Predictors of Sinonasal Improvement After Highly Effective Modulator Therapy in Adults with Cystic Fibrosis.

OBJECTIVES: The 22-question SinoNasal Outcome Test (SNOT-22) assesses chronic rhinosinusitis (CRS) severity. We aimed to identify predictors of SNOT-22 score improvement following highly effective modulator therapy (HEMT) initiation and to corroborate the SNOT-22 minimal clinically important difference (MCID) in adults with cystic fibrosis (CF). METHODS: Prospective observational data was pooled from four studies across 10 US centers investigating people with CF (PwCF) and CRS. Three studies evaluated HEMT's impact on CRS. For participants enrolled prior to HEMT initiation, SNOT-22 scores were obtained at baseline and after 3-6 months of HEMT. Multivariate regression identified predictors of improvement. Cronbach's alpha and four distribution-based methods were used to assess internal consistency and calculate the MCID of the SNOT-22. RESULTS: A total of 184 PwCF participated with mean baseline SNOT-22 scores ranging from 18.1 to 56.7. Cronbach's alpha was ≥0.90 across sites. Participants at sites with pre- and post-HEMT data reported improvement in SNOT-22 scores after initiating HEMT (all p < 0.05). Worse baseline SNOT-22 score (odds ratio (OR): 1.05, p < 0.001, 95% CI: 1.02-1.08), F508del homozygosity (OR: 4.30, p = 0.040, 95% CI: 1.14-18.99), and absence of prior modulator therapy (OR: 4.99, p = 0.017, 95% CI: 1.39-20.11) were associated with greater SNOT-22 improvement. The mean MCID calculated via distribution-based methods was 8.5. CONCLUSION: Worse baseline sinonasal symptoms, F508del homozygosity, and absence of prior modulator therapy predicted greater improvement after HEMT initiation. The mean MCID for SNOT-22 in PwCF is 8.5 points, similar to non-CF individuals with CRS, and provides a threshold specifically for PwCF. The SNOT-22 has strong internal consistency in PwCF. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Environmental impact on pediatric epistaxis and the utility of diagnostic studies: A single-institutional review.

OBJECTIVES: Pediatric epistaxis is a multifactorial disease entity. The objective of this study is to determine the socioeconomic and air-quality contributions to pediatric epistaxis. The study also evaluates the utility of diagnostic lab work as a predictor of bleeding rates and need for operative intervention. METHODS: A case series of pediatric patients treated in an outpatient Otolaryngology clinic at a tertiary care children's hospital in 2021 for epistaxis was performed. Patients with nasal bone trauma (n = 8), consult while inpatient (n = 7), and those with nasal masses (n = 2) were excluded; 181 patients met inclusion criteria. Demographic, clinical, socioeconomic, and air quality (tropospheric ozone, particulate matter) data were recorded. Associations with persistent bleeding and operative interventions were evaluated using logistic regression, Wilcoxon rank-sum, and Spearman rank correlation. RESULTS: Of the 181 patients, 75 (41.4%) were female. Forty-six of 181 (25.4%) had associated allergic symptoms. Twenty-six patients had allergy testing; 14/26 (53.8%) of these had positive results. Re-bleeding was more common in those with allergic symptoms (OR: 2.42, 95% CI: 1.22-4.78, p = 0.01). Patients with re-bleeding lived in counties with more days with ozone over the US standard (median 5 days, range 0-32 days) compared with those with no re-bleeding (median 3 days, range 0-32 days, p = 0.007). There was also an association between the number of visits for re-bleed and percent below poverty level (ρ = 0.259, p = 0.03) as well as the number of days with particulate matter levels over the US standard (ρ = 0.343, p = 0.01). Coagulopathy was present in 9/54 (16.7%) patients, with the majority being Von Willebrand disease (5/54, 9.3%). Easy bruising was not significantly associated with positive lab results. CONCLUSIONS: Environmental pollution, living in a zip code with more residents below the poverty level, and allergic rhinitis were positively associated with recurrent epistaxis. Understanding the geographic background of presenting patients may help direct workup and treatment options.

