Pediatric Postoperative Outcomes for Severe and Very Severe Obstructive Sleep Apnea Syndrome.

OBJECTIVE: To determine if increasing obstructive sleep apnea syndrome (OSAS) severity, as determined by preoperative polysomnography data, is an independent risk for respiratory complications and level of follow-up care after adenotonsillectomy or tonsillectomy. METHODS: A retrospective analysis of patients ≤21 years of age with severe OSAS (obstructive apnea-hypopnea index [OAHI] >10) undergoing adenotonsillectomy or tonsillectomy. Patients were categorized based on preoperative polysomnography data (PSG). Outcome measures including respiratory complications were collected via chart review. Logistic regression was used in the analysis of all parameters, and Wilcoxon Rank Sum tests were used for analysis of both OAHI and oxygen saturation nadir as continuous variables. All surgeries were performed at Johns Hopkins Hospital, a tertiary care center. RESULTS: We identified 358 patients with severe OSAS who had adenotonsillectomy or tonsillectomy. OAHI >40 and oxygen saturation nadir <80% were significantly associated with postoperative respiratory complications. Increasing OAHI and O2 saturation <80% was each associated with unplanned continuous positive airway pressure (CPAP) initiations postoperatively. There was no association between hypercarbia and presence of any complications. CONCLUSION: Patients with very severe OSAS (preoperative OAHI ≥40) as determined by preoperative PSG may be at higher risk of developing respiratory complications postoperatively. However, there does not appear to be a linear association with increasing severity of OAHI on regression analysis. Further research is needed to understand factors associated with complications in severe and very severe OAHI. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

Promoting a Sleep-friendly Environment by Minimizing Overnight Room Entries.

Introduction: Despite its importance in illness recovery, the sleep of hospitalized children is frequently interrupted. This quality improvement intervention aimed to reduce overnight room entries by minimizing unnecessary interventions. Methods: This study occurred at a university-affiliated children's hospital on the hospital medicine services from March 26, 2021, to April 14, 2022. The intervention included order set changes and the implementation of a rounding checklist designed to address factors most closely associated with sleep disruption and overnight room entries. The outcome measure was overnight (10 pm to 6 am) room entries, counted using room entry sensors. Process measures reflected the intervention targets (overnight vital sign orders, medication administration, and intravenous fluid use). The method of analysis was statistical process control charting. Results: After identifying special cause variation, the average number of overnight room entries decreased from 8.1 to 6.8, a 16% decrease. This decrease corresponded with the implementation of a rounding checklist. However, there continued to be variability in average room entries, suggesting a process lacking ongoing stability. During this period, avoidance of overnight medications and intravenous fluid increased by 28% and 17%, respectively. Conclusions: Implementing a rounding checklist to a broad patient population decreased overnight room entries. However, future work is needed to better understand the factors associated with sustaining such an improvement.

Obstructive sleep apnea screening in children with asthma.

RATIONALE: Obstructive sleep apnea is highly prevalent in children with asthma, particularly in obese children. The sleep-related breathing disorder screening questionnaire has low screening accuracy for obstructive sleep apnea in children with asthma. Our goal was to identify the questions on the sleep-related breathing disorder survey associated with obstructive sleep apnea in children with asthma. METHODS: Participants completed the survey, underwent polysomnography and their body mass index z-score was measured. Participants with survey scores above 0.33 were considered high risk for obstructive sleep apnea and those with an apnea-hypopnea index ≥ 2 events/h classified as having obstructive sleep apnea. Logistic regression was used to examine the association of each survey question and obstructive sleep apnea. Positive and negative predictive values were calculated to estimate screening accuracy. RESULTS: The prevalence of obstructive sleep apnea was 40% in our sample (n = 136). Loud snoring, morning dry mouth, and being overweight were the survey questions associated with obstructive sleep apnea. The composite survey score obtained from all 22 questions had positive and negative predictive values of 51.0% and 65.5%, while the combined model of loud snoring, morning dry mouth, and being overweight had positive and negative predictive values of 60.3% and 77.6%. On the other hand, the body mass index z-score alone had positive and negative predictive values of 76.3% and 72.2%. CONCLUSIONS: The body mass index z-score is useful for obstructive sleep apnea screening in children with asthma and should be applied routinely given its simplicity and concerns that obstructive sleep apnea may contribute to asthma morbidity.

A Trial of Intranasal Corticosteroids to Treat Childhood OSA Syndrome.

