Type one chiari malformation as a cause of central sleep apnea and hypoventilation in children.

OBJECTIVES: Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. METHODS: This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008-2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging. RESULTS: We included 104 children with CM1 with a median age of 7 (interquartile range (IQR) 5-13) years. The median extent of tonsillar descent (TD) was 13 (IQR 10-18) mm. Syringomyelia was present in 19 children (18%). Of all children, 53 (51%) had normal PSG, 35 (34%) showed periodic breathing or central apnea and hypopnea index ≥5 h-1, and 16 (15%) displayed features of compensated central hypoventilation and end-tidal or transcutaneous carbon dioxide 99th percentile level above 50 mmHg. TD had the best predictive value for central breathing disorders. In a linear model, both age (61%) and TD (39%) predicted median breathing frequency (R = 0.33, p < 0.001). CONCLUSIONS: Although severe CSA is a rare complication of brainstem compression in pediatric patients with CM1, short arousal-triggered episodes of periodic breathing and mild compensated central hypoventilation are common. TD shows the best but still poor prediction of the presence of a central breathing disorder. This highlights the use of PSG in patient evaluation. Posterior fossa decompression surgery effectively treats central breathing disorders.

Effect of adenotonsillectomy on central sleep apnea: A scoping review.

OBJECTIVE: The purpose of this study was to assess the effects of adenotonsillectomy (A&T) on reducing central sleep apnea (CSA) in children and adolescents with obstructive sleep apnea (OSA). METHODS: A review of the PubMed database was conducted. Two researchers independently reviewed the articles from the literature search and selected papers for further review if they met inclusion criteria. Included studies were prospective studies and case series whose patients were children 18 years or younger undergoing adenotonsillectomy for obstructive sleep apnea with reported pre and postoperative central apnea indexes (CAI). RESULTS: Of the 107 articles initially identified, 18 underwent full length review, and ultimately 15 for final review. All studies reported marked improvement of central sleep apnea indexes after adenotonsillectomy. Two studies found resolution of CSA in 66.7 % and 73.7 % of patients respectively. The remaining four studies found significant reductions in CAI in 43.9 %-93 % of patients. The degree of reduction varied from 40.9 % to 80 %. DISCUSSION: Adenotonsillectomy improves and at times resolves CSA in pediatric patients with concomitant OSA.

Images: Atypical presentation of congenital central hypoventilation syndrome in an infant with central and obstructive sleep apnea.

Congenital central hypoventilation syndrome (CCHS), a rare disease caused by paired-like homeobox 2B variants, affects control of breathing. We report on a 21-month-old boy with CCHS caused by a novel nonpolyalanine repeat mutation, neuroblastoma, severe obstructive and central sleep apnea, and sleep-related hypoxemia without hypoventilation. At 10 months, due to persistent central sleep apnea during serial polysomnography, bilevel positive airway pressure therapy was initiated despite the absence of hypoventilation. Nonpolyalanine repeat mutations are associated with severe phenotypes requiring continuous assisted ventilation, Hirschsprung's disease, and neural crest tumors; however, our patient had a relatively milder respiratory phenotype requiring sleep-only assisted ventilation without tracheostomy. Although alveolar hypoventilation is the hallmark of CCHS, our patient lacked hypoventilation. Bilevel positive airway pressure could be considered in some infants with CCHS requiring sleep-only assisted ventilation for tracheostomy avoidance. Our case demonstrates the expanding phenotypic spectrum in CCHS and the importance of formulating an individualized care plan. CITATION: Fain ME, Raghunandan S, Pencheva B, Leu RM, Kasi AS. Images: atypical presentation of congenital central hypoventilation syndrome in an infant with central and obstructive sleep apnea. J Clin Sleep Med. 2024;20(3):478-481.

The OSA patient journey: pathways for diagnosis and treatment among commercially insured individuals in the United States.

