Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis

Gilberto, Nelson; Custódio, Sara; Colaço, Tiago; Santos, Ricardo; Sousa, Pedro; Escada, Pedro

doi:10.1007/s00405-020-05792-4

Cited by 38 publications

(51 citation statements)

References 83 publications

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“…However, in the remaining 34%, CC is incidentally diagnosed by routine otoscopy or during otologic screening. 6 Based on its histopathology, CC can be divided 2 types: the closed type, which presents as an epithelial cyst without keratin exposure, and the open type, characterized by exposure of the cholesteatoma matrix that forms part of the middle ear mucosa. 7 The most common site of CC is the anterosuperior quadrant of the middle ear, but it does not consistently remain confined there.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic Surgery of Congenital Cholesteatoma in the Anterior Epitympanic Recess: A Case Report of a Rare Location

2020

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Congenital cholesteatoma is a whitish mass in the middle ear medial to an intact tympanic membrane. It is often without symptoms and therefore incidentally diagnosed. Pediatric congenital cholesteatoma generally starts as a small pearl-like mass in the middle ear cavity that eventually expands to involve the ossicles, epitympanum, and mastoid. The location, size, histopathological type, and extent of the mass must be evaluated to select the appropriate surgical method. Although microscopic ear surgery has traditionally been performed to remove congenital cholesteatoma, a recently introduced alternative is endoscopic surgery, which allows a minimally invasive approach and has better visualization. Here, we report the first known case of a patient with congenital cholesteatoma in the anterior epitympanic recess and discuss the utility of an endoscopic approach in the removal of a congenital cholesteatoma in the hidden area within the middle ear.

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Section: Discussionmentioning

confidence: 99%

Endoscopic Surgery of Congenital Cholesteatoma in the Anterior Epitympanic Recess: A Case Report of a Rare Location

2020

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“…In most recent congenital cholesteatoma case series (eg, Cho et al or Mostafa et al) as well as meta-analyses (Gilberto et al), authors note that the diagnosis is most commonly incidental, although patients can present with hearing loss, discharge, or facial paralysis and have a history of otitis media. 11,[17][18][19] Because the ossicular chain is affected early in the pathologic progression, damage to the ossicles and subsequent hearing loss may be the first indicators of IIC. Newly diagnosed isolated hearing loss in children with unremarkable audiologic history and normal otoscopy, combined with a CT scan showing abnormalities and discontinuity of the ossicular chain, should alert physicians to the possibility of this diagnosis.…”

Section: Presentation and Symptomatologymentioning

confidence: 99%

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features

et al. 2020

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Objectives: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. Methods: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children’s Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. Results: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. Conclusion: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

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“…Furthermore, facial paralysis might be caused by erosion of the facial canal and disturbances of the equilibrium organ due to detraction of the semicircular fistulas. Fortified inflammation causes mastoiditis, meningitis, intracranial abscesses, and sinus vein thrombosis [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

“…The removal of the external auditory canal is considered to be the most effective procedure to allow complete cholesteatoma removal. However, the preservation or restoration of the hearing ability at the same time requires a high level of surgical expertise [ 5 , 8 , 13 , 14 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

Surgical Guidance for Removal of Cholesteatoma Using a Multispectral 3D-Endoscope

Wisotzky

Rosenthal

Wege

et al. 2020

Sensors

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We develop a stereo-multispectral endoscopic prototype in which a filter-wheel is used for surgical guidance to remove cholesteatoma tissue in the middle ear. Cholesteatoma is a destructive proliferating tissue. The only treatment for this disease is surgery. Removal is a very demanding task, even for experienced surgeons. It is very difficult to distinguish between bone and cholesteatoma. In addition, it can even reoccur if not all tissue particles of the cholesteatoma are removed, which leads to undesirable follow-up operations. Therefore, we propose an image-based method that combines multispectral tissue classification and 3D reconstruction to identify all parts of the removed tissue and determine their metric dimensions intraoperatively. The designed multispectral filter-wheel 3D-endoscope prototype can switch between narrow-band spectral and broad-band white illumination, which is technically evaluated in terms of optical system properties. Further, it is tested and evaluated on three patients. The wavelengths 400 nm and 420 nm are identified as most suitable for the differentiation task. The stereoscopic image acquisition allows accurate 3D surface reconstruction of the enhanced image information. The first results are promising, as the cholesteatoma can be easily highlighted, correctly identified, and visualized as a true-to-scale 3D model showing the patient-specific anatomy.

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Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis

Cited by 38 publications

References 83 publications

Endoscopic Surgery of Congenital Cholesteatoma in the Anterior Epitympanic Recess: A Case Report of a Rare Location

Endoscopic Surgery of Congenital Cholesteatoma in the Anterior Epitympanic Recess: A Case Report of a Rare Location

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features

Surgical Guidance for Removal of Cholesteatoma Using a Multispectral 3D-Endoscope

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