Pediatric Postoperative Outcomes for Severe and Very Severe Obstructive Sleep Apnea Syndrome.

OBJECTIVE: To determine if increasing obstructive sleep apnea syndrome (OSAS) severity, as determined by preoperative polysomnography data, is an independent risk for respiratory complications and level of follow-up care after adenotonsillectomy or tonsillectomy. METHODS: A retrospective analysis of patients ≤21 years of age with severe OSAS (obstructive apnea-hypopnea index [OAHI] >10) undergoing adenotonsillectomy or tonsillectomy. Patients were categorized based on preoperative polysomnography data (PSG). Outcome measures including respiratory complications were collected via chart review. Logistic regression was used in the analysis of all parameters, and Wilcoxon Rank Sum tests were used for analysis of both OAHI and oxygen saturation nadir as continuous variables. All surgeries were performed at Johns Hopkins Hospital, a tertiary care center. RESULTS: We identified 358 patients with severe OSAS who had adenotonsillectomy or tonsillectomy. OAHI >40 and oxygen saturation nadir <80% were significantly associated with postoperative respiratory complications. Increasing OAHI and O2 saturation <80% was each associated with unplanned continuous positive airway pressure (CPAP) initiations postoperatively. There was no association between hypercarbia and presence of any complications. CONCLUSION: Patients with very severe OSAS (preoperative OAHI ≥40) as determined by preoperative PSG may be at higher risk of developing respiratory complications postoperatively. However, there does not appear to be a linear association with increasing severity of OAHI on regression analysis. Further research is needed to understand factors associated with complications in severe and very severe OAHI. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

ACE2 and TAS2R38 receptor expression in pediatric and adult patients in the nasal and oral cavity.

Objective: To investigate differences in angiotensin-converting-enzyme-2 (ACE2) and bitter taste receptor (TAS2R38) expression between patient age groups and comorbidities to characterize the pathophysiology of coronavirus 19(COVID-19) pandemic. ACE2 is the receptor implicated to facilitate SARS-CoV-2 infections and levels of expression may correlate to the severity of COVID-19 infection. TAS2R38 has many non-gustatory roles in disease, with some evidence of severe COVID-19 disease in certain receptor phenotypes. Methods: We conducted a prospective cohort study and collected nasal and lingual tissue from healthy pediatric (n = 22) and adult (n = 25) patients undergoing general anesthesia for elective procedures. RNA isolation and qPCR were performed with primers targeting ACE2 and TAS2R38. Results: A total of 25 adult (52% male; 44% obese) and 22 pediatric (50% male; 36% obese) patients were enrolled, pediatric tissue had 43% more nasal ACE2 RNA expression than adults with a median fold change of 0.69 (IQR 0.37, 0.98) in adults and 0.99 (IQR 0.74, 1.43) in children (p < .05). There were no differences between the age groups in ACE2 expression of lingual tissue (p = .14) or TAS2R38 expression collected from either nasal (p = 049) or lingual tissue (p = .49). Stratifying for obesity yielded similar differences between nasal ACE2 expression between adults and children with median fold change of 0.56 (IQR 0.32, 0.87) in adults and 1.0 (IQR 0.82, 1.52) in children (p < .05). Conclusions: ACE2 receptor expression is higher in nasal tissue collected from children compared to adults, suggesting COVID-19 infectivity is more complicated than ACE2 and TAS2R38 mRNA expression. Level of Evidence: NA.

Upper lip tie: A novel classification scale with improved inter-rater reliability.

Objectives: Upper lip tie (ULT) is a clinical condition with restrictive attachment of the superior labial frenulum (SLF), which may inhibit flanging of the lips. Objective outcome studies are lacking in part due to unreliable classification systems that rely solely upon a single attachment parameter of the SLF. This study's objectives are to describe a novel 3-point classification system for ULT and compare its inter-rater reliability to the Kotlow and Stanford systems. Methods: Five raters used the Kotlow and Stanford systems, as well as our novel 3-point scale to score images of the SLF from 20 newborns seen at our institution between September 1, 2017 and April 1, 2018. Newborn birth weight, gestational age, and demographic data were collected from the infant's medical record. Fleiss's kappa was used to calculate inter-rater reliability for all classification systems. Results: The parameters for our novel 3-point classification system for ULT were as follows: length from alveolar edge to frenulum gingival attachment, length of frenulum on stretch, and free-lip to total-lip length ratio. Our novel scale yielded the highest inter-rater reliability of 0.41, compared to 0.24 and 0.25 under the Kotlow and Stanford systems. Conclusion: While the Kotlow and Stanford systems are based upon a single anatomical parameter, our novel 3-point classification scale uses three oral parameters that encompass anatomical points of attachment as well as the maximal length of the ULT on stretch. Our classification scheme is the first to incorporate a functional parameter of the SLF, and thereby more fully characterizes ULT.Level of Evidence: Level 4.

