Surgical management of Rathke cleft cysts in pediatric patients: a single institution experience.

OBJECTIVE: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and optimal management of growing or symptomatic RCCs. We present our institutional experience with the surgical management of RCCs. METHODS: We performed a retrospective study of consecutive RCC patients ≤ 18 years old treated surgically at our institution between 2006 and 2022. RESULTS: Overall, 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 had a histopathological diagnosis of RCC, 58% female and 42% male. The mean age was 13.2 ± 4.2 years. Presenting symptoms included headache (58%), visual changes (32%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and subsequently demonstrated growth on serial imaging. Six percent presented with symptomatic intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients and preoperative visual deficits improvement in 55% after surgery. New pituitary axis deficits were seen in 9.7% of patients. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging at a mean follow-up was 62.9 ± 58.4 months. Recurrence requiring reoperation occurred in 32% of patients. Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSION: Due to their secretory epithelium, pediatric RCCs may demonstrate rapid growth and can cause symptoms due to local mass effect. Surgical management of symptomatic or growing pediatric RCCs via cyst fenestration or partial resection of the cyst wall can be performed safely, with good neurologic outcomes. There is a nontrivial risk of endocrinologic injury, and long-term follow up is needed due to high recurrence rates.

Hospital Admissions in Pediatric Patients With Tracheostomies Based on Rurality and Insurance Status.

OBJECTIVE: Determine whether rurality or public insurance status is associated with greater 30-day readmission after tracheostomy in pediatric patients. STUDY DESIGN: Retrospective cohort. SETTING: Pediatric Health Information System (PHIS) Database. METHODS: Patients within PHIS who underwent tracheostomy from 2013 to 2017 were included. Rural status was defined by rural-urban commuting area codes. Insurance status was based on the primary payer. All-cause 30-day readmissions and tracheostomy-related readmissions were recorded. Multivariate logistic regression was performed to test for differences in readmissions between cohorts. RESULTS: Among patients, 1092 were rural, and 4329 were publicly insured, with no significant association between rurality and insurance. Compared to nonrural patients, rural patients were more frequently white, less frequently ventilator dependent, and more likely discharged home rather than to a care facility. Publicly insured patients were more frequently non-white. Twenty-eight percent of patients were readmitted within 30 days of discharge. Odds of 30-day readmission were lower in rural patients (odds ratio [OR]: 0.80, 95% confidence interval [CI]: 0.68-0.95, p = .01) but higher in publicly insured (OR: 1.24, 95% CI: 1.09-1.42, p = .001) controlling for age at tracheostomy, sex, race, and ventilator dependence. The odds of tracheostomy-related admission did not differ by rurality but were higher in publicly insured children (1.39, 95% CI: 1.03-1.88, p = .03). CONCLUSION: Readmission within 30 days following tracheostomy was more likely in publicly insured patients and less likely in rural patients. These findings help identify at-risk patients when considering discharge planning and follow-up. More work is needed to understand long-term tracheostomy outcomes in these groups.

Effect of Local Anesthetic Injections on Subjective Pain Scales in Pediatric Tonsillectomies: A Meta-analysis.

OBJECTIVE: To assess the effect of local anesthetic injection on subjective pain scores for pediatric tonsillectomies on postoperative days (PODs) 0 and 1. DATA SOURCES: A comprehensive literature search was conducted with the PubMed, Embase, Web of Science, and Scopus databases. REVIEW METHODS: A 2-researcher team following the PRISMA guidelines performed a systematic review and meta-analysis. Studies with original data and a saline injection control arm were selected. Corresponding authors were contacted for missing data. The collated data were analyzed with RevMan version 5.4 (Review Manager; Cochrane Collaboration). Random effects modeling and standard mean difference were utilized to control for heterogeneity. Surgical technique subgroup analysis was performed. RESULTS: A total of 319 publications were identified, and 8 articles with 13 experimental arms (627 participants) were selected for meta-analysis. Local anesthetic injection was compared with placebo with saline injection. The standard mean difference for POD 0 pain was -0.81 (95% CI, -1.16 to -0.46; P < .00001) in favor of local anesthetic. The standard mean difference for POD 1 was -1.13 (95% CI, -1.48 to -0.78; P < .00001) in favor of local anesthetic. Subgroup analysis by surgical technique showed a less robust effect for cautery excision (-1.01 [95% CI, -1.37 to -0.66]) vs cold excisional technique (-1.19 [95% CI, -1.64 to -0.74]), with or without cautery-based hemostasis. CONCLUSIONS: A local anesthetic injection during a pediatric tonsillectomy reduces postoperative pain on POD 0 and 1. Further analysis on total narcotic use and postoperative complications would benefit surgeon decision making.

