Drug-Induced Sleep Endoscopy Predicts Subjective Outcomes of Adenotonsillectomy.

OBJECTIVES: Persistent obstructive sleep apnea (OSA) after adenotonsillectomy (AT) has been reported in 20%-30% of children. The aim of this study was to determine the predictive value of drug-induced sleep endoscopy (DISE) at the time of AT on subjective AT outcomes. METHODS: This was a prospective cohort study of children aged 2-18 years being treated with AT for sleep disordered breathing (SDB) with one or more risk factors for AT failure: age >7 years, obesity, severe baseline OSA, Black race. All underwent DISE at the time of AT. Potential predictors of subjective AT outcome included age, sex, obesity, and DISE patterns. Multivariable linear regression was used to model predictors of post-AT Pediatric Sleep Questionnaire (PSQ) and OSA-18 outcomes. RESULTS: Pre- and post-AT PSQ/OSA-18 responses were available from 194 children. Mean age was 9.3 ± 3.5 years, with 59% obese, 50% female, and 67% White. After AT, mean PSQ score decreased from 0.60 ± 0.19 to 0.28 ± 0.22, p < 0.001 and mean OSA-18 score decreased from 66 ± 21 to 37 ± 18, p < 0.001. The most frequent sites of obstruction were the tonsils (92%), nasal airway (77%), adenoids (64%), and velopharynx (65%). Multivariable regression modeling demonstrated worse outcomes with obesity, male gender, and multilevel obstruction that included the nasal airway and tongue base in addition to adenotonsillar obstruction. CONCLUSIONS: Persistent OSA and symptom burden after AT was common in this population. Obesity, male gender, and multilevel obstruction during DISE were all associated with worse subjective outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Congenital Cytomegalovirus Testing Outcomes From the ValEAR Trial.

OBJECTIVE: To determine the positivity rate of congenital cytomegalovirus (cCMV) testing among universal, hearing-targeted CMV testing (HT-cCMV) and delayed targeted dried blood spot (DBS) testing newborn screening programs, and to examine the characteristics of successful HT-cCMV testing programs. STUDY DESIGN: Prospective survey of birth hospitals performing early CMV testing. SETTING: Multiple institutions. METHODS: Birth hospitals participating in the National Institutes of Health ValEAR clinical trial were surveyed to determine the rates of cCMV positivity associated with 3 different testing approaches: universal testing, HT-cCMV, and DBS testing. A mixed methods model was created to determine associations between successful HT-cCMV screening and specific screening protocols. RESULTS: Eighty-two birth hospitals were surveyed from February 2019 to December 2021. Seven thousand six hundred seventy infants underwent universal screening, 9017 infants HT-cCMV and 535 infants delayed DBS testing. The rates of cCMV positivity were 0.5%, 1.5%, and 7.3%, respectively. The positivity rate for universal CMV screening was less during the COVID-19 pandemic than that reported prior to the pandemic. There were no statistically significant drops in positivity for any approach during the pandemic. For HT-cCMV testing, unique order sets and rigorous posttesting protocols were associated with successful screening programs. CONCLUSION: Rates of cCMV positivity differed among the 3 approaches. The rates are comparable to cohort studies reported in the literature. Universal CMV prevalence decreased during the pandemic but not significantly. Institutions with specific order set for CMV testing where the primary care physician orders the test and the nurse facilitates the testing process exhibited higher rates of HT-cCMV testing.

When is pre-operative imaging required for craniofacial dermoid cysts/sinuses? A review.