STUDY OBJECTIVES: The aims of this study were to characterize obstructive sleep apnea (OSA) care pathways among commercially insured individuals in the United States and to investigate between-groups differences in population, care delivery, and economic aspects. METHODS: We identified adults with OSA using a large, national administrative claims database (January 1, 2016-February 28, 2020). Inclusion criteria included a diagnostic sleep test on or within ≤ 12 months of OSA diagnosis (index date) and 12 months of continuous enrollment before and after the index date. Exclusion criteria included prior OSA treatment or central sleep apnea. OSA care pathways were identified using sleep testing health care procedural health care common procedure coding system/current procedural terminology codes then selected for analysis if they were experienced by ≥ 3% of the population and assessed for baseline demographic/clinical characteristics that were also used for model adjustment. Primary outcome was positive airway pressure initiation rate; secondary outcomes were time from first sleep test to initiation of positive airway pressure, sleep test costs, and health care resource utilization. Associations between pathway type and time to treatment initiation were assessed using generalized linear models. RESULTS: Of 86,827 adults with OSA, 92.1% received care in 1 of 5 care pathways that met criteria: home sleep apnea testing (HSAT; 30.8%), polysomnography (PSG; 23.6%), PSG-Titration (19.8%), Split-night (14.8%), and HSAT-Titration (3.2%). Pathways had significantly different demographic and clinical characteristics. HSAT-Titration had the highest positive airway pressure initiation rate (84.6%) and PSG the lowest (34.4%). After adjustments, time to treatment initiation was significantly associated with pathway (P < .0001); Split-night had shortest duration (median, 28 days), followed by HSAT (36), PSG (37), PSG-Titration (58), and HSAT-Titration (75). HSAT had the lowest sleep test costs and health care resource utilization. CONCLUSIONS: Distinct OSA care pathways exist and are associated with differences in population, care delivery, and economic aspects. CITATION: Wickwire EM, Zhang X, Munson SH, et al. The OSA patient journey: pathways for diagnosis and treatment among commercially insured individuals in the United States. J Clin Sleep Med. 2024;20(4):505-514.

Association and risk factors of pediatric pulmonary hypertension with obstructive sleep apnea: A national study utilizing the Kids' Inpatient Database (KID).

STUDY OBJECTIVE: Assess the prevalence of and risk factors for pediatric pulmonary hypertension (PH) in the 2016 Kids' Inpatient Database (KID), including obstructive sleep apnea (OSA) and obesity. METHODS: Retrospective cross-sectional cohort study utilizing 6,081,132 weighted pediatric discharges from the 2016 KID. Study variables included age, length of stay, mortality, gender, hospital region, primary payer, race, median household income for patient's ZIP code, OSA, central sleep apnea (CSA), obesity, Down syndrome, sickle cell disease (SCD), thalassemia, congenital heart disease (CHD), hypertension, asthma and chronic lung disease of prematurity (CLDP). PH was the primary outcome of interest. Bivariate and multivariable logistic regression models were utilized with odds ratios and 95 % confidence intervals. RESULTS: The mean age was 3.76 years, the mean hospital length of stay was 3.85 days, 48.9 % were male, 52.6 % had government health insurance, 51.0 % were White, 16.1 % were Black, 21.1 % were Hispanic, 5.0 % were Asian or Pacific Islander, 0.80 % were Native American and 6.1 % identified as "other". The prevalence of PH was 0.21 % (12,777 patients). There were 37,631 patients with OSA and the prevalence of PH among this cohort was 3.3 %, over 10x greater than the overall prevalence of PH in the 2016 KID (0.21 %). Risk factors associated with PH included CLDP, CHD, Down syndrome, asthma, OSA, CSA, hypertension, SCD, obesity, race/ethnicity, government insurance, age, male gender (p < 0.0001), and hospital region (p = 0.0002). CONCLUSIONS: Several risk factors were independently associated with PH, such as OSA, CSA, obesity, asthma, and insurance status. Prospective multi-institutional studies are needed to assess the relationships between these risk factors, severity metrics, and causative links in the development of PH; in addition to identifying children with OSA who are most likely to benefit from cardiopulmonary screening prior to adenotonsillectomy. LEVEL OF EVIDENCE: Level III.

Central sleep apnea in patients with pontine tegmental cap dysplasia treated with supplemental oxygen: a case report.

This is the first case report of sleep-disordered breathing in patients with pontine tegmental cap dysplasia, a very rare neurological disorder characterized by an anatomic malformation in the pons. Patients present with hypotonia, cognitive dysfunction, and cranial nerve palsies (eg, hearing loss, trigeminal anesthesia, and swallow dysfunction). Extensive studies have demonstrated the relevance of different pontine neuronal nuclei in breathing regulation, which are structurally abnormal in pontine tegmental cap dysplasia. We present detailed polysomnography data for 3 patients aged 41 years, 20 years, and 1.5 years revealing significant central sleep apnea. We discuss our experience with managing their sleep-disordered breathing in the setting of multiple cranial nerve palsies and corneal anesthesia, and its relative contraindication of noninvasive positive pressure ventilation treatment. CITATION: Ju Wang J-D, Doherty D, Ramirez J-M, Chen M. Central sleep apnea in patients with pontine tegmental cap dysplasia treated with supplemental oxygen: a case report. J Clin Sleep Med. 2023;19(4):843-849.

