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.

Supratentorial and cervical extensions of a giant middle ear cholesteatoma.

INTRODUCTION: Cholesteatomas are slowly progressive and well demarcated lesions but if not treated, they can spread to the inner ear and brain and lead to serious neurological complications. CASE REPORT: A 59-year old male was referred to our practice. His initial imaging showed that a huge intracranial mass has spread to the neck. Beyond surgical exploration, a sizable temporal bone lesion with extensive keratinisation was observed which later on histopathological exam turned out to be a cholesteatoma. CONCLUSIONS: Cholesteatomas may be aggressive lesions by rapidly growing and gross destructive features. In such cases, more attempts should be given to ruling out any malignant neoplastic tissues.

Variability of the retrotympanum and its association with mastoid pneumatization in cholesteatoma patients.

PURPOSE: This study aimed to investigate the variability of the retrotympanum in patients undergoing surgical treatment for cholesteatoma. METHODS: We included 59 ears of patients undergoing middle ear surgery for cholesteatoma who had preoperative computed tomography scans. A retrospective analysis of the medical records was conducted. The sinus tympani (ST), subtympanic sinus (STS) and facial recess (FR) were classified into types A-C based on the relationship of their extension to the facial nerve. The mastoid and petrous apex were assessed and categorized as normal pneumatized or sclerotic. RESULTS: Type A extension was the most frequently found in all sinuses (ST 64%, FR 77%, STS 69%), Type B extension was found more often in ST (34%) and STS (24%) than in FR (15%). A very deep extension was found only rarely (ST 2%, FR 8%, STS 7%). A sclerotic mastoid was found in 67% of cases. Those cases showed a statistically significant difference regarding retrotympanum pneumatization when compared with normal mastoid. CONCLUSION: The most frequent variant of retrotympanic pneumatization in relation to the facial nerve was type A in all subsites in cholesteatoma patients. The variability among patients with cholesteatoma is different to previously published results in healthy subjects. Moreover, the pneumatization of the retrotympanum is associated with mastoid pneumatization.

Clinical Analysis of 85 Cases of External Auditory Canal Cholesteatoma Surgery under Specialized Endoscopy.

Objective: To investigate the clinical characteristics, surgical experience, and surgical outcomes of external auditory canal cholesteatoma (EACC) surgery under endoscopic otolaryngoscopy. Methods: A retrospective analysis of 85 EACC cases admitted to the Department of Otolaryngology, Renji Hospital, Shanghai Jiaotong University School of Medicine, from January 2016 to February 2021 was performed, followed by retrospective analysis of clinical data to explore the feasibility and clinical characteristics of all-oral endoscopic EACC surgery. A total of 85 EACC patients (90 ears) with a mean age of 49.93 ± 14.87 years were included in the study. According to Udayabhanu staging, 43 ears (47.78%) were stage I, 40 ears (44.44%) were stage II, and 7 ears (7.78%) were stage III. All patients underwent transendoscopic surgery. Results: 79 ears (87.78%) underwent endoscopic EACC resection alone (+external auditory canal tumor resection/tympanostomy tube insertion), 9 ears (10%) underwent endoscopic EACC resection+tympanostomy+tympanoplasty, 1 ear (1.11%) underwent endoscopic EACC resection+tympanoplasty, and 2 ears (2.22%) underwent EACC resection+otolaryngotomy+tympanoplasty+auditory chain reconstruction endoscopically. Of these, 7 ears (7.78%) underwent auricular cartilage-chondroplasty and 2 ears (2.22%) underwent auricular cartilage membrane repair. All patients were reviewed at 1 week, 2 weeks, 1 month, 3 months, 6 months, and 1 year postoperatively. One patient with stage II external auditory atresia had a recurrence after 6 months and underwent endoscopic ear surgery (ESS) again. One patient with stage 2 atresia recurred after 1 year and again underwent endoscopic ear surgery. The rest of the patients recovered well after the surgery, and the grafts healed well. Conclusion: EACC surgery through the external ear canal under a dedicated endoscope is a safe, reliable, and effective method. Patients with stage I and II external auditory canal cholesteatoma surgery under endoscopy have a rapid postoperative recovery with significant hearing improvement, and stage IIIA patients can also achieve good results under strict evaluation of indications.

[Endoscopic transnasal approach in surgical treatment of petrous temporal bone cholesteatoma extending towards the clivus. Three clinical observations and literature review]. / Endoskopicheskii transnazal'nyi dostup v khirurgicheskom lechenii kholesteatom piramidy visochnoi kosti, rasprostranyayushchikhsya v oblast' skata. Tri klinicheskikh nablyudeniya i obzor literatury.

