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.

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.

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.

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.

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.

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.

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.

The role of tympanostomy tubes in surgery for acquired retraction pocket cholesteatoma.

OBJECTIVE: 1. To determine whether intraoperative tympanostomy tubes (TT) during surgery for acquired retraction pocket cholesteatoma (ARPC) can decrease recurrence of ARPC and retraction pockets (RP). 2. To determine the need for subsequent TT in children that did not initially receive TT. STUDY DESIGN: Retrospective review of children who underwent primary surgery for ARPC. SETTING: Tertiary care children's hospital. SUBJECTS AND METHODS: Audiometry, operative reports, office findings, and recurrence of ARPC and RP were analyzed. Univariate analysis with Fisher's exact test, Wilcoxon rank-sum and t test, as well as multivariate analysis with logistical regression, were used for statistical analyses. RESULTS: 21 patients had TT at initial surgery and 34 patients did not. The recurrence of ARPC was 24% for the TT Group versus 44% for the no TT group (p=0.09). The incidence of RP was 33% in the TT group versus 41% in the no TT groups (p=0.35). 35% of the no TT group subsequently required TT. Audiometric outcomes were not different between groups. TT placement did not significantly affect the odds of recurrent ARPC and RP (95% CI 0.12-1.83, p=0.28 for ARPC and 95% CI 0.30-4.60, p=0.82 for RP). However, the odds of recurrent ARPC and RP were significantly increased in children with stapes and malleus erosion by cholesteatoma. Children with stapes and malleus involvement had 5.28 and 11.8 times higher odds of recurrent ARPC compared to those without ossicular erosion (95% CI 1.09-25.6, p=0.04 stapes and 95% CI 1.58-88.3, p=0.02 malleus). Similarly, children with malleus involvement had an 18.6 times higher odds of recurrent RP (95% CI 1.62-214, p=0.02) compared to those who did not. Incus erosion was not a significant predictor for recurrent ARPC and RP. CONCLUSION: There was no statistical difference in recurrence or audiometric outcomes between patients who underwent TT versus those who did not during initial surgery for ARPC. However malleus and stapes erosion were significantly associated with recurrent ARPC and RP. These findings suggest that TT at initial ARPC surgery did not play a role in preventing recurrence, but malleus and incus erosion, was predictive of recurrent disease. A larger, prospective study of TT at initial surgery for ARPC is needed.

Endoscopic endonasal surgery for benign fibro-osseous lesions of the pediatric skull base.

OBJECTIVES/HYPOTHESIS: To describe the presentation, treatment, and outcomes of benign fibro-osseous tumors involving the skull base in a pediatric population. METHODS: Retrospective chart review from January 2002 to September 2013 of pediatric patients (ages 0-18 years) who underwent endoscopic endonasal surgery (EES) for benign fibro-osseous tumors involving the skull base. RESULTS: Fourteen patients were identified with an age range of 2.7 to 17.9 years (mean, 12.5 years). Six juvenile ossifying fibromas, five benign fibro-osseous lesions, two osteomas, and one fibrous dysplasia were treated. Ocular symptoms and nasal obstruction were the most common presenting symptoms in nine (64%) and six (43%) of patients, respectively; five (36%) presented with proptosis and four (29%) with diplopia. Two (14%) patients had cranial nerve VI palsy. Transsellar and transclival approaches were used in five (36%) of patients. Orbital and optic nerve decompressions were the most common components of the approaches performed in nine (64%) of the surgeries. Gross total resection (GTR) was achieved with single-stage surgery in 10 (71%) patients; two additional patients underwent staged GTR. Two intraoperative cerebrospinal fluid (CSF) leaks occurred and were repaired endoscopically. There were no postoperative CSF leaks or infectious complications. Two patients had transient diplopia, and two had transient diabetes insipidus, all of which resolved. The mean follow-up was 13.8 months. Two patients had a recurrence, and both required additional EES achieving GTR. CONCLUSIONS: EES for benign fibro-osseous tumors of the skull base is a safe and effective treatment for excision of these lesions in the pediatric population. LEVEL OF EVIDENCE: 4.

The impact of nasal surgery on sleep quality: a prospective outcomes study.

