Nonocclusive small bowel infarction in familial dysautonomia syndrome

The authors report a case of a 14-year-old boy with familial dysautonomia ( FD) in whom a small-bowel infarction developed during a dysautonomic crisis . Atypical features of the presentation included hypotension with prolonged fever and abdominal distension. The authors postulate that the bowel infar ction was caused by prolonged hypoperfusion. It is recognized that the smal l bowel in normal subjects can adapt to periods of ischemia without irrever sible injury. The authors speculate that the known abnormal systemic cardio vascular regulation in patients with FD may adversely affect splanchnic blo od flow, which led to the catastrophic consequences in this case. This repo rt draws attention to the risk of significant ischemic complications during a dysautonomic crisis, especially in the face of atypical features, and em phasizes the challenging cardiovascular management of such patients. Copyri ght (C) 1999 by W.B. Saunders Company.