The presence of a submucous cleft palate in patients with isolated cleft lip and middle ear dysfunction.

PURPOSE: Recent studies have suggested that children with an isolated cleft lip (CL) are more likely to develop middle ear disease and eustachian tube dysfunction (ETD) compared to the general population. This may be related to abnormal palatal musculature or an undiagnosed submucosal cleft palate (SMCP). We aim to determine the prevalence of SMCP in patients with CL who exhibit ETD. MATERIALS AND METHODS: A retrospective chart review was performed for children with an isolated CL requiring tympanostomy tubes over a 20-year period at an academic tertiary care medical center. Demographic, clinical, and surgical data were collected. RESULTS: Three hundred twelve patients had an isolated CL, and 29 (9.3 %) children required tympanostomy tubes. Of those, nine (31 %) were found to have a SMCP (7 males, 6 Caucasian). The average age at CL repair was 3.94 ± 1.03 months, and the average age at tympanostomy tube placement was 13.68 ± 13.8 months. All nine patients had chronic otitis media, with four having mild conductive hearing loss and three having moderate conductive hearing loss. The SMCP was diagnosed at the time of CL diagnosis (4), after CL diagnosis with the diagnosis of chronic otitis media/ETD (2) and after a diagnosis of chronic otitis media/ETD. CONCLUSION: Middle ear disease or eustachian tube dysfunction in a patient with an isolated cleft lip should raise suspicion for an accompanying undiagnosed SMCP.

Otitis Media in Children with Down Syndrome Is Associated with Shifts in the Nasopharyngeal and Middle Ear Microbiotas.

Background: Otitis media (OM) is defined as middle ear (ME) inflammation that is usually due to infection. Globally, OM is a leading cause of hearing loss and is the most frequently diagnosed disease in young children. For OM, pediatric patients with Down syndrome (DS) demonstrate higher incidence rates, greater severity, and poorer outcomes. However, to date, no studies have investigated the bacterial profiles of children with DS and OM. Method: We aimed to determine if there are differences in composition of bacterial profiles or the relative abundance of individual taxa within the ME and nasopharyngeal (NP) microbiotas of pediatric OM patients with DS (n = 11) compared with those without DS (n = 84). We sequenced the 16S rRNA genes and analyzed the sequence data for diversity indices and relative abundance of individual taxa. Results: Individuals with DS demonstrated increased biodiversity in their ME and NP microbiotas. In children with OM, DS was associated with increased biodiversity and higher relative abundance of specific taxa in the ME. Conclusion: Our findings suggest that dysbioses in the NP of DS children contributes to their increased susceptibility to OM compared with controls. These findings suggest that DS influences regulation of the mucosal microbiota and contributes to OM pathology.

Hearing outcomes in children with pneumococcal meningitis in the PCV13 era.

INTRODUCTION: Streptococcus pneumoniae, is associated with the highest incidence of post-meningitic SNHL. The exact impact of 13-valent pneumococcal conjugate vaccine (PCV) on pediatric SNHL from pneumococcal meningitis is unknown. We aimed to identify clinical factors associated with post-meningitic SNHL (pmSNHL) from pneumococcal meningitis and describe its rates based on three time periods: pre-PCV, PCV-7 and PCV13 eras. METHODS: A retrospective case-control study was performed for patients 18 years and younger diagnosed with pneumococcal meningitis from January 1, 2010 to December 31, 2020 at Children's Hospital Colorado. Demographic and clinical risk factors between those with or without SNHL were compared. Detailed hearing outcomes of those with resulting SNHL are described. RESULTS: 23 patients with CSF cultures or Meningitis/Encephalitis Panel positive for pneumococcal meningitis were identified. Twenty patients both survived the infection and had audiologic evaluation. Six patients had pmSNHL, with 50 % affected bilaterally. The rate of pmSNHL from S. pneumoniae in the PCV-13 era at our institution was similar to historical rates from the pre-PCV and PCV-7 eras. Similar proportions of patients with pmSNHL completed PCV vaccination (66.7 %) compared to those without (71.4 %). Non-PCV-13 serotypes were responsible 83 % of patients with pmSNHL versus 57 % of patients without pmSNHL. CONCLUSIONS: Despite high rates of PCV-13 uptake in our cohort, pmSNHL was still common, severe, and commonly associated with non-PCV-13 serotypes. Non-PCV-13 serotypes may be contributing to the persistently high rate of post-meningitic SNHL and the severity of SNHL. Newer pneumococcal conjugate vaccines with expanded serotypes may help mitigate the SNHL associated with pneumococcal meningitis.

