Secondary Postoperative Hemorrhage in the Pediatric Tonsillectomy Patient- is there a correlation between hemorrhage and tonsilloliths?

INTRODUCTION: Tonsillectomy with or without adenoidectomy is one of the most common ambulatory procedures performed in children under 15. One rare yet serious complication of tonsillectomy is postoperative hemorrhage. Chronic tonsillitis, which is an indication for tonsillectomy, has been shown to have an increased risk for postoperative hemorrhage. Tonsilloliths or tonsil stones have been associated with cryptic tonsillitis. This 2020-2021 study examined whether tonsilloliths were a risk factor for post-tonsillectomy hemorrhage in a convenience sample of 187 pediatric patients. METHODS: This was a cross-institutional 12-month retrospective cohort study investigating pediatric patients who had undergone tonsillectomy. Exclusion criteria included patients who had received prior airway surgeries (e.g., supraglottoplasty), patients with significant comorbidities such as chromosomal abnormalities or congenital disorders, and patients with pre-existing bleeding disorders. Demographic, clinical, and operative data was extracted from each chart. Postoperative adverse events and bleeding were also recorded. These factors were then compared between the tonsillolith and no tonsillolith patient groups. RESULTS: A total of 187 pediatric patients met the inclusion criteria. Seventy-three (39%) of the patients had tonsilloliths and 114 (61%) did not have tonsilloliths at the time of surgery. The tonsillolith subgroup had a higher median age (10 vs 3, P < 0.001) when compared to the no tonsillolith subgroup. The most common indication for tonsillectomy was obstructive sleep apnea/sleep disordered breathing (N= 148, 79.1%). There was no statistical difference found between presence of tonsillolith and indication for surgery (P = 0.06). Only five (2.7%) of sample patients experienced postoperative bleeding and there was no association found between postoperative bleeding and presence of tonsilloliths (P = 0.38). CONCLUSION: In the current study there was no association found between the presence of tonsilloliths (indicating low grade chronic inflammation) and hemorrhage after tonsillectomy. Continued larger sample evaluations of possible risk factors for post-tonsillectomy hemorrhage patterns are encouraged.

Squamous Cell Carcinoma of the Temporal Bone Arising from Cholesteatoma: A Case Report and Review of the Literature.

Objective Present a case of squamous cell carcinoma of the temporal bone (SCCTB) arising in a 61-year-old female with a prior history of cholesteatoma and persistent otologic symptoms and review the current literature regarding this disease presentation. Setting Tertiary academic center. Patient A 61-year-old female with a history of left ear cholesteatoma for which she had undergone surgery 54 years prior. The patient presented with a persistent history of otorrhea since first surgery and developed exacerbation of symptoms just prior to presentation at our department. The clinical picture was highly suspicious of cholesteatoma recurrence. However, the biopsy was consistent with squamous cell carcinoma. Intervention Surgical debulking of the lesion was followed by a brief course of radiation therapy later halted by the patient due to side effect intolerance. Conclusion SCCTB may arise from cholesteatoma. A high index of suspicion for SCCTB should be maintained in patients with a prior history of cholesteatoma and evidence of a temporal bone mass with persistent otologic symptoms.

Dural Contact to the Malleus Head in Patients with Superior Semicircular Canal Dehiscence (SSCD): Case Series and Review of SSCD and Tegmen Defects.

OBJECTIVE: Report a series of cases in which patients have concomitant superior semicircular canal dehiscence (SSCD) and a dehiscent tegmen tympani with Dural contact to the malleus head (DCMH). METHODS: An analysis of radiologic and audiologic data in 4 patients who presented with SSCD and DCMH at a tertiary care institution. A pertinent literature review was performed. RESULTS: Four patients (5 ears) had SSCD and DCMH. In 3 patients with unilateral DCMH, the mean maximum air-bone gap was 15 dB in the ear with DCMH compared to 50 dB in the ear without DCMH. Of the 5 ears with DCMH, the mean air conduction threshold at 250 Hz was 17 dB compared to 42 dB in the 3 ears without DCMH. CONCLUSIONS: We report the findings of DCMH in a series of 4 patients with bilateral SSCD. This limited series suggests that ears with SSCD and DCMH have less of an air-bone gap than would be expected, as 1 would expect an additive effect of DCMH and SSCD on the air-bone gap.

