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PURPOSE: PIK3CA-related overgrowth spectrum (PROS) conditions of the head and neck are treatment challenges. Traditionally, these conditions require multiple invasive interventions, with incomplete malformation removal, disfigurement, and possible dysfunction. Use of the PI3K inhibitor alpelisib, previously shown to be effective in PROS, has not been reported in PIK3CA-associated head and neck lymphatic malformations (HNLMs) or facial infiltrating lipomatosis (FIL). We describe prospective treatment of 5 children with PIK3CA-associated HNLMs or head and neck FIL with alpelisib monotherapy. METHODS: A total of 5 children with PIK3CA-associated HNLMs (n = 4) or FIL (n = 1) received alpelisib monotherapy (aged 2-12 years). Treatment response was determined by parental report, clinical evaluation, diary/questionnaire, and standardized clinical photography, measuring facial volume through 3-dimensional photos and magnetic resonance imaging. RESULTS: All participants had reduction in the size of lesion, and all had improvement or resolution of malformation inflammation/pain/bleeding. Common invasive therapy was avoided (ie, tracheotomy). After 6 or more months of alpelisib therapy, facial volume was reduced (range 1%-20%) and magnetic resonance imaging anomaly volume (range 0%-23%) were reduced, and there was improvement in swallowing, upper airway patency, and speech clarity. CONCLUSION: Individuals with head and neck PROS treated with alpelisib had decreased malformation size and locoregional overgrowth, improved function and symptoms, and fewer invasive procedures.
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Fosfatidilinositol 3-Quinasas , Tiazoles , Niño , Humanos , Fosfatidilinositol 3-Quinasas/genética , Mutación , Fosfatidilinositol 3-Quinasa Clase I/genética , Tiazoles/uso terapéuticoRESUMEN
Pyruvate dehydrogenase complex deficiency (PDCD) is a rare X-linked disorder that affects glucose metabolism. There are several case reports describing the anesthetic management of patients with PDCD. This is the first case report to describe the anesthetic management of a PDCD patient requiring spontaneous ventilation using dexmedetomidine and ketamine.
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Anemia Hemolítica Congénita no Esferocítica , Anestesia , Anestésicos , Dexmedetomidina , Ketamina , Niño , Humanos , Piruvato Quinasa/deficiencia , Errores Innatos del Metabolismo del PiruvatoRESUMEN
OBJECTIVES: The applications of using ultrasound for the evaluation and management of otolaryngologic diagnoses are expanding. The purpose of this study was to evaluate the current experience of ultrasound training in otolaryngology residency programs. METHODS: All allopathic and osteopathic otolaryngology residency programs in the United States were surveyed online via an e-mailed survey link to the resident representatives of the Section for Residents and Fellows in Training of the American Academy of Otolaryngology-Head and Neck Surgery. We present a descriptive analysis of the survey results. RESULTS: A total of 110 responses were obtained from resident representatives at MD and DO otolaryngology residency programs, representing a response rate of 94.8%. Forty-four percent of residents reported that they would not feel comfortable with performing ultrasound-guided procedures after residency; 43% reported that they do not perform ultrasound procedures as a part of their residency training; and 60% of those trainees performing ultrasound procedures do not log the procedures. Twenty-three percent of residents did not have access to an ultrasound machine. Most respondents (71%) desired more exposure to diagnostic and/or interventional ultrasound training during residency. CONCLUSIONS: Although current experience is variable, there is a strong interest in increasing resident skill acquisition in ultrasound training among otolaryngology residents. Some barriers to these goals may be a lack of trained faculty members using ultrasound and insufficient recording mechanisms for residents performing ultrasound procedures.
