Colesteatoma congénito: caso clínico y revisión de literatura / Congenital cholesteatoma: clinical case and literature review

El colesteatoma congénito (CC) es una lesión benigna de epitelio escamoso queratinizado que puede afectar diferentes aéreas del hueso temporal con predominio en el oído medio. El CC es una patología poco frecuente que se presenta en pacientes pediátricos y clínicamente se manifiesta como una lesión blanquecina detrás de un tímpano indemne. La mayoría de los pacientes no presenta historia de hipoacusia, otorrea, infección, perforación o cirugía otológica previa. Se analiza el caso de un prescolar con CC que consultó con trastorno de sueño sin sintomatología otológica, pero con otoscopía alterada como hallazgo clínico. En la resonancia magnética nuclear (RMN) con secuencia de difusión, se evidenciaron hallazgos sugerentes de lesión colesteatomatosa en oído medio. Se realizó tratamiento quirúrgico endoscópico combinado con remoción completa de la lesión compatible histológicamente con CC y reconstrucción funcional con prótesis inactiva con resultado auditivo satisfactorio. El CC requiere alta sospecha diagnóstica por pediatras y otorrinolaringólogos, siendo imprescindible realizar un examen físico acucioso que incluya otoscopía de rutina, aunque el paciente no manifieste síntomas otológicos. El tratamiento es quirúrgico y debe considerar uso de endoscópico para asegurar una extracción completa de la lesión. En algunos casos es requerido realizar una reconstrucción auditiva para asegurar un óptimo resultado funcional.

Congenital cholesteatoma (CC) is a benign lesion of keratinized squamous epithelium that can affect different areas of the temporal bone, predominantly in the middle ear. CC is a rare pathology that occurs in pediatric patients and clinically manifests as a white lesion behind an intact eardrum. Most patients do not have a history of hearing loss, otorrhea, infection, perforation, or previous otologic surgery. The following, is the case of an infant with CC who consulted with a sleep disorder without otological symptoms but with altered otoscopy as a clinical finding. Nuclear magnetic resonance (NMR) with diffusion sequence with findings compatible with a cholesteatomatous lesion in the middle ear. Endoscopic surgical treatment was performed combined with complete removal of the lesion histologically compatible with CC and functional reconstruction with inactive prosthesis with satisfactory hearing results. CC requires high diagnostic suspicion by paediatrics and otorhinolaryngologists, and it is essential to perform a thorough physical examination that includes routine otoscopy even if the patient does not show otological symptoms. Treatment is surgical and endoscopic use should be considered to ensure complete removal of the lesion. In some cases, hearing reconstruction is required to ensure optimal functional results.

Cholesteatoma labyrinthine fistula: prevalence and impact / Fístula labiríntica por colesteatoma: prevalência e impacto

Abstract Introduction: Labyrinthine fistula is one of the most common complications associated with cholesteatoma. It represents an erosive loss of the endochondral bone overlying the labyrinth. Reasons for cholesteatoma-induced labyrinthine fistula are still poorly understood. Objective: Evaluate patients with cholesteatoma, in order to identify possible risk factors or clinical findings associated with labyrinthine fistula. Secondary objectives were to determine the prevalence of labyrinthine fistula in the study cohort, to analyze the role of computed tomography and to describe the hearing results after surgery. Methods: This retrospective cohort study included patients with an acquired middle ear cholesteatoma in at least one ear with no prior surgery, who underwent audiometry and tomographic examination of the ears or surgery at our institution. Hearing results after surgery were analyzed according to the labyrinthine fistula classification and the employed technique. Results: We analyzed a total of 333 patients, of which 9 (2.7%) had labyrinthine fistula in the lateral semicircular canal. In 8 patients, the fistula was first identified on image studies and confirmed at surgery. In patients with posterior epitympanic and two-route cholesteatomas, the prevalence was 5.0%; and in cases with remaining cholesteatoma growth patterns, the prevalence was 0.6% (p = 0.16). In addition, the prevalence ratio for labyrinthine fistula between patients with and without vertigo was 2.1. Of patients without sensorineural hearing loss before surgery, 80.0% remained with the same bone conduction thresholds, whereas 20.0% progressed to profound hearing loss. Of patients with sensorineural hearing loss before surgery, 33.33% remained with the same hearing impairment, whereas 33.33% showed improvement of the bone conduction thresholds' Pure Tone Average. Conclusion: Labyrinthine fistula must be ruled out prior to ear surgery, particularly in cases of posterior epitympanic or two-route cholesteatoma. Computed tomography is a good diagnostic modality for lateral semicircular canal fistula. Sensorineural hearing loss can occur post-surgically, even in previously unaffected patients despite the technique employed.

