Airway and Feeding Outcomes in Pierre Robin Sequence: A Comparison of Three Management Strategies.

BACKGROUND: Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery. METHODS: All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate. RESULTS: 67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy. CONCLUSION: The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon.

A large collection of real-world pediatric sleep studies.

Despite being crucial to health and quality of life, sleep-especially pediatric sleep-is not yet well understood. This is exacerbated by lack of access to sufficient pediatric sleep data with clinical annotation. In order to accelerate research on pediatric sleep and its connection to health, we create the Nationwide Children's Hospital (NCH) Sleep DataBank and publish it at Physionet and the National Sleep Research Resource (NSRR), which is a large sleep data common with physiological data, clinical data, and tools for analyses. The NCH Sleep DataBank consists of 3,984 polysomnography studies and over 5.6 million clinical observations on 3,673 unique patients between 2017 and 2019 at NCH. The novelties of this dataset include: (1) large-scale sleep dataset suitable for discovering new insights via data mining, (2) explicit focus on pediatric patients, (3) gathered in a real-world clinical setting, and (4) the accompanying rich set of clinical data. The NCH Sleep DataBank is a valuable resource for advancing automatic sleep scoring and real-time sleep disorder prediction, among many other potential scientific discoveries.

Age-Dependent Responsiveness to Smoke Alarm Signals Among Children.

OBJECTIVES: Although it has been established that smoke alarms have more difficulty awakening children from sleep than adults, no attempt has been previously made to characterize how smoke alarm responsiveness changes with age during childhood. The objective of this study is to evaluate the age-dependent responsiveness to various smoke alarm signals among children 5 to 12 years old. METHODS: The effect of age on children's response to 4 types of smoke alarms (human voice, hybrid voice-tone, low-frequency tone, and high-frequency tone) was evaluated using combined data from 3 previous studies. RESULTS: There were 540 subjects (median age 9 years; 51.7% male). The proportion of children who awakened demonstrated a statistically significant (P < .001) increase of 3.1% to 7.6% for each additional year of age between 5 and 12 years old for the 4 alarm types. Similarly, child age showed a statistically significant (P < .001) effect on the proportion who escaped for each of the 4 alarm types. The proportion of subjects who awakened or escaped did not differ significantly by sex for any of the alarm types. Median time-to-awaken and median time-to-escape decreased with increase in child age for all alarm types. CONCLUSIONS: This study demonstrates the substantial influence of child age on the effectiveness of audible smoke alarms during childhood. Among 12-year-olds, only 56.3% escaped within 1 minute (and 67.6% within 2 minutes) to a high-frequency tone. However, a hybrid voice-low-frequency tone alarm is >96% effective at awakening and prompting escape within 1 minute among children 9 years and older.

What Are the Factors Affecting Total Sleep Time During Video Polysomnography in Infants?

OBJECTIVE: The aim of the study is to investigate factors affecting total sleep time (TST) during infant polysomnography (PSG) and assess if <4 hours of TST is sufficient for accurate interpretation. STUDY DESIGN: Overall, 242 PSGs performed in 194 infants <6 months of chronological age between March 2013 and December 2015 were reviewed to identify factors that affect TST, including age of infant, location and timing of study, presence of medical complexity, and presence of nasal tubes. A continuum of apnea-hypopnea index (AHI) in relation to TST was reviewed. Data were examined in infants who had TST <4 hours and low AHI. RESULTS: Greater TST (p < 0.001) was noted among infants during nocturnal PSGs, at older chronological and post-menstrual ages, and without medical complexity. The presence of nasogastric/impedance probes reduced TST (p = 0.002). Elevated AHIs were identified even in PSGs with TST <4 hours. Short TST may have affected interpretation and delayed initial management in one infant without any inadvertent complications. CONCLUSION: Clinical factors such as PMA and medical complexity, and potentially modifiable factors such as time of day and location of study appeared to affect TST during infant PSGs. TST < 4 hours can be sufficient to identify high AHI allowing physician interpretation. KEY POINTS: · Less than 4 hours of TST is enough for interpretation of infant polysomnography.. · Shorter TST appears related to infant age, medical complexity, and higher apnea-hypopnea index.. · Modifiable factors seen with higher TST were time of day, environment, and presence of nasal tubes..

