Spontaneous colonic ischemia in a patient with Riley-Day syndrome.
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.
Applegate
K E
KE
Department of Radiology, Children's Hospital, Boston, Massachusetts 02115, USA.
Sargent
S K
SK
D002648:Child; D003106:Colon / Q000098:blood supply* / Q000000981:diagnostic imaging; D004402:Dysautonomia, Familial / Q000150:complications*; D005260:Female; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D007511:Ischemia / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography