Laparoscopic sleeve gastrectomy in children and adolescents with Prader-Willi syndrome: a matched-control study



      Obesity is a leading cause of mortality and morbidity in Prader-Willi syndrome (PWS).


      To study weight loss and growth after laparoscopic sleeve gastrectomy (LSG) in pediatric patients with PWS compared with those without the syndrome.


      Academic center with a standardized care pathway for pediatric bariatric surgery as a part of a prospective clinical outcome study on children and adolescents undergoing weight loss surgery.


      Clinical data of all PWS patients who underwent LSG were abstracted from our prospective database, which included all pediatric patients who underwent bariatric surgery. These data were then compared with a 1:3 non-PWS group matched for age, gender, and body mass index (BMI). Data for up to 5 years follow-up were analyzed.


      The 24 PWS patients (mean age 10.7; 6<8 yr old, range 4.9–18) had a preoperative BMI of 46.2±12.2 kg/m2. All PWS patients had obstructive sleep apnea (OSA), 62% had dyslipidemia, 43% had hypertension, and 29% had diabetes mellitus. BMI change at the first, second, third, fourth, and fifth annual visits was –14.7 (n = 22 patients), –15.0 (n = 18), 12.2 (n = 13), –12.7 (n = 11), and –10.7 (n = 7), respectively, in the PWS group, whereas the non-PWS group had a BMI change of –15.9 (n = 67), –18.0 (n = 50), –18.4 (n = 47), –18.9 (n = 26), and –19.0 (n = 20), respectively. No significant difference was observed in postoperative BMI change (P = .2–.7) or growth (postoperative height z-score P value at each annual visit = .2–.8); 95% of co-morbidities in both groups were in remission or improved, with no significant difference in the rate of co-morbidity resolution after surgery (P = .73). One PWS patient was readmitted 5 years after surgery with recurrence of OSA and heart failure. No other readmissions occurred, and there were no reoperations, postoperative leaks, or other complications. No mortality or major morbidity was observed during the 5 years of follow-up. Among the PWS patients who reached their follow-up visit time points the total follow-up rate was 94.1%, whereas in the non-PWS group it was 97%. All patients who missed a follow-up visit were subsequently seen in future follow-ups, and no patient was lost to follow-up in either group.


      PWS children and adolescents underwent effective weight loss and resolution of co-morbidities after LSG, without mortality, significant morbidity, or slowing of growth. LSG should be offered to obese PWS patients with heightened mortality particularly because no other effective alternative therapy is available.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Surgery for Obesity and Related Diseases
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Butler M.G.
        Prader-Willi syndrome: current understanding of cause and diagnosis.
        Am J Med Genet. 1990; 35: 319-332
      1. Butler M.G. Lee P.D.K. Whitman B.Y. Management of Prader-Willi syndrome. 3rd ed. Springer-Verlag, New York2006: 1-550
        • Chen C.
        • Visootsak J.
        • Dills S.
        • Graham Jr, J.M.
        Prader-Willi syndrome: an update and review for the primary pediatrician.
        Clin Pediatr (Phila). 2007; 46: 580-591
        • Tauber M.
        • Diene G.
        • Molinas C.
        • Hebert M.
        Review of 64 cases of death in children with Prader-Willi syndrome (PWS).
        Am J Med Genet A. 2008; 146 A: 881-887
        • Butler M.G.
        Prader-Willi syndrome: obesity due to genomic imprinting.
        Curr Genomics. 2011; 12 (204–15.6)
        • McCandless S.E.
        Clinical report-health supervision for children with Prader-Willi syndrome.
        Pediatrics. 2011; 127: 195-204
        • Alqahtani A.R.
        • Antonisamy B.
        • Alamri H.
        • Elahmedi M.
        • Zimmerman V.A.
        Laparoscopic sleeve gastrectomy in 108 obese children and adolescents aged 5 to 21 years.
        Ann Surg. 2012; 256: 266-273
        • Alqahtani A.
        • Alamri H.
        • Elahmedi M.
        • Mohammed R.
        Laparoscopic sleeve gastrectomy in adult and pediatric obese patients: a comparative study.
        Surg Endosc. 2012; 26: 3094-3100
        • Alqahtani A.R.
        • Elahmedi M.O.
        • Al Qahtani A.
        Co-morbidity resolution in morbidly obese children and adolescents undergoing sleeve gastrectomy.
        Surg Obes Relat Dis. 2014; 10: 842-850
        • Alqahtani A.R.
        • Elahmedi M.
        • Alqahtani Y.A.
        Bariatric surgery in monogenic and syndromic forms of obesity.
        Semin Pediatr Surg. 2014; 23: 37-42
        • Scheimann A.O.
        • Nadler E.E.
        • Driscoll D.J.
        • et al.
        Laparoscopic sleeve gastrectomy in 108 obese children and adolescents aged 5 to 21 years by Alqahtani AR, Antonisamy B, Alamri H, Elahmedi M, Zimmerman VA.
        Ann Surg. Epub. 2013 Sep 16;
        • Scheimann A.O.
        • Butler M.G.
        • Gourash L.
        • Cuffari C.
        • Klish W.
        Critical analysis of bariatric procedures in Prader-Willi syndrome.
        J Pediatr Gastroenterol Nutr. 2008; 46: 80-83
        • Alqahtani A.R.
        Reply to letter: "Laparoscopic sleeve gastrectomy in 108 obese children and adolescents aged 5 to 21 years.".
        Ann Surg. Epub. 2013 Aug 30;
      2. Alqahtani AR, Elahmedi M. Pediatric bariatric surgery: the clinical pathway. Obes Surg 25(5):910–921.