Petrous temporal bone Cholesteatoma is widely described in the literature and accounts for up to 9% of all neoplasms of this localization. These cholesteatomas rarely spread towards the clivus. Isolated clival cholesteatomas are described only as single cases. There is currently no consensus on the choice of surgical approach for resection of similar neoplasms. OBJECTIVE: To demonstrate the possibilities of endoscopic transnasal approach in surgery for clival and petrous cholesteatoma. MATERIAL AND METHODS: This article presents 3 clinical cases: 2 patients with apical cholesteatoma of petrous part of temporal bone extending to the clivus and 1 patient with massive petrous temporal bone cholesteatoma extending towards the clivus according to the classification of Sanna M. All patients underwent endoscopic transnasal surgery. RESULTS AND CONCLUSION: In our opinion, endoscopic transnasal approach is optimal for resection of similar neoplasms. Total and subtotal resection was performed in 2 and 1 case, respectively. However, there are certain limitations of this approach in accessing the most lateral parts of the neoplasm. Nevertheless, endoscopic transnasal approach ensures resection of petrous temporal bone cholesteatoma extending to the clivus without the risk of damage to acoustic-facial cranial nerves. It is especially significant in patients without their baseline dysfunction.

Is it useful to use computerized tomography image-based artificial intelligence modelling in the differential diagnosis of chronic otitis media with and without cholesteatoma?

OBJECTIVE: Cholesteatoma is an aggressive form of chronic otitis media (COM). For this reason, it is important to distinguish between COM with and without cholesteatoma. In this study, the role of artificial intelligence modelling in differentiating COM with and without cholesteatoma on computed tomography images was evaluated. METHODS: The files of 200 patients who underwent mastoidectomy and/or tympanoplasty for COM in our clinic between January 2016 and January 2021 were retrospectively reviewed. According to the presence of cholesteatoma, the patients were divided into two groups as chronic otitis with cholesteatoma (n = 100) and chronic otitis without cholesteatoma (n = 100). The control group (n = 100) consisted of patients who did not have any previous ear disease and did not have any active complaints about the ear. Temporal bone computed tomography (CT) images of all patients were analyzed. The distinction between cholesteatoma and COM was evaluated by using 80% of the CT images obtained for the training of artificial intelligence modelling and the remaining 20% for testing purposes. RESULTS: The accuracy rate obtained in the hybrid model we used in our study was 95.4%. The proposed model correctly predicted 2952 out of 3093 CT images, while it predicted 141 incorrectly. It correctly predicted 936 (93.78%) of 998 images in the COM group with cholesteatoma, 835 (92.77%) of 900 images in the COM group without cholesteatoma, and 1181 (98.82%) of 1195 images in the normal group. CONCLUSION: In our study, it has been shown that the differentiation of COM with and without cholesteatoma with artificial intelligence modelling can be made with highly accurate diagnosis rates by using CT images. With the deep learning modelling we proposed, the highest correct diagnosis rate in the literature was obtained. According to the results of our study, we think that with the use of artificial intelligence in practice, the diagnosis of cholesteatoma can be made earlier, it will help in the selection of the most appropriate treatment approach, and the complications can be reduced.

Absceso mastoideo como presentación de colesteatoma congénito en preescolar ¿cuál es el origen? / Mastoid abscess as congenital cholesteatoma presentation in a child what is the origin?

Resumen El colesteatoma congénito es una entidad que puede manifestarse con una amplia variedad de síntomas o ser silente durante largo tiempo y constituir un hallazgo incidental. Una vez diagnosticada es importante valorar su extensión y el compromiso de estructuras adyacentes, para lograr una adecuada planificación quirúrgica, eliminando la enfermedad y manteniendo la mejor funcionalidad posible. Se presenta un caso de colesteatoma congénito infantil.

Abstract Congenital cholesteatoma is an entity that can manifest with a wide variety of symptoms or be silent for a long time and constitute an incidental finding. Once diagnosed, it is important to assess the extension to apply the most efficient treatment, eliminating the disease and providing functionality if possible. A case of congenital cholesteatoma in a child is presented.

Could computed tomography histogram analysis add value to the diagnosis of cholesteatoma?

