Operative Surveillance of Airway Hemangiomas in PHACE Syndrome.

OBJECTIVE: PHACE is a rare syndrome that can present with airway hemangiomas. Management for these patients is variable and the utilization of operative endoscopic airway evaluation has not been described. The objectives of this study were to identify the incidence of airway symptoms in patients being evaluated for PHACE syndrome and determine the utility of operative endoscopy. METHODS: An IRB-approved retrospective cohort study was conducted on consecutive pediatric patients with head and neck infantile hemangioma (IH) evaluated in a multi-disciplinary vascular anomalies center between 2013 and 2019. Patients were included if they were being worked up for PHACE syndrome and had an otolaryngology evaluation. Demographics, clinical, and surgical variables were collected. RESULTS: There were 317 patients with head and neck IH. Thirty-six patients met inclusion criteria. The majority of patients were female (31/36; 86.1%) and less than half of the patients (15/36; 41.7%) were eventually diagnosed with PHACE syndrome. Median age at presentation was 2 months (range 0-82 months). A total of 28/36 (77.8%) of patients were managed with propranolol. The majority of the patients presented without aerodigestive symptoms; however, 16/36 (44.4%) of patients presented with symptoms such as stridor, hoarseness, and dysphagia. A total of 20/36 (55.6%) of patients underwent operative endoscopy. A total of 8/20 (40.0%) of patients who underwent operative endoscopy had operative intervention. Of the entire cohort, only 2/15 (13.3%) patients diagnosed with PHACE were found to have a subglottic hemangioma. Both patients presented with stridor. CONCLUSION: Operative endoscopy remains useful in the workup of PHACE syndrome to identify subglottic hemangiomas, however there may be relatively low yield in asymptomatic patients. In office flexible laryngoscopy may be a less invasive means to examine the subglottic region. A multi-center prospective study would be necessary to evaluate incidence of subglottic hemangiomas in asymptomatic patients evaluated for PHACE.

Quality of Life in Patients With Recurrent and Second Primary Head and Neck Cancer.

OBJECTIVE: In the setting of similar outcomes, quality of life (QOL) measures can be utilized to compare treatment modalities in head and neck squamous cell carcinoma (HNSCC). We evaluate QOL and symptoms in patients treated for primary, second primary, and recurrent HNSCC. STUDY DESIGN: Retrospective cohort study. SETTING: Head and neck cancer survivorship clinic. METHODS: We identified patients seen between 2016 and 2019. QOL and symptoms were assessed with the University of Washington Quality of Life (UW-QOL) questionnaire, 10-item Eating Assessment Tool, 8-item Patient Health Questionnaire, 7-item Generalized Anxiety Disorder, and Neck Disability Index. Regression analysis was utilized to explore associations and compare QOL outcomes. RESULTS: Our cohort comprised 662 patients: 546 with primary HNSCC, 34 with second primary HNSCC, and 82 with recurrent HNSCC. Multimodality therapy was associated with lower UW-QOL Physical Subscale (UW-QOL-PS) vs single modality: chemoradiation therapy (-12.17 [95% CI, -16.57 to -7.78]) and surgery + postadjuvant treatment (-12.11 [-16.06 to -8.16]). Multimodality therapy was also associated with lower UW-QOL Social-Emotional Subscale (UW-QOL-SS): chemoradiation therapy (-6.70 [-11.41 to -1.99]) and surgery + postadjuvant treatment (-7.41 [-11.63 to -3.19]). Recurrence (-14.42 [-18.80 to -10.04]) and second primary (-11.15 [-17.71 to -4.59]) demonstrated lower UW-QOL-PS vs primary. Radiation for recurrence or second primary had worse UW-QOL-PS (-10.43 [-19.27 to -1.59]) and UW-QOL-SS (-10.58 [-18.76 to -1.54]) and higher Eating Assessment Tool (6.08 [1.39-10.77]) than surgery alone. Surgery + postadjuvant treatment showed worse UW-QOL-PS (-12.65 [-23.76 to -1.54]) and UW-QOL-SS (-12.20 [-22.38 to -2.03]). CONCLUSION: Multimodality therapy, particularly with recurrent and second primary HNSCC, is more likely to contribute to diminished QOL and symptoms. This important consideration should play a role in framing informed discussions with patients regarding treatment.

Factors affecting obstructive sleep apnea patients' use of upper airway stimulation treatment.

STUDY OBJECTIVES: Upper airway stimulation (UAS) is an alternative treatment for obstructive sleep apnea that must be activated nightly. Although the implanted device offsets the mask- or pressure-related side effects often associated with continuous positive airway pressure therapy, some UAS recipients do not use the therapy consistently. This study qualitatively explored factors associated with UAS usage in obstructive sleep apnea patients. METHODS: Semistructured interviews were conducted with 24 obstructive sleep apnea patients who received UAS treatment. Twelve patients were categorized as high users with mean usage of ≥ 4 hours/night and 12 were categorized as low users with < 4 hours/night or nonuse. Interviews explored patients' experiences regarding barriers and facilitators to UAS use and their advice for new UAS recipients. Demographic and clinical data including the Insomnia Severity Index and Generalized Anxiety Disorder Scale were collected. RESULTS: Compared to high users, low users had higher levels of insomnia (mean Insomnia Severity Index: 3.6 vs 15.2, respectively) and anxiety (mean Generalized Anxiety Disorder Scale: 3.4 vs 6.9). High users reported more positive experiences with UAS treatment, such as improvements in symptoms and convenience of treatment, as facilitators of use. Low users tended to focus on the negative aspects of treatment, particularly stimulation-related discomfort and associated sleep disturbance. CONCLUSIONS: Insomnia with or without anxiety contributes to differing patient-reported experiences in high vs low user groups, with increased insomnia symptoms among low users. Improved understanding of the specific barrier and facilitators of UAS adherence may drive better long-term use and more personalized management strategies, including concomitant insomnia treatment. CLINICAL TRIALS REGISTRATION: Registry: ClinicalTrials.gov; Name: Stimulation Therapy for Apnea: Reporting Thoughts (START); URL: https://clinicaltrials.gov/ct2/show/NCT04768543; Identifier: NCT04768543. CITATION: Luyster FS, Ni Q, Lee K, et al. Factors affecting obstructive sleep apnea patients' use of upper airway stimulation treatment. J Clin Sleep Med. 2022;18(9):2207-2215.

Cost analysis of sialendoscopy for the treatment of salivary gland disorders in children.

INTRODUCTION: Recent advances in Otolaryngology have changed the diagnosis and therapy for salivary gland disorders. Sialendoscopy-assisted surgery is a minimally invasive, conservative procedure for functional preservation of the affected gland. The goals of this study are to assess the indications, use, and outcomes of pediatric sialendoscopy at a tertiary pediatric institution as well as to analyze the direct cost related to the diagnosis and treatment of patients with sialolithiasis and Juvenile Recurrent Parotitis managed with sialendoscopy. METHODS: Retrospective cohort study of pediatric patients undergoing diagnostic and/or therapeutic sialendoscopy at a tertiary level children's hospital between 2012 and 2020. Demographic, clinical, surgical variables and direct hospital costs 1 year before and after the sialendoscopy procedure were collected and analyzed. RESULTS: Twenty-two pediatric patients were included. There was male predominance (59.3%). The most common indication for sialendoscopy was Juvenile Recurrent Parotitis. Average age of onset was 6.5 years for patients with JPR and 14.2 years for patients with sialolithiasis. All patients had an average of 4.5 episodes before the first procedure. 8 patients required repeat sialendoscopy for recurrent symptoms. Mean total hospital costs were significantly higher in patients with JRP 1 year before and after the sialendoscopy ($4308.8 vs. $3330) compared to patients with sialolithiasis. Costs of the sialendoscopy and related expenses including anesthesia and PACU cost were similar in both studied groups ($13,506 vs. $13,022.9). Complete resolution of symptoms was achieved in 14 patients with JRP and all patients with sialolithiasis. CONCLUSION: Sialendoscopy is a low-risk procedure that aids in the treatment for pediatric salivary gland disorders. The costs related to sialendoscopy are substantial and patients with JRP incur higher hospital preoperative and postoperative costs compared to patients with sialolithiasis.

Utilization of Diagnostic Testing for Renal Anomalies and Congenital Heart Disease in Patients with Microtia.

