the whole arm. In the newborn it is unnecessary to localize the anatomic lesion precisely. Rather, one should make a careful joint-by-joint examination, recording the
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Figure 7. Brachial plexus injury in the newborn. Three-month-old infant exhibiting the typical posture of Erb's palsy (headwaiter's tip position).
remaining active musculature and range of motion. This will proVide an excellent record for later assessment of the patient's progress. Treatment The primary goal is to avoid contractures of the involved joints by preserving a full range of passive motion. The importance of maintaining normal joint motion must be emphasized to the parents. 8 If there is return of neurologic function, the reinnervated musculature will require supple joints; and if there is no return, prevention of contractures will allow a greater latitude in choice of reconstructive procedures. Indiscriminate use of rigid bracing is to be discouraged, as harmful contractures may develop. Similarly, pinning the arm to the bed can lead to additional injury by the infant's tugging and pulling on the relatively insensitive extremity. Judicious use of bracing in concert with physical therapy is helpful, such as splinting the fingers in extension or splinting the wrist to avoid a volar contracture. Most neurologic recovery is within the first 6 months but may extend
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Figure 8. A and B, Sprengel's deformity in a six-year-old child.
to 18 months. 1 Less than 20 per cent obtain full neurologic return. l Reconstructive procedures are considered when the child is older and can cooperate with preoperative evaluation of remaining muscle function. The development of newer microsurgical techniques has stimulated interest in direct surgical repair of the lesion, but results are preliminary. 28 Sprengel's Deformity (Congenital Elevation of the Scapula) The scapula is elevated and hypoplastic. The affected side of the neck is fuller and shorter, with a decrease in the cervicoscapular line and the appearance of torticollis (Fig. 8). Abduction of the affected shoulder is limited due to a decrease in the scapulocostal motion. Scapulohumeral joint motion is usually within normal limits. There is no right or left preponderance and one third are bilateral. Associated congenital scoliosis is common and a third of those affected will have renal anomalies. l2 Approximately one third have an omovertebral bone, an osseous and cartilaginous structure originating in the upper part of the scapula and attaching to the spinous process of the cervical vertebrae. This abnormal bar, occasionally in combination with a contracture of levator scapulae muscles, may further limit scapular motion. Approximately one third of the children have sufficient deformity to warrant surgical intervention. 4 Treatment of Sprengel's deformity in properly selected patients can provide considerable cosmetic benefit.4 It can restore a more natural contour of the shoulders and neck as well as apparent increase in neck length. If an omovertebral bone is present,. its removal may permit an increase in neck and shoulder motion. 4 Surgery is usually
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recommended between 3 and 5 years of age. In the older child there is an increased risk of injury to the brachial plexus from stretching or compression from the clavicle. Cleidocranial Dysostosis This is a hereditary condition that results in incomplete formation of the clavicles and skull but also may involve other skeletal structures. There may be complete absence of the clavicle or loss of a small segment in the mid or outer portion. 9 The defect is usually bilateral (82 per cent).21 There is delayed closure of the cranial sutures and fontanels and incomplete development of the pubis. Severely affected children may have scoliosis and anomalies of the mandible, teeth, and small bones of the hands and feet. The defect in the pubis may be quite alarming and has been mistaken for erosion from a tumor. Clinically, the typical patient has a large head, small face, long neck, drooping shoulders, narrow chest, and short stature. For most it is not a disabling condition, and many have a long and productive life. 21 Congenital Pseudarthrosis of the Clavicle This is a rare lesion, usually within the middle third of the clavicle and present at birth, and spontaneous healing does not occur. The deformity may become larger and more conspicuous as the child grows, with a false joint developing between the enlarged ends of the clavicle. 24 The shoulder on the affected side tends to droop lower and forward, nearer the midline than the normal side. There is a variable degree of painless motion between the ends of the clavicle but seldom any functional problems. 24, 33 The condition may be confused with a simple fracture, cleidocranial dysostosis, or neurofibromatosis. Recently, Lloyd-Roberts and coworkers have noted that the condition is predominantly right sided, and they suggest that the lesion may be due to pressure on the developing clavicle by the subclavian artery, which is normally at a higher level on the right side. 17 Surgery is recommended only for those who have an unsightly lump, pain, or shoulder weakness. Congenital Absence of the Pectoralis Musculature This is often incorrectly referred to as Poland's syndrome, which includes syndactyly and hypoplasia of the ipsilateral upper extremity. The deformity at times can be minimal and the patient and family completely unaware of the absent muscle. A well-developed, full, curving anterior axillary fold is absent. The affected side of the chest is flat and the nipple is high. Male children are more frequently affected than are female children. In addition, there may be associated hypoplastic or absent breast and nipple. Less frequently reported are hypoplasia of a rib, herniation of the lung, elevated scapula, and scoliosis. 15 All have absence of the costal head of the pectoralis major and, in most, the pectoralis minor is also absent. Only 13 per cent of patients have the true Poland's syndrome. Few have any functional impairment. 15 Occasionally, a fibrous remnant of the pectoralis muscle may require resection if it limits abduction or extension.