Elexacaftor-tezacaftor-ivacaftor decreases pseudomonas abundance in the sinonasal microbiome in cystic fibrosis.

BACKGROUND: Chronic rhinosinusitis (CRS) is common in individuals with cystic fibrosis (CF) and is marked by chronic inflammation and episodes of infection that negatively impact quality of life. Several studies have shown that elexacaftor-tezacaftor-ivacaftor (ETI) improves symptoms and examination findings in CF-CRS. The current study determines the effect of ETI on the sinonasal microbiota in CF. METHODS: Sinonasal samples were collected under endoscopic visualization before and after starting ETI. Samples were subjected to 16S amplicon sequencing and sequences were processed with the QIIME2 pipeline with subsequent analysis using the vegan R-package. RESULTS: Twenty-nine individual baseline samples and 23 sample pairs pre-/post-ETI were available. At baseline, the cohort had samples dominated by Staphylococcus, and alpha diversity was lower than that of a published reference set of individuals without sinonasal disease. Individuals with prior sinus surgery had lower alpha diversity as measured by Shannon Index, Observed Richness, and Faith's phylogenetic diversity Index. Beta diversity differed between individuals with and without allergic rhinitis, with higher Staphylococcus abundance in those with allergic rhinitis. No change in alpha or beta diversity was seen after a median of 9 months on ETI. With ETI, the Pseudomonas genus and the genus containing Burkholderia decreased in samples containing these taxa at baseline. Pseudomonas abundance decreased with treatment as measured by qPCR. Core sinonasal microbiome members Staphylococcus, Corynebacterium, and Streptococcus were unchanged, while Moraxella increased with ETI. CONCLUSIONS: Treatment with ETI leads to a reduction in Pseudomonas abundance within the sinonasal microbiome of individuals with Pseudomonas at baseline.

Use of patient-specific 3D printed models in pre-operative counseling for pediatric skull base surgery.

OBJECTIVES: Pediatric cranial base pathology is anatomically complex and surgical treatment is oftentimes difficult to conceptualize for patients and their families. Three-dimensional (3D) models of the sinuses and cranial base have the potential to enhance patient understanding in numerous domains. Our objective is to assess the use of 3D models in pre-operative parental and patient counseling prior to endoscopic endonasal skull base surgery in the pediatric population. METHODS: A survey was designed to assess parent and patient-perceived utility of 3D-printed models in surgical counseling prior to pediatric skull base surgery. RESULTS: A total of 10 patients were included. The median age was 9 years (range = 5 months-15 years). Pathology included juvenile nasopharyngeal angiofibroma (JNA) (N = 4), fibrous dysplasia of the maxilla and sphenoid (N = 1), juvenile ossifying fibroma (N = 1), nasal dermoid (N = 2, one with intracranial extension), encephalocele (N = 1), and parapharyngeal ectopic glial tissue (N = 1). Nearly all parents agreed or strongly agreed that 3D printed models were helpful in explaining the patient's skull base pathology (N = 10), surgical plan (N = 10), and possible complications (N = 9). All parents strongly agreed that 3D models should be used routinely in pre-operative counseling for endoscopic endonasal surgery. According to a majority of parents, patients older than 4 years old found the models helpful in understanding their pathology (75%) and surgery (88%). CONCLUSION: By allowing direct three-dimensional visualization of the target pathology, 3D models serve as a useful adjunct in enhancing patient comprehension of the pathologic entity, planned surgery, and potential complications prior to pediatric endoscopic endonasal skull base surgery.

Otorhinologic Disorders in 22q11.2 Deletion Syndrome.