Residual OSA in Down syndrome: does body position matter?

STUDY OBJECTIVES: To examine children with Down syndrome with residual obstructive sleep apnea (OSA) to determine if they are more likely to have positional OSA. METHODS: A retrospective chart review of children with Down syndrome who underwent adenotonsillectomy at a single tertiary children's hospital was conducted. Children with Down syndrome who had a postoperative polysomnogram with obstructive apnea-hypopnea index (OAHI) > 1 event/h, following adenotonsillectomy with at least 60 minutes of total sleep time were included. Patients were categorized as mixed sleep (presence of ≥ 30 minutes of both nonsupine and supine sleep), nonsupine sleep, and supine sleep. Positional OSA was defined as an overall OAHI > 1 event/h and a supine OAHI to nonsupine OAHI ratio of ≥ 2. Group differences are tested via Kruskal-Wallis test for continuous variables and Fisher's exact tests for categorical. RESULTS: There were 165 children with Down syndrome who met inclusion criteria, of which 130 individuals had mixed sleep. Patients who predominately slept supine had a greater OAHI than mixed and nonsupine sleep (P = .002). Sixty (46%) of the mixed-sleep individuals had positional OSA, of which 29 (48%) had moderate/severe OSA. Sleeping off their backs converted 14 (48%) of these 29 children from moderate/severe OSA to mild OSA. CONCLUSIONS: Sleep physicians and otolaryngologists should be cognizant that the OAHI may be an underestimate if it does not include supine sleep. Positional therapy is a potential treatment option for children with residual OSA following adenotonsillectomy and warrants further investigation. CITATION: Lackey TG, Tholen K, Pickett K, Friedman N. Residual OSA in Down syndrome: does body position matter? J Clin Sleep Med. 2023;19(1):171-177.

Olfactory testing as COVID-19 screening in school children; A prospective cross-sectional study.

BACKGROUND: Little is known about olfactory changes in pediatric COVID-19. It is possible that children under-report chemosensory changes on questionnaires, similar to reports in adults. Here, we aim to describe COVID-19-related olfactory dysfunction in outpatient children. We hypothesized that children with COVID-19 will demonstrate abnormal olfaction on smell-identification testing at a higher rate than children with negative COVID-19 testing. METHODS: A prospective cross-sectional study was undertaken from June 2020-June 2021 at a tertiary care pediatric hospital. A consecutive sample of 205 outpatients aged 5-21 years undergoing severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) PCR testing were approached for this study. Patients with prior olfactory dysfunction were excluded. Participants were given a standard COVID-19 symptom questionnaire, a Smell Identification Test (SIT) and home-odorant-based testing within 2 weeks of COVID-19 testing. Prior to study enrollment, power calculation estimated 42 patients to determine difference in rates of SIT results between groups. Data were summarized with descriptive statistics. RESULTS: Fifty-one patients underwent smell identification testing (23 positive (45%) and 28 negative (55%) for COVID-19; mean age 12.7 years; 60% female). 92% of all patients denied subjective change in their sense of smell or taste but only 58.8% were normosmic on testing. There was no difference in screening questionnaires or SIT scores between COVID-19 positive and negative groups. CONCLUSIONS: Unlike adults, there was no statistical difference in olfactory function between outpatient COVID-19 positive and negative children. Our findings suggest a discrepancy between objective and patient-reported olfactory function in pediatric patients, and poor performance of current screening protocols at detecting pediatric COVID-19.

Tonsillectomy for Obstructive Sleep-Disordered Breathing: Should They Stay, or Could They Go?

