Acute Airway Obstruction: An Unusual Presentation of Posttransplant Lymphoproliferative Disorder in a Renal Transplant Recipient.

Posttransplant lymphoproliferative disorder is a life-threatening complication after solid-organ transplants. In adults, recipients of heart transplants have the highest risk, whereas renal transplant recipients have the lowest risk among all solid-organ transplants. The most common site for posttransplant lymphoproliferative disorders are gastrointestinal tract followed by the graft itself. Airway involvement in posttransplant lymphoproliferative disorder is rarely encountered. We report a case of a 26-year-old renal allograft recipient who presented to the emergency room with airway obstruction necessitating an emergency tracheostomy. Imaging revealed a left tonsillar mass extending into the nasopharynx and retropharyngeal space causing complete oropharyngeal occlusion. Endoscopic biopsy from nasopharyngeal mass showed a diffuse large B-cell lymphoma and was Ebstein-Barr virus positive. Reduction in immunosuppression and treatment with posttransplant lymphoproliferative disorder-1 risk-stratified approach resulted in complete remission.

Airway Complications in a Patient With Ehlers-Danlos Syndrome: A Case Report.

A female patient, known to have hypermobile Ehlers-Danlos syndrome (hEDS), underwent several elective gastroscopies under sedation in different hospitals. Except for a single incident of mild laryngospasm during emergence, all procedures were uneventful. On that occasion, following the procedure in the postanesthesia care unit, the patient suffered severe airway obstruction, and standard airway rescue techniques exacerbated adequate ventilation. After the removal of all stimuli and maintaining only an indirect oxygen supply via a mask in front of her face, her airway improved, and the patient fully recovered after 17 minutes. After the gastroscopy, physical examination revealed that the patient had an extremely flexible trachea that could be completely moved outside the midline to the extreme right and left. For the subsequent procedures, an airway plan was developed in conjunction with the patient and resulted in uncomplicated perianesthetic care. This case report serves to alert readers to the risk of adverse airway events in patients with EDS and suggests an alternative approach to avoid such complications. When patients receive care in different hospitals, adequate documentation is essential and adequate preoperative assessment is crucial. This case study demonstrates the value of patient-coproduction care plans.

Large congenital cervical mass in a neonate: prenatal diagnosis and postnatal management of teratoma: a case report.

INTRODUCTION: Cervical teratomas are rare congenital neoplasms that can cause neonatal airway obstruction if large. CASE PRESENTATION: The female Persian neonate displayed respiratory distress at birth, with a 7 cm × 8 cm cystic solid mass identified on the left side of the neck. Antenatal ultrasonography revealed polyhydramnios. Despite initial stabilization, the infant required intubation and mechanical ventilation due to persistent respiratory distress. Imaging confirmed a cystic mass compressing the trachea, ruling out cystic hygroma. Surgical resection on postnatal day 17 revealed a 10 cm × 10 cm solid cystic structure, histologically identified as an immature teratoma. CONCLUSION: Despite risks of poor fetal and postnatal outcome from large cervical teratomas, early surgical resection after airway stabilization can result in recovery. Proper multidisciplinary management of respiratory distress from such tumors is paramount.

Effects of adenoid hypertrophy on nasopharyngeal airway ventilation: A computational fluid dynamics study.

OBJECTIVES: Adenoid hypertrophy causes impaired nasopharyngeal airways (NA) ventilation. However, it is difficult to evaluate the ventilatory conditions of NA. Therefore, this study aimed to analyze the nasopharyngeal airway resistance (NARES) based on computational fluid dynamics simulations and the nasopharyngeal airway depth (NAD) and adenoid hypertrophy grade measured on cephalometric cone-beam computed tomography images and determine the relationship between NAD and grade and NARES to ultimately assess using cephalometric measurements whether NA has airway obstruction defects. METHODS: Cephalogram images were generated from cone-beam computed tomography data of 102 children (41 boys; mean age: 9.14 ± 1.43 years) who received orthodontic examinations at an orthodontic clinic from September 2012 to March 2023, and NAD and adenoid grade and NARES values were measured based on computational fluid dynamics analyses using a 3D NA model. Nonlinear regression analyses were used to evaluate the relationship between NARES and NAD and correlation coefficients to evaluate the relationship between grade and NARES. RESULTS: NARES was inversely proportional to the cube of NAD (R2 = 0.786, P < 0.001), indicating a significant relationship between these variables. The resistance NARES increased substantially when the distance NAD was less than 5 mm. However, adenoid Grade 4 (75 % hypertrophy) was widely distributed. CONCLUSIONS: These study findings demonstrate that the ventilatory conditions of NA can be determined based on a simple evaluation of cephalogram images. An NAD of less than 5 mm on cephalometric images results in NA obstruction with substantially increased airflow resistance.

