Segmental Reflex Sympathetic Dystrophy: Clinic; 11and Scintigraphic . Crl :eria Samuel C. Kline, MD, Virginia Beach, VA, Lawrence E. Holder, MD, FACR, Baltimore, MD Clinical and scintigraphic criteria are proposed for the diagnosis of segmental reflex sympathetic dystrophy. Eight patients met previously described clinical criteria for reflex sympathetic dystrophy with involvement limited to only a portion of the hand. The delayed phase of the three-phase radionuclide bone scan was found to be highly sensitive (100%) for this small group of patients. Consecutive bone scans (n = 127) performed during a 6-month period for a variety of upper extremity problems were reviewed, and a segmentally diffuse pattern of tracer uptake was found to be highly specific (98%) for segmental reflex sympathetic dystrophy. Recognition and documentation of a more localized form of reflex sympathetic dystrophy will allow earlier recognition and treatment, which is an important factor in a successful outcome for managing pain dysfunction disorders. (J Hand Surg 1993;18A:853-859.)
This study presents a group of patients evaluated by three-phase radionuclide bone scanning (TPBS) for segmental reflex sympathetic dystrophy (RSD). They were referred by a group of trained hand surgeons practicing at a tertiary referral center who felt that a clinical diagnosis of RSD was justified despite the absence of total hand involvement. The appreciation of a more localized form of RSD and the presence of an objective marker of involvement may aid in the early diagnosis and management of symptoms resulting from sympathetic dysfunction. Although RSD, as we have defined it, may be part of a broader spectrum of sympathetic maintained pain syndromes,“* RSD in the hand is so well established that we have retained this terminology. Criteria for the clinical diagnosis of segmental From the Department of Hand Surgery, The Raymond M. Curtis Hand Center and the Department of Nuclear Medicine, The Union Memorial Hospital, Baltimore, MD. Received for publication July 24, 1991; accepted in revised form Jan. 25, 1993. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Samuel C. Kline, MD, Virginia Beach Orthopaedic Associates, 1853 Old Donation Parkway, Virginia Beach, VA 23454.
RSD were based on those previously described for regional RSD, I-8 but with anatomic involvement limited to less than the entire hand. These criteria included diffuse pain, loss of function, and autonomic dysfunction. Since radionuclide scintigraphy is now considered one of the most sensitive and specific tests for regional RSD,2,7-‘0 we evaluated the potential of TPBS to provide objective evidence of segmental RSD. Our experience with TPBS in establishing sensitivity, specificity, and predictive value data is presented. Materials
and Methods
Patient Selection Six patients meeting our clinical criteria for segmental RSD were specifically referred for evaluation by TPBS. In addition, all 127 hand scans performed in the Department of Hand Surgery of the Union Memorial Hospital from January to June 1990 were reviewed, and two more patients with this clinical syndrome were found. The criteria (Table 1) were those well established and accepted for regional RSD2*’ ; signs and symptoms involved less than the entire hand, most often only a single ray, and occasionally two or three rays. Very often the The Journal of Hand Surgery
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Table 1. Criteria for Reflex Sympathetic Dystrophy Diffuse, nonanatomic pain frequently out of proportion to the initiating cause Loss of function including joint stiffness and motion impairment Objective evidence of autonomic dysfunciton including atrophy of the skin or subcutaneous tissue, edema, temperature changes, sweating
