Homozygous deletion of the DSG3 terminal exon associated with acantholytic blistering of the oral and laryngeal mucosa.

We report a novel homozygous 49.6 kb deletion of chromosome 18q12.1 involving the last exon of DSG3 in dizygotic twins with phenotype consistent with acantholytic blistering of the oral and laryngeal mucosa (ABOLM). The twin siblings presented predominantly with friability of the laryngeal and respiratory mucosa. This is only the second report in the literature of this unusual autosomal recessive blistering disorder. The diagnosis explains the mucosal phenotype of a pemphigus-like disorder without evidence of autoimmune dysfunction. The exclusion of an autoimmune basis has management implications. The deletion also involved the DSG2 gene, which is associated with arrhythmogenic right ventricular dysplasia (ARVD). The affected siblings and heterozygous parents do not show any cardiac phenotype at this time. Functional studies would further clarify how deletions resulting in loss of function of DSG3 may cause the reported phenotypes of DSG3-related ABOLM.

Two-Year Outcomes After Pediatric In-Office Tympanostomy Using Lidocaine/Epinephrine Iontophoresis and an Automated Tube Delivery System.

OBJECTIVE: Evaluate 2-year outcomes after lidocaine/epinephrine iontophoresis and tympanostomy using an automated tube delivery system for pediatric tube placement in-office. STUDY DESIGN: Prospective, single-arm. SETTING: Eighteen otolaryngology practices. METHODS: Children age 6 months to 12 years indicated for tympanostomy were enrolled between October 2017 and February 2019. Local anesthesia of the tympanic membrane was achieved via lidocaine/epinephrine iontophoresis and tympanostomy was completed using an automated tube delivery system (the Tula® System). An additional Lead-In cohort of patients underwent tube placement in the operating room (OR) under general anesthesia using only the tube delivery system. Patients were followed for 2 years or until tube extrusion, whichever occurred first. Otoscopy and tympanometry were performed at 3 weeks, and 6, 12, 18, and 24 months. Tube retention, patency, and safety were evaluated. RESULTS: Tubes were placed in-office for 269 patients (449 ears) and in the OR for 68 patients (131 ears) (mean age, 4.5 years). The median and mean times to tube extrusion for the combined OR and In-Office cohorts were 15.82 (95% confidence interval [CI]: 15.41-19.05) and 16.79 (95% CI: 16.16-17.42) months, respectively. Sequelae included ongoing perforation for 1.9% of ears (11/580) and medial tube displacement for 0.2% (1/580) observed at 18 months. Over a mean follow-up of 14.3 months, 30.3% (176/580) of ears had otorrhea and 14.3% (83/580) had occluded tubes. CONCLUSION: In-office pediatric tympanostomy using lidocaine/epinephrine iontophoresis and automated tube delivery results in tube retention within the ranges described for similar grommet-type tubes and complication rates consistent with traditional tube placement in the OR.

Tracheostomy Outcomes in Patients With COVID-19 at a New York City Hospital.

Objective: Tracheostomies have been performed in patients with prolonged intubation due to COVID-19. Understanding outcomes in different populations is crucial to tackle future epidemics. Study Design: Prospective cohort study. Setting: Tertiary academic medical center in New York City. Methods: A prospectively collected database of patients with COVID-19 undergoing open tracheostomy between March 2020 and April 2020 was reviewed. Primary endpoints were weaning from the ventilator and from sedation and time to decannulation. Results: Sixty-six patients underwent tracheostomy. There were 42 males (64%) with an average age of 62 years (range, 23-91). Patients were intubated for a median time of 26 days prior to tracheostomy (interquartile range [IQR], 23-30). The median time to weaning from ventilatory support after tracheostomy was 18 days (IQR, 10-29). Of those sedated at the time of tracheostomy, the median time to discontinuation of sedation was 5 days (IQR, 3-9). Of patients who survived, 39 (69%) were decannulated. Of those decannulated before discharge (n = 39), the median time to decannulation was 36 days (IQR, 27-49) following tracheostomy. The median time from ventilator liberation to decannulation was 14 days (IQR, 8-22). Thirteen patients (20.0%) had minor bleeding requiring packing. Two patients (3%) had bleeding requiring neck exploration. The all-cause mortality rate was 10.6%. No patients died of procedural causes, and no surgeons acquired COVID-19. Conclusion: Open tracheostomies were successfully and safely performed at our institution in the peak of the COVID-19 pandemic. The majority of patients were successfully weaned from the ventilator and sedation. Approximately 60% of patients were decannulated prior to hospital discharge.

The Safety and Efficacy of Tracheostomy in Patients Diagnosed With COVID-19: An Analysis of 143 Patients at a Major NYC Medical Center.

