Résumé
Le syndrome de Prader-Willi (SPW) est une maladie génétique caractérisée par une hypotonie néonatale, une petite taille, un hypogonadisme, un retard mental et une hyperphagie compulsive expliquée, en partie, par un taux de ghréline plasmatique élevée. L’obésité est une cause majeure de morbidité et de la mortalité chez ces patients. La prise en charge diététique avec restriction alimentaire est incapable d’induire une perte de poids stable. La chirurgie bariatrique restrictive s’est avérée impuissante à obtenir une perte de poids stable. Pour un nombre limité de patients, il a été rapporté des résultats favorables après dérivation biliopancréatique (BPD). Nous rapportons un cas de SPW ayant bénéficié d’un BPD laparoscopique, suivi pendant quatre ans. Il s’agissait d’une patiente de 28 ans avec un SPW et une obésité de 123 kg, IMC = 58 kg/m2. Il n’y a pas eu de complication périopératoire. Quatre ans après l’intervention, le poids était de 101 kg et l’IMC de 47 kg/m2. Le diabète initialement insulinodépendant était devenu contrôlable par antidiabétique oral. Il n’y a pas eu d’hypoprotéinémie, ni de déficit en vitamines.
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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Sodji, M., Catheline, J.M., Chebib, J. et al. Syndrome de Prader-Willi : traitement chirurgical par bypass biliopancréatique coelioscopique. Obes 6, 172–176 (2011). https://doi.org/10.1007/s11690-011-0285-6
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DOI: https://doi.org/10.1007/s11690-011-0285-6