OBJECTIVE: Investigate incidence and natural history of otologic and sinonasal disease associated with 22q11.2 deletion syndrome. STUDY DESIGN: Case series. SETTING: Tertiary care children's hospital. METHODS: Charts from consecutive children born 2000 to 2018 with a diagnosis of 22q11.2 deletion, DiGeorge, or velocardiofacial syndrome based on the International Classification of Diseases (ICD)-9 and ICD-10 codes were reviewed. Otologic and rhinologic diagnoses and surgeries and immune and microbiologic laboratory findings were collected from the medical record. RESULTS: After the exclusion of patients with no 22q11.2 deletion (n = 101), otologic care at an outside hospital (n = 59), and loss to follow-up prior to 3 years of age (n = 22), 128 were included. Males comprised 80 (62.5%) patients, 115 (89.8%) were white, and the median age at genetic confirmation of 22q11.2 deletion was 119 days (range 0 days to 14.6 years). Recurrent acute otitis media (RAOM), chronic otitis media with effusion, chronic rhinosinusitis, and recurrent acute sinusitis were diagnosed in 54 (42.2%), 37 (28.9%), 10 (7.8%), and 8 (6.3%), respectively. Tympanostomy tubes were placed in 49 (38.3%). Adenoidectomy and sinus surgery were performed in 38 (29.7%) and 4 (3.1%), respectively. Neither immunoglobulin nor cluster of differentiation deficiency increased the odds of RAOM diagnosis, tympanostomy tube placement, or chronic/recurrent sinusitis. Methicillin-resistant Staphylococcus aureus was the most common organism in sinus cultures (4/13, 30.8%). Streptococcus pneumonia dominated otorrhea cultures (11/21, 52.4%). CONCLUSION: Approximately half of children with 22q11.2 deletion may experience otologic disease that often requires surgical management. Future studies will utilize a larger cohort to examine the role of immunodeficiency in otologic and rhinologic disease in this population.

Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis.

BACKGROUND: Many individuals with cystic fibrosis (CF) have chronic rhinosinusitis resulting in nasal obstruction, sinus infections, and repeated surgeries. Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator therapy approved for individuals aged 6 years or older with CF who have at least one F508del allele or other responsive mutation. The current study tests the hypothesis that ELX/TEZ/IVA improves sinonasal disease in CF. METHODS: The study was a pre/post, observational cohort study conducted at two sites. Participants underwent a study visit prior to starting ELX/TEZ/IVA and a second visit at a median of 9 months on therapy. Each visit included sinus CT scan, rigid nasal endoscopy, and sweat chloride measurement. Symptoms were measured with the 22 item Sinonasal Outcome Test at scheduled intervals during the study. Regression models were used to test for improvement in symptoms, endoscopy, and CT scales. RESULTS: The study enrolled 34 individuals, with a median age of 27 years (range 12-60). Symptoms improved within 7 days of therapy and plateaued by day 28. Endoscopic crusting resolved and nasal polyposis improved, with a decrease in size or resolution of polyps. Sinus opacification and mucosal thickening improved on CT radiographs with treatment. CONCLUSIONS: Sinonasal symptoms improved rapidly and durably for at least 180 days on ELX/TEZ/IVA therapy. Objective measures of disease including endoscopic and CT findings improved with ELX/TEZ/IVA.

Long Term Incidence and Outcomes of Sinonasal and Otologic Disease in Patients With Pyriform Aperture Stenosis and Choanal Atresia.

ABSTRACT: Pyriform aperture stenosis (PAS) and choanal atresia (CA) are 2 anatomic causes of newborn nasal obstruction. The goal of management of PAS and CA is to establish a patent nasal airway, often requiring surgery. No previous study has sought to assess the long term sinonasal and otologic disease incidence and outcomes in the PAS and CA population after surgical intervention. The goal of this study was to investigate whether surgical intervention in PAS and CA is correlated with the long-term development of sinonasal disease or otologic disease (either recurrent acute otitis media or chronic otitis media with effusion). Patients with a diagnosis of PAS or CA who underwent surgical intervention were retrospectively identified. Pertinent demographic risk factors, medical and syndromic diagnoses, number of surgical interventions, types of surgical interventions, and presence of sinonasal and otologic diseases were assessed. Fifty-three patients were included in the study: 8 patients with PAS and 45 with CA. The average follow-up time was 2.9 years. No PAS patients developed otologic or sino-nasal disease. Four of 45 patients with CA developed recurrent acute sinusitis (3 non-syndromic and 1 syndromic) and 19 of 45 patients developed otologic disease (9 non-syndromic and 10 syndromic). Coloboma, Heart, Choanal Atresia, Growth Retardation, Genitourinary, Ear Syndrome and unilateral CA correlated significantly with the subsequent development of otologic disease; however, the number of surgeries did not. This study suggests that surgery for PAS and CA do not increase the risk of long-term development of sinonasal or otologic disease.

