High Prevalence of Cochlear Nerve Deficiency in Pediatric Patients With Cochlear Aperture Stenosis.

OBJECTIVE: Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS. STUDY DESIGN: Retrospective study. SETTING: Tertiary care center. METHODS: We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI. RESULTS: Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartile range [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears. CONCLUSION: CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis.

Congenital Cytomegalovirus Testing Outcomes From the ValEAR Trial.

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

Electrode array positioning after cochlear reimplantation from single manufacturer.

OBJECTIVE: To investigate whether revision surgery with the same device results in a change in three key indicators of electrode positioning: scalar location, mean modiolar distance (M¯), and angular insertion depth (AID). METHODS: Retrospective analysis of a cochlear implant database at a university-based tertiary medical center. Intra-operative CT scans were obtained after initial and revision implantation. Electrode array (EA) position was calculated using auto-segmentation techniques. Initial and revision scalar location, M¯, and AID were compared. RESULTS: Mean change in M¯ for all ears was -0.07 mm (SD 0.24 mm; P = 0.16). The mean change in AID for all ears was -5° (SD 67°; P = 0.72). Three initial implantations with pre-curved EAs resulted in a translocation from Scala Tympani (ST) to Scala Vestibuli (SV). Two remained translocated after revision, while one was corrected when revised with a straight EA. An additional five translocations occurred after revision. CONCLUSIONS: In this study examining revision cochlear implantation from a single manufacturer, we demonstrated no significant change in key indicators of EA positioning, even when revising with a different style of electrode. However, the revision EA is not necessarily confined by the initial trajectory and there may be an increased risk of translocation.

Cochlear Implantation in Very Young Children With Single-Sided Deafness.

OBJECTIVE: Cochlear implants (CIs) for single-sided deafness (SSD) have only been approved for patients 5 years and older despite data supporting that younger children can also benefit from implantation. This study describes our institution's experience with CI for SSD in children 5 years and younger. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. METHODS: A case series with chart review identified 19 patients up to age 5 years who underwent CI for SSD between 2014 and 2022. Baseline characteristics, perioperative complications, device usage, and speech outcomes were collected. RESULTS: The median age at CI was 2.8 (range, 1.0-5.4) years, with 15 (79%) patients being below age 5 at implantation. Etiologies of hearing loss were idiopathic (n = 8), cytomegalovirus (n = 4), enlarged vestibular aqueduct (n = 3), hypoplastic cochlear nerve (n = 3), and meningitis (n = 1). The median preoperative pure-tone average was 90 (range, 75-120) and 20 (range, 5-35) dB eHL in the poor and better hearing ears, respectively. No patients had postoperative complications. Twelve patients achieved consistent device use (average, 9 h/d). Three of the seven who were not consistent users had hypoplastic cochlear nerves and/or developmental delays. The three patients with available preoperative and postoperative speech testing showed significant benefits, and five patients with available postoperative testing demonstrated speech recognition in the implanted ear when isolated from the better ear. CONCLUSION: CI can safely be performed in younger children with SSD. Patients and families accept early implantation, as evidenced by consistent device use, and derive notable benefits in speech recognition. Candidacy can be broadened to include SSD patients under age five years, particularly individuals without hypoplastic cochlear nerves or developmental delay.

Fine-Needle Aspiration Biopsy of Pediatric Salivary Gland Tumors: Analysis of Patient Tolerability, Sedation Requirement, and Procedural Complication.

OBJECTIVE: Fine-needle aspiration (FNA) biopsy is the standard diagnostic tool recommended by consensus management guidelines for preoperative evaluation of salivary gland tumors in adults. However, its utility in the pediatric population remains debated due to a paucity of data and inherited challenges of pediatric management (patient cooperation, the need for sedation, and procedural complications). METHODS: Consecutive series of 92 FNA biopsies of pediatric salivary gland lesions with available procedural data were included for retrospective analysis. Patient demographics, procedural characteristics, and complications were assessed. RESULTS: Sixty-three patients (68%) tolerated FNA without sedation. Sedation need was significantly associated with younger age, concurrent non-FNA procedure requiring sedation, ultrasound guidance, interventional radiologist as the proceduralist, and radiology suite as the facility setting. The sedation rates for children, and early, middle, and late adolescents were 69%, 32%, 12%, and 10%, respectively, with an optimal cutoff point of ≤12 years for age derived from receiver operating characteristic curve analysis. No significant procedural complications were observed. Sedation did not provide significantly better diagnostic yield. CONCLUSION: FNA biopsy of salivary gland tumors is safe, well tolerated by the pediatric population, and can be effectively performed in an outpatient setting without sedation in most cases. FNA biopsy is a useful tool in the preoperative management of pediatric patients with salivary gland tumors.

