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JOURNAL ARTICLE
Spontaneous colonic ischemia in a patient with Riley-Day syndrome.
Pediatric Radiology 1995
Familial dysautonomia, or Riley-Day syndrome, is a hereditary disturbance in the autonomic and peripheral sensory nervous systems, first described by Riley, Day, and colleagues in 1949 [1, 2]. Previous reports of myocardial infarction and avascular necrosis in bone suggest that these patients are at risk for ischemia at certain organ sites [3, 4]. We report a case of spontaneous colonic ischemia and stricture which resulted in colocutaneous fistula formation and eventual colonic resection in a child with Riley-Day syndrome.
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