Adenotonsillectomy may help resolve obstructive sleep apnea in children with Prader-Willi syndrome

(Medical Xpress)—Children with Prader-Willi syndrome may receive relief from sleep disorders after undergoing an adenotonsillectomy, suggests a new study from Nationwide Children's Hospital published in the November print issue of the Archives of Otolaryngology-Head and Neck Surgery

" with Prader-Willi syndrome are at risk for as growth hormone commonly used to treat their condition can cause the tonsils and adenoids to enlarge," said the study's lead author Kris Jatana, MD, FAAP, with Otolaryngology Head & Neck Surgery at Nationwide Children's.

" in a low dose is extremely beneficial to most patients with Prader-Willi syndrome," said David Repaske, PhD, MD  co-director of the Prader Willi Center and chief of Endocrinology, Metabolism & Diabetes at Nationwide Children's. "This beneficial effect has nothing to do with growth, but rather, it markedly improves patients' ability to eat, sit, stand, walk or run depending on their developmental stage and due to a positive effect on their low muscle tone."

At Nationwide Children's, Prader-Willi patients undergo an annual study and are evaluated for potential if obstructive apnea events are present. To evaluate the efficacy of adenotonsillectomy in the treatment of in Prader-Willi syndrome, investigators at Nationwide Children's performed a retrospective chart review. Thirteen patients met the study criteria and were categorized based on severe, moderate or mild apnea/hypopnea indexes and obstructive hypoxia.

Findings showed that 89 percent of the patients with mild-to-moderate obstructive sleep apnea or obstructive hypoventilation normalized after receiving adenotonsillectomy. Of the four children with severe obstructive sleep apnea, two normalized after surgery and two continued to have severe apneas.

"These findings suggest that adenotonsillectomy is effective in most children with Prader-Willi syndrome who demonstrate mild to moderate obstructive sleep apnea, but may not be curative in children with severe forms of the condition," said Dr. Jatana, also a faculty member at The Ohio State University College of Medicine.

Dr. Jatana and his team of investigators stress that patients should receive a repeat sleep study six-to-eight weeks postoperatively since an increase in central apneas can occur in some Prader-Willi children after upper airway surgical intervention.

Prader-Willi Syndrome is treated at Nationwide Children's by a multidisciplinary team which emphasizes early intervention and a proactive approach to assessment of and treatment for the possible complications that can be associated with PWS. The team includes an endocrine nurse coordinator, endocrinologists, a geneticist, a genetic counselor and a dietitian that attend each PWS Clinic. The team collaborates with clinicians from a number of supporting disciplines, and patients are referred to Ophthalmology, ENT, Sleep Medicine, Behavioral Health, Urology, Physical/Occupational/Speech Therapy and Dermatology as needed.

Related Stories

Oxytocin promises hope in Prader-Willi syndrome

Jun 24, 2011

Prader-Willi syndrome is a rare genetic disorder which affects one child in 25,000. Children born with this syndrome have a range of complex neurological and developmental problems which continue into adult life. These can ...

A new way of looking at Prader-Willi Syndrome

Nov 15, 2012

An Australian study reveals that people with the rare genetic disorder known as Prader-Willi Syndrome may have an impaired autonomic nervous system. This discovery opens up a new way of looking at the insatiable appetite ...

Updated recommendations for sleep apnea in children

Aug 27, 2012

(HealthDay)—The American Academy of Pediatrics (AAP) is revising its recommendations for the diagnosis and management of obstructive sleep apnea syndrome (OSAS) in children and adolescents, according to ...

Recommended for you

Sleep-disordered breathing linked to functional decline

Dec 06, 2014

(HealthDay)—For older women, sleep-disordered breathing (SDB) is associated with functional decline, according to a study published in the November issue of the Journal of the American Geriatrics Society.

Sleep apnea linked to poor aerobic fitness

Nov 24, 2014

People with moderate to severe obstructive sleep apnea may have an intrinsic inability to burn high amounts of oxygen during strenuous aerobic exercise, according to a new study led by researchers at University ...

Sleep apnea may contribute to kidney disease progression

Nov 14, 2014

Sleep apnea may accelerate kidney function decline in diabetic patients with kidney disease, according to a study that will be presented at ASN Kidney Week 2014 November 11-16 at the Pennsylvania Convention Center in Philadelphia, ...

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.