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

Abstract

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...

Intratympanic membrane cholesteatoma after traumatic tympanic membrane perforation: a case report

“…The etiology of ITMC remains unclear [ 2 , 6 , 7 ]. ITMC can occur due to iatrogenic or traumatic tympanic membrane perforation.…”

“…In patients with recurrent otitis media and local inflammation, metaplasia within the tympanic membrane may be induced, resulting in ITMC [ 2 ]. ITMC may develop from basal cell layer proliferation of the tympanic membrane into protruding cones due to an inflammatory process [ 4 , 6 ].…”

“…They reported that ITMC could be congenital because most patients were young children, with a mean age of 3 years, and there was a significant positive correlation between patient age and ITMC size, indicating that the congenital cholesteatoma gradually grew over time [ 1 , 2 ]. Reports have suggested that the persistence of embryonic epithelial rests, which do not disappear after they contribute to tympanic membrane and tympanic ring development, could result in ITMC [ 6 ]. In this case, the patient had been aware of the ITMC after the traumatic tympanic membrane perforation and was 12 years of age at diagnosis.…”

“…ITMC may also spontaneously disappear if it ruptures into the EAC in the early stage and the keratin debris is completely discharged [ 7 – 9 ]. In comparison, several reports mentioned that surgical removal should be performed as soon as possible because ITMC could increase in size and expand into the middle ear [ 2 , 6 ]. If ITMC would rupture or enlarge into the middle ear, it could result in middle ear cholesteatoma [ 8 ].…”

The Significance of Staging in the Treatment of Congenital Cholesteatoma in Children

“…Because the growth of CC is closely linked to patient age and delays in detection and treatment may facilitate extension of the disease, early diagnosis, and surgical intervention is crucial. 9-11…”