OBJECTIVE: (1) Evaluate the impact of nasal airway surgery on sleep quality using validated outcome measurements, (2) compare the utility of Epworth Sleepiness Scale (ESS) versus Pittsburgh Sleep Quality Index (PSQI) as a reflection of sleep quality, and (3) identify perioperative variables that might correlate with a beneficial effect of nasal surgery on sleep quality. STUDY DESIGN: Prospective outcome study of patients with symptomatic nasal obstruction undergoing nasal airway surgery. SETTING: Academic medical center. METHODS: Patients completed the Nasal Obstruction Symptom Evaluation (NOSE) scale, ESS, PSQI, and Ease-of-Breathing and Sleep Quality Likert scales preoperatively and 3 months postoperatively. A nonparametric analysis compared pre- and postoperative values, and associations were examined using Spearman correlations. RESULTS: Sixty-one patients completed the study. Mean NOSE scores decreased significantly from 68.2 preoperatively to 17.5 three months after surgery. Mean ESS scores and PSQI scores improved (P < .0001) over that same interval (7.5 to 5.3 and 7.8 to 4.6, respectively). There was a correlation seen between the degree of change in both NOSE scores and Ease-of-Breathing scores and the change in sleep quality measured using the PSQI or Sleep Quality Likert scores. The PSQI correlated better with Sleep Quality Likert scores than the ESS. Overall, 86.9% of subjects reported subjective improvement in sleep quality postoperatively. CONCLUSION: In patients undergoing nasal airway surgery there may be a secondary improvement in subjective sleep quality. The degree of change in sleep quality correlates with the severity of nasal obstruction preoperatively and the degree of improvement in obstruction with surgery.

Congenital cholesteatoma: predictors for residual disease and hearing outcomes.

OBJECTIVE: To determine predictive factors for residual disease and hearing outcomes of surgery for congenital cholesteatoma (CC). DESIGN: Retrospective record review of surgery for CC from January 1, 1998, through December 31, 2010. The initial extent of CC was staged using the system as defined by Potsic et al. SETTING: Tertiary care children's hospital. PATIENTS: Eighty-one children (82 ears) underwent a total of 230 operations for CC. The mean (SD) age was 5.3 (2.9) years, and the mean follow-up was 4.3 years. INTERVENTION: Initial and subsequent operations for CC and audiologic evaluations. MAIN OUTCOME MEASURES: Statistical analyses were performed to determine factors associated with increased residual disease for CC and poorer hearing outcomes. RESULTS: Higher initial stage of disease, erosion of ossicles, and removal of ossicles were significantly associated with increased likelihood of residual CC (46%, 50%, and 51%, respectively; P < .001). More extensive disease at initial surgery was associated with poorer final hearing outcomes (P < .05). Other significant findings included CC medial to the malleus (41.5%) or incus (54.3%), abutting the incus (51.3%) or stapes (63%), or enveloping the stapes (50%); all patients had increased residual disease (all P < .05). Excellent audiometric results (air-bone gap of ≤20 decibel hearing level) were obtained in 63 (77%) of the 82 ears. CONCLUSIONS: More extensive initial disease, ossicular erosion, and the need for ossicular removal were associated with residual disease. On the basis of our data, the best chance for completely removing CC at initial surgery involves removing involved ossicles if they are eroded, if the CC is abutting or enveloping the incus or stapes, if the CC is medial to the malleus or incus, or if the matrix of the CC is violated. These results may help guide surgeons to achieve the best results for their patients.

Perioperative incidence and management of hyponatremia in vWD patients undergoing adenotonsillectomy.

OBJECTIVES/HYPOTHESIS: To analyze the incidence and severity of hyponatremia in patients receiving synthetic desmopressin (DDAVP) in the perioperative setting of oropharyngeal surgery in the treatment of von Willebrand disease and to propose a standardized protocol for perioperative fluid resuscitation and postoperative sodium monitoring after DDAVP administration. STUDY DESIGN: Retrospective medical record review. METHODS: A retrospective medical record review in an academic pediatric medical center was conducted. From October 1, 2002, to February 1, 2009, all patients undergoing adenotonsillectomy and receiving DDAVP preoperatively for the treatment of von Willebrand disease were identified. A total of 76 patients were identified by initial database review; 63 patients were included in the study, and 13 patients were excluded secondary to incomplete data. DDAVP dose and timing, perioperative fluid volume and composition, and postoperative sodium levels were collected. Extreme adverse events related to hyponatremia were recorded. RESULTS: Forty-seven of 63 (74.6%) patients developed some degree of hyponatremia after DDAVP administration, and six of 63 (9.5%) patients developed extreme hyponatremia, with the degree of hyponatremia related to the volume of perioperative fluid resuscitation. The sodium nadir occurred within 9 to 20 hours after DDAVP administration. No serious adverse events related to hyponatremia were recorded during the study period. CONCLUSIONS: The incidence of hyponatremia in children receiving DDAVP for prophylaxis of intraoperative bleeding following oropharyngeal surgery is high. The degree of hyponatremia is related to the perioperative fluid volume administered. A protocol for DDAVP administration, perioperative fluid resuscitation, and postoperative sodium monitoring that aims to reduce the incidence of hyponatremia in this population is proposed.