Effectiveness of Adenotonsillectomy and Risk of Velopharyngeal Insufficiency in Children With Prader-Willi Syndrome

Padia, Reema; Muntz, Harlan R.; Pfeffer, Kathleen D.

doi:10.1177/0003489417729834

Cited by 9 publications

(32 citation statements)

References 21 publications

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“…Only one patient had an adenotonsillectomy between the first and second polygraph, her AHI worsening from 1.5 to 11.5 after surgery. This finding suggests that adenotonsillectomy may be less successful in SRS patients than in the general population, as already reported for PWS patients (29)(30)(31). Furthermore, an ENT intervention concerning tonsils in SRS patients can result in a voice change and worsen a preexisting high-pitched voice, quite common in these patients.…”

Section: Discussionsupporting

confidence: 51%

Sleep disordered breathing in Silver−Russell syndrome patients: a new outcome

et al. 2019

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Context: Imprinting disorders (ID), such as Prader-Willi syndrome (PWS), are associated with sleepdisordered breathing (SDB). No data are available for Silver-Russell syndrome (SRS), another ID which shares clinical features with PWS, although many patients describe excessive daytime sleepiness, disturbed sleep, and snoring. Objective: to characterize sleep in children with SRS and evaluate the impact of recombinant growth hormone (rGH) therapy. Patients and Methods: we performed a retrospective analysis of sleep recordings in 40 patients with molecularly-proven SRS (methylation anomaly in 11p15 (n=32) or maternal uniparental disomy of chromosome 7 (n=16)). Sleep recordings were either polygraphy or polysomnography (n=16). 34 patients received rGH therapy. Results: We collected 61 sleep recordings. The mean apnea-hypopnea index (AHI) was 3.4 events/hour (0-12.4), with a mean central AHI of 0.5 events/hour (0-2.4). SDB was identified in 73.8% (n=45) of the recordings and was severe in 4.9%. SDB was present in 86.4% of patients before rGH therapy and was severe in 13.6%. AHI worsened for five of 12 patients with sleep recordings before and after rGH therapy initiation, reaching mild impairment. The mean rGH dose was 32.3µg/kg/day (12.9-51.4), with a mean IGF-I plasma levels of 1.7 SDS (-1.9-6.6). Conclusion: Most patients with SRS present SDB with an obstructive profile, possibly explained by narrowing of the airways and lymphoid organ hypertrophy. We recommend systematic ear-nose-throat evaluation of SRS patients and polysomnography if there are clinical anomalies, preferably before initiating rGH therapy, to monitor and adapt the management of patients with SDB.

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Section: Discussionsupporting

confidence: 51%

Sleep disordered breathing in Silver−Russell syndrome patients: a new outcome

et al. 2019

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“…A systematic review showed that A&T was effective in reducing OSA for some PWS children, but alternative treatments may be considered, given the only moderate response rate (59). OAHI decreased after A&T in 22 children, but a significant number had persistent OSA (79). Velopharyngeal dysfunctions (VPD) may occur after A&T (80).…”

Section: Prader-willi Syndromementioning

confidence: 99%

Sleep-disordered breathing in paediatric setting: existing and upcoming of the genetic disorders

Zaffanello¹,

Antoniazzi²,

Tenero³

et al. 2018

Ann. Transl. Med.

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Childhood obstructive sleep apnea syndrome (OSAS) is characterized by anatomical and functional upper airway abnormalities as pathophysiological determinants, and clinical symptoms are frequently clear. OSAS is widely described in rare genetic disorders, such as achondroplasia, Down syndrome, Prader-Willi syndrome, Pierre Robin sequence, and mucopolysaccharidosis. Craniofacial and upper airway involvement is frequently morbid conditions. In children with genetic diseases, the clinical symptoms of OSAS are often slight or absent, and related morbidities are usually more severe and can be observed at any age. The present review is aimed to updating the discoveries regarding OSAS on Achondroplasia, Down syndrome, Prader-Willi syndrome, Pierre Robin sequence, Sickle cell disease, or encountered in our clinical practice (Ehlers-Danlos syndrome, Ellis-van Creveld syndrome, Noonan syndrome). Two additional groups of genetic disorders will be focused (mucopolysaccharidoses and osteogenesis imperfecta). The flowing items are covered for each disease: (I) what is the pathophysiology of OSAS? (II) What is the incidence/prevalence of OSAS? (III) What result from the management and prognosis? (IV) What are the recommendations? Considering the worries of OSAS, such as inattention and behavioural problems, daytime sleepiness, failure to thrive, cardiological and metabolic complications, the benefit of a widespread screening and the treatment in children with genetic diseases is undoubtful. The goals of the further efforts can be the inclusion of various genetic diseases into guidelines for the screening of OSAS, updating the shreds of evidence based on the research progression.

