Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus

Objectives/Hypothesis: Create a competency-based assessment tool for pediatric tracheotomy. Study Design: Blinded, modified, Delphi consensus process. Methods: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as “keep” or “remove,” and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. Results: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as “keep,” and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. Conclusions: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. Level of Evidence: 5 Laryngoscope, 130:2700–2707, 2020

Novel variants in GNAI3 associated with auriculocondylar syndrome strengthen a common dominant negative effect

Auriculocondylar syndrome is a rare craniofacial disorder comprising core features of micrognathia, condyle dysplasia and question mark ear. Causative variants have been identified in PLCB4, GNAI3 and EDN1, which are predicted to function within the EDN1-EDNRA pathway during early pharyngeal arch patterning. To date, two GNAI3 variants in three families have been reported. Here we report three novel GNAI3 variants, one segregating with affected members in a family previously linked to 1p21.1-q23.3 and two de novo variants in simplex cases. Two variants occur in known functional motifs, the G1 and G4 boxes, and the third variant is one amino acid outside of the G1 box. Structural modeling shows that all five altered GNAI3 residues identified to date cluster in a region involved in GDP/GTP binding. We hypothesize that all GNAI3 variants lead to dominant negative effects.CRANIRAREUniversite Paris Descartes-Sorbonne Paris Cite Pole de Recherche et d'Enseignement SuperieurAgence Nationale de la Recherche (project EvoDevoMut)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)National Health and Medical Research Council of AustraliaUniv São Paulo, Inst Biociencias, Dept Genet & Biol Evolut, Ctr Pesquisas Genoma Humano & Celulas Tronco, BR-05508090 São Paulo, BrazilUniv Paris 05, Sorbonne Paris Cite, INSERM, U1163, Paris, FranceUniv São Paulo, HRCA, Dept Clin Genet, Bauru, BrazilUniv Melbourne, Royal Childrens Hosp, Murdoch Childrens Res Inst, Victorian Clin Genet Serv, Melbourne, Vic, AustraliaUniv Melbourne, Dept Paediat, Melbourne, Vic, AustraliaRoyal Childrens Hosp, Dept Plast & Maxillofacial Surg, Melbourne, Vic, AustraliaHosp Sick Children, Dept Otolaryngol Head & Neck Surg, Toronto, ON M5G 1X8, CanadaUniv São Paulo, Inst Biosci, BR-05508090 São Paulo, BrazilLeiden Univ, Med Ctr, Leiden Genome Technol Ctr, Leiden, NetherlandsUniversidade Federal de São Paulo, Inst Ciencia & Tecnol, Sao Jose Dos Campos, BrazilHop Necker Enfants Malad, AP HP, Dept Genet, Paris, FranceUniversidade Federal de São Paulo, Inst Ciencia & Tecnol, Sao Jose Dos Campos, BrazilUniversite Paris Descartes-Sorbonne Paris Cite Pole de Recherche et d'Enseignement Superieur: SPC/JFG/2013-031National Health and Medical Research Council of Australia: 607431Web of Scienc

Development of an International Odor Identification Test for Children: The Universal Sniff Test

Objective: To assess olfactory function in children and to create and validate an odor identification test to diagnose olfactory dysfunction in children, which we called the Universal Sniff (U-Sniff) test.  Study design: This is a multicenter study involving 19 countries. The U-Sniff test was developed in 3 phases including 1760 children age 5-7 years. Phase 1: identification of potentially recognizable odors; phase 2: selection of odorants for the odor identification test; and phase 3: evaluation of the test and acquisition of normative data. Test—retest reliability was evaluated in a subgroup of children (n = 27), and the test was validated using children with congenital anosmia (n = 14).  Results: Twelve odors were familiar to children and, therefore, included in the U-Sniff test. Children scored a mean ± SD of 9.88 ± 1.80 points out of 12. Normative data was obtained and reported for each country. The U-Sniff test demonstrated a high test—retest reliability (r27 = 0.83, P < .001) and enabled discrimination between normosmia and children with congenital anosmia with a sensitivity of 100% and specificity of 86%.  Conclusions: The U-Sniff is a valid and reliable method of testing olfaction in children and can be used internationally

The effect of dexamethasone on post-tonsillectomy nausea, vomiting and bleeding Efeito da dexametasona sobre sangramento, vômito e náusea pós-amigdalectomia