OBJECTIVE: Congenital ear anomalies are associated with congenital cardiac and renal defects. Renal ultrasounds, electrocardiogram, and echocardiogram can be utilized for diagnosis of these concurrent defects. No standard of care exists for the workup of patients with microtia. The goals of this study were to describe the utilization of diagnostic testing for cardiac and renal anomalies and to identify their prevalence in patients with microtia. STUDY DESIGN: Case series with chart review. SETTING: Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center. SUBJECTS AND METHODS: This study is an Institutional Review Board-approved retrospective review of consecutive patients born between 2002 and 2016 who were diagnosed with microtia and seen in the otolaryngology clinic at a tertiary care children's hospital. Demographics, sidedness and grade of microtia, comorbid diagnoses, and details of renal and cardiovascular evaluations were recorded. Factors associated with retroperitoneal ultrasound and cardiac testing were assessed with logistic regression. RESULTS: Microtia was present in 102 patients, and 98 patients were included as they received follow-up. Microtia was associated with craniofacial syndrome in 34.7% of patients. Renal ultrasound was performed in 64.3% of patients, and 12.9% of patients with ultrasounds had renal aplasia. Cardiac workup (electrocardiogram or echocardiogram) was completed in 60.2% of patients, and of this subset, 54.2% had a congenital heart defect. CONCLUSION: Diagnostic testing revealed renal anomalies and cardiac defects in patients with isolated microtia at a higher rate than in the general population. This suggests the need for further evaluation of the role of routine screening in patients with microtia.

Expansile Superabsorbent Polymer Ball Foreign Body in the Ear.

BACKGROUND: Superabsorbent polymers (SAPs) are materials that can absorb large quantities of water. Small spherical SAPs are commonly marketed as toys for children. CASE REPORT: We report the case of a 4-year-old female who presented to a pediatric emergency department with a small, marble-sized SAP that was placed in her ear by herself during the course of play at daycare. On examination, the object was translucent and difficult to visualize. After multiple attempts at removal, the object was partially removed using an ear curette. The patient sustained a small tympanic membrane perforation. During a follow-up operative intervention by otorhinolaryngology, the remainder of the object was removed using a suction device and the tympanic membrane was repaired. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: While large SAPs associated with intestinal obstruction have been removed from the market, smaller SAPs can present a health risk when placed in orifices such as the ear or nose. Cases of foreign body SAPs have been misdiagnosed as ear infections and treated with topical antibiotics, which can lead to enlargement of the foreign body. Irrigation techniques and topical medications should not be used. SAPs are friable and can break into multiple pieces during removal attempts. Blunt tools, such as ear curettes, may be best suited for their removal. If the item breaks up during removal or if removal fails, urgent consultation with an otorhinolaryngologist is recommended.

Radiodensity of the Ostiomeatal Complex in Recurrent Acute Rhinosinusitis.

Objective Recurrent acute rhinosinusitis (RARS) can be an elusive diagnosis due to the lack of clinical and radiographic findings in between acute episodes. This study aims to identify objective computed tomography (CT) characteristics in RARS. Study Design Case series with chart review. Setting Tertiary academic medical center. Subjects and Methods Patients meeting diagnostic criteria for RARS were identified retrospectively from the senior author's practice. Patients were diagnosed with RARS if they experienced 3 or more episodes of acute sinusitis within the past year with complete resolution of symptoms in between episodes. At least 1 episode was confirmed via CT or endoscopy. CT scans showing prior surgery were excluded. The scans for these patients were compared with those from a normal control group. Bone radiodensity of the entire ostiomeatal complex (OMC), including the ethmoid bulla, middle turbinate, and uncinate, was measured in Hounsfield units. Maximum radiodensity was noted for each side. Results A total of 16 patients meeting inclusion criteria for RARS were compared with 16 healthy patients. The mean Lund-MacKay score was 1.6 in the RARS group and 1.3 in the control group. The maximum radiodensity of the OMC was significantly higher in the RARS group (556) compared with that of the control group (327) ( P < .0001). Conclusion Patients with RARS had significantly greater radiodensity of the OMC compared with those in the control group despite minimal differences in mucosal disease. Radiodensity measurement of the OMC using Hounsfield units may help to identify patients with this elusive diagnosis.

Coblation of suprastomal granulomas in tracheostomy-dependent children.

