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To examine children with Down syndrome with residual obstructive sleep apnea (OSA) to determine if they are more likely to have positional OSA.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 OAHI1 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.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 (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.Lackey TG, Tholen K, Pickett K, Friedman N. Residual OSA in Down syndrome: does body position matter?

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 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 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, 2021.

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.

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. CITATION: Tholen K, Meier M, Kloor J, Friedman, N. Persistent OSA in obese children: does body position matter? Clin J Sleep Med. 2021;17(2):227–232.

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.

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.

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.