ECEESPE2025 Oral Communications Oral Communications 15: Metabolism, Nutrition and Obesity (6 abstracts)
1"Bambino Gesù" Children Hospital, Endocrinology and Diabetology Unit, Rome, Italy; 2Sapienza University of Rome, Department of Experimental Medicine, Rome, Italy; 3Fondazione Policlinico Universitario A. Gemelli, Center for Rare Diseases and Congenital Defects, Rome, Italy; 4"Bambino Gesù" Children Hospital, General Paediatric Surgery Unit, Rome, Italy; 5"Bambino Gesù" Children Hospital, Research unit for Preventive and Predictive Medicine, Rome, Italy
JOINT3694
Introduction: Prader Willi Syndrome (PWS) is a rare genetic disorder (1:20,000 live births) characterized by hypothalamic dysfunction, leading to endocrine complications including early-onset, and life-threatening obesity. Controlling weight is particularly challenging due to behavioural disorders and an intense hyperphagic drive. In cases of progressive obesity, bariatric surgery, such as laparoscopic sleeve gastrectomy (LSG), may be considered. While LSG is effective in non-syndromic adolescents and young adults with severe obesity, evidence of its long-term efficacy in PWS remains limited.
Patients and methods: We enrolled 16 patients affected by genetically confirmed PWS, and 32 sex-, age- and BMI-matched non-syndromic control subjects (OB) with severe obesity (BMI-standard deviation score [SDS] >3), who underwent LSG. The whole cohort included 24 males (50%), with a median age at surgery of 16.2 years [range: 8.1-26.7]. All subjects underwent comprehensive endocrine and metabolic evaluation, received personalised nutritional counselling, as well as psychological assessment and multidisciplinary support before and after surgery.
Results: Baseline median weight, BMI and BMI-SDS were 117.6 [IQR 97.3-131.9] vs 122.3 [111.5-144.0] Kg (P=0.130), 47.0 [41.1-53.4] vs 44.3 [40.7-49.5] Kg/m2 (P=0.252), and +5.2 [4.3-6.3] vs +4.5 [4.0-5.0] (P=0.032) in the PWS and OB groups, respectively. Patients were followed-up for a median of 4.8 [3.6-7.1] years. Changes in mean BMI-SDS at each year were significantly lower in PWS compared to OB patients: year 1 (-30.6±19.9% vs -55.7±23.6%, P=0.008), year 2 (-29.8±31.2% vs -65.5±28.5%, P=0.008), year 3 (-8.4±31.8% vs -65.0±33.4%, P=0.006), year 4 (-9.1±31.6% vs -62.1±30.4%, P=0.006), year 5 (-18.5±34.5% vs -58.5±35.1%, P=0.014). The number of comorbidities (intermediate hyperglycaemia or diabetes mellitus, hypertension, hypercholesterolemia, hypertriglyceridemia and obstructive sleep apnea) decreased from 2.6±1.5 to 1.9±1.2 in the PWS group (P=0.051), and from 1.1±0.9 to 0.5±0.2 in the OB group (P=0.125).
Conclusions: LSG results in less sustained weight loss in PWS than in OB over a 5-year period. LSG should be considered in PWS selected patients with complicated obesity when a rapid weight loss is needed. However, these findings highlight the need for alternative or adjunctive therapeutic strategies to address the complex pathophysiology of obesity in PWS and improve long-term outcomes.