Persistent mass after treatment for orbital rhabdomyosarcoma.

PURPOSE: To report a case of orbital rhabdomyosarcoma and highlight the treatment approach to the dilemma of a residual mass. OBSERVATIONS: An eleven-year-old boy was diagnosed with Stage 1, Group III embryonal rhabdomyosarcoma in the orbit. After completing a 24-week treatment regimen of chemotherapy and radiation, imaging showed a large persistent mass with erosion through the medial wall. It was uncertain whether the erosion was due to radiation osteonecrosis or to advancing tumor, creating a treatment dilemma for the providers. A repeat biopsy was planned. During the procedure, the mass was completely excised due to ease of removal, and the biopsy showed completely treated tumor. MRI surveillance at four years follow up showed that the patient remains tumor-free. CONCLUSIONS AND IMPORTANCE: Rhabdomyosarcoma was once a disease with a very poor outcome, but advances in imaging, chemotherapy, and radiation therapy have improved the prognosis of these patients. What was once a surgical disease treated with morbid resection is now predominantly a medical disease diagnosed with biopsy and treated with chemotherapy and radiation. However, such patients may have a residual mass after completing treatment. This situation presents a challenge, as it may not be clear whether the persistent mass is active tumor or treated tumor. This report describes the presentation and management of such a case in the orbit and demonstrates that a residual orbital mass may remain and represent completely treated tumor.

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations.

BACKGROUND: Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing loss and dysphonia. Given these manifestations of CF, otolaryngologists are frequently involved in the care of patients with CF; however, there is limited consensus on optimal management of sinonasal, otologic, and laryngologic symptoms. METHODS: The Cystic Fibrosis Foundation convened a multidisciplinary team of otolaryngologists, pulmonologists, audiologists, pharmacists, a social worker, a nurse coordinator, a respiratory therapist, two adults with CF, and a caregiver of a child with CF to develop consensus recommendations. Workgroups developed draft recommendation statements based on a systematic literature review, and a ≥80% consensus was required for acceptance of each recommendation statement. RESULTS: The committee voted on 25 statements. Eleven statements were adopted recommending a treatment or intervention, while five statements were formulated recommending against a specific treatment or intervention. The committee recommended eight statements as an option for select patients in certain circumstances, and one statement did not reach consensus. CONCLUSION: These multidisciplinary consensus recommendations will help providers navigate decisions related to otolaryngology consultation, medical and surgical management of CF-CRS, hearing, and voice in individuals with CF. A collaborative and multidisciplinary approach is advocated to best care for our patients with CF. Future clinical research is needed utilizing standardized, validated outcomes with comprehensive reporting of patient outcome, effects of modulator therapies, and genetic characteristics to help continue to advance care, decrease morbidity, and improve the quality of life for individuals with CF.

Prevalence, variability, and predictors of sinus surgery in pediatric patients with cystic fibrosis.

INTRODUCTION: Chronic rhinosinusitis is common among individuals with cystic fibrosis (CF) and has an impact on quality of life. Sinus surgery is a treatment option, but minimal literature exists regarding prevalence and indications. METHODS: Using the linked CF Foundation Patient Registry (CFFPR) - Pediatric Health Information Systems (PHIS) database, we investigated variability in receipt of surgery, predictors of surgery, and time to first surgery. We included individuals less than 18 receiving care between 2006 and 2015 at a CF Foundation care program that is also a PHIS-participating-hospital. We used logistic regression to examine predictors of receipt of surgery and a Kaplan-Meier curve to examine time to first surgery among those born 2005-2007. RESULTS: There were 11,545 children and adolescents and 2156 (18.7%) received at least one surgery. Variation in number of surgeries was observed across hospitals (median: 63 [IQR, 33-110]). There was an inconsistent pattern between receipt of surgery and markers of disease severity; those receiving surgery having increased odds of treatment use and pulmonary exacerbations and decreased odds of lower lung function and body mass index. Among the cohort of young children, 159 (14%) had at least one surgery with a median age at first surgery of 5.6 (IQR, 3.9-7.0). CONCLUSIONS: The use of sinus surgery is frequent, but variable, among children and adolescents. Clinical factors are associated with receipt of surgery, but further understanding is needed on other factors that impact variability in use. Our study indicates the need for additional evaluation of the management of CF-related CRS and indications for surgery.