BACKGROUND: To investigate the potential role of computed tomography (CT) histogram analysis in differentiating cholesteatoma (CHS) and non-cholesteatoma (NCHS). METHODS: We evaluated 77 temporal bone CT images (from November 2016 to February 2020) that were obtained preoperatively (mean age, 37.10±17.27 years in CHS; 36.72±16.08 years in NCHS group). Histogram analyses of the resulting XML files were conducted using the R Project 3.3.2 program. ROC analysis was used to find threshold values, and the diagnostic efficiency of these values in differentiating CHS-NCHS was determined. RESULTS: The CT images of 41 CHS (53.25%) and 36 NHCS cases (46.75%) were evaluated. There was a statistically significant difference between the CHS and NCHS group in terms of the mean, maximum, and median values (p = 0.036, p = 0.006, p = 0.043). When examining the ROC curve obtained from the mean of these parameters, area under the curve (AUC) is determined as 0.638, and when the threshold value is selected as 42.55, the mean value was determined to have a sensitivity of 86.50% and specificity of 56.10% in differentiating CHS-NCHS.

Identifying epithelial borders in cholesteatoma surgery using narrow band imaging.

PURPOSE: To quantify changes in the perceived epithelial border with narrow band imaging (NBI) and white light imaging (WLI) during cholesteatoma surgery and to objectify possible benefits of NBI in otology. METHODS: Perioperative digital endoscopic images were captured during combined approach tympanoplasty at our tertiary referral center using WLI and NBI (415 nm and 540 nm wavelengths). Sixteen otologic surgeon defined the epithelial borders within 16 identical WLI and NBI photos. Pixels of these selections were calculated to analyze the quantitative difference between WLI and NBI. A questionnaire also analyzed the qualitative differences. RESULTS: Sixteen otologic surgeons participated in the study. Stratified per photo, only two photos yielded a significant difference: less pixels were selected with NBI than WLI. A Bland-Altman plot showed no systemic error. Stratified per otologist, four participants selected significantly more pixels with WLI than with NBI. Overall, no significant difference between selected pixels was found. Sub-analyses of surgeons with more than 5 years of experience yielded no additional findings. Despite these results, 60% believed NBI could be advantageous in defining epithelial borders, of which 83% believed NBI could reduce the risk of residual disease. CONCLUSION: There was no objective difference in the identification of epithelial borders with NBI compared to WLI in cholesteatoma surgery. Therefore, we do not expect the use of NBI to evidently decrease the risk of residual cholesteatoma. However, subjective assessment does suggest a possible benefit of lighting techniques in otology.

Surgical corridors for congenital aural atresia with otogenic cerebellar abscess and lateral sinus thrombosis.

The following is a case report of an adolescent with mental retardation who had congenital aural atresia with contralateral congenital facial palsy. She developed multiple intracranial complications (cerebellar abscess and lateral sinus thrombosis) due to cholesteatoma. We managed her in a multidisciplinary approach. This report discusses case management, emphasising the meticulous intraoperative steps taken in identifying the landmarks and precautions adopted to avoid postoperative facial palsy and other complications.

Combined Endoscopic Endonasal and Contralateral Transmaxillary Approach for Petrous Cholesteatoma: 2-Dimensional Operative Video.

This 59-yr-old man presented with headache, dizziness, diplopia, and right-side hearing impairment for years. The objective degree of hearing impairment was not available. Magnetic resonance imaging (MRI) showed a right petrous apex lesion centered behind the right petrous internal carotid artery and extending lateral to the medial aspect of the right internal auditory canal. A combined endoscopic endonasal and left contralateral transmaxillary (CTM) approach was performed, and gross-total resection was achieved. Peeling the cyst wall from the dura resulted in minor weeping. It was covered with a left-sided, vascularized nasoseptal flap. His dizziness and diplopia improved immediately after the surgery. Histopathology revealed an epidermoid cyst. In this surgical video, we demonstrate the key steps of the CTM approach for access to the petrous apex posterior to the petrous internal carotid artery (ICA). The patient gave informed consent for surgery and video recording.

Rapid diffusion-weighted MRI for the investigation of recurrent temporal bone cholesteatoma.

BACKGROUND: Suspected cholesteatoma recurrence is commonly investigated with magnetic resonance imaging (MRI) of the temporal bone. Non-echo planar diffusion-weighted imaging (non-EP DWI) has become the sequence of choice. PURPOSE: To assess the agreement between an MRI protocol incorporating both non-EP DWI and contrast-enhanced sequences, and a shortened protocol without contrast-enhanced sequences in the assessment of suspected cholesteatoma recurrence. MATERIALS AND METHODS: One hundred consecutive MRIs, consisting of T2-weighted, non-EP DWI and pre- and post-contrast T1-weighted sequences, were reviewed by two radiologists at a tertiary referral centre. Agreement between the two protocols was assessment by means of a weighted Cohen kappa coefficient. RESULTS: We found a near perfect agreement between the two protocols (kappa coefficient with linear weighting 0.98; 95% confidence interval 0.95-1.00). There were two cases in which the two protocols were discordant. In both cases, the lesion measured <3 mm and images were degraded by artefact at the bone-air interface. The shortened protocol without post-contrast sequences yielded a 32% reduction in acquisition time. CONCLUSION: When non-EP DWI is available, contrast-enhanced sequences can be omitted in the vast majority of cases without compromising diagnostic accuracy. Contrast-enhanced sequences may provide additional value in equivocal cases with small (<3 mm) lesions or in cases where images are degraded by artefact.

