A Case of Tonsillectomy and Adenoidectomy in Child With Down Syndrome With Obstructive Sleep Apnea

Abstract Introduction Specialized health care guidelines for children with Down Syndrome (DS) published by the American Academy of Pediatrics (AAP) provided specific recommendations based on the higher risk needs of individuals with DS. Obstructive sleep apnea (OSA) is reported to be present in 50–79% of individuals with DS. According to the AAP guideline, all individuals with DS should have a polysomnography (PSG) evaluating for OSA by 4 years old and then screened by history and physical exam annually thereafter. An interim analysis of an ongoing Down Syndrome Research study was evaluated to determine rate of adherence to these guidelines. Methods The Dimensional, Sleep, and Genomic Analyses of Down Syndrome to Elucidate Phenotypic Variability study enrolled down syndrome patients 30 months and older, as well as first degree relatives to participate. Patients completed a standardized clinical sleep interview, childhood sleep habits questionnaire and was asked to complete 2 week sleep diary, actigraphy and polysomnography. We aimed to characterize the rate of PSG completion by 4 years of age, number of research PSGs completed and rate of OSA identified on research PSG. Results A total of 31 patients were consented. The median patient age was 10 years old with a slight female predominance (15F:12M). 27 patients completed the sleep interview and 19 successfully completed a scorable polysomnography. Only 7 patients had completed a PSG previously by age of 4 years. 11 of 19 studies demonstrated obstructive sleep apnea ranging from mild to severe severity (1.7–42.5/hr). REM AHI (range 1.2–58.2/hr, mean 19/hr and median 12.3/hr) demonstrated increased severity. Conclusion Despite AAP guidelines recommending universal PSG evaluation by the age of 4 years of age, only 26% of patients interviewed has a PSG successfully completed previously. Additional recommendations by AAP include yearly surveillance of symptoms although there is poor correlation between parent report and polysomnogram results. Of the 19 research completed PSGs, 58% demonstrated OSA with the mean and median results consistent with moderate to severe OSA and worsening during REM sleep. Improved effort to successfully obtain PSG in this population is needed. Further study is ongoing to evaluate the relationship to other health and cognitive outcomes. Support (if any) NIMH

Download Full-text

Tongue-Based Procedures in Treating Refractory Obstructive Sleep Apnea in Down Syndrome Patients: A Systematic Review and Meta-Analysis

FACE ◽

10.1177/2732501621992447 ◽

2021 ◽

Vol 2 (1) ◽

pp. 65-70

Author(s):

Paul B. Lee ◽

Michael T. Chung ◽

Jared Johnson ◽

Jordyn Lucas ◽

Caitlin R. Priest ◽

...

Keyword(s):

Systematic Review ◽

Obstructive Sleep Apnea ◽

Down Syndrome ◽

Sleep Apnea ◽

Nerve Stimulation ◽

Hypoglossal Nerve ◽

Meta Analysis ◽

Apnea Hypopnea Index ◽

Hypoglossal Nerve Stimulation ◽

Obstructive Sleep

Objective: There is a high prevalence of obstructive sleep apnea (OSA) in pediatric and adult Down Syndrome (DS) patients that is refractory to adenotonsillectomy and continuous positive airway pressure. Newer treatment modalities have emerged with improved outcomes. The objective is to provide an updated systematic review and meta-analysis to analyze the clinical outcomes of OSA in pediatric and adult DS patients with hypoglossal nerve stimulation using Inspire, midline posterior glossectomy plus lingual tonsillectomy (MPG + LT), and combined genioglossus advancement plus radiofrequency (GGS + RF). Methods: A comprehensive literature search of PubMed and Google Scholar was performed followed by a meta-analysis. Studies with preoperative and post-operative Apnea Hypopnea Index (AHI) values were included with patients serving as their own control. Results: Across 5 studies, 56 patients were analyzed. The mean reduction in AHI was statistically significant before vs. after procedure ( P < .001 for hypoglossal nerve stimulation using Inspire with a paired 2-tailed t-test and P = .031 for MPG + LT). Although individual patient AHI values were unavailable in the GGS + RF study, the standard difference in mean AHI was also significant for GGS + RF with P = .001. Device malfunction was the most common complication for Inspire while postoperative bleeding was observed for MPG + LT and nasopharyngeal obstruction and retropalatal collapse were observed for GGS + RF. Conclusion: This review reveals significant improvement in AHI with Inspire, MPG + LT, and GGS + RF for DS patients with refractory OSA. Further investigation is needed for comparison between these 3 therapies.

