Faculty Bibliography

Flexible laryngoscopy, the gold-standard evaluation of the larynx and the pharynx, is one of the most commonly performed procedures in otolaryngology. During the coronavirus disease 2019 (COVID-19) pandemic, flexible laryngoscopy represents a risk for patients and an occupational hazard for otolaryngologists and any clinic staff involved with the procedure or endoscope reprocessing. Here we present a set of recommendations on flexible laryngoscopy performance during the pandemic, including patient selection, personal protective equipment, and endoscope disinfection, based on a consensus reached during a virtual webinar on March 24, 2020, attended by approximately 300 participants from the American laryngology community.

BACKGROUND:Some petroclival meningiomas cause trigeminal nerve compression leading to disabling trigeminal neuralgia. Tumor resection and nerve decompression can offer pain relief but may not be feasible in all patients. Simultaneous stereotactic radiosurgery (SRS) to the tumor and nerve is another option. It is an effective means of treating meningiomas and trigeminal neuralgia separately, but there is limited data regarding the efficacy and outcomes of their concomitant treatment. CASE DESCRIPTION/METHODS:We present our series of four patients who presented with trigeminal neuralgia secondary to a petroclival mass causing compression of the trigeminal nerve. All patients underwent SRS to both the petroclival mass and trigeminal nerve in a single-session. The average margin tumor dose was 12.25Gy (range, 12 to 12.5Gy) and average maximum trigeminal nerve dose was 80Gy (range, 75 to 85Gy). Barrow Neurologic Institute (BNI) Pain Intensity Scores in all patients prior to intervention were a grade IV or V. At last follow-up (average = 29.8 months), all patients were pain free (BNI I or IIIA). Two patients experienced reduced facial sensation in either one or all three distributions. No brainstem edema was seen. CONCLUSION/CONCLUSIONS:This series highlights the benefit and safety of simultaneous treatment of petroclival tumors and the trigeminal nerve in a single session for patients affected by tumor related trigeminal neuralgia.

The novel Coronavirus (COVID-19) has created a deadly pandemic that is now significantly impacting the United States. Otolaryngologists are considered high risk for contracting disease, as the virus resides in the nasal cavity, nasopharynx, and oropharynx. While valuable work has been publicized regarding several topics in Rhinology, we discuss other aspects of our specialty in further detail. There are several issues regarding Rhinologic practice that need to be clarified both for the current epidemic as well as for future expected "waves." In addition, as the pandemic dies down, guidelines are needed to optimize safe practices as we start seeing more patients again. These include protocols pertinent to safety, in-office Rhinologic procedures, the substitution of imaging for endoscopy, and understanding the appropriate role of telemedicine. We discuss these aspects of Rhinology as well as practical concerns relating to telemedicine and billing, as these issues take on increasing importance for Rhinologists both in the present and the future.

BACKGROUND:The prevalence of survivors of oropharyngeal cancer (OPC) is increasing due to improved survival for individuals with human papillomavirus (HPV)-related disease. Although elderly survivors of OPC are known to have a high burden of comorbidities, to the authors' knowledge it is unknown how this compares with a similar cohort without a history of cancer. METHODS:The current retrospective, cross-sectional study included individuals with a first incident primary diagnosis of OPC from 2004 through 2011 from the Surveillance, Epidemiology, and End Results (SEER)-Medicare-linked databases and matched controls. The baseline prevalence and subsequent incidence of comorbid conditions were identified. The association between comorbidity and overall survival was evaluated. RESULTS:A total of 2497 eligible patients with OPC were matched to 4994 noncancer controls. Baseline comorbidity was higher in cases (Charlson Comorbidity Index >0 for 48.5% of cases vs 35.8% of controls). At 5 years, cases were more likely than controls to develop comorbidities. Survivors of OPC were at high risk (≥20% cumulative prevalence by 5 years) of developing several comorbidities, including cardiovascular diseases, cerebrovascular disease, chronic obstructive pulmonary disease, and tobacco abuse, and were at moderately high risk (10%-19% cumulative prevalence) of developing other conditions including carotid artery occlusive stroke, alcohol abuse, depression, and anxiety. In both cases and controls, the presence of the majority of comorbidities either at the time of diagnosis or during the follow-up period was associated with worse survival. CONCLUSIONS:Patients with OPC have a higher comorbidity burden compared with matched controls, both at baseline and during survivorship, the majority of which are associated with decreased survival. Oncologic surveillance of survivors of OPC should include screening for highly prevalent conditions.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.RESULTSThirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5-autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.

