Outcome of biliopancreatic diversion in subjects with Prader-Willi Syndrome.
BACKGROUND
In Prader-Willi Syndrome (PWS), mental retardation and compulsive hyperphagia cause early obesity, the co-morbidities of which lead to short life-expectancy, with death usually occurring in their 20s. Long-term weight loss is mandatory to lengthen the survival; therefore, the lack of compliance in voluntary food restriction requires a surgical malabsorptive approach.


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 delaying the meeting between food and biliopancreatic juices, causes an intestinal malabsorption. Indication for BPD was BMI > 40 or > 35 with metabolic complications. Preoperative mean age was 21 +/- 5 years, mean weight 127 +/- 26 kg, and mean Body Mass Index (BMI, kg/m2) 53 +/- 10. According to Holm's criteria, all of the subjects had a total score > or = 8. 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 weight loss (%EWL) was 59 +/- 15 at 2 years and 56 +/- 16 at 3 years, and then progressive regain occurred; at 5 years %EWL was 46 +/- 22 and at 10 years 40 +/- 27. Spearman rank test failed to demonstrate any correlation between weight loss at 5 years and patient data, except with lifestyle score (Spearman r = 0.8548, p < .0001). Current mean age is 31 +/- 7 years.


CONCLUSIONS
BPD has to be considered for its value in prolonging and qualitatively improving the PWS patient's life.
Marinari
G M
GM
Semeiotica Chirurgica R, University of Genoa School of Medicine, Largo Rosanna Benzi 8, 16132 Genova, Italy. marinari@unige.it
Camerini
G
G
Novelli
G B
GB
Papadia
F
F
Murelli
F
F
Marini
P
P
Adami
G F
GF
Scopinaro
N
N
D000328:Adult; D000368:Aged; D015904:Biliopancreatic Diversion / Q000009:adverse effects / Q000379:methods / Q000523:psychology; D001786:Blood Glucose / Q000032:analysis; D015992:Body Mass Index; D002784:Cholesterol / Q000097:blood; D005215:Fasting; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007361:Intelligence Tests; D008017:Life Expectancy; D008019:Life Style; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011218:Prader-Willi Syndrome / Q000097:blood / Q000175:diagnosis / Q000401:mortality / Q000523:psychology / Q000601:surgery*; D011788:Quality of Life; D016019:Survival Analysis; D016896:Treatment Outcome; D015431:Weight Loss
D001786:Blood Glucose; D002784:Cholesterol