Pediatric Tonsil Cancer: A National and Institutional Perspective.

OBJECTIVE: To evaluate childhood and adolescent tonsil cancer incidence and to identify the clinical characteristics indicative of those patients who would benefit from urgent operative intervention. STUDY DESIGN: The Surveillance, Epidemiology and End Results 18 database, inclusive of national cancer statistics from 1973 to 2013, provided quantitative tonsil cancer incidence data. An institutional retrospective chart review of pediatric patients diagnosed with tonsil malignancy from January 2013 to January 2017 identified supplementary qualitative clinical presentation information. RESULTS: The Surveillance, Epidemiology and End Results 18 database included 138 pediatric patients with tonsil cancer with an age-adjusted incidence rate of 0.021/100 000 patients per year. The majority of cases were unilateral (79.7%), and there was both a male and Caucasian predominance. Non-Hodgkin lymphoma (84.1%) was the most common malignancy, of which Burkitt lymphoma (31.1%), diffuse large B-cell lymphoma (26.8%), and follicular lymphoma (10.1%) were the most common subtypes. Five tonsillar malignancy patients were identified upon institutional chart review. The majority likewise had non-Hodgkin lymphoma and all shared a history of rapid tonsillar enlargement over ≤12 weeks. Significant tonsillar asymmetry was present in 4 patients. Four patients additionally exhibited prominent cervical lymphadenopathy. CONCLUSIONS: Pediatric tonsil cancer is rare, with non-Hodgkin lymphoma accounting for the majority of pediatric tonsillar malignancies. A high index of suspicion is appropriate in children who present with relatively rapid tonsil enlargement, tonsillar asymmetry characterized by a difference in tonsillar size of ≥2 degrees on the Brodsky scale, or concurrent prominent cervical lymphadenopathy.

Ambulatory Visits for Otitis Media before and after the Introduction of Pneumococcal Conjugate Vaccination.

OBJECTIVE: To examine the long-term trend of otitis media (OM)-associated ambulatory visits from 1997 to 2014 and to evaluate the impact of 7-valent and 13-valent pneumococcal conjugate vaccines (PCV7 and PCV13) on OM ambulatory visits. STUDY DESIGN: We examined OM-associated ambulatory visits in children, using the National Ambulatory Medical Care Survey and National Hospital Ambulatory Medical Care Survey. RESULTS: A total of 24 148 OM-related visits were identified from 363 240 ambulatory visits. OM visits accounted for 9.5% of all ambulatory visits in 1997-1999, 7.1% in 2002-2009, and 5.5% in 2012-2014. Between the pre-PCV7 and the post-PCV13 period, annual physician's office visits for OM declined from 826 to 387 visits per 1000 children among children younger than 2 years of age. Compared with the pre-PCV7 period, office visit rates for OM significantly declined by 51% (95% CI 42%-58%) among children younger than 2 years of age and by 37% (95% CI 23%-48%) among children 2-4 years of age during the post-PCV13 period. Among children younger than 2 years of age, emergency department visits for OM declined by 47% (95% CI 36%-55%) during the post-PCV13 period, and hospital outpatient visits declined by 30% (95% CI 8%-48%) during the PCV13-transition period. OM-related visits declined across sex, race/ethnicity, health insurance status, and geographic region. CONCLUSION: Our nationwide study shows that ambulatory visits for OM have declined following the introduction of PCV7 and PCV13. Future research on the evolving microbiology of OM and continued monitoring of the epidemiology of pneumococcal disease are needed.

Juvenile Nasopharyngeal Angiofibroma: Outcomes Analysis Based Upon Hospital Volume.