Resumo Introdução: A fístula labiríntica é uma das complicações mais comuns associadas ao colesteatoma. Representa uma perda erosiva do osso endocondral que recobre o labirinto. As razões para a ocorrência da fístula labiríntica induzida pelo colesteatoma ainda são mal compreendidas. Objetivo: Avaliar pacientes com colesteatoma, a fim de identificar possíveis fatores de risco ou achados clínicos associados à fístula labiríntica. Os objetivos secundários foram determinar a prevalência de fístula labiríntica no estudo de coorte, analisar o papel da tomografia computadorizada e descrever os resultados auditivos após a cirurgia. Método: Este foi um estudo de coorte retrospectivo. Foram incluídos pacientes com colesteatoma adquirido de orelha média em pelo menos um lado sem cirurgia prévia que haviam sido submetidos à audiometria e tomografia computadorizada de orelha ou cirurgia em nossa instituição. Os resultados auditivos após a cirurgia foram analisados de acordo com a classificação de fístula labiríntica e da técnica empregada. Resultados: Analisamos um total de 333 pacientes, dos quais 9 (2,7%) apresentavam fístula labiríntica no canal semicircular lateral. Em 8 pacientes, a fístula foi identificada na tomografia computadorizada e confirmada durante a cirurgia. Em pacientes com colesteatomas epitimpânicos posteriores e de via dupla, a prevalência foi de 5,0%; e nos casos com padrão de crescimento de colesteatoma remanescente, a prevalência foi de 0,6% (p = 0,16). Além disso, a taxa de prevalência de fístula labiríntica entre pacientes com e sem vertigem foi de 2,1. Dos pacientes sem perda auditiva neurossensorial antes da cirurgia, 80,0% permaneceram com os mesmos limiares de condução óssea, enquanto 20,0% progrediram para perda auditiva profunda. Dos pacientes com perda auditiva neurossensorial antes da cirurgia, 33,33% permaneceram com a mesma deficiência auditiva, enquanto 33,33% apresentaram melhora da média de dos limiares de condução óssea aos tons puros. Conclusão: A fístula labiríntica deve ser descartada antes do procedimento cirúrgico, particularmente nos casos de colesteatomas epitimpânicos posteriores e de dupla via. A tomografia computadorizada é uma boa modalidade diagnóstica para a fístula do canal semicircular lateral. A perda auditiva neurossensorial pode ocorrer pós-cirurgicamente, mesmo em pacientes previamente não afetados, a despeito da técnica empregada.

Sinus tympani y recidiva en cirugía de colesteatoma / Sinus tympani and recidiva in cholesteatoma surgery

RESUMEN Introducción: El sinus tympani (ST) es una de las áreas que más presenta colesteatoma residual. Recientemente se ha clasificado en 3 tipos de acuerdo a su morfología. Objetivos: Determinar el tipo de ST en los pacientes sometidos a cirugía de colesteatoma y analizar su impacto como factor de recidiva. Material y método: Revisión de fichas clínicas de pacientes sometidos a cirugía de colesteatoma entre los años 2004 y 2015 en el Hospital Regional de Concepción. Análisis de la tomografía axial computarizada (TAC) preoperatoria y posterior evaluación clínica de los pacientes operados mediante mastoidectomía canal wall down (CWD). Resultados: En el periodo descrito se operaron 271 oídos. El 60% de los casos analizados presentó ST tipo A y 40% ST tipo B. Se identificaron 12 casos de recidiva, 3 ST tipo B y 9 ST tipo A, sin diferencia estadísticamente significativa entre ambos. Discusión: Distinto a lo reportado en la literatura el tipo de ST más frecuente en nuestro estudio fue el tipo A, lo que podría corresponder a una variable étnica. Conclusión: El estudio preoperatorio con TAC es una herramienta útil para evaluar el tipo y compromiso del ST. Las diferencias anatómicas entre ST tipo A y B parece no ser un factor determinante de recidiva en mastoidectomías CWD.

ABSTRACT Introduction: Sinus tympani (ST) is one of the areas with the most residual cholesteatoma. Recently it has been classified in 3 types according to its morphology. Aim: To determine the type of ST in patients undergoing cholesteatoma surgery and to analyze its impact as a relapse factor. Material and method: Review of clinical files of patients submitted to cholesteatoma surgery between 2004 and 2015 at the Regional Hospital of Concepción. Preoperative computed axial tomography (CT) analysis and subsequent clinical evaluation of patients operated by canal wall down mastoidectomy (CWD). Results: In the described period 271 ears were operated. 60% of the cases analyzed had ST type A and 40% ST type B. Twelve cases of relapse were identified, 3 ST type B and 9 ST type A, with no statistically significant difference between the two. Discussion: Unlike to what is reported in the literature, the most common ST type in our study was type A, which could correspond to an ethnic variable. Conclusion: The preoperative study with CT is a useful tool to evaluate the type and commitment of ST. The anatomical differences between ST type A and B seems not to be a determinant factor of relapse in CWD mastoidectomies.

accuracy of clinical and radiologic diagnosis of cholesteatoma

This is a retrospective study for the cases of chronic suppurative otitis media with cholesteatoma. The aim of work was to throw light upon the variable clinical presentations and radiological findings among those patients as well as to study the operative details done. The study included 135 patients proved by surgical exploration to be complicated by cholesteatoma formation. These cases were admitted during a period of two years from 1988 to the end of 1989