To the Editor:
We read with great interest the recent article by Alqahtani et al1 describing a single center’s experience with laparoscopic sleeve gastrectomy between 2008 and 2011 performed on 108 children and adolescents between 5 and 21 years of age. Of note, the study included a subgroup of 13 children, including 9 with genetic obesity syndromes associated with hyperphagia (7 children with Prader-Willi syndrome and 2 children with Bardet-Biedl syndrome). Eight of 13 children (62%) in the subgroup were younger than 14 years, including a 5-year-old girl with Prader-Willi syndrome with a preoperative body mass index of 31.8 kg/m2 and a history of obstructive sleep apnea and cardiac arrests. Outcome data were reported for only 1 of 13 children in the younger than 14 years subgroup, a decline in body mass index from 31.8 to 17.1 kg/m2 reported at 12 months after sleeve gastrectomy for the 5-year-old girl with Prader-Willi syndrome. Additional outcome data for the other 8 children with genetic causes of hyperphagia and the genetic syndrome diagnostic criteria for these children were unavailable in the article.
Prader-Willi syndrome is a complex genetic disorder resulting from loss of the paternal copy of chromosome 15q11.2-13 and is considered one of the most common causes of severe obesity affecting an estimated 350,000 to 400,000 individuals globally.2 Patients with Prader-Willi syndrome have an underlying defect in satiety, altered pain threshold, decreased ability to vomit, and increased risk for development of gastric dilation and necrosis.3-5 Bardet-Biedl syndrome is a pleiotropic autosomal recessive disorder affecting ciliary function (prevalence rates ranging from 1 in 125,000 to 1 in 160,000 for individuals of European ancestry to 1 in 18,000 among consanguineous populations) accompanied by an increased risk for the development of severe obesity and diabetes mellitus.6,7 Published data regarding outcomes of bariatric procedures in patients with genetic and hypothalamic conditions associated with hyperphagia such as Prader-Willi or Bardet-Biedl are quite limited and in many series fairly disappointing.
The results of a patient with Bardet-Biedl syndrome who underwent Roux-en-Y gastric bypass with sustained drop in body mass index 52.3 to 34.9 kg/m2 at 42 months postoperatively with improvement in hypertension was published by Daskalakis et al8 in 2009. Deaths have been reported among individuals with Prader-Willi syndrome after restrictive bariatric procedures including laparoscopic silicone gastric banding and BioEnterics intragastric balloon placement.9,10 A long-term review of outcomes of bariatric surgery among individuals with Prader-Willi syndrome revealed suboptimal results in comparison with obese controls, advocating the use of a supervised hypocaloric diet with micronutrient supplementation, exercise, and restricted access to food, rather than the bariatric surgery procedures offered at that time, which did not include laparoscopic sleeve gastrectomy.11
We would request that the authors publish additional information regarding the diagnostic criteria of the individual cases, including genetic confirmation where available, and individual longitudinal outcomes for their children with hyperphagic disorders after sleeve gastrectomy, ideally with followup over several years. This would provide invaluable information for the clinical management of individuals with genetic syndromes associated with hyperphagia and morbid obesity, such as Prader-Willi and Bardet-Biedl syndromes, and to determine whether, indeed, sleeve gastrectomy is a procedure that should be considered in this unique patient population.
Footnotes
Disclosure: There are no funding received for this letter. The authors declare no conflicts of interest.
Contributor Information
Ann O. Scheimann, Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins School of Medicine, Baltimore, MD.
Evan E. Nadler, Division of Pediatric Surgery, Children’s National Medical Center, The George Washington University School of Medicine & Health Sciences, Washington, DC.
Daniel J. Driscoll, Pediatric Genetics and Metabolism, University of Florida College of Medicine, Gainesville, FL.
Merlin G. Butler, Departments of Psychiatry, Behavioral Sciences and Pediatrics, University of Kansas Medical Center, Kansas City, KS.
Jennifer L. Miller, Division of Endocrinology, Department of Pediatrics, University of Florida, Gainesville, FL.
Tania P. Markovic, Metabolism and Obesity Services, Royal Prince Alfred Hospital, Boden Institute of Obesity, Nutrition, Exercise, and Eating Disorders, University of Sydney, Sydney, Australia.
Anthony P. Goldstone, Metabolic and Molecular Imaging Group, MRC Clinical Sciences Centre, Imperial College London, Imperial Centre for Endocrinology, Hammersmith Hospital, London, United Kingdom.
REFERENCES
- 1.Alqahtani AR, Antonisamy B, Alamri H, et al. Laparascopic sleeve gastrectomy in 108 obese children and adolescents aged 5 to 21 years. Ann Surg. 2012;256:266–273. [DOI] [PubMed] [Google Scholar]
- 2.Butler MG, Hanchett JM, Thompson T. Clinical findings and natural history of Prader-Willi syndrome In: Butler MG, Lee PDK, Whitman BY, eds. Management of Prader-Willi Syndrome. 4th ed. New York: Springer; 2006:3–5. [Google Scholar]
- 3.Holm VA, Cassidy SB, Butler MG, et al. Prader-Willi syndrome: consensus diagnostic criteria. Pediatrics. 1993;91:398–402. [PMC free article] [PubMed] [Google Scholar]
- 4.Wharton RH, Wang T, Graeme Cook F, et al. Acute idiopathic gastric dilation with gastric necrosis in individuals with Prader-Willi syndrome. Am J Med Genet. 1997;73:437–441. [DOI] [PubMed] [Google Scholar]
- 5.Stevenson DA, Heinemann J, Angulo M, et al. Gastric rupture and necrosis in Prader-Willi syndrome. J Pediatr Gastroenter Nutr. 2007;45: 272–274. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Klein D, Amman F. The syndrome of Lawrence-Moon-Bardet-Biedl and allied disease in Switzerland. Clinical, genetic, and epidemiological studies. J Neurol Sci. 1969;9:479–513. [DOI] [PubMed] [Google Scholar]
- 7.Moore SJ, Green JS, Fan Y, et al. Clinical and genetic epidemiology of Bardet-Biedl syndrome in Newfoundland: a 22 year prospective population-based cohort study. Am J Med Genet. 2005;132A:352–360. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Daskalakis M, Till H, Kiess W, et al. Roux-en-Y gastric bypass in an adolescent patient with Bardet-Biedl syndrome, a monogenic obesity disorder. Obes Surg. 2010;20:121–125. [DOI] [PubMed] [Google Scholar]
- 9.Chelala E, Cadiere GB, Favretti F, et al. Conversions and complications in 185 laparascopic adjustable silicone gastric banding cases. Surg Endosc. 1997;11:268–271. [DOI] [PubMed] [Google Scholar]
- 10.DePeppo F, DiGiorgio 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. [DOI] [PubMed] [Google Scholar]
- 11.Scheimann AO, Butler MG, Cuffari C, et al. Critical analysis of bariatric procedures in Prader-Willi syndrome. J Pediatr Gastroenter Nutr. 2008;46:80–83. [DOI] [PMC free article] [PubMed] [Google Scholar]