Introduction. Congenital cholesteatoma is a pearly white mass that rarely originates from the mastoid process. Case Report. A 21-year-old male patient presented to our department with severe right mastoid pain and postauricular fluctuant swelling for 23 days. There was no preceding history of ear complaints and examination showed a normal right ear drum. Emergency exploration of the mastoid process was done on the same day and revealed localized cholesteatoma limited only to the mastoid cavity. Conclusion. Despite a rarity, the mastoid process should be always put in mind as a site of origin for congenital cholesteatoma

Hazem M Abdel Tawab

Naveen Kumar Gopal

Salim M Sloma Tabook

CiteSeerX

Case Report
Congenital Cholesteatoma Localized to the Mastoid Cavity and
Presenting as a Mastoid Abscess
Salim M. Sloma Tabook,1 Hazem M. Abdel Tawab,1,2 and Naveen Kumar Gopal1
1Department of Otorhinolaryngology, Sultan Qaboos Hospital, Salalah, Oman
2Department of Otorhinolaryngology, Faculty of Medicine, Cairo University, Giza, Egypt
Correspondence should be addressed to Hazem M. Abdel Tawab; hazemabdeltawwab77@yahoo.com
Received 18 February 2015; Revised 1 April 2015; Accepted 6 April 2015
Academic Editor: Chung-Feng Hwang
Copyright © 2015 Salim M. Sloma Tabook et al.This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Introduction. Congenital cholesteatoma is a pearly white mass that rarely originates from the mastoid process. Case Report. A 21-
year-old male patient presented to our department with severe right mastoid pain and postauricular fluctuant swelling for 23 days.
There was no preceding history of ear complaints and examination showed a normal right ear drum. Emergency exploration of the
mastoid process was done on the same day and revealed localized cholesteatoma limited only to the mastoid cavity. Conclusion.
Despite a rarity, the mastoid process should be always put in mind as a site of origin for congenital cholesteatoma.
1. Introduction
Congenital cholesteatoma (CC) is a pearly white keratinized
stratified squamous epithelium that arises in the middle
ear cleft. Some diagnostic criteria had been suggested to
differentiate it from the acquired cholesteatoma. According
to Levenson et al.’s revision, congenital cholesteatoma is a
pearly white mass medial to an intact tympanic membrane
with normal pars tensa and pars flaccida, with no history
of ear discharge or ear drum perforation or any otological
procedure [1].
Congenital cholesteatoma may originate from five dif-
ferent sites in the temporal bone: the petrous bone, the
cerebellopontine angle, the middle ear cavity, the external ear
canal, and the mastoid process. The mastoid process appears
to be the least affected and the rarest site that congenital
cholesteatoma may arise from [2].
In this study, we present a rare case of mastoid abscess
as the only presentation of congenital cholesteatoma in the
mastoid process.
2. Case Report
A 21-year-old male Yemeni patient presented to the Otorhi-
nolaryngology Department at Sultan Qaboos Hospital in
Salalah,Oman, with a history of rightmastoid pain of twenty-
three- day duration that did not respond to multiple different
courses of antibiotics. No preceding history of upper respira-
tory tract infection was found. The patient did not complain
from diminution of hearing or tinnitus or previous history
of ear discharge or operations. No history of ear trauma
was presented by the patient. The full otorhinolaryngological
examination was done. The right ear drum was intact with
normal appearance together with the right external auditory
canal with no signs of congestion or inflammation. The left
ear was normal and tuning fork tests were having within
normal results. The right postauricular region showed a
tender fluctuant cystic swelling, oval in shape and measuring
2.5 × 3 cm.
The overlying skin was congested red but not attached to
the underlying swelling. A defect in the mastoid bone had
been felt during the examination. The rest of examination of
the ears and the rest of nose and throat examination were
normal.
X-ray mastoid Schuller view was done and revealed
opacification of the right mastoid with a picture of large
mastoid cavity (Figure 1).
A decision was made for emergency right mastoid explo-
ration to drain the abscess and evaluate the cause.
Hindawi Publishing Corporation
Case Reports in Otolaryngology
Volume 2015, Article ID 305494, 3 pages
http://dx.doi.org/10.1155/2015/305494
2 Case Reports in Otolaryngology
Figure 1: Opacification and large mastoid cavity as seen in the
patient’s X-ray mastoid.
Informed consent was taken from the patient after expla-
nation of the details of the surgical procedure.
Under general anesthesia, a right postauricular incision
was done and surprisingly a rapid gush of pus appeared after