Predictors of overnight postoperative respiratory complications in obese children undergoing adenotonsillectomy for obstructive sleep apnea.

OBJECTIVES: Current clinical guidelines from the American Academy of Otolaryngology Head & Neck Surgery (AAO-HNS) recommend a preoperative polysomnogram (PSG) for obese patients prior to adenotonsillectomy (T&A). An overnight admission is recommended for children with severe (AHI >10) obstructive sleep apnea (OSA), citing a higher incidence of post-operative respiratory complications (PRCs) and need for respiratory support. Routine admission of obese children based on AHI >10 alone after T&A may place undue strain on hospital resources and increase healthcare costs, especially considering that many of these children have uncomplicated postoperative courses. In this study, we sought to identify variables from the pre-operative PSG and post-anesthesia care unit (PACU) that could more accurately predict overnight PRCs and indicate the need for a post-surgical admission after T&A. METHODS: A single-center retrospective chart review was performed on a cohort of 155 obese children who underwent adenotonsillectomy for OSA. Inclusion criteria included patients 17 years of age and younger who had BMI 95th percentile or greater, underwent preoperative polysomnography, and were admitted overnight after T&A. Overnight respiratory complications were defined as an O2 desaturation under 92%, the need for overnight airway support, a respiratory support regression, respiratory depression, and bronchospasm/laryngospasm. Multivariable binary logistic regression analysis, point-biserial correlation, and Chi-square tests were performed to assess relationship of BMI z-score, polysomnography parameters, and PACU events with overnight respiratory complications. RESULTS: Lower O2 saturation nadirs on polysomnography were an independent predictor of respiratory complications overnight (OR = 0.953, 95% CI = 0.91-0.99, P = 0.021), as was sleep time with O2 saturation less than 90% (OR = 1.04, 95% CI = 1.00-1.07, P = 0.048). A prediction model with preoperative and postoperative variables significant on simple logistic regression yielded a ROC curve with AUC 0.89 (95% CI 0.82, 0.96). At a cutoff point of O2 saturation nadir less than 80%, overnight PRCs were predicted with 70.8% sensitivity and 75.2% specificity. At a cutoff point of greater than 0.5% of sleep time spent with O2 < 90% on PSG, overnight PRCs were predicted with 82.6% sensitivity and 62% specificity. Obstructive apneas (OAI) was not predictive of PRCs. BMI percentile was not significantly correlated with overnight respiratory complications, but BMI z-score was significantly correlated with overnight respiratory depression and an overnight airway event. CONCLUSIONS: O2 saturation nadir on PSG and time spent with oxygen saturation <90% (TST90) on PSG were found to be independent predictors of overnight postoperative respiratory complications after adenotonsillectomy in obese children. In addition to reaffirming existing guidelines for postoperative admission of patients with O2 saturation nadir on PSG <80%, these findings also suggest considering postoperative admission for obese patients who experience >0.5% sleep time with O2 sat <90% during PSG due to increased risk of overnight postoperative respiratory complications.

Completion of postoperative polysomnography for children with severe obstructive sleep apnea: A quality improvement project.