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Competencia Clínica/estadística & datos numéricos , Internado y Residencia , Otolaringología/educación , Ultrasonido/educación , Humanos , Ultrasonografía , Estados UnidosRESUMEN
BACKGROUND: Gaps between evidence-based research and clinical-public health practice have been evident for decades. One of the aims of medical student research is to close this gap. Accordingly, evaluating individual and environmental factors that influence participation of medical students in research are needed to understand and identify potential targets for action. This study aims to identify characteristics of medical student researchers in Colombia and the associated factors with scientific publications. METHODS: A cross-sectional study of Colombian medical students involved in research using a validated, self-administered, online survey. The survey was distributed through the Colombian Association of Medical Students' Associations (ASCEMCOL). Data sets were analyzed using descriptive and summary statistics. Bivariate analysis and a multiple logistic regression model were conducted to identify predictors of scientific publications. RESULTS: A total of 133 responses were analyzed from students at 12 Colombian cities and 20 higher-education institutions. Although 94% of responders had at least one research proposal, only 57% had completed a project, and 17% had published their findings. Barriers for undertaking research included time restrictions and a lack of mentorship. Motivational factors included opportunity to publish findings and good mentorship. Students planning to do a specialization (OR = 3.25; 95% Confidence interval [CI] = 1.27-8.30), innovators (OR = 3.52; 95%CI = 1.30-9.52) and committed (OR = 3.39; 95%CI = 1.02-11.29), those who had previously published their findings (OR 9.13 IC95% 2.57-32.48), and were further in their medical education (OR 2.26 IC95% 1.01-5.07), were more likely to publish scientific papers. CONCLUSIONS: Our findings describe medical students understanding of the process of conducting research in Colombia. Although there appears to be motivation to participate in research, very few students achieve publication. Barriers such as time constraints and mentorship seem to play a critical role. This highlights opportunities where barriers to research can be overcome in medical school and other levels.
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Investigación Biomédica/estadística & datos numéricos , Internado y Residencia , Publicaciones Periódicas como Asunto , Investigadores/educación , Facultades de Medicina , Investigación Biomédica/educación , Colombia/epidemiología , Estudios Transversales , Medicina Basada en la Evidencia , Femenino , Conocimientos, Actitudes y Práctica en Salud , Humanos , Masculino , Mentores , Guías de Práctica Clínica como Asunto , Estudiantes de Medicina , Adulto JovenRESUMEN
OBJECTIVE: The objective of this study was to develop and assess multidisciplinary advanced surgical planning (ASP) sessions using three dimensional (3D) printed models for cervicothoracic slide tracheoplasty (CST). We hypothesized that these sessions would improve surgeon confidence, streamline intraoperative planning, and highlight the utility of 3D modeling. METHODS: 3D-printed patient-specific trachea models were used in pre-operative ASP sessions consisting of a multidisciplinary case discussion and hands-on slide tracheoplasty simulation. Participants completed a survey rating realism, utility, impact on the final surgical plan, and pre- and post-session confidence. Statistical analysis was performed via Wilcoxon and Kruskal-Wallis tests. RESULTS: Forty-eight surveys were collected across nine sessions and 27 different physicians. On a 5-point Likert scale, models were rated as "very realistic", "very useful" (both median of 4, IQR 3-4 and 4-5, respectively). Overall confidence increased by 1.4 points (+/- 0.7, p < 0.0001), with the largest change seen in those with minimal prior slide tracheoplasty experience (p = 0.005). Participants felt that the sessions "strongly" impacted their surgical plan or anticipated performance (median 4, IQR 4-5), regardless of training level or experience. CONCLUSION: 3D-printed patient-specific models were successfully implemented in ASP sessions for CST. Models were deemed very realistic and very useful by surgeons across multiple specialties and training levels. Surgical planning sessions also strongly impacted the final surgical plan and increased surgeon confidence for CST. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3395-3401, 2024.