Optimizing smoke alarm signals: Testing the effectiveness of children's smoke alarms for sleeping adults.

BACKGROUND: Being asleep is an important risk factor for death during a residential fire; however, the high-frequency tone smoke alarms in many homes will not adequately awaken children who are old enough to self-rescue. In a series of previous studies, we identified smoke alarm signals that effectively awaken children 5-12 years old and prompt their escape. Because it is impractical to have separate alarms for children and adults in a household, the purpose of this study is to test whether alarms that are effective in awakening children and prompting their escape are also effective among adults. METHODS: Using a randomized, non-blinded, repeated measures design, 150 adults 20-49 years old were exposed during stage 4 sleep to four different smoke alarms. Statistical tests included the Kaplan-Meier estimator, generalized Wilcoxon test, and hazard ratios with Wald's 95% confidence intervals. RESULTS: The median age of study subjects was 30.0 years and 67.3% were female. Almost all (n = 149) subjects awakened and performed the escape procedure to all four alarms; one individual did not awaken or escape to the high-frequency tone alarm. The median time-to-awaken was 2.0 s for the high-frequency tone alarm and 1.0 s for the other three alarms. The median time-to-escape for the high-frequency tone alarm was 12.0 s, compared with 10.0 s for the low-frequency tone alarm and 9.0 s each for the female and male voice alarms. All pairwise comparisons between the high-frequency tone alarm and each of the other three alarms were statistically significant for the probability functions for time-to-awaken and time-to-escape. There were no significant differences in these outcome measures between the latter three alarms, except for female voice versus low-frequency tone alarms for time-to-escape. CONCLUSIONS: All alarms performed well, demonstrating that smoke alarms developed for the unique developmental requirements of sleeping children are also effective among sleeping adults. Compared with a high-frequency tone alarm, use of these alarms may reduce residential fire-related injuries and deaths among children, while also successfully alerting adult members of the household.

Comparison of the effectiveness of female voice, male voice, and hybrid voice-tone smoke alarms for sleeping children.

BACKGROUND: To test whether children awaken from slow wave sleep and perform an escape procedure better in response to a smoke alarm that uses a male voice, female voice, combination of a low-frequency tone plus a female voice (hybrid alarm), or high-frequency tone. METHODS: Using a randomized, non-blinded, repeated-measures design, 188 children aged 5-12 years were exposed during stage 4 slow wave sleep to four smoke alarms. RESULTS: Among study subjects, 84.6%, 87.2%, 88.8%, and 56.4% awakened and 84.0%, 86.7%, 88.8%, and 55.3% successfully performed the escape procedure within 5 min of alarm onset in response to the male voice, female voice, hybrid, and high-frequency tone alarms, respectively, while the median time-to-escape was 12.0, 12.0, 13.0, and 96.5 s for these four alarms, respectively. All pairwise comparisons between the high-frequency tone alarm and each of the other three alarms were statistically significant for the proportions of subjects who awakened or escaped and for time-to-awaken and time-to-escape. There were no significant differences in these outcome measures between the latter three alarms. CONCLUSIONS: Use of the male or female voice or hybrid alarms in children's sleep areas may reduce residential fire-related injuries and deaths among children old enough to perform self-rescue. IMPACT: The male voice, female voice, and hybrid alarms were each significantly more effective than a high-frequency tone alarm in awakening children aged 5-12 years from slow wave sleep and prompting their performance of an escape procedure. There were no significant differences in the effectiveness of the male voice, female voice, and hybrid alarms when compared with each other. Use of these alarms in children's sleep areas may reduce residential fire-related injuries and deaths among children old enough to perform self-rescue.