OBJECTIVE: To evaluate the management of juvenile nasopharyngeal angiofibroma (JNA) from a national perspective with outcomes comparison based on hospital volume. STUDY DESIGN: Ten-year Pediatric Health Information Systems (PHIS) data analysis. METHODS: The PHIS database was queried for the diagnosis of JNA. Data regarding demographics, surgical approach, embolization, length of stay, charges, readmission, and revision surgery was collected and analyzed. Hospitals were classified as low volume if fewer than 10 cases and high volume if greater than or equal to 10 cases during the study period. A random effects model compared outcomes based on hospital volume. RESULTS: A total of 287 JNA patients were identified with a mean age of 13.8 (± 2.7) years. Nine hospitals were classified as high volume, accounting for a total of 121 patients. The mean length of hospitalization, blood transfusion rate, and 30-day readmissions did not differ significantly by hospital volume. Patients cared for at high-volume institutions were less likely to require postoperative mechanical ventilation (8.3% vs. 25.0%; adjusted RR = 0.32; 95% CI 0.14-0.73; p < 0.01) or return to the operating room for residual disease than patients admitted to low-volume hospitals (7.4% vs. 20.5%; adjusted RR = 0.38; 95% CI 0.18-0.79; p = 0.01). CONCLUSIONS: The management of JNA is complex from both an operative and perioperative management standpoint. Over the past decade, nearly half (42.2%) of JNA patients have been managed at nine institutions in the United States. These centers have significantly lower rates of postoperative mechanical ventilation and the need for revision surgery. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3216-3220, 2023.

Vestibular pneumolabyrinth: why assessment with temporal bone computed tomography utilizing dynamic focal spot mode is important for the diagnosis.

We present an interesting and relatively uncommon case of vestibular pneumolabyrinth in a young child post-trauma. His initial clinical exam and imaging studies of the head and cervical spine were negative. He subsequently developed nystagmus and a dedicated temporal bone study demonstrated a subtle fracture and vestibular pneumolabyrinth. Temporal bone fractures can be difficult to appreciate, and therefore, associated findings of fluid in the middle ear, stapes dislocation, or vestibular pneumolabyrinth must be carefully evaluated. Temporal bone computed tomography is a high resolution study, utilizing dynamic focal spot mode which leads to increased sampling and resolution, thereby reducing aliasing artifacts but a longer scan time and increased radiation dose. CT head and cervical spine normally obtained without using this technique leads to aliasing artifacts where even the normal endolymph in the inner ear structures appear hypodense mimicking pneumolabyrinth, thereby obscuring true pneumolabyrinth. It is important to be aware of this finding and technique-related artifact, if a temporal bone injury is suspected, to ensure an earlier diagnosis and optimum management.

Improvement of Pulmonary Function in Cystic Fibrosis Patients following Endoscopic Sinus Surgery.

OBJECTIVE/HYPOTHESIS: To compare pre-and post-operative pulmonary function relative to disease severity in cystic fibrosis (CF) patients following endoscopic sinus surgery (ESS). STUDY DESIGN: Retrospective chart review. METHODS: Patients with CF who underwent ESS between January 1996 and July 2018 were identified, with subsequent study exclusions based upon surgical indications or incomplete records. CF disease severity was based upon percentage predicted of forced expiratory volume in 1 second (%FEV1) with <40% considered severe disease, 40% to 70% as moderate disease, and >70% as mild disease. The changes in %FEV1 before and after ESS were examined using multivariable mixed-effects models controlling for age, gender, genotype, medications, nutritional status, diabetes status, microbiology results, extent of surgery, and number of surgeries. RESULTS: A total of 427 surgeries were performed in 188 patients during the study period. Mean age at first ESS was 12.7 years (SD 6.0 years, range 4-38) and 54.8% were females. The effect of ESS varied by severity of lung disease. After surgery, %FEV1 increased by 8.1% (95% CI: 2.3, 13.9%) among patients with severe lung disease and by 3.0% (95% CI: 0.7, 5.2%) among patients with moderate disease. %FEV1 also increased by 7.3% (95% CI: 4.2, 10.5%) among patients with mild disease whose %FEV1 value was 70% to 80% at baseline. No improvement was observed in patients with a baseline %FEV1 >80%. CONCLUSIONS: When controlling for important confounding factors, lung function improved following ESS among CF patients with severe and moderate disease and in select patients with mild disease. This improvement was sustained at 12 months following surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1930-1938, 2021.

Validation of the Nasal Obstruction Symptom Evaluation Scale in Pediatric Patients.