Concurrent Occurrence of Congenital Ossicular Anomaly and Localized Cholesteatoma: Series of 10 Cases.

OBJECTIVE: The objective of this study is to describe the clinical features, managements and outcomes of a rare coexistence of congenital ossicular anomaly and localized cholesteatoma. A literature review on these cases and each congenital disorder is also presented. METHODS: A retrospective chart review was performed on patients diagnosed with congenital ossicular anomaly with concurrent localized cholesteatoma from 2008 to 2017. Clinical data of these patients were collected. RESULTS: A total of 10 patients were identified. All patients presented with unilateral hearing loss. Pure-tone audiometry showed conductive hearing loss in all affected ears with an average air conduction (AC) threshold of 59 dB. High-resolution computed tomography scans of the temporal bone diagnosed ossicular anomaly for 90% (9/10); however, only 50% (5/10) had a diagnosis of localized cholesteatoma. A transcanal exploratory tympanotomy under the microscope was performed to discover whether the localized tiny-sized cholesteatoma around the ossicular chain did not have direct contact with the ossicular chain, which could be diagnosed as congenital cholesteatoma. We removed the localized cholesteatoma and reconstructed the ossicular chain in each patient. All localized cholesteatomas were found in the posterior-superior quadrant of the middle ear. Ossicular chain anomalies were associated with the incus and/or the stapes in all cases. Hearing improvement was achieved in each of the 6 patients who were followed up postoperatively, with an average AC threshold of 35 dB. The clinical features of congenital ossicular anomaly with concurrent congenital cholesteatoma were compared with those of each congenital disorder. The pathogenesis of each condition was also discussed. CONCLUSIONS: Congenital ossicular anomaly with concurrent congenital cholesteatoma is rare. It shares similar clinical features with congenital ossicular anomaly occurring alone, therefore awareness should be raised for a possible concurrent congenital cholesteatoma which was easy to miss in the diagnosis (50%) by the radiologist. A patient's hearing level can be improved by removal of the cholesteatoma and reconstruction of the ossicular chain. Localized cholesteatoma does not usually show residuals or recurrence.

Congenital External Auditory Canal Cholesteatoma Resected by a Transcanal Endoscopic Approach.

OBJECTIVE: To describe the first case of congenital external auditory canal cholesteatoma resected via a transcanal endoscopic approach and describe the possible pathologenesis of cholesteatoma. PATIENT: A 2-year-old female patient presented with a cholesteatoma that extended from the floor of the external auditory bony canal to the inferior quadrant of her right tympanic membrane. INTERVENTION: Otological examinations and computed tomography were performed. The cholesteatoma was resected using a transcanal endoscopic approach. RESULTS: Pathologic examination confirmed the diagnosis of external auditory canal cholesteatoma. The patient's postoperative hearing was observed to be normal. Cholesteatoma did not recur during the 2-year follow-up period. Computed tomography scan revealed an isodense mass, partly surrounded by bony tissue, on the floor of the external auditory canal, outside of the inferior quadrant of an intact tympanic membrane. A minor malformation of the bony tissue, covering the inferiolateral surface of the cholesteatoma, was observed. The proposed mechanisms of pathogenesis are: 1) the embryonic developmental deficits of the meatal plug, during its differentiation into squamous epithelium, cause the arrest of ectodermal tissues, 2) a remnant of the squamous epithelium becomes trapped in the niche of the foramen tympanicum. CONCLUSION: A rare case of congenital external auditory canal cholesteatoma, located on the inferior external auditory canal, was diagnosed and resected using a transcanal endoscopic approach. It was possibly caused by a minor anomaly of the first branchial cleft or by a remnant of the squamous epithelium trapped in the foramen tympanicum.

Evolving Treatment of Frontal Sinus Cholesteatoma: A Case Report.