BACKGROUND: Intranasal corticosteroids (INCS) are frequently used to treat OSA syndrome (OSAS) in children. However, their efficacy has not been rigorously tested. RESEARCH QUESTION: Do INCS result in improved OSAS symptoms, polysomnography findings, behavior, and quality of life compared with placebo? STUDY DESIGN AND METHODS: In this randomized, double-blind, placebo-controlled trial, children with OSAS aged 5 to 12 years (N = 134) were randomized 2:1 to receive 3 months of INCS or placebo. Children in the INCS arm were then re-randomized to receive 9 months of INCS or placebo. Polysomnography, symptoms, and neurobehavioral findings were measured at baseline, 3 months, and 12 months. The primary outcome was change in obstructive apnea hypopnea index (OAHI) at 3 months, available for 122 children. The secondary outcome was OAHI change at 12 months, available for 70 children. RESULTS: Median (interquartile range) age and OAHI at baseline for the entire group were 7.9 (6.3 to 9.9) years and 5.8 (3.6 to 9.7) events per hour. OAHI changes at 3 months (-1.72 [-3.91 to 1.92] events per hour) and 12 months (-1.2 [-4.22 to 1.71] events per hour) were not different between the two groups (P = not significant). OSAS symptoms and neurobehavioral results did not differ between the INCS and placebo groups at 3 and 12 months. The 38 children who received INCS for 12 months reported a significant OAHI decrease from 7.2 (3.62 to 9.88) events per hour to 3.7 (1.56 to 6.4) events per hour (P = .039). INTERPRETATION: In children with OSAS, treatment with INCS did not result in significant polysomnography, neurobehavioral, or symptom changes at 3 and 12 months of treatment. Twelve months of INCS treatment resulted in a statistically significant but not clinically relevant OAHI reduction. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02180672; URL: www. CLINICALTRIALS: gov.

Cardiopulmonary Testing before Pediatric Adenotonsillectomy for Severe and Very Severe Obstructive Sleep Apnea Syndrome.

OBJECTIVES/HYPOTHESIS: Adenotonsillectomy is first-line treatment for pediatric obstructive sleep apnea syndrome (OSAS) when not otherwise contraindicated. There is concern severe OSAS increases risk of comorbid cardiopulmonary abnormalities, such as ventricular hypertrophy or pulmonary hypertension, which preoperative testing could detect. Our objective is to determine if there is a severity of pediatric OSAS where previously undetected cardiopulmonary comorbidities are likely. STUDY DESIGN: Retrospective chart review. METHODS: We performed a retrospective review of 358 patients ≤21 years with severe OSAS who underwent adenotonsillectomy at a tertiary hospital June 1, 2016 to June 1, 2018. We extracted demographics, comorbidities, polysomnography, and preoperative tests. Wilcoxon rank-sum and logistic regression estimated associations of OSAS severity (based on obstructive apnea-hypopnea index [OAHI], hypoxia, hypercarbia) with preoperative echocardiograms and chest X-rays (CXRs). RESULTS: Mean age was 5.9 (±3.6) years and 52% were male. Mean OAHI and oxygen saturation nadir were 30.3 (±23.8) and 80.7% (±9.2), respectively. OAHI ≥60 was associated with having a preoperative echocardiogram (OR, 3.8; 95% CI, 1.7-8.5) or CXR (OR, 3.0; 95% CI, 1.4-6.8) compared to OAHI 10-59. There were no significant associations between OSAS severity and test abnormalities. The presence of previously diagnosed cardiopulmonary comorbidities was associated with abnormalities on echocardiogram (OR, 36; 95% CI, 4.1-320.1) and CXR (OR, 4.1; 95% CI, 1.2-14.4). CONCLUSIONS: Although pediatric patients with very severe OSAS (OAHI ≥60) underwent more pre-adenotonsillectomy cardiopulmonary tests, OSAS severity did not predict abnormal findings. Known cardiopulmonary comorbidities may be a better indication for cardiopulmonary testing than polysomnographic parameters, which could streamline pre-adenotonsillectomy evaluation and reduce cost. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2361-2368, 2021.

Best practice guidelines in managing the craniofacial aspects of skeletal dysplasia.