OBJECTIVE/HYPOTHESIS: The Nasal Obstruction Symptom Evaluation (NOSE) is a disease specific quality of life instrument developed and validated in adults. The objective of this study is to evaluate the validity of the NOSE scale for pediatric nasal obstruction. We also examined the effect of septoplasty with bilateral inferior turbinate reduction in this population. STUDY DESIGN: A validation study at a tertiary care children's hospital. METHODS: Thirty-eight pediatric patients who underwent septoplasty and bilateral inferior turbinate reduction between 2014 and 2018 were included. Patients were administered the NOSE instrument on the day of their clinic evaluation, the day of surgery, and at their 6 to 8-week post-operative appointment. A sample of 40 pediatric patients with non-rhinologic complaints was also included. Confirmatory factor analysis was performed to evaluate the factorial validity of the NOSE instrument. RESULTS: Of the 78 patients included, the mean age was 15.4 years (SD 3.4). In the confirmatory factor analysis, factor loadings were all significant and ranged from 0.95 to 0.99. Internal consistency reliability using Omega and maximal reliability H indices were well above recommended standards (Omega = 0.983 and maximal H = 0.988). Test-retest reliability was also adequate. Mean NOSE scores significantly improved following surgery (from 96.7 [SD 6.2] to 8.8 [SD 7.8]; mean difference = -87.9; 95% CI: -84.5, -91.3; P < .001). Similar improvements were observed across age groups. CONCLUSIONS: The NOSE scale is a valid and reliable quality of life instrument for pediatric patients with nasal obstruction. Nasal septoplasty with bilateral turbinate reduction substantially improved symptoms of nasal obstruction. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2594-E2598, 2021.

Pediatric Subperiosteal Abscess Secondary to Acute Sinusitis: A Systematic Review and Meta-analysis.

OBJECTIVES/HYPOTHESIS: The surgical versus medical management of subperiosteal abscess can be controversial. The purpose of this study was to summarize current literature and provide pooled analyses to help direct management decisions. STUDY DESIGN: Systematic review and meta-analysis. METHODS: Patients <18 years old with subperiosteal abscess secondary to acute sinusitis were reviewed, and a meta-analysis was conducted. Studies including five or more patients written in English were the primary search focus. RESULTS: Thirty-eight studies met inclusion criteria for the systematic review, and seven studies contained sufficient data for the meta-analysis. A total of 1,167 patients between the ages of 10 days and 18 years were included. Eleven sign/symptom categories were identified, with restricted ocular motility (n = 289, 45.9%), proptosis (n = 277, 44%), and fever (n = 223, 35.4%) being most frequent. Staphylococcus aureus was the most common pathogen isolated from cultures. Patients with abscess volume greater than the threshold specified in each individual study were over three times more likely to require surgical intervention compared to those with smaller abscess volume (pooled risk ratio [RR] = 3.61, 95% confidence interval [CI]: 2.40-5.44). Proptosis and gaze restriction also significantly predicted surgical intervention (pooled RR = 1.65: 95% CI: 1.29-2.12 for proptosis/pooled RR = 1.90; 95% CI: 1.20-3.00 for gaze restriction). CONCLUSIONS: Approximately half of patients with a subperiosteal abscess undergo surgical drainage. Abscess volume appears to be the most significant predictive risk factor. Detailed data from future studies regarding radiologic and ophthalmologic parameters are needed to provide more definitive values predictive of which patients are likely to fail medical therapy. LEVEL OF EVIDENCE: 2a Laryngoscope, 2020.

Juvenile nasopharyngeal angiofibroma in prepubertal males: A diagnostic dilemma.

OBJECTIVES/HYPOTHESIS: To highlight the presentation and management of juvenile nasopharyngeal angiofibroma (JNA) in prepubertal children. STUDY DESIGN: Single-institution 10-year retrospective review. METHODS: All identified cases of pathologically confirmed JNA in children <10 years of age were assessed from a gender, imaging and embolization findings, tumor stage, surgical approach, and clinical outcomes standpoint, and compared to a group of stage-matched older patients from the same time period. RESULTS: Of 45 patients over the 10-year study period, four male children between 8 to 9.8 years of age were identified. One patient had University of Pittsburgh Medical Center stage 1 disease, and the other three had stage 3 disease at presentation. A malignant process other than JNA was of concern preoperatively in two of the four children due to a combination of aggressive imaging characteristics and an absence of pterygopalatine fossa involvement. Such pterygopalatine fossa involvement was comparatively uniformly present in a group of stage-matched JNA patients aged 15 to 21 years. All four prepubescent children underwent surgical resection via transnasal endoscopic approach following ipsilateral sphenopalatine artery embolization without the need for blood transfusion. There were no recurrences in three of the four cases at a median follow-up duration of 2.3 years (range, 0.8-6.4 years). CONCLUSIONS: JNA may pose a diagnostic challenge in prepubertal males due to the atypical age at presentation and absence of classic imaging characteristics. Successful endoscopic transnasal resection is possible despite anatomic constrictions. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1777-1783, 2019.