Impact of upper airway obstruction management in Robin Sequence on need for myringotomy tubes.

OBJECTIVE: Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT). METHODS: Retrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups. RESULTS: One hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30-3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management. CONCLUSION: Most RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.

Analyzing the characteristics of respiratory microbiota after the placement of an airway stent for malignant central airway obstruction.

Malignant central airway stenosis is treated with airway stent placement, but post-placement microbial characteristics remain unclear. We studied microbial features in 60 patients post-stent placement, focusing on changes during granulation tissue proliferation. Samples were collected before stent (N = 29), after stent on day 3 (N = 20), and after granulation tissue formation (AS-GTF, N = 43). Metagenomic sequencing showed significant respiratory tract microbiota changes with granulation tissue. The microbiota composition, dominated by Actinobacteria, Firmicutes, and Proteobacteria, was similar among the groups. At the species level, the AS-GTF group exhibited significant differences, with Peptostreptococcus stomatis and Achromobacter xylosoxidans enriched. Analysis based on tracheoesophageal fistula presence identified Tannerella forsythia and Stenotrophomonas maltophilia as the main differential species, enriched in the fistula subgroup. Viral and fungal detection showed Human gammaherpesvirus 4 and Candida albicans as the main species, respectively. These findings highlight microbiota changes after stent placement, potentially associated with granulation tissue proliferation, informing stent placement therapy and anti-infective treatment optimization. IMPORTANCE: Malignant central airway stenosis is a life-threatening condition that can be effectively treated with airway stent placement. However, despite its clinical importance, the microbial characteristics of the respiratory tract following stent insertion remain poorly understood. This study addresses this gap by investigating the microbial features in patients with malignant central airway stenosis after stent placement, with a specific focus on microbial changes during granulation tissue proliferation. The findings reveal significant alterations in the diversity and structure of the respiratory tract microbiota following the placement of malignant central airway stents. Notably, certain bacterial species, including Peptostreptococcus stomatis and Achromobacter xylosoxidans, exhibit distinct patterns in the after-stent granulation tissue formation group. Additionally, the presence of tracheoesophageal fistula further influences the microbial composition. These insights provide valuable references for optimizing stent placement therapy and enhancing clinical anti-infective strategies.

A New Distal Pharyngeal Airway Device Associated with a Reduced Need for Chin-Lift and Jaw-Thrust Maneuvers in Sedated Patients.

Oropharyngeal airways (OPA) or nasopharyngeal airways (NPA) sometimes require chin-lift or jaw-thrust (CLJT) maneuvers to relieve airway obstruction which creates the burden of continuous hands-on care by the anesthesia provider. A new distal pharyngeal airway device (DPA) was used on 63 successive ambulatory surgery patients to assess the frequency of patients requiring manual CLJT maneuvers to prevent airway obstruction. Results were then compared with a contemporaneous group of patients who had used OPA or NPA devices for similar procedures. Patients using the DPA had a 38.5% lower rate of CLJT maneuvers compared with the combined OPA/NPA groups (22.2% of 63 vs. 60.7% of 163, P ≤ .001). Moreover, the results for the DPA group were close to those of the natural airway group (22.2% of 62 vs. 24.8% of 233, P = .66) Results were similar for a sub-set of the above groups who required deep sedation or deep extubation. CLJT maneuvers were common in this ambulatory surgery setting. The new DPA device was associated with a reduced need for such manual maneuvers when compared with similar patients who received OPA or NPA devices and is comparable with the rate for natural airways.

