Concurrent hypertrophic pyloric stenosis and gastroschisis: An unusual presentation of a rare association

Gastroschisis and hypertrophic pyloric stenosis rarely occur in the same patient, with few reports of this unusual association published to date. We describe a neonate with prenatally diagnosed gastroschisis treated with interval silo reduction and closure. Following definitive repair, the patient had a 30-day interval of difficulty feeding and occasional emesis that progressed to projectile vomiting. Upper GI series was consistent with hypertrophic pyloric stenosis. Upon surgical exploration, multiple gastric adhesions were found to cause a 180° organoaxial gastric volvulus with aberrant relocation of the pylorus to the left upper quadrant. Following adhesiolysis and reduction of the volvulus, a Ramstedt pyloromyotomy was performed on the hypertrophied pylorus. Postoperatively, the patient's diet was advanced as tolerated, and he was discharged home on postoperative day 10.