Objective: Pediatric patients with severe obstructive sleep apnea (OSA) are at risk for residual OSA following tonsillectomy with/without adenoidectomy (T ± A). We initiated a quality improvement (QI) project to increase the percentage of postoperative (postop) polysomnography (PSG) completion to identify residual OSA. Methods: This is a prospective QI project carried out at a tertiary pediatric academic hospital. Children ≤18 years of age who underwent T ± A for severe OSA were included. Our Specific, Measurable, Attainable, Relevant, and Time-based (SMART) aim was to increase the percentage of completed postop PSGs in this cohort from a baseline of 70% to95% by May 31, 2021. We focused on patient education and leveraged both clinical decision support and reporting functionalities of the electronic medical record for project implementation. Results: During the pre-intervention period between January 1, 2019 to June 30, 2020, 472 patients met the inclusion criteria with an average age of 8.6 years (SD 4.6). The rate of postop PSG completion was 69.7% (SD 11.4%) with an average time of 99 days (SD 66) between surgery and the postop PSG. A shift was observed starting in September 2020, and the PSG completion rate improved to 94.9% by September 30, 2021. Post-intervention, there were 178 patients with an average age of 9.3 years (SD 4.9). The average time between surgery and the postop PSG was significantly reduced to 57 days (SD 16; p < .001). Conclusions: Through a multidisciplinary approach, we successfully completed our SMART aim. With the establishment of QI infrastructure, our goal is to deliver better care in a sustainable fashion using QI methodology.

Pediatric basilar skull fractures from multi-level falls: A systematic review and retrospective analysis.

OBJECTIVE: Multi-level fall (MLF) accounts for 26.5%-37.7% of traumatic pediatric basilar skull fractures (BSFs). There is a dearth of information concerning recommendations for work-up, diagnosis, treatment, and otolaryngological follow-up of pediatric basilar skull fractures secondary to MLFs. Through a systematic literature review and retrospective review of an institution's trauma experience, we sought to identify clinical findings among pediatric MLF patients that indicate the need for otolaryngological follow-up. METHODS: A two-researcher team following the PRISMA guidelines performed a systematic literature review. PubMed, Web of Science, and EBSCO databases were searched August 16th, 2020 and again on November 20th, 2021 for English language articles published after 1980 using search terms Pediatric AND (fall OR "multi level fall" OR "fall from height") AND ("basilar fracture" OR "basilar skull fracture" OR "skull base fracture" OR "skull fracture"). Simultaneously, an institutional trauma database and retrospective chart review was performed for all patients under age 18 who presented with a MLF to a pediatric tertiary care center between 2007 and 2018. RESULTS: 168 publications were identified and 13 articles reporting pediatric basilar skull fracture data and MLF as a mechanism of injury were selected for review. MLF is the most common etiology of BSF, accounting for 26.5-37.7% of pediatric BSFs. In the retrospective review, there were 180 cases of BSF from MLF in the study period (4.2%). BSF and fall height were significantly associated (p < 0.001), as well as presence of a CSF leak and fall height (p = 0.02), intracranial hemorrhage (ICH) (p = 0.047), and BSF fracture type (p < 0.001). However, when stratified by age, these associations were only present in the younger group. Of those with non-temporal bone BSFs (n = 71), children with hemotympanum (n = 7) were approximately 18 times more likely (RR 18.3, 95% CI 1.89 to 177.02) than children without hemotympanum (n = 64) to have hearing loss at presentation (28.6% vs. 1.6% of patients). CONCLUSIONS: MLF is the most common cause of pediatric basilar skull fractures. However, there is limited information on the appropriate work-up or otolaryngologic follow-up for this mechanism of injury. Our retrospective review suggests fall height is predictive for BSF, ICH, and CSF leak in younger children. Also, children with non-temporal bone BSFs and hemotympanum may represent a significant population requiring otolaryngology follow-up.

Maturation of the sella turcica and parasellar region: Surgical relevance for anterior skull base approaches in pediatric patients.