patient had a hypersensitivity component consisting of hyperalgesia (reduced pain threshold with increased pain response to normal stimuli such as light touch of the skin), hyperpathia (prolonged pain after stimulation), or allodynia (marked pain from nonnoxious stimulation).2~4 Patients were assigned to clinical groups based on presenting symptoms (Table 2). Group 1 consisted of patients with segmental RSD. Symptoms compatible with this syndrome were present for an average of 3 months, with a range of 1-4 months. There were four women and four men, ranging in age from 29 to 57 years. Group 2 consisted of patients felt to have regional RSD in accordance with the criteria described.*y3T7 Group 3 was composed of patients with diffuse hand pain without other criteria for RSD. Group 4 was composed of patients with focal symptoms from possible fractures, infections, tumors, or unexplained pain. Group 5 was a miscellaneous group of patients referred for vascular problems or for evaluation of incidental x-ray film findings. Follow-up of patients in group 1 and in those patients whose TPBS pattern met the criteria for segmental RSD (Table 3) ranged from 6 months to 9 years (mean, 2.5 years). Since the purpose of this study was not to define the usefulness of TPBS in other conditions, no specific follow-up of patients
Table 2. Presenting Clinical Examination Group (n)
Segmental RSD (8) Regional RSD (23) Diffuse pain, not RSD (8) Focal pain (89)
Other (5)
Symptoms
without a clinical history suggestive of segmental RSD or a segmental TPBS pattern was undertaken. Studies
Scintigraphic
Three-phase bone scanning of the hands was performed following a standard technical protocol.‘*’ The images were evaluated without any clinical or historical data, using well-established criteria for normal and abnormal studies.3,7-9 The images were graded as normal, focally abnormal, diffusely abnormal involving the entire hand (regional), or diffusely abnormal involving less than the entire hand (segmental) (Table 3). The criteria for a diffusely abnormal scan used in previous studies of regional RSD3,7*8 were used to develop criteria for the segmental RSD pattern. As a minimum for segmental RSD, we required diffuse juxta-articular uptake in the distal interphalangeal, proximal interphalangeal, and metacarpophalangeal joints of a single ray, although the shafts themselves and carpometacarpal and intercarpal joints were also often involved. A focally abnormal scan included single or multiple discrete foci of increased uptake involving discrete anatomic structures. Although all three phases of the TPBS were evaluated, results, as for studies on regional RSD, were based only on the delayed images. Representative
Case Histories
Case 1: Segmental RSD With Multiple Ray Involvement. A 40-year-old man sustained distal interpha-
langeal dislocations of his ring and middle fingers, treated by closed reduction and early splinting. He presented 4 months after his initial injury complaining of severe hand pain. Examination revealed swelling, stiffness, tenderness, and hypersensitivity limited to his middle, ring, and little fingers. The
and Bone Scan Findings in 133 Patients Focal Abnormal Increase
Segmental RSD Pattern
Regional RSD Pattern
8 2t
0 8
0 7
0
2
1* 0
4 0
Normal
Patients With Two Findings*
2
0 8 4
0 2 0
49 3
41 2
6 0
* Eight patients had more than one finding on three-phase bone scan, most often a focal abnormality superimposed on more diffuse abnormal increased uptake. t Both of these patients had an apparent segmental RSD pattern superimposed on a more regional RSD pqttern. $ This patient had prolonged cast immobilization for snuffbox tenderness, RSD, we considered his scan a false positive during statistical analysis. RSD, reflex sympathetic dystrophy.
and although he may have had segmental
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Table 3. Diagnostic
Normal scan
Focal abnormality
Regional pattern
Segmental pattern
Bone Scan Patterns
Individual carpal bones are not defined. Metacarpophalangeal joints are defined with one half to two times more activity than the metacarpal or phalangeal shafts. It is less easy to distinguish proximal interphalangeal joints and even more difficult to distinguish the distal interphalangeal joints. Single or multiple foci of increased
uptake involving a discrete anatomic structure. Diffuse increased uptake involving the entire hand with periarticular accentuation including the radioand ulnocarpal, intercarpal, carpometacarpal, metacarpophalangeal, and interphalangeal joints. Wrist activity is increased, and proximal interphalangeal activity is more prominent than distal interphalangeal activity. Diffuse increased uptake involving at least a single ray, but less than the entire hand, with periarticular accentuation of at least the metacarpophalangeal and interphalangeal joints of the involved ray(s).