Do Sleeping Children Respond Better to a Smoke Alarm That Uses Their Mother's Voice?

OBJECTIVE: Being asleep at the time of a residential fire increases fire-related fatality risk. This study tested whether children awaken from slow wave sleep and perform an escape procedure better to a voice smoke alarm that uses their mother's voice compared with a female stranger's voice or a low-frequency tone alarm. METHODS: Using a randomized, nonblinded, repeated measures design, 176 children 5 to 12 years old were exposed during stage 4 slow wave sleep to 4 smoke alarm signals: 1) the voice of the child's mother, 2) the voice of a female stranger, 3) low-frequency 500 Hz square wave tone, and 4) conventional residential high-frequency tone. The alarms were assessed regarding their ability to awaken the children and prompt their performance of an escape procedure. RESULTS: Among the 176 subjects, 78.4%, 83.0%, 88.1%, and 49.4% awakened and 78.4%, 81.3%, 85.8%, and 48.3% successfully performed the escape procedure within 5 minutes of alarm onset in response to the mother's voice, stranger's voice, low-frequency tone, and high-frequency tone alarms, respectively; while the median time-to-escape was 23.0, 24.0, 41.5, and >300 seconds for these 4 alarms, respectively. CONCLUSIONS: The 2 voice alarms and low-frequency tone alarm significantly outperformed the high-frequency tone alarm, with the low-frequency tone alarm and female stranger's voice alarm performing best. Compared with the voice of a female stranger, personalizing the alarm message with the voice of the child's mother did not increase alarm effectiveness. These findings provide important information for development of an effective and practical smoke alarm for children.

Pediatric obstructive sleep apnea screening questionnaire and post-operative outcomes: A prospective observational study.

INTRODUCTION: Obstructive Sleep Apnea (OSA) and Sleep Disordered Breathing (SDB) in children tend to be a more complex and multifactorial disease than in adults. Although adult screening tools, such as the STOP-BANG questionnaire, their application limited in pediatrics. We used our previously described 6-point questionnaire to identify OSA in children and evaluated its use for predicting post-operative respiratory events. METHODS: Children from 3 to 18 years of age presenting for surgery were eligible. Exclusion criteria were emergency surgery or refusal to participate. A 6-question survey regarding symptoms of OSA/SDB was administered preoperatively. Neck circumference was measured. Height and weight were recorded from preoperative data and the body mass index (BMI) percentile obtained. RESULTS: 749 patients were enrolled in the study. 707 patients were in the final analysis (359 boys and 348 girls, mean age 12 ±â€¯4 years). The median 6-item questionnaire score was 1 (interquartile range: 0, 2) and 186 (26%) scored ≥ 2 of 6 points. Children with predicted OSA (yes on ≥ 2 questions) were more likely than without predicted OSA to require supplemental oxygen in the PACU (24% vs. 17%; 95% confidence interval [CI] of difference: -0.3%, 13%; p = 0.049). Amongst 681 patients with available data on Post Anesthesia Care Unit (PACU) length of stay (LOS), prolonged LOS (>1 h) was not more likely among children with predicted OSA (42%) compared to those without predicted OSA (39%; 95% CI of difference: -5%, 11%; p = 0.479). Outcomes assessed after PACU discharge noted no differences. Specifically, overnight hospital stay was required in 33% of patients with predicted OSA as compared to 29% of those without (95% CI of difference: -4%, 11%; p = 0.399). On POD 0, supplemental oxygen was used on the inpatient ward for 6% of patients with predicted OSA compared to 4% of patients without predicted OSA (95% CI of difference: -2%, 6%; p = 0.272). CONCLUSION: The incidence of OSA/SDB is under-appreciated in children presenting for non-otolaryngological surgical procedures. Although patients judged to have OSA on the 6-item question may need for supplemental oxygen longer in the PACU, no other outcomes differences were noted.

