Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea

Diercks, Gillian R.; Keamy, Donald G.; Kinane, T. Bernard; Skotko, Brian G.; Schwartz, Allen D.; Grealish, Ellen; Dobrowski, John M.; Soose, Ryan J.; Hartnick, Christopher J.

doi:10.1542/peds.2015-3663

Cited by 43 publications

(30 citation statements)

References 12 publications

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“…It may be that even a moderate surgical success rate is viewed as better than suboptimal or minimal use of PAP. Given the concerns for the effectiveness of such surgeries for improving sleep in children with DS, it is encouraging that research continues to assess, refine and improve surgical procedures for children with DS and OSA, using refined surgical procedures or novel tools, such as a hypoglossal nerve stimulator 33,34 . These research efforts are needed to continue to assess and improve surgical outcomes, as are research to improve PAP tolerance and adherence in this population.…”

Section: Discussionmentioning

confidence: 99%

Use of Sleep Evaluations and Treatments in Children with Down Syndrome

Esbensen

Beebe

Byars

et al. 2016

J Dev Behav Pediatr

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Objective To characterize practice patterns regarding sleep evaluation and intervention among children with Down syndrome (DS). Method Data were obtained from electronic health records from 2009–2013 for a retrospective cohort of 954 children with DS, ages 5–21 years during the time sampled. ICD-9 diagnoses were used to identify children with obstructive sleep apnea and/or behavioral sleep disturbances. Primary outcomes were confirmed by participation in an overnight diagnostic polysomnography (PSG) and/or documented provision of specified sleep interventions including positive airway pressure, otolaryngology (ENT) surgery, sleep medication and behavioral sleep therapy. Results Overall, 47.7% of children with DS had undergone PSG, 39.1% had diagnosed sleep problems, and of those diagnosed with sleep problems, 81.2% had received sleep intervention. Consistent with best practice clinical care, sleep treatments matched the diagnosed sleep problems. Age, gender, and race, but not body mass index, were associated with PSG completion rate and occurrence rates for ENT surgery and sleep medication usage. Body mass index was associated with obstructive sleep apnea. Conclusion Despite high rates of reported sleep problems in children with DS, less than half underwent PSG. Children diagnosed with sleep problems received treatment consistent with their sleep diagnosis. However, age and gender were associated with differential rates of treatment delivery that was incongruous with prevalence rates for diagnosed sleep problems. These findings underscore the importance of screening for sleep problems in children with DS, and referring for and providing appropriate targeted sleep interventions.

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Section: Discussionmentioning

confidence: 99%

Use of Sleep Evaluations and Treatments in Children with Down Syndrome

Esbensen

Beebe

Byars

et al. 2016

J Dev Behav Pediatr

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“…There is often persistence of disease following tonsillectomy and adenoidectomy, as well as frequent noncompliance with positive pressure ventilation . As a result, an Food and Drug Administration (FDA)‐approved trial was organized to evaluate the safety and efficacy of UAS in the pediatric DS population (http://ClinicalTrials.gov NCT02344108) . Implantation in the first patient was well tolerated, with significant improvement in OSA severity and eventual tracheostomy decannulation …”

Section: Introductionmentioning

confidence: 99%

“…The pediatric population presents a greater variety in body habitus, including the size of the thorax. Previously, stimulator placement has been performed through three incisions . One neck incision is made for placement of the hypoglossal nerve stimulation lead, and the implantable pulse generator (IPG) and pleural sensing lead are placed through two separate chest incisions.…”

Section: Introductionmentioning

confidence: 99%

Modified surgical approach to hypoglossal nerve stimulator implantation in the pediatric population

2017

Self Cite

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Upper airway stimulation with the hypoglossal nerve stimulator is a promising treatment modality for severe obstructive sleep apnea in carefully selected patients with Down syndrome. The pediatric population presents a greater variety in body habitus, including thorax size. A modified surgical approach, utilizing a medially placed, single chest incision, instead of two separate chest incisions, provides an alternative that is particularly useful for pediatric patients with small stature. As this technology is evaluated for Food and Drug Administration clearance in the pediatric population, it is important to consider modifications in surgical technique, partnering prior surgical experience with the technical support of company representatives. Laryngoscope, 128:1490-1492, 2018.