Clinical presentation of paradoxical vocal fold motion or laryngeal dyskinesia in infants.

OBJECTIVES: Paradoxical vocal fold motion (PVFM) is not well-characterized in infants. Sex- and race/ethnicity-based differences have been described in older children with PVFM. This study's objectives are to characterize demographic and clinical characteristics of infants diagnosed with PVFM and investigate sex- and race-specific differences in presentation. METHODS: We retrospectively reviewed infants ≤1 year of age diagnosed with PVFM at our institution from 2009 to 2019. Patient demographics, symptoms, and findings on flexible laryngoscopy are described. Sex- and race/ethnicity-based differences were assessed using Fisher's exact test analyses. RESULTS: We identified 22 infants who were diagnosed with PVFM. The average age (range) at diagnosis was 5.7 (0.25-12.0) months, and 45.5% were male. The majority (54.6%) of patients identified as non-Hispanic White. Common comorbidities included GERD (45.5%) and chronic rhinitis (13.6%). Stridor was the only presenting symptom in the majority of patients (95.4%). The most common episode triggers were crying (45.5%), feeding (27.3%), and gastric reflux (9.1%). On flexible laryngoscopy, PVFM was observed in 95.5% of patients. A third of patients (31.8%) were misdiagnosed as having reactive airway disease or laryngomalacia prior to evaluation by otolaryngology. No sex- and race/ethnicity-based differences in presentation were identified. CONCLUSION: We present the largest case series of PVFM in infants. We found sparse clinical signs/symptoms other than stridor and a high incidence of misdiagnosis, which supports the importance of objective flexible laryngoscopy for the evaluation of stridor in this age group. Previously reported sex- and race/ethnicity-based differences in presentation of PVFM were not observed in this cohort of infants.

Application of the Milan System for Reporting Salivary Gland Cytopathology in pediatric patients: An international, multi-institutional study.

BACKGROUND: Pediatric salivary gland fine-needle aspiration (FNA) is uncommon with a higher frequency of inflammatory lesions and a small proportion of malignancies. This international, multi-institutional cohort evaluated the application of the Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) and the risk of malignancy (ROM) for each diagnostic category. METHODS: Pediatric (0- to 21-year-old) salivary gland FNA specimens from 22 international institutions of 7 countries, including the United States, England, Italy, Greece, Finland, Brazil, and France, were retrospectively assigned to an MSRSGC diagnostic category as follows: nondiagnostic, nonneoplastic, atypia of undetermined significance (AUS), benign neoplasm, salivary gland neoplasm of uncertain malignant potential (SUMP), suspicious for malignancy (SM), or malignant. Cytology-histology correlation was performed where available, and the ROM was calculated for each MSRSGC diagnostic category. RESULTS: The cohort of 477 aspirates was reclassified according to the MSRSGC as follows: nondiagnostic, 10.3%; nonneoplastic, 34.6%; AUS, 5.2%; benign neoplasm, 27.5%; SUMP, 7.5%; SM, 2.5%; and malignant, 12.4%. Histopathologic follow-up was available for 237 cases (49.7%). The ROMs were as follows: nondiagnostic, 5.9%; nonneoplastic, 9.1%; AUS, 35.7%; benign neoplasm, 3.3%; SUMP, 31.8%; SM, 100%; and malignant, 100%. Mucoepidermoid carcinoma was the most common malignancy (18 of 237; 7.6%), and it was followed by acinic cell carcinoma (16 of 237; 6.8%). Pleomorphic adenoma was the most common benign neoplasm (95 of 237; 40.1%). CONCLUSIONS: The MSRSGC can be reliably applied to pediatric salivary gland FNA. The ROM of each MSRSGC category in pediatric salivary gland FNA is relatively similar to the ROM of each category in adult salivary gland FNA, although the reported rates for the different MSRSGC categories are variable across institutions.