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Modelos Anatómicos , Impresión Tridimensional , Tráquea , Humanos , Tráquea/cirugía , Procedimientos de Cirugía Plástica/métodosRESUMEN
OBJECTIVE: Families preferring to receive care in a language-other-than-English have disparities in access to care. We studied the effect of implementing an ambulatory outbound scheduling team on the timeliness of scheduling referrals to pediatric otolaryngology. We hypothesized this intervention could increase access to care. STUDY DESIGN: Retrospective cohort analysis. SETTING: Tertiary care academic center. METHODS: Data were abstracted from the hospital's enterprise database for patients referred to Otolaryngology over 3 years (October 2019-August 2022; 7675 referrals). An outbound scheduling team was created April 2021 and tasked with calling out to schedule referrals within one business day of receipt. Referral lag was compared across patient cohorts before and after the scheduling intervention. Log-transformed linear regression models were used to assess the impact of the scheduling intervention on referral lag for language cohorts. RESULTS: The median preintervention referral lag was 6 days (interquartile range [IQR] 2-18), which was reduced to 1 day postintervention (IQR 0-5; P < .001). Preintervention language-other-than-English families had a median referral lag of 8 days (IQR 2-23), which was 1.27 times higher than for patients speaking English (P < .001). With implementation of the scheduling intervention, language-other-than-English families were scheduled in a median of 1 day (IQR 0-6), and the disparity in timeliness of scheduling was eliminated (P = .131). Postintervention, referral lag was reduced by 58% in the English and 64% in the language other than English cohorts. CONCLUSION: Implementation of an outbound ambulatory scheduling process reduces referral lag for all patients and eliminated a disparity in referral lag for language-other-than-English families.
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Citas y Horarios , Otolaringología , Derivación y Consulta , Humanos , Derivación y Consulta/organización & administración , Estudios Retrospectivos , Otolaringología/organización & administración , Niño , Factores de Tiempo , Femenino , Accesibilidad a los Servicios de Salud/organización & administración , Masculino , Preescolar , LenguajeRESUMEN
BACKGROUND: Sinonasal neoplasms, whether benign and malignant, pose a significant challenge to clinicians and represent a model area for multidisciplinary collaboration in order to optimize patient care. The International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors (ICSNT) aims to summarize the best available evidence and presents 48 thematic and histopathology-based topics spanning the field. METHODS: In accordance with prior International Consensus Statement on Allergy and Rhinology documents, ICSNT assigned each topic as an Evidence-Based Review with Recommendations, Evidence-Based Review, and Literature Review based on the level of evidence. An international group of multidisciplinary author teams were assembled for the topic reviews using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format, and completed sections underwent a thorough and iterative consensus-building process. The final document underwent rigorous synthesis and review prior to publication. RESULTS: The ICSNT document consists of four major sections: general principles, benign neoplasms and lesions, malignant neoplasms, and quality of life and surveillance. It covers 48 conceptual and/or histopathology-based topics relevant to sinonasal neoplasms and masses. Topics with a high level of evidence provided specific recommendations, while other areas summarized the current state of evidence. A final section highlights research opportunities and future directions, contributing to advancing knowledge and community intervention. CONCLUSION: As an embodiment of the multidisciplinary and collaborative model of care in sinonasal neoplasms and masses, ICSNT was designed as a comprehensive, international, and multidisciplinary collaborative endeavor. Its primary objective is to summarize the existing evidence in the field of sinonasal neoplasms and masses.