OBJECTIVE: Traditional and extended transnasal transsphenoidal approaches provide direct access to a variety of anterior skull base pathologies. Despite increased utilization of transnasal approaches in children, anatomic studies on pediatric skull base maturation are limited. We herein perform a surgically relevant morphometric analysis of the sella and parasellar regions during pediatric maturation. METHODS: Measurements of sellar length (SL), sellar depth (SDp), sellar diameter (SDm), interclinoid distance (ID), intercavernous distance (ICD), and the presence of sphenoid sinus pneumatization (SSP), and sphenoid sinus type (SST) were made on thin-cut CT scans from 60 patients (evenly grouped by ages 0-3, 4-7, 8-11 12-15, 16-18, and >18 years) for analysis. Data were analyzed by sex and age groups using t-tests and linear regression. RESULTS: Sella and parasellar parameters did not differ by sex. SL steadily increased from 8.5 ± 1.2 mm to 11.5 ± 1.6 mm throughout development. SDp and SDm increased from 6.0 ± 0.9 mm to 9.3 ± 1.4 mm and 9.0 ± 1.6 mm to 14.4 ± 1.8 mm during maturation, with significant interval growth from ages 16-18 to adult (p < 0.01). ID displayed significant growth from ages 0-3 to 4-7 (18.0 ± 2.4 mm to 20.7 ± 1.9 mm; p = 0.002) and ICD from ages 0-3 to 8-11 (12.0 ± 1.8 mm to 13.5 ± 2.1 mm; p < 0.001), without further significant interval growth. SSP was not seen in patients < 3, but was 100% by ages 8-11. SSTs progressed from conchal/presellar (60% at ages 4-7) to sellar/postsellar (80% at adulthood). CONCLUSION: The sella and parasellar regions have varied growth patterns with development. Knowledge of the expected maturation of key anterior skull base structures may augment surgical planning in younger patients.

Anesthetic injections and analgesia use in pediatric post-tonsillectomy patients: A meta-analysis and systematic review.

OBJECTIVE: To assess the effect of local anesthetic injection on post-operative analgesia and complications for pediatric tonsillectomies on post-operative day (POD) 0 and 1. METHODS: A 2-researcher team following the PRISMA guidelines performed a systematic review and meta-analysis. The databases Pubmed.gov, Embase, Web of Science, and Scopus were used. Studies written with original data utilizing a saline injection control arm were selected. Corresponding authors were contacted for missing data, which was used when possible. The collated data was analyzed with Review Manager (RevMan) Version 5.4., The Cochrane Collaboration, 2020. Surgical technique subgroup analysis was performed. RESULTS: 319 publications were identified and 8 articles with 12 total experimental arms (529 participants) were selected. Local anesthetic injection was compared to saline injection. The standard mean difference for time until first analgesic was 0.68 min [0.26, 1.09](P = 0.001) in favor of local anesthetic. The odds ratio (OR) for patients receiving one or more narcotic doses on POD 0 was 0.22 [0.06, 0.84](P = 0.03) in favor of local anesthetic. CONCLUSIONS: Use of an intraoperative anesthetic injection during pediatric tonsillectomy can help improve initial recovery as it delays the need for narcotic medication and may reduce postoperative narcotic usage on POD 0 and 1. In addition, a local anesthetic does not increase the risk of postoperative bleeding or dehydration.

Management and outcome of extreme pediatric obstructive sleep apnea.

OBJECTIVES: Classify post-adenotonsillectomy (AT) respiratory support, identify variables that predict these interventions, and evaluate outcomes in children with extreme obstructive sleep apnea (OSA). METHODS: Retrospective chart analysis was performed on patients found to have apnea/hypopnea index (AHI) > 100 events/h. Patients with chronic diseases other than obesity were excluded. RESULTS: Forty-one subjects were studied, average age of 11.4 ± 4.3 years, majority (73.1%) were Hispanic, with a mean total AHI (TAHI) of 128.1 ± 22.9/h. Twenty-eight (68.3%) patients underwent AT. Lower age (P < 0.001), lower BMI Z-score (P < 0.01), higher OAHI (P < 0.05) were associated with having surgery. Eleven out of 28 (39.3%) surgical patients required respiratory support (oxygen or positive airway pressure) postoperatively. Longer % total sleep time SpO2 <90% during PSG (P < 0.05) and lower SpO2 nadir (P < 0.05) were associated with requiring airway support. No patients experienced mortality, reintubation, or hospital readmission following AT, with majority (71.4%) discharged 1 day post-operatively. Eleven (57.9%) of the 19 patients who had a postoperative PSG had residual OSA, defined as AHI >5 events/h, but there was a significant improvement in TAHI (P < 0.01). CONCLUSION: Our findings confirm the need for postoperative observation in a controlled setting for patients with extreme OSA undergoing AT. Although at higher risk of needing respiratory support, those patients undergoing AT for extreme OSA did not require re-intubation post-operatively or suffer serious harm. Barring contraindications to AT, surgery may still be a first-line therapy for some children with extreme OSA.