Diagnosis and patterns of hearing loss in children with severe developmental delay.

INTRODUCTION & OBJECTIVE: Children with cognitive delay often experience challenges with obtaining hearing thresholds through behavioral audiometry (BA). This necessitates sedated Auditory Brainstem Response (sABR) testing. This study aimed to evaluate diagnostic and hearing patterns in children with Down Syndrome (DS), Autism Spectrum Disorder (ASD), Global Developmental delay (GDD), and Cerebral Palsy (CP) who were unable to complete reliable BA testing due to severe cognitive delay. METHODS: Retrospective chart review on a cohort of children aged 0.5-18 years with a diagnosis of DS, ASD, GDD, or CP who underwent sABR due to unsuccessful BA testing. This was performed at a tertiary care institution from 2014 to 2019. Testing patterns and audiometric data were collected. RESULTS: Across 15 DS, 39 ASD, 10 GDD, and 11 CP patients, the average time from first nondiagnostic BA to sABR ranged from 8.6 months (in GDD) to 21.8 months (in DS). The average number of BAs performed before sABR ranged from 1.6 (in ASD and GDD) to 2.7 (in DS). Hearing loss (HL) was diagnosed in 10%, 13%, 36% and 46% of patients with GDD, ASD, CP and DS respectively. Up to 75% of the HL was sensorineural (in CP patients). CONCLUSION: In children with significant cognitive delays, a high incidence of HL (especially SNHL) was identified, therefore high suspicion for HL should be held in these patients. Multiple unsuccessful BAs contribute to prolonged time to diagnosis and treatment, thus prompt sABR should be performed in patients whose severe cognitive delay inhibits reliable testing with BA.

Pediatric laryngeal electromyography technique for vocal fold immobility using bipolar double hookwire electrodes.

Vocal fold immobility (VFI) is a common cause of dysphonia and dysphagia in children. Laryngeal electromyography (LEMG) is an important adjunctive test in its diagnosis and treatment. In this study, we present an alternative technique in which bipolar double hookwire electrodes allow simultaneous placement and recordings from the bilateral thyroarytenoid and posterior cricoarytenoid muscles. Details of the technique are shown for a 5 y/o F with history of Neurofibromatosis Type 2 with left VFI and aspiration and dysphonia. This modified technique for pediatric LEMG has the potential to be a useful tool in predicting return of laryngeal function.

Utilization of diagnostic testing for pediatric sensorineural hearing loss.

BACKGROUND/OBJECTIVE: Sensorineural hearing loss is a common diagnosis among children. The diagnostic workup varies widely among practitioners. This study's aim was to assess the utilization of diagnostic testing for SNHL and determine the yield of each test. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. SUBJECTS: 827 patients with a diagnosis of SNHL from January 1, 2011 to January 1, 2015. RESULTS: 746 patients met inclusion criteria. Temporal bone imaging was performed on 561 (75%) of patients with 224 (40%) having positive results that explained the etiology of the SNHL. Congenital SNHL was more likely to be associated with abnormal imaging than acquired SNHL (109/299 versus 106/316 respectively) (p = 0.001). Unilateral SNHL was more likely to be associated imaging abnormalities than bilateral SNHL (101/221 and 123/340 respectively) (p = 0.028). Genetic testing was performed on 244 (33%) patients, of which 94 (39%) had abnormalities. Positive genetics results were more common with bilateral than unilateral SNHL (82/191 and 12/53 respectively) (p = 0.007). There was no statistically significant difference in the utility of genetic testing for congenital and acquired SNHL (p = 0.0836). Cytomegalovirus (CMV) testing was available for 104 (14%) of patients with 13 (12.5%) being positive and consistent with congenital CMV. Electrocardiogram, urinalysis, and Lyme titers were less useful. CONCLUSIONS: Imaging and genetic testing had the highest yield in the evaluation of children with SNHL and were the most commonly performed. CMV testing was valuable in neonates who failed newborn hearing screening.