It has been stated, that the administration of Dexamethasone has an impact on the morbidity following tonsillectomy. OBJECTIVE: To re-calculate the blood values for Dexamethasone when given as fixed doses and to evaluate the effect of Dexamethasone on post-operative nausea, vomiting and bleeding rates following tonsillectomy. MATERIALS AND METHODS: The charts of 272 children (2-15 years) who had undergone tonsillectomy were analyzed. The rates of post-operative nausea, vomiting and bleeding in relation to Dexamethasone were calculated-in general and different doses (0 mg/kg, 0.15 mg/kg). STUDY DESIGN: Retrospective cohort study. RESULTS: Dexamethasone was administered in 121 children (43.7%) according to the preference of the anesthesist (mean dose: 0.2 +/- 0.12 mg/kg; range: 0.04 - 0.62 mg/kg). There was no significant difference in nausea and vomiting (p=0.953) or bleeding (p=0.827) across groups receiving or not receiving Dexamethasone. Stratification into three different groups of Dexamethasone concentration also did not identify a dose-related risk of postoperative nausea or vomiting (p=0.98) or bleeding (p=0.71). CONCLUSION: At least under common non-controlled conditions in the clinic, Dexamethasone does not appear to have an effect on nausea or vomiting or bleeding following tonsillectomy.É conhecido o impacto da administração de dexametasona sobre a morbidade no pós-operatório de amigdalectomia. OBJETIVO: Recalcular os valores séricos para dexametasona quando administrada em doses fixas e avaliar seus efeitos sobre as taxas de náusea, vômito e sangramento no pós-operatório de amigdalectomia. MATERIAIS E MÉTODOS: Analisamos os prontuários de 272 crianças (idades entre 2-15 anos) submetidas a amigdalectomias. As taxas de náusea, vômitos e sangramentos foram calculadas para a dexametasona em geral e em diferentes doses (0 mg/kg; 0,15 mg/ kg). TIPO DE ESTUDO: Coorte retrospectivo. RESULTADOS: A dexametasona foi administrada em 121 crianças (43,7%), baseado na preferência do anestesista (dose média: 0,2 +/- 0,12 mg/kg; variação: 0,04 - 0,62 mg/kg). Não houve diferença significativa em termos de náuseas e vômitos (p=0,953) ou sangramento (p=0,827) entre os grupos de pacientes que receberam e não receberam dexametasona. Mesmo a estratificação em três grupos de diferentes concentrações de dexametasona não identificou risco dose-dependente de náusea ou vômito pós-operatório (p=0,98) ou sangramento (p=0,71). CONCLUSÃO: Pelo menos sob condições não-controladas normais da clínica, a dexametasona parece não ter efeito sobre a incidência de náuseas, vômito ou sangramento após amigdalectomia

Olfactory testing in children using objective tools: comparison of Sniffin’ Sticks and University of Pennsylvania Smell Identification Test (UPSIT)

Abstract Background Detection of olfactory dysfunction is important for fire and food safety. Clinical tests of olfaction have been developed for adults but their use in children has been limited because they were felt to be unreliable in children under six years of age. We therefore administered two olfactory tests to children and compared results across tests. Methods Two olfactory tests (Sniffin’ Sticks and University of Pennsylvania Smell Identification Test (UPSIT)) were administered to 78 healthy children ages 3 to 12 years. Children were randomized to one of two groups: Group 1 performed the UPSIT first and Sniffin’ Sticks second, and Group 2 performed Sniffin’ Sticks first and UPSIT second. Results All children were able to complete both olfactory tests. Performance on both tests was similar for children 5 and 6 years of age. There was an age-dependent increase in score on both tests (p < .01). Children performed better on the Sniffin’ Sticks than the UPSIT (65.3% versus 59.7%, p < .01). There was no difference in performance due to order of test presentation. Conclusions The Sniffin’ Sticks and UPSIT olfactory tests can both be completed by children as young as 5 years of age. Performance on both tests increased with increasing age. Better performance on the Sniffin’ Sticks than the UPSIT may be due to a decreased number of test items, better ability to maintain attention, or decreased olfactory fatigue. The ability to reuse Sniffin’ Sticks on multiple children may make it more practical for clinical use

Attenuating Cardiac Pulsations within the Cochlea: Structure and Function of Tortuous Vessels Feeding Stria Vascularis

The mammalian ear has an extraordinary capacity to detect very low-level acoustic signals from the environment. Sound pressures as low as a few μPa (−10 dB SPL) can activate cochlear hair cells. To achieve this sensitivity, biological noise has to be minimized including that generated by cardiovascular pulsation. Generally, cardiac pressure changes are transmitted to most peripheral capillary beds; however, such signals within the stria vascularis of the cochlea would be highly disruptive. Not least, it would result in a constant auditory sensation of heartbeat. We investigate special adaptations in cochlear vasculature that serve to attenuate cardiac pulse signals. We describe the structure of tortuous arterioles that feed stria vascularis as seen in corrosion casts of the cochlea. We provide a mathematical model to explain the role of this unique vascular anatomy in dampening pulsatile blood flow to the stria vascularis.Peer Reviewe

Corrosion casting of the subglottis following endotracheal tube intubation injury: a pilot study in Yorkshire piglets

Abstract Purpose Subglottic stenosis can result from endotracheal tube injury. The mechanism by which this occurs, however, is not well understood. The purpose of this study was to examine the role of angiogenesis, hypoxia and ischemia in subglottic mucosal injury following endotracheal intubation. Methods Six Yorkshire piglets were randomized to either a control group (N=3, ventilated through laryngeal mask airway for corrosion casting) or accelerated subglottic injury group through intubation and induced hypoxia as per a previously described model (N=3). The vasculature of all animals was injected with liquid methyl methacrylate. After polymerization, the surrounding tissue was corroded with potassium hydroxide. The subglottic region was evaluated using scanning electron microscopy looking for angiogenic and hypoxic or degenerative features and groups were compared using Mann–Whitney tests and Friedman’s 2-way ANOVA. Results Animals in the accelerated subglottic injury group had less overall angiogenic features (P=.002) and more overall hypoxic/degenerative features (P=.000) compared with controls. Amongst angiogenic features, there was decreased budding (P=.000) and a trend toward decreased sprouting (P=.037) in the accelerated subglottic injury group with an increase in intussusception (P=.004), possibly representing early attempts at rapid revascularization. Amongst hypoxic/degenerative features, extravasation was the only feature that was significantly higher in the accelerated subglottic injury group (P=.000). Conclusions Subglottic injury due to intubation and hypoxia may lead to decreased angiogenesis and increased blood vessel damage resulting in extravasation of fluid and a decreased propensity toward wound healing in this animal model

Two congenital cases of pigmented epithelioid melanocytoma with unique clinical and genetic features

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