        • Gunay-Aygun M.
        • Schwartz S.
        • Heeger S.
        • O׳Riordan M.A.
        • Cassidy S.B.
        The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria.
        Pediatrics. 2001; 108: e92
        • Holm V.A.
        • Cassidy S.B.
        • Butler M.G.
        • et al.
        Prader-Willi syndrome: consensus diagnostic criteria.
        Pediatrics. 1993; 91: 398-402
        • Angulo M.
        • Cataletto M.
        • Butler M.G.
        Prader-Willi syndrome: a review of clinical, genetic and endocrine findings.
        J Endocrinol Invest. Epub. 2015 Jun 11;
        • Bittel D.C.
        • Butler M.G.
        Prader-Willi syndrome: clinical genetics, cytogenetics and molecular biology.
        Expert Rev Mol Med. 2005; 7: 1-20
        • Horsthemke B.
        • Wagstaff J.
        Mechanisms of imprinting of the Prader-Willi/Angelman region.
        Am J Med Genet A. 2008; 146A: 2041-2052
        • Al-Qahtani A.
        Surgical approaches to pediatric obesity.
        in: Ferry R.J. Management of Pediatric obesity and diabetes. Springer, New York2011: 221-248
        • Cole T.J.
        The LMS method for constructing normalized growth standards.
        Eur J Clin Nutr. 1990; 44: 45-60
        • Butler M.G.
        • Lee J.
        • Manzardo A.M.
        • et al.
        Growth charts for non-growth hormone treated Prader-Willi syndrome.
        Pediatrics. 2015; 135: e126-e135
      3. Flegal KM, Cole TJ. Construction of LMS parameters for the Centers for Disease Control and Prevention 2000 growth charts. Atlanta: Centers for Disease Control and Prevention, 2013

      4. Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents; National Heart, Lung, and Blood Institute. Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents: summary report. Pediatrics 2011;128 Suppl 5:S213-56.