OBJECTIVES/HYPOTHESIS: Children who do not require oxygen beyond 3 hours after surgery and pass a sleep room air challenge (SRAC) are safe for discharge regardless of polysomnogram (PSG) results or comorbidities. STUDY DESIGN: Cross-sectional prospective study. METHODS: All children observed overnight undergoing an adenotonsillectomy for obstructive sleep-disordered breathing were prospectively recruited. Demographic, clinical, and PSG characteristics were stratified by whether the patient had required oxygen beyond 3 hours postoperatively (prolonged oxygen requirement [POR]) and compared using t test, chi-squared test, or Fisher's exact test depending on distribution. Optimal cut points for predicting POR postsurgery were calculated using receiver operating characteristic curves. The primary analysis was performed on the full cohort via logistic regression using POR as the outcome. Significant characteristics were analyzed in a logistic regression model, with significance set at P < .05. RESULTS: A total of 484 participants met the inclusion criteria. The mean age was 5.65 (standard deviation = 4.02) years. Overall, 365 (75%) did not have a POR or any other adverse respiratory event. In multivariable logistic regression, risk factors for POR were an asthma diagnosis (P < .001) and an awake SpO2 <96% (P = .005). The probability of a POR for those without asthma and a SpO2 ≥ 96% was 18% (95% confidence interval: 14-22). Age, obesity, and obstructive apnea/hypopnea index were not associated with POR. CONCLUSIONS: In conclusion, all children in our study who are off oxygen within 3 hours of surgery and passed a SRAC were safe for discharge from a respiratory standpoint regardless of age, obesity status, asthma diagnosis, and obstructive apnea/hypopnea index. Additional investigations are necessary to confirm our findings. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1675-1681, 2022.

Pediatric Primary Tympanoplasty Outcomes With Autologous and Non-autologous Grafts.

OBJECTIVE: To compare rates of successful tympanic membrane (TM) closure in primary pediatric tympanoplasty between various autologous and non-autologous tissues. METHODS: A retrospective chart review was performed examining all primary pediatric tympanoplasties over a 20-year period at a single institution. RESULTS: In 564 pediatric tympanoplasties, no statistically significant difference existed between success rates of autologous and non-autologous grafts (p = 0.083). Compared with fascia, the hazard ratios (and 95% confidence intervals [CI]) for failure for each graft were as follows: human pericardial collagen (HR 0.90, CI 0.54-1.50, p = 0.680), porcine submucosal collagen (HR 1.07, CI 0.56-2.05, p = 0.830), human acellular dermal collagen (HR 1.66, CI 0.95-2.87, p = 0.073), and "multiple grafts" (HR 0.72, CI 0.26-1.98, p = 0.520). Survival curves demonstrated that 75% of graft failures occurred by 6 months after surgery, the rest occurring between 6 and 12 months postoperatively. Larger perforations encompassing more than or equal to 50% of the TM had lower success rates (HR 1.50, CI 1.02-2.21, p = 0.041) than smaller perforations encompassing less than 50% of the TM. Age was not correlated with success (HR 0.98, CI 0.93-1.03, p = 0.390). CONCLUSION: This study found that non-autologous collagen grafts provide equivalent rates of healing when compared with autologous tissue in primary pediatric tympanoplasty. In addition to the potential for reduced operative time and donor site morbidity, these materials provide a viable graft alternative in fascia-depleted ears.Level of Evidence: Level 4.

Tonsillectomy Outcomes for Children With Severe Obesity.

OBJECTIVES/HYPOTHESIS: To determine the success of an adenotonsillectomy (T&A) in treating children with severe obesity utilizing a more accurate obesity scale. STUDY DESIGN: Retrospective cohort. METHODS: A retrospective cohort of children with obesity between 5 and 10 years of age who underwent a T&A at Children's Hospital of Colorado (CHCO) was used. This study also utilized publicly available data from the Childhood Adenotonsillectomy Trial (CHAT) study. The cohort was divided into three obesity classes using age- and sex-specific body mass index (BMI) expressed as a percentage of the 95th percentile (%BMIp95) and compared for operative success differences. RESULTS: There were 132 patients included in our primary analysis, with obesity distribution as follows: Class 1 to 53 patients (40%), Class 2 to 45 patients (34%), and Class 3 to 34 patients (26%). Overall, 52 patients (35.9%) experienced a cure (obstructive apnea/hypopnea index [OAHI] <1), with 27 (52%) patients in Class 1 obesity, 18 (35%) in Class 2, and 7 (13%) in Class 3. Class 3 had a significantly lower obstructive sleep apnea cure rate compared with Class 1 patients (P = .013), but after adjusting for covariates, this difference was no longer present (P > .05). There was no significant difference in the preoperative to postoperative percent change in mean oxygen saturation (P = .82 CHCO, P = .43 CHAT), oxygen nadir (P = .20 CHCO, P = .49 CHAT), or OAHI (P = .12 CHCO, P = .26 CHAT) between the obesity classes. CONCLUSION: After adjusting for covariates, children with Class 3 obesity are as likely to be cured with a T&A as those with Class 1 obesity. A T&A should be considered a first line treatment for all children with obesity. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:461-469, 2022.