Demographic Differences in Clinical Presentation of Pediatric Paradoxical Vocal Fold Motion (PVFM).

OBJECTIVES: Paradoxical vocal fold motion (PVFM) is involuntary closure of the vocal folds during inspiration, often presenting in children and young adults. Although common symptoms and triggers are known, differences in clinical presentation based on patient demographics are unknown. This study characterizes differences in clinical presentation of pediatric PVFM based on age, sex, and race/ethnicity. METHODS: We reviewed electronic medical records of patients 0-21 years old with PVFM based on ICD codes from 2009 to 2019 within a tertiary academic health system. Demographics, symptoms, triggers, concurrent diagnoses, and laryngoscopy findings were abstracted. Odds ratios (ORs) and 95% confidence intervals (CI) were estimated using logistic regression. RESULTS: Among 96 individuals the mean age was 10.6 years (standard deviation ±6.5) and 66 (69%) were female. In comparison to 13-21 year olds, those 0-2 years more often had PVFM observed on laryngoscopy (OR = 17.84, 95% CI: 3.14-101.51) and had less shortness of breath (OR = 0.01, 95% CI: 0.00-0.09). Those 3-12 years had more asthma (OR = 3.07, 95% CI: 1.07-8.81) and cough (OR = 6.12, 95% CI: 1.77-21.13). Both 0-2 (OR = 0.07, 95% CI: 0.02-0.24) and 3-12 year olds (OR = 0.13, 95% CI: 0.04-0.40) presented less with activity as a trigger. Racial/ethnic minorities were more likely to present with pharyngeal findings (eg mucosal inflammation, adenotonsillar hypertrophy) on laryngoscopy (OR = 4.58, 95% CI: 1.45-15.37) compared to non-Hispanic Whites. Differences in clinical presentation by sex were not observed. CONCLUSION: We identified several differences in symptoms, triggers, and laryngoscopy findings in pediatric PVFM based on age and race/ethnicity. Associations between sex and clinical presentation were not observed.

Repair of Giant Anterior Skull Base Encephalocele Containing Intralesional Eloquent Brain: Technical Note.

BACKGROUND: Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. OBJECTIVE: To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. METHODS: A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. RESULTS: We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. CONCLUSION: The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

Cardiopulmonary Testing before Pediatric Adenotonsillectomy for Severe and Very Severe Obstructive Sleep Apnea Syndrome.

OBJECTIVES/HYPOTHESIS: Adenotonsillectomy is first-line treatment for pediatric obstructive sleep apnea syndrome (OSAS) when not otherwise contraindicated. There is concern severe OSAS increases risk of comorbid cardiopulmonary abnormalities, such as ventricular hypertrophy or pulmonary hypertension, which preoperative testing could detect. Our objective is to determine if there is a severity of pediatric OSAS where previously undetected cardiopulmonary comorbidities are likely. STUDY DESIGN: Retrospective chart review. METHODS: We performed a retrospective review of 358 patients ≤21 years with severe OSAS who underwent adenotonsillectomy at a tertiary hospital June 1, 2016 to June 1, 2018. We extracted demographics, comorbidities, polysomnography, and preoperative tests. Wilcoxon rank-sum and logistic regression estimated associations of OSAS severity (based on obstructive apnea-hypopnea index [OAHI], hypoxia, hypercarbia) with preoperative echocardiograms and chest X-rays (CXRs). RESULTS: Mean age was 5.9 (±3.6) years and 52% were male. Mean OAHI and oxygen saturation nadir were 30.3 (±23.8) and 80.7% (±9.2), respectively. OAHI ≥60 was associated with having a preoperative echocardiogram (OR, 3.8; 95% CI, 1.7-8.5) or CXR (OR, 3.0; 95% CI, 1.4-6.8) compared to OAHI 10-59. There were no significant associations between OSAS severity and test abnormalities. The presence of previously diagnosed cardiopulmonary comorbidities was associated with abnormalities on echocardiogram (OR, 36; 95% CI, 4.1-320.1) and CXR (OR, 4.1; 95% CI, 1.2-14.4). CONCLUSIONS: Although pediatric patients with very severe OSAS (OAHI ≥60) underwent more pre-adenotonsillectomy cardiopulmonary tests, OSAS severity did not predict abnormal findings. Known cardiopulmonary comorbidities may be a better indication for cardiopulmonary testing than polysomnographic parameters, which could streamline pre-adenotonsillectomy evaluation and reduce cost. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2361-2368, 2021.