BACKGROUND:To the authors' knowledge, the question of whether human papillomavirus (HPV) infection is associated with outcomes in patients with sinonasal squamous cell carcinoma (SNSCC) is not well studied at this time. In the current study, the authors investigated patterns of HPV testing and its association with survival in patients with SNSCC using the National Cancer Data Base. METHODS:The authors selected all SNSCC cases diagnosed between 2010 and 2016. HPV testing practices, clinicodemographic factors, treatments, and survival were analyzed. Multivariable Cox regression and propensity score-matched survival analyses were performed. RESULTS:A total of 6458 SNSCC cases were identified. Of these, only 1523 cases (23.6%) were tested for HPV and included in the current study. The median patient age was 64 years and the majority had advanced stage tumors (overall AJCC stage III-IV, 721 patients; 62.1%). HPV-positive SNSCC comprised 31.5% (447 of 1418 cases) of the final study cohort. Among 15 hospitals that routinely tested nonoropharyngeal SCCs for HPV, the percentage of HPV-positive SNSCCs was smaller (24.6%; P = .04). Patients with HPV-positive SNSCC were younger (aged 60 years vs 65 years; P < .001), with tumors that were more likely to be high grade (55.3% vs 41.7%; P < .001), and attributed to the nasal cavity (62.2% vs 44.0%; P < .001). HPV-positive SNSCC was associated with significantly improved overall survival in multivariable regression analysis (hazard ratio, 0.45; 95% CI, 0.28-0.72 [P = .001]) and propensity score-matched (hazard ratio, 0.61; 95% CI, 0.38-0.96 [P = .03]) analyses controlling for clinicodemographic and treatment factors. CONCLUSIONS:Currently, only a minority of patients with SNSCC are tested for HPV. However, a sizable percentage of SNSCC cases may be HPV related; furthermore, HPV-positive SNSCC is associated with improved overall survival. Routine HPV testing may be warranted in patients with SNSCC.

OBJECTIVE:To identify risk factors and interventions affecting length of hospitalization (LOH) and clinical outcome in children with intracranial complications of rhinosinusitis. METHODS:Retrospective chart review of 12 children hospitalized at 2 academic medical centers for intracranial complications of rhinosinusitis over the past 5 years. RESULTS:12 patients were identified with an average age at presentation of 13 years old. 92% were male and 75% were African American. The most common presenting symptoms were fever and headache. Localizing neurological symptoms including hemiparesis and aphasia, in addition to seizures occurred in 33% of patients and increased LOH significantly (33 versus 15 days, p = 0.03). Epidural (EA) and subdural abscesses (SA) were the most common intracranial complications. 58% of patients were initially treated with a combination of open neurosurgical (ON) intervention and endoscopic sinus surgery (ESS) and LOH was significantly shorter for these patients compared to those treated otherwise (14 versus 31 days, p = 0.02). Streptococcus species were the most common group of bacteria identified in 75% of cases, with S. anginosus accounting for 42% of cases. The overall average LOH was 21 days with 92% of patients having complete resolution of symptoms by time of discharge. CONCLUSIONS:Treatment of intracranial complications of acute rhinosinusitis can have favorable outcomes after appropriate surgical management. Localizing neurologic symptoms and seizures portend longer hospital stay and recovery time. Shorter hospital stay was seen in those undergoing early combined ON and ESS interventions.

Nasal septal perforations can cause issues of epistaxis, whistling, crusting, saddle deformity, and obstruction, which motivate patients to seek surgical repair. Numerous methods of septal perforation repair have been described, with surgical success rates ranging from 52% to 100%, but few studies address situations with concomitant septal deviation. In treating patients with septal perforation and deviation, both issues should be addressed for optimal outcomes. While routine septoplasty involves the removal of septal cartilage, septal perforation repair involves the addition of interposition grafts. The composite chondromucosal septal rotation flap harmoniously combines these seemingly conflicting goals as an effective and efficient technique for septal perforation repair. We present 3 patients successfully treated for their septal perforation and septal deviation concurrently with this technique.