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“…Ear-nose-throat (ENT) surgeons have been reported to be the number one specialty that follows individuals with CHARGE syndrome throughout their life, due to the high prevalence of head and neck dysfunction Hartshorne et al, 2016). A recent case report described the success of an implanted hypoglossal nerve stimulator to treat refractory severe obstructive sleep apnea in an adolescent with Down syndrome (Diercks et al, 2016). This has not yet been described in CHARGE syndrome, but has great potential due to the high prevalence of cranial nerve dysfunction (see section below).…”

Section: Craniofacial Anomaliesmentioning

confidence: 99%

Gastrointestinal and feeding difficulties in CHARGE syndrome: A review from head‐to‐toe

Blake

Hudson

2017

American J of Med Genetics Pt C

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CHARGE syndrome is an autosomal dominant genetic condition that is primarily diagnosed based on clinical features, with genetic testing available for confirmation.The CHARGE mnemonic stands for some of the common characteristics: coloboma, heart defects, atresia/stenosis of the choanae, retardation of growth/development, genitourinary anomalies, and ear abnormalities (CHARGE). However, many of the common clinical features are not captured by this mnemonic, including cranial nerve dysfunction, considered by some to be one of the major diagnostic criteria. Over 90% of individuals experience feeding and gastrointestinal dysfunction, which carries great morbidity and mortality. The aim of this review is to examine the nature of gastrointestinal (GI) symptoms and feeding difficulties in CHARGE syndrome, focusing on their underlying pathology, associated investigations, and available treatment options. We also provide information on available tools (for parents, clinicians, and researchers) that are important additions to the lifelong healthcare management of every individual with CHARGE syndrome. We review how cranial nerve dysfunction is one of the most important characteristics underlying the pervasive GI and feeding dysfunction, and discuss the need for future research on gut innervation and motility in this genetic disorder. trachea-eosophageal fistula, developmental delay) contribute directly to feeding and GI issues, highlighting the pervasiveness of these dysfunctions (Blake et al., 1998;Verloes, 2005). The structural anomalies, motor impairment, and oral sensory impairment all contribute to these issues, and are all potential treatment targets.The aim of this review is to comprehensively summarize the existing literature on the nature of GI and feeding dysfunction in CHARGE syndrome, including the pathophysiology, symptomology, and treatment options. | METHODSAn electronic systematic search was conducted via PubMed using the following search terms: CHARGE syndrome and GI, feeding, reflux, or cranial nerve. There were no restrictions placed on article type or year of publication. Titles and abstracts were reviewed for relevance.Reference lists of identified articles were reviewed for inclusion of any additional relevant articles. The articles were summarized and divided into type of feeding or GI problem, from mouth to anus. The original list of articles was then reappraised to identify any missing topics. | RESULTSNinety-seven articles were identified using the search strategy. Titles and abstracts were screened for relevance to the topic and any duplicates, which resulted in the exclusion of 49 articles. The remaining 48 articles' reference lists were reviewed for additional relevant articles. A final total of 71 articles were included for review. Their findings are summarized below, from head-to-toe. Table 1 displays a summary of the phenotypic characteristics of CHARGE syndrome, FIGURE 1 A highlight of the multitude of factors contributing to gastrointestinal and feeding dysfunction in CHARGE syndrome FIGU...

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Hypoglossal Nerve Stimulator Implantation in an Adolescent With Down Syndrome and Sleep Apnea

Cited by 43 publications

References 12 publications

Use of Sleep Evaluations and Treatments in Children with Down Syndrome

Use of Sleep Evaluations and Treatments in Children with Down Syndrome

Modified surgical approach to hypoglossal nerve stimulator implantation in the pediatric population

Gastrointestinal and feeding difficulties in CHARGE syndrome: A review from head‐to‐toe

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