BACKGROUND: Recognition and appropriate management of the craniofacial manifestations of patients with skeletal dysplasia are challenging, due to the rarity of these conditions, and dearth of literature to support evidence-based clinical decision making. METHODS: Using the Delphi method, an international, multi-disciplinary group of individuals, with significant experience in the care of patients with skeletal dysplasia, convened to develop multi-disciplinary, best practice guidelines in the management of craniofacial aspects of these patients. RESULTS: After a comprehensive literature review, 23 initial statements were generated and critically discussed, with subsequent development of a list of 22 best practice guidelines after a second round voting. CONCLUSIONS: The guidelines are presented and discussed to provide context and assistance for clinicians in their decision making in this important and challenging component of care for patients with skeletal dysplasia, in order standardize care and improve outcomes.

Laryngoscopy Grade Improvement and Difficult Airway Resolution in Infants With Robin Sequence Undergoing Mandibular Distraction Osteogenesis: A Multi-Institutional Study.

OBJECTIVE: Mandibular distraction osteogenesis (MDO) aims to relieve tongue-based airway obstruction in Robin Sequence (RS). We investigated direct laryngoscopy grade (DLG) improvement and difficult airway (DA) resolution following MDO. DESIGN: Retrospective cohort analysis. SETTING: Three tertiary care institutions. PATIENTS: Sixty-four infants with RS who underwent a single MDO procedure in their first year of life were identified from January 2010 to January 2019. MAIN OUTCOME MEASURES: The primary outcome was DLG pre- and post-MDO. Secondary outcomes included DA designation, pre- and post-MDO polysomnographic assessment for obstructive sleep apnea (OSA), length of stay, need for gastrostomy, and major/minor adverse events. RESULTS: Median DLG improved from II pre-MDO to I at the time of distractor removal (n = 43, P < .001). No significant change was seen in patients with a third recorded time point (eg, palatoplasty; n = 78, P = .52). Twenty-six (47%) of 55 patients were designated as DA pre-MDO, and 10 (18%) of 55 patients retained the label post-MDO (P < .01). Five (50%) of these 10 patients appeared to be inappropriately retained. Median obstructive apnea-hypopnea index improved from 38.6 (range 31.2-62.8) pre-MDO to 2.9 (range 1-3.9) post-MDO (n = 12; P = .002). CONCLUSION: Mandibular distraction osteogenesis allowed for DLG improvement that was stably maintained as well as functional improvement in OSA, with minimal morbidity. Difficult airway designation persisted in the electronic record of some infants despite clinical resolution.

Outcomes of Adenotonsillectomy for Obstructive Sleep Apnea in Prader-Willi Syndrome: Systematic Review and Meta-analysis.

OBJECTIVES: Prader-Willi syndrome (PWS) increases the risk of obstructive sleep apnea (OSA) due to obesity, hypotonia, and abnormal ventilatory responses. We evaluated post-adenotonsillectomy complications, polysomnography changes, and quality of life in children with OSA and PWS. STUDY DESIGN: Systematic review and meta-analysis. METHODS: We conducted a systematic review and meta-analysis by searching PubMed, Embase, Cochrane, Web of Science, and Scopus. Two researchers independently reviewed studies about adenotonsillectomy for OSA in patients <21 years with PWS. We extracted study design, patient numbers, age, complications, polysomnography, and quality of life. We pooled postoperative changes in apnea hypopnea index (AHI) for meta-analysis. We applied Methodological Index for Nonrandomized Studies (MINORS) criteria to assess study quality. RESULTS: The initial search yielded 169 studies. We included 68 patients from eight studies with moderate to high risk of bias. Six studies reported on complications and 12 of 51 patients (24%) had at least one. Velopharyngeal insufficiency was the most commonly reported complication (7/51, 14%). We included seven studies in meta-analysis. Mean postoperative improvement in AHI was 7.7 (95% CI: 4.9-10.5). Postoperatively 20% (95% CI: 3%-43%) had resolution of OSA with AHI < 1.5 while 67% (95% CI: 50%-82%) had improvement from severe/moderate OSA to mild/resolved (AHI < 5). Two studies evaluated quality of life and demonstrated improvement. CONCLUSIONS: Children with PWS undergoing adenotonsillectomy for OSA have a substantial risk of postoperative complications that may require additional interventions, especially velopharyngeal insufficiency. Despite improvements in polysomnography and quality of life, many patients had residual OSA. This information can be used to counsel families when considering OSA treatment options. Laryngoscope, 131:898-906, 2021.

Predicting polysomnographic severity thresholds in children using machine learning.