incision of the periosteum. The bony defect was identified
and widened. A large pearly white sac with whitish flakes
had been seen completely filling the mastoid cavity and
widening the mastoid antrum and also encroaching on the
facial ridge and reaching posteriorly to the sigmoid sinus and
posterosuperiorly to the sinodural angle (Figure 2).
The sac was completely delivered in total (Figure 3), and
the wound was closed after insertion of a drain in the large
cavity left after removal of the sac.
The specimen was sent for histopathology that confirmed
the diagnosis of cholesteatoma.The patient had no complica-
tions intraoperatively or in the postoperative period. Stitches
and ear pack had been removed ten days after the operation
and the patient had been followed up for three months later
on with clean wound site and no recurrence of the swelling.
3. Discussion
Different studies and theories have been suggested to explain
the origin of congenital cholesteatoma as metaplasia theory
[3], invagination theory [4], epithelial rest theory [5], and
implantation theory [6].
It is somehow difficult to apply the metaplasia theory in
our case in this paper as it is strange thatmetaplasiamay affect
the mastoid process leaving a completely normal middle ear
cavity. This is supported by Friedberg’s study in 1994 [6].
The invagination theory of the ectoderm can be applied;
however, it is extremely rare. Also, the epithelial rest theory
can be applied if epidermoid formation happens to the
underdeveloped mastoid during the fetal life and grows later
into congenital cholesteatoma yet this theory is also rare [7].
The implantation theory might be accepted in our case.
Canalis et al., in 2002, suggested that cranial cholesteatoma
can arise with entrapment of squamous epithelium in the
suture during the period ofmastoid fontanelle closure leading
Figure 2: Largely pearly white mass expanding the mastoid cavity.
to the formation of congenital cholesteatoma in the mastoid
process [8].
The most accepted theory for the development of con-
genital cholesteatoma is the epithelial cell rests. It depends
on Teed’s initial observation of an epidermal structure in
a 5-month human fetus in “the dorsal lateral pole of the
tympanum, just medial to the neck of the malleus” [9].
These rests are ectodermal implants in the fusion plates
between the first and second branchial arches that appear
around 10 weeks at the junction of the first branchial cleft and
pouch systems [10].
Levenson et al. [1] postulated that these rests helped in the
middle ear and tympanic membrane development and that
they are initially dormant. These epithelial cell rests undergo
rapid proliferation before resorption around 33 weeks of
gestation. In cases where incomplete resorption is the case, it
is thought that congenital cholesteatoma will form. Levenson
et al. [1] postulated that the epidermal rests fail to undergo
involution because of continuous and chronic irritation.
Michaels [11] confirmed these rests of epithelial cells his-
tologically in 54% of the fetal temporal bones examined and
postulated that their persistence was the cause of congenital
cholesteatoma.
Mastoid abscess was the first presentation of congen-
ital cholesteatoma in a 21-year-old male patient in our
study. Migirov et al. presented seven cases with mastoid
subperiosteal abscess as the first presentation of congenital
cholesteatoma in the pediatric population, of whom two
patients presented with normal intact tympanic membrane.
All the seven patients had no history of middle ear disease
[12].
Hidaka et al., in 2010, reported an adult case with
acute mastoiditis as the first presentation of congenital
cholesteatoma with no extension in the attic or aditus ad
antrum as seen in the operation and suggested that any
adult with mastoiditis should be evaluated for congenital
cholesteatoma. They also mentioned that, including their
case, only four cases till that time presentedwithmastoid pain
or swelling [13]. Another presentation of mastoid congenital
Case Reports in Otolaryngology 3
(a) (b)
Figure 3: (a) shows the sac after delivery from the mastoid cavity before its removal. (b) shows the sac.
cholesteatoma mentioned in the literature was a stricture in
the external auditory canal with intact tympanic membrane
[14].
According to Lee et al. and Hong et al.’s reviews in 2007
and 2014, about 30 cases of mastoid congenital cholesteatoma
existed [7, 15] towhichwe add the case presented in this study.
Conflict of Interests
The authors have no conflict of interests.
References
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Presenting as a Mastoid Abscess

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Presenting as a Mastoid Abscess

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