BACKGROUND: A 15 year old girl with Prader-Willi Syndrome (PWS) died of gastric rupture. Systematic literature research revealed seven case reports of PWS patients with acute gastric dilatation, two had a lethal course. The objective of this study was to determine if delayed gastric emptying in PWS patients might contribute to gastric dilatation. METHODS: Gastric emptying was measured in eight patients with PWS by nucleotid scintigraphy after a standardized test meal. RESULTS: Median age was 17.8 years (range 10.1-19.5). Median BMI of the male patients was 29.5 (range 18.4-34.8), of the female patients 28 (range 20.0-44.8). Half time of gastric emptying was delayed in five of the eight patients (median 78.5 minutes, range 59-134). CONCLUSION: Scintigraphic measurement of gastric emptying in eight PWS patients revealed delay in comparison to normal values. This might be a risk factor for gastric dilatation and rupture in patients with PWS.
BACKGROUND: A 15 year old girl with Prader-Willi Syndrome (PWS) died of gastric rupture. Systematic literature research revealed seven case reports of PWSpatients with acute gastric dilatation, two had a lethal course. The objective of this study was to determine if delayed gastric emptying in PWSpatients might contribute to gastric dilatation. METHODS: Gastric emptying was measured in eight patients with PWS by nucleotid scintigraphy after a standardized test meal. RESULTS: Median age was 17.8 years (range 10.1-19.5). Median BMI of the male patients was 29.5 (range 18.4-34.8), of the female patients 28 (range 20.0-44.8). Half time of gastric emptying was delayed in five of the eight patients (median 78.5 minutes, range 59-134). CONCLUSION: Scintigraphic measurement of gastric emptying in eight PWSpatients revealed delay in comparison to normal values. This might be a risk factor for gastric dilatation and rupture in patients with PWS.