Figure 1. Segmental reflex sympathetic dystrophy with multiple ray involvement in a 40-year-old man. Delayed image, palmar view. Intense diffuse increased tracer accumulation in the right wrist and ulnar three rays when compared to left side. Juxta-articular accentuation is noted in those rays, as is a subtle relative increase in the second right ray. Right tracer activity is represented by black dots on white background.
index finger and thumb were nontender with good range of motion. X-ray films revealed juxta-articular osteoporosis without other abnormalities. Threephase bone scans demonstrated intense juxta-articular uptake throughout the middle, ring, and little fingers and the wrist (Fig. 1). The patient underwent intense physical therapy and psychiatric evaluation. Despite these efforts, continued pain and loss of motion resulted in permanent disability 9 years after his injury. Case 2: Segmental RSD With Isolated fZay fnvolvemed. A 44-year-old woman sustained a crush in-
jury to her proximal interphalangeal joint and was splinted for a closed acute central slip injury without radiographic abnormalities. A year after successful management of her injury, she presented with pain and swelling of her proximal interphalangeal joint. Aspiration was negative for infection or crystalline deposition, and the patient underwent extensor tenolysis. Postoperatively she developed further swelling, tenderness, and hypersensitivity and was unable to move her finger. Two months after her surgery, TPBS demonstrated diffuse increased uptake involving her middle ray (Fig. 2). She underwent intense physical therapy and a series of stellate gan-
Figure 2. Segmental reflex sympathetic dystrophy with isolated ray involvement in a 44-year-old woman. Delayed image, palmar view. Moderate intense diffuse increased tracer accumulation in the left third ray. Juxta-articular accentuation was noted. There is slight additional accentuation at the site of healing underlying crush injury at the proximal interphalangeal joint. Minimal relative increased activity in adjacent second ray.
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Figure 3. Segmental reflex sympathetic dystrophy with
isolated ray involvement in a 52-year-old woman. Delayed image, palmar view. Mildly intense diffuse increased tracer accumulation in the right second ray, with juxtaarticular accentuation at metacarpophalangeal and interphalangeal joints. Incidental mild focal activity was thought to be nonspecific biomechanical stress response (noninflammatory arthritis) at right first interphalangeal and third proximal interphalangeal and left first carpalmetacarpal joints. glion blocks with good results. Six months later she had residual stiffness and mild tenderness to deep pressure, but no hypersensitivity. Case 3: Segmental RSD With Isolated Ray InvolveA 52-year-old woman underwent multiple surgical procedures for removal of subungual condylomas over a B-month period. She presented with a nailbed deformity, severe hypersensitivity along the entire index finger, and stiffness and edema involving the interphalangeal and metacarpophalangeal joints. There was no evidence of infection. The TPBS demonstrated diffuse increased uptake involving her index ray (Fig. 3). She underwent stellate ganglion blocks and intensive physical therapy. Eight months later she was pain-free, nontender, and had only slightly decreased motion of her interphalangeal joints.
ment.
Results The eight patients in group 1 who met the strict criteria for segmental RSD were found to have a recognizable scan pattern. They demonstrated diffuse increased uptake in the involved ray, including periarticular uptake at the interphalangeal and meta-
carpophalangeal joints. Increased uptake was also found in the carpal region (n = 3) and occasionally in an adjacent ray (n = 2), but with less intense uptake than in the involved ray. One patient also demonstrated a slightly increased background activity of his entire hand; however, uptake in the involved ray was clearly more intense. For this select group of patients, the TPBS was very sensitive (100%) in evaluating segmental RSD involvement . These patients are described in more detail in Table 4. Of the 127 sequential TPBSs evaluated to obtain specificity and predictive value data, 5 patients had a scintigraphic pattern consistent with segmental RSD. Two of these patients also had clinical findings and were included in group 1. One patient demonstrated segmental scintigraphic abnormalities of his thumb and carpal region. He was felt to have deQuervain’s disease in association with prolonged cast immobilization for snuffbox tenderness. The bone scan was obtained to rule out scaphoid fracture. This patient was not available for long-term follow-up evaluation. For statistical purposes he was considered to have a false positive result for segmental RSD. The other two patients, also classified as false positive for segmental RSD, were clinically felt to have regional RSD. They had more intense segmental tracer uptake superimposed on the diffuse pattern of regional RSD. One of these patients had rheumatoid arthritis. She had severe middle finger pain and swelling superimposed on more diffuse changes compatible with regional RSD. The other patient demonstrated “radial-to-ulnar fade,” a pattern of regional RSD with slight radial accentuation of tracer uptake. We incidentally had noted this pattern in other patients evaluated for regional RSD.
