[Acute gastric volvulus secondary to asplenia in a child with Smith-Lemli-Opitz syndrome].

An acute gastric volvulus in a 10 years old boy with Smith-Lemli-Opitz syndrome is reported. He was immediately operated on and an organoaxial volvulus secondary to spleen agenesia was found. No other visceral malformation was present. Because of gastric ischemia, a second-look procedure was performed 48 hours later in order to asses necrosis extent. Gastric fundus had to be resected and the stomach was definitively fixed to the abdominal wall. Clinical, radiological and therapeutic characteristics of this unusual pathology are reviewed.