Application of the Milan System for Reporting Pediatric Salivary Gland Cytopathology: Analysis of histologic follow-up, risk of malignancy, and diagnostic accuracy.

BACKGROUND: The diagnosis and management of salivary gland tumors in pediatric patients can be challenging. The utility of fine-needle aspiration (FNA) cytopathology and the performance of the Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) in this age group have not been systematically assessed. The paucity of data has contributed to the controversial role of FNA cytopathology in the presurgical management of these patients. METHODS: The authors retrospectively analyzed 104 pediatric salivary gland FNAs (2000-2020). A correlation with the available histopathologic follow-up (n = 54) was performed. The distribution percentages, the risk of neoplasm (RON), and the risk of malignancy (ROM) were assessed for each category of the MSRSGC. RESULTS: The overall sensitivity, specificity, negative predictive value, and positive predictive value of pediatric salivary gland FNAs were 80%, 97%, and 92%, respectively. The RON values for the nondiagnostic, nonneoplastic, atypia of undetermined significance, benign neoplasm, salivary gland neoplasm of uncertain malignant potential, suspicious for malignancy, and malignant categories were 60%, 11%, 100%, 100%, 100%, 100%, and 100%, respectively, whereas the ROM values were 0%, 11%, 100%, 6%, 67%, 100%, and 100%, respectively. The percentage of nonneoplastic FNAs was greater in comparison with the adult population (52% vs 8%). All neoplasms in patients aged 0 to 10 years were malignant, whereas benign neoplasms occurred only in patients aged ≥11 years; this supported an inverse correlation between age and malignancy rate in salivary gland neoplasms. CONCLUSIONS: FNA cytopathology demonstrates excellent diagnostic performance in differentiating malignant and benign pediatric salivary gland lesions. The MSRSGC is a valuable tool for standardization of the reporting and preoperative risk stratification of these lesions.

Working towards consensus in the management of pediatric chronic rhinosinusitis in cystic fibrosis.

OBJECTIVE: The prevalence of chronic rhinosinusitis (CRS), defined by mucosal thickening on imaging, approaches 100% in the cystic fibrosis (CF) population. CRS is associated with significant morbidity in CF, including its ability to trigger pulmonary exacerbations. CRS in CF is typically managed by pediatricians, otolaryngologists and pulmonologists. This survey evaluates the variance in practice patterns of CRS in CF amongst specialists. METHODS: This is a cross-sectional, electronic survey in which maximum variation purposive sampling was used by a multi-disciplinary group of pediatric, otolaryngology and pulmonology providers in order to select a survey population with expertise in CRS in CF patients. The survey was distributed to 381 practitioners from September to October 2019. RESULTS: 175 participants responded (45% response rate). Ten (of 54) statements achieved 75% consensus agreement. Consensus statements included: The decision to pursue surgical intervention for CRS in CF is a multi-disciplinary approach (94%; n = 146); maximal medical management should include nasal saline irrigation (93%; n = 142), topical steroids (75%; n = 117), maximal medical management should not include intravenous steroids (79%; n = 122); image guidance in surgery is necessary for all surgery involving the frontal sinuses (77%; n = 43), and all revision surgery(80%, n = 45); the appropriate setting for sinus surgery in a CF patient varies depending on patient presentation (89%; n = 133); post-operative regimen should include nasal saline (93%; n = 137); but does depend on the severity of disease discovered intra-operatively (84%; n = 124); post-operative antibiotics should be guided by intra-operative culture data (82%; n = 121). CONCLUSIONS: There is a great deal of variation amongst specialists in the treatment of CRS in CF, however 10 statements met consensus criteria and should be considered when forming clinical care guidelines in this population.

Chronic rhino-sinusitis treatment in children with cystic fibrosis: A cross-sectional survey of pediatric pulmonologists and otolaryngologists.