The Genetic and Molecular Determinants of Juvenile Nasopharyngeal Angiofibroma: A Systematic Review.

OBJECTIVE: Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular tumor of unknown etiology. Studies investigating the molecular and genetic determinants of JNA are limited by small sample size and inconsistent approaches. The purpose of this study is to examine all eligible JNA studies in aggregate, applying qualitative analysis to highlight areas of particular relevance, including potential targets for therapeutic intervention. METHODS: The PubMed, MEDLINE, Embase, Web of Science, Cochrane, and CINAHL databases were screened with inclusion and exclusion criteria applied to all citations. Manuscripts investigating the genetic determinants, histopathogenesis, and heritability of juvenile nasopharyngeal angiofibroma were included. Non-English studies, case reports, and articles focusing on clinical management without original data were excluded. Full text articles were obtained. A qualitative synthesis of data was performed. RESULTS: A total of 59 articles met criteria for inclusion. These were divided into 6 categories based on the primary topic or target discussed, (1) steroid hormone receptors, (2) chromosomal abnormalities, (3) growth factors, (4) genetic targets, (5) molecular targets, (6) Wnt cell signaling, and (7) studies that overlapped multiple of the aforementioned categories. Although relatively low n values prevent definitive conclusions to be drawn, a predominance of certain molecular targets such as vascular endothelial growth factor (VEGF) and Wnt/ß-catenin pathway intermediaries is apparent. CONCLUSIONS: Although the etiology of JNA remains elusive, contemporary molecular genetic investigation holds promise for risk stratification and could form the basis of a modernized staging system. A multicenter clinical registry and linked tissue bank would further promote the search for JNA specific biomarkers.

Balloon Sinuplasty Utilization in the Pediatric Population: A National Database Perspective.

OBJECTIVE: Balloon sinuplasty (BS) is a surgical management option in the treatment of chronic rhinosinusitis. The purpose of this study was to examine BS utilization among children with a national database. STUDY DESIGN: Retrospective review. SETTING: National pediatric database. SUBJECTS AND METHODS: All cases of children aged ≤18 years who underwent BS or traditional endoscopic sinus surgery (ESS) 5 years before and after the introduction of BS billing codes were studied with the Pediatric Health Information System database. We evaluated overall trends, demographics, performing physicians, readmissions, and cost data. RESULTS: A total of 14,079 patients met inclusion criteria: 13,555 underwent traditional ESS and 524 had a BS procedure. There was no significant increase in BS rates between 2011 and 2016. BS was more commonly performed among younger children than ESS (median age [interquartile range], 6 years [4-10] vs 9 years [6-13]; P < .001). There were 23 (4.4%) readmissions within 30 days in the balloon cohort versus 474 (3.5%) in the ESS cohort. The median cost of balloon maxillary antrostomy (US $6560 [$5420-$8250]) was higher than that of traditional maxillary antrostomy (US $5630 [$4130-$7700], P < .001). Physicians who performed BS had a larger volume of ESS procedures when compared with those who did not perform BS. CONCLUSION: Rates of BS performance in the pediatric population have not increased over time. Results showed no difference in readmission rates between BS and ESS. BS was associated with higher costs as compared with ESS. The role of BS in the pediatric chronic rhinosinusitis population remains unclear.

Changes in Scope of Procedures Performed by Pediatric Otolaryngologists in the Past Decade.