OBJECTIVE: To determine the optimal surgical strategy for performing tracheostomy in COVID-19 patients. BACKGROUND: Many ventilated COVID-19 patients require prolonged ventilation. We do not know if tracheostomy will improve their care. Given the paucity of data on this topic, the optimal surgical approach has yet to be elucidated. METHODS: This is a cohort study of 143 ventilator dependent COVID-19 patients undergoing tracheostomy at an academic medical center from April 15th to May 15th, 2020, with follow up until June 1, 2020. We included adult patients admitted to a NYC medical center with COVID-19 who required invasive mechanical ventilation for greater than 2 weeks who were unable to be extubated and determined to have reasonable chance of recovery and fit defined tracheostomy candidate criteria. Patients underwent either a percutaneous tracheostomy (PT) or open surgical tracheostomy (ST) performed by 1 of 3 surgical services. RESULTS: One hundred forty-three patients underwent tracheostomy, 58 (41%) via a ST, and 85 (59%) via a PT. There were no significant differences in patient characteristics between the 2 groups, except that more patients who had a history of extracorporeal membrane oxygenation underwent PT (11% vs 2%, P = 0.049). There were no statistical differences observed between the PT and ST groups with regard to bleeding complications (3.5%vs 10.3%, P = 0.099), tracheostomy related complications (5.9% vs 8.6%, P = 0.528), inpatient death (12% vs 5%, P = 0.178), discharge from hospital (39% vs 36%, P = 0.751) or surgeon illness (0% vs 0%, P = 1). CONCLUSION AND RELEVANCE: The rapid formation of a multi-disciplinary team allows for the efficient evaluation and performance of a large volume of tracheostomies in a resource-limited setting. Bedside tracheostomy in COVID-19 does not cause additional harm to patients if performed after 2 weeks from intubation. It also seems to be safe for proceduralists to perform in this timeframe. The manner of tracheostomy does not change outcomes significantly if it is performed safely and efficiently.

Lessons learned from safe tracheostomy aftercare taskforce implemented during COVID-19 pandemic.

PURPOSE: At the height of the COVID-19 pandemic, our institution instituted a Safe Tracheostomy Aftercare Taskforce (STAT) team to care for the influx of patients undergoing tracheostomies. This review was undertaken to understand this team's impact on outcomes of tracheostomy care. METHODS: We compared retrospective data collected from patients undergoing tracheostomies at our institution from February to June 2019, prior to creation of the STAT team, to prospectively collected data from tracheostomies performed from February to June 2020, while the STAT team was in place and performed statistical analysis on outcomes of care such as decannulation prior to discharge, timely tube change, and post-discharge follow-up. RESULTS: We found that the STAT team significantly increased rate of decannulation prior to discharge (P < 0.0005), performance of timely trach tube change when indicated (P < 0.05), and rates of follow-up for tracheostomy patients after discharge from the hospital (P < 0.0005). CONCLUSION: The positive impact of the STAT team on outcomes of patient care such as decannulation prior to discharge, timely tube change, and post-discharge follow-up makes a strong case for its continuation even in non-pandemic times.

In-Office Tympanostomy Tube Placement in Children Using Iontophoresis and Automated Tube Delivery.

OBJECTIVES/HYPOTHESIS: Evaluate technical success, tolerability, and safety of lidocaine iontophoresis and tympanostomy tube placement for children in an office setting. STUDY DESIGN: Prospective individual cohort study. METHODS: This prospective multicenter study evaluated in-office tube placement in children ages 6 months through 12 years of age. Anesthesia was achieved via lidocaine/epinephrine iontophoresis. Tube placement was conducted using an integrated and automated myringotomy and tube delivery system. Anxiolytics, sedation, and papoose board were not used. Technical success and safety were evaluated. Patients 5 to 12 years old self-reported tube placement pain using the Faces Pain Scale-Revised (FPS-R) instrument, which ranges from 0 (no pain) to 10 (very much pain). RESULTS: Children were enrolled into three cohorts with 68, 47, and 222 children in the Operating Room (OR) Lead-In, Office Lead-In, and Pivotal cohorts, respectively. In the Pivotal cohort, there were 120 and 102 children in the <5 and 5- to 12-year-old age groups, respectively, with a mean age of 2.3 and 7.6 years, respectively. Bilateral tube placement was indicated for 94.2% of children <5 and 88.2% of children 5 to 12 years old. Tubes were successfully placed in all indicated ears in 85.8% (103/120) of children <5 and 89.2% (91/102) of children 5 to 12 years old. Mean FPS-R score was 3.30 (standard deviation [SD] = 3.39) for tube placement and 1.69 (SD = 2.43) at 5 minutes postprocedure. There were no serious adverse events. Nonserious adverse events occurred at rates similar to standard tympanostomy procedures. CONCLUSIONS: In-office tube placement in selected patients can be successfully achieved without requiring sedatives, anxiolytics, or papoose restraints via lidocaine iontophoresis local anesthesia and an automated myringotomy and tube delivery system. LEVEL OF EVIDENCE: 2b Laryngoscope, 130:S1-S9, 2020.

Congenital lesions of epithelial origin.

Defects of embryologic development give rise to a variety of congenital lesions arising from the epithelium and are among the most common congenital lesions of the head and neck in the pediatric population. This article presents several congenital lesions of epithelial origin, including congenital midline cervical cleft, pilomatrixoma, dermoid, foregut duplication cysts, and preauricular sinuses and pits. In addition, the management of these lesions is reviewed.