INTRODUCTION: Cholesteatomas are lined by squamous epithelium, contain keratin debris, and can cause bony erosion. Although commonly found in the middle ear space and mastoid, cholesteatomas may develop in adjacent structures including the paranasal sinuses. Frontal sinus cholesteatoma (FSC) is a rare condition with fewer than 30 reported cases. The aims of this study are to describe the clinical presentation, diagnostic imaging, and endoscopic treatment of FSC and to review the literature focusing on the pathogenesis, diagnosis, and historical and contemporary treatments of FSC. CASE PRESENTATION: A 45-year-old man presented with a 1-week history of right eyelid and forehead swelling. Results of computed tomography scans and magnetic resonance images revealed a right frontal sinus lesion of soft-tissue density with bony dehiscence along the superior orbit and posterior table. He underwent right-sided endoscopic sinus surgery at a tertiary care center in January 2017. Intraoperatively, the frontal sinus contained keratin debris suggestive of FSC. This suspicion was confirmed postoperatively by pathologic analysis after subtotal resection. DISCUSSION: The pathogenesis of frontal sinus cholesteatoma varies based on its cause (congenital vs acquired). Clinical diagnosis remains challenging but is aided by nasal endoscopy, computed tomography, and magnetic resonance imaging. Historically, FSC has been managed by total extirpation through open approaches, which can entail substantial morbidity. With sophisticated endoscopic sinus instrumentation and image guidance, FSC can be successfully treated via an endoscopic approach. Serial débridements and washouts in an outpatient setting may adequately manage the residual disease in the postoperative period.

Congenital Cholesteatoma of the Sphenoid, Occipital, and Temporal Bones: 54-Year Follow Up.

OBJECTIVE: To provide long-term follow up of a unique patient with history of massive petrous apex congenital cholesteatoma. PATIENT: 75-year-old man who presented at age 18 with left Gradenigo-like syndrome. INTERVENTION: Staged left radical mastoidectomy and open transsphenoidal marsupialization. Followed with routine in-office cholesteatoma debridement. MAIN OUTCOME MEASURES: Bony erosion on computed tomography (CT), monitoring for new symptoms. RESULTS: Interval development of sensorineural component of hearing loss due to cochlear fistulization. Very slow skull base bony erosion continues without neck destabilization. No new or recurrent cranial neuropathies have developed. CONCLUSIONS: Marsupialized cholesteatoma may be followed with routine debridement and interval imaging for monitoring over many decades.

Correlation of pre-operative computed tomography, intra-operative findings and surgical outcomes in revision tympanomastoidectomy.

OBJECTIVE: To correlate pre-operative computed tomography findings, intra-operative details and surgical outcomes with cholesteatoma recurrence in revision tympanomastoidectomy. METHODS: This retrospective, non-randomised, single-institution cohort study included 42 patients who underwent pre-operative computed tomography imaging and revision surgery for recurrent chronic otitis media. Twelve disease localisations noted during revision surgery were correlated with pre-operative temporal bone computed tomography scans. A matched pair analysis was performed on patients with similar intra-operative findings, but without pre-operative computed tomography scans. RESULTS: Pre-operative computed tomography identified 25 out of 31 cholesteatoma recurrences. Computed tomography findings correlated with: recurrent cholesteatoma when attic opacification and ossicular chain involvement were present; and revision surgery type. Sinodural angle disease, posterior canal wall erosion and dehiscent dura were identified as predictors of canal wall down tympanomastoidectomy. Patients with pre-operative computed tomography scans had a higher rate of cholesteatoma recurrence, younger age at diagnosis of recurrent disease, more revision surgical procedures and less time between previous and revision surgical procedures (all p < 0.05). CONCLUSION: Pre-operative imaging and intra-operative findings have important clinical implications in revision surgery for chronic otitis media. Performing pre-operative computed tomography increases diagnosis accuracy and reduces the time required to diagnose recurrent disease.

Congenital Cholesteatoma in a Case of Congenital Aural Atresia: A Case Report.

Congenital external canal atresia is one of the congenital ear anomalies that can occur in patients. Similarly, congenital cholesteatoma is also another congenital disease that is often diagnosed in early adulthood. Both the above-mentioned diseases can occur independently but the presence of both these entities is a rare occurrence and needs a high degree of suspicion aided by a computed tomography scan to make the diagnosis. We are presenting a case of a sixteen-year-old patient who presented with unilateral ear anomaly, earache, facial palsy, and postaural swelling and was diagnosed as a right sided congenital aural atresia with congenital cholesteatoma. He was surgically managed with right-sided modified radical mastoidectomy with canaloplasty and closure of mastoid fistula under general anesthesia.