The Turkish Journal of Pediatrics
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Tympanic Membrane Cholesteatoma: A Rare Finding
Sinan Atmaca, Ender Seçkin, Mehmet Koyuncu
Department of Otolaryngology and Head and Neck Surgery, Ondokuz Mayıs University Faculty of Medicine, Samsun,
|We report a rare finding of tympanic membrane cholesteatoma in a twoyear-
Tympanic membrane cholesteatoma without trauma or surgery to the ear
is a rare entity, with few cases documented in the literature. The exact
etiology of this lesion is still unclear. The presentation, clinical course and
management are discussed.
A whitish spot on the tympanic membrane should raise suspicion for
cholesteatoma. Early diagnosis and treatment are imperative to allow an easy
removal and avoid middle ear involvement.
cholesteatoma, tympanic membrane, congenital.
|Congenital cholesteatoma was first described
by House1 in 1953. Derlacki and Clemis
proposed the clinical criteria to establish
the diagnosis of middle ear (ME) congenital
cholesteatoma, which were later revised by
Levenson et al. (Table I). The incidence of
congenital cholesteatoma of the ME comprises
2% to 5% of all cholesteatomas4-6. Congenital
cholesteatomas arising from the tympanic
membrane (TM) are even less common4,6-11.
Although the number of studies regarding
congenital cholesteatoma of the ME has
increased over the years, there are few reports
on TM cholesteatoma.Cholesteatoma
should be considered in the differential
diagnosis of white TM lesion.
Early treatment is essential to avoid progressive
destruction. We present a two-yearold
girl with left TM cholesteatoma located in
the posterior-inferior quadrant.
| ||Table I. Criteria for Definition of Congenital
Cholesteatomas by Levenson et al.|
|Case Presentation |
|A two-year-old girl was referred to our
outpatient department by her pediatrician for
further investigation of the whitish spot on the
left TM. Otomicroscopic examination revealed
the presence of a white pearl, measuring 2 mm
in diameter, located at the posterior-inferior
quadrant of the TM (Fig. 1). The parents denied
history of otitis media, ear trauma, otorrhea,
or prior otologic surgery. Tympanograms
were A type with intact acoustic reflexes
bilaterally, and the parents believed she was
responding appropriately to sound stimuli. A
high resolution computed tomography of the
temporal bones showed a round-shaped tissue
located inside the TM without involvement
of the ossicles or the ME space. Surgery was
performed through a transcanal approach, and
the cholesteatoma pearl was peeled off from
the TM (Fig. 2). After removal, the fibrous
layer of the TM was intact and no further
action was taken. Histopathology confirmed
a cholesteatoma. At the three-month followup,
otoscopic examination was normal with
no signs of recurrence, and the tympanogram
was type A as well.|
| ||Fig 1. White pearl located at the posteriorinferior
quadrant of the left tympanic membrane.|
Congenital cholesteatoma is now a relatively
common diagnosis due to the recognition of the
disease by otolaryngologists and pediatricians,
but congenital cholesteatoma arising from the
TM is very uncommon. There are several
theories as to the pathophysiology of congenital
cholesteatomas. The “epithelial rest” theory
postulates that persistence of epidermoid
formations, derived from the first branchial
groove, at the junction of the Eustachian tube
| ||Fig. 2. White pearl measuring 2 mm in diameter
and ME, results in congenital cholesteatomas. The “metaplasia” theory supports
transformation of inflamed ME mucosa into
stratified squamous epithelium. These
two theories may explain the formation of
congenital cholesteatomas of the ME, but the
origin of the TM cholesteatomas may be best
explained by Ruedi’s theory. He postulated that
small inflammatory injuries to the TM in utero
produce small perforations in the epithelium,
through which the squamous epithelial basal
layer proliferates into protruding cones. These
cones could then form TM cholesteatomas if
retained within an intact TM. Our case also
supports Ruedi’s theory with the involvement
of the epithelial layer of the TM with an intact
fibrous layer. Tympanoplasty with an overlay
or a combined overlay-underlay technique
can be an important source of acquired TM
cholesteatoma. A whitish spot on the TM
should raise suspicion for cholesteatoma. Otomicroscopy is the gold standard
for differentiating TM cholesteatoma from
tympanosclerosis. Surgery is mandatory
because these lesions may potentially involve
the ossicles and ME. In our case,
early diagnosis and treatment before violation
of the fibrous layer provided easy removal of
the disease and avoided a myringoplasty with
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