Download Full-text

0771 Severe Obstructive Sleep Apnea In Children With Down Syndrome

SLEEP ◽

10.1093/sleep/zsy061.770 ◽

2018 ◽

Vol 41 (suppl_1) ◽

pp. A287-A287

Author(s):

J N Mian ◽

B Gunaratnam ◽

E Senthilvel

Keyword(s):

Obstructive Sleep Apnea ◽

Down Syndrome ◽

Sleep Apnea ◽

Severe Obstructive Sleep Apnea ◽

Children With Down Syndrome ◽

Obstructive Sleep

Download Full-text

Treatment Considerations for Obstructive Sleep Apnea in Pediatric Down Syndrome

Children ◽

10.3390/children8111074 ◽

2021 ◽

Vol 8 (11) ◽

pp. 1074

Author(s):

Erica Gastelum ◽

Marcus Cummins ◽

Amitoj Singh ◽

Michael Montoya ◽

Gino Luis Urbano ◽

...

Keyword(s):

Obstructive Sleep Apnea ◽

Down Syndrome ◽

Sleep Apnea ◽

Treatment Guidelines ◽

Negative Impact ◽

Pediatric Population ◽

Healthy Children ◽

Obstructive Sleep ◽

Treatment Considerations ◽

Additional Therapy

Children with Down syndrome (DS) are at high risk for developing obstructive sleep apnea (OSA) compared to children without DS. The negative impact of OSA on health, behavior, and cognitive development in children with DS highlights the importance of timely and effective treatment. Due to the higher prevalence of craniofacial and airway abnormalities, obesity, and hypotonia in patients with DS, residual OSA can still occur after exhausting first-line options. While treatment commonly includes adenotonsillectomy (AT) and continuous positive airway pressure (CPAP) therapy, additional therapy such as medical management and/or adjuvant surgical procedures need to be considered in refractory OSA. Given the significant comorbidities secondary to untreated OSA in children with DS, such as cardiovascular and neurobehavioral consequences, more robust randomized trials in this patient population are needed to produce treatment guidelines separate from those for the general pediatric population of otherwise healthy children with OSA. Further studies are also needed to look at desensitization and optimization of CPAP use in patients with DS and OSA.

Download Full-text

Obstructive Sleep Apnea in Children With Down Syndrome

PEDIATRICS ◽

10.1542/peds.88.1.132 ◽

1991 ◽

Vol 88 (1) ◽

pp. 132-139

Author(s):

Carole L. Marcus ◽

Thomas G. Keens ◽

Daisy B. Bautista ◽

Walter S. von Pechmann ◽

Sally L. Davidson Ward

Keyword(s):

Obstructive Sleep Apnea ◽

Down Syndrome ◽

Sleep Apnea ◽

Arterial Oxygen Saturation ◽

Sleep Apnea Syndrome ◽

Arterial Oxygen ◽

Children With Down Syndrome ◽

Obstructive Sleep ◽

Apnea Syndrome ◽

End Tidal

Children with Down syndrome have many predisposing factors for the obstructive sleep apnea syndrome (OSAS), yet the type and severity of OSAS in this population has not been characterized. Fifty-three subjects with Down syndrome (mean age 7.4 ± 1.2 [SE] years; range 2 weeks to 51 years) were studied. Chest wall movement, heart rate, electrooculogram, end-tidal Po2 and Pco2, transcutaneous Po2 and Pco2, and arterial oxygen saturation were measured during a daytime nap polysomnogram. Sixteen of these children also underwent overnight polysomnography. Nap polysomnograms were abnormal in 77% of children; 45% had obstructive sleep apnea (OSA), 4% had central apnea, and 6% had mixed apneas; 66% had hypoventilation (end-tidal Pco2, >45 mm Hg) and 32% desaturation (arterial oxygen saturation <90%). Overnight studies were abnormal in 100% of children, with OSA in 63%, hypoventilation in 81%, and desaturation in 56%. Nap studies significantly underestimated the presence of abnormalities when compared to overnight polysomnograms. Seventeen (32%) of the children were referred for testing because OSAS was clinically suspected, but there was no clinical suspicion of OSAS in 36 (68%) children. Neither age, obesity, nor the presence of congenital heart disease affected the incidence of OSA, desaturation, or hypoventilation. Polysomnograms improved in all 8 children who underwent tonsilletomy and adenoidectomy, but they normalized in only 3. It is concluded that children with Down syndrome frequently have OSAS, with OSA, hypoxemia, and hypoventilation. Obstructive sleep apnea syndrome is seen frequently in those children in whom it is not clinically suspected. It is speculated that OSAS may contribute to the unexplained pulmonary hypertension seen in children with Down syndrome.

Download Full-text