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Neoplasias de Cabeza y Cuello , Hipersensibilidad , Neoplasias de los Senos Paranasales , Humanos , Calidad de Vida , Neoplasias de los Senos Paranasales/terapia , Neoplasias de los Senos Paranasales/patologíaRESUMEN
Heterochromatin protein 1 (HP1) proteins are "gatekeepers" of epigenetic gene silencing that is mediated by lysine 9 of histone H3 methylation (H3K9me). Current knowledge supports a paradigm whereby HP1 proteins achieve repression by binding to H3K9me marks and interacting to H3K9 histone methyltransferases (HMTs), such as SUV39H1, which methylate this residue on adjacent nucleosomes thereby compacting chromatin and silencing gene expression. However, the mechanism underlying the recruitment of this epigenetic regulator to target gene promoters remains poorly characterized. In the current study, we reveal for the first time a mechanism whereby HP1 is recruited to promoters by a well characterized Krüppel-like transcription factor (KLF), in a sequence-specific manner, to mediate complex biological phenomena. A PXVXL HP1-interacting domain identified at position 487-491 of KLF11 mediates the binding of HP1α and KLF11 in vitro and in cultured cells. KLF11 also recruits HP1α and its histone methyltransferase, SUV39H1, to promoters to limit KLF11-mediated gene activation. Indeed, a KLF11ΔHP1 mutant derepresses KLF11-regulated cancer genes, by inhibiting HP1-SUV39H1 recruitment, decreasing H3K9me3, while increasing activation-associated marks. Biologically, impairment of KLF11-mediated HP1-HMT recruitment abolishes tumor suppression, providing direct evidence that HP1-HMTs act in a sequence-specific manner to achieve this function rather than its well characterized binding to methylated chromatin without intermediary. Collectively, these studies reveal a novel role for HP1 as a cofactor in tumor suppression, expand our mechanistic understanding of a KLF associated to human disease, and outline cellular and biochemical mechanisms underlying this phenomenon, increasing the specificity of targeting HP1-HMT complexes to gene promoters.
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Proteínas de Ciclo Celular/metabolismo , Proteínas Cromosómicas no Histona/metabolismo , Regulación Neoplásica de la Expresión Génica/fisiología , Genes Supresores de Tumor/fisiología , Enfermedades Metabólicas/genética , Neoplasias/genética , Proteínas Represoras/metabolismo , Animales , Proteínas Reguladoras de la Apoptosis , Células CHO , Muerte Celular/fisiología , División Celular/fisiología , Línea Celular Tumoral , Senescencia Celular/fisiología , Cromatina/fisiología , Homólogo de la Proteína Chromobox 5 , Cricetinae , Femenino , Humanos , Enfermedades Metabólicas/metabolismo , Enfermedades Metabólicas/patología , Ratones , Ratones Desnudos , Trasplante de Neoplasias , Neoplasias/metabolismo , Neoplasias/patología , Regiones Promotoras Genéticas/fisiología , Receptores CXCR4/genética , Transcripción Genética/fisiología , Trasplante HeterólogoRESUMEN
INTRODUCTION: LatinX populations are rapidly growing in the USA, but still report lower levels of patient centered care and satisfaction when compared to their non-LatinX white counterparts. This review encompasses literature which describes patient experience instruments that (1) evaluate LatinX experience, (2) have validated Spanish versions, or (3) measure language-concordant care experiences. METHODS: A scoping review of literature in Ovid Medline, CINAHL, and PsycINFO was conducted. Articles were excluded if they were not applicable to the health care industry, did not include a patient experience instrument, or did not include LatinX or Spanish-speaking individuals within their study population. Data extraction was performed for concepts measured, study size, population, health care setting, and languages validated. RESULTS: This review identified 224 manuscripts. Of these, 81 met full inclusion criteria and represented 60 unique instruments. These covered six categories: general patient experience (43%, n = 26/60), experiences of discrimination/mistrust (12%, n = 7/60), cultural factors (10%, n = 6/60), patient-provider relationship (10%, n = 6/60), and communication (8%, n = 5/60). The remaining instruments measured multiple categories (17%, n = 10/60). Just over one third of instruments (n = 24, 5 pediatric, 19 adult) were validated in Spanish and an additional 14 (23%) were validated in English alone. Finally, 4 (7%) instruments were identified which were developed for use in a language concordant setting. CONCLUSION: Many instruments were identified which evaluate LatinX patient experience; however, none was both validated in Spanish and measured in all key categories of experience described above. Additionally, few instruments were developed for holistic evaluation of patient experience in pediatric or language concordant care settings.
