Improvement in quality of life after adenotonsillectomy in a child with Prader Willi syndrome

Wong, Chin-Pang; Ng, Daniel; Tracy, M.; Chau, Christy S. K.; Chow, Pok-yu; Kwok, Ka‐li

doi:10.1007/s11325-009-0297-8

Cited by 12 publications

(9 citation statements)

References 8 publications

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“…In general, 74.4% of the studies involved largely behavioural approaches, 13.3% incorporated some 'equipment', 18,57,66,72,77,103,[115][116][117][118]120,123 and 12.2% were surgical approaches. 101,[104][105][106][107][108][109][110]113,114,121 Table I, the alphabetical listing of commonly applied behavioural approaches to problematic paediatric sleeping, shows considerable overlap with the management approaches implemented in a population with developmental disabilities. Apart from sleep ecology management, few approaches were specifically created for children with developmental disabilities.…”

Section: Discussionmentioning

confidence: 99%

Non‐pharmacological management of problematic sleeping in children with developmental disabilities

Spruyt

Curfs

2014

Develop Med Child Neuro

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Modifying sleep management strategies to meet the specific needs of children with developmental disabilities is encouraged, and studies that look beyond sleep quality or sleep quantity are required. It is also advocated that modifications to sleep hygiene, sleep regularity, and sleep ecology in a population with developmental disabilities are rigorously investigated. Finally, daytime somnolence should not be overlooked when aiming to optimize sleep in children with developmental disabilities across the ages and stages of their lives. There were several limitations in the research findings of problematic sleep in children with developmental disabilities. In general, the sleep problems and the developmental disabilities investigated were multicomponent in nature. It is likely that management approaches impacted those problems on multiple levels or through diverse 'therapeutic' pathways. There is a need for randomized controlled trials and more objective measures that quantify improved sleep or wake states.

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Section: Discussionmentioning

confidence: 99%

Non‐pharmacological management of problematic sleeping in children with developmental disabilities

Spruyt

Curfs

2014

Develop Med Child Neuro

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“…There were 4 studies included in this analysis. [37][38][39][40] Seven children had a normal AHI level after AT (41.18%; 2 were normal at baseline). Six children had mild OSA (35.29%), one had moderate OSA (5.88%), and 3 had severe OSA (17.65%) following surgery.…”

Section: Adenotonsillectomy As a Treatment For Osamentioning

confidence: 99%

Prader Willi Syndrome and Obstructive Sleep Apnea: Co-occurrence in the Pediatric Population

Sedky

Bennett

Pumariega

2014

Journal of Clinical Sleep Medicine

127

117

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Background: A high prevalence of obstructive sleep apnea (OSA) occurs in children with Prader-Willi syndrome (PWS). Yet, due in part to the relatively small samples previously used, the prevalence of OSA has varied greatly across studies. It is also unclear if factors such as age, gender, body mass index (BMI), or type of genetic imprinting are associated with increased risk for OSA among children with PWS. Objectives: To evaluate the (a) prevalence of OSA, as well as narcolepsy, in pediatric populations diagnosed with PWS; (b) effects of age, gender, body mass index, and genetic imprinting on OSA severity; and (c) effi cacy of adenotonsillectomy (AT) for decreasing OSA severity in this population. Methods: All studies assessing OSA among children with PWS through August 2013 were identifi ed using the PubMed/Medline, Psych Info, Cochrane library, and Google Scholar data bases. Results: Fourteen studies of children diagnosed with PWS and who were assessed for OSA using polysomnography (PSG) met inclusion criteria (n = 224 children). The prevalence of OSA across studies was 79.91% (n = 179/224). Among youths with OSA, 53.07% had mild OSA, 22.35% moderate OSA, and 24.58% severe OSA. Narcolepsy was found to occur in 35.71% of children with PWS. Adenotonsillectomy was associated with improvement in OSA for most children with PWS. However, residual OSA was present in the majority of cases post-surgery. Conclusion: This study confi rms the high prevalence of OSA and narcolepsy among children with PWS. Screening for OSA and narcolepsy among children with PWS is recommended. In addition, while adenotonsillectomy was effective in reducing OSA for some children, alternative treatments may need to be considered, given the only moderate response rate.

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“…In addition, early treatment improves the outcomes of individuals with PWS [Eiholzer et al, 2001;Fong and De, 2003;Goldstone, 2004;Wong et al, 2010;McCandless, 2011;Cassidy et al, 2012]. Prenatal diagnosis is crucial for families who may decide to terminate the pregnancy if the option is available to them.…”

Section: Introductionmentioning

confidence: 99%

Prader–Willi syndrome can be diagnosed prenatally

Gross

Rabinowitz

Gross‐Tsur

et al. 2014

American J of Med Genetics Pt A

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The aim of this study was to characterize the fetal phenotype of a cohort of individuals with confirmed diagnoses of Prader-Willi syndrome (PWS), a severe multi-system genetic disorder, diagnosed by a specific methylation test. We interviewed mothers of 106 individuals with PWS to obtain information about the pregnancy of their affected child. For 47 pregnancies of children younger than 10 years, we also reviewed the obstetric ultrasound and detailed obstetric history from medical records. We compared the PWS pregnancies with those of the sibling closest in age and with the general population. McNemars, Chi-square and Fisher exact tests were used for statistical analyses. Decreased fetal movements, small for gestational age (SGA), asymmetrical intrauterine growth (increased head/abdomen circumferences ratio) and polyhydramnios were found in 88%, 65%, 43%, and 34%, respectively (P < 0.001 vs. siblings and P < 0.0001 vs. the general population for all measurements). No severe morphological abnormalities were found. A combination of 2, 3, and 4 abnormalities was found in 27%, 29%, and 24% of pregnancies, respectively. Fourteen out of 15 umbilical artery Doppler studies were within the normal range (93%). The rare combination of asymmetrical intrauterine growth and polyhydramnios was found in 34% of PWS pregnancies (P < 0.0001 vs. the general population). Prenatal genetic screening for PWS by methylation testing is indicated when any combination of polyhydramnios, SGA or asymmetric intrauterine growth, with normal Doppler studies is present, particularly when asymmetrical intrauterine growth and polyhydramnios coexist.

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Improvement in quality of life after adenotonsillectomy in a child with Prader Willi syndrome

Cited by 12 publications

References 8 publications

Non‐pharmacological management of problematic sleeping in children with developmental disabilities

Non‐pharmacological management of problematic sleeping in children with developmental disabilities

Prader Willi Syndrome and Obstructive Sleep Apnea: Co-occurrence in the Pediatric Population

Prader–Willi syndrome can be diagnosed prenatally

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