Outcome of biliopancreatic diversion in subjects with Prader-Willi syndrome

Background: In Prader-Willi Syndrome (PWS), mental retardation and compulsi ve hyperphagia cause early obesity, the co-morbidities of which lead to sho rt life-expectancy, with death usually occurring in their 20s. Long-term we ight loss is mandatory to lengthen the survival; therefore, the lack of com pliance in voluntary food restriction requires a surgical malabsorptive app roach. Methods: 15 PWS subjects were submitted to biliopancreatic diversion (BPD) and followed (100%) for a mean period of 8.5 (4-13) years. BPD consists of a distal gastrectomy with a long Roux-en-Y reconstruction which, by delayin g the meeting between food and biliopancreatic juices, causes an intestinal malabsorption. Indication for BPD was BMI >40 or >35 with metabolic compli cations. Preoperative mean age was 21 +/-5 years, mean weight 127 +/- 26 kg , and mean Body Mass Index (BMI, kg/m(2)) 53 +/- 10. According to Helm's cr iteria, all of the subjects had a total score greater than or equal to8, IQ assessment was performed in each subject, with a mean score of 72 +/- 10. An arbitrary lifestyle score was given to each subject. Results: No perioperative complications were observed. Percent excess weigh t loss (%EWL) was 59 +/- 15 at 2 years and 56 +/- 16 at 3 years, and then p rogressive regain occurred; at 5 years %EWL was 46 +/- 22 and at 10 years 4 0 +/- 27. Spearman rank test failed to demonstrate any correlation between weight loss at 5 years and patient data, except with lifestyle score (Spear man r=0.8548, p<.0001), Current mean age is 31<plus/minus>7 years. Conclusion: BPD has to be considered for its value in prolonging and qualit atively improving the PWS patient's life.