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Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.

Introduction: Flexible nasolaryngoscopy (FNL) is a common, uncomfortable procedure performed to assess the upper airway in infants. Oral sucrose is used during various painful procedures in infants but has not been used during FNL. Our objective was to understand the impact of oral sucrose on discomfort in infants undergoing FNL. Methods: Infants (<12‐months‐old) undergoing FNL in the otolaryngology clinic were randomized to treatment (0.5 mL 24% oral sucrose) or standard management (no sucrose). Sucrose was administered <2 min prior to FNL performed by a single endoscopist. Outcome measures included: EVENDOL pain scale and cry duration and visit duration. Infant discomfort was measured by a second observer who was blinded to treatment group. Results: Forty‐seven infants were included, 23 were treated with sucrose and 24 with standard management. The median (IQR) age was 3.0 (2–5.7) months. There were no significant differences in age, weight, or sex across groups. The median (IQR) duration of FNL was 35.2 (26.5–58.4) and 36.4 (28.9–51.8) seconds for treatment and standard management groups, respectively. Mean (SD) EVENDOL scores were significantly lower in the sucrose group [4.9 (2.0)] than standard group (6.7 [2.1]) (p = 0.003). Mean cry duration after FNL was significantly shorter in the sucrose group (29.9 [20.4] seconds) than the standard group (52.7.0 [40.6] seconds) (p = 0.02). Median (IQR) visit duration did not differ across groups (1.1 [0.9–1.3] vs. 1.1 [0.7–1.4] h [p = 0.15]). Conclusion: Oral sucrose given before FNL reduced EVENDOL scores and cry duration after FNL and did not prolong clinic visits in this randomized pilot study. Level of Evidence: 2 Laryngoscope, 134:3826–3831, 2024 [ABSTRACT FROM AUTHOR]

Objective: To achieve consensus on critical steps and create an assessment tool for actual and simulated pediatric tracheostomy emergencies that incorporates human and systems factors along with tracheostomy‐specific steps. Methods: A modified Delphi method was used. Using REDCap software, an instrument comprising 29 potential items was circulated to 171 tracheostomy and simulation experts. Consensus criteria were determined a priori with a goal of consolidating and ordering 15 to 25 final items. In the first round, items were rated as "keep" or "remove". In the second and third rounds, experts were asked to rate the importance of each item on a 9‐point Likert scale. Items were refined in subsequent iterations based on analysis of results and respondents' comments. Results: The response rates were 125/171 (73.1%) for the first round, 111/125 (88.8%) for the second round, and 109/125 (87.2%) for the third round. 133 comments were incorporated. Consensus (>60% participants scoring ≥8, or mean score >7.5) was reached on 22 items distributed across three domains. There were 12, 4, and 6 items in the domains of tracheostomy‐specific steps, team and personnel factors, and equipment respectively. Conclusions: The resultant assessment tool can be used to assess both tracheostomy‐specific steps as well as systems factors affecting hospital team response to simulated and clinical pediatric tracheostomy emergencies. The tool can also be used to guide debriefing discussions of both simulated and clinical emergencies, and to spur quality improvement initiatives. Level of Evidence: 5 Laryngoscope, 133:3588–3601, 2023 [ABSTRACT FROM AUTHOR]

Background: Complete and accurate documentation of surgical procedures is essential for optimizing patient care, yet significant variation in operative notes persists within and across institutions. We sought to reach consensus on the most important components of an operative note for pediatric microlaryngoscopy and bronchoscopy. Methods: A modified Delphi consensus process was used. A checklist for operative documentation, created by fellowship‐trained pediatric otolaryngologists‐head and neck surgeons, was sent to surgeons identified as experts in pediatric laryngoscopy and bronchoscopy. In the first round, items were rated as "keep" or "remove". In the second round, each item was rated on a 7‐point Likert scale for importance. The mean score of each item was calculated to determine if consensus was reached. Results: Overall, 43/74 (58.1%) surgeons responded to our survey. After two rounds of editing, 28 components reached consensus, 24 were near consensus, and 26 did not reach consensus. Items that reached final consensus had mean (SD) ratings of 6.12 (0.94) (range, 5.31–6.72). Conclusion: Pediatric otolaryngologists identified as bronchoscopy experts were able to create a checklist of essential components of an operative note for pediatric laryngoscopy and bronchoscopy using a Delphi method. Items reaching consensus included procedure name, description of breathing, grade of airway view, description of normal anatomic structures, grade of subglottic stenosis if present, presence and description of tracheobronchomalacia, presence of fistulae, cleft and rings, and several special cases including foreign body and tracheostomy management, as well as end of procedure disposition and complications. Level of Evidence: 5 Laryngoscope, 133:1234–1238, 2023 [ABSTRACT FROM AUTHOR]