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Comunicación , Accesibilidad a los Servicios de Salud , Hispánicos o Latinos , Lenguaje , Satisfacción del Paciente , Atención Dirigida al Paciente , Adulto , Niño , Humanos , Hispánicos o Latinos/psicología , Hispánicos o Latinos/estadística & datos numéricos , Satisfacción del Paciente/etnología , Satisfacción del Paciente/estadística & datos numéricos , Atención Dirigida al Paciente/estadística & datos numéricos , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Estados Unidos/epidemiologíaRESUMEN
OBJECTIVES: Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number. MATERIALS: Consecutive HNLM patients (n = 199) between 2010 and 2017, aged 0-18 years. METHODS: ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ2 ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT). RESULTS: Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p < 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR = 0.09, 95% CI 0.02-0.36, p < 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR = 0.47, 95% CI 0.33-0.66, p < 0.001). CONCLUSION: Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:956-962, 2023.
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Cabeza , Anomalías Linfáticas , Humanos , Masculino , Lactante , Femenino , Cuello , Anomalías Linfáticas/cirugía , Resultado del Tratamiento , Escleroterapia/métodosRESUMEN
We sought to assess the feasibility of virtually assisted personalized tracheostomy tube (vapTT) implementation for patients with congenital airway anomalies (CAAs) and persistent tracheostomy tube (TT)-related respiratory failure at a tertiary pediatric hospital. Three patients (0-18 years) with CAAs and recurrent TT-related respiratory complications were managed with vapTT over 5 years. Patients underwent airway computed tomography acquisition with 3-dimensional reconstruction and TT virtual modeling for shape customization. Models were transferred to Bivona for fabrication based on industry-standard materials and processes. Clinical information and tracheoscopies assessing position, obstruction, and granulation were reviewed. Patients demonstrated resolution of visualized TT-related obstruction, granulation, or ulceration and de-escalation of respiratory support. Clinical events requiring urgent tracheoscopy decreased in all 3 patients. Sufficient relief of critical airway obstruction allowed progression of medical care and/or discharge. VapTTs are feasible for patients with CAA. This new frontier in personalized devices may serve uniquely challenging patient populations for whom standard treatments have failed.
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Obstrucción de las Vías Aéreas , Traqueostomía , Humanos , Niño , Traqueostomía/métodos , Obstrucción de las Vías Aéreas/etiología , Tomografía Computarizada por Rayos X , Complicaciones Posoperatorias/cirugía , Estudios RetrospectivosRESUMEN
BACKGROUND: Recent studies have described the use of telehealth for pediatric surgical care during the COVID-19 pandemic. We aimed to evaluate equity in telehealth use by comparing rates of utilization and satisfaction with pediatric surgical telemedicine among Hispanic patients. METHODS: We conducted a retrospective cohort study of patients seen by a surgical subspecialty provider in the outpatient setting at a quaternary pediatric hospital between April 1 and June 30, 2020. Patients evaluated in the same three-month period in 2019 were analyzed as a historic control. Differences in Family Experience Survey (FES) responses based on race and ethnicity and preferred language of care were assessed using univariable and multivariable generalized linear modeling. RESULTS: The pandemic cohort included fewer patients of Hispanic ethnicity and fewer Spanish-speakers. After controlling for visit type, comparison of Spanish-speaking and English-speaking patients revealed that Spanish-speaking families had significantly lower scores for FES items that evaluated healthcare provider explaining (IRR 0.74, 95% CI: 0.61-0.90), listening (IRR 0.76, 95% CI: 0.63-0.92), and time spent with the family (IRR 0.73, 95% CI: 0.60-0.89). There were no differences in FES responses based on insurance status or degree of medical complexity. CONCLUSIONS: Telehealth services were less commonly used among Hispanic and Spanish-speaking patients. Language may differentially affect family satisfaction with healthcare and telehealth solutions. Strategies to mitigate these inequities are needed and may include strengthening interpreter services and providing language-concordant care. LEVEL OF EVIDENCE: Level IV.