BACKGROUND: Approximately 500,000 children undergo tonsillectomy and adenoidectomy (T&A) annually for treatment of obstructive sleep disordered breathing (oSDB). Although polysomnography is beneficial for preoperative risk stratification in these children, its expanded use is limited by the associated costs and resources needed. Therefore, we used machine learning and data from potentially wearable sensors to identify children needing postoperative overnight monitoring based on the polysomnographic severity of oSDB. METHODS: Children aged 2-17 years undergoing polysomnography were included. Six machine learning models were created using (i) clinical parameters and (ii) nocturnal actigraphy and oxygen desaturation index. The prediction performance for polysomnography-derived severity of oSDB measured by apnea hypopnea index (AHI) >2 and >10 were evaluated. RESULTS: One hundred and ninety children were included. One hundred and eight were male (57%), mean age was 6.7 years [95% confidence interval; 6.1, 7.2], and mean AHI was 10.6 [7.8, 13.4]. Predictive performance utilizing clinical parameters was poor for both AHI > 2 (accuracy range: 48-56% for all models) and AHI > 10 (50-61%). Combining oximetry and actigraphy improved the accuracy to 87-89% for AHI > 2 and 95-96% for AHI > 10. CONCLUSIONS: Machine learning with oximetry and actigraphy identifies most children needing overnight monitoring as determined by polysomnographic severity of oSDB, supporting a potential resource-conscious screening pathway for children undergoing T&A. IMPACT: We provide proof of principle for the utility of machine learning, oximetry, and actigraphy to screen for severe obstructive sleep apnea syndrome (OSAS) in children. Clinical parameters perform poorly in predicting the severity of OSAS, which is confirmed in the current study. The predictive accuracy for severe OSAS was improved by a smaller subset of quantifiable physiologic parameters, such as oximetry. The results of this study support a lower cost, patient-friendly screening pathway to identify children in need of in-hospital observation after surgery.

Sleep disordered breathing in Marfan syndrome: Value of standard screening questionnaires.

BACKGROUND: A high prevalence of sleep disordered breathing (SDB) has been reported in persons with Marfan syndrome (MFS), a single gene disorder of connective tissue resulting in premature death from aortic rupture. The burden of SDB and accompanying hemodynamic stress could warrant broad screening in this population. Our goal was to assess the utility of traditional SDB screening tools in our sample of persons with MFS. METHODS: Participants were recruited during an annual Marfan Foundation meeting and Marfan status confirmed using the Ghent criteria. Screening questionnaires were administered and SDB assessed by home sleep testing. We assessed accuracy of screening tools using receiver-operating characteristic curve analyses. RESULTS: The prevalence of moderate-severe SDB was 32% in our sample of 31 MFS participants. The Stop-Bang questionnaire had the highest positive predictive value (PPV) of 60% and the highest negative predictive value (NPV) of 100% using the high- and moderate-risk cut-offs, respectively, and the Berlin questionnaire had a PPV of 50% and an NPV of 92.3% at the high-risk cut-off. When those with mild SDB were included, the Stop-Bang and the Sleep Apnea Clinical Score (SACS) questionnaires demonstrated useful screening accuracies with PPVs of 94.7% and 92.9%, and NPVs of 63.6% and 47.1%, respectively, at the moderate-risk cut-offs. CONCLUSION: A survey of SDB in a sample of persons with MFS reveals not only a high burden of SDB but also that conventional screening instruments have utility if adapted appropriately. Future studies should validate the utility of these screening tools given concerns that SDB may contribute to progression of aortic pathology in MFS.

The association of obstructive sleep apnea and left ventricular hypertrophy in obese and overweight children with history of elevated blood pressure.

Obesity is a potent cardiovascular disease (CVD) risk factor and is associated with left ventricular hypertrophy (LVH). Obstructive sleep apnea (OSA) is common among individuals with obesity and is also associated with CVD risk. The authors sought to determine the association of OSA, a modifiable CVD risk factor, with LVH among overweight/obese youth with elevated blood pressure (EBP). This was a cross-sectional analysis of the baseline visit of 61 consecutive overweight/obese children with history of EBP who were evaluated in a pediatric obesity hypertension clinic. OSA was defined via sleep study or validated questionnaire. Children with and without OSA were compared using Fisher's exact tests, Student's t tests, and Wilcoxon rank sum test. Multivariable logistic regression evaluated the association between OSA and LVH. In this cohort, 71.7% of the children had LVH. Children with OSA were more likely to have LVH (85.7% vs 59.4%, P = 0.047). OSA was associated with 4.11 times greater odds of LVH (95% CI 1.15, 14.65; P = 0.030), remaining significant after adjustment for age, sex, race, and BMI z-score (after adjustment for hypertension, P = 0.051). A severe obstructive apnea-hypopnea index (AHI >10) was associated with 14 times greater odds of LVH (95% CI 1.14, 172.64, P = 0.039). OSA was significantly associated with LVH among overweight/obese youth with EBP, even after adjustment for age, sex, race, and BMI z-score. Those with the most severe OSA (AHI >10) had the greatest risk for LVH. Future studies exploring the impact of OSA treatment on CVD risk in children are needed.