A Randomized Trial of a Self-Administered Parenting Intervention for Infant and Toddler Insomnia.

Self-administered treatments may overcome access barriers to evidence-based care for pediatric sleep problems. Two hundred thirty-nine families participated in a randomized controlled trial with 3 study arms: a DVD intervention condition (the Sleep Easy Solution), a Website comparison condition, and a Wait-List comparison condition. The primary outcome was trichotomous-Do you consider your child's sleep a problem? (not a problem at all, a small problem, a very serious problem). DVD was superior to Wait-List in terms of the primary outcome ( P = .03; odds ratio = 0.44; 95% confidence interval = 0.21-0.93). Similarly, regarding secondary outcomes, DVD was superior to Wait-List in terms of longer continuous sleep periods ( P = .003), more favorable perceptions of the child's overall sleep ( P = .001), and higher parental confidence in managing the child's sleep ( P = .001). Results suggest that the DVD intervention is a promising self-administered treatment for pediatric insomnia.

Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.

BACKGROUND: Home oxygen therapy is often required in children with chronic respiratory conditions. This document provides an evidence-based clinical practice guideline on the implementation, monitoring, and discontinuation of home oxygen therapy for the pediatric population. METHODS: A multidisciplinary panel identified pertinent questions regarding home oxygen therapy in children, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach to rate the quality of evidence and strength of clinical recommendations. RESULTS: After considering the panel's confidence in the estimated effects, the balance of desirable (benefits) and undesirable (harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were developed for or against home oxygen therapy specific to pediatric lung and pulmonary vascular diseases. CONCLUSIONS: Although home oxygen therapy is commonly required in the care of children, there is a striking lack of empirical evidence regarding implementation, monitoring, and discontinuation of supplemental oxygen therapy. The panel formulated and provided the rationale for clinical recommendations for home oxygen therapy based on scant empirical evidence, expert opinion, and clinical experience to aid clinicians in the management of these complex pediatric patients and identified important areas for future research.

Effectiveness of a Voice Smoke Alarm Using the Child's Name for Sleeping Children: A Randomized Trial.

OBJECTIVES: To test maternal voice alarm effectiveness under residential conditions and determine whether personalizing the maternal voice alarm message with the child's first name improves effectiveness. STUDY DESIGN: Using a randomized, nonblinded, repeated measures design, we compared 3 maternal voice smoke alarms with respect to their ability to awaken 176 children 5-12 years old from stage 4 slow-wave sleep and prompt their performance of an escape procedure. A conventional residential high-frequency tone smoke alarm was used as a comparative reference. Children's sleep stage was monitored in a residence-like research setting. RESULTS: Maternal voice alarms awakened 86%-91% of children and prompted 84%-86% to escape compared with 53% awakened and 51% escaped for the tone alarm. A sleeping child was 2.9-3.4 times more likely to be awakened by each of the 3 voice alarms than the tone alarm. The median time to awaken was 156 seconds for the tone alarm and 2 seconds for each voice alarm. The proportions of children who awakened and escaped differed significantly between the tone alarm and each voice alarm, but no significant differences were found between each pair of the voice alarms, regardless of whether the child's first name was included in the alarm message. CONCLUSIONS: The maternal voice alarms significantly outperformed the tone alarm under residential conditions. Personalizing the alarm message with the child's first name did not increase alarm effectiveness. These findings have important implications for development of an effective and practical smoke alarm for children. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01169155.

Improving Communication Delay of Outpatient Sleep Study Results to Pediatric Otolaryngology Patients and Families.