        • Chervin R.D.
        • Weatherly R.A.
        • Garetz S.L.
        • et al.
        Pediatric sleep questionnaire: prediction of sleep apnea and outcomes.
        Arch Otolaryngol Head Neck Surg. 2007; 133: 216-222
        • Marcus C.L.
        • Brooks L.J.
        • Draper K.A.
        • et al.
        Diagnosis and management of childhood obstructive sleep apnea syndrome.
        Pediatrics. 2012; 130: 576-584
        • Einfeld S.L.
        • Kavanagh S.J.
        • Smith A.
        • Evans E.J.
        • Tonge B.J.
        • Taffe J.
        Mortality in Prader-Willi syndrome.
        Am J Ment Retard. 2006; 111: 193-198
        • Schrander-Stumpel C.T.
        • Curfs L.M.
        • Sastrowijoto P.
        • Cassidy S.B.
        • Schrander J.J.
        • Fryns J.P.
        Prader-Willi syndrome: causes of death in an international series of 27 cases.
        Am J Med Genet A. 2004; 124A: 333-338
        • Ho A.Y.
        • Dimitropoulos A.
        Clinical management of behavioral characteristics of Prader-Willi syndrome.
        Neuropsychiatr Dis Treat. 2010; 6: 107-118
        • De Peppo F.
        • Di Giorgio G.
        • Germani M.
        • et al.
        BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits.
        Obes Surg. 2008; 18: 1443-1449
        • Anderson A.E.
        • Soper R.T.
        • Scott D.H.
        Gastric bypass for morbid obesity in children and adolescents.
        J Pediatr Surg. 1980; 15: 876-881
        • Marinari G.M.
        • Camerini G.
        • Novelli G.B.
        • et al.
        Outcome of biliopancreatic diversion in subjects with Prader-Willi Syndrome.
        Obes Surg. 2001; 11: 491-495
        • Siemensma E.P.
        • Tummers–de Lind van Wijngaarden R.F.
        • Festen D.A.
        • et al.
        Beneficial effects of growth hormone treatment on cognition in children with Prader-Willi syndrome: a randomized controlled trial and longitudinal study.
        J Clin Endocrinol Metab. 2012; 97: 2307-2314
        • Craig M.E.
        • Cowell C.T.
        • Larsson P.
        • et al.
        Growth hormone treatment and adverse events in Prader-Willi syndrome: data from KIGS (the Pfizer International Growth Database).
        Clin Endocrinol (Oxf). 2006; 65: 178-185
        • Eiholzer U.
        Deaths in children with Prader-Willi syndrome. A contribution to the debate about the safety of growth hormone treatment in children with PWS.
        Horm Res. 2005; 63: 33-39
        • Nagai T.
        • Obata K.
        • Tonoki H.
        • et al.
        Cause of sudden, unexpected death of Prader-Willi syndrome patients with or without growth hormone treatment.
        Am J Med Genet A. 2005; 136: 45-48
        • Nixon G.M.
        • Rodda C.P.
        • Davey M.J.
        Longitudinal association between growth hormone therapy and obstructive sleep apnea in a child with Prader-Willi syndrome.
        J Clin Endocrinol Metab. 2011; 96: 29-33
        • Carel J.C.
        • Ecosse E.
        • Landier F.
        • et al.
        Long-term mortality after recombinant growth hormone treatment for isolated growth hormone deficiency or childhood short stature: preliminary report of the French SAGhE study.
        J Clin Endocrinol Metab. 2012; 97: 416-425
        • Farooqi I.S.
        • O׳Rahilly S.
        Monogenic obesity in humans.
        Annu Rev Med. 2005; 56: 443-458
        • Choquet H.
        • Meyre D.
        Genetics of obesity: what have we learned?.
        Curr Genomics. 2011; 12: 169-179
        • Butler M.G.
        • Bittel D.C.
        Plasma obestatin and ghrelin levels in subjects with Prader-Willi syndrome.
        Am J Med Genet A. 2007; 143A: 415-421
        • Erdie-Lalena C.R.
        • Holm V.A.
        • Kelly P.C.
        • Frayo R.S.
        • Cummings D.E.
        Ghrelin levels in young children with Prader-Willi syndrome.
        J Pediatr. 2006; 149: 199-204
        • Haqq A.M.
        • Stadler D.D.
        • Rosenfeld R.G.
        • et al.
        Circulating ghrelin levels are suppressed by meals and octreotide therapy in children with Prader-Willi syndrome.
        J Clin Endocrinol Metab. 2003; 88: 3573-3576
        • Holsen L.M.
        • Savage C.R.
        • Martin L.E.
        • et al.
        Importance of reward and prefrontal circuitry in hunger and satiety: Prader-Willi syndrome vs simple obesity.
        Int J Obes (Lond). 2012; 36: 638-647
        • Karamanakos S.N.
        • Vagenas K.
        • Kalfarentzos F.
        • Alexandrides T.K.
        Weight loss, appetite suppression, and changes in fasting and postprandial ghrelin and peptide-YY levels after Roux-en-Y gastric bypass and sleeve gastrectomy: a prospective, double blind study.
        Ann Surg. 2008; 247: 401-407
        • Ochner C.N.
        • Gibson C.
        • Shanik M.
        • Goel V.
        • Geliebter A.
        Changes in neurohormonal gut peptides following bariatric surgery.
        Int J Obes (Lond). 2011; 35: 153-166
        • Fong A.K.
        • Wong S.K.
        • Lam C.C.
        • Ng E.K.
        Ghrelin level and weight loss after laparoscopic sleeve gastrectomy and gastric mini-bypass for Prader-Willi syndrome in Chinese.
        Obes Surg. 2012; 22: 1742-1745