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COVID-19 , Telemedicina , Niño , Humanos , Satisfacción del Paciente , Pandemias , Estudios Retrospectivos , Hispánicos o LatinosRESUMEN
Management of tongue venous malformations can be challenging in the pediatric population due to their heterogeneity in presentation, extent of involvement and functional compromise. It is important to recognize the value of various treatment options in order to guide management of each patient in an individualized fashion. Here we describe a series of patients with tongue venous malformations that are managed using diverse modalities to illustrate the relative benefits and risks of each technique. The challenges of venous malformation treatment can be mitigated by tailoring the approach to each individual patient and malformation. This case series also emphasizes the need and importance of working in the setting of a multidisciplinary vascular anomalies team.
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Embolización Terapéutica , Malformaciones Vasculares , Niño , Humanos , Embolización Terapéutica/métodos , Escleroterapia/métodos , Lengua , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/terapia , Venas/anomalíasRESUMEN
Analysis of premalignant tissue has identified the typical order of somatic events leading to invasive tumors in several cancer types. For other cancers, premalignant tissue is unobtainable, leaving genetic progression unknown. Here, we demonstrate how to infer progression from exome sequencing of primary tumors. Our computational method, PhylogicNDT, recapitulated the previous experimentally determined genetic progression of human papillomavirus-negative (HPV-) head and neck squamous cell carcinoma (HNSCC). We then evaluated HPV+ HNSCC, which lacks premalignant tissue, and uncovered its previously unknown progression, identifying early drivers. We converted relative timing estimates of driver mutations and HPV integration to years before diagnosis based on a clock-like mutational signature. We associated the timing of transitions to aneuploidy with increased intratumor genetic heterogeneity and shorter overall survival. Our approach can establish previously unknown early genetic progression of cancers with unobtainable premalignant tissue, supporting development of experimental models and methods for early detection, interception and prognostication.
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Carcinoma de Células Escamosas , Neoplasias de Cabeza y Cuello , Infecciones por Papillomavirus , Lesiones Precancerosas , Humanos , Carcinoma de Células Escamosas de Cabeza y Cuello/genética , Carcinoma de Células Escamosas/genética , Carcinoma de Células Escamosas/patología , Neoplasias de Cabeza y Cuello/genética , Lesiones Precancerosas/genéticaRESUMEN
OBJECTIVES: Patients born with bilateral head and neck lymphatic malformations (BHNLMs) often require multiple invasive treatments, including tracheostomy. We hypothesized that primary targeted medical therapy (pTMT) with diagnostic needle aspiration reduces the need for invasive therapy such as surgical resection and/or sclerotherapy. METHODS: Retrospective case review was performed of infants with BHNLMs (Grade 2 or De Serres stage IV and V) treated only at our institution from 2000 to 2021. Patients were divided into two cohorts: those managed with pTMT and those managed with observation, sclerotherapy, or surgical intervention (non-pTMT). Data regarding interventions, clinical outcomes, morbidity, and mortality were analyzed with descriptive statistics. RESULTS: Nine children with BHNLMs met inclusion criteria. Three (33%) were in the pTMT cohort and six (66%) were non-pTMT. Eight (89%) malformations were genotyped, and all demonstrated hotspot PIK3CA variants. All pTMT patients had sirolimus initiated in the first month of life and underwent needle aspiration of malformation cyst fluid for cell-free DNA samples. All pTMT patients tolerated medical therapy. For the non-pTMT cohort, primary treatment included none (deceased, n = 1, 17%), observation with needle aspiration (n = 1, 17%), surgical resection (n = 2, 33%), or combination surgery and sclerotherapy (n = 2, 33%). Intubation duration, intensive care and initial hospital length of stay were not different between cohorts. Four non-pTMT patients (67%) required tracheostomy, and two (33%) died prior to discharge. All pTMT patients survived and none required tracheostomy. Non-pTMT patients required a median of two invasive therapies prior to discharge (IQR 1-4) and a mean total of 13 over the course of their lifetime (IQR 1-16), compared to the pTMT group who did not require any lifetime invasive therapy, even after initial pTMT and discharge home. CONCLUSION: This study compares patients with BHNLMs (Grade 2) treated with pTMT versus those treated with observation or invasive therapy. Patients treated with pTMT required no surgical or invasive procedural treatment of their malformations, no tracheostomy placement, no unplanned readmissions after discharge, and had no mortalities. Needle aspiration was useful as a therapeutic adjunct for cell-free DNA diagnosis of PIK3CA variants, which guided TMT.