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“…Hypotonia, facial dysmorphic features, obesity, and adenotonsillar hypertrophy may be the possible causes of OSA in children with PWS. 10,16,17,43,49,52…”

Section: Discussionmentioning

confidence: 99%

“…All 6 studies reported the data for AHI changes postoperatively. 43,44,[46][47][48][49] The AHI results were 13.1 events/h (95% CI, 11.0-15.1) before surgery and 4.6 events/h (95% CI, 4.1-5.1) after surgery. Overall, the mean change in postoperative AHI was a reduction of 8.0 events/ h (95% CI, 210.8 to 25.1; Figure 2) and was statistically significant.…”

Section: Outcomes Of Surgery In Children With Pwsmentioning

confidence: 99%

“…Hypotonia, facial dysmorphic features, obesity, and adenotonsillar hypertrophy may be the possible causes of OSA in children with PWS. 10,16,17,43,49,52 The prevalence of PWS has been 1:10,000 to 1:30,000 among live children. 4,5 However, this meta-analysis identified only 46 children with PWS who received T&A and had undergone PSG before and after the surgery.…”

Section: Association Between Osa and Pws In Childrenmentioning

confidence: 99%

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Adenotonsillectomy for the Treatment of Obstructive Sleep Apnea in Children with Prader‐Willi Syndrome: A Meta‐analysis

Lee

Hsu

et al. 2019

Otolaryngol.--head neck surg.

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Objective Adenotonsillectomy outcomes in obstructive sleep apnea (OSA) treatment among children with Prader-Willi syndrome (PWS) remain unclear. This study aimed to elucidate the effectiveness of adenotonsillectomy in OSA treatment among children with PWS. Data Source PubMed, MEDLINE, Embase, and Cochrane Review up to February 2019. Review Methods The registry number of the protocol published on PROSPERO was CRD42015027053. Two authors independently searched the relevant database. Polysomnography outcomes in these children were examined, including net postoperative changes in the apnea-hypopnea index (AHI), net postoperative changes in the minimum and mean oxygen saturation, the overall success rate for a postoperative AHI <1, and the overall success rate for a postoperative AHI <5. Results Six studies with 41 patients were analyzed (mean age, 5.0 years; 55% boys; mean sample size, 6.8 patients). All children had PWS and received adenotonsillectomy for the treatment of OSA. The AHI was 13.1 events per hour (95% CI, 11.0-15.1) before surgery and 4.6 events per hour (95% CI, 4.1-5.1) after surgery. The mean change in the AHI was a significant reduction of 8.0 events per hour (95% CI, −10.8 to −5.1). The overall success rate was 21% (95% CI, 11%-38%) for a postoperative AHI <1 and 71% (95% CI, 54%-83%) for a postoperative AHI <5. Some patients developed velopharyngeal insufficiency postoperatively. Conclusion Adenotonsillectomy was associated with OSA improvement among children with PWS. However, residual OSA was frequently observed postoperatively in these patients.

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Effectiveness of Adenotonsillectomy and Risk of Velopharyngeal Insufficiency in Children With Prader-Willi Syndrome

Cited by 9 publications

References 21 publications

Sleep disordered breathing in Silver−Russell syndrome patients: a new outcome

Sleep disordered breathing in Silver−Russell syndrome patients: a new outcome

Sleep-disordered breathing in paediatric setting: existing and upcoming of the genetic disorders

Adenotonsillectomy for the Treatment of Obstructive Sleep Apnea in Children with Prader‐Willi Syndrome: A Meta‐analysis

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