Understanding Bias in Surgery: Perceived Cultural Similarity Between Surgeons and Patient Families.

OBJECTIVE: We describe surgeon and parent perceptions of similarity toward each other and evaluate differences in the perceptions of similarity by race. STUDY DESIGN: Observational cohort analysis. SETTING: Three outpatient sites. METHODS: Following consultations for children undergoing evaluation for 1 of 3 surgical procedures (tonsillectomy, hernia repair, circumcision), surgeons and parents rated their perception of cultural similarity toward each other on a 6-point Likert scale. Surgeon evaluation of 9 parent characteristics was measured with 7-point Likert scales. Regression analyses were performed to identify predictors of greater surgeon-perceived similarity and to assess associations of perceived similarity with evaluation of parent characteristics. RESULTS: Most parents were women (n = 38, 84%), whereas surgeons were primarily men (n = 7, 54%). Of 45 parents, 23 (51%) were non-White, whereas only 4 of 13 clinicians (31%) were non-White. Mean perceived similarity score was 21.7 for parents (range, 10-24) and 18.2 for surgeons (range, 10-24). There was no difference in parent-perceived similarity based on race (White vs non-White parents, mean [SD] = 22.3 [3.4] vs 21.1 [3.0]; P = .26). Surgeons perceived greater similarity with White parents (odds ratio = 4.78; 95% CI, 1.02-22.54; P = .04) and parents with higher income (odds ratio = 11.84; 95% CI, 1.32-106.04; P = .03). Greater perceived similarity by the surgeons was associated with more positive assessments of parent personality characteristics. CONCLUSION: Surgeons perceived similarity more commonly with White parents, while parents' perception of similarity to surgeons was uniform regardless of parent race. Elucidating biases of surgeons may help to tailor interventions promoting culturally competent, equitable communication and decision making for elective surgery.

The Effect of Language Barriers at Discharge on Pediatric Adenotonsillectomy Outcomes and Healthcare Contact.

OBJECTIVE: Effective delivery of discharge instructions and access to postoperative care play a critical role in outcomes after pediatric surgery. Previous studies in the pediatric emergency department suggest that caregivers with language barriers have less comprehension of discharge instructions despite use of interpretation services. However, the impact of language barriers during discharge on surgical outcomes in a pediatric surgical setting has not been studied. This study examined the effect of parental language during discharge on number and mode of healthcare contact following pediatric adenotonsillectomy. METHODS: A retrospective cohort study was conducted on children who underwent adenotonsillectomy at a tertiary care pediatric academic medical center from July 1, 2016 to June 1, 2018. Data were collected on consecutive patients with non-English-speaking caregivers and a systematic sampling of patients with English-speaking caregiver. Surgery-related complications and healthcare contacts within 90 days after discharge were collected. Two-tailed t tests, χ2 tests, and logistic regression were performed to assess the association between parental primary language and incidence of healthcare contact after surgery. RESULTS: A total of 136 patients were included: 85 English-speaking and 51 non-English-speaking. The groups were comparable in age, sex, and comorbidities. The non-English group had more patients with public insurance (86% vs. 56%; P < .001). Number of encounters and types of complications following discharge were similar, but the non-English group was more likely to utilize the emergency department compared to phone calls (OR, 9.3; 95% CI, 2.3-38.2), even after adjustment for insurance type (OR, 7.9; 95% CI, 1.6-39.4). CONCLUSION: Language barriers at discharge following pediatric otolaryngology surgery is associated with a meaningful difference in how patients utilized medical care. Interventions to improve comprehension and access may help reduce preventable emergency department visits and healthcare costs.

Assessment of Parental Choice Predisposition for Tonsillectomy in Children.

