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 infarction was caused by prolonged hypoperfusion. It is recognized that the small bowel in normal subjects can adapt to periods of ischemia without irreversible injury. The authors speculate that the known abnormal systemic cardiovascular regulation in patients with FD may adversely affect splanchnic blood flow, which led to the catastrophic consequences in this case. This report draws attention to the risk of significant ischemic complications during a dysautonomic crisis, especially in the face of atypical features, and emphasizes the challenging cardiovascular management of such patients. Copyright (C) 1999 by W.B. Saunders Company.