Discussion Segmental RSD, as we have characterized it, is a localized disorder closely related to regional RSD and can be defined by similar clinical and diagnostic criteria applied to a smaller anatomic region of involvement. If loss of function, diffuse pain, and evidence of sympathetic dysfunction are confined to a portion, rather than the entire hand, a diagnosis of segmental RSD should be considered. Results of this study demonstrate that TPBS is both highly sensitive (100%) and specific (98%) for the diagnosis of segmental RSD. The positive predictive value was 0.727, and the negative predictive value was 1.0 (Table 5). Specificity and negative predictive value in this study may have been overestimated since we accepted the clinical evaluation regarding the pres-
The Journal of Hand Surgery / Vol. 18A No. 5 September
Table 4. Patients With Segmental AgelSex
Initiating Factor
52/F (case 3) condyloma; multiple surgical procedures on index finger
40/M (case 1)
DIP dislocation of middle and ring fingers
35/M
Crush injury of thumb with segmental nerve loss
57/F
Overuse syndrome; middle finger
44/F (case 2)
Closed central slip injury of middle finger PIP joint
SO/F
MP and PIP joint sprain of index finger
49/M
Laceration of middle fingertip
29/M
Tip amputation middle finger
CMC, carpometacarpal;
Physical Examination Tenderness, decreased range of motion, hypersensitivity limited to index finger Duration: 4 months Tenderness, decreased range of motion, hypersensitivity, limited to ulnar side of hand Duration: 4 months Tenderness, decreased range of motion, hypersensitivity, limited to thumb ray and CMC joint Duration: I month Tenderness, decreased range of motion, hypersensitivity, limited to middle linger Duration: 3 months Tenderness, decreased range of motion, hypersensitivity, limited to middle ray Duration: 2 months Tenderness, decreased range of motion, hypersensitivity Duration: 3 months Tenderness, decreased range of motion, hypersensitivity limited to middle finger Duration: 3 months Tenderness, decreased range of motion, hypersensitivity limited to middle finger Duration: 4 months
DIP, distal interphalangeal,
Reflex Sympathetic Bone Scan Findings
857
Dystrophy Treatment (Response)
Follow-up .._. _...__ ~ 8 months Nontender. slightly decreased range of motion
Diffuse increased uptake, index ray
Stellate + Therapy +
Diffuse increased uptake involving ulnar 3 rays and wrist
Psychiatric counseling Therapy +
Diffuse increased uptake involving thumb ray and CMC joint
Stellate + Therapy + Surgery -
1 year Still undergoing treatment for severe thumb pain
Diffuse increased uptake involving middle ray with slight increase involving adjacent rays
Therapy t Surgery -
8 months Nontender with moderate loss of PIP joint motion
Diffuse uptake involving middle ray with mild increased uptake of adjacent index ray
Stellate + Therapy +
8 months Residual loss of motion with minimal tenderness and no hypersensitivity
Diffuse increased uptake limited to index ray
Stellate Therapy +
1.5 years Residual disability with pain and stiffness
Diffuse uptake limited to middle ray
Stellate + Therapy +
4 years Full range of motion. nontender
Diffuse increased uptake middle ray with slightly increased background activity involving rest of hand
Stellate Therapy + Surgery +
8 months Good range of motion with minimal tenderness and no hypersensitivity
MP, metacarpophalangeal;
ence of “segmental” symptoms provided at the time of presentation. Although most patients were referred by trained hand surgeons, it is possible that with an increased awareness of this entity, an occasional patient with a normal or focal scan pattern may have had clinical symptoms suggestive of segmental RSD. The segmental RSD pattern described in this paper has been uncommonly encountered
1993
+
9 years Nontender with permanent disability based on loss of motion
PIP, proximal interphalangeal.