OBJECTIVE: We undertook this quality improvement project to improve communication of outpatient pediatric sleep study results to families in a consistent and timely manner. METHODS: Based on the Institute for Healthcare Improvement quality improvement methodology, multiple key drivers were identified, including standardizing documentation and communication for sleep study results among the otolaryngology department, sleep center, and families. Meaningful interventions included developing standard electronic medical record documentation and utilizing otolaryngology nurses and advanced practice nurses to assist with communication by sending the results from the sleep center to both the referring otolaryngology provider and the triage nurses. The primary outcome measure was the monthly proportion of sleep studies communicated by the otolaryngology department to families within 3 business days. RESULTS: Average monthly sleep study results communicated to families within 3 business days increased from 31% to 92.9% over the study period ( P < .0001). Sleep study results were personally communicated via telephone and voicemail in 60.88% and 34.0% of cases, respectively. Approximately 50.0% of families receiving voicemails later contacted our department for their children's study results. DISCUSSION: Novel documentation strategies and involvement of our entire clinical team (physicians, nurses, and advanced practice nurses), allowed us to significantly improve the consistency and timeliness of our communication of outpatient sleep study results to families in a proactive manner. IMPLICATIONS FOR PRACTICE: With time-sensitive clinical test results, such as those from pediatric sleep studies, intra- and interdepartmental collaboration and standardization of the communication process and documentation may allow for more expedient care of children with suspected obstructive sleep apnea.

Sleep-disordered breathing and reaction time in children.

BACKGROUND: The incidence of obstructive sleep apnea (OSA) and sleep-disordered breathing (SDB) in children exceeds the availability of polysomnography (PSG) to definitively diagnose OSA and identify children at higher risk of perioperative complications. As sleep deficits are associated with slower reaction times (RTs), measuring RT may be a cost-effective approach to objectively identify SDB symptoms. AIM: The aim of this study is to compare RT on a standard 10-minute psychomotor vigilance test (PVT) based on children's history of OSA/SDB. METHODS: Children, 6-11 years of age, were enrolled from two different clinical groups. The SDB group included children undergoing adenotonsillectomy with a clinical history of SDB, OSA, or snoring. The control group included children with no history of SDB, OSA, or snoring who were scheduled for surgery other than adenotonsillectomy. RT was measured via 10-minute PVT (Ambulatory Monitoring Inc., Ardsley, NY, USA). Median RT was calculated for each patient based on all responses to stimuli during the PVT assessment and was compared to published age-sex-specific norms. The proportion of children exceeding RT norms was compared between study groups. RESULTS: The study included 72 patients (36/36 male/female, median age 7 years), 46 with SDB and 26 without SDB. There was no difference in the RT between the two groups. Fifty-four percent of patients with SDB exceeded norms for median RT vs 42% of control patients (95% CI of difference: - 12, 36; P=0.326). CONCLUSION: Approximately half of the patients in both groups exceeded published norms for median RT on PVT. Despite its convenience, measurement of RT did not distinguish between patients with probable SDB/OSA for preoperative risk stratification.

Sedation with Propofol for Bronchoscopy in Cystic Fibrosis Lung Transplant Recipients.

INTRODUCTION: Flexible fiberoptic bronchoscopy (FFB) plays an important role in the surveillance of cystic fibrosis (CF) patients after lung transplantation (LTx). With rapid onset and clearance, propofol provides a safe and efficient method for sedation during FFB, yet sedation requirements for CF patients are not well described. OBJECTIVES: Due to pharmacokinetic differences for other classes of drugs in CF patients, this study was performed to examine propofol requirements for sedation during bronchoscopy in lung transplant recipients with CF. METHODS: A single-center retrospective cohort study was performed to examine propofol sedation requirements during outpatient surveillance. FFB procedures with transbronchial biopsy (TBB) in post-LTx recipients between 2009 and 2014 were conducted. RESULTS: A total of 40 FFB procedures with TBB were performed 20 CF (11 females), 20 non-CF (11 females). Mean (± SD) age was 25.6 ± 9.2 (range 13-42) years and 22.2 ± 10.8 (range 11-39) years for the CF and non-CF groups, respectively. Propofol requirements were significantly higher in the CF patients compared to the non-CF patients. Mean (± SD) propofol dose for CF patients was 334 ± 86 versus 214 ± 88 mg for non-CF patients (p < 0.001). Mean (± SD) propofol dose per weight (mg/kg) was 6.5 ± 2.1 for CF patients versus 3.8 ± 1.6 for non-CF patients (p < 0.001). CONCLUSIONS: Compared to a non-CF cohort, CF lung transplant recipients required higher dosages of propofol for sedation during FFB with TBB.