IMPORTANCE: Monitoring current trends in pediatric otolaryngology will help adjust our training and practice paradigms in a way that ensures the long-term viability of the specialty. OBJECTIVES: To gauge the current scope of pediatric otolaryngology (ORL) practice within and outside of the United States and to identify changes in caseload over the past decade. DESIGN, SETTING, AND PARTICIPANTS: An online survey was sent to pediatric ORL chairs and/or fellowship directors at 42 institutions in the United States and abroad. For 59 procedures, respondents were asked to estimate the percentage of cases performed by their department, determine whether this percentage has changed over the past 10 years (2006-2016), and identify any other specialties performing the procedure. Data were collected during a 2-week period in October 2016, from October 7 through 21, and analyzed from November 2016 through February 2017. MAIN OUTCOMES AND MEASURES: Main outcomes included the percentage of operations currently performed by the respondent's department for each procedure; whether this percentage has decreased, increased, or remained the same over the past decade; other specialties that perform each procedure; and any procedures added to or eliminated from the respondent's practice over the past decade. RESULTS: Respondents from 33 of the 42 academic institutions completed the survey (23 in the United States and 10 international; 79% response rate). Respondents reported the least involvement in procedures pertaining to facial plastic and reconstructive surgery, aerodigestive endoscopy, and congenital anomalies. Conversely, a mean (SD) of 91% (7%) reported performing 90% to 100% of otology, airway, rhinology, and general procedures. A mean (SD) of 82% (11%) reported that their department's involvement in each procedure has remained the same from 2006 to 2016. CONCLUSIONS AND RELEVANCE: The specialty of pediatric ORL has evolved over the past decade. There has been a notable decline in involvement in facial plastic and reconstructive surgery and treatment of vascular malformations and esophageal disorders. The management of thyroid disease is in flux. Monitoring current trends to adjust training and practice paradigms will ensure the long-term viability of the specialty.

Microbiology of the Upper and Lower Airways in Pediatric Cystic Fibrosis Patients.

Objective To evaluate the microbiology of the upper and lower airways in pediatric cystic fibrosis (CF) patients who underwent sinus surgery. Study Design Retrospective case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods A total of 201 paired sinus and pulmonary cultures from 105 CF patients were identified between 1996 and 2014. Demographics and culture results were analyzed. Results The mean age of patients was 11.2 ± 5.4 years (range, 1-27 years), and approximately one-half were female. Methicillin-sensitive Staphylococcus aureus was the most common pathogen overall. A significantly higher prevalence of Pseudomonas aeruginosa (32% for pulmonary and 37% for sinus cultures) was observed in older patients versus younger patients ( P < .001). There was low to moderate agreement between sinus and pulmonary cultures (Kappa statistic range, 0.03-0.56). The prevalence of methicillin-resistant S aureus (MRSA) increased significantly for lower respiratory tract culture (from 5% to 16%) and sinus culture (from 5% to 27%) between 1996-2004 and 2010-2014 ( P = .016 and P < .001, respectively). The prevalence of positive sinus cultures increased from 40% to 85% between 1996-2004 and 2010-2014 ( P = .018). Patients with pulmonary MRSA were more likely to be coinfected with pulmonary P aeruginosa (risk ratio, 2.4; 95% CI, 1.2-4.8; P = .015) or Aspergillus fumigatus (risk ratio, 2.2; 95% CI, 1.2-4.8; P = .035). Conclusions There is low to moderate correlation between pulmonary and sinus pathogens in CF patients. This is important to consider when treating infections. The prevalence of MRSA in sinus cultures has increased over time and warrants further investigation.

Nasal versus tracheobronchial biopsies to diagnose primary ciliary dyskinesia: A meta-analysis.

OBJECTIVES/HYPOTHESIS: To systematically review the literature regarding the efficacy of different biopsy sites and methods to obtain an adequate ciliary sample for ultrastructural examination with electron microscopy (EM) for the diagnosis of primary ciliary dyskinesia (PCD). STUDY DESIGN: Systematic review and meta-analysis. METHODS: A literature search was conducted with respect to the diagnosis of PCD. English studies with five or more subjects were included. Successful biopsy was defined as an adequate ciliary sample to make or exclude the diagnosis of PCD. RESULTS: Eight studies met inclusion criteria. These studies included 1,993 patients who underwent 2,299 ciliary biopsies. Included studies were level 3 or 4 evidence. The weighted pooled proportion of obtaining an adequate specimen from a nasal biopsy was 76% (95% confidence interval [CI], 64%-86%) versus 66% (95% CI, 62%-69%) for a tracheobronchial site (P = 0.10). The pooled proportion of obtaining an adequate sample was 68% for both brush and forceps biopsy groups (95% CI, 58%-77% and 54%-81%, respectively). Nasal scraping yielded a higher proportion of adequate specimens (pooled proportion of 92%; 95% CI, 82%-99%) than other techniques (P = 0.002). CONCLUSION: Tissue biopsy is one component of diagnosing PCD. We found no significant difference between biopsy sites in terms of obtaining an adequate ciliary sample for EM evaluation. This suggests that nasal biopsy should be preferred for patients old enough to tolerate an office biopsy without the need for general anesthesia. The merits of nasal versus tracheobronchial biopsy in the operating room depend more on the risks and benefits of the procedure being performed. LEVEL OF EVIDENCE: NA Laryngoscope, 127:6-13, 2017.