Influence of Pediatric Endoscopic Endonasal Skull Base Resections on Midface and Skull Base Development.

OBJECTIVE: The purpose of this study is to use imaging data to determine if endoscopic endonasal surgery (EES) for skull base tumor resection interrupts skull base growth and development, resulting in an atrophic midface skeletal structure, compared to matched normal controls. METHODS: Data were collected by a retrospective chart review done on children aged 16 years and below who underwent endoscopic tumor resection and had pre- and postoperative magnetic resonance imaging with relevant midface anatomy. 121 normal controls were matched to 20 EES patients by age and gender. Three measurements related to midface anatomy were taken from 1 sagittal T1 slice and 1 axial T2 slice of each scan. Statistical analysis was used to compare growth measures between cases and controls. RESULTS: Twenty patients who underwent EES between November 2015 and April 2018 met our inclusion criteria. The mean age of the patients, 11 males and 9 females, was 10 years, and 8 patients (38%) were aged 7 years or younger. Six patients who had a high-flow CSF leak obtained a nasoseptal flap. A student T test and multivariate regression analysis found that EES did not affect midface and skull base growth. Among the variables assessed, age appears to be the only driver of growth. CONCLUSION: There were no identified differences in craniofacial growth in pediatric patients undergoing EES for skull base tumor resection as compared to the control group. EES does not appear to significantly interfere with midface/skull base development and is a good surgical option for pediatric patients.

The accuracy of a portable sleep monitor to diagnose obstructive sleep apnea in adolescent patients.

STUDY OBJECTIVES: The efficacy of portable-monitor (PM) sleep testing in children is not well understood. While most studies have evaluated PM in a lab setting, the utility of PM in the home environment is relatively unknown. We sought to determine whether home PM accurately diagnoses obstructive sleep apnea in adolescents and to assess patient satisfaction with home PM sleep testing. METHODS: We evaluated adolescents (age 12-18 years) with suspected obstructive sleep apnea using a PM device. In addition to in-laboratory polysomnography (PSG), all participants had PM testing performed twice, once in their home and once concurrent to in-laboratory PSG. PM was compared to PSG using 2 primary outcomes: the apnea-hypopnea index and oxygen desaturation index. All participants were approached for interview to evaluate their experience with PM sleep testing. RESULTS: Twenty adolescents participated. Bland-Altman analysis comparing the apnea-hypopnea index and oxygen desaturation index determined by home or in-laboratory PM to in-laboratory PSG revealed mostly agreement; however, some deviations were observed when either parameter was markedly increased. While PM testing tended to underestimate the apnea-hypopnea index, the diagnostic agreement between home PM and PSG was 80% (by the White-Westbrook method). Most preferred PM to PSG and found PM easy to very easy to set up. CONCLUSIONS: In a small cohort of adolescents, our study supports the application of home PM in the diagnosis of suspected obstructive sleep apnea. Until studies implementing PM using larger cohorts become readily available, the findings from this preliminary study could contribute to adolescents receiving sleep apnea therapy more promptly. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Identifier: NCT03748771. At the time of issue publication, this registration is not publicly available because the trial includes a device that is not approved or cleared for use in pediatric populations. Once the device is FDA cleared, the registration will become public.

Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria. Here, we categorize these patients as syndrome of undifferentiated recurrent fever (SURF), and identify the unique features which distinguish them from the PFAPA syndrome. SURF patients were more likely to report gastrointestinal symptoms of nausea, vomiting and abdominal pain, and experienced inconsistent responses to on-demand steroid therapy compared to PFAPA patients. For this previously undefined cohort, an optimal course of therapy remains uncertain, with medical and surgical therapies largely driven by parental preference. A subset of patients with SURF underwent tonsillectomy with complete resolution. Flow cytometric evaluation demonstrates leukocytic populations distinct from PFAPA patients, with reduced CD3+ T cell numbers. SURF patient tonsils were predominantly characterized by an IL-1 signature compared to PFAPA, even during the afebrile period. Peripheral blood signatures were similar between groups suggesting that PFAPA and SURF patient tonsils have localized, persistent inflammation, without clinical symptoms. These data suggest that SURF is a heterogenous syndrome on the autoinflammatory disease spectrum.

Pediatric Nasal Chondromesenchymal Tumors: Case Report and Review of the Literature.

INTRODUCTION: Nasal chondromesenchymal tumors (NCMT) are rare benign neoplasms that usually present in children <1 year of age. They can display rapid growth and significant local bony remodeling that can mimic a malignant process. Of the ∼50 published cases to date, few have documented the need for neurosurgical intervention. We herein report a NCMT in an infant treated with a staged cranial and transnasal approach, as well as summarize the available literature on this pathology. CASE REPORT: A newborn male with a compromised airway was noted to have a large sinonasal lesion. After stabilization, MRI demonstrated a 4-cm enhancing mass with diffuse sinus involvement and significant extension into the anterior cranial fossa, with displacement of the optic apparatus and hypothalamic pituitary axis. After an initial biopsy, the patient underwent a bifrontal craniofacial approach at 2 months of age, followed by a second-stage transnasal endoscopic approach at 15 months which resulted in a complete resection. There were no neurosurgical complications. Pathology was consistent with a NCMT. DISCUSSION: Although rare, neurosurgical involvement is critical for the treatment of NCMTs with intracranial extension. Staged cranial and endonasal endoscopic approaches may be needed for complete resection of such lesions.

Pediatric Basilar Skull Fracture Mechanisms and Trends From 2007 to 2018.

OBJECTIVES: To evaluate the specific mechanism and trend of injury resulting in pediatric basilar skull fractures. STUDY DESIGN: Retrospective chart review of a trauma database. SETTING: Tertiary care children's hospital. METHODS: Patients ≤18 years old with basilar skull fractures were identified via the trauma database for admissions from 2007 to 2018. Patients were identified with ICD-9 codes (801.0, 801.1, 801.2, 801.3 or 801.4) and ICD-10 codes (S02.1, S02.10, S02.11, S02.19) for skull base fractures (International Classification of Diseases, Ninth Revision and Tenth Revision). RESULTS: A total of 729 patients were included: 251 females and 478 males. The 2 most common mechanisms of injury are multilevel falls and unhelmeted rider falls. Multilevel falls occur more in the toddler age group (average age, 4 years), and unhelmeted rider falls are seen in the older age group (average age, 11.2 years). Helmeted rider and motor vehicle accident basilar skull injuries are relatively uncommon. There was a spike in television/entertainment center mechanisms of injury in toddlers from 2007 to 2011 but has since decreased. CONCLUSION: Pediatric basilar skull fractures are costly to the health care system, as patients spent more time in intensive care unit beds with a charge limited to hospital rooms between $1.7 and $2.7 million per year. The protective effect of helmets is demonstrated by unhelmeted rider injuries being the second-most common mechanism of basilar skull fractures and by helmeted rider injuries being rare. The small proportion of basilar skull fractures from motor vehicle accidents is an example of policy and behavioral changes resulting in decreased injury.

Multidisciplinary clinic for care of children with complex obstructive sleep apnea.