OBJECTIVES: Children with cystic fibrosis (CF) have a high incidence of chronic rhinosinusitis (CRS); however, no clinical care guidelines currently exist for the management of CRS in these patients. As a result, there is variation in the treatment of CRS in children, especially when it comes to the frequency of surgery for nasal polyposis. METHODS: A 28-question survey was sent to pediatric otolaryngologists (POs) and pulmonologists (PPs) who care for pediatric CF patients. Questions assessed the level of agreement that practitioners had with various approaches to CRS care in pediatric CF patients. RESULTS: Responses from 114 POs and 50 PPs were included in our final analysis. Each group demonstrated significantly different approaches to the medical and surgical management of CRS in pediatric CF patients. POs prefer multi-modal approach while PPs prefer single-modal approaches. With respect to medical management, PPs incline towards IV antibiotics while POs tend toward oral steroids. CONCLUSION: POs and PPs strongly agree that CRS has an impact on overall disease state and quality of life of pediatric CF patients. However, POs and PPs significantly differ in their approach to treating CRS, demonstrating a potential need for clinical care guidelines for the management these common sequelae of CF.

The Role of the Adenoids in Pediatric Chronic Rhinosinusitis.

There are several mechanisms by which the adenoids contribute to pediatric chronic rhinosinusitis (PCRS), particularly with children aged 12 years and younger. Understanding the role that the adenoids play in PCRS is crucial when attempting to treat these patients. A literature review was performed to address this problem and provide information surrounding this topic. This review will provide a better understanding of how adenoids contribute to PCRS, and also of the medical and surgical treatment options.

Update on sinus disease in children with cystic fibrosis: advances in treatment modalities, microbiology, and health-related quality-of-life instruments.

PURPOSE OF REVIEW: There is a lack of consensus with regards to the diagnosis and treatment of sinus disease in children with cystic fibrosis. Here, we review literature from the past 18 months in order to highlight the way forward in this contentious field. RECENT FINDINGS: Most of the literature (from the past 18 months) on sinus disease in pediatric cystic fibrosis focused on treatment approaches, bacteriology and immunology, and health-related quality-of-life (HRQOL) instruments. Quality studies have demonstrated that functional endoscopic sinus surgery (FESS) is as safe in children with or without cystic fibrosis; that the microbiology of the paranasal sinus in children with cystic fibrosis is different than that of their lungs; and, that HRQOL instruments may prove useful in determining sinonasal disease severity in children with cystic fibrosis. SUMMARY: Medical and surgical approaches appear to be viable in the treatment of sinonasal disease in pediatric cystic fibrosis; the microbiology and immunology of pediatric cystic fibrosis is proving more complex and nuanced than initially believed; and, HRQOL instruments show promise in reconciling differences between observable and clinically relevant sinus disease in pediatric cystic fibrosis patients.

Tympanic Membrane Perforation Repair Using Porcine Small Intestinal Submucosal Grafting.

OBJECTIVE: To evaluate the use of porcine small intestinal submucosal grafts for tympanic membrane repair. PATIENTS: Adult and pediatric patients with tympanic membrane perforations with and without chronic otitits media, and perforations after removal of cholesteatoma. INTERVENTION: Endoscopic or microscopic tympanic membrane repair using porcine small intestinal submucosal grafts (Biodesign). MAIN OUTCOME MEASURE(S): Perforation closure, bone and air pure-tone averages (PTA), air-bone gap (ABG), and word recognition scores (WRS) were recorded as outcome measures. RESULTS: Thirty-seven patients were included with a mean age of 25.4 years (range, 6-75), 57% men. Twenty-six cases (70%) were performed endoscopically and 34 (92%) had concomitant cartilage grafting. Three patients (8%) had postoperative pinpoint (<1% surface area) perforation, and two patients (5%) had postoperative perforation, with an overall success rate of 86.5%. The mean improvement in air-bone gap was 7.6 dB and (p = 0.006). There were no statistically significant differences in closure rates when comparing primary versus revision cases, endoscopic versus microscopic cases, size of perforation, cholesteatoma, concomitant mastoidectomy, age, tobacco exposure, or comorbid diabetes mellitus. Patients with concomitant cartilage graft were more likely to be successful when compared with those without cartilage graft (p = 0.04). CONCLUSIONS: Porcine small intestinal submucosal grafts are effective in the repair of the tympanic membrane. These grafts are an excellent choice in total endoscopic cases as it avoids incisions necessary for allograft harvest.

Clinical chronic rhinosinusitis outcomes in pediatric patients with cystic fibrosis.