HIDEA syndrome: A rare cause of congenital hypoventilation in a premature infant.

BACKGROUND: HIDEA (hypotonia, hypoventilation, intellectual disability, dysautonomia, epilepsy and eye abnormalities) syndrome is a rare and novel disease. We describe a premature patient who required extensive work up for his hypoventilation with a diagnosis of HIDEA syndrome. CASE DESCRIPTION: The patient was born to a pair of consanguineous parents at 32-week gestation. His intermittent bradypnoea requiring significant respiratory support during his postnatal clinical course was atypical for bronchopulmonary dysplasia and this required further extensive work up to look for a cause for his hypoventilation. A trio whole exon sequencing was done which identified homozygous variants in P4HTM, in keeping with the diagnosis of autosomal recessive HIDEA syndrome. He is currently doing well on BiPAP 18 cm H2O / 8 cm H2O, Rate 30 breaths per minute in room air and full nasogastric feeding. He also has cortical blindess and severe global developmental delay. CONCLUSION: Early diagnosis is crucial to optimise adequate ventilatory management including early tracheostomy as many require lifelong continuous or intermittent ventilation. This minimises the complications of chronic hypoxia and reduces mortality risk.

Images: Sleep-disordered breathing and hypoventilation in a child with obesity and hypothalamic dysfunction.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare and potentially lethal disorder of respiratory control, autonomic, and hypothalamic dysfunction of unknown etiology. We report a 15-year-old girl with ROHHAD who developed hyperphagia and rapid weight gain of 16 kg between 2.5 and 4 years of age and cardiorespiratory arrest at 4 years. Initial polysomnography showed central sleep apnea and severe oxygen desaturations without hypoventilation. Mild obstructive sleep apnea and intermittent hypoxemia were identified at 4.5 years, following which nocturnal bilevel positive airway pressure therapy was initiated. At 6 years, she developed sleep-related hypoventilation, and subsequent polysomnograms continued to show obstructive sleep apnea and hypoventilation requiring bilevel positive airway pressure. Clinicians interpreting polysomnograms should become familiar with the evolution of sleep-disordered breathing in ROHHAD and that hypoventilation may develop over time. Our case highlights the importance of serial polysomnography in patients with ROHHAD and optimal ventilatory management. CITATION: Ghosh R, Malik M, Daley TC, Kasi AS. Images: Sleep-disordered breathing and hypoventilation in a child with obesity and hypothalamic dysfunction. J Clin Sleep Med. 2022;18(1):339-342.

Optimal NIV Medicare Access Promotion: Patients With Central Sleep Apnea: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society.

This document summarizes suggestions of the central sleep apnea (CSA) Technical Expert Panel working group. This paper shares our vision for bringing the right device to the right patient at the right time. For patients with CSA, current coverage criteria do not align with guideline treatment recommendations. For example, CPAP and oxygen therapy are recommended but not covered for CSA. On the other hand, bilevel positive airway pressure (BPAP) without a backup rate may be a covered therapy for OSA, but it may worsen CSA. Narrow coverage criteria that require near elimination of obstructive breathing events on CPAP or BPAP in the spontaneous mode, even if at poorly tolerated pressure levels, may preclude therapy with BPAP with backup rate or adaptive servoventilation, even when those devices provide demonstrably better therapy. CSA is a dynamic disorder that may require different treatments over time, sometimes switching from one device to another; an example is switching from BPAP with backup rate to an adaptive servoventilation with automatic end-expiratory pressure adjustments, which may not be covered. To address these challenges, we suggest several changes to the coverage determinations, including: (1) a single simplified initial and continuing coverage definition of CSA that aligns with OSA; (2) removal of hypoventilation terminology from coverage criteria for CSA; (3) all effective therapies for CSA should be covered, including oxygen and all PAP devices with or without backup rates or servo-mechanisms; and (4) patients shown to have a suboptimal response to one PAP device should be allowed to add oxygen or change to another PAP device with different capabilities if shown to be effective with testing.

Variable phenotypes in congenital central hypoventilation syndrome with PHOX2B nonpolyalanine repeat mutations.