Sleep disordered breathing in bronchopulmonary dysplasia.

BACKGROUND: There are limited data on the effect of bronchopulmonary dysplasia (BPD) on sleep disordered breathing (SDB). We hypothesized that both the severity of prematurity and BPD would increase the likelihood of SDB in early childhood. Our secondary aim was to evaluate the association of demographic factors on the development of SDB. METHODS: This is a retrospective study of patient factors and overnight polysomnogram (PSG) data of children enrolled in our BPD registry between 2008 and 2015. Association between PSG results and studied variables was assessed using multiple linear regression analysis. RESULTS: One-hundred-forty children underwent at least one sleep study on room air. The mean respiratory disturbance index (RDI) was elevated at 9.9 events/hr (SD: 10.1). The mean obstructive apnea-hypopnea index (OAHI) was 6.5 (9.1) events/hr and the mean central event rate of 3.0 (3.7) events/hr. RDI had decreased by 22% or 1.5 events/hour (95%CI: 0.6, 1.9) with each year of age (P = 0.005). Subjects with more severe respiratory disease had 38% more central events (P = 0.02). Infants exposed to secondhand smoke had 2.4% lower (P = 0.04) oxygen saturation nadirs and a pattern for more desaturation events. Non-white subjects were found to have 33% higher OAHI (P = 0.05), while white subjects had a 61% higher rate of central events (P < 0.001). CONCLUSIONS: RDI was elevated in a selected BPD population compared to norms for non-preterm children. BPD severity, smoke exposure, and race may augment the severity of SDB. RDI improved with age but was still elevated by age 4, suggesting that this population is at risk for the sequelae of SDB.

An Official American Thoracic Society Clinical Practice Guideline: Pediatric Chronic Home Invasive Ventilation.

BACKGROUND: Children with chronic invasive ventilator dependence living at home are a diverse group of children with special health care needs. Medical oversight, equipment management, and community resources vary widely. There are no clinical practice guidelines available to health care professionals for the safe hospital discharge and home management of these complex children. PURPOSE: To develop evidence-based clinical practice guidelines for the hospital discharge and home/community management of children requiring chronic invasive ventilation. METHODS: The Pediatric Assembly of the American Thoracic Society assembled an interdisciplinary workgroup with expertise in the care of children requiring chronic invasive ventilation. The experts developed four questions of clinical importance and used an evidence-based strategy to identify relevant medical evidence. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to formulate and grade recommendations. RESULTS: Clinical practice recommendations for the management of children with chronic ventilator dependence at home are provided, and the evidence supporting each recommendation is discussed. CONCLUSIONS: Collaborative generalist and subspecialist comanagement is the Medical Home model most likely to be successful for the care of children requiring chronic invasive ventilation. Standardized hospital discharge criteria are suggested. An awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child's care. Standardized equipment for monitoring, emergency preparedness, and airway clearance are outlined. The recommendations presented are based on the current evidence and expert opinion and will require an update as new evidence and/or technologies become available.

Polysomnography in preterm infants and children with chronic lung disease.