Cost analysis of sialendoscopy for the treatment of salivary gland disorders in children.

INTRODUCTION: Recent advances in Otolaryngology have changed the diagnosis and therapy for salivary gland disorders. Sialendoscopy-assisted surgery is a minimally invasive, conservative procedure for functional preservation of the affected gland. The goals of this study are to assess the indications, use, and outcomes of pediatric sialendoscopy at a tertiary pediatric institution as well as to analyze the direct cost related to the diagnosis and treatment of patients with sialolithiasis and Juvenile Recurrent Parotitis managed with sialendoscopy. METHODS: Retrospective cohort study of pediatric patients undergoing diagnostic and/or therapeutic sialendoscopy at a tertiary level children's hospital between 2012 and 2020. Demographic, clinical, surgical variables and direct hospital costs 1 year before and after the sialendoscopy procedure were collected and analyzed. RESULTS: Twenty-two pediatric patients were included. There was male predominance (59.3%). The most common indication for sialendoscopy was Juvenile Recurrent Parotitis. Average age of onset was 6.5 years for patients with JPR and 14.2 years for patients with sialolithiasis. All patients had an average of 4.5 episodes before the first procedure. 8 patients required repeat sialendoscopy for recurrent symptoms. Mean total hospital costs were significantly higher in patients with JRP 1 year before and after the sialendoscopy ($4308.8 vs. $3330) compared to patients with sialolithiasis. Costs of the sialendoscopy and related expenses including anesthesia and PACU cost were similar in both studied groups ($13,506 vs. $13,022.9). Complete resolution of symptoms was achieved in 14 patients with JRP and all patients with sialolithiasis. CONCLUSION: Sialendoscopy is a low-risk procedure that aids in the treatment for pediatric salivary gland disorders. The costs related to sialendoscopy are substantial and patients with JRP incur higher hospital preoperative and postoperative costs compared to patients with sialolithiasis.

Efficacy of inferior turbinate reduction in pediatric patients: a prospective analysis.

BACKGROUND: Chronic nasal congestion is a common presentation in the pediatric setting. For patients who fail to respond to maximal medical therapy and adenoidectomy, inferior turbinate reduction (ITR) may be a reasonable adjuvant surgical approach. Our objective was to prospectively evaluate nasal congestion symptoms and daily medication use in the year following ITR in children with and without allergic rhinitis (AR). METHODS: Patients younger than 18 years undergoing ITR alone or in combination with nonsinonasal procedures were enrolled. Scores from the Nasal Obstruction Symptom Evaluation (NOSE) survey were collected preoperatively and at 3, 6, and 12 months postoperatively from 96 patients. Number and type of pertinent daily medications utilized were also analyzed using Wilcoxon signed-rank and McNemar's tests. Patients with and without comorbid AR were then compared using Wilcoxon rank-sum tests. RESULTS: Median (range) NOSE sum scores significantly improved from preoperative median of 65 out of 100 points (0-100) to 20 (0-100), (z = 7.12, p < 0.001) at 12 months postoperatively. The median number of daily medications was significantly reduced from 1 (range, 0-4) preoperatively to 0 (range, 0-5) postoperatively (z = 2.38, p = 0.017). Fifty-six (58.3%) patients had AR. AR patients received more medications preoperatively and postoperatively compared with those without AR (z = -2.96, p = 0.003; z = -2.23, p = 0.024). At 12 months, NOSE scores remained significantly greater in patients with AR compared with those without AR (ß = 11.6; 95% confidence interval [CI], 0.676-22.5; p = 0.038). CONCLUSION: Our data highlight the need for a multimodal approach to chronic nasal congestion, particularly in children with AR.

Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients.