Importance: The decision to proceed with tonsillectomy to treat pediatric obstructive sleep-disordered breathing (OSDB) often falls on individual families. Despite emphasis on shared decision-making between parents and surgeons about tonsillectomy for OSDB, the extent to which parents have already decided about surgery prior to the child's consultation is not known. Objective: To identify predictors of parent choice predisposition for surgical treatment of OSDB with tonsillectomy and describe its association with parent-clinician communication. Design, Setting, and Participants: Observational cohort study conducted at 3 outpatient clinical sites (urban-based outpatient center, suburban off-site outpatient center, and community-based medical center) associated with a large academic center. A total of 149 parents of children undergoing their initial otolaryngology consultation for OSDB were identified through clinic scheduling records and deemed eligible for participation in this study. Of the 149 parents, a volunteer sample of 64 parents (42.9%) agreed to participate and have their consultation audiorecorded. Of these 64 participants, 12 parents were excluded because their child had previously been evaluated for OSDB by a specialist. Main Outcomes and Measures: The primary outcomes and measures were treatment choice predisposition scale (a measure of the strength of a patient's treatment decision prior to entering a medical consultation), parent communication behaviors coded in consultation audiorecordings (substantive questions asked, introduced medical jargon, expression of treatment preference, and scores on the OSDB and Adenotonsillectomy Knowledge Scale for parents). Results: A total of 52 parent participants were included in the final analysis. Most parent participants were female (n = 48; 92%); 50% (n = 26) of parents were non-Hispanic White, 37% (n = 19) were Black, 10% (n = 5) were Hispanic/Latino, and 4% (n = 2) self-reported race/ethnicity as "Other." Mean (range) choice predisposition was 6.84 (2-10), with 22 parents (42%) more predisposed to choose tonsillectomy. Parents more predisposed to choose tonsillectomy used more medical jargon during the consultation (odds ratio [OR], 3.95; 95% CI, 1.16-15.15) and were less likely to ask questions (OR, 0.22; 95% CI, 0.05-0.87). Parental predictors of greater predisposition toward choosing surgery were White race (OR, 7.31; 95% CI, 1.77-39.33) and prior evaluation by a pediatrician for OSDB (OR, 6.10; 95% CI, 1.44-33.34). Conclusions and Relevance: In this cohort study of parents of children with OSDB, many parents were predisposed to choose treatment with tonsillectomy prior to initial surgical consultation, which may lessen engagement and influence 2-way communication. In this cohort, greater predisposition for tonsillectomy was observed in non-Hispanic White parents and parents of patients who had been previously evaluated by a pediatrician for OSDB. Understanding parent choice predisposition for surgery may promote improved communication and parental engagement during surgical consultations. It may also help direct education about sleep and tonsillectomy to nonsurgical forums.

Laryngoscopy Grade Improvement and Difficult Airway Resolution in Infants With Robin Sequence Undergoing Mandibular Distraction Osteogenesis: A Multi-Institutional Study.

OBJECTIVE: Mandibular distraction osteogenesis (MDO) aims to relieve tongue-based airway obstruction in Robin Sequence (RS). We investigated direct laryngoscopy grade (DLG) improvement and difficult airway (DA) resolution following MDO. DESIGN: Retrospective cohort analysis. SETTING: Three tertiary care institutions. PATIENTS: Sixty-four infants with RS who underwent a single MDO procedure in their first year of life were identified from January 2010 to January 2019. MAIN OUTCOME MEASURES: The primary outcome was DLG pre- and post-MDO. Secondary outcomes included DA designation, pre- and post-MDO polysomnographic assessment for obstructive sleep apnea (OSA), length of stay, need for gastrostomy, and major/minor adverse events. RESULTS: Median DLG improved from II pre-MDO to I at the time of distractor removal (n = 43, P < .001). No significant change was seen in patients with a third recorded time point (eg, palatoplasty; n = 78, P = .52). Twenty-six (47%) of 55 patients were designated as DA pre-MDO, and 10 (18%) of 55 patients retained the label post-MDO (P < .01). Five (50%) of these 10 patients appeared to be inappropriately retained. Median obstructive apnea-hypopnea index improved from 38.6 (range 31.2-62.8) pre-MDO to 2.9 (range 1-3.9) post-MDO (n = 12; P = .002). CONCLUSION: Mandibular distraction osteogenesis allowed for DLG improvement that was stably maintained as well as functional improvement in OSA, with minimal morbidity. Difficult airway designation persisted in the electronic record of some infants despite clinical resolution.

Systemic Bevacizumab (Avastin) for Juvenile-Onset Recurrent Respiratory Papillomatosis: A Systematic Review.