OBJECTIVES: Dermoid cysts/sinuses (DCS) are congenital masses occurring along lines of embryonic fusion. Midline DCS carry a risk of intracranial extension. Pre-operative computed tomography (CT) or magnetic resonance imaging (MRI) are the primary imaging modalities used and based on the results, the need to involve a neurosurgical team in the resection is determined. Although less so, non-midline locations are also at risk for intracranial communication. This study aims to quantify our institutional experience with both midline and lateral DCS for intracranial extension and discuss potential need for preoperative imaging in all DCS cases. METHODS: Institutional Review Board approval was obtained. Pediatric patients ages 0-18 years with DCS presenting to the pediatric otolaryngology, plastic surgery, and neurosurgery clinics from 2005 to 2020 were retrospectively reviewed. Data collected included patient demographics, imaging modality, location, size, complications, and presence/absence of intracranial extension. DCS location included nasoethmoidal (NE), periorbital, frontotemporal (FT), and scalp. Lesions were further classified as midline and non-midline. RESULTS: 205 patients with surgically removed DCS were included for analysis. Mean age at surgery was 3 years. MRI was the most common imaging modality used (60.5%), followed by US (18%), CT (18%) and plain films (1%). Locations were: NE (69, 34%), periorbital (67, 33%), FT (28, 14%), and scalp (41, 20%). 105 DCS were midline: NE (69), periorbital (7), and scalp (29). Of these, 29 (28%) had intracranial extension: NE (8), scalp (21). 100 DCS were non-midline: periorbital (60), FT (28) and scalp (12). Of these, 7 (7%) had intracranial extension: periorbital (3), FT (3) and scalp (1). CONCLUSION: The risk of intracranial extension of midline craniofacial DCS is well established. We have shown that there is a percentage of lateral DCS which carry a risk for intracranial extension, and for which the involvement of a neurosurgical team may be required. Given the potential benefit, pre-operative imaging of all lateral head and neck DCS may be prudent to screen for intracranial extension.

A Systematic Review and Meta-Analysis: Timing of Elective Removal of Tympanostomy Tubes.

OBJECTIVE(S): Tympanostomy tube (TT) placement is the most common surgical procedure in children. Less than 10% of TT do not self-extrude. This study is a systematic review (SR) on elective TT removal in the pediatric population: timing, perforation rates, and role of simultaneous repair. A PICOTS (population, intervention, comparison, outcome, timing, setting) question was formulated: In pediatric patients who have retained TT, what is the preferred time to elective removal of such tubes, and what are the outcomes in terms of perforation rates? Does an intervention at the time of TT removal improve perforation rates? STUDY DESIGN: Systematic review and meta-analysis. METHODS: We searched four major electronic databases: EMBASE, MEDLINE, CDSR, CCRCT for articles published prior to 02/19/20. EndNote® was used to gather references, review abstracts, and obtain full text articles. Inclusion criteria were studies reporting patients aged 0 to 18 years undergoing elective TT removal with follow-up greater than 3 months. Exclusion criteria included patients >18 years, duplicate patient series, or case series with fewer than five patients. Articles that were not available in English, not available in full text, and those that only addressed long-acting TT were excluded. Data were pooled and meta-analysis was conducted to examine how timing of TT removal, patching of the tympanic membrane, or any TM intervention at TT removal affected outcomes. RESULTS: A total of 1,064 references were found. We identified 63 unique studies for full text review. Of these, 17 were selected for SR. MINORS (Methodological Index for Nonrandomized Studies) scores were low-revealing high bias among the studies. Reported perforation rates after elective TT removal ranged from 0% to 57%. Four studies had data suitable for comparative meta-analysis, which showed a significant increase in perforation rates after elective removal of TT after 3 years compared to removal prior to 3 years (OR 2.89; CI 1.78-4.69). No difference in perforation rates were identified when TM intervention vs. no intervention at time of TT removal was performed (six studies: OR 1.21; CI 0.71-2.07). No difference in perforation rates was identified when the type of TM intervention was compared, including freshening of TM edges, to patching with various materials (paper, fat, gelfoam®/gelfilm®, Trichloroacetic acid) (three studies: OR 1.07; CI 0.52-2.19). CONCLUSION: From the data reviewed in this SR and meta-analysis, elective TT removal at or prior to 3 years' retention showed decreased perforation rates. However, TM intervention at the time of TT removal was not shown to lower perforation rates. In the absence of tube complications such as granuloma formation, nonfunctional tube, or chronic tube otorrhea, it may be reasonable to wait up to 3 years to electively remove a retained TT. Laryngoscope, 132:2063-2070, 2022.

Drug-Induced Sleep Endoscopy Findings in Children With Obstructive Sleep Apnea With vs Without Obesity or Down Syndrome.