STUDY OBJECTIVES: Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting the autonomic nervous system that is caused by variants in the paired-like homeobox 2B (PHOX2B) gene. About 10% of patients with CCHS have nonpolyalanine repeat mutations (NPARM) that are associated with severe phenotypes requiring continuous assisted ventilation, Hirschsprung's disease, and increased neural crest tumor risk. However, some patients with NPARM have milder phenotypes. Our objective was to describe the phenotypes in patients with CCHS PHOX2B NPARM. METHODS: Retrospective case series of patients with CCHS PHOX2B NPARM was conducted at 2 children's hospitals to evaluate their phenotypes. RESULTS: We identified 8 patients with CCHS PHOX2B NPARM aged 3-31 years. Seven patients were diagnosed in infancy and 1 patient at 2 years of age. All patients presented with respiratory depression in the first 2 months of life. Only 1 patient was identified with a severe phenotype requiring continuous assisted ventilation, Hirschsprung's disease, and a neural crest tumor, which was resected. Five patients required positive pressure ventilation via tracheostomy only during sleep and 2 patients required oxygen only during sleep. Four patients had Hirschsprung's disease and 1 patient had a cardiac pacemaker due to a bradyarrhythmia. None of the patients had echocardiographic abnormalities. CONCLUSIONS: Patients with CCHS PHOX2B NPARM can have variable phenotypes, emphasizing the importance of implementing a plan of care that is individualized for each patient. The type of NPARM and their respective location on the PHOX2B gene may play a critical role in the severity of phenotypes displayed by each patient. CITATION: Kasi AS, Li H, Jurgensen TJ, Guglani L, Keens TG, Perez IA. Variable phenotypes in congenital central hypoventilation syndrome with PHOX2B nonpolyalanine repeat mutations. J Clin Sleep Med. 2021;17(10):2049-2055.

Central Sleep Apnea Predicts Pulmonary Complications After Cardiac Surgery.

BACKGROUND: Postoperative major pulmonary complications (MPCs) continue to be leading causes of increased morbidity and death after cardiac surgery. Although various risk factors have been identified, reports on the association between sleep-disordered breathing (SDB) and postoperative MPCs remain inconclusive. RESEARCH QUESTION: What is the incidence of the composite end point postoperative MPCs? What are predictors for postoperative MPCs in patients without SDB, with OSA, and with central sleep apnea (CSA) who undergo cardiac surgery? STUDY DESIGN AND METHODS: In this subanalysis of the ongoing prospective observational study "Impact of Sleep-disordered breathing on Atrial Fibrillation and Perioperative complications in Patients undergoing Coronary Artery Bypass grafting Surgery (CONSIDER AF)," preoperative risk factors for postoperative MPCs were examined in 250 patients who underwent cardiac surgery. Postoperative MPCs (including respiratory failure, acute respiratory distress syndrome, pneumonia, or pulmonary embolism) were registered prospectively within the first seven postoperative days. Presence and type of SDB were assessed the night prior to surgery with the use of portable SDB-monitoring. RESULTS: Patients with SDB experienced significantly more often postoperative MPCs than patients without SDB (24% vs 7%; P < .001). Multivariable logistic regression analysis showed that CSA (OR, 4.68 [95% CI, 1.78-12.26]; P = .002), heart failure (OR, 2.65 [95% CI, 1.11-6.31]; P = .028), and a history of transient ischemic attack or stroke (OR, 2.73 [95% CI, 1.07-6.94]; P = .035) were associated significantly with postoperative MPCs. Compared with patients without MPCs, those with postoperative MPCs had a significantly longer hospital stay (median days, 9 [25th/75th percentile, 7/13] vs 19 [25th/75th percentile, 11/38]; P < .001). INTERPRETATION: Among established risk factors for postoperative MPCs, CSA, heart failure, and history of transient ischemic attack or stroke were associated significantly with postoperative MPCs. Our findings contribute to the identification of patients who are at high-risk for postoperative MPCs. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT02877745.

Severe central apnea secondary to cerebellar dysplasia in a child: look past Joubert syndrome.

