Colesteatoma congénito: revisión de la literatura / Congenital cholesteatoma: literature review

El colesteatoma congénito es una entidad clínica única y desafiante, que se caracteriza por acumulación anormal de queratina en el oído medio, medial a la membrana timpánica. Se presenta, mayoritariamente, en el género masculino, con una incidencia estimada de 0.12 por 100.000 habitantes, representando el 4% a 24% de los colesteatomas en población pediátrica y un 2% a 5% del total de colesteatomas. Su origen aún es controversial, siendo la teoría más aceptada, la del arresto epitelial. Su diagnóstico es clínico, variando la sintomatología según severidad del compromiso, presentándose desde hallazgo incidental, hipoacusia de conducción, hasta presentar otalgia y perforación timpánica. Las imágenes se consideran un apoyo complementario preoperatorio. El tratamiento es quirúrgico, con diferentes técnicas disponibles, las cuales se deben definir de manera individual en el caso de cada paciente. Es fundamental su diagnóstico y manejo precoz, para lograr un tratamiento oportuno con menor tasa de complicaciones y compromiso a largo plazo. A continuación, se presenta una revisión de la literatura respecto de esta patología, para difusión en nuestro medio.

Congenital cholesteatoma (CC) is a unique and challenging clinical entity characterized by abnormal accumulation of keratin in the middle ear, medial to the tympanic membrane, being more frequent in the male gender, with an estimated incidence of 0.12 per 100,000 inhabitants. It represents 4% to 24% of cholesteatomas in the pediatric population and 2% to 5% of all cholesteatomas. Its cause is still controversial, the most accepted theory being epithelial arrest. The diagnosis is clinical, varying the symptoms according to the severity of the compromise, from incidental finding, through conduction hearing loss, to presenting otalgia and tympanic perforation. Images are considered additional preoperative support. Treatment is predominantly surgical, with different techniques available, which must be defined individually. Its early discovery and management are essential to achieve proper treatment with a lower rate of complications and long-term commitment. We present a review of the literature regarding CC to provide information relevant to our area of expertise.

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.

Intracranial epidermoid cyst in a cat / Cisto epidermoide intracraniano em um gato

ABSTRACT: Epidermoid cysts, also known as epidermal and keratin cysts, or congenital cholesteatomas are benign congenital non-neoplastic tumors, rarely observed in the brain of humans and some animal species (dogs, horses, mice, and rats). Histologically, they are composed of laminated, concentrically arranged keratin surrounded by a thin layer of stratified squamous epithelium. We describe a case of intracranial epidermoid cyst in a 6-year-old mixed-breed male cat in southern Brazil. The patient presented central vestibular syndrome. Given the poor prognosis and the fact that it belonged to a shelter with many dogs and cats, the owner requested euthanasia, and a thorough post-mortem examination was conducted immediately after death. The definitive diagnosis was based on histopathological findings. To the best of our knowledge, this is the first report of an intracranial epidermoid cyst in a cat.

RESUMO: Cisto epidermoide, também denominado cisto epidermal, cisto de queratina ou colesteatoma congênito é um tumor não neoplásico, benigno e congênito raramente encontrado no encéfalo de humanos e de algumas poucas espécies animais (cães, equinos, camundongos e ratos). Histologicamente, esse tumor é composto por queratina laminada concentricamente arranjada e circundada por uma fina parede de epitélio escamoso estratificado. Descreve-se um caso de cisto epidermoide intracraniano em um gato, macho, sem raça definida, de seis anos de idade, no sul do Brasil. O paciente foi encaminhado para atendimento veterinário por apresentar sinais de síndrome vestibular central. Devido ao mau prognóstico e por pertencer a um abrigo com muitos cães e gatos, o proprietário optou pela eutanásia e o paciente foi encaminhado para a realização de necropsia. O diagnóstico definitivo foi baseado nos achados histopatológicos. Pelo conhecimento dos autores, este é o primeiro relato de um cisto epidermoide intracraniano em um gato.

