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Surgery and PWS

Surgery offers hope to children and adolescents with PWS

Credit: Walter Siegmund
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=0.73)

Obesity is a leading cause of complications and death in children suffering from Prader-Willi syndrome (PWS), yet there are few effective treatment options for these patients. In the study. ‘Sleeve Gastrectomy in Children and Adolescents with Prader-Willi Syndrome: A Matched Control Study.’, published in Surgery for Obesity and Related Disease researchers found that bariatric surgery, specifically laparoscopic sleeve gastrectomy (LSG), resulted in substantial weight loss with no apparent adverse effect on growth in a small group of severely overweight patients with PWS. While bariatric surgery is considered controversial for PWS, the research team is encouraged by their positive results.

PWS is a rare genetic condition that causes a wide range of problems including a constant desire to consume food, which is driven by a permanent feeling of hunger. This can easily lead to dangerous weight gain, and in fact, in PWS obesity is a leading cause of death and related problems such as obstructive sleep apnoea, dyslipidaemia, hypertension and diabetes mellitus.

Aayed R Alqahtani

"Questions are raised regarding the safety of bariatric surgery in PWS patients, the degree and sustainability of weight loss and resolution of related health problems, long term results, as well as the effect on growth and skeletal maturity," said lead investigator, Dr Aayed R Alqahtani of the Department of Surgery at King Saud University College of Medicine, Riyadh, Saudi Arabia. “These concerns stem from the fact that the pathophysiology of obesity in those patients is unique and differs from what is observed in the general population.”

The study was carried out at King Saud University College of Medicine, which is an academic centre with a standardised care pathway for paediatric bariatric surgery. Alqahtani and co-investigators examined weight loss and growth after LSG in 24 children and adolescents with PWS aged between five and 18 years old. The outcomes data were then compared with a 1:3 non-PWS group matched for age, gender, and BMI. Data for up to five years follow-up were analysed.

Outcomes

The 24 PWS patients (mean age 10.7; 6<8 yr old, range 4.9–18) had a preoperative BMI46.2±12.2. All PWS patients had obstructive sleep apnoea, 62% had dyslipidaemia, 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=0.2–.7) or growth (p=0.28); 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=0.73).

One PWS patient was readmitted five years after surgery with recurrence of obstructive sleep apnoea 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 five 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.

"Our study indicates that bariatric surgery should be recommended for paediatric PWS patients; our results are unmatched by any other treatment. All of our patients experienced significant weight loss following LSG. There were no deaths or major complications, no significant morbidity, and no slowing of growth," said Alqahtani. "Most of the weight loss occurred within the first two years after surgery and patients successfully reduced food intake and felt satiated by smaller amounts of food due to reduced stomach capacity."

The authors conclude that, “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.”

"Although the use of surgery in pre-adolescents with special needs is uncharted territory, the results are of interest, particularly since there has been very limited experience with modern bariatric procedures in this patient population," said Dr Thomas Inge, professor of surgery and pediatrics from Cincinnati Children's Hospital Medical Center. "While it is clearly not possible to make treatment recommendations for use of surgery in this complex population without further research to examine the physiologic impact, these initial findings should at least prompt a new conversation about prospective and more comprehensive studies to examine safety and efficacy of modern weight loss procedures and newer medications in patients with PWS."

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