Prader-Willi syndrome-associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review.

BACKGROUND

Prader-Willi syndrome (PWS) is a congenital chromosomal disorder characterized by compulsive hyperphagia and the early development of obesity. Obesity is identified as the main cause of morbidity and mortality in PWS individuals. Thus, body weight reduction is of major importance for a prolonged survival. PATIENT-METHOD: A 20-year-old female patient with PWS was referred to our department for surgical treatment of her obesity. At admission, her body weight was 153 kg, and her body mass index (BMI) was 74.33 kg/m(2). The patient underwent biliopancreatic diversion with duodenal switch, as well as cholecystectomy and appendicectomy. The volume of the gastric remnant was 100 mL, and the lengths of the gastric and common limbs were 250 and 60 cm, respectively.

RESULTS

Eighteen months after the operation, the patient lost 63 kg with no considerable changes in her eating habits. Her sleep disturbances and sleep apnea disappeared, and her social life dramatically improved.

CONCLUSIONS

Biliopancreatic diversion with duodenal switch seems to be a good method for the treatment of PWS-associated obesity because it offers good results in weight loss without the need for revision, good quality of life, and a chance for a prolonged survival.