Management of complex pediatric laryngotracheal stenosis with skin graft reconstruction.

OBJECTIVES: For pediatric patients with laryngotracheal stenosis, the ultimate goal is creation of a safe, functional airway. Unfortunately, wound healing in a hollow structure can complicate repair attempts, leading to restenosis. Herein, we present our experience using skin-grafting techniques in two complex pediatric laryngotracheal stenosis cases, leading to successful decannulation or speech production. METHODS: A chart review was performed examining the evaluation and management of two pediatric patients with laryngotracheal stenosis despite prior reconstructive attempts. Patient history, bronchoscopic evaluation, intra-operative technique, post-operative management, treatment outcomes, and complications were noted. Harvesting and preparation of the split-thickness skin grafts (STSG) proceeded in a similar manner for each case. Stenting material varied based on the clinical scenario. RESULTS: Using this technique, our patient with a Type 3 glottic web achieved substantial improvement in exercise tolerance, as well as vocal strength and quality. In addition, our aphonic patient could vocalize for the first time since her laryngotracheal injury. CONCLUSIONS: Temporary endoluminal stenting with skin graft lining can reproduce epithelial continuity and provide "biological inhibition" to enhance the wound healing process. When previous reconstructive efforts have failed, use of STSG can be considered in the management of complex pediatric laryngotracheal stenosis.

Hypoglossal Nerve Stimulation in Adolescents With Down Syndrome and Obstructive Sleep Apnea.

IMPORTANCE: Obstructive sleep apnea (OSA) affects up to 60% of children with Down syndrome (DS) and may persist in half of patients after adenotonsillectomy. Children with DS who have persistent OSA often do not tolerate treatment with positive pressure airway support devices or tracheotomy for their residual moderate to severe OSA. The hypoglossal nerve stimulator is an implantable device that delivers an electrical impulse to anterior branches of the hypoglossal nerve in response to respiratory variation, resulting in tongue base protrusion that alleviates upper airway obstruction in adults. OBJECTIVE: To determine whether hypoglossal nerve stimulation is safe and effective in children with DS. DESIGN, SETTING, AND PARTICIPANTS: Case series of the first 6 adolescents with DS to undergo hypoglossal nerve stimulator implantation. Participants were 6 children and adolescents (12-18 years) with DS and severe OSA (apnea hypopnea index [AHI] > 10 events/h) despite prior adenotonsillectomy. INTERVENTION: Inspire hypoglossal nerve stimulator placement. MAIN OUTCOMES AND MEASURES: Patients were monitored for adverse events. Adherence to therapy was measured by hours of use recorded by the device. Efficacy was evaluated by comparing AHI and OSA-18, a validated quality-of-life instrument, scores at baseline and follow-up. RESULTS: In 6 patients (4 male, 2 female; aged 12-18 years), hypoglossal nerve stimulator therapy was well tolerated (mean use, 5.6-10.0 h/night) and effective, resulting in significant improvement in OSA. At 6- to 12-month follow-up, patients demonstrated a 56% to 85% reduction in AHI, with an overall AHI of less than 5 events/h in 4 children and less than 10 events/h in 2 children. Children also demonstrated a clinically significant improvement (mean [SD] overall change score, 1.5 [0.6]; range, 0.9-2.3) on the OSA-18, a validated quality-of-life instrument. CONCLUSIONS AND RELEVANCE: Hypoglossal nerve stimulation was well tolerated and effective in the study population, representing a potential therapeutic option for patients with DS and refractory OSA after adenotonsillectomy who are unable to tolerate positive pressure airway devices. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT2344108.

Time-driven activity-based costing to estimate cost of care at multidisciplinary aerodigestive centers.

