Management of Persistent, Post-adenotonsillectomy Obstructive Sleep Apnea in Children: An Official American Thoracic Society Clinical Practice Guideline.

Background: Obstructive sleep apnea (OSA) is the most common sleep-related breathing disorder. Although adenotonsillectomy is first-line management for pediatric OSA, up to 40% of children may have persistent OSA. This document provides an evidence-based clinical practice guideline on the management of children with persistent OSA. The target audience is clinicians, including physicians, dentists, and allied health professionals, caring for children with OSA. Methods: A multidisciplinary international panel of experts was convened to determine key unanswered questions regarding the management of persistent pediatric OSA. We conducted a systematic review of the relevant literature. The Grading of Recommendations, Assessment, Development, and Evaluation approach was used to rate the quality of evidence and the strength of the clinical recommendations. The panel members considered the strength of each recommendation and evaluated the benefits and risks of applying the intervention. In formulating the recommendations, the panel considered patient and caregiver values, the cost of care, and feasibility. Results: Recommendations were developed for six management options for persistent OSA. Conclusions: The panel developed recommendations for the management of persistent pediatric OSA based on limited evidence and expert opinion. Important areas for future research were identified for each recommendation.

Sleep and respiratory outcomes in neonates with Pierre Robin sequence: a concise review.

There are no standardized management algorithms for neonates with Pierre Robin sequence. Currently available literature is variable in terms of outcomes assessed across studies. In this paper, we have aimed to summarize the currently available literature on longitudinal sleep and respiratory outcomes in Pierre Robin sequence neonates with a focus on identifying gaps in literature and areas for future research development.

Sleep Outcomes in Neonates with Pierre Robin Sequence Undergoing External Mandibular Distraction: A Longitudinal Analysis.

BACKGROUND: Robin sequence is a common cause of upper airway obstruction in newborns. Herein, we report sleep outcomes in neonates undergoing external mandibular distraction osteogenesis. METHODS: In this retrospective, 14-year, single-institution study of neonates with Robin sequence undergoing mandibular distraction osteogenesis, we compare respiratory parameters and sleep architecture before versus after surgery. RESULTS: Thirty-one neonates were included; age was 13 days (interquartile range, 5 to 34 days) at preoperative polysomnography and 80 days (interquartile range, 50 to 98 days) at postoperative polysomnography. All neonates had severe obstructive sleep apnea at baseline (defined as pre-operative obstructive apnea hypopnea index ≥ 10). Postoperatively, there was a significant reduction in obstructive apnea hypopnea index [38.3 (interquartile range, 23.4 to 61.8) preoperatively versus 9.4 (interquartile range, 5.3 to 17.1) postoperatively; p < 0.0001], and a significant improvement in sleep efficiency and oxygen saturation nadir. Although 26 neonates (84 percent) had a 50 percent reduction in obstructive apnea hypopnea index postoperatively, all neonates had obstructive sleep apnea, and 15 neonates (48 percent) had persistent severe obstructive sleep apnea following surgery. CONCLUSIONS: We report the largest cohort of sleep outcomes in neonates with Robin sequence and severe obstructive sleep apnea undergoing external mandibular distraction osteogenesis. Although the severity of obstructive sleep apnea improves postoperatively, the disease persists in all neonates. We propose that neonates undergo polysomnography before and soon after mandibular distraction osteogenesis to objectively assess improvement in obstructive sleep apnea, as they may require additional evaluation for sites of multilevel airway obstruction and treatment. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Longitudinal Sleep Outcomes in Neonates With Pierre Robin Sequence Treated Conservatively.

STUDY OBJECTIVES: Although Pierre Robin sequence (PRS) is a major cause of neonatal obstructive sleep apnea (OSA), longitudinal studies reporting evolution with age are lacking. This study aimed to describe changes in sleep-related respiratory parameters and sleep architecture in neonates with PRS treated conservatively (defined for this paper as treatment without tracheostomy or mandibular distraction). METHODS: A retrospective, 14-year, single-institution study of neonates with PRS who underwent diagnostic polysomnography (PSG) and at least one follow-up PSG. Those treated with surgery were excluded. Data were analyzed using a mixed-effects model with subject-specific random effect. RESULTS: In a cohort of 21 infants, baseline PSG (mean age 0.9 ± 0.7 months) showed a total apnea-hypopnea index (AHI) of 24.3 ± 3.6 events/h, obstructive apnea-hypopnea index (OAHI) of 13.4 ± 1.6 events/h, central apnea index of 10.2 ± 3.2 events/h, and an arousal index of 28.3 ± 1.3 events/h (variables reported as least square means ± standard error of the mean). There was a significant reduction in AHI, OAHI, arousal index, and percentage of REM sleep with advancing age. Although 71% of infants achieved full oral feeds by one month of age, some infants remained underweight during infancy. CONCLUSIONS: These neonates with PRS and OSA, treated conservatively, had an improvement in OAHI with advancing age with the median age of OSA resolution at 15 months. Factors potentially responsible include craniofacial growth and maturational changes of respiratory control. Further studies are necessary to determine the long-term effects of conservative management on growth and neurodevelopmental outcomes in these infants.