Utility of screening questionnaire and polysomnography to predict postoperative outcomes in children.

INTRODUCTION: The prevalence of pediatric obstructive sleep apnea (OSA) has increased concurrently with the increasing prevalence of obesity. We have previously validated a short questionnaire predicting the occurrence of OSA on polysomnography (PSG). This follow-up study assessed the utility of the questionnaire in predicting postoperative outcomes. METHODS: Children undergoing surgery and completing a sleep study were prospectively screened for OSA using a short questionnaire. Procedures within 1 year of PSG were included in the analysis. Questionnaires were scored according to a cutoff previously deemed optimal for predicting OSA (apnea-hypopnea index ≥ 5) on the sleep study. Postoperative outcomes included prolonged (>60 min) length of stay (LOS) in the post-anesthesia care unit (PACU) and oxygen requirement in the PACU. RESULTS: The study cohort included 185 patients (100/85 male/female) age 8 ± 4 years, undergoing adenotonsillectomy (n = 109), other ear, nose, and throat (ENT) procedures (n = 18), or non-ENT procedures (n = 58). There were 45 patients with OSA documented by PSG and 122 patients identified as likely to have OSA according to questionnaire responses (89% sensitivity, 41% specificity). PACU LOS was prolonged in 55/181 (30%) cases and supplemental oxygen was used in the PACU in 29/181 (16%) cases. In separate multivariable models, supplemental oxygen use in the PACU was more common if a patient scored ≥2/6 points on the short questionnaire scale (OR = 5.0; 95% CI: 1.3, 19.9; p = 0.023) or if the patient was diagnosed with OSA on PSG (OR = 4.6; 95% CI: 1.6, 13.5; p = 0.005). Neither OSA on PSG nor questionnaire score ≥2/6 were associated with prolonged PACU stay. CONCLUSION: Both OSA diagnosis based on the AHI and the questionnaire scale achieved comparable predictive value for the need for oxygen use in the PACU. The utility of the questionnaire in predicting rare adverse events (e.g., unplanned admission or rapid response team activation) remains to be determined. Our preliminary results support using a brief questionnaire scale for preoperative risk stratification among children with suspected OSA who have not had a formal sleep study.

Impact of functional status and medical comorbidities on tracheostomy decannulation in pediatric patients.

PURPOSE: To determine if medical or functional factors influence the ability of a pediatric patient with a tracheostomy to tolerate decannulation. Retrospective evaluation of patients at a tertiary Children's Hospital undergoing evaluation with capped tracheostomy polysomnogram (cPSG) for possible tracheostomy decannulation. Charts were reviewed for demographic information, functional status, cPSG characteristics, and success or failure of decannulation. Statistical analysis was performed to determine which patient factors were predictive of successful decannulation. A total of 139 sleep studies were analyzed in 104 unique children followed for at least 1 year after a cPSG was performed to determine readiness for decannulation. At 1 year after most recent PSG, 79.8% of children were decannulated. There was no significant association between any single comorbid condition and the ability to decannulate. There was no association between individual or total functional status score and successful decannulation. Patients with at least 3 comorbid conditions investigated and a total functional score less than 7 were less likely to be decannulated successfully than other patients (71% vs. 93%, p= 0.04). Functional status and comorbid conditions do not independently predict successful decannulation. Regular multi-disciplinary team reevaluation is indicated in patients with lower functional status, as removal of tracheostomy tube may be successfully accomplished.