OBJECTIVE: While adenotonsillectomy (AT) remains first line therapy for pediatric obstructive sleep apnea (OSA), management of children who are not candidates for AT or who have residual OSA post AT varies and spans across multiple specialties. We aim to report our experience in managing this population through a multidisciplinary sleep clinic composed of specialists in pediatric dentistry, otolaryngology, plastic surgery, and pulmonary/sleep medicine. STUDY DESIGN: Retrospective chart review. METHOD: The medical records of children attending our complex sleep apnea clinic were reviewed. Data pertaining to demographics, underlying diagnoses, prior evaluation and treatment, recommendations, and initial therapy were collected. RESULT: Two-hundred and thirty patients (mean age 10.7 ± 5.1 years, 62.2% male) were assessed. Underlying conditions included Trisomy 21 (n = 65, 28.2%), other genetic syndromes (n = 37, 16.1%), obesity in an otherwise typically developing child (n = 36, 15.2%), cerebral palsy (n = 27, 11.7%), and craniofacial syndromes (n = 7, 3.0%). Mean obstructive apnea hypopnea index (OAHI) was 14.2 events/hour at first clinic visit, and the majority of children had previously undergone at least one upper airway surgery (n = 168, 73.0%), primarily adenotonsillectomy. Recommended initial treatment plans included positive airway pressure (PAP) therapy (n = 108, 47.0%), surgery (n = 75, 32.6%), allergy management (n = 52, 22.6%), and/or weight loss (n = 34, 14.8%). Patients prescribed PAP therapy with follow up data were found to be adherent 43.9% of the time. Surgical patients with post-operative polysomnography had pre-operative OAHI 15.6 ± SD13.4 decrease to 10.7 ± 14.2 events/hour (p = 0.61). CONCLUSION: Genetic conditions and obesity were the most common underlying diagnoses cared for in the complex sleep apnea clinic. Patients presented with severe OSA, most having already had upper airway surgery. Management plans were frequently adjusted, and we observed improvement in SDB in a sub-segment of patients, suggesting benefit to a coordinated, multi-disciplinary approach.

Incidence of cure and residual obstructive sleep apnea in obese children after tonsillectomy and adenoidectomy stratified by age group.

OBJECTIVES: Current clinical guidelines by the American Academy of Pediatrics recommends adenotonsillectomy (T&A) as the first-line treatment for pediatric OSA. However, obese children experience a decreased incidence of cure from T&A compared to non-obese children, with obesity increasing risk of residual post-operative OSA by up to 3.7-fold. In addition to obesity, increased age has also been linked to more severe baseline OSA, among other factors. In this study, we examined how age effects the post-operative outcome in obese children with OSA. METHODS: A retrospective chart review was performed to assess post-operative T&A polysomnography outcomes of obese children. Inclusion criteria included patients who were 17 years old and younger, underwent T&A, were obese and had both pre- and post-operative sleep studies. The patients were split into 3 different groups based on their age: Group 1 (0-6 years old), Group 2 (7-11 years old), and Group 3 (12-17 years old). RESULTS: 55 patients were included in the study: 13 in Group 1, 20 in Group 2, and 22 in Group 3. For Groups 1, 2, and 3 respectively, data averages were BMI percentile 99.20, 98.49, and 98.92 (P = 2.77); z-score 2.79, 2.36, and 2.45 (P = 0.026), tonsil size 3.17, 3.15, and 3.23 (P = 0.898), adenoid size 2.42, 2.05, and 1.77 (P = 0.015), time between the preoperative and postoperative PSG 179, 240, and 202 days (P = 0.481), and time from surgery to postoperative PSG 126, 170, and 127 days (P = 0.544). The average preoperative oAHI was 52.56, 41.23, and 43.49 (P = 0.732), post-operative oAHI was 1.94, 4.79, and 4.44 (P=.417); and change in oAHI was 50.62, 36.44, and 39.25 (P = 0.617). When comparing the age group of 0-6-year-olds to the older remaining patients, the post-operative oAHI was the only variable to show a significant difference between the two-groups with a P value of 0.038. The percentage of patients with post-operative resolution of OSA (oAHI<2), mild, moderate, and severe OSA, respectively, were 53%, 29%, 9%, and 9% for all patients, 70%, 23.1%, 7%, and 0% for group 1; 50%, 35%, 5%, and 10% for group 2; and 45%, 27%, 13%, and 13% for group 3. The percent of the patients requiring post-surgical nighttime airway support were 18%, 7%, 15%, and 26% for Groups All, 1, 2, and 3, respectively. CONCLUSION: We found that despite having the highest rates of obesity and the most severe OSA, obese patients under 7 years old performed better following T&A, with greater cure rate, overall reduction of oAHI, and decreased need for post-surgical nighttime airway support.