Importance: Persistent obstructive sleep apnea after adenotonsillectomy is common in children with Down syndrome or obesity. Drug-induced sleep endoscopy could help to identify anatomic differences in these patients that might affect surgical decision-making. Objective: To assess drug-induced sleep endoscopy findings in surgically naive children with obstructive sleep apnea with obesity or Down syndrome and compare these findings with children without obesity or Down syndrome. Design, Setting, and Participants: This cross-sectional analysis of data from a prospective cohort study of patients enrolled between May 1, 2015, and December 31, 2019, was conducted at an academic tertiary care children's hospital and included a consecutive sample of surgically naive children (age 2-18 years) who underwent drug-induced sleep endoscopy at the time of adenotonsillectomy for sleep-disordered breathing. Indications for sleep endoscopy included severe sleep apnea, age older than 7 years, obesity, African American race, and Down syndrome. Exposures: Drug-induced sleep endoscopy. Main Outcomes and Measures: Sleep endoscopy findings were scored according to the Sleep Endoscopy Rating Scale. Ratings at 6 anatomic levels for children with obesity and those with Down syndrome were compared with controls without obesity or Down syndrome using several measures of effect size (Cohen d, Cramer V, and η2). Results: A total of 317 children (158 girls [50%]; 219 [69%] White, 20 [6%] Black, and 103 [34%] Hispanic; mean [95% CI] age, 9.6 [9.2-10.0] years) were included, of whom 115 (36%) were controls without obesity or Down syndrome, 179 (56%) had obesity without Down syndrome, and 23 (7%) had Down syndrome. The mean apnea-hypopnea index was 16 (95% CI, 13-19), and the mean minimum O2 saturation was 83% (95% CI, 81%-85%). Compared with controls without obesity or Down syndrome, children with Down syndrome demonstrated greater overall obstruction (mean sleep endoscopy rating scale total score of 5.6 vs 4.8; Cohen d, 0.46), and greater tonsillar (percentage of complete obstruction: 65% vs 54%), tongue base (percentage of complete obstruction: 26% vs 12%), and arytenoid obstruction (percentage of at least partial obstruction, 35% vs 6%). Children with obesity had greater tonsillar (percentage of complete obstruction, 74% vs 54%) and less base of tongue obstruction (percentage of complete obstruction, 2% vs 12%) compared with controls. Conclusions and Relevance: In this cohort study, surgically naive children with obesity with obstructive sleep apnea had predominantly tonsillar obstruction, whereas children with Down syndrome demonstrated greater obstruction of the tonsils, tongue base, and arytenoids compared with controls. Routine drug-induced sleep endoscopy should be considered in surgically naive children with Down syndrome to help inform the surgical plan.

Endoscopic ear surgery in children with down syndrome.

INTRODUCTION: Children with Down syndrome (DS) have a high incidence of chronic middle ear disease. Surgery to manage this disease is challenging due to the severity of illness and narrow ear canal dimensions. Endoscopic ear surgery is used to manage tympanic membrane and middle ear disease with the advantages of improved visualization and avoidance of post-auricular incisions. However, its application in children with DS has not been reported. We aim to compare the outcomes of endoscopic versus microscopic ear surgery in children with DS. METHODS: All patients with DS who underwent tympanoplasty without mastoidectomy between 2012 and 2018 were identified, and their charts retrospectively reviewed. Rate of residual perforation, hearing, surgical time, and surgical details were recorded. RESULTS: 37 surgeries in 26 patients were identified that met inclusion criteria. Two subgroups were analyzed. The first included 14 cases that were done using traditional microscopic visualization (MV). The second included 17 cases that had substantial or exclusive use of endoscopic visualization (EES). Due to a learning curve, the number of cases done endoscopically increased over time. The average age in MV was 13.9 years vs 11.0 in EES. The MV cases included 2 with cholesteatoma vs 4 in EES. In cases with adequate follow up, residual perforations were found in 1/13 MV, and 4/17 EES. All of the residual perforation cases in EES used acellular porcine submucosa grafts. None of the cases in MV used this material. Average air bone gap reduction was seen in both groups; 4.2 dB in MV, 9.8 dB in EES. Average surgical time was similar between groups; 124 min in MV, 115 min in EES. All cases in MV required a post-auricular incision and approach to the middle ear. Only four cases in EES required this approach. Six cases in EES did not require any incision outside of the ear canal for either graft harvest or middle ear approach. CONCLUSION: Endoscopic and microscopic ear surgery in children with DS have similar outcomes. There were no statistical differences in hearing results, surgical times, or residual tympanic membrane perforations, although the rate of perforations in the endoscopic group trended higher. Most endoscopic cases did not require conversion to a post-auricular approach. Endoscopic surgery allows some DS patients to avoid any incision outside of the ear canal.