NONE: We report the case of a female patient aged 12 years referred to our pediatric sleep unit with a history of central sleep apnea associated with transient episodes of tachypnea on polysomnography recordings. The patient was otherwise healthy, with no personal or family medical history, and had a normal physical and neuropsychological examination. Brain magnetic resonance imaging showed signs of cerebellar vermis dysplasia but without the classical features of the molar tooth sign. The rest of the workup (genetic tests, blood tests, cardiac investigations) was normal except for an increased peripheral chemosensitivity to carbon dioxide and oxygen. The patient was successfully treated with bilevel positive airway pressure. This case report highlights the importance of performing brain magnetic resonance imaging in patients with central sleep apnea to study the cerebellum, beyond the brainstem area. Cerebellar malformations can be found even in the absence of any other neurological condition.

Positional impairment of gas exchange during diaphragm pacing alleviated by increasing amplitude settings in congenital central hypoventilation syndrome.

None: Diaphragm pacing (DP) by phrenic nerve stimulation is a modality of chronic ventilatory support in individuals with congenital central hypoventilation syndrome (CCHS). We report a 9-year-old girl with CCHS who uses DP without tracheostomy during sleep. Her parents report hypoxemia and hypercapnia related to positional changes of the body during sleep requiring frequent adjustment of pacer settings. Overnight polysomnography was performed to titrate DP settings that showed adequate gas exchange in the supine position, but intermittent hypoxemia and hypercapnia were noted in the left decubitus position without obstructive sleep apnea occurring. Subsequently, the DP amplitude settings were increased during polysomnography, thereby identifying and treating positional hypoxemia and hypercapnia in various body positions. Our case emphasizes the importance of polysomnography in children with CCHS using DP to monitor for sleep-disordered breathing and titration of DP settings to achieve optimal oxygenation and ventilation with different body positions during sleep.

Central sleep apnea in children with obstructive sleep apnea syndrome and improvement following adenotonsillectomy.

BACKGROUND: Although the pathogenesis of central and obstructive events seems to be different, these two entities may somehow be related. We aimed to determine whether, as reported in previous research, the number of central sleep apnea (CSA) cases in a population of children with obstructive sleep apnea syndrome (OSAS) was greater than in patients without obstructive events, and if CSA worsens with increasing OSAS severity. As a second objective, we analyzed changes in central apnea index (CAI) after adenotonsillar surgery compared to changes when no surgery has been performed. METHODS: We retrospectively reviewed nocturnal polysomnography (PSG) data from children between 1 and 14 years of age with no neurological conditions or syndromes. Patients with CAI values greater than 5 per hour were diagnosed as having CSA. Improvements of greater than 50% in CAI on repeat PSG were considered to represent a real change. RESULTS: Data were available from 1279 PSG studies, resulting in 72 children with a CAI greater than 5 per hour (5.6%). Patients with OSAS showed a higher CAI (2.16) compared with those without OSAS (1.17), and this correlation increased with higher degrees of obstructive apnea severity. When adenotonsillectomy was performed due to OSAS, the CAI decreased by 1.37. The average decrease in PSG values was only 0.38 in cases where no surgery was performed. CONCLUSION: The results of this study suggest that although CSA is perceived to be mostly associated with central nervous system ventilatory control, there may be a connection with airway obstruction and in children with CSA and OSA diagnosis adenotonsillectomy may improve both conditions.

Severe Positional Central Sleep Apnea in an Asymptomatic Adult With a PHOX2B Frameshift Mutation.

ABSTRACT: We report an unusual case of an adult patient carrying a germline PHOX2B frameshift mutation and hence was diagnosed with congenital central hypoventilation syndrome. He came to medical attention after the mutation was identified in his daughter who presented with hypoventilation and a neuroblastoma. Although PHOX2B mutations are usually associated with a phenotype of congenital hypoventilation, severe autonomic dysfunction and neural crest tumors, our patient had no complaints at the time of presentation. At polysomnography we found severe positional hypercapnic central sleep apnea, partly responsive to positional therapy. Eventually, he was titrated to noninvasive ventilation with resolution of the central breathing events and, in hindsight, a more refreshing sleep than before. Clinicians working in sleep medicine need to be aware of the variable expression of this rare condition to prevent late cardiorespiratory and neurocognitive complications.

Postoperative complications after elective coronary artery bypass grafting surgery in patients with sleep-disordered breathing.