Treatment of malignant airway obstruction with Y-shape sigma stent loaded with I125 seeds installed via rigid bronchoscopy.

PURPOSE: To summarize and analyze the safety and efficacy of a Y-shape Sigma stent loaded with I125 in patients with inoperable malignant main airway obstruction. METHODS: This study was approved by the Institutional Ethics Committee, and a written informed consent was obtained from each participant. A Y-shape Sigma stent loaded with I125 was placed under vision from rigid bronchoscopy. The primary endpoint was alleviation of symptoms and improvement of Karnofsky Performance Status (KPS) score, and the secondary endpoint was complications and technical success. RESULTS: From November 2018 through June 2023, total 33 patients with malignant airway obstruction were palliatively treated by installing Y-shape Sigma stents loaded with I125. The airway lumen was immediately restored and the average airway opening significantly increased to 70 ± 9.4% after the procedure from baseline 30.2 ± 10.5% (p < 0.05). Average KPS score was improved from baseline 30.0 ± 10.0 to 70.0 ± 10.0 (p < 0.05) as well as PaO2 from baseline 50.1 ± 15.4 mmHg to 89.3 ± 8.6 mmHg (p < 0.05). The technical success rate of placing the stent in this study was 73%, and adverse events or complications including bleeding, I125 loss, and airway infection occurred during or after the procedure. CONCLUSION: Placement of Y-shape Sigma stents under vision from rigid bronchoscopy in the patients with malignant airway obstruction is feasible and it immediately alleviates dyspnea and significantly improves quality of life.

Prevalence, stability, and clinical significance of an isolated low FEV1 spirometry pattern in children.

OBJECTIVES: In adults, an isolated low FEV1 pattern (an FEV1 below the lower limit of normal with a preserved FVC and FEV1/FVC) has been associated with the risk of developing airway obstruction. Our objective was to examine the prevalence, stability, and clinical significance of an isolated low FEV1 pattern in the pediatric population. METHODS: We conducted a retrospective study of spirometries from children ages 6-21 years and categorized tests into spirometry patterns according to published guidelines and recent literature. In a subgroup of tests with an isolated low FEV1 pattern, we evaluated spirometry technique. We also examined the association of having a test with an isolated low FEV1 pattern with clinical markers of disease severity in a subgroup of children with cystic fibrosis (CF). RESULTS: The isolated low FEV1 pattern was uncommon across the 29,979 tests included (n = 645 [2%]). In the 263 children with an isolated low FEV1 pattern who had a follow-up test performed, the most frequent spirometry pattern at last test was normal (n = 123 [47%]). A primary diagnosis of CF was associated with increased odds of having at least one test with an isolated low FEV1 pattern (OR = 8.37, 95% CI = 4.70-15.96, p < .001). The spirometry quality in a subgroup of tests with an isolated low FEV1 pattern (n = 50) was satisfactory. In the subgroup of children with CF (n = 102), those who had a test with an isolated low FEV1 pattern had higher odds of using oral antibiotics in the last 12 months than those who had a normal pattern (OR = 3.50, 95% CI = 1.15-10.63, p = .03). CONCLUSIONS: The isolated low FEV1 pattern can occur repeatedly over time, usually transitions to a normal pattern, is not due to a poor spirometry technique, and could be clinically relevant in children with chronic lung diseases.

Prevalence of airway patency and air pocket in critically buried avalanche victims - a scoping review.