Hamartomas and midline anomalies in association with infantile hemangiomas, PHACE, and LUMBAR syndromes.

BACKGROUND/OBJECTIVE: The pathogenesis of infantile hemangiomas (IH), PHACE, and LUMBAR syndromes remains unknown. We aim to describe histopathologic features of midline anomalies associated with IH, including patients with PHACE and LUMBAR syndromes. METHODS: A multicenter retrospective chart review was performed to identify patients with IH, PHACE, and LUMBAR syndrome with histopathologic specimens from sternal or midline anomalies. A total of 18 midline lesions from 13 patients were included. Out of 18, 14 midline lesions underwent both histopathologic and clinical review. Three hamartoma-like chin plaques and one supraumbilical raphe underwent only clinical review. RESULTS: All 13 patients had midline lesions and IH. Histopathologic diagnoses were as follows: rhabdomyomatous mesenchymal hamartoma (3), folliculosebaceous cystic hamartoma (1), fibroepithelial polyp (1), verrucous epidermal hyperplasia with vascular proliferation and fibroplasia (1), congenital midline cervical cleft (1), pericardium with fibrosis (1), fibrous components with increased collagen (1), atrophic skin/membrane (3), angiolipomatous mass with neural components (1), and lipomatous mass (1). Due to the retrospective nature of this study, it was not possible to obtain pathology slides for all midline lesions that had previously been biopsied or resected. We show clinically and histopathologically a new association between PHACE syndrome and rhabdomyomatous mesenchymal hamartoma (RMH), in addition to demonstrating the association between PHACE syndrome and chin hamartomas. We also display histopathologic findings seen in midline lesions resected from LUMBAR patients. CONCLUSION: Rhabdomyomatous mesenchymal hamartoma is thought to be related to aberrations of mesenchymal cells during development; therefore, this may provide clues to the pathogenesis of IH and related syndromes.

Laryngomalacia and Swallow Dysfunction.

OBJECTIVES: Laryngomalacia is an established cause of stridor and sleep-disordered breathing in children. However, the relationship between laryngomalacia and dysphagia has not been well characterized. The objectives of this study were to (1) describe the patient characteristics, symptoms, and prevalence of dysphagia in children with laryngomalacia and (2) examine the effectiveness of supraglottoplasty in improving feeding. METHODS: This was a retrospective study of patients with laryngomalacia who underwent a modified barium swallow study (MBSS) at a tertiary academic pediatric medical center between March 1, 2014, and March 1, 2018. Patients were excluded if they did not undergo a MBSS. Comorbidities, airway and feedings symptoms, MBSS results, and surgical history were recorded from each patient's electronic medical record. RESULTS: Forty-four children met inclusion/exclusion criteria. The median age at presentation was 96 days. There was a male predominance (66%). About one-third had a genetic or neuromuscular comorbidity. Most children had stridor (93%) and feeding difficulty (86%), while 50% had parent-reported poor weight gain. Fifty-seven percent of patients had evidence of penetration or aspiration on MBSS. All patients with a positive MBSS had dysphagia symptoms. Fifty-seven percent of patients underwent supraglottoplasty. Postoperatively, 92% reported improvement in dysphagia symptoms. CONCLUSION: Dysphagia is prevalent among a subset of children with laryngomalacia. Symptomatic children may benefit from a swallow evaluation to help determine the need for further intervention. Children who undergo supraglottoplasty for laryngomalacia have improved dysphagia at follow-up, though the amount of improvement directly attributable to surgery is unclear and warrants further investigation.

Simultaneous intra-operative sclerotherapy and surgical resection of cervicofacial venous malformations.