PURPOSE: Pediatric cystic fibrosis (CF) patients have a variable onset, severity, and progression of sinonasal disease. The objective of this study was to identify genotypic and phenotypic factors associated with CF that are predictive of sinonasal disease, recurrent nasal polyposis, and failure to respond to standard treatment. METHODS: A retrospective case series was conducted of 30 pediatric patients with CF chronic rhinosinusitis with and without polyps. Patient specific mutations were divided by class and categorized into high risk (Class I-III) and low risk (Class IV-V). Severity of pulmonary and pancreatic manifestations of CF, number of sinus surgeries, nasal polyposis and recurrence, age at presentation to Otolaryngology, and Pediatric Sinonasal Symptom Survey (SN-5)/Sinonasal Outcome Test (SNOT-22) scores were examined. RESULTS: 27/30 patients (90%) had high risk mutations (Class I-III). 21/30 (70.0%) patients had nasal polyposis and 10/30 (33.3%) had recurrent nasal polyposis. Dependence on pancreatic enzymes (23/27, 85.2% vs 0/3, 0.0%, p = 0.009) and worse forced expiratory volumes (FEV1%) (mean 79, SD 15 vs mean 105, SD 12, p = 0.009) were more common in patients with high risk mutations. Insulin-dependence was more common in those with recurrent polyposis (5/10, 50% vs 2/20, 10%, p = 0.026). There was no statistical difference in ages at presentation, first polyps, or sinus surgery, or in polyposis presence, recurrence, or extent of sinus surgery based on high risk vs. low risk classification. CONCLUSION: CF-related diabetes was associated with nasal polyposis recurrence. Patients with more severe extra-pulmonary manifestations of CF may also be at increased risk of sinonasal disease.

Clinical and Radiologic Characterization of Frontal Sinusitis in the Pediatric Population.

INTRODUCTION: Frontal sinusitis in the pediatric population is a disease that has not been thoroughly studied or characterized. The goals of this study are to characterize the clinical presentation, radiologic variables, treatment modalities, complications, and prognosis associated with acute and chronic frontal sinus disease in the pediatric population. METHODS: IRB-approved retrospective cohort study of pediatric patients who were diagnosed with acute (AFS) or chronic frontal sinusitis (CFS) and underwent frontal sinus surgery at a tertiary level Children's Hospital from 2006 to 2016. Patients with AFS were compared to patients with CFS. Statistical analysis completed using chi-square test or Fisher's exact test, statistical significance set at P < .05. RESULTS: A total of 19 patients with AFS and 15 patients with CFS were analyzed. There was a male predominance in AFS and female predominance in CFS (P < .05).AFS patients were less likely to have allergies, prior sinus disease, or significant comorbidities (P < .05).Additionally, AFS patients presented with constitutional, neurologic, and ocular symptoms. The CFS group had predominantly sinonasal symptoms. CT-scan analysis showed that AFS patients had higher prevalence of complex frontal anatomy (Type-II cells, concha bullosa) compared with CFS patients (P < .05). Culture results were positive in 78% of the AFS group, with S. Anginosus (53%), Anaerobes (20%), and normal flora (17%). In the CFS group cultures were positive in 60% of the patients, 56% grew normal flora, 13% H. Influenzae, 6.5% Pseudomonas, and 24.5% other species. CFS patients were more likely to have persistent sinus disease and require repeat sinus procedures (P < .05). CONCLUSION: There are 2 distinct presentations of frontal sinus disease in the pediatric population. Patients with AFS vary significantly from those with CFS. Males, ages 13 to 18 years old, who cultured positive for S. Anginosus (former S.Milleri) dominated the AFS subgroup. Whereas as female patients with a history of allergic rhinitis and muco-cilliary disease were more prominent in the CRS subgroup. Correct identification and understanding of these 2 different entities are crucial for the appropriate short and long-term patient management.

The microbiome of pediatric patients with chronic rhinosinusitis.