Complications of turbinate reduction surgery in combination with tonsillectomy in pediatric patients.

OBJECTIVES/HYPOTHESIS: To examine whether the addition of turbinoplasty to tonsillectomy and adenoidectomy (T&A) increases the risk of postoperative complications. STUDY DESIGN: Retrospective cohort study of children (18 years old and younger) who underwent tonsillectomy and/or turbinoplasty between July 1, 2013 and June 30, 2015 using the 2016 Pediatric Health Information System administrative database METHODS: Patients were divided into three groups: 1) T&A and turbinoplasty, 2) T&A alone, and 3) turbinoplasty alone. Postoperative revisit, hemorrhage requiring cautery, and blood transfusion rates were compared between groups. RESULTS: A total of 75,761 patients met inclusion criteria: 3,079 underwent both T&A and turbinoplasty, 72,043 underwent T&A alone, and 639 underwent turbinoplasty alone. The rate of 14-day relevant revisits after T&A in combination with turbinate reduction surgery was not significantly higher than that of T&A alone (9.4% vs. 8.6%; P = .11). The revisit rate after turbinoplasty alone was 1.4%. Indications for revisits did not differ between the T&A and turbinoplasty group versus T&A alone group (P = .23). Furthermore, the rates of hemorrhage requiring cauterization was similar between the two groups (1.4% vs. 1.5%; P = .64). Twenty-one patients who underwent T&A alone required blood transfusion after they were readmitted; no cases in the other two groups required blood transfusion. CONCLUSIONS: Turbinoplasty and T&A performed together do not increase the risk of postoperative revisit or hemorrhage requiring cauterization, and can therefore be considered as a combined procedure. Pediatric patients will benefit from avoiding the additional risk of multiple anesthetics and repeated intubation. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1920-1923, 2017.

Nasal septal perforation in children: Presentation, etiology, and management.

OBJECTIVE: The presentation, etiology, and treatment of nasal septal perforation have been described in the adult literature; however, reports in the pediatric population are limited. In this study, we review our experience with pediatric nasal septal perforations with a focus on presentation, pathogenesis, management, and outcomes of surgical repair. METHODS: A retrospective chart review was performed on pediatric patients diagnosed with nasal septal perforations from 1998 to 2015. Data regarding patient demographics, perforation characteristics, and treatment were extracted and analyzed. RESULTS: Twenty-seven patients met inclusion criteria. Mean age was 10.8 years (range 2 months-17 years). Nasal crusting (n = 19, 73%) and epistaxis (n = 15, 58%) were the most common complaints at presentation. The most common etiologies were trauma (n = 9, 33%), iatrogenic sources (n = 5, 19%), and neoplasm (n = 3, 11%). Septal perforations were primarily located in the anterior septum (n = 17, 81%) and the average size was 0.9 cm (±0.37) in diameter. Four patients were managed with a nasal septal button. Successful closure was achieved in four out of six patients (66.7%) who underwent surgical repair. CONCLUSIONS: In our series, septal perforations in children occurred most frequently due to digital nasal trauma, and crusting was the most common symptom. Factors to consider prior to repair include symptomatology, the etiology of the perforation, co-morbidities, ability to comply with post-operative care/restrictions, availability of adjacent tissue/grafts, and potential effects on nasal growth. Even with careful consideration of these factors, successful closure was limited to two-thirds of patients who were offered repair.

The presentation and management of granulomatosis with polyangiitis (Wegener's Granulomatosis) in the pediatric airway.