Spontaneous migration of a congenital intratympanic membrane cholesteatoma / 영남의대학술지

Congenital intratympanic membrane cholesteatoma (ITMC) is a rare type of congenital cholesteatoma located within the tympanic membrane. This lesion tends to increase in size over time. The development of ITMC can cause several complications such as hearing impairment, dizziness, facial palsy, and intracranial complications, similar to any other cholesteatoma. The treatment of congenital cholesteatoma requires the removal of the lesion through surgery, because disease progression induces bony destruction of the nearby tissue. Most patients presenting with this cholesteatoma type are also treated with primary surgical removal. However, we recently experienced a case of an ITMC that showed a natural transition to an external auditory canal cholesteatoma.

Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma

OBJECTIVES: As endoscopic instrumentation, techniques and knowledges have significantly improved recently, endoscopic ear surgery has become increasingly popular. Transcanal endoscopic ear surgery (TEES) can provide better visualization of hidden areas in the middle ear cavity during congenital cholesteatoma removal. We aimed to describe outcomes for TEES for congenital cholesteatoma in a pediatric population. METHODS: Twenty-five children (age, 17 months to 9 years) with congenital cholesteatoma confined to the middle ear underwent TEES by an experienced surgeon; 13 children had been classified as Potsic stage I, seven as stage II, and five as stage III. The mean follow-up period was 24 months. Recurrence of congenital cholesteatoma and surgical complication was observed. RESULTS: Congenital cholesteatoma can be removed successfully via transcanal endoscopic approach in all patients, and no surgical complications occurred; only one patient with a stage II cholesteatoma showed recurrence during the follow-up visit, and the patient underwent revision surgery. The other patients underwent one-stage operations and showed no cholesteatoma recurrence at their last visits. Two patients underwent second-stage ossicular reconstruction. CONCLUSION: Although the follow-up period and number of patients were limited, pediatric congenital cholesteatoma limited to the middle ear cavity could be safely and effectively removed using TEES.

Clinical Aspects and Surgical Outcomes of Congenital Cholesteatoma in 93 Children: Increasing Trends of Congenital Cholesteatoma from 1997 through 2012

BACKGROUND AND OBJECTIVES: The recent increase in the reported incidence of congenital cholesteatoma (CC) may be secondary to the widespread use of otoendoscopy as well as an increased awareness of these lesions among primary care physicians. However, little research about CC has been conducted in a large group of patients. This study aimed to analyze the clinical characteristics of CC including the annual number of patients, symptoms, age at diagnosis, stage and type of disease, surgical techniques, recurrence, and postoperative complications. SUBJECTS AND METHODS: Retrospective chart review was performed for patients who met the inclusion criteria between January 1997 and June 2012. RESULTS: Ninety-three patients underwent surgery for CC. The age at operation ranged from 12 months to 17 years (mean age, 6.1 years). The number of patients was less than 4 per year until 2005, but increased to more than 10 per year since 2008. CC was most commonly reported as an incidental finding (58.1%). The operative procedures included the transcanal myringotomy approach (46.2%), canal wall up mastoidectomy (37.6%), tympanoplasty (8.6%), and canal wall down mastoidectomy (7.5%). The recurrence rate was 20.4% and the complication rate was 12.9%. No patients with stage I CC had complications. CONCLUSIONS: This study showed that the incidence of CC has recently increased notably. Most patients with stage I and II CC were completely cured by transtympanic surgery, and complication and recurrence rates increased according to the extent of disease. Early detection of CC is important to facilitate minimally invasive surgery and to reduce complication and recurrence rates.

A Case of Two Isolated Congenital Cholesteatomas Presented in Middle Ear Cavity / 대한이비인후과학회지

Middle ear cholesteatoma is considered to be congenital in origin when there is an intact tympanic membrane with no prior history of otorrhea, tympanic perforation or otologic procedure. However, congenital cholesteatoma is a relatively rare disease entity for which a variety of theories regarding its pathogenesis have been suggested as follows: epidermoid formation, migration of squamous epithelium, implantation, metaplasia, and acquired inclusion theory. However, the pathogenesis of congenital cholesteatoma remains unclear. There are two pathologic types of congenital cholesteatoma, "closed" and "open". We present a rare case of congenital cholesteatoma arising from middle ear cavity in a condition of two completely isolated closed congenital cholesteatomas, which were successfully removed via transcanal approach. Also we review the literature and discuss the theories on the development of two separate congenital cholesteatomas.

