Abstract
Prader-Willi syndrome (PWS) is a genetic disorder characterized by neonatal hypotonia, short stature, hypogonadism, mental retardation and compulsive hyperphagia partially explained by high plasma ghrelin, an orexigenic substance secreted by the stomach. Obesity is a major cause of increased morbidity and mortality in these patients. Dietary restriction alone is unable to achieve a permanent weight loss, thus surgical treatment has been attempted, but gastric restrictive operations were unsuccessful. In a small number of patients, favourable results have been reported with biliopancreatic diversion (BPD). We report a PWS case treated by laparoscopic BPD. A 28-year-old female PWS patient with morbid obesity (123 kg; BMI: 58 kg/m2) was operated. There was no complication. After 4 years, she lost 22 kg with a BMI of 47 kg/m2. This modest weight loss can be explained by insufficient food compliance. Yet, diabetes mellitus was controlled without insulin. Neither hypoproteinemia nor vitamin deficiency was observed. Laparoscopic BPD was safe, with a benefit regarding diabetes.
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