OBJECTIVES: To review simultaneous intra-operative sclerotherapy (IOS) with immediate surgical resection for the treatment of cervicofacial venous malformations (VMs) at a single institution. While pre-operative sclerotherapy (POS) has been reported in the literature, simultaneous intra-operative sclerotherapy and surgery in the operating room has not. METHODS: The database from the Hemangioma and Vascular Birthmarks Clinic was reviewed. All patients in both groups had biopsy-proven VMs. RESULTS: IOS was used in 11 surgical patients with average age 17 years. Sclerotherapy was performed with sodium tetradecyl sulfate 3%, absolute alcohol or bleomycin. Immediately after IOS, and under the same anesthetic, all patients had either complete resection or debulking of the VMs. Eight patients had complete resolution of their VM and 3 had improvement. Average duration of the combined procedures done under a single anesthetic was 121 min. The POS approach was used for 6 surgical patients with average age 7 years. Sclerotherapy agents used were absolute alcohol or sodium tetradecyl sulfate 3%. All patients underwent complete resection of the VM 24-72 h after sclerotherapy under a separate surgical session. Five patients experienced complete resolution of their VM and one has had further sclerotherapy for recurrent disease. Interventional Radiology suite sclerotherapy times were on average 70 min. Surgical times were on average 142 min. Total combined anesthesia times for the two procedures added together were 212 min. Treatment time was significantly shorter in the IOS group (p = 0.0015). CONCLUSIONS: Simultaneous IOS at the time of surgical resection has been successful in our hands. IOS has the advantage of a single procedure and decreased cost to the patient. In the era of reducing pediatric exposure to anesthesia, this approach is especially attractive in the pediatric population. As well, at approximately $100/minute cost to the patient to be in either the Interventional Radiology Suite or in the operating room, the reduced length of the procedures seen in the IOS approach results in lower overall cost to the patient.

Multicenter Interrater Reliability in the Endoscopic Assessment of Velopharyngeal Function Using a Video Instruction Tool.

OBJECTIVE: Assess interrater agreement of endoscopic assessment of velopharyngeal (VP) function before and after viewing the video instruction tool (VIT). We hypothesized improvement in interrater agreement using the Golding-Kushner scale (GKS) after viewing the VIT. STUDY DESIGN: Prospective study. SETTING: Multi-institutional. METHODS: Sixteen fellowship-trained pediatric otolaryngologists who treat velopharyngeal insufficiency (VPI) rated 50 video segments using the GKS before and after watching the VIT. Raters assessed gap size percentage and lateral pharyngeal wall (LPW), soft palate (SP), and posterior pharyngeal wall (PPW) movement. Intraclass correlation coefficient was estimated for these continuous measures. Raters also indicated the presence of a palatal notch, Passavant's ridge, and aberrant pulsations (categorical variables). Fleiss κ coefficient was used for categorical variables. Wilcoxon signed-rank test was performed on the difference between the pre/post individual video ratings. RESULTS: Reliability improved for all continuous variables after watching the instructional video. The improvement was significant for PPW (0.22-0.30, P < .001), SP (left: 0.63-0.68, P < .001 and right: 0.64-0.68, P = .001), and LPW (left: 0.49-0.54, P = .01 and right: 0.49-0.54, P = .09) but not significant for gap size (0.65-0.69, P = .36). Among categorical variables, agreement on Passavant's ridge significantly improved (0.30-0.36, P = .03). CONCLUSION: Exposure to a video instruction tool improves interrater agreement of endoscopic assessment of VP function. Significant improvement was observed in our primary end points, specifically posterior pharyngeal wall movement, soft palate movement, and lateral pharyngeal wall movement. There was less impact of the VIT on the interrater agreement of the categorical variables, palatal notch, Passavant's ridge, and aberrant pulsations.

Is Routine Electrocardiography Necessary Before Initiation of Propranolol for Treatment of Infantile Hemangiomas?

