ACUTE IDIOPATHIC GASTRIC DILATATION WITH GASTRIC NECROSIS IN INDIVIDUALS WITH PRADER-WILLI-SYNDROME

Individuals with Prader-Willi syndrome (PWS) have excessive appetite w ith the ability to consume large quantities of food, Absence of vomiti ng and a high pain threshold are considered manifestations of the diso rder, We present 6 patients with PWS with acute dramatic gastric diste ntion, In 3 young adult women with vomiting and apparent gastroenterit is, clinical course progressed rapidly to massive gastric dilatation w ith subsequent gastric necrosis, One individual died of overwhelming s epsis and disseminated intravascular coagulation, In 2 children, gastr ic dilatation resolved spontaneously, Gastrectomy specimens-in 2 cases subtotal and distal, in the other with accompanying partial duodenect omy and pancreatectomy-showed similar changes, All cases demonstrated signs of ischaemic gastroenteritis, All specimens showed diffuse mucos al infarction with multifocal transmural necrosis, Vascular dilatation and small bifrin thrombi were apparent within the infarcted areas, Th ese 6 women with PWS had acute idiopathic gastric dilatation, It is po ssible that a predisposition to acute gastric dilatation may be relate d to abnormal gastric homeostasis on a genetic basis, Understanding th e mechanisms responsible for this event could increase the understandi ng of gastrointestinal and appetite regulation in individuals with PWS . (C) 1997 Wiley-Liss, Inc.