OBJECTIVES: Juvenile onset recurrent respiratory papillomatosis (JORRP) can cause severe or disseminated disease. Surgical treatment may be inadequate. Systemic bevacizumab has shown initial success for severe JORRP. The objective of this systematic review was to assess usage, effectiveness, and safety of this treatment. METHODS: We searched PubMed, Embase, and Web of Science for studies of humans with JORRP treated with systemic bevacizumab. Two researchers independently reviewed the studies to determine inclusion and aggregate data on patient characteristics, dosing protocols, treatment response, adverse events, and level of evidence. RESULTS: Of 80 identified articles, 12 studies were included detailing 20 distinct cases. At a mean age of 12.8 years (range = 1-43 years) patients received initial dosing of 5 to 10 mg/kg of bevacizumab followed by ongoing doses at a mean 3-week intervals (range = 2-5 weeks). All patients had clinically significant disease reduction with reduced need for surgery. Six patients (30%) had complete response in at least one involved anatomic site. Eleven (55%) required no surgery after initiating treatment. There was recurrence in all four patients whose treatment was stopped, but had rapid improvement with treatment resumption. Six (30%) experienced mild or moderate adverse events. CONCLUSIONS: Marked improvement in severe JORRP has been reported from systemic bevacizumab. Treatment protocols vary, and treatment discontinuation was not feasible in any reported patient. Based on currently available data, systemic bevacizumab can be considered for severe JORRP as it appears to be well tolerated and effective. A clinical trial could enhance the understanding of its safety and efficacy for this indication. Laryngoscope, 131:1138-1146, 2021.

Outcomes of Adenotonsillectomy for Obstructive Sleep Apnea in Prader-Willi Syndrome: Systematic Review and Meta-analysis.

OBJECTIVES: Prader-Willi syndrome (PWS) increases the risk of obstructive sleep apnea (OSA) due to obesity, hypotonia, and abnormal ventilatory responses. We evaluated post-adenotonsillectomy complications, polysomnography changes, and quality of life in children with OSA and PWS. STUDY DESIGN: Systematic review and meta-analysis. METHODS: We conducted a systematic review and meta-analysis by searching PubMed, Embase, Cochrane, Web of Science, and Scopus. Two researchers independently reviewed studies about adenotonsillectomy for OSA in patients <21 years with PWS. We extracted study design, patient numbers, age, complications, polysomnography, and quality of life. We pooled postoperative changes in apnea hypopnea index (AHI) for meta-analysis. We applied Methodological Index for Nonrandomized Studies (MINORS) criteria to assess study quality. RESULTS: The initial search yielded 169 studies. We included 68 patients from eight studies with moderate to high risk of bias. Six studies reported on complications and 12 of 51 patients (24%) had at least one. Velopharyngeal insufficiency was the most commonly reported complication (7/51, 14%). We included seven studies in meta-analysis. Mean postoperative improvement in AHI was 7.7 (95% CI: 4.9-10.5). Postoperatively 20% (95% CI: 3%-43%) had resolution of OSA with AHI < 1.5 while 67% (95% CI: 50%-82%) had improvement from severe/moderate OSA to mild/resolved (AHI < 5). Two studies evaluated quality of life and demonstrated improvement. CONCLUSIONS: Children with PWS undergoing adenotonsillectomy for OSA have a substantial risk of postoperative complications that may require additional interventions, especially velopharyngeal insufficiency. Despite improvements in polysomnography and quality of life, many patients had residual OSA. This information can be used to counsel families when considering OSA treatment options. Laryngoscope, 131:898-906, 2021.

Mandibular Reconstruction Following Central Giant Cell Granuloma Resection in Primary Dentition: A Case for the Use of a Costochondral Graft.

Central giant cell granuloma is a benign, intraosseous lesion that may affect the pediatric craniofacial skeleton, particularly the mandible. When surgery is indicated, the role of the craniofacial surgeon is to ameliorate the sequelae of ablative surgery by restoring facial symmetry, ensuring appropriate postoperative occlusion, and allowing for adequate interincisal opening, all in the setting of a growing craniofacial skeleton. Herein, we report the case of a 3-year-old female presenting for reconstruction after resection of the right hemimandible proximal to the unerupted first permanent molar. We highlight the various reconstructive challenges associated with mandibular reconstruction during primary dentition and make a case for the use of a costochondral graft, with a successful outcome demonstrated at 2 years of follow-up.

Transoral Endoscopic Vestibular Thyroglossal Duct Cyst Excision.