BACKGROUND: In recent years propranolol has become the treatment of choice for infantile hemangiomas (IHs). There is broad variation in the approach to propranolol initiation in clinical practice. This retrospective study explored the effectiveness of routine pre-treatment ECG in screening infants being considered for systemic treatment with propranolol. METHODS: All patients seen in the outpatient pediatric dermatology clinics at Oregon Health and Sciences University (OHSU) and The Mayo Clinic Rochester (MCR), as well as those seen in multidisciplinary vascular anomalies clinics, who had ECGs obtained prior to planned initiation of propranolol for treatment of IH from 2008 to 2013, were identified. A total of 162 patients were included in the study. RESULTS: We found that 43% (69) of routine ECGs were read as abnormal, leading to 28 formal consultation appointments with pediatric cardiologists. After either formal consultation or informal discussion with cardiology, no patients with initially "abnormal" ECGs were ultimately excluded from treatment with propranolol based on routine ECG findings. Additionally no patients in our cohort experienced an adverse effect during treatment that could have been predicted or prevented by ECG prior to initiation of the propranolol. CONCLUSION: Our findings suggest that routine ECG may not be necessary or helpful in the vast majority of patients treated with propranolol for IHs.

Quality of life improvement after pressure equalization tube placement in Down syndrome: A prospective study.

OBJECTIVE: To evaluate quality-of-life changes after bilateral pressure equalization tube placement with or without adenoidectomy for the treatment of chronic otitis media with effusion or recurrent acute otitis media in a pediatric Down syndrome population compared to controls. STUDY DESIGN: Prospective case-control observational study. METHODS: The OM Outcome Survey (OMO-22) was administered to both patients with Down syndrome and controls before bilateral tube placement with or without adenoidectomy and at an average of 6-7 months postoperatively. Thirty-one patients with Down syndrome and 34 controls were recruited. Both pre-operative and post-operative between-group and within-group score comparisons were conducted for the Physical, Hearing/Balance, Speech, Emotional, and Social domains of the OMO-22. RESULTS: Both groups experienced improvement of mean symptom scores post-operatively. Patients with Down syndrome reported significant post-operative improvement in mean Physical and Hearing domain item scores while control patients reported significant improvement in Physical, Hearing, and Emotional domain item scores. All four symptom scores in the Speech domain, both pre-operatively and post-operatively, were significantly worse for Down syndrome patients compared to controls (p ≤ 0.008). CONCLUSIONS: Surgical placement of pressure equalizing tubes results in significant quality of life improvements in patients with Down syndrome and controls. Problems related to speech and balance are reported at a higher rate and persist despite intervention in the Down syndrome population. It is possible that longer follow up periods and/or more sensitive tools are required to measure speech improvements in the Down syndrome population after pressure equalizing tube placement ± adenoidectomy.

Use of intravenous propranolol for control of a large cervicofacial hemangioma in a critically ill neonate.

Cervicofacial segmental infantile hemangiomas (IH) may result in airway obstruction requiring use of propranolol to induce hemangioma regression and reestablish the airway. We present the first case using intravenous (IV) propranolol for control of airway obstruction and rapid expansion of cervicofacial IH in the setting of necrotizing enterocolitis (NEC) impaired gastrointestinal function. Intravenous dosing of propranolol was tolerated well in a critically ill neonate with multisystem complications of prematurity.

Implementation of a Pediatric Posttonsillectomy Pain Protocol in a Large Group Practice.

OBJECTIVE: In response to the increased risk of respiratory failure and death after tonsillectomy related to codeine use, Kaiser Permanente Northwest restricted use of opioids in patients <7 years old via electronic health record (EHR). However, opioids could be prescribed at physician discretion by overriding the EHR. This study aims to examine protocol compliance in a large group practice using EHR order sets and complication rates as compared with historical data. STUDY DESIGN: Case series with chart review. SETTING: Ambulatory care within a health maintenance organization. SUBJECTS AND METHODS: Procedural codes were used to identify children <7 years old who underwent tonsillectomy or adenotonsillectomy approximately 1.5 years before and after implementation of EHR protocol (n = 437). Primary outcome was opioid pain prescriptions received by patients. Secondary outcomes were emergency or urgent care utilization, postoperative bleeding, nausea, vomiting, dehydration, death, and reasons for prescribing opioid pain medication after EHR protocol implementation. Chi-square analysis and Fischer's exact testing were used to compare differences in event rates. RESULTS: Implementation of an age-based narcotic protocol significantly decreased physician narcotic prescribing from 82.2% to 15.4% (P < .0001). The most common reason for narcotic prescription after the intervention was the report of inadequate pain control by phone call (35%). There was no significant difference in rate of emergency or urgent care utilization between pre- and postimplementation groups (4% vs 6%, P = .29). CONCLUSIONS: Implementation of an age-based narcotic restriction for posttonsillectomy patients using an EHR order set is an effective and safe way to influence physician prescription practices.