OBJECTIVE: To determine the utility of overnight polysomnography (PSG) in assessing pulmonary reserve in stable preterm children with chronic lung disease (CLD). STUDY DESIGN: A retrospective review and descriptive study of overnight PSGs and clinic visits of preterm infants/children less than 3 years of age who were diagnosed with bronchopulmonary dysplasia at discharge from the hospital and enrolled in the Johns Hopkins CLD patient registry between 2008 and 2010. RESULTS: Sixty-two clinically stable patients underwent at least one overnight polysomnogram for clinical indications. The majority of patients were referred for oxygen titration (71%). PSGs from first studies revealed a mean respiratory disturbance index (RDI) of 8.2 ± 10.1 events/hr and a mean O(2) saturation (SaO(2) ) nadir of 86.2 ± 5.7%. In patients who underwent more than one PSG (n = 23), a significant decrease in RDI (P < 0.001) was found between the first study (mean age: 8.0 ± 3.3 months) and second study (mean age: 13.4 ± 5.2 months). Outpatient clinical measures of mean room air SaO(2) and respiratory rate were not predictive of PSG measures of RDI and SaO(2) nadir. CONCLUSION: Mean RDI was higher in stable preterm infants/children with CLD compared to previously published controls. RDI decreased with age in stable preterm infants/children with CLD suggesting improved pulmonary reserve with age. Outpatient clinical measures (respiratory rate and room air SaO(2) ) did not correlate with RDI and SaO(2) nadir indicating that overnight PSG is more sensitive in assessing pulmonary reserve than outpatient clinical measures.

Primary snoring and growth failure in a patient with cystic fibrosis.

An 8-year-old female with pancreatic-insufficiency cystic fibrosis presented with recurrent pharyngitis, and reduction in body mass index and height velocity during the previous 2 years. Her symptoms (eg, snoring and restless sleep) suggested obstructive sleep apnea, and physical examination revealed tonsillar hypertrophy. While her respiratory disturbance index on nocturnal polysomnography was normal, there was evidence of prolonged periods of snoring, associated with hypercapnia. Adenotonsillectomy decreased the snoring, improved her sleep, and in the 18-month follow-up period she had substantial weight-gain and growth improvement. This case demonstrates that adenotonsillar hypertrophy associated with recurrent pharyngitis and primary snoring might hinder growth in a patient with cystic fibrosis.

Perioperative management of children with obstructive sleep apnea.

Obstructive sleep apnea syndrome (OSA) affects 1%-3% of children. Children with OSA can present for all types of surgical and diagnostic procedures requiring anesthesia, with adenotonsillectomy being the most common surgical treatment for OSA in the pediatric age group. Thus, it is imperative that the anesthesiologist be familiar with the potential anesthetic complications and immediate postoperative problems associated with OSA. The significant implications that the presence of OSA imposes on perioperative care have been recognized by national medical professional societies. The American Academy of Pediatrics published a clinical practice guideline for pediatric OSA in 2002, and cited an increased risk of anesthetic complications, though specific anesthetic issues were not addressed. In 2006, the American Society of Anesthesiologists published a practice guideline for perioperative management of patients with OSA that noted the pediatric-related risk factor of obesity, and the increased perioperative risk associated with adenotonsillectomy in children younger than 3 yr. However, management of OSA in children younger than 1 yr-of-age was excluded from the guideline, as were other issues related specifically to the pediatric patient. Hence, many questions remain regarding the perioperative care of the child with OSA. In this review, we examine the literature on pediatric OSA, discuss its pathophysiology, current treatment options, and recognized approaches to perioperative management of these young and potentially high-risk patients.

Polysomnographic values in adolescents with ataxia telangiectasia.

Most adolescents with ataxia telangiectasia (A-T) develop progressive bulbar muscle weakness and decreased pulmonary reserve. The purpose of this study was to define the patterns of sleep and respiration during sleep, and to identify sleep-related breathing problems in subjects with A-T. To address these issues, overnight polysomnography was performed on 12 adolescents with A-T. Eleven of the 12 subjects completed overnight polysomnography. The median age was 16 years (range, 13-20 years). All subjects in the study were wheelchair-bound and the median forced vital capacity (% predicted of normal) was 44% (range, 16-82%). The mean sleep efficiency was 72.6% with a mean apnea hypopnea index (AHI) of 0.7 events/hr (range, 0-2.2). The majority of apnea/hypopneas were REM related. The mean central apnea index was 0.1 events/hr (range, 0-0.2). The mean oxygen saturation nadir was 92.7% (range, 87-96) and the mean peak end-tidal carbon dioxide ET(CO(2) ) value was 53.8 mm Hg (range, 49-60). Two of 11 subjects had ET(CO(2) ) values >or=50 mm Hg for more than 50% of total sleep time. In this study, the majority of A-T adolescents had infrequent partial or complete upper airway obstructions during sleep and minimal nighttime hypoxemia. They did, however, have decreased sleep efficiency most likely, due in part, to their underlying neurological condition. This decrease in total sleep time may underestimate hypoventilation. Based on these findings, overnight polysomnography should be considered in adolescents with A-T, particularly in those in which there is a clinical suspicion of sleep related breathing abnormalities.