LX Congenital Cholesteatoma of the Middle Ear and Mastoid

Derlacki, Eugene L.; Clemis, Jack D.

doi:10.1177/000348946507400313

Cited by 171 publications

(111 citation statements)

References 14 publications

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“…We should suspect a congenital form in cases with an early age without a history of ear trauma, surgery, or middle ear infection. There are diagnostic criteria for the clinical diagnosis of congenital middle ear cholesteatoma (i.e., development behind an intact normal looking TM, without a previous history of aural infections, and arising from epithelial inclusions), but there are none for congenital ITMC [2] .…”

Section: Discussionmentioning

confidence: 99%

Congenital Intratympanic Cholesteatoma in an Adult Patient: A Case Report and Review of the Literature

Pedruzzi

Mion²,

Comacchio³

2016

Int Adv Otol

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Congenital cholesteatoma within the tympanic membrane is an uncommon entity, with only few cases being documented. The aetiopathogenesis of this lesion is still unknown; however, when cholesteatoma develops in subjects without any history of previous ear inflammation, as in the case we report here, an embryologic origin is deeply suspected. An acquired origin is hypothesized in patients with a previous history of an inflammatory process of the external or middle ear because of the proliferation of the basal cell layer of the tympanic membrane epithelium. We report a rare case of congenital cholesteatoma of the tympanic membrane in an adult patient and review the literature KEYWORDS: Congenital intratympanic cholesteatoma, intratympanic cholesteatoma, congenital cholesteatoma of the tympanic membrane, cholesteatoma of the tympanic membrane Barbara Pedruzzi, Marta Mion, Francesco ComacchioDepartment of Otolaryngology, Azienda Ospedaliera di Padova, Padova, Italy Case ReportIn 1936, Teed [3] cited the first five cases of ITMC to support the theory of a congenital origin of cholesteatomas. From the English literature, he cited the first case reported by Hinton in 1863 [4] and one other case.We identified a list of potential citations for inclusion in this review, following which the titles and abstracts on this list were screened by two independent reviewers.All the retrieved full-texts were included in the review by consensus of all the authors ( Figure 5).As a quality assessment strategy, the included studies were methodologically appraised according to the National Institute for Health and Clinical Excellence's levels of evidence (Table 1): all the studies could be categorized as evidence level 3.We analyzed the data from these cases, including our own case, to study their demographic details, and clinical features and management, for a total of 42 patients and 20 articles, including our own research (Table 2). Table 2 summarizes the data found in the English literature [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] . 120J Int Adv Otol 2016; 12(1): 119-24 The age range was 5 months to 57 years, with 27 (64.3%) cases involving patients less than 4 years of age, out of which seven (16.7%) were 1-year old or less. There was a history of one or more episodes of otitis media (n=14) when ITMC was noticed on otoscopy. The lesion was first noted by pediatricians (n=14), by general practitioners (n=4), or by otolaryngologists (n=16). In three cases, the analyses were conducted post-mortem during autoptic exams [9] . This highlights the need for routine otoscopy by primary care physicians to detect abnormalities and make an early referral to otolaryngologists. ITMC should always be considered in the differential diagnosis of white lesions in TM. Otomicroscopy can help differentiate ITMC from tympanosclerosis. Only in one case had the lesion occupied the whole TM, while the umbo was involved in nine patients; this region is the most depressed part of TM, where the manubrium of the malleus is fir...

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Section: Discussionmentioning

confidence: 99%

Congenital Intratympanic Cholesteatoma in an Adult Patient: A Case Report and Review of the Literature

Pedruzzi

Mion²,

Comacchio³

2016

Int Adv Otol

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“…As the histology is similar in congenital and acquired cholesteatoma, the distinction is made on clinical grounds. Here in this case we consider it as congenital by the presence of an intact tympanic membrane, no history of ear discharge or otological surgery and the sac limited to the mastoid antrum, so satisfying the criteria proposed by Derlaki and Clemis [4].…”

Section: Discussionmentioning

confidence: 99%

“…On examination congenital cholesteatoma appears as a white round mass medial to an intact tympanic membrane, usually at the anterosuperior quadrant [3]. This epidermoid tissue is considered as congenital cholesteatoma only if it fulfill the criteria of Derlaki and Clemis [4] (i) mass medial to an intact tympanic membrane; (ii) a normal pars tensa and flaccida, (iii) no history of tympanic membrane perforation, otorrhoea, trauma or previous otological procedures, and (iv) prior bouts of otitis media or middle ear effusion are not grounds for excluding possibility of congenital cholesteatoma as it is very rare for a child to have no episodes of otitis media in first 5 years [5,6]. The presentation of congenital cholesteatoma as Bezold's abscess is rare.…”

Section: Introductionmentioning

confidence: 99%

Congenital Cholesteatoma of Temporal Bone with Bezold’s Abscess: Case Report

Janardhan

Peram

Palukuri

et al. 2011

Indian J Otolaryngol Head Neck Surg

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Congenital cholesteatoma is a rare entity. It may originate at various sites in the temporal bone, for example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or in the external auditory canal. The least common site being the mastoid process. Most common presentation is a retrotympanic pearly white mass with no previous history of ear discharge, perforation or any ear surgery. It can lead to various complications, both intracranial and extracranial, some of which may be life threatening. Bezold's abscess is an extracranial complication which is usually seen in children following acute otitis media with mastoiditis. Here we present a rare case of a 60 year old patient with congenital cholesteatoma complicating to Bezold's abscess. After necessary investigations patient underwent surgery for complete removal of cholesteatoma and the abscess drainage.

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“…Congenital cholesteatomas are epithelial remains found in ears with intact tympanic membrane and without previous history of infections 9 . According to Valvassori 10 , they are found in four regions of the temporal bone: tympanic-mastoid, petrous apex, cerebello-pontine angle and jugular foramen.…”

Section: Classificationmentioning

confidence: 99%

Algumas considerações sobre colesteatomas adquiridos pediátricos e adultos

Dornelles

Costa

Meurer

et al. 2005

Rev. Bras. Otorrinolaringol.

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Authors debate about cholesteatomas, from the first time this word was employed, by Muller, in 1838, until the recent updates. They dissert about its definition, etiology and pathology and present basic concepts about its biology. They also make a wide review about pediatric cholesteathoma, its epidemiology and biology, and compare it with adult cholesteatoma. Finally, they describe some articles about ossicle chain erosion and its correlation with cholesteatoma perimatrix, collagen and collagenase.

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LX Congenital Cholesteatoma of the Middle Ear and Mastoid

Cited by 171 publications

References 14 publications

Congenital Intratympanic Cholesteatoma in an Adult Patient: A Case Report and Review of the Literature

Congenital Intratympanic Cholesteatoma in an Adult Patient: A Case Report and Review of the Literature

Congenital Cholesteatoma of Temporal Bone with Bezold’s Abscess: Case Report

Algumas considerações sobre colesteatomas adquiridos pediátricos e adultos

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