Sleeve Gastrectomy in Paediatric and Transition Age Patients with Prader-Willi Syndrome: A 5-Year Longitudinal Study

Area of Science:

• Pediatric Endocrinology
• Bariatric Surgery
• Genetics and Rare Diseases

Background:

• Prader-Willi syndrome (PWS) is a complex genetic disorder associated with severe obesity and significant cardiometabolic comorbidities.
• Effective long-term weight management strategies for pediatric and transition-age patients with PWS are limited.
• Laparoscopic sleeve gastrectomy (LSG) is a bariatric procedure increasingly considered for severe obesity, but its long-term efficacy in PWS requires evaluation.

Purpose of the Study:

• To assess the long-term effectiveness of LSG in managing severe obesity in pediatric and transition-age patients with PWS.
• To compare the outcomes of LSG in patients with PWS to matched non-syndromic individuals with obesity.
• To evaluate the impact of LSG on obesity-related comorbidities in PWS.

Main Methods:

• A single-center, longitudinal, retrospective cohort study.
• Reviewed electronic health records of 110 patients with PWS and 268 subjects undergoing LSG.
• Included 16 PWS patients with severe obesity undergoing LSG (PWS-LSG), 32 matched non-syndromic obese patients (OB-LSG), and 16 PWS patients receiving lifestyle intervention only (PWS-LS).
• Primary endpoint: change in Body Mass Index Standard Deviation Score (BMI-SDS) percentage at 5 years.

Main Results:

• PWS-LSG group showed an initial BMI-SDS decrease of ~26% at 1-2 years, with a partial rebound to -16.9% at 5 years (p=0.012).
• OB-LSG group demonstrated a more substantial and sustained BMI-SDS reduction of -57.4% at 5 years (p<0.001 within-group; p=0.002 vs PWS-LSG).
• Obesity-related comorbidities decreased in PWS-LSG (2.69 to 1.94; p=0.041) and OB-LSG (1.25 to 0.56; p<0.001) but increased in PWS-LS (1.56 to 2.44; p=0.004).

Conclusions:

• LSG in PWS patients leads to clinically significant early weight loss with partial maintenance at 5 years, though with attenuated long-term benefits compared to non-syndromic peers.
• Despite the reduced efficacy, LSG appears to alter the disease trajectory in PWS, contrasting with the progressive worsening observed with lifestyle intervention alone.
• LSG offers a potential therapeutic option for improving obesity and associated comorbidities in pediatric and transition-age PWS patients.