OBJECTIVES/HYPOTHESIS: Providing high-value healthcare to patients is increasingly becoming an objective for providers including those at multidisciplinary aerodigestive centers. Measuring value has two components: 1) identify relevant health outcomes and 2) determine relevant treatment costs. Via their inherent structure, multidisciplinary care units consolidate care for complex patients. However, their potential impact on decreasing healthcare costs is less clear. The goal of this study was to estimate the potential cost savings of treating patients with laryngeal clefts at multidisciplinary aerodigestive centers. STUDY DESIGN: Retrospective chart review. METHODS: Time-driven activity-based costing was used to estimate the cost of care for patients with laryngeal cleft seen between 2008 and 2013 at the Massachusetts Eye and Ear Infirmary Pediatric Aerodigestive Center. Retrospective chart review was performed to identify clinic utilization by patients as well as patient diet outcomes after treatment. Patients were stratified into neurologically complex and neurologically noncomplex groups. RESULTS: The cost of care for patients requiring surgical intervention was five and three times as expensive of the cost of care for patients not requiring surgery for neurologically noncomplex and complex patients, respectively. Following treatment, 50% and 55% of complex and noncomplex patients returned to normal diet, whereas 83% and 87% of patients experienced improved diets, respectively. Additionally, multidisciplinary team-based care for children with laryngeal clefts potentially achieves 20% to 40% cost savings. CONCLUSIONS: These findings demonstrate how time-driven activity-based costing can be used to estimate and compare patient costs in multidisciplinary aerodigestive centers. LEVEL OF EVIDENCE: 2c. Laryngoscope, 127:2152-2158, 2017.

PD-L1 expression and CD8+ infiltration shows heterogeneity in juvenile recurrent respiratory papillomatosis.

INTRODUCTION: Tumor immunotherapy have broadened therapeutic options for tumor treatment. The role of immune function in juvenile recurrent respiratory papillomatosis (JRRP) has not been investigated. Applying immunoblockade inhibitors as a novel disease treatment is unclear. Our study, for the first time, evaluates immune infiltration and immuno-suppressive molecule expression in JRRP. Our study provides insights in possibly treating this disease with tumor immunotherapies. We aimed to determine expression of programmed death-ligand 1 (PD-L1), a cancer escape protein, and presence of CD8+ T cell infiltration in tumor microenvironment. MATERIAL AND METHODS: Seven patients with JRRP (mean age: 7.43; age range 3-17) in this study routinely have their tumors surgical debulked at Massachusetts Eye and Ear Infirmary. Following surgery, samples were de-identified and sent to pathology where they were stained and analyzed. RESULTS: Six out of seven patients expressed PD-L1 on tumor cells to various extents. Three patients showed concurrent PD-L1 expression on tumor cells and abundant CD8+ tumor infiltrating lymphocytes as well as PD-L1+ stromal lymphocytes, while PD-L1 expression on tumor cells were not associated with CD8+ tumor infiltrating T cells nor PD-L1+ stromal lymphocytes in the other three patients. HPV 6/11 and p16 was detected in all the patients. There appeared to be no correlation between either PD-L1 expression and CD8+ infiltration and clinical severity as measured by both the number of surgeries per year or Derkay score. CONCLUSIONS: Despite a small cohort, the expression of p16 and HPV 6/11 in all of the patients confirms the tissues were HPV tumor cells. PD-L1 expression was detected in the vast majority of tumor samples, while inflammatory cell compartments showed a higher degree of variation. Expression of PD-L1 on tumor cells but not inflammatory cells raises the possibility of a tumor cell intrinsic manner of PD-L1 expression. In contrast, a group of patients showed PD-L1 positivity in both tumor and inflammatory cells along with abundant CD8+ tumor infiltrating lymphocytes, suggesting adoptive immune resistance in these tumors and potential benefits from tumor immunotherapy.