Tonsillectomy and hematologic malignancy: Should routine pediatric tonsillectomy specimens be sent to pathology?

OBJECTIVES: Routine pediatric tonsillectomy ± adenoidectomy (T ± A) is one of the most common procedures for children worldwide, accounting for approximately 2000 procedures per year at our institution. To determine the utility of pathologic analysis of routine, nonsuspicious pediatric tonsil specimens, we investigated the incidence of hematologic and lymphoid malignancy diagnosed at the time of or following T ± A. METHODS: Retrospective review of patients 0-18 years undergoing T ± A between 2012 and 2020 with or without pathologic analysis. RESULTS: Included were 14,141 patients who underwent routine T ± A (mean age 11 ± 4.6 years, 48% female). Of these, tonsils of 2464 patients were sent to pathology, where zero were found to harbor malignancy. Seven patients (0.050%) developed malignancy after T ± A. Of these, 4 had unremarkable tonsils per pathology, and 3 did not have tonsils analyzed. There were 5 cases of Acute Lymphocytic Leukemia (ALL, 0.035%), 1 case of Acute Myeloid Leukemia (0.007%), and 1 case of Lymphoma (0.007%). The average length of time from T ± A to diagnosis was 2.4 ± 1.8 years. CONCLUSION: No cases of occult malignancy were identified in specimens from routine T ± A with pathologic analysis, even among patients who later developed malignancy. This study suggests that sending routine pediatric T ± A specimens for formal pathologic analysis is an inefficient use of resources without appreciably improving the quality and safety of patient care.

Pediatric Head and Neck Tumors Associated with Li-Fraumeni Syndrome.

INTRODUCTION: Cancer predisposition syndromes are germline pathogenic variants in genes that greatly raise the risk of developing neoplastic diseases. One of the most well-known is Li-Fraumeni syndrome (LFS), which is due to pathogenic variants in the TP53 gene. Children with LFS have higher risks for multiple malignancies before adulthood, often with rare and aggressive subtypes. OBJECTIVE: To examine head and neck manifestations of LFS in children treated at a tertiary children's hospital over a 20-year period. METHODS: A retrospective review of LFS children with neoplastic disease presenting in traditional Otolaryngologic head and neck subsites from 2000 to 2019, with patient charts reviewed for relevant clinical, imaging, and operative data. RESULTS: Of the 40 LFS patients initially identified, 27 neoplastic tumors were identified in 20 children within this cohort (20 primary, 7 second primary). Head and neck subsites aside from the brain or orbit were involved in 22% (6/27) of these tumors, representing 20% (4/20) of primary tumors and 29% (2/7) of second primary tumors. Both second primaries within the head and neck were within the radiation fields of the first primary tumor. The mean ages at primary and second primary diagnosis were 4.6 years (SD 3.5) and 12 years (SD 1.4), respectively. The male/female ratio was 1:6 among all patients with head and neck tumors. All 6 head and neck tumors were sarcomas. Rhabdomyosarcoma (N = 3, 50%) was the most common pathology, and the other 3 demonstrated rare tumor pathological subtypes (synovial cell sarcoma, pleomorphic myxoid liposarcoma, mandibular osteosarcoma). The neck was the most common subsite (75%) within this group for primary tumor presentation. CONCLUSION: This study identifies a high potential for head and neck involvement in children with LFS, which has not been previously described in the literature. Otolaryngological care should be included in a multidisciplinary care team surveilling these patients.

Parental Perception of Weight Status for Adenotonsillectomy Patients.