BACKGROUND: This study aimed to compare the microbiota of pediatric patients with chronic rhinosinusitis (CRS) who are undergoing adenoidectomy to treat their disease with that of healthy control patients. METHODS: Patients undergoing adenoidectomy-only for obstructive sleep apnea (n = 50) and CRS (n = 37) were recruited. Preoperative 22-item Sino-Nasal Outcome Test (SNOT-22) or Sinus and Nasal Quality of Life Survey (SN-5) were collected. Each patient had samples collected from their nasopharynx (adenoid bed) and nasal cavity (sinus) at the onset of surgery. 16S ribosomal ribonucleic acid (rRNA) gene sequencing was subsequently performed to obtain per sample taxonomic abundances. Statistical analyses included permutational multivariate analysis of variance (PERMANOVA), alpha (within sample) diversity measures, and changes in taxonomic abundance. RESULTS: Moraxella was the most abundant organism. Nasopharyngeal swabs demonstrated higher alpha diversity compared to the nasal cavity. The diversity was not different based on CRS vs obstructive history. There was an increase in diversity with increasing age, and eczema contributed to a greater difference in diversity between the nasopharynx and nasal cavity. Diversity was not affected by adenoid size; however, use of nasal steroids, inhaled steroids, and antihistamines influenced diversity in both the nasopharynx and nasal cavity. Nasopharyngeal samples were higher in relative abundance for Fusobacterium, Prevotella, Porphyromonas, and Campylobacter compared to the nasal cavity. CONCLUSION: The nasopharynx and nasal cavity differed in both microbiota composition and diversity. In contrast, no significant difference in composition or diversity were found in CRS vs control patients. Ecological changes in the nasopharyngeal and sinus site may contribute to the etiology for adenoid hypertrophy in both healthy controls and CRS patients.

The Pediatric Sinus Staging System: A Computed Tomography-Based Approach to Grading Pediatric Sinus Disease.

OBJECTIVES/HYPOTHESIS: Pediatric chronic rhinosinusitis (CRS) is a prevalent disease with few objective measurements available to predict which patients will require surgical intervention. The Lund-Mackay (LM) score for computed tomography (CT) scans is one objective data point available for the adult population; however, a dedicated scoring system in the pediatric population has not been popularized. We present a Pediatric Sinus Staging System (PSSS) that considers both opacification and the varying developmental stages of each sinus. STUDY DESIGN: Retrospective chart review. METHODS: We analyzed CT scans of pediatric patients with a diagnosis of CRS. Both LM and PSSS scores were calculated for each scan. Groups were formed based on treatment outcomes and included patients who were treated successfully with medical therapy and/or adenoidectomy (med/adenoid), patients who required functional endoscopic sinus surgery (FESS), and patients who required revision FESS. RESULTS: Overall, 76 patient scans were reviewed. PSSS values were significantly less than LM for the control group (P = .001) and significantly higher for patients with cystic fibrosis (P = .027) and with CRS with polyps (P = .001). The ideal cutoff for PSSS to distinguish between med/adenoid and single FESS treatment with a sensitivity 90.6% and specificity of 50.0% was ≥2. CONCLUSIONS: The PSSS gives a more descriptive score by accounting for the opacification and pneumatization of each sinus. Our current results show similar values between PSSS and LM scores, which suggests internal validity. In addition, a PSSS score of ≥2 may help physicians better counsel families on the likelihood of requiring FESS. Further investigation is needed to fully validate the PSSS. LEVEL OF EVIDENCE: 3b Laryngoscope, 131:E642-E648, 2021.

Silent sinus syndrome in children.

INTRODUCTION: Silent sinus syndrome (SSS) is defined as a progressive enophthalmos and hypoglobus associated with maxillary sinus atelectasis. There is extremely limited literature describing SSS in children. The goals of this study are to characterize SSS in children through an IRB approval retrospective chart review of cases identified through a large health system-wide imaging database and to compare the presentation and outcomes of patients who underwent surgery versus those who were observed. METHODS: A radiology database of over 26 million reports from 2003 to 2017 was searched to identify children aged 1-18 years diagnosed with maxillary sinus hypoplasia or SSS on CT scan. Chart review was performed on the identified children including clinical presentation, eye symptoms, surgical treatment, and outcome. RESULTS: Eighty-three children were identified to have maxillary sinus hypoplasia. Eighty-one patients had maxillary sinus opacification and 57 patients had hypoglobus or enophthalmos characteristic of SSS. Thirty-two patients (47%) were seen by a specialist and 19 had surgery. The majority of patients (55%) had headache as their presenting symptom. There were no statistically significant differences in the clinical presentation between those who received surgery and those who were observed clinically. CONCLUSIONS: Silent sinus syndrome can present at any age. The majority of cases of maxillary sinus hypoplasia will have the orbital floor changes characteristic of SSS. Headaches are a common presenting symptom. Close follow up of pediatric patients is advised and early intervention may be favorable to prevent long term orbital changes and complications.

