Pyloric stenosis associated with malrotation

Three cases of pyloric stenosis associated with malrotation are presented. In one case, the diagnosis was made synchronously, and in two cases, pyloric stenosis was noted following correction of malrotation. All three patients underwent pyloromyotomy and Ladd's procedure. These cases differ from the few reported hereditary cases of pyloric stenosis associated with malrotation, which have congenitally shortened bowel, functional obstruction, and poor prognosis. The unusual finding of pyloric stenosis and malrotation occurring together in the first patient was diagnosed with upper gastrointestinal series, obtained because of a high index of suspicion despite a palpable pyloric tumor and positive ultrasound examination. The second and third cases illustrate that vomiting following Ladd's procedure may be due to pyloric stenosis. When clinical findings do not support the presumed diagnosis, further investigation prior to operation is recommended so that rare concommitant pathology can be corrected.