Results of Biliopancreatic Diversion in Two Patients with Prader-Willi Syndrome

Abstract

Prader-Willi Syndrome (PWS) is a genetic disorder characterized by hypotonia, mental retardation or learning disability, hyperphagia and compulsive eating due to hypothalamic dysfunction. Obesity is a major cause of increased morbidity and mortality among patients with PWS. Gastric restrictive surgery has been associated with partial breakdown of the staple-line in PWS. We report two patients with PWS associated with morbid obesity and obstructive sleep apnea who underwent biliopancreatic diversion (BPD). A 27-year-old male with BMI 52 kg/m(2) and a 20 year-old female with BMI 64 kg/m(2) underwent BPD. No perioperative complications were observed. After BPD, the male's BMI was 36.7 kg/m(2) at 12 months and the female's BMI was 48.4 kg/m(2) at 28 months, with excess weight loss 58% and 48%, respectively. They developed loose stools associated with eating. These patients have shown a considerable improvement in hypersomnia and respiratory difficulties. BPD proved to be an effective approach to weight loss in PWS, resulting in improvement of sleep apnea, behavior problems and quality of life.

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

“…Yet, in these patients, a drastic body weight reduction is mandatory to reduce the risk of cardio-respiratory and metabolic complications and to improve life expectancy. In the last three decades, several bariatric surgical techniques were applied to treat morbid obesity in PWS patients, such as trunkal vagotomy [13], jejunoileal bypass [14,15], vertical banded gastroplasty [16,17], adjustable silicon gastric banding [18], gastric bypass [19], and biliopancreatic diversion (BPD) [20][21][22][23][24][25][26]. However, a higher morbidity and mortality rate in these patients, compared to those with simple obesity, has been reported [27].…”

Abordagem terapêutica da obesidade na Síndrome de Prader-Willi

“…De acordo com uma revisão recente que fizemos da literatura, a derivação biliopancreática (DBP), procedimento malabsortivo, parece ser uma abordagem cirúrgica mais adequada para esses pacientes (51). Nesta revisão, relatamos a cirurgia de DBP pela técnica de Scopinaro em dois portadores da SPW, que foi eficaz na redução de peso e manutenção prolongada, com redução do IMC de 52 kg/m 2 para 36,7 kg/m 2 em um homem de 27 anos, acompanhado por 12 meses, e de 64 kg/m 2 para 48,8 kg/m 2 em uma paciente do sexo feminino, de 20 anos, seguida por 28 meses, com melhora marcante da função respiratória, além de melhora do comportamento agressivo, resultando em melhor qualidade de vida (51).…”

Roux-en-Y Gastric Bypass in an Adolescent Patient with Bardet–Biedl Syndrome, a Monogenic Obesity Disorder

“…However, apart from this form, there are several reports of multi-system genetic disorders related to obesity. Anti-obesity surgery has been previously reported for the Prader-Willi syndrome, the most common genetic cause of obesity due to variations in genomic imprinting of chromosome 15 [2][3][4]. In patients with Prader-Willi syndrome, gastric bypass and biliopancreatic diversion are the procedures that have been implemented mostly with varied degree of success [5].…”