Airway and Feeding Outcomes of Mandibular Distraction, Tongue-Lip Adhesion, and Conservative Management in Pierre Robin Sequence: A Prospective Study.

BACKGROUND: Pierre Robin sequence is characterized by mandibular retrognathia and glossoptosis resulting in airway obstruction and feeding difficulties. When conservative management fails, mandibular distraction osteogenesis or tongue-lip adhesion may be required to avoid tracheostomy. The authors' goal was to prospectively evaluate the airway and feeding outcomes of their comprehensive approach to Pierre Robin sequence, which includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion. METHODS: A longitudinal study of newborns with Pierre Robin sequence treated at a pediatric academic medical center between 2010 and 2015 was performed. Baseline feeding and respiratory data were collected. Patients underwent conservative management if they demonstrated sustainable weight gain without tube feeds, and if their airway was stable with positioning alone. Patients who required surgery underwent tongue-lip adhesion or mandibular distraction osteogenesis based on family and surgeon preference. Postoperative airway and feeding data were collected. RESULTS: Twenty-eight patients with Pierre Robin sequence were followed prospectively. Thirty-two percent had a syndrome. Ten underwent mandibular distraction osteogenesis, eight underwent tongue-lip adhesion, and 10 were treated conservatively. There were no differences in days to extubation or discharge, change in weight percentile, requirement for gastrostomy tube, or residual obstructive sleep apnea between the three groups. No patients required tracheostomy. The greatest reduction in apnea-hypopnea index occurred with mandibular distraction osteogenesis, followed by tongue-lip adhesion and conservative management. CONCLUSIONS: Careful selection of which patients with Pierre Robin sequence need surgery, and of the most appropriate surgical procedure for each patient, can minimize the need for postprocedure tracheostomy. A comprehensive approach to Pierre Robin sequence that includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion can result in excellent airway and feeding outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

Effect of Severity of Esophageal Acidification on Sleep vs Wake Periods in Infants Presenting with Brief Resolved Unexplained Events.

OBJECTIVES: To describe the pattern of gastroesophageal reflux (GER) events in wake and sleep states with increasing acid reflux index (ARI) in neonates and to test the hypothesis that GER-related symptoms are frequent in ARI >7% in wake state. STUDY DESIGN: Infants underwent 24-hour pH-impedance studies with 6-hour concurrent video-polysomnography studies. Data were stratified based on the 24-hour ARI (% duration that esophageal pH is <4) into ARI < 3% (normal), ARI 3 ≥ to ≤7% (intermediate), and ARI >7% (abnormal). GER frequency, clearance mechanisms, and symptoms were distinguished during wake state and sleep state. RESULTS: Total wake and sleep duration was similar (P ≥ .2) in all ARI groups. Acidic events were frequent with increasing ARI in wake state vs sleep state (P ≤ .03). The symptom index increased with increasing ARI (P ≤ .02) in both wake state and sleep state. Acid clearance time increased with increasing ARI in wake state (P ≤ .02). In ARI > 7% vs ARI ≤ 7%, frequency of acidic GER events was higher (P ≤ .02) in wake state and sleep state; proximal migration of acid (P = .03) and acid clearance time were higher in wake state (P = .0005) only. Symptom index was higher in ARI >7% vs ARI ≤ 7% in wake state (P < .0001), comparable in normal vs intermediate (P = .4), and higher in abnormal vs intermediate (P = .0004) groups. CONCLUSIONS: Severe esophageal acid exposure (ARI >7%) is associated with increased reflux-associated symptoms in wake state. Sleep state appears to be protective regardless of ARI, likely because of greater chemosensory thresholds. Attention to posture and movements during wake state can be helpful. Scrutiny for non-GER etiologies should occur for infants presenting with life-threatening symptoms.