OBJECTIVE: Granulomatosis with polyangiitis (GPA) is a necrotizing inflammatory disease that can affect the airway. The purpose of this study was to present a case of pediatric laryngotracheal GPA and provide management recommendations based on a thorough review of the literature. DATA SOURCES: Retrospective chart and literature review REVIEW METHODS: A pediatric patient with laryngotracheal and pulmonary manifestations of GPA who underwent chemotherapy and intralesional corticosteroid injection is described. An extensive literature review of pediatric GPA affecting the larynx/trachea was also performed. RESULTS: A pediatric patient presented with acute respiratory distress. Flexible laryngoscopy revealed a laryngeal mass. Magnetic resonance imaging showed circumferential subglottic stenosis, and chest computed tomography demonstrated multiple pulmonary nodules. Laryngeal and tracheal biopsy revealed granulation tissue and primary vasculitis. Labs demonstrated positive cytoplasmic antineutrophil cytoplasmic antibody, consistent with GPA. Methylprednisone, rituximab, cyclophosphamide, and intralesional steroid injection resulted in remission after 12 weeks. Review of the literature revealed two pediatric cases series and 10 case reports of GPA affecting the larynx or trachea. CONCLUSIONS: There is a higher prevalence of GPA of the airway in children when compared to adults. Biopsy of the airway lesion may not be necessary and has lower diagnostic yield compared to other GPA subsites. Medical management includes induction therapy followed by maintenance therapy once the disease is in remission. Most patients will require a surgical intervention to maintain the airway. LEVEL OF EVIDENCE: NA Laryngoscope, 127:233-240, 2017.

To stent or not to stent? A meta-analysis of endonasal congenital bilateral choanal atresia repair.

OBJECTIVES: The use of nasal stents as a postoperative adjunct following repair of choanal atresia remains controversial. The study objective was to systematically review the literature regarding the efficacy and safety of stenting following transnasal endoscopic repair of bilateral choanal atresia. STUDY DESIGN: Systematic review with meta-analysis. METHODS: A comprehensive search in PubMed, EMBASE, CINAHL, and the Cochrane Library was conducted. Inclusion criteria included articles written in the English language with five or more subjects and clear intervention data and outcomes. Two independent reviewers screened studies for eligibility, appraised the level of evidence, extracted data, and resolved discrepancies by consensus. Successful surgery was defined as the absence of restenosis. RESULTS: Of 154 identified studies, 15 met inclusion criteria. Levels of evidence varied from level 3 to 4. Mean age at surgery ranged from 5 days to 25 months. Thirteen studies (n = 167 patients) included patients who were stented; the weighted pooled proportion of successful surgery was 65% (95% confidence interval [CI], 49-76%). Mean duration of stenting ranged from 48 hours to 16 weeks. Six studies (n = 42) evaluated patients who were not stented; the weighted pooled proportion of successful surgery was 64% (95% CI, 42-84%). Complications associated with stenting included alar injury, vestibular stenosis, columellar tear, and stent dislodgement or blockage. CONCLUSIONS: Success rates for bilateral choanal atresia repair were similar with and without the use of nasal stents. The use of nasal stents may be associated with more complications. There is insufficient data to determine if mitomycin C is a useful therapeutic adjunct.

Endoscopic Radiofrequency Ablation-Assisted Resection of Juvenile Nasopharyngeal Angiofibroma: Comparison with Traditional Endoscopic Technique.

OBJECTIVES: To evaluate the feasibility of radiofrequency surgical instrumentation for endoscopic resection of juvenile nasopharyngeal angiofibroma (JNA) and to test the hypothesis that endoscopic radiofrequency ablation-assisted (RFA) resection will have superior intraoperative and/or postoperative outcomes as compared with traditional endoscopic (TE) resection techniques. STUDY DESIGN: Case series with chart review. SETTING: Two tertiary care pediatric hospitals. SUBJECTS AND METHODS: Twenty-nine pediatric patients who underwent endoscopic transnasal resection of JNA from January 2000 to December 2014. RESULTS: Twenty-nine patients underwent RFA (n = 13) or TE (n = 16) JNA resection over the 15-year study period. Mean patient age was not statistically different between the 2 groups (P = .41); neither was their University of Pittsburgh Medical Center classification stage (P = .79). All patients underwent preoperative embolization. Mean operative times were not statistically different (P = .29). Mean intraoperative blood loss and the need for a transfusion were also not statistically different (P = .27 and .47, respectively). Length of hospital stay was not statistically different (P = .46). Recurrence rates did not differ between groups (P = .99) over a mean follow-up period of 2.3 years. CONCLUSION: There were no significant differences between RFA and TE resection in intraoperative or postoperative outcome parameters.