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REFERENCES 1. Adler, J. B., and Patterson, R L.: Erb's palsy: Long-term results of treatment in 88 cases. J. Bone Joint Surg., 49A:I052-1064, 1967. 2. Brooks, B.: Pathologic changes in muscle as a result of disturbances of circulation. Arch. Surg., 5:188-216, 1922. 3. Calabro, J. J., Katz, R. M., and Maltz, B. A.: A critical reappraisal of juvenile rheumatoid arthritis. Clin. Orthop., 74:101-119, 1971. 4. Carson, W. G., Lovell, W. W., and Whitesides, T. E., Jr.: Congenital elevation of the scapula. Surgical correction by the Woodward procedure. J. Bone Joint Surg., 63A:11991207, 1982. 5. Canale, S. T., Griffin, D. W., and Hubbard, C. N.: Congenital muscular torticollis. A long-term follow-up. J. Bone Joint Surg., 64A:81O-816, 1982. 6. Cattell, H. S., and Filtzer, D. L.: Pseudo subluxation and other normal variations in the cervical spine in children. J. Bone Joint Surg., 47A:1295-1309, 1965. 7. Coventry, M. B., and Harris, L. E.: Congenital muscular torticollis in infancy; some observations regarding treatment. J. Bone Joint Surg., 41A:815-822, 1959. 8. Eng., G. D., Koch, B., and Smokvina, M.: Brachial plexus palsy in neonates and children. Arch. Phys. Med. Rehab., 59:458-464, 1978. 9. Fairbanks, T. A. H.: Cranio-cleido-dysostosis. J. Bone Joint Surg., 31B:608-617, 1949. 10. Fielding, J. W., and Hawkins, R. J.: Fixed rotatory subluxation of the atlanto-axial joint. J. Bone Joint Surg., 59A:37-44, 1977. 11. Gibson, E. A., and Carroll, N.: Congenital pseudarthrosis of the clavicle. J. Bone Joint Surg., 52B:629-643, 1970. 12. Hensinger, R. N., Lange, J. R, and MacEwen, G. D.: The Klippel-Feil syndrome: A constellation of related anomalies. J. Bone Joint Surg., 56A:1246-1253, 1974. 13. Hensinger, R. N., and MacEwen, G. D.: Congenital anomalies of the spine. In Rothman, R. N., and Simeone, F. A. (eds.): The Spine. Vol. 2. Philadelphia, W. B. Saunders Company, 1982, pp. 188-316. 14. Hummer, C. D., Jr., and MacEwen, G. D.: The coexistence of torticollis and congenital dysplasia of the hip. J. Bone Joint Surg., 54A:1255-1256, 1972. 15. Ireland, D. C. R., Takayama, N., and Flatt, A. E.: Poland's syndrome. A review of 43 cases. J. Bone Joint Surg., 58A:52-58, 1976. 16. Ling, C. M., and Low, Y. S.: Sternomastoid tumor and muscular torticollis. Clin. Orthop., 86:144-150, 1972. 17. Lloyd-Roberts, G. D., Apley, A. G., and Owen, R: Reflections upon the aetiology of congenital pseudarthrosis of the clavicle with a note on cranio-cleido-dysostosis. J. Bone Joint Surg., 57B:24-29, 1975. 18. Kopits, S. E., Perovic, M. N., McKusick, J. A., et al.: Congenital atlanto axial dislocations in various forms of dwarfism. J. Bone Joint Surg., 54A:1349-1350, 1972. 19. MacDoneld, c.: Sternomastoid tumor and muscular torticollis. J. Bone Joint Surg., 51B:432-443, 1969. 20. MacEwen, G. D., Winter, R B., and Hardy, J. H.: Evaluation of kidney anomalies in congenital scoliosis. J. Bone Joint Surg., 54A:1451-1454, 1972. 21. Outland, T., and Sherk, H.: Cleidocranial dysostosis: The hereditary aspects. Am. J. Dis. Child., 123:218-221, 1972. 22. Quam, L., and Smith, D. W.: The VATER association-vertebral defects, anal atresia, IE fistula, esophageal atresia, radial and renal dysplasia-a spectrum of associated defects. J. Pediatr., 82:104-107,1973. 23. Ramenofsky, M. L., Buyse, M., Goldberg, M. J., et al.: Gastroesophageal reflux and torticollis. J. Bone Joint Surg., 60A:1140-1141, 1978. 24. Quinlan, W. R., Brady, P. G., and Regan, B. F.: Congenital pseudarthrosis of the clavicle. Acta Orthop. Scand., 51 :489-492, 1980. 25. Sanner, G., and Bergstrom, B.: Benign paroxysmal torticollis in infancy. Acta Paediatr. Scand., 68:219-223, 1979. 26. Sarant, J. B., and Morrissy, R T.: Idiopathic torticollis: Sternocleidomastoid myopathy and accessory neuropathy. Muscle Nerve, 4:374, 380, 1981. 27. Scheckter, L. S., Smith, A., and Pearl, M.: Intervertebral disc calcification in childhood. Am. J. Dis. Child., 123:608-611, 1972.
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28. Solonen, K. A., Telaranta, T., and Ryoppy, S.: Early reconstructions of birth injuries of the brachial plexus. J. Pediatr. Orthop., 1:367-370, 1981. 29. Sonnabend, D. H., Taylor, T. K. F., and Chapman, C. K.: Intervertebral disc calcification syndromes in children. J. Bone Joint Surg., 64B:25-31, 1982. 30. Staheli, L. T.: Muscular torticollis: Late results of operative treatment. Surgery, 69:469473, 1971. 31. Sullivan, R. C., Bruwer, A. J., and Harris, L.: Hypermobility of the cervical spine in children: A pitfall in the diagnosis of cervical dislocation. Am. J. Surg., 95:636-640, 1958. 32. Swischuck, L. E.: Anterior displacement of C2 in children: Physiologic or pathologic? A helpful differentiating line. Radiology, 122:759-763, 1977. 33. Wall, J. J.: Congenital pseudarthrosis of the clavicle. J. Bone Joint Surg., 52A:1003-1009, 1970. C4554 Orthopedics University of Michigan 1405 East Ann Ann Arbor, Michigan 48109