|IMAGES IN RHEUMATOLOGY
|Year : 2018 | Volume
| Issue : 4 | Page : 277-279
Complex regional pain syndrome with an unusual aetiology
Punit Pruthi1, Pramod Arora2, Kunal Bahrani3, Manoj Mittal4
1 Department of Internal Medicine and Rheumatology, Asian Institute of Medical Sciences, Faridabad, Haryana, India
2 Department of Nuclear Medicine, Asian Institute of Medical Sciences, Faridabad, Haryana, India
3 Department of Neurology, Asian Institute of Medical Sciences, Faridabad, Haryana, India
4 Department of Radiodiagnosis, Asian Institute of Medical Sciences, Faridabad, Haryana, India
|Date of Web Publication||18-Nov-2018|
Dr. Punit Pruthi
Asian Institute of Medical Sciences, Sector 21A, Faridabad - 121 005, Haryana
Source of Support: None, Conflict of Interest: None
Keywords: Carpal tunnel syndrome, complex regional pain syndrome, reflex sympathetic dystrophy
|How to cite this article:|
Pruthi P, Arora P, Bahrani K, Mittal M. Complex regional pain syndrome with an unusual aetiology. Indian J Rheumatol 2018;13:277-9
Complex regional pain syndrome (CRPS) is a pain disorder characterized by spontaneous pain usually triggered by a noxious stimulus, most commonly fracture, soft-tissue injury, and surgery. Among the nontraumatic causes of CRPS, carpal tunnel syndrome (CTS) is not often considered as an etiological differential. We present a case of untreated idiopathic CTS precipitating CRPS.
A 52-year-old male patient without any comorbidities presented to us with complaints of pain and paresthesia over the right hand of 6 months' duration. The symptoms first developed in thumb, index, and middle fingers with nocturnal exacerbation, and for the past 2 months progressed to involve whole of the right hand and distal forearm along with diffuse swelling of the hand. The pain was constantly present, with exacerbation on touch and movement at joints of hand resulting in gross limitation of his activities. There were no constitutional features, and he denied any history of trauma or surgical intervention. One month before the presentation, he also developed similar symptoms in his left hand, but with much less severity and without any swelling.
On examination, the right hand was diffusely swollen and warm, with marked tenderness to touch, allodynia, reduced range of motion, and mild flexion contracture of fingers [Figure 1]a. His left-hand examination was normal.
|Figure 1: (a): Diffuse swelling of the right hand with mild flexion contracture of fingers. (b) Near normalization of swelling of the right hand, but with persisting mild contracture|
Click here to view
On further evaluation, his laboratory parameters including hematological, metabolic profile, rheumatoid serology, and inflammatory markers were normal. Musculoskeletal ultrasonography was done which revealed diffuse subcutaneous edema of the right hand without any evidence of synovitis. His nerve conduction velocity study was suggestive of bilateral Grade-3 CTS as per the neurophysiological grading scale. Plain radiograph of both hands and triple phase radioisotope bone scan was done which revealed findings highly suggestive of CRPS in the right upper limb [Figure 2].
|Figure 2: (a) Plain radiograph of both hands (posteroanterior view) showing diffuse osteoporosis of bones of the right hand with periarticular accentuation, also reduced joint space at intercarpal and interphalangeal joints. (b-d) Triple-phase radioisotope bone scan. (b) Phase 1 - Increased flow in hand and wrist on the right side. (c) Phase 2 - Increased blood pool in whole of the right hand and wrist. (d) Phase 3 - Increased periarticular tracer uptake in wrist, metacarpophalangeal and interphalangeal joints on the right side|
Click here to view
The patient fulfilled the Budapest diagnostic criteria for CRPS; he had continuing pain disproportionate to the inciting event, hyperalgesia, vasomotor changes in the form of a rise in temperature of the hand, sudomotor changes in the form of edema and motor finding of decreased range of motion. No other condition could be found that could have explained the clinical scenario. Chronology of symptoms strongly implicated CTS as the inciting cause of the right upper limb CRPS.
The patient was treated with pregabalin, prednisolone (tapered over 1 month), tramadol, alendronate, physiotherapy, nocturnal splinting, and counseling. On follow-up, corticosteroid was injected under ultrasonic guidance around both the median nerves. His symptoms gradually improved within 6 weeks of therapy with a reduction in swelling and improved range of motion, but unrelenting mild flexion contracture [Figure 1]b.
CRPS has been traditionally divided into Type-1 and Type-2, depending on the absence or presence of a preceding peripheral nerve injury., Type-1 also known as “reflex sympathetic dystrophy” represents approximately 90% of clinical presentations whereas Type-2 also known as “causalgia” represents the remaining 10% of cases.,
The pain of CRPS is regional but not in a specific nerve territory or dermatome and is usually disproportionate to the usual course of any known trauma or other lesion.
The diagnosis of CRPS is clinical and is made as per the Budapest criteria.
CTS is considered to be the most frequent compressive mononeuropathy seen in clinical practice. CTS as a cause of CRPS is mainly described in relation to trauma or surgery as evident from a large retrospective cohort of 1043 patients. In this study, CTS was a cause of CRPS in 7% of total cases. Among the Type-1 CRPS cases (patients without nerve injury), only 3% were attributed to CTS, in contrast to 36% of Type-2 CRPS patients (having median nerve injury).
The radiographs of affected limb are usually normal in early CRPS, but in later stages findings include patchy osteoporosis with periarticular accentuation and loss of joint space., Triple-phase bone scintigraphy may help to diagnose cases presenting with early disease in which it shows increased radiotracer uptake on the symptomatic side., However, a negative bone scan does not rule out the diagnosis of CRPS, as uptake may be reduced in the later stages of the disease.,
The treatment of CRPS is multidisciplinary which includes physiotherapy, psychotherapy, and drugs such as tricyclic antidepressants, antiepileptics, bisphosphonates, corticosteroids, and calcitonin., Progression to the chronic stage significantly worsens the prognosis, and in such cases, modalities such as transcutaneous electrical nerve stimulation, spinal cord stimulation, regional sympathetic blockade, surgical sympathectomy, and occupational therapy have been used with limited success.,
In around 10% of cases of CRPS, no precipitating event is identified. In the absence of trauma, CTS should be actively considered as an inciting cause, as early diagnosis leads to a better outcome in CRPS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Harden RN, Bruehl S, Stanton-Hicks M, Wilson PR. Proposed new diagnostic criteria for complex regional pain syndrome. Pain Med 2007;8:326-31.
Gorodnik R. Complex regional pain syndrome. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 6th
ed. Philadelphia: Elsevier; 2015. p. 683-9.
Atroshi I, Englund M, Turkiewicz A, Tägil M, Petersson IF. Incidence of physician-diagnosed carpal tunnel syndrome in the general population. Arch Intern Med 2011;171:943-4.
Ott S, Maihöfner C. Signs and symptoms in 1,043 patients with complex regional pain syndrome. J Pain 2018;19:599-611.
Birklein F, O'Neill D, Schlereth T. Complex regional pain syndrome: An optimistic perspective. Neurology 2015;84:89-96.
Bussa M, Guttilla D, Lucia M, Mascaro A, Rinaldi S. Complex regional pain syndrome type I: A comprehensive review. Acta Anaesthesiol Scand 2015;59:685-97.
[Figure 1], [Figure 2]