Assessment of pediatric obstructive sleep apnea using a drug-induced sleep endoscopy rating scale.

OBJECTIVES/HYPOTHESIS: Assess the reliability of a Sleep Endoscopy Rating Scale (SERS) and its relationship with pediatric obstructive sleep apnea (OSA) severity. STUDY DESIGN: Retrospective case series of pediatric patients who underwent drug-induced sleep endoscopy (DISE) at the time of surgery for OSA from January 1, 2013 to May 1, 2014. METHODS: Three blinded otolaryngologists scored obstruction on DISE recordings as absent (0), partial (+1), or complete (+2) at six anatomic levels: nasal airway, nasopharynx, velopharynx, oropharynx, hypopharynx, and arytenoids. Ratings were summed for a SERS total score (range, 0-12). Reliability was calculated using a κ statistic with linear weighting. SERS ratings and obstructive apnea-hypopnea index (OAHI) were compared using Spearman correlation. A receiver operating characteristic (ROC) analysis determined the ability of the SERS total score to predict severe OSA (OAHI >10). RESULTS: Thirty-nine patients were included (mean age, 8.3 ± 5.1 years; 36% obese; mean OAHI, 19.1 ± 23.7). Intrarater and inter-rater reliability was substantial-to-excellent (κ = 0.61-0.83) and fair-to-substantial (κ = 0.33-0.76), respectively. Ratings correlated best with OAHI for the oropharynx (r = 0.54, P = .02), hypopharynx (r = 0.48, P = .04), and SERS total score (r = 0.75, P = .002). In ROC analysis, a SERS total score ≥6 demonstrated sensitivity/specificity of 81.8%/87.5%, respectively, and correctly classified 84% of patients. CONCLUSIONS: The SERS can be applied reliably in children undergoing DISE for OSA. Ratings of the oropharynx, hypopharynx, and SERS total score demonstrated significant correlation with OSA severity. A SERS total score ≥6 was an accurate predictor of severe OSA. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1492-1498, 2016.

Propranolol-responsive cranial nerve palsies in a patient with PHACES syndrome.

PHACES syndrome is a neurocutaneous disorder characterized by the presence of segmental hemangiomas with associated anomalies of the posterior fossa, cerebral vasculature, cardiovascular system, eyes, and ventral or midline structures. We present the first case of propranolol-responsive congenital trigeminal and facial nerve palsies secondary to an intracranial hemangioma in a patient with PHACES syndrome.

Predisposition to Childhood Otitis Media and Genetic Polymorphisms within the Toll-Like Receptor 4 (TLR4) Locus.

BACKGROUND: Predisposition to childhood otitis media (OM) has a strong genetic component, with polymorphisms in innate immunity genes suspected to contribute to risk. Studies on several genes have been conducted, but most associations have failed to replicate in independent cohorts. METHODS: We investigated 53 gene polymorphisms in a Finnish cohort of 624 cases and 778 controls. A positive association signal was followed up in a tagging approach and tested in an independent Finnish cohort of 205 cases, in a British cohort of 1269 trios, as well as in two cohorts from the United States (US); one with 403 families and the other with 100 cases and 104 controls. RESULTS: In the initial Finnish cohort, the SNP rs5030717 in the TLR4 gene region showed significant association (OR 1.33, P = .003) to OM. Tagging SNP analysis of the gene found rs1329060 (OR 1.33, P = .002) and rs1329057 (OR 1.29, P = .003) also to be associated. In the more severe phenotype the association was stronger. This finding was supported by an independent Finnish case cohort, but the associations failed to replicate in the British and US cohorts. In studies on TLR4 signaling in 20 study subjects, the three-marker risk haplotype correlated with a decreased TNFα secretion in myeloid dendritic cells. CONCLUSIONS: The TLR4 gene locus, regulating the innate immune response, influences the genetic predisposition to childhood OM in a subpopulation of patients. Environmental factors likely modulate the genetic components contributing to the risk of OM.

