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ABSTRACT
Reflex sympathetic dystrophy is a syndrome characterized by pain in one or more extremities, usually associated with vasomotor changes. Its occurrence in childhood has long been thought to be rare. We describe six cases of pediatric reflex sympathetic dystrophy and suggest that this syndrome could be underdiagnosed in children and adolescents. Psychologic problems frequently play a role in this disorder, which often can be treated conservatively. We also point out that the diagnosis is mainly clinical. An early diagnosis can avoid unnecessary tests and potentially can improve response to treatment, and prognosis. (J Child Neurol 1999;14:363-368).
Reflex sympathetic dystrophy, also known by a variety of other names (algodystrophy, causalgia, Sudeck's atrophy, reflex neurovascular dystrophy) is a syndrome characterized by swelling of a limb associated with pain, and with vasomotor changes. Its etiology is still obscure: Several pathogenetic theories have been proposed.1 The occurrence of reflex sympathetic dystrophy is considered rare in children,2,3 but it has been suggested recently that this syndrome could be underestimated and misdiagnosed in the pediatric age group.4
Successful treatment, which could allow for the avoidance of complications, is based on early recognition of the disease. For this reason, we report six patients, seen in three pediatric referral centers, who demonstrate that reflex sympathetic dystrophy can occur frequently in childhood and that the diagnosis is primarily based on clinical grounds. The diagnostic work-up for these patients is often quite extensive; since emotional problems frequently play a role, multiple unnecessary investigations and referrals could perpetuate signs and symptoms.
CASE REPORTS
Case 1
A 9-year-old girl was admitted to a Pediatric Unit with a history of severe pain in the left ankle and increasing disability. Her medical history was unremarkable except for Henoch-Schonlein purpura at the age of 5 years. Six months prior to admission the patient started complaining of recurrent episodes of pain in her left ankle. The pain, absent at night, was described as burning and the girl abandoned the use of the affected limb. However, during painless periods she was normal and no joint abnormalities were noted. Two weeks before admission the pain worsened for 3 consecutive days, and after a short interval of improvement, worsened again, now involving the left knee and calf. The left ankle...