OBJECTIVE: Weight status can affect outcomes in pediatric adenotonsillectomy performed for obstructive sleep disordered breathing. Parents frequently underestimate their child's weight and are unaware weight status may affect adenotonsillectomy success. Accurate understanding of a child's weight status is important for shared decision making with the family and perioperative care. The purpose of this study is to analyze the accuracy of the parent's perception of their child's weight status. METHODS: A retrospective analysis was performed of prospective data collected from families of children undergoing adenotonsillectomy from June 2018 through June 2019. RESULTS: A total of 522 children met the inclusion criteria. Two hundred and thirty-two children were either overweight (n = 46, 9%) or obese (n = 186, 36%). Among parents of this cohort whose children were overweight or obese, 74 (32%) erroneously reported that their child was normal weight. For the 290 nonoverweight children, 99% of parents accurately reported weight status. After adjusting for ethnicity, race, BMI%, and sex, for every 1-year increase in age of the child, the odds of the parent correctly identifying their child as overweight increased by a factor of 1.18 (95% CI: 1.09, 1.27). CONCLUSION: One-third of families with children who were overweight or obese undergoing adenotonsillectomy for obstructive sleep disordered breathing underestimated their child's weight. This study highlights the need to facilitate family understanding of weight status' potential impact on both obstructive sleep disordered breathing severity and adenotonsillectomy success, especially for younger children. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2121-2125, 2021.

Persistent OSA in obese children: does body position matter?

STUDY OBJECTIVES: The objective of this study was to determine if positional therapy is a viable treatment alternative for obese children with persistent obstructive sleep apnea (OSA). METHODS: A retrospective review was performed of children who underwent an adenotonsillectomy for OSA from 2014 to 2017. Children were included if they had a body mass index ≥ 95th percentile and underwent a postoperative polysomnogram. Subjects fell into one of three categories: mixed sleep (the presence of ≥ 30 minutes of both nonsupine and supine sleep), nonsupine sleep, and supine sleep. Cure was defined as an OSA/apnea-hypopnea index of < 1 events/h. Paired t tests were used to assess the differences, and a linear model adjusting for obesity class, age at procedure, and sex was performed to assess the differences between nonsupine and supine sleep. RESULTS: There were 154 children who met the inclusion criteria. Using a paired t test, supine sleep position had a significantly higher average OSA/apnea-hypopnea index (7.9 events) compared with nonsupine (OSA/apnea-hypopnea index of 4.1); P value was < .01 for the 60 children with mixed sleep. Forty-three children had predominantly nonsupine sleep and 33 predominantly supine sleep, and a McNemar's test comparing these children showed that those sleeping in the nonsupine position were significantly more likely to be cured than those in the supine position (P < .001). CONCLUSIONS: Sleep physicians and otolaryngologists should be cognizant of positional treatment when consulting with families and note that the postoperative polysomnography may be inaccurate if it does not include supine sleep. Positional therapy as a potential treatment option for obese children with persistent OSA after adenotonsillectomy warrants further investigation.

Three-Dimensional Printed Patient Models for Complex Pediatric Spinal Surgery.

Background: Pediatric spinal deformity surgeries are challenging operations that require considerable expertise and resources. The unique anatomy and rarity of these cases present challenges in surgical training and preparation. We present a case series illustrating how 3-dimensional (3-D) printed models were used in preoperative planning for 3 cases of pediatric spinal deformity surgery. Case Series: Patient 1 was a 6-year-old male with scoliosis secondary to an L3 hemivertebra and severe congenital heart disease who underwent excision of the L3 hemivertebra and L2-L4 spinal fusion. Patient 2 was an 11-year-old male with an L2 hemivertebra and lumbar kyphosis who underwent excision of the L2 hemivertebra and T12-L4 spinal fusion. Patient 3 was a 6-year-old female with Down syndrome who presented with atlantoaxial instability and acute lymphoblastic leukemia. She underwent occipital-cervical spinal fusion and decompression. Prior to surgery, 3-D printed models of the patients' spines were created based on computed tomography (CT) imaging. Conclusion: The anatomic complexity and risk of devastating neurologic consequences in spine surgery call for careful preparations. 3-D models enable more efficient and precise surgical planning compared to the use of 2-dimensional CT/magnetic resonance images. The 3-D models also make it easier to visualize patient anatomy, allowing patients and their families who lack medical training to interpret and understand cross-sectional anatomy, which in our experience, enhanced the consultations.