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Case Report

Adult Congenital Cholesteatoma


Maesyara Adinda Sari, Harry A Asroel

Introduction

Congenital cholesteatoma is defined by Derlacki and Clemis as an


embryonic rest of epithelial tissue in the ear without tympanic membrane
perforation and without a history of ear infection. Levenson et al. have
modified the definition of a congenital cholesteatoma to include a normal
pars flaccida and pars tensa, no history of prior otorrhea, and no history
prior to otologic procedures. Prior episodes of otitis media without otorrhea
are not criteria for excluding congenital origin (Lim, Yoon, Kang 2012 ;
Meyer, Strunk Jr., Lambert 2014).

Congenital cholesteatoma manifest as a whitish mass with intact


tympanic membrane. it is a relative rare disease consisting of
approximately 2% of the entire cholesteatoma cases. Recently, with the
increase of interest on this disease, its detection rate is increased, and
thus the ratio of congenital cholesteatoma to the entire cholesteatomas
has been reported to be from 3.7% to 24% (Park et al. 2009).

The diagnosis criteria for congenital cholesteatoma include an


intact tympanic membrane and the absence of a history of otologic
procedures, otorrhea, perforation or granulation. Concerning the location
of congenital cholesteatoma, Levenson et al. have reported that it is
located primarily in the anterior area of the tympanic cavity (Park et al.
2009 ; Yamatodani et al. 2013)

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Recurrences rates of congenital cholesteatoma should be lower
than acquired forms of the disease since the pathophysiology does not
involve eustachian tube dysfunction (Goh et al. 2010).

The treatment for congenital cholesteatoma is surgery (Goh et al.


2010).

In this case report, we describe a woman who presented with a


congenital cholesteatoma of the right ear and we treated with canal wall
down mastoidectomy surgery.

Case Report

A 48-year-old female came to ENT departement of private general


hospital on January 1st 2016 with headache in the last 1 year as a chief
complaint. She also complained hearing loss on the right ear. There was
history of vertigo and there was no history of otorrhea, trauma or facial
asymmetry. We suggested patient to had canal wall down mastoidectomy
surgery but the patient refused.

A month later, on 2nd February she came to ENT departement of the


same private general hospital with the same headache as the chief
complaint but it became worse and heavy.

Physical examination of the oropharynx and nose were normal. On


otologic examination revealed there is destruction at superoposterior
external auditory canal, tympanic membrane was intact (Fig. 1). There is
no fistula at postauricular region (Fig. 2). The left ear was normal.

The temporal CT Scan showed an opacity of the right and left


mastoid air cells, normal right and left external or internal auditory canal,
and there is no bone destruction. Conclusion : Bilateral Mastoiditis.

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Perioperative hematologic laboratory finding and chest x-ray was normal
(Fig. 3).

Fig. 1 Tympanic membrane was intact and destruction at superoposterior


external auditory canal,

Fig. 2 Normal postauricular region

The patient was diagnosed by clinical history and physical


examination as congenital cholesteatoma on right ear. Patient underwent
canal wall down mastoidectomy surgery under general anesthesia on
February 3rd 2016.

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After desinfection of the left postauricular region with povidone
iodine and alcohol 70%, a marker was made on the 0,5 cm from sulcus
retroauricular. Subcutis infiltration with pehacaine dilution was done along
the marker (Fig. 4). A curvelinear incision was made at the marker from
helical rim to mastoid tip, from the cutis and subcutis layer until loose
areolar tissue. Later, we had incision toward posterior ear canal. Each the
superficial and deep temporal muscle fascia was harvest as a graft. A T-
shaped incision was then made on the soft tissue over the mastoid. The
soft tissue was elevated by resparatorium until the entire mastoid cortex
and spine of Henle was exposed (Fig. 5).

Fig. 3 CT Scan imaging of temporal bone showed bilateral


mastoiditis

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The mastoid cortex was exposed with retractor. The posterior wall
ear canal, temporal line and spine of Henle were identified. Then we
started drilling mastoid cortex at MacEwen triangle area. The drilling was
continued until the antrum. We observed mastoid and tympanic cavity
under the microscope. We found matrix of cholesteatome in superior
region of the tympanic cavity and we saw a destruction at superoanterior
region that continue to the external ear canal (Fig. 6). Then we did a canal
wall down. The eustachian tube was plugged with bone that we harvested
from the temporal bone itself. And the last, we did a meatoplasty. Then the
postauricular wound was closed layer by layer and the external auditory
canal was packed with cotton wick soaked in antibiotic ointment.

Fig. 4 Subcutis infiltration with pehacaine dilution

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Fig. 5 mastoid cortex and spine of Henle was exposed

After surgery, the patient was given antibiotic, steroid, analgetic, H2


antagonist and antihaemorrhagic, all the medication administrated
intravenously. There was no facial nerve paresis. The suture was taken off
at 4-6 days after sugery.