Objectives: Chronic rhinosinusitis and nasal polyposis are common conditions in cystic fibrosis (CF). Approximately 2-3% of pediatric CF patients per year have sinus disease requiring surgery. It has been well established that there is a significant negative impact on quality of life associated with chronic rhinosinusitis (CRS) in the non-CF patient population. However, the impact of CRS on the pediatric CF population remains uncertain. The purpose of this article is to review the current state of outcome measures for CRS in pediatric CF patients. Data Sources: PubMed and EMBASE literature review. Methods: PubMed and EMBASE electronic databases were searched using Boolean searches that incorporated mesh headings and plain language for quality of life, symptom evaluation, pediatric patients, and sinusitis/rhinosinusitis. Studies were included if the study primarily evaluated a pediatric Cystic Fibrosis-Chronic Rhinosinusitis (CF-CRS) population and the primary outcome measure was quality of life evaluation. Results: The search yielded 34 unique articles. A total of 7 articles met inclusion criteria. Conclusions: Despite the high frequency of chronic rhinosinusitis in the pediatric CF patient population, its impact on quality of life is not well understood. Currently there is a lack of a validated disease specific quality of life instruments available to assess the impact of CRS on the pediatric CF patient population. Level of Evidence: 5.

Evaluating the sinus and Nasal Quality of Life Survey in the pediatric cystic fibrosis patient population.

INTRODUCTION: The Sinus and Nasal Quality of Life Survey (SN-5) is a validated quality of life (QOL) questionnaire for chronic rhinosinusitis in patients age 2-12. Its utility in the cystic fibrosis (CF) has been studied, but not yet validated. The purpose of this study is to determine the effectiveness of the SN-5 for evaluation of sinonasal symptoms in the pediatric CF population. METHODS: This retrospective study analyzed SN-5 surveys completed between 2012 and 2015 by pediatric CF patients and caregivers. Baseline and follow-up overall QOL scores and specific symptom scores were obtained from surveys completed in the three-year span. Non-parametric statistics were conducted to identify differences in survey data. RESULTS: A total of 165 patients completed baseline and follow-up surveys. The overall QOL of the patient cohort did not change over the duration of the study (p = 0.660). Thirty-seven patients indicated higher overall QOL, with all five symptom scores showing significant improvement. Analysis by age group showed that QOL was significantly correlated with all five symptoms for children ages 0-4. In patients 5-12 years, overall QOL was only correlated with sinus infection (r = -0.3090, p = 0.01). QOL was significantly correlated with sinus infection (r = -0.2903, p = 0.04) and allergy symptoms (r = -0.5644, p < 0.01) in patients >12 years of age. CONCLUSION: There remains a need for a validated CRS QOL tool for children with CF. Though the SN-5 has previously been described as a potential instrument, our data suggest that it may be more valuable in children ages 0-4.

Pediatric post-tonsillectomy hemorrhage in the setting of post-transplantation immunosuppression.

INTRODUCTION: Long-term immunosuppressants form an integral part of therapy for post-transplantation patients. Immunosuppressants may also have an anticoagulant effect, and little is known about their effects on bleeding risk after adenotonsillectomy. Our objective was to investigate whether there is an increased observed rate of post-tonsillectomy hemorrhage in a population of pediatric patients on long-term immunosuppressants after solid organ transplantation, compared to healthy controls. METHODS: This was a retrospective chart review of pediatric patients with a history of renal or heart transplant undergoing adenotonsillectomy at our institution between 2000 and 2014. All patients underwent tonsillectomy with monopolar electrocautery. Retrieved data included perioperative medications, occurrence of post-operative bleeding and associated treatment. For comparison, we obtained a population of age-matched controls with no history of immunosuppression who underwent the same procedure. RESULTS: A total of 34 patients meeting criteria were identified, of which 3 (8.82%) suffered a postoperative bleed. Forty-seven controls were obtained, with a total of 2 (4.26%) postoperative hemorrhages (p = 0.65). Two of the post-transplantation patients who bled postoperatively required cauterization in the operating room. None of the controls required surgical treatment. The incidences of postoperative bleeding requiring surgical treatment were 5.88% and 0%, respectively (p = 0.17). CONCLUSION: We failed to demonstrate an increased risk of bleeding after undergoing adenotonsillectomy in our cohort of post-transplantation pediatric patients on chronic immunosuppression. Future research, likely requiring a multi-institutional effort, could stratify by immunosuppressive agent to elucidate bleeding risk with specific medications.

Characteristics of superior orbital subperiosteal abscesses in children.