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Cabeza , Anomalías Linfáticas , Niño , Lactante , Humanos , Estudios Retrospectivos , Resultado del Tratamiento , Cuello , Anomalías Linfáticas/cirugía , EscleroterapiaRESUMEN
Despite society recommendations that cross-sectional imaging be obtained following removal of ingested button batteries, there is no published consensus on how it effectively guides clinical management. This single institution survey demonstrates a lack of uniformity by clinicians regarding which imaging findings impact management decisions, highlighting the need for further guidelines.
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Cuerpos Extraños , Niño , Suministros de Energía Eléctrica/efectos adversos , Esófago/diagnóstico por imagen , Cuerpos Extraños/diagnóstico por imagen , Cuerpos Extraños/cirugía , Humanos , Imagen por Resonancia Magnética , Encuestas y CuestionariosRESUMEN
OBJECTIVES/HYPOTHESIS: We hypothesized that the use of three-dimensional (3D) printed tracheal models to reproducibly simulate surgical technique variations in slide tracheoplasty would demonstrate the quantitative impact of surgical variables on postoperative tracheal dimensions. STUDY DESIGN: Prospective analysis of three-dimensional printed surgical simulation models. METHODS: Slide tracheoplasty was performed on 3D printed long segment tracheal stenosis models with combinations of tracheal transection incision angle (90°, 45° beveled superior to inferior, 45° beveled inferior to superior) and tracheal transection location relative to the stenosis (at midpoint, 2 mm each superior and inferior to midpoint). Postoperative computed tomography (CT) scans measured changes in tracheal length, volume, and cross-sectional area compared to controls. Statistical analysis was performed using one-way analysis of variance and unpaired two-tailed t-tests. RESULTS: Slide tracheoplasty yielded 27 reconstructed tracheas. On average, slide tracheoplasty reduced total tracheal length by 36%. Beveled tracheal incisions yielded 9.5% longer final tracheas than straight transection incisions (P < .0001). Cross-sectional area at the stenosis midpoint increased from 9.0 mm2 to 45 mm2 but did not vary with technique (P > .05). Total tracheal luminal volume increased from 900 mm3 to 1378 mm3 overall and was largest with beveled incisions (P = .03). More material was discarded with straight incisions compared to beveled (89 mg vs. 19 mg, P < .0001). CONCLUSIONS: Beveled tracheal transection incisions resulted in increased tracheal length, longer anastomotic segments, increased volume, and reduced tissue waste as compared to straight incisions. Offsetting the incision from the midpoint of stenosis did not significantly affect reconstructed tracheal morphology. Using 3D printed models for surgical simulation can be helpful for the quantitative study of the effect isolated surgical variables on technical outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1306-1312, 2022.