BACKGROUND: Sleep-disordered breathing (SDB) may increase the risk of postoperative complications in patients after cardiac surgery. This study evaluated the length of hospital stay as well as postoperative cardiac, respiratory, and renal complications after elective coronary artery bypass grafting (CABG) in patients without SDB, with central sleep apnea (CSA), or with obstructive sleep apnea (OSA). METHODS: The presence and type of SDB had been assessed with polygraphic recordings in 100 patients the night before elective CABG surgery. SDB was defined as an apnea-hypopnea index (AHI) of ≥ 15/h. Prolonged length of hospital stay (LOS) and postoperative hemodynamic instability due to any cause were retrospectively evaluated as primary endpoints and cardiac, respiratory, and renal complications as secondary endpoints. RESULTS: 37% of patients had SDB, 14% CSA, and 23% OSA. LOS differed significantly between patients without SDB and those with CSA and OSA [median (25;75. percentile): 8.0 days (7.5;11.0) vs. 9.5 days (7.0;12.5) vs. 12.0 days (9.0;17.0), Kruskal-Wallis test between three groups: p = 0.023; OSA vs. no SDB: p = 0.005]. AHI was significantly associated with prolonged LOS [> 9 days; odds ratio (OR) (95% confidence interval): 1.047 (1.001;1.095), p = 0.044]. Prolonged need of vasopressors (≥ 48 h) was observed in 36% of patients without SDB, in 64% with CSA, and in 62% with OSA (p = 0.037). AHI was significantly associated with prolonged (≥ 48 h) need of vasopressors [OR (95% CI): 1.052 (1.002;1.104), p = 0.040], independent of any confounders. CONCLUSIONS: SDB, particularly OSA, is associated with prolonged LOS after CABG, independent of known confounders. Prolonged LOS in patients with SDB may be due to increased postoperative hemodynamic instability due to any cause.

Obstructive Sleep Apnea in a Severely Obese Child With Combined Central Sleep Apnea and Sleep-Related Hypoventilation Disorder Caused by a Medullary Tumor.

ABSTRACT: A medullary tumor is a relatively rare disease that causes severe and complicated respiratory disorders, including sleep-related breathing disorders, due to dysregulation of respiratory control. A severely obese 12-year-old male was admitted to our hospital for worsening dyspnea and received a diagnosis of type II respiratory failure. Although obstructive sleep apnea (OSA) and/or obesity hypoventilation syndrome were suspected, a polysomnogram obtained during a nap (nap study) revealed central sleep apnea (CSA) and sleep-related hypoventilation disorder (SRHD) in addition to OSA. Brain magnetic resonance imaging showed a mass in the medulla oblongata. The patient received a diagnosis of CSA/SRHD caused by a medullary tumor, and with OSA. A partial brainstem tumor resection was performed. Noninvasive positive airway pressure therapy was initiated, and was continued after surgery. However, the patient died unexpectedly, 20 months after surgery. When children with sleep problems caused by OSA present with atypical symptoms of OSA, a neurological examination and polysomnography should be conducted to detect CSA and SRHD.

Primary central sleep apnea and anesthesia: a retrospective case series.

PURPOSE: Primary (idiopathic) central sleep apnea (PCSA) is a rare central sleep-related breathing disorder characterized by increased chemoreceptor sensitivity to partial pressure of carbon dioxide, which manifests as hyperventilation followed by apnea during non-rapid eye movement sleep. The purpose of this retrospective study was to describe the postoperative course of patients who had PCSA and underwent procedures requiring anesthetic management. METHODS: Patients who received a diagnosis of PCSA at our institution and required procedural anesthesia between 1 January 2010 and 1 June 2016 underwent a comprehensive review of their health records with a focus on identifying respiratory complications. RESULTS: Ten patients (nine males, one female) underwent 47 procedures requiring anesthetic management: 20 (43%) under general anesthesia, 25 (53%) with monitored anesthetic care, and two (4%) with regional anesthesia. Procedures were complicated by second-degree heart block in one patient and pneumonia in another two (one had Ivor-Lewis esophagectomy and the other bronchoscopy to evaluate worsening lung infiltration). Hypoxemia (oxyhemoglobin saturation < 90% for three minutes) developed in three patients during anesthesia recovery. One was possibly due to PCSA-a 73-yr-old male with alcoholic cirrhosis who was moderately sedated and hypoxemic after orthopedic surgery; his oxygenation improved with an adaptive servoventilator positive airway pressure device and supplemental oxygen. His underlying medical condition or level of sedation may have contributed to hypoxemia. The other patients became hypoxemic after bronchoscopy. No other cases were complicated by postoperative respiratory compromise. CONCLUSIONS: No major adverse outcomes were related to PCSA postoperatively. Nevertheless, continuation of home positive airway pressure therapy during anesthesia recovery was useful in one patient who had cirrhosis and postoperative hypoxemia.