Congenital short bowel syndrome associated with appendiceal agenesis and functional intestinal obstruction

Congenital short bowel with functional intestinal obstruction and absence of appendix vermiformis is a very rare condition with poor prognosis. Seventeen cases of congenital short bowel have been reported previously in the literature. The etiology is unknown. In this report, a case of congenital short bowel, combined with functional intestinal obstruction, mal-rotation, and absence of the appendix vermiformis, is presented and the pathogenesis discussed.