OBJECTIVE: Suprastomal granulomas pose a persistent challenge for tracheostomy-dependent children. They can limit phonation, cause difficulty with tracheostomy tube changes and prevent decannulation. We describe the use of the coblator for radiofrequency plasma ablation of suprastomal granulomas in five consecutive children from September 2012 to January 2016. METHOD: Retrospective case series at a tertiary medical center. RESULTS: The suprastomal granuloma could be removed with the coblator in all 5 cases. Three were removed entirely endoscopically and 2 required additional external approach through the tracheal stoma for complete removal. There were no intraoperative or postoperative complications. One patient was subsequently decannulated and 2 patients have improved tolerance of their speaking valves. Two patients remain ventilator dependent, but their bleeding and difficulty with tracheostomy tube changes resolved. Three of the patients have had subsequent re-evaluation with bronchoscopy, demonstrating resolution or markedly decreased size of the granuloma. This technique is time efficient, simple and minimizes risks associated with other techniques. The relatively low temperature and use of continuous saline irrigation with the coblator device minimizes the risk of airway fires. Additionally, the risk of hypoxia from keeping a low fractional inspiratory oxygen level (FIO2) to prevent fire is avoided. The concurrent suction in the device decreases blood and tissue displacement into the distal airway. CONCLUSION: Coblation can be used safely and effectively with an endoscopic or external approach to remove suprastomal granulomas in tracheostomy-dependent children. More studies that are larger and have longer follow-up are needed to evaluate the use of this technique.

Intranasal fluticasone associated with delayed tympanostomy tube placement in children with eustachian tube dysfunction.

OBJECTIVES: Pediatric patient caregivers may prefer to avoid a surgical intervention and request a medical management option for eustachian tube dysfunction (ETD). However, there are limited published data evaluating the efficacy of intranasal fluticasone in the medical management of ETD as an alternative to tympanostomy tube placement. The objectives of this study were to: 1) determine if intranasal fluticasone (INF) prevented tympanostomy tube placement in children with ETD, and 2) describe differences in patient response to INF related to cleft lip and/or palate (CLP) and Down syndrome. METHODS: Case series with planned chart review at a Tertiary academic hospital. We reviewed pediatric patients treated with INF for ETD. Inclusion criteria included ETD, no prior intranasal or oral steroid therapy, and no prior tympanostomy tube placement. Outcomes included time-to- tympanostomy tube placement with or without INF and therapy compliance. Kaplan-Meier survival analyses with log-rank tests and Fisher's exact tests were used to examine outcome variables. RESULTS: 676 fulfilled inclusion criteria. 393 (58.7%) were male, and 355 (52.5%) Caucasian with mean age of 27.1 months old. 92 (13.6%) had CLP and 46 (6.8%) had Down Syndrome. 266 (39.4%) received INF, and 202 (88.2%) were compliant at their next visit. 474 (70.1%) had tympanostomy tubes placed. Children treated with INF were less likely to have tympanostomy tubes placed than children not treated (52.6% vs. 81.5%; p < 0.0001). Using survival analyses, INF use was associated with significantly longer mean time-to-tympanostomy tube than no INF use (199.4 vs. 133.7 days; p < 0.0001). INF did not reduce time-to-tympanostomy tube in patients with CLP (p = 0.05) or Down Syndrome (p = 0.27). CONCLUSION: INF significantly reduces the number of children requiring tympanostomy tube placement for ETD. The CLP and Down Syndrome anatomical variants may attenuate INF efficacy. Further in vivo characterization of INF action on eustachian tube tissues will help further substantiate these observations.

Practice patterns in supraglottoplasty and perioperative care.

OBJECTIVES: Supraglottoplasty is the first-line surgical treatment for severe laryngomalacia. The purpose of this study is to determine the current trends of practice patterns in managing children who require supraglottoplasty. METHODS: A 25-question survey regarding supraglottoplasty techniques and perioperative management was sent by e-mail to 274 physician members of the Society for Ear, Nose and Throat Advances in Children (SENTAC). RESULTS: 101 surgeons responded and 72% of respondents were in academic practice (p < 0.0001). All four United States regions, Canada and the United Kingdom were represented. The most commonly reported age of patients undergoing supraglottoplasty was 1-3 months (62% of respondents). Indications include worsening airway symptoms (43%), failure to thrive (41%) and worsening feeding (10%). The majority of respondents (89%) treat these patients for reflux with 54% prescribing PPIs and 41% prescribing H2 blockers. Cold steel is the most popular surgical technique (73%) followed by laser (14%), microdebrider (10%) and coblator (3%) (p < 0.0001). Most respondents (92%) administer intraoperative steroids with the majority of them choosing dexamethasone (99%). Perioperative antibiotics are administered by 23% of respondents. Almost all respondents admit their patients for post-operative observation (99%) and 53% of these admit to PICU rather than step-down or floor status. The level of care is associated with the number of supraglottoplasties performed per year (p = 0.009) and with the geographic region (p = 0.02). Surgeons who perform fewer supraglottoplasties tend to admit to a higher level of care. Those in the South and Northeast regions tend to admit more to floor status. Only 13% routinely keep patients intubated post-operatively. CONCLUSIONS: This provider survey study highlights some significant variations and trends in practice patterns of otolaryngologists who perform supraglottoplasty. The majority utilizes anti-reflux pharmacotherapy; however, there is no consensus in which type. The method for supraglottoplasty also varies with cold steel being the most popular, although no single method has been shown to be superior. There is variation in post-operative care with trends for keeping patients extubated and admitting them to an intensive care. With the importance of safe, effective, and also cost-conscious care, further studies are needed to understand the optimal management of those who undergo supraglottoplasty.