over the years in our center. where over 300 upper extremity TPBSs are performed annually, many after finger crushing injuries. There have been several individual case reports suggesting the possibility of segmental or localized RSD6,r0-“; however, none of these were defined by strict clinical or diagnostic criteria. Segmental osteoporosis was demonstrated in x-ray tilms in as-
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Table 5. Sensitivity,
Specificity, Value Results
Dystrophy
and Predictive
Final Diagnosis Segmental RSD
No Segmental RSD
Scan positive for
8
3
segmental RSD Scan negative for segmental RSD
0
122
Sensitivity = TP TFN
8 = 8+0
= 1.0
122 = .976 122 + 3
Specificity = TNT+NFP = -
TP
8
.727 =8+3= TN 122 . Negative predictive value = TN + FN = 122 = 1.0 Positive predictive value = TP + FP
RSD, reflex sympathetic dystrophy; TP, true positive; FN, false negative; TN, true negative; FP, false positive.
sociation with nerve injury” and neuropathy.i3 Helms” noted osteoporosis of the ulnar two digits of a hand following minor fractures and concluded that this segmental distribution was best explained by neural pathway transmission. The non-English literature has included references to focal osteoporosis, believed to be a more localized form of Sudek’s atrophy. ‘0,i4,i5 In these reports, there was no mention of autonomic dysfunction, nor were radionuclide bone scans obtained. Mackinnon and Dellon in their book on surgery of the peripheral nerve included a delayed radionuclide bone image of a patient who they believed to have signs and symptoms of RSD confined to a single digit following trivial injury to an index finger.6 Kozin mentioned single digit RSD but gave no examples.4 Two of the three false positive studies were in patients with the diffuse pattern of regional RSD with more intense segmental uptake in isolated rays. One of the patients had rheumatoid arthritis, which was not a diagnostic problem since his examination was clearly that of regional RSD. The other patient also was not a diagnostic problem, since his examination was clearly that of regional RSD. Perhaps the differential uptake was related to subtle subclinical variations in use of the rays. Segmental RSD provides further circumstantial evidence that there is a spectrum of sympathetically mediated pain syndromes and may have implications for the pathophysiology of these apparently closely related conditions. The anatomic boundaries of involvement in RSD
or sympathetically mediated pain syndromes vary from the small area illustrated by this study to those patients who have involvement of their entire extremity from the shoulder to the fingertips.‘*4*s*16*‘7 Some authors have reported bilateral symptoms or bilateral x-ray film abnormalities,5*‘8 although in our experience this is very rare. These variations suggest that a combination of local and regional factors may all act on a common final pathway, with the process initiated at several levels and anatomically limited by proximity to the final common pathway. Mackinnon7 has postulated a variety of tissue components and neurotransmitters that may be active in these processes. These substances are produced both locally from tissue injury and more generally in response to sympathetic nervous stimulation. The chemical milieu created by these substances may be the final common pathway that produces all the clinical stigmata, radiographic changes, and radionuclide tracer uptake classically associated with RSD. The boundary between normal and abnormal response to an injury may be crossed when the local chemical milieu is enhanced or sustained by abnormal sympathetic activity, which may be activated at varying levels, sometimes central and sometimes more peripheral. The vast majority of individuals with painful hand and finger injuries do not demonstrate the clinical or scintigraphic abnormalities demonstrated by the small group of patients in this series. However, when recovery is abnormally prolonged and symptoms are out of proportion to the clinical injury, the contribution of sympathetic dysfunction should be considered. Management of patients with sympathetically mediated pain syndromes requires accurate diagnosis of the sympathetic component of their disorder in addition to an exhaustive search for anatomic sources serving as a triggering mechanism 1,2,4,18-20 The authors appreciate the advice and assistance of colleagues associated with the Curtis Hand Center and the nuclear medicine technologists who were especially conscientious in producing quality images. Nancy Hawkins typed the manuscript and Henri Hessels of the Radiology Photography Department of The Johns Hopkins Hospital prepared the illustrations.