OBJECTIVES/HYPOTHESIS: Superior pediatric orbital subperiosteal abscesses (SPAs) are less common than medial ones, and clinical features specific to patients with superior SPAs have not been well defined. Clinical characteristics between patients with superior and medial SPAs are compared to determine whether superior location is a risk factor for surgical intervention. STUDY DESIGN: Retrospective cohort study. METHODS: The target population consisted of patients diagnosed with an SPA and seen by the pediatric otolaryngology service at a tertiary children's hospital between January 2010 and October 2014. Imaging characteristics including proptosis, hypoglobus, intraorbital air, and abscess volume as well as treatment interventions were reviewed. RESULTS: Forty patients between 5 and 17 years of age treated for an orbital SPA were identified. Thirteen patients were identified as having superior SPAs; 27 had medial SPAs. The average ages in the two groups were 10.92 and 9.26 years, respectively. The odds ratio for surgical treatment per each increasing year of age was 1.5 (P = .004). The proportion of patients requiring surgery was significantly different between the groups (12/13 superior vs. 13/27 medial, P = .01). The predominant organism group cultured in surgical patients was Streptococcus anginosus (8/24, 29.17%). Superior SPA patients had significantly more proptosis, hypoglobus, and abscess volume on computed tomography scan. CONCLUSIONS: Patients with superior SPAs may present with more advanced disease, leading to a higher rate of characteristics such as proptosis, hypoglobus, and intraorbital air, factors that would predispose to surgical drainage. We found that abscess volume was the most predictive of surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:735-740, 2017.

Surgical Approaches to First Branchial Cleft Anomaly Excision: A Case Series.

Objectives. First branchial cleft anomalies (BCAs) constitute a rare entity with variable clinical presentations and anatomic findings. Given the high rate of recurrence with incomplete excision, identification of the entire tract during surgical treatment is of paramount importance. The objectives of this paper were to present five anatomic variations of first BCAs and describe the presentation, evaluation, and surgical approach to each one. Methods. A retrospective case review and literature review were performed. We describe patient characteristics, presentation, evaluation, and surgical approach of five patients with first BCAs. Results. Age at definitive surgical treatment ranged from 8 months to 7 years. Various clinical presentations were encountered, some of which were atypical for first BCAs. All had preoperative imaging demonstrating the tract. Four surgical approaches required a superficial parotidectomy with identification of the facial nerve, one of which revealed an aberrant facial nerve. In one case the tract was found to travel into the angle of the mandible, terminating as a mandibular cyst. This required en bloc excision that included the lateral cortex of the mandible. Conclusions. First BCAs have variable presentations. Complete surgical excision can be challenging. Therefore, careful preoperative planning and the recognition of atypical variants during surgery are essential.

Inter-hospital variation in the frequency of sinus surgery in children with cystic fibrosis.

BACKGROUND: Chronic rhinosinusitis and nasal polyposis are common conditions in cystic fibrosis (CF). Approximately 2-3% of pediatric CF patients per year have sinus disease requiring surgery. The purpose of this study was to evaluate the variation of sinus surgery rates in pediatric CF patients across multiple US pediatric hospitals. METHODS: The Pediatric Health Information System (PHIS) compiles inpatient administrative data from 42 pediatric hospitals. We conducted a retrospective analysis of PHIS for the period January 1, 2008 to January 1, 2011 to evaluate frequency of sinus surgery at each hospital. We identified CF patients and sinus surgery during inpatient encounters using ICD-9 codes. Demographic data and data for each hospital on hospital size, number of pediatric otolaryngologists, average FEV1, and percentage of patients meeting minimum care guidelines were collected. Twenty-nine hospitals were included in analysis using mixed-effects logistic regression models for occurrence of sinus surgery. RESULTS: We identified 5,194 CF patients, accounting for 18,788 unique encounters among 29 hospitals. 880 patients underwent 1,397 sinus operations. Total number of CF patients at each institution ranged from 39 to 364 and total number of sinus surgeries ranged from 4 to 205, over the 3-year period. Variation in the rate of sinus surgery with hospital encounter was observed (1-24%). Hospital-average lung function (P = 0.56), number of otolaryngologists (P = 0.65) were not found to be predictors of sinus surgery. The size of the CF center (P = 0.01), hospital size (P = 0.05), and age at admission (P ≤ 0.0001) were associated with an increased frequency of sinus surgery. However, with multivariable analysis, only size of the CF center and age of admission remained statistically significant predictors of surgery with admission. CONCLUSIONS: There is large variation in the incidence of sinus surgery for CF in 29 of the largest freestanding pediatric hospitals. This study highlights remarkable variation in clinical practice and underscores the need for further research into the indications and benefits of sinus surgery in pediatric patients with CF. Pediatr Pulmonol. 2015; 50:231-235. © 2014 Wiley Periodicals, Inc.