The use and cost-effectiveness of intraoperative navigation in pediatric sinus surgery.

OBJECTIVES/HYPOTHESIS: There are consensus statements about when to use intraoperative navigation (IN) in adult sinus surgery. However, no corresponding guidelines exist for pediatrics. Our objectives included: 1) assess the demographic and operative factors associated with IN use and 2) calculate the cost-effectiveness of IN use. STUDY DESIGN: Retrospective chart review. METHODS: One hundred nineteen pediatric patients undergoing sinus surgery between 2003 and 2016 were reviewed. Demographic and surgical factors were collected from medical records. Costs associated with use of IN were gathered from billing records. RESULTS: Of the 119 patients, 60 underwent sinus surgery with navigation (wIN) and 59 underwent surgery without navigation (sIN). Children in the wIN group had more complex surgeries with more sinuses opened (P = .008). Individual attending surgeon and presence of trainee were associated with increased use of IN (P < .001 for both). IN resulted in a median of 31.5 minutes longer surgical time (P < .001). IN had an incremental cost/effectiveness ratio (ICER) of $22,378 for each year without revision surgery for patients with acute disease. However, for patients with chronic disease, the probability of undergoing a second surgery was the same between wIN and sIN groups, and navigation was not cost-effective (ICER of -$3,583). CONCLUSIONS: IN use did not decrease complications or rates of revision surgery. It was used primarily as an educational tool or to increase confidence in intraoperative identification of landmarks. However, the use of IN added surgical time and was not cost-effective. Further research must be completed to determine when IN is indicated in pediatric sinus surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 2019.

Pediatric nasal septal perforation.

OBJECTIVE: 1.) Describe demographic and clinical characteristics of pediatric nasal septal perforations (NSP), 2.) Analyze efficacy of treatment modalities in symptom management and resolution of pediatric NSP, 3.) Describe the surgical technique of external rhinoplasty with vascularized nasal septal flaps in the treatment of nasal septal perforations. METHODS: IRB-approved retrospective chart review of pediatric patients ages 0-18 years with nasal septal perforations treated at a tertiary care pediatric otolaryngology practice. Demographic and clinical characteristics including gender, age, race, and presenting symptoms, and location, size, and etiology of perforation were collected. Outcomes including persistence of perforation and symptoms at 1 year and most recent visit were recorded. A total of 20 patients were included. Statistical analysis used Fisher's t-test for categorical variables and Wilcoxon rank-sum for continuous variables. RESULTS: Median age was 167.5 months (1.5-221.0). The most common etiology was iatrogenic (40%), followed by button battery (20%). Thirty percent of patients underwent surgical repair. Fifty percent of patients who underwent surgical repair achieved closure of their perforation at most recent follow up. CONCLUSION: Pediatric NSP is a challenging issue with limited literature to date. Iatrogenic causes (40%) and button batteries (20%) were the most common etiologies of nasal septal perforation in our study. We introduce an advancement in our center's surgical technique with a case illustration with repair via external rhinoplasty and bilateral vascularized nasal septal flaps. Future larger studies may further elucidate characteristics and treatment modalities associated with successful closure.

The Natural Growth Rate of Residual Juvenile Angiofibroma.