Cost analysis and outcomes of a second-look tympanoplasty-mastoidectomy strategy for cholesteatoma.

OBJECTIVES/HYPOTHESIS: To analyze cost and compare cholesteatoma recidivism and hearing outcomes with single-stage and second-look operative strategies. STUDY DESIGN: Retrospective review and cost analysis. METHODS: Adult and pediatric patients who underwent a tympanoplasty with mastoidectomy for cholesteatoma with a single-stage or second-look operative strategy were identified. Variables included procedure approach, residual or recurrent cholesteatoma, ossicular chain reconstruction frequency, and operative complications. Audiologic outcomes included pre-/postoperative air bone gap (ABG) and word recognition score (WRS). Cost analysis included charges for consultation and follow-up visits, surgical procedures, computed tomography temporal bone scans, and audiology visits. RESULTS: One hundred and six patients had a tympanoplasty with mastoidectomy for cholesteatoma, with 80 canal wall-up procedures (CWU) as initial approach. Of these, 46 (57.5%) CWU patients had a planned second look. Two (4.3%) CWU patients had recurrent cholesteatoma and 20 (43.4%) had residual identified at second look. Four (11.7%) single-stage CWU strategy patients developed recurrent cholesteatoma. There was no significant difference in pre-/postoperative ABG and WRS between second look and single stage (P > 0.05). Compared to second-look patients, single-stage patients had significantly fewer postoperative visits (6.32 vs. 10.4; P = 0.007), and significantly lower overall charges for care ($23,529. vs. $41.411; P < 0.0001). CONCLUSION: The goal of cholesteatoma surgery is to produce a safe ear, and a second-look strategy after CWU has historically been used to evaluate for recurrent or residual disease. The cholesteatoma recurrence rate at a second look after a CWU tympanoplasty-mastoidectomy is low. Costs of operative procedures are a significant proportion of healthcare resource expenditures. Considering the low rate of cholesteatoma recurrence and relatively high cost of care, implementation of a second-look strategy should be individually tailored and not universally performed. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:2574-2579, 2016.

A computational analysis of nasal vestibule morphologic variabilities on nasal function.

Although advances in computational modeling have led to increased understanding of nasal airflow, not much is known about the effects of normal sinonasal anatomic variabilities on nasal function. In this study, three distinct variations in the human nasal vestibule airspace that have not been previously described were identified. Computational fluid dynamics modeling of nasal airflow profile in each identified variation of nasal vestibule phenotype was conducted to assess the role of these phenotypes on nasal physiology. Three-dimensional reconstructions of the nasal geometry in sixteen subjects with normal radiographic sinonasal images were created and each respective unilateral nasal cavity was classified as Notched, Standard, or Elongated phenotype based nasal vestibule morphology. Steady state, laminar and incompressible flow simulations were performed in the nasal geometries under physiological, pressure-driven conditions with constant inspiratory pressure. Results showed that at localized regions of the unilateral nasal cavity, average resistance was significantly different among nasal vestibule phenotypes. However, global comparison from nostril to choana showed that average resistance was not significantly different across phenotypes; suggesting that with normal anatomic variations, the nose has a natural compensatory mechanism that modulates localized airflow in order to achieve a desired amount of global airflow.

Surgical Correction of an Extensive Skull Base Defect as a Result of Pneumocele.

Pneumocele is a condition involving the pathologic expansion of paranasal sinuses with corresponding bony erosion of the skull base. Extensive pneumocele can lead to pneumocephalus, especially in patients with long-term cerebrospinal fluid shunts. Here, the authors present a case of pneumocele in an adult with a history of congenital hydrocephalus managed with a ventriculoperitoneal shunt. The patient underwent reconstruction of a posterior table defect with a pericranial flap followed by frontal sinus obliteration with abdominal fat.