Radiation exposure from videofluoroscopic swallow studies in children with a type 1 laryngeal cleft and pharyngeal dysphagia: A retrospective review.

INTRODUCTION: Radiation exposure is recognized as having long term consequences, resulting in increased risks over the lifetime. Children, in particular, have a projected lifetime risk of cancer, which should be reduced if within our capacity. The objective of this study is to quantify the amount of ionizing radiation in care for children being treated for aspiration secondary to a type 1 laryngeal cleft. With this baseline data, strategies can be developed to create best practice pathways to maintain quality of care while minimizing radiation exposure. METHODS: Retrospective review of 78 children seen in a tertiary pediatric aerodigestive center over a 5 year period from 2008 to 2013 for management of a type 1 laryngeal cleft. The number of videofluoroscopic swallow studies (VFSS) per child was quantified, as was the mean effective dose of radiation exposure. The 78 children reviewed were of mean age 19.9 mo (range 4 mo-12 years). All children were evaluated at the aerodigestive center with clinical symptomatology and subsequent diagnosis of a type 1 laryngeal cleft. Aspiration was assessed via VFSS and exposure data collected. Imaging exams where dose parameters were not available were excluded. RESULTS: The mean number of VFSS each child received during the total course of treatment was 3.24 studies (range 1-10). The average effective radiation dose per pediatric VFSS was 0.16 mSv (range: 0.03 mSv-0.59 mSv) per study. Clinical significance was determined by comparison to a pediatric CXR. At our facility a CXR yields an effective radiation dose of 0.017 mSv. Therefore, a patient receives an equivalent total of 30.6 CXR over the course of management. CONCLUSIONS: Radiation exposure has known detrimental effects particularly in pediatric patients. The total ionizing radiation from VFSS exams over the course of management of aspiration has heretofore not been reported in peer reviewed literature. With this study's data in mind, future developments are indicated to create innovative clinical pathways and limit radiation exposure.

Modified Best-Practice Algorithm to Reduce the Number of Postoperative Videofluoroscopic Swallow Studies in Patients With Type 1 Laryngeal Cleft Repair.

IMPORTANCE: There is no consensus as to the timing of videofluoroscopic swallow studies (VFSSs) in determining resolving aspiration after laryngeal cleft repair. There is a growing literature on the effect of radiation exposure in children. OBJECTIVE: To modify a previously published best-practice algorithm based on a literature review and our clinical experience to maintain the quality of care provided after successful type 1 laryngeal cleft repair, while reducing the total number of postoperative VFSSs by 10% or greater. DESIGN, SETTING, AND PARTICIPANTS: The previously published algorithm was modified by a multidisciplinary group at a tertiary care academic medical center (Massachusetts Eye and Ear) and was prospectively applied to 31 children who underwent type 1 laryngeal cleft repair from January 1, 2013, to February 28, 2015. MAIN OUTCOMES AND MEASURES: The number of VFSSs obtained in the first 7 months after surgery was compared with the peer-reviewed literature and with a retrospective cohort of 27 patients who underwent type 1 laryngeal cleft repair from January 1, 2008, to December 31, 2012. RESULTS: The study cohort comprised 31 patients. Their ages ranged from 10 to 48 months, with a mean (SD) age of 23.94 (9.93) months, and 19% (6 of 31) were female. The mean (SD) number of postoperative VFSSs per patient before and after implementation of the algorithm was 1.22 (0.42) and 1.03 (0.55), respectively. The use of the algorithm reduced the number of VFSSs by 0.19 (95% CI, -0.07 to 0.45). This reduction in radiation exposure is equivalent to 1.47 chest radiographs per child per course of care. Surgical success was 87% (27 of 31) compared with our group's previously published success rate of 78% (21 of 27) (absolute difference, 0.09; 95% CI, -0.17 to 0.34). CONCLUSIONS AND RELEVANCE: This modified algorithm to help guide decisions on when and how often to obtain VFSSs after type 1 laryngeal cleft repair can limit patients' radiation exposure, while maintaining high surgical success rates.