Diagnosis and management of gastrointestinal perforations in childhood dermatomyositis with particular reference to perforations of the duodenum.

Four children with dermatomyositis were recently seen with gastrointestinal perforations. The sites of perforation in the four cases were: (1) the duodenum, esophagus, and colon; (2) the duodenum; (3) the distal stomach; and (4) the traverse colon. The gastric and transverse colon perforations were intraperitoneal and easily diagnosed. The gastric perforation was treated successfully by partial gastrectomy. The patient with the colon perforation underwent exteriorization; death occurred from cerebral complications possibly related to vasculitis. Both duodenal perforations were posterior in the distal descending portion. Enzymatic dissection into the right lower quadrant produced confusing clinical and radiographic signs and extensive retroperitoneal necrosis. Successful treatment was obtained by partial gastrectomy, sump drainage of the perforation, and parenteral nutrition. Gastrointestinal perforation is a well-recognized complication of vasculitis in childhood dermatomyositis. In particular, perforations of the distal duodenum, as reported by others, are associated with delay in diagnosis and high mortality.