Gastric rupture and necrosis in Prader-Willi syndrome

DA Stevenson, J Heinemann, M Angulo… - Journal of pediatric …, 2007 - journals.lww.com
DA Stevenson, J Heinemann, M Angulo, MG Butler, J Loker, N Rupe, P Kendell, SB Cassidy…
Journal of pediatric gastroenterology and nutrition, 2007journals.lww.com
Hyperphagia and obesity are common features in individuals with Prader-Willi syndrome
(PWS). Demographic and cause-of-death data from individuals with PWS were obtained
through a national support organization. Four reports of unexpected mortality due to gastric
rupture and necrosis were found in 152 reported deaths, accounting for 3% of the causes of
mortality. Four additional individuals were suspected to have gastric rupture. Vomiting and
abdominal pain, although rare in PWS, were frequent findings in this cohort. The physician …
Abstract
Hyperphagia and obesity are common features in individuals with Prader-Willi syndrome (PWS). Demographic and cause-of-death data from individuals with PWS were obtained through a national support organization. Four reports of unexpected mortality due to gastric rupture and necrosis were found in 152 reported deaths, accounting for 3% of the causes of mortality. Four additional individuals were suspected to have gastric rupture. Vomiting and abdominal pain, although rare in PWS, were frequent findings in this cohort. The physician should consider an emergent evaluation for gastric rupture and necrosis in individuals with PWS who present with vomiting and abdominal pain.
Lippincott Williams & Wilkins