References 1. Amadio PC. Pain dysfunction syndromes. J Bone Joint Surg 1988;70A:944-9. 2. Amadio PC, Mackinnon SE, Merritt WH, Body GS, Terzis SK. Reflex sympathetic dystrophy syndrome: consensus report of an ad hoc committee of the American Association for Hand Surgery on the definition of reflex sympathetic dystrophy syndromes. J Plast Reconstr Surg 1991;87:371-5.
The Journal of Hand Surgery I Vol. 18A No. 5 September 1993 3. Holder LE, Mackinnon SE. Reflex sympathetic dystrophy in the hands: clinical and scintigraphic criteria. Radiology 1984;152:517-22. 4. Kozin F. Reflex sympathetic dystrophy syndrome. Bull Rheum Dis 1986;36: l-8. 5. Kozin F, Soin JS, Ryan LM, Carrera GF, Wortmann RL. Bone scintigraphy in the reflex sympathetic dystrophy syndrome. Radiology 1981;138:437-43. 6. Mackinnon SE, Dellon AL. Surgery of the peripheral nerve. New York: Thieme Medical 1988:492-503. 7. Mackinnon SE, Holder LE. The use of three-phase radionuclide bone scanning in the diagnosis of reflex sympathetic dystrophy. J Hand Surg 1984;9A:556-63. 8. Maurer AH, Holder LE, Espinola DA, Rupani HD, Wilgis EFS. Three-phase radionuclide scintigraphy of the hand. Radiology 1983;146:761-75. 9. Demangeat JL, Constantinesco A, Brunot B, Foucher G, Fariot JM. Three-phase bone scanning in reflex sympathetic dystrophy of the hand. J Nucl Med 1988; 29:26-32. 10. Faulong L. Dystrophie reflex dur membre superiere. Rev Rhum Ma1 Osteoartic 1952;19:554-63. 11. Chester MH. Segmental manifestation of reflex sympathetic dystrophy limited to one finger. Anesthesiology 1990;73:558-61. 12. Helms CA, O’Brien ET, Katzberg RW. Segmental
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reflex sympathetic dystrophy syndrome. Radiology 1980;135:67-8. Karasick S, Karasick D. Case report 193: diagnosis: segmental reflex sympathetic dystrophy syndrome affecting both hands. Skeletal Radio1 1982;8: 151-2. Dammann F. Isoliertes Sudeck-Syndrom nur an drei Fingern: Bietrag zur Entstehung eines peripher-neurogenin Sudeck-Syndroms. Mschr Unfallheilk 1972; 75: 13-22. Rauvault PP, Bouvier M. Les Algodystrophies. Rev Prat 1966;16:2529-45. Greyson ND, Tepperman PS. Three-phase bone studies in hemiplegia with reflex sympathetic dystrophy and the effect of disuse. J Nucl Med 1984;25:423-9. Resnick DR, Niwayama G. Diagnosis of bone and joint disorders. Philadelphia: WB Saunders 1988: 2034-47. Linson MA, Leffert R, Todd DP. The treatment of upper extremity reflex sympathetic dystrophy with prolonged continuous stellate ganglion blockade. J Hand Surg 1983;8:153-9. Schutzer SF, Gossling MR. The treatment of reflex sympathetic dystrophy syndrome. J Bone Joint Surg 1984;66A:625-29. Simon H, Carlson DH. The use of bone scanning in the diagnosis of reflex sympathetic dystrophy. Clin Nucl Med 1980:5:116-21.