INTRODUCTION: Survival of critically buried avalanche victims is directly dependent on the patency of the airway and the victims' ability to breathe. While guidelines and avalanche research have consistently emphasized on the importance of airway patency, there is a notable lack of evidence regarding its prevalence. OBJECTIVE: The aim of this review is to provide insight into the prevalence of airway patency and air pocket in critically buried avalanche victims. METHODS: A scoping review was done in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline extension for scoping reviews. MEDLINE and Cochrane databases, as well as additional manual searching was performed to identify literature reporting data on airway patency and the presence of an air pocket in critically buried avalanche victims. After eliminating duplicates, we screened abstracts and main texts to identify eligible studies. RESULTS: Of 4,109 studies identified 154 were eligible for further screening. Twenty-four publications and three additional data sources with a total number of 566 cases were included in this review. The proportion of short-term (< 35 min) to long-term burial (≥ 35 min) in the analysed studies was 19% and 66%, respectively. The burial duration remained unknown in 12% of cases. The prevalence of airway patency in critically buried avalanche victims was 41% while that of airway obstruction was 12%, with an overall rate of reporting as low as 50%. An air pocket was present in 19% of cases, absent in 46% and unknown in 35% of the cases. CONCLUSION: The present study found that in critically buried avalanche victims patent airways were more than three times more prevalent than obstructed, with the airway status reported only in half of the cases. This high rate of airway patency supports the ongoing development and the effectiveness of avalanche rescue systems which oppose asphyxiation in critically buried avalanche victims. Further effort should be done to improve the documentation of airway patency and the presence of an air pocket in avalanche victims and to identify factors affecting the rate of airway obstruction.

The effects of bystander interventions for foreign body airway obstruction on survival and neurological outcomes: Findings of the MOCHI registry.

INTRODUCTION: Foreign body airway obstruction (FBAO) is a life-threatening condition. We aimed to quantify the impact of bystander FBAO interventions on survival and neurological outcomes. METHODS: We conducted a Japan-wide prospective, multi-center, observational study including all FBAO patients who presented to the Emergency Department from April 2020 to March 2023. Information on bystander FBAO interventions was collected through interviews with emergency medical services personnel. Primary outcomes included 1-month survival and favorable neurologic outcome defined as Cerebral Performance Category 1 or 2. We performed a multivariable logistic regression and a Cox proportional hazards modeling to adjust for confounders. RESULTS: We analyzed a total of 407 patients in the registry who had the median age of 82 years old (IQR 73-88). The FBAO incidents were often witnessed (86.5%, n = 352/407) and the witnesses intervened in just over half of the cases (54.5%, n = 192/352). The incidents frequently occurred at home (54.3%, n = 221/407) and nursing home (21.6%, n = 88/407). Common first interventions included suction (24.8%, n = 101/407) and back blow (20.9%, n = 85/407). The overall success rate of bystander interventions was 48.4% (n = 93/192). About half (48.2%, n = 196/407) survived to 1-month and 23.8% patients (n = 97/407) had a favorable neurological outcome. Adjusting for pre-specified confounders, bystander interventions were independently associated with survival (hazard ratio, 0.55; 95% CI, 0.39-0.77) and a favorable neurological outcome (adjusted OR, 2.18; 95% CI, 1.23-3.95). CONCLUSION: Bystander interventions were independently associated with survival and favorable neurological outcome, however, they were performed only in the half of patients.

Benign multinodular goitre presenting as acute decompensated type two respiratory failure.

Acute airway obstruction is a life-threatening complication of benign goitre mostly occurring in cases of known progressing goitres. The index presentation of goitre with decompensated type two respiratory failure is an exceedingly rare and a diagnostically challenging presentation. We discuss the case of a woman in her 50 s, who had been diagnosed with asthma by her general practitioner, but during admission was found to have a large goitre with retrosternal extension causing critical tracheal compression. She presented with acute decompensated type two respiratory failure. We explore the diagnostic confounding posed by the patient's background of asthma and describe the initial management of the patient with non-invasive ventilation by the emergency department. The diagnosis of upper airway obstruction was not apparent which is an interesting anomaly in this case. She underwent an emergency hemithyroidectomy and recovered with a resolution of her respiratory symptoms. Histology confirmed benign multinodular hyperplasia.

Air leak test in the Paediatric Intensive Care Unit (ALTIPICU): rationale and protocol for a prospective multicentre observational study.