Management of Airway Obstruction in Infants With Pierre Robin Sequence.

BACKGROUND: Pierre Robin sequence (PRS)-related airway obstruction is often treated surgically; however, objective measures predicting the need for surgery are poorly defined. METHODS: A retrospective chart review was performed on 171 neonates with PRS. Infants were grouped based upon intervention modality: nonsurgical (conservative) or surgical [mandibular distraction osteogenesis (MDO) or tracheostomy]. Demographic data, physical examination findings, and study results were compared between groups to determine risk factors for surgical intervention, and to predict long-term success or failure of those interventions. RESULTS: The most significant, objective risk factor among those receiving surgery was a poor preintervention sleep study [obstructive index (OI): 42.4 versus 12.9 for the conservative treatment group; P < 0.001]. Only 11% of those treated conservatively had an OI >20, whereas 67.5% of those treated surgically met this severity measure. Of those receiving surgery, tracheostomy was associated with neurologic impairment (P = 0.030) and low birth weight (P = 0.046) compared with the MDO group. Together with syndromic status, these risk factors were useful for predicting failure of MDO to avoid subsequent tracheostomy (test sensitivity and specificity were 64.2% and 100.0%, respectively). No long-term differences in speech or micrognathia were detected between the 3 groups; however, those treated conservatively or with MDO had improved long-term feeding and airway obstruction outcomes compared with the tracheostomy group. CONCLUSIONS: Surgical intervention for PRS-related tongue-based airway obstruction should be strongly considered with an OI >20. Tracheostomy should be reserved for complex patients with concomitant syndromic diagnosis, neurologic impairment, and low birth weight.

Pediatric Obstructive Sleep Apnea.

Screening for obstructive sleep apnea (OSA) with in-laboratory polysomnography is recommended for children with sleep disordered breathing. Adenotonsillectomy is the first-line therapy for pediatric OSA, although intranasal steroids and montelukast can be considered for those with mild OSA and continuous positive airway pressure for those with moderate to severe OSA awaiting surgery, poor surgical candidates or persistent OSA. Bony or soft tissue upper airway surgery is reasonable for children failing medical management or those with persistent OSA following adenotonsillectomy. Weight loss and oral appliance therapy are also useful. A multi-modality approach to diagnosis and treatment is preferred.

Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacin.

Pseudomonas aeruginosa (PsA) is a highly prevalent bacterial organism recovered from the lungs of cystic fibrosis (CF) patients and chronic PsA infection is linked to progressive pulmonary function decline. The eradication and treatment of this organism from CF airways is particularly challenging to CF care providers. Aerosolized antibiotics that target PsA help to slow down growth, maintain lung function and reduce the frequency of pulmonary exacerbations. In this review, we discuss the currently available inhaled antibiotics for management of PsA lung infections in CF patients, with a focus on liposomal amikacin for inhalation (LAI). LAI is a unique formulation of amikacin under development that enhances drug delivery and retention in CF airways via drug incorporation into neutral liposomes. Factors such as once-daily dosing, mucus and biofilm penetration and potentially prolonged off-drug periods make LAI a potentially attractive option to manage chronic PsA lung infections in CF patients.

Nebulized liposomal amikacin for the treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients.

INTRODUCTION: Chronic lung infection with Pseudomonas aeruginosa (PsA) is associated with more-rapid pulmonary decline and acute pulmonary exacerbations in patients with cystic fibrosis (CF). The treatment and eradication of this organism from CF airways is particularly difficult, making it the focus of many therapeutic endeavors. Inhaled antibiotics used for PsA treatment help to suppress growth of the organism, maintain lung function and reduce the frequency of pulmonary exacerbations. AREAS COVERED: Herein, the authors discuss the currently available inhaled antibiotics for management of lung infections in CF patients. They also describe the recent results from clinical trials of agents under development, with a focus on liposomal amikacin for inhalation (LAI, Arikace™). The article also provides a summary of the results from relevant articles published in the English. EXPERT OPINION: LAI is a unique formulation of amikacin that enhances drug delivery and retention in CF airways via incorporation into neutral liposomes. Results of a recent Phase II trial suggest that LAI, with the capacity for once-daily dosing and prolonged off-drug periods, may be an attractive choice of inhaled antibiotic to manage PsA lung infections in CF patients. Further data from Phase III studies assessing the efficacy and safety of LAI should better elucidate its potential.