OBJECTIVE: Thyroglossal duct cysts (TGDCs) are relatively common congenital midline neck masses that are treated with surgical excision. Traditionally these are removed along with any associated tract and the central portion of the hyoid bone through an anterior neck incision. Some patients with TGDCs want to avoid an external neck scar. METHODS: We describe the details of a transoral endoscopic vestibular excision of a TGDC and the associated hyoid bone in an adolescent patient. RESULTS: This novel approach was successful and there were no complications. CONCLUSION: We propose that cervical TGDCs can be safely and completely removed with this approach in appropriately selected patients while avoiding a neck scar.

Adherence to American Academy of Otolaryngology-Head and Neck Surgery Clinical Practice Guidelines: A Systematic Review.

OBJECTIVE: Clinical practice guidelines synthesize and disseminate the best available evidence to guide clinical decisions and increase high-quality care. Since 2004, the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) has published 16 guidelines. The objective of this review was to evaluate clinicians' adherence to these guidelines' recommendations as measured in the literature. DATA SOURCES: We searched PubMed, Embase, and Web of Science on August 29, 2019, for studies published after June 1, 2004. REVIEW METHODS: We systematically identified peer-reviewed studies in English that reported clinician adherence to AAO-HNSF guideline recommendations. Two authors independently reviewed and abstracted study characteristics, including publication date, population, sample size, guideline adherence, and risk of bias. RESULTS: The search yielded 385 studies. We excluded 331 studies during title/abstract screening and 32 more after full-text review. The remaining 22 studies evaluated recommendations from 8 of the 16 guidelines. The Otitis Media with Effusion, Polysomnography, Tonsillectomy, and Sinusitis guidelines were studied most. Study designs included retrospective chart reviews (7, 32%), clinician surveys (7, 32%), and health care database analyses (8, 36%). Studies reported adherence ranging from 0% to 99.8% with a mean of 56%. Adherence varied depending on the recommendation evaluated, type of recommendation, clinician type, and clinical setting. Adherence to the polysomnography recommendations was low (8%-65.3%). Adherence was higher for the otitis media with effusion (76%-90%) and tonsillectomy (43%-98.9%) recommendations. CONCLUSIONS: Adherence to recommendations in the AAO-HNSF guidelines varies widely. These findings highlight areas for further guideline dissemination, research about guideline adoption, and quality improvement.

Transoral robotic surgery for upper airway pathology in the pediatric population.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to present one of the largest case series of pediatric transoral robotic surgery (TORS) in the upper airway demonstrating a wide range of ages and indications. STUDY DESIGN: A retrospective case series at an academic tertiary referral center from August 2010 to September 2014. METHODS: The da Vinci surgical robot (Intuitive Surgical, Inc., Sunnyvale, CA) was used on 16 pediatric patients for 18 procedures. A variety of upper airway pathologies and reconstructions in children with a wide range of ages and weights were treated. No lingual tonsillectomies or base-of-tongue reductions were included. RESULTS: Sixteen children (6 males) underwent 18 TORS procedures, including resection of hamartoma (n = 1), repair of laryngeal cleft (n = 7), removal of saccular cyst (n = 2), release of pharyngeal or esophageal strictures (n = 2), and excision of lymphatic malformations (n = 4). Patient ages ranged from 14 days to 15 years. There were no intraoperative complications. All patients had successful robotic access, and no patients had conversions to open or traditional endoscopic surgery. Hospital courses varied with duration ranging from 1 to 20 days. The median follow up was 22 months. CONCLUSION: Applying TORS to the pediatric population can be feasible and safe for appropriate airway pathologies. Because many patients are small in size, there is inherent risk in using robotic instruments and scopes transorally. Pearls in this series include a standardized two-robot experienced attending team and longitudinal airway follow-up. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:247-251, 2017.

Camptodactyly and the 22q11.2 deletion syndrome.

We describe a 5-day-old male with minor facial anomalies, a congenital laryngeal web, severe laryngomalacia, and prominent fixed flexion of the proximal interphalangeal joints of digits 2 through 5 bilaterally. A whole genome SNP microarray analysis identified a 2.55 Mb interstitial deletion of 22q11.21, typical of that seen in the DiGeorge and Velocardiofacial syndromes. A review of the literature identifies 10 other cases with camptodactyly. Camptodactyly appears to be an associated but rarely reported anomaly in patients with the 22q11.2 microdeletion syndrome. © 2016 Wiley Periodicals, Inc.