Congenital short-bowel and malrotation: Clinical presentation and outcome of six affected offspring in three related families

Newborns with congenital short small bowel associated with malrotation and intestinal dysmotility have a uniformly bad prognosis. However, few long-te rm survivors have been reported, suggesting that the disorder is not invari ably fatal. The majority of cases previously reported were familial. We rep ort on six affected siblings in three related families. The aim of this rep ort is to assess the mode of inheritance, the expression of this disorder, and to point to the correlation between the onset of gastrointestinal sympt oms and the outcome.