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Procedimientos de Cirugía Plástica , Herida Quirúrgica , Estenosis Traqueal , Constricción Patológica/cirugía , Humanos , Lactante , Procedimientos de Cirugía Plástica/métodos , Estudios Retrospectivos , Herida Quirúrgica/cirugía , Tráquea/diagnóstico por imagen , Tráquea/cirugía , Estenosis Traqueal/diagnóstico por imagen , Estenosis Traqueal/cirugía , Resultado del TratamientoRESUMEN
OBJECTIVE: Suprastomal collapse and granulation are common sequelae of pediatric tracheostomy. We present the first measure of suprastomal obstructive pathology, the Seattle Suprastomal Safety Score (5S), an instrument with 2 domains: collapse and granulation. STUDY DESIGN: Cross-sectional repeated testing survey. SETTING: Electronic survey. METHODS: A library of images was assembled from still pictures of the suprastomal area in 50 patients who previously underwent trachea-bronchoscopy at a quaternary children's hospital. Five pediatric otolaryngologists and 2 pediatric pulmonologists reviewed the images in random, blinded fashion and provided 5S scores. Participants repeated this process 2 to 4 weeks later. Interrater agreement was calculated with an intraclass correlation coefficient (ICC) with a 2-way random-effects model and Fleiss's κ. Intrarater agreement was measured with an ICC using a 2-way mixed-effects model as well as with test-retest correlations using Spearman rank coefficient. All measures were performed separately on collapse and granulation domains. RESULTS: ICC for interrater agreement was 0.88 (95% CI, 0.82-0.93) for collapse and 0.97 (95% CI, 0.96-0.98) for granulation, indicating almost perfect agreement. Fleiss's κ demonstrated moderate agreement for collapse and almost perfect agreement for granulation. ICC for intrarater agreement was 0.95 (95% CI, 0.93-0.97) and 0.99 (95% CI, 0.98-0.99) for collapse and granulation, respectively, indicating almost perfect agreement. Spearman rank correlation for test-retest demonstrated substantial agreement for collapse and almost perfect agreement for granulation. CONCLUSION: The 5S demonstrates excellent interrater and intrarater agreement, making it highly reliable as a novel measure of suprastomal collapse and granulation in tracheostomy-dependent pediatric patients.
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Tráquea , Traqueostomía , Broncoscopía , Niño , Estudios Transversales , Humanos , Reproducibilidad de los Resultados , Estudios Retrospectivos , Tráquea/cirugía , Traqueostomía/métodosRESUMEN
Describe a novel use for a kinase inhibitor, imatinib, in young children with a known activated somatic mutation in PDGFR-beta. Two patients with infantile myofibromatosis treated with imatinib. Case description of evaluation, diagnosis and treatment decisions for infantile myfibromatosis of the head and neck. Description of medical therapy for infantile myofibromatosis in these patients. For function threatening myofibromas of a known genotype, in infants, targeted medical therapy is a treatment option.
RESUMEN
BACKGROUND: Enhancing patient outcomes in an array of surgical procedures in the head and neck requires the maintenance of complex regional functions through the protection of cranial nerve integrity. This review and consensus statement cover the scope of cranial nerve monitoring of all cranial nerves that are of practical importance in head, neck, and endocrine surgery except for cranial nerves VII and VIII within the temporal bone. Complete and applied understanding of neurophysiologic principles facilitates the surgeon's ability to monitor the at-risk nerve. METHODS: The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) identified the need for a consensus statement on cranial nerve monitoring. An AAO-HNS task force was created through soliciting experts on the subject. Relevant domains were identified, including residency education, neurophysiology, application, and various techniques for monitoring pertinent cranial nerves. A document was generated to incorporate and consolidate these domains. The panel used a modified Delphi method for consensus generation. RESULTS: Consensus was achieved in the domains of education needs and anesthesia considerations, as well as setup, troubleshooting, and documentation. Specific cranial nerve monitoring was evaluated and reached consensus for all cranial nerves in statement 4 with the exception of the spinal accessory nerve. Although the spinal accessory nerve's value can never be marginalized, the task force did not feel that the existing literature was as robust to support a recommendation of routine monitoring of this nerve. In contrast, there is robust supporting literature cited and consensus for routine monitoring in certain procedures, such as thyroid surgery, to optimize patient outcomes. CONCLUSIONS: The AAO-HNS Cranial Nerve Monitoring Task Force has provided a state-of-the-art review in neural monitoring in otolaryngologic head, neck, and endocrine surgery. The evidence-based review was complemented by consensus statements utilizing a modified Delphi method to prioritize key statements to enhance patient outcomes in an array of surgical procedures in the head and neck. A precise definition of what actually constitutes intraoperative nerve monitoring and its benefits have been provided.