INTRODUCTION: In children, respiratory distress due to upper airway obstruction (UAO) is a common complication of extubation. The quantitative cuff-leak test (qtCLT) is a simple, rapid and non-invasive test that has not been extensively studied in children. The objective of the ongoing study whose protocol is reported here is to investigate how well the qtCLT predicts UAO-related postextubation respiratory distress in paediatric intensive care unit (PICU) patients. METHODS AND ANALYSIS: Air Leak Test in the Paediatric Intensive Care Unit is a multicentre, prospective, observational study that will recruit 900 patients who are aged 2 days post-term to 17 years and ventilated through a cuffed endotracheal tube for at least 24 hours in any of 19 French PICUs. Within an hour of planned extubation, the qtCLT will be performed as a sequence of six measurements of the tidal volume with the cuff inflated then deflated. The primary outcome is the occurrence within 48 hours after extubation of severe UAO defined as combining a requirement for intravenous corticosteroid therapy and/or ventilator support by high-flow nasal cannula and/or by non-invasive ventilation or repeat invasive mechanical ventilation with a Westley score ≥4 with at least one point for stridor at each initiation. The results of the study are expected to identify risk factors for UAO-related postextubation respiratory distress and extubation failure, thereby identifying patient subgroups most likely to require preventive interventions. It will also determine whether qtCLT appears to be a reliable method to predict an increased risk for postextubation adverse events as severe UAO. ETHICS AND DISSEMINATION: The study was approved by the Robert Debré University Hospital institutional review board (IRB) on September 2021 (approval #2021578). The report of Robert Debré University Hospital IRB is valid for all sites, given the nature of the study with respect to the French law. The results will be submitted for publication in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: NCT05328206.

A narrative review of oncologic emergencies in patients with head and neck cancers: initial management and the role of radiation therapy.

BACKGROUND AND OBJECTIVE: Head and neck cancers (HNCs) encompass a complex group of malignancies with high morbidity, often leading to critical emergencies such as pain crises, airway obstruction and hemorrhage. This review aims to outline an evidence-based approach to the multidisciplinary management of HNC oncologic emergencies with a focus on the role of emergent radiotherapy (RT). METHODS: A literature search was performed using Medline, Embase and the Cochrane Central Register of Controlled Trials databases with a focus on three common oncological emergencies using the following keywords: "head and neck cancer", "radiation OR radiotherapy", "pain", "bleeding OR haemorrhage", and "airway obstruction". All English language articles published up to April 2022 were screened to identify studies pertaining to the management of oncologic emergencies in HNC. KEY CONTENT AND FINDINGS: The management of oncologic emergencies in HNC present a unique set of challenges that require early recognition and aggressive treatment. In this narrative review, we summarize the evidence supporting the role of RT in the management of HNC patients presenting with pain crisis, malignant airway obstruction and acute haemorrhage. We demonstrate that while RT can be used as a primary or adjunct therapy, optimal management depends on the involvement of a multi-disciplinary team that includes head and neck surgeons, interventional radiology and palliative care. CONCLUSIONS: RT plays a critical role in the multidisciplinary management of HNC oncological emergencies. Further prospective and comparative studies are needed to assess optimal management strategies.

Early respiratory interventional therapy combined with antifungal agent for endobronchial cryptococcosis: A case report and literature review.

RATIONALE: Cryptococcosis presenting as endobronchial obstruction and lung collapse is an extremely rare occurrence. While these patients were treated with antifungal agents, unfortunately, half of them showed a suboptimal response. PATIENT CONCERNS: A 45-year-old immunocompetent male was admitted to the hospital due to a cough, yellow phlegm, and dyspnea persisting for 5 months. Chest computer tomography revealed a mass in the right main bronchus accompanied by right lower lobe atelectasis. DIAGNOSES: Endobronchial cryptococcosis presenting as endobronchial obstruction and lung collapse. INTERVENTIONS: Early rigid bronchoscopic therapy was performed to resect endobronchial obstruction, which combined with antifungal agent. OUTCOMES: The patient recovered well with completely clinical and radiologic resolution at 1 year follow-up. LESSONS: This case provides a good example of successful utilization of the early respiratory interventional therapy combined with antifungal agent in obstructive endobronchial cryptococcosis.

Effective Differences between 2D and 3D Planned Brachytherapy in Lung Cancer: An Institutional Retrospective Study.