Objectives Examine the postoperative growth rate of residual juvenile angiofibroma (JA) in a large series of patients relative to pediatric growth parameters and other prognostic factors. Establish an algorithm for postoperative surveillance of patients with JA. Design Retrospective case series. Setting Tertiary referral academic center. Participants Pediatric patients undergoing surgical resection of JA between September 2005 and June 2015. Main Outcome Measures Postoperative recurrence and tumor growth rates. Results Thirty-eight patients were identified with a mean follow-up of 24.1 months. Sixty-eight percent (26/34) of patients achieved gross total resection, and 32% (12/38) had persistent postoperative disease. Of those with postoperative residual tumor, all had preoperative skull base involvement and residual blood supply from the internal carotid artery following embolization. Sixty-seven percent (8/12) of patients with postoperative residual tumor demonstrated radiographic stability of disease with scans being completed approximately every 6 months. Radiographic rates of disease progression ranged from 4.1 to 9.22 mm/year. Conclusions Patients with preoperative skull base involvement and residual vascularity following preoperative embolization are more likely to have residual postoperative disease, and a longer postoperative follow-up is warranted in these patients. Progression of residual disease occurred approximately 7.5 months postoperatively. Younger patients may be more likely to need further intervention for postoperative residual disease, and postoperative imaging at 6-month intervals appears appropriate.

Outcomes of adenoidectomy-alone in patients less than 3-years old.

OBJECTIVES: 1. Determine the percentage of patients under the age of 3 undergoing adenoidectomy-alone who require subsequent management of residual sleep disordered breathing (SDB).2. Characterize complications following adenoidectomy and determine if any perioperative factors are associated with intra-operative or post-operative complications and outcomes. METHODS: Case series with chart review was conducted including children seen at a tertiary care children's hospital between 2008 and 2012. Consecutive patients under the age of 3 who underwent adenoidectomy-alone were identified by billing codes. After excluding those with syndromes, partial adenoidectomies, and those without follow-up, 148 patients were included. Predictors of requiring additional surgery for SDB were evaluated using log-rank tests or Cox proportional hazards regression. RESULTS: Median age at time of initial adenoidectomy was 27.5 months (range 11-36 months) and the patient population was comprised of 66.2% males (n = 98/148) and 89.2% Caucasians (n = 132/148). 56.5% (n = 74/131) of patients continued to have residual symptoms of SDB and 34.5% (n = 51/148) underwent additional surgical intervention. Multivariable survival analysis revealed GERD (HR, 6.21; CI, 1.29-29.77, p = .022) and tonsil size (HR, 4.07; CI, 1.57-10.51, p = .004) were significant predictors of additional surgery in this group of patients under the age of 3. There was no observed difference in intra- and post-operative complication rates between patients with and without additional operative intervention. CONCLUSIONS: Residual SDB symptoms following adenoidectomy in patients less than 3 years of age are common and require additional surgery at a high rate. Medical comorbidities such as GERD and large tonsil size may help predict the need for additional surgery.

Risk factors for cerebrospinal fluid leak in pediatric patients undergoing endoscopic endonasal skull base surgery.

OBJECTIVES: To determine the risk factors associated with cerebrospinal fluid (CSF) leak following endoscopic endonasal surgery (EES) for pediatric skull base lesions. METHODS: Retrospective chart review of pediatric patients (ages 1 month to 18 years) treated for skull base lesions with EES from 1999 to 2014. Five pathologies were reviewed: craniopharyngioma, clival chordoma, pituitary adenoma, pituitary carcinoma, and Rathke's cleft cyst. Fisher's exact tests were used to evaluate the different factors to determine which had a statistically higher risk of leading to a post-operative CSF leak. RESULTS: 55 pediatric patients were identified who underwent 70 EES's for tumor resection. Of the 70 surgeries, 47 surgeries had intraoperative CSF leaks that were repaired at the time of surgery. 11 of 47 (23%) surgeries had post-operative CSF leaks that required secondary operative repair. Clival chordomas had the highest CSF leak rate at 36%. There was no statistical difference in leak rate based on the type of reconstruction, although 28% of cases that used a vascularized flap had a post-operative leak, whereas only 9% of those cases not using a vascularized flap had a leak. Post-operative hydrocephalus and perioperative use of a lumbar drain were not significant risk factors. CONCLUSIONS: Pediatric patients with an intra-operative CSF leak during EES of the skull base have a high rate of post-operative CSF leaks. Clival chordomas appear to be a particularly high-risk group. The use of vascularized flaps and perioperative lumbar drains did not statistically decrease the rate of post-operative CSF leak.