Fig.6 perforation at superoposterior region that continue to the


external ear canal

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Discussion

Congenital cholesteatoma is a relatively rare disease, defined as a


whitish mass lesion in the middle ear cleft behind an intact tympanic
membrane early in life. The term congenital has been used rather
conventionally because the pathogenesis of congenital cholesteatoma
remains unclear and suggested hypotheses range from formation during
fetal period to an acquired condition in infant (Lim, Yoon, Kang 2012).

Otoscopy typically shows a pearly white mass, medial to the intact


tympanic membrane, primarily in the anterosuperior quadrant, without a
preceding history of otorrhoea, tympanic membrane perforation or surgery.
It can present at any age from birth to early adulthood. Congenital
cholesteatoma may be asymptomatic or present as a conductive hearing
loss. Other presentations include otalgia, vertigo and facial palsy. The later
symptoms indicate erosion into the semicircular canals or the Fallopian
canal, respectively. Symptom of headache and fever may herald
impending intracranial complication (Mahanta et al. 2007 ; Mattingly &
Chan 2016).

The pathogenesis of congenital cholesteatomas is incompletely


understood. Congenital cholesteatoma is diagnosed on the basis of a
specific set of criteria : (1). Cholesteatoma sac medial to an intact
tympanic membrane, (2). A normal tympanic membrane, and (3). No
history of tympanic membrane perforation, otorrhoea, trauma or surgery
(Mahanta et al. 2007 ; Meyer, Strunk Jr., Lambert 2014).

In this patient we found a normal tympanic membrane and there


was no history of tympanic membrane perforation, otorrhoea, trauma or
previous surgery. Althought in physical examination of the ear we found
there was external ear canal destruction at the superoposterior region.

The majority of cases involve the anterosuperior quadrant although


they can arise from other parts of the tympanic membrane. If located in the

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posterosuperior quadrant, Congenital cholesteatoma can involve the
ossicular chain even in the absence of a conductive hearing loss. As the
histology is similiar in congenital and acquired cholesteatoma, the
distinction is made by the presence of an intact tympanic membrane
perforation or surgery (Mahanta et al. 2007)

In this case, it is located in the posterosuperior quadrant and we


suspended that there was ossicular chain involved because this patient
complained about hearing loss which is in accordance with if it is located
in the posterosuperior quadrant, it can involve the ossicular chain.

Postic et al. classified congenital cholesteatoma of the midle ear in


4 stages. Stage I denotes disease limited to only one quadrant. Stage II
means disease involving multiple quadrants without involvement of the
ossicles or mastoid. Stage III represent ossicular involvement with no
disease in the mastoid. stage IV disease occupying the mastoid (Solmaz
et al. 2015).

Based on Postic et a. classification, this patient classified in to


stage III, which is represent ossicular involvement because of the patient
complained hearing loss and there was no disease in the mastoid.

The treatment for congenital cholesteatoma is surgery. the goal of


the surgery is complete removal of disease with restoration of hearing if
possible (Goh et al. 2010).

Conclusion

We have reported a case of adult congenital cholesteatoma in a 48-


year-old female with a headache as achief complaint. She also
complained about hearing loss on the right ear. From the history and

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physical examination we diagnosed patient with adult congenital
cholesteatoma. We managed this patient with radical mastoidectomy
surgery and meatoplasty with a good result.

References

Goh B, Faizah A, Salina H, Asma A, Saim L. 2010. Congenital


Cholesteatoma : Delayed Diagnosis and its Consequences Medical
Journal of Malaysia.65(3).pp.189-91

Lee J., 2012. Noninfectious Disorder of the Ear Essential Otolaryngology


Head and Neck Surgery, 10th Edition, pp.338-350

Lim H, Yoon T, Kang W. 2012. Congenital Cholesteatoma : Clinical


Features and Growth Patterns American Journal of Otolaryngology
Heand and Neck Medicine and Surgery.33.pp.538-42

Mattingly J and Chan K. 2016. Cholesteatoma ENT Secret. 4th edition.


pp.278-81

Meyer T, Strunk Jr C and Lambert P. 2014. Cholesteatoma Baileys Head


& Neck Surgery Otolaryngology. 5th Edition. pp..2433-45

Mahanta V, Uddin F, Mohan S, Sharp J. 2007. Non-classical Presentation


of Congenital Cholesteatoma Tann R Coll Surg Engl.pp.1-3

Park K, Park S, Chang K, Jung M, Yeo S. 2009. Congenital Middle Ear


Cholesteatoma in Children; Retrospective Review of 35 Cases.
Journal Korean Medical Science, 24. pp. 126-31

Yamatodani et al. 2013. Congenital Middle ear Cholesteatoma :


Experience From 26 surgical Cases. Annals of Otology, Rhinology &
Laryngology. 122(5). pp.316-21

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Solmaz F, Akduman D, Haksever M, Gundogdu E, Mescioglu A. 2015.
Atypical Presentation of Congenital Cholesteatoma in Adult Case
with Good Hearing Result Annals of Medicine and Surgery. 4. pp.26-
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