Failure of biliopancreatic diversion in Prader-Willi syndrome

Background: Prader-Willi syndrome (PWS) is the most common genetic obesity. Excessive weight gain follows failure-to-thrive in early infancy; in adole scents and young adults, excess body weight can exceed 100%. The hyperphagi a associated with PWS is responsible for the early mortality. Dietary restr iction, alone or combined with anorexic drugs, are ineffective to induce a permanent weight loss. Thus, surgical treatment of morbid obesity in PWS ha s been attempted, but gastric restrictive operations are unable to produce stable weight loss. In a small number of patients, favorable results have b een reported with biliopancreatic diversion (BPD). Case Report: A 24-year-old woman with PWS, Pickwickian, at age 21 weighed 8 0 kg (BMI = 50) and underwent BPD. Results: 3 years after the BPD she regained 21 of the 26 kg lost; somnolenc e and respiratory difficulties were the same as before surgery. The patient now presents severe reduction of bone mass density, hypochromic anemia, hy poproteinemia, and diarrhea associated with eating. Conclusion: The regain of weight following BPD suggests that this procedure alone is not adequate for long-term control of obesity in PWS.