Outcomes of tympanostomy tube placement in children with Down syndrome--a retrospective review.

OBJECTIVES: Tympanostomy tubes are commonly used for treatment of chronic otitis media with effusion (COME) or recurrent acute otitis media (RAOM) in patients with Down syndrome, but hearing outcomes in this population have been mixed, and complications appear to be common. We aim to characterize outcomes and complications associated with tympanostomy tube placement in this population. METHODS: Retrospective review. All patients with Down syndrome presenting to a tertiary academic pediatric otolaryngology practice over a ten year period from 2002 to 2012 who received tympanostomy tubes for COME, RAOM, or hearing loss were reviewed. RESULTS: Long term follow up data was obtained in 102 patients, with average follow up 4.7 years. COME was the primary indication for tube placement in 100/102 (98%). Less than half of these patients (44%) initially failed their newborn hearing screen. Post operative hearing was found to be normal or near normal for the better hearing ear in 85/99 (85.9%), and normal to near normal in bilateral ears in 71/99 (71%). A majority (63.7%) of patients required two or more sets of tubes during the follow up period. Long term complications were common and were significantly increased if the patient required three or more sets of tubes, including chronic perforation (36.6% vs 8.2%, p<0.001), atelectasis (29.3% vs 1.6%, p<0.0001), and cholesteatoma (14.6% vs 0%, p=0.003). CONCLUSIONS: COME is a frequent problem in Down syndrome, and the majority of patients will require two or more sets of tubes during their childhood and achieve normal postoperative hearing. Long term complications of otitis media appear to be more common in this population and appear to correlate with increasing number of tubes placed. More investigation is required to determine optimal treatment strategies for COME in patients with Down syndrome.

Prenatal diagnosis of fetal cervicofacial anomalies.

PURPOSE OF REVIEW: To review the current literature on prenatal diagnosis of cervicofacial anomalies that may require neonatal intervention or that require prenatal counseling by a maternal-fetal medicine team and otolaryngology, head and neck surgery. RECENT FINDINGS: Ultrasound and MRI imaging are complementary in the prenatal diagnosis of fetal anomalies that may present with the need for the head and neck surgeon to assist with airway management at delivery or that require prenatal counseling. Team approaches to delivery of at-risk infants have improved and there is more experience with the ex-utero intrapartum treatment (EXIT) procedure. The importance of planning and simulation for EXIT is essential prior to delivery. Future directions include in-utero gene therapy and fetal surgery. SUMMARY: Advances in detection and treatment of fetuses at risk for airway obstruction at birth and for accurate diagnosis of facial clefting will allow teams to continue to improve outcomes for these infants. Increased experience of such teams will allow refinement of protocols and indices for decision making regarding which fetuses will need treatment on placental support and which will do well with traditional delivery with treatment teams on standby. This will allow improved risk profiles for both mother and child.

Innate immunity gene single nucleotide polymorphisms and otitis media.

OBJECTIVE: Toll-like receptors (TLR) activate the innate immune system. Single nucleotide polymorphisms (SNPs) in TLR genes are linked to increased susceptibility to infections. TLR4-deficient mice have increased incidence and duration of otitis media. We hypothesize that SNPs in TLR genes are more common in otitis-prone children than in children without a history of otitis media. METHODS: Cases (n=70) included children undergoing surgery for otitis media. Control subjects (n=70) included children undergoing surgery for non-otologic indication. Genomic DNA was extracted from blood samples. RT-PCR genotyping was performed for TLR2 (rs5743708), TLR4 (rs4986790 and rs4986791), TLR9 (rs5743836 & rs187084), and CD14 (rs2569190). RESULTS: There were no significant differences between the groups in family history, day care, smoke exposure, allergies or prevalence of the SNPs. The most common pre-op diagnosis in control subjects was obstructive sleep apnea (OSA). CONCLUSIONS: TLR2, TLR4, TLR9 and CD14 gene SNPs were not more prevalent in otitis-prone children.