Sleep disordered breathing in children with trisomy 13 and trisomy 18.

PURPOSE: While the prevalence of obstructive sleep apnea (OSA) is well documented in trisomy 21, there has been little published about the incidence in trisomy 13 (T13) and trisomy 18 (T18). Trisomies 13, 18, and 21 have overlapping clinical features that make patients prone to OSA. Because the literature regarding OSA in T13 and T18 children is limited, we performed a retrospective chart review to investigate the characteristics of these patients. METHODS: We reviewed the medical records of children with T13 or T18 seen at seen at a single urban tertiary children's hospital for sleep disordered breathing from 1/1/10 to 5/1/18. Candidates were selected based on ICD-9 diagnosis and procedural codes. RESULTS: We identified 21 T18 patients that had documented symptoms of SDB, of which 3 were diagnosed with OSA, 11 had clinical SDB, and 7 had snoring. Of the T13 patients, 10 had documented symptoms of SDB, of which 1 patient was diagnosed with OSA, 7 with clinical SDB, and 2 with snoring. In both T13 and T18 patients, anatomical features included micrognathia/mandibular hypoplasia, small mouth/small airway, midface hypoplasia, abnormal/difficult airway, glossoptosis, hypotonia, and GERD. Endoscopic findings included laryngomalacia and/or tracheomalacia, adenoid and lingual tonsil hypertrophy, and inferior turbinate hypertrophy. Surgical interventions performed in T13 and T18 patients included adenoidectomy, lingual tonsillectomy, and tracheostomy. Of the 32 T13 and T18 patients, 15 had to be intubated for respiratory insufficiency. CONCLUSION: The results of our study suggest that T13 and T18 patients are at increased risk for OSA due to common features found in this population. These findings indicate a need for otolaryngologist intervention to increase both survival and quality of life in this population.

Approaching the Sella through the Nonpneumatized Sphenoid in Pediatric Patients.

Objective The purpose of this study is to specifically assess pediatric patients with nonpneumatized sphenoid sinuses who have undergone transsphenoidal resections of skull base tumors and assess the complications and outcomes. Methods Data was collected by a retrospective chart review done on children ages 7 and under who underwent endoscopic tumor resection and had a partially or completely nonpneumatized sphenoid sinus on preoperative computed tomography imaging. Surgical data collected included surgical corridor, gross total versus subtotal resection, repair method, use of septal flap, intraoperative and postoperative cerebrospinal fluid leak, and estimated blood loss. Results Six patients were identified that fit our inclusion criteria who underwent surgery between November 2015 and April 2018 (3 males, 3 females; average age = 4.28 years). Tumor pathologies include three craniopharyngiomas, Rathke cleft cyst, meningocele, and neuroblastoma. All cases involved varying percentages of sphenoid sinus pneumatization. All cases except one craniopharyngioma and the neuroblastoma required removal of cancellous bone to access pathology. Degree of sphenoid pneumatization tended to be greater with age and resulted in less intraoperative bleeding. The two youngest patients with completely nonpneumatized sphenoid sinuses lost 61.73 and 17.52% of their total blood volume intraoperatively. Procedures were able to be adequately performed and pathology completely addressed with minimal postoperative complications and no postoperative CSF leaks. Conclusions Intraoperative challenges including hemorrhage are likely in nonpneumatized patients, and thus surgeons should be prepared with adequate vascular access and blood products. Hemorrhage and total blood volume loss are increased in the younger patients with no sphenoid pneumatization. Despite the additional challenges, a nonpneumatized sphenoid sinus is not a contraindication for an endoscopic resection in the pediatric population.