Colesteatoma congénito del conducto auditivo externo. Caso clínico / Congenital cholesteatoma of the external auditory meatus. Case report / Colesteatoma congênito do canal auditivo externo. Caso

Se expone el caso poco frecuente de colesteatoma congénito del conducto auditivo externo en un lactante de 6 meses de edad que se presentó inicialmente como absceso.

We review the unusual presentation of a congenital cholesteatoma of the external auditory meatus in a 6 months old infant, initially presented as an abscess.

O caso raro de colesteatoma congênito do canal auditivo externo em crianças de 6 meses de idade, que apresentou inicialmente como abscesso está exposto.

The Clinical Effectiveness of the Minimally Invasive Transcanal Myringotomy for the Removal of Early Stage Congenital Cholesteatoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Because of the wide application of the endoscopic diagnostic system and increased amount of attention for early stage congenital cholesteatoma (CC) recently, the detection of CC in stages I and II has increased. In this study, we investigated the effectiveness of minimally invasive transcanal myringotomy (MITM), and compared its results with those of postauricular approach (PAA) in early stage CC consisting of stage I and II of Potsic's classification. SUBJECTS AND METHOD: We retrospectively studied 70 patients who were diagnosed with CC after surgery performed by an otologist from June 2006 to June 2013. Thirty-two patients were in early stage CC consisting stage I and II. Of the 32 patients, MITM was performed on 20 and PAA was performed on 12. We analyzed the characteristics of disease, operation time, hospitalization period, recurrence and complications according to the stage and each operation. RESULTS: There were no significant differences between the stages and types of the disease between the MITM and the PAA group (p>0.05). The operation time and hospitalization period for the MITM group were significantly shorter than for the PAA group (p<0.001). There was no recurrence in the two groups during the average follow-up period of 33 months follow-up. Tympanic membrane perforation occurred in one patient in the MITM group. One patient presenting an external aural epidermal cyst in the PAA group was treated with surgical removal. CONCLUSION: The MITM is an efficacious and feasible method to remove early stage CC.

Risk Factors of Post-Operative Recidivism in Congenital Cholesteatoma of the Middle Ear / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: There is no general consensus on the risk factors affecting the recurrence or residual disease (recidivism) after surgery of congenital cholesteatoma of the middle ear. In this study, we analyzed clinical characteristics of recidivistic cases after the surgery and compared those of non-recurrent cases to investigate the risk factors regarding the post-operative recidivism of the disease. SUBJECTS AND METHOD: Data were collected from retrospective chart reviews and computerized database of patients who have underwent surgeries at Kangdong and Hallym University Sacred Heart Hospital during the last 24 years. All surgeries, consisting of 95 primary cases and 24 revision cases, were performed by the same surgeon. RESULTS: Recurrence was detected in 24 cases of the 95 patients (including 4 cases, of which primary surgeries were done at other hospitals). Among the risk factors affecting post-operative recidivism such as sex, age, symptom, disease duration, type of cholesteatoma, mastoid pneumatization, ossicle status, and stage of disease, factors of longer symptom duration, poor mastoid pneumatization, older age, poor mastoid pneumatization, advanced or recurrent cholesteatoma were significantly related to the recidivism of disease. CONCLUSION: Longer symptom duration, poor mastoid pneumatization, and advanced or recurrent cholesteatoma were revealed as high risk factors for post-operative recidivism in this study.

Congenital Cholesteatoma: Diagnosis and Management / 대한이비인후과학회지

Incidence of congenital cholesteatoma of the middle ear seems to be increasing due to recent developments in diagnostic skill including the use of endoscopy. As residual or recurred cholesteatoma after incomplete removal of the disease is also on the rise as well, a necessity has been emerging for the systematic approach for diagnosis and management of the disease. In this paper, author wishes to help the novice of the ear surgery by introducing a novel staging system and treatment algorithm for the disease, which were developed through author's surgical experience of more than one hundred cases over twenty years as well as the literature review.