Background and Objectives: Advanced lung cancer is usually manifested by endoluminal tumor propagation, resulting in central airway obstruction. The objective of this study is to compare the high dose rate brachytherapy treatment outcomes in non-small-cell lung cancer (NSCLC) depending on the treatment planning pattern-two-dimension (2D) or three-dimension (3D) treatment planning. Materials and Methods: The study was retrospective and two groups of patients were compared in it (a group of 101 patients who underwent 2D planned high-dose-rate endobronchial brachytherapy (HDR-EBBT) in 2017/18 and a group of 83 patients who underwent 3D planned HDR-EBBT between January 2021 and June 2023). Results: In the group of 3D planned brachytherapy patients, there was a significant improvement in terms of loss of symptoms of bronchial obstruction (p = 0.038), but no improvement in terms of ECOG PS (European Cooperative Oncology Group Performance Status) of the patient (p = 0.847) and loss of lung atelectasis (if there was any at the beginning of the disease) (p = 0.781). Two-year overall survival and time-to-progression periods were similar for both groups of patients (p = 0.110 and 0.154). Fewer treatment complications were observed, and 91.4% were in 3D planned brachytherapy (BT) patients. Conclusions: Three-dimensionally planned HDR-EBBT is a suggestive, effective palliative method for the disobstruction of large airways caused by endobronchial lung tumor growth. Independent or more often combined with other types of specific oncological treatment, it certainly leads to the loss of symptoms caused by bronchial obstruction and the improvement of the quality of life of patients with advanced NSCLC. Complications of the procedure with 3D planning are less compared to 2D planned HDR-EBBT.

Association between heated tobacco product use and airway obstruction: a single-centre observational study, Japan.

BACKGROUND: While heated tobacco products (HTPs) may affect pulmonary function, the evidence supporting the utility of screening for HTP use in clinical settings is insufficient. We examined the association between HTP use and airway obstruction after switching from cigarettes. METHOD: The study subjects were patients aged ≥20 years undergoing surgery from December 2021 to September 2022 who completed spirometry and reported tobacco (cigarette and HTP) use status during the preoperative assessment. Airway obstruction was defined as forced expiratory volume in 1 s to forced vital capacity ratio below the lower limit of normal. Current tobacco use was defined as past-30-day use. Multivariable Poisson regression analysis was performed to examine the associations between HTP use and airway obstruction by adjusting for demographic characteristics, lifetime cigarette smoking (pack-year) and duration of smoking cessation. RESULTS: Overall (N=2850, 55.4% women, mean age 62.4), 4.6% and 10.7% reported current HTP use and cigarette smoking, respectively. 16.8% had airway obstruction. Airway obstruction was more common among current HTP-only users (adjusted prevalence ratio (APR)=2.32), current cigarette-only smokers (APR=2.57) and current dual users (APR=2.82) than never-tobacco users. Among current tobacco users (N=398), the prevalence of airway obstruction was not significantly different between HTP-only users and cigarette-only smokers. Among former cigarette smokers (>30-day cigarette quitters) (N=1077), current HTP users had 1.42 times the increased prevalence of airway obstruction than never-HTP users after adjusting for cigarette pack-year; a stronger association was observed when the analysis was restricted to ≥5-year cigarette quitters (N=772) (APR=1.96, vs never HTP users). CONCLUSION: Current HTP use was associated with airway obstruction among patients with cancer who had completely switched from cigarettes even after quitting smoking for a long period. Patients should be routinely screened for HTP use and advised to quit any tobacco.

Airway obstruction in two children with congenital central hypoventilation syndrome and review of the literature.

Congenital central hypoventilation syndrome (CCHS) is an autosomal dominant disease that is caused by heterozygous mutations in the paired-like homeobox 2B gene (PHOX2B). Madani et al. described an abnormally high degree of not only central apnea but also obstructive and mixed apnea in Phox2b27Ala/+newborn mice. Newborns with CCHS must undergo polysomnography for obstructive respiratory events in order to guide the optimal ventilation strategy if oxygen desaturation, bradycardia, and malaise persist under noninvasive ventilation. Newborns and infants with CCHS must be systematically tested for obstructive apnea, especially in cases of inefficient noninvasive ventilation.