| Clinical study
of congenital cholesteatoma of the middle ear.Nippon
Jibiinkoka Gakkai Kaiho. 2004
Nov;107(11):998-1003.
We studied 35 ears of 34 patients
with congenital cholesteatoma who were operated between June 1992 and
May 2003, focusing on the localization and progression of congenital
cholesteatoma. Patients were 2 to 55 years of age. Congenital
cholesteatoma was diagnosed based on two intraoperative findings: 1)
no continuity between the tympanic membrane and cholesteatoma, 2) no
presence of perforation, retraction, or granulation of the tympanic
membrane. All patients had closed-type cholesteatoma, and none formed
open-type cholesteatoma, which grows as a flat sheet of epidermis. The
primary site of congenital cholesteatoma was classified into 3 types;
1) anterior-superior quadrant, 2) posterior-superior quadrant, and 3)
epitympanic, and the origin of these three types of congenital
cholesteatoma was difficult to explain by a single theory. We operated
on 31 ears by canal wall up tympanoplasty and on 4 ears by canal wall
down tympanoplasty. On all but 4 ears, we performed planned-staged
operation, including second-look operations, in case of recurrence or
residual cholesteatoma. Improvement in hearing after surgery was seen
in 22 of the 26 ears treated and followed up for more than 6 months
after surgery. By drawing all localization of congenital cholesteatoma
in 35 ears, we studied the progression of congenital cholesteatoma and
speculated on the original primary site. Congenital cholesteatoma in
restricted areas consequently implies good results in hearing after
surgery, making earlier diagnosis and treatment desirable.
Occult contralateral congenital
cholesteatoma: is the epidermoid formation theory enough?Am
J Otolaryngol. 2004 Jul-Aug;25(4):285-9
Bilateral congenital
cholesteatomas are rare entities. Nine cases have been previously
described in the literature. Many different etiologies for the
development of congenital cholesteatoma have been proposed. The case
of a five-year-old boy with bilateral congenital cholesteatoma is
discussed. A lesion of the left ear was apparent clinically. However,
the right-sided lesion was silent and was demonstrated only by
radiologic exam. Theories of pathogenesis are reviewed.
Clinical evaluation of congenital
cholesteatoma of the middle ear.Nippon
Jibiinkoka Gakkai Kaiho. 2003 Aug;106(8):797-807.
We conducted a retrospective
study to identify the clinical features and surgical observations of
congenital cholesteatoma. Sixty patients were diagnosed and underwent
surgery for congenital cholesteatoma between April 1987 and May 2002.
All diagnoses were made on the basis of two operative findings: 1. the
tympanic membrane manifested neither retraction, perforation, nor
granulation. 2. the tympanic membrane was not continuous with the
cholesteatoma. In this series, congenital cholesteatoma accounted for
7% of all cholesteatomas (853 ears). The patient age ranged from 2 to
48 years. The male to female ratio was 4:1. Seventeen patients had
multiple cholesteatoma. Fifty-three patients exhibited closed-type
cholesteatomas, while the remaining 7 patients had open-type
cholesteatomas that had formed as a flat surface of the epidermis.
Patients with open-type cholesteatomas presented with a much more
pronounced conductive hearing loss and ossicular erosion or
malformation. Twenty-two patients with relatively small cholesteatomas
were analyzed to estimate the origin of their cholesteatomas. Of the
22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had
posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at
the time of detection was older in the PSQ-type group than in the ASQ-type
group and the frequency of ossicular erosion or malformation was more
prominent in the PSQ-type group than in the ASQ-type group. The
primary site of origin was thought to be the portion between the
tympanic ostium of the auditory canal and the semicanal for tensor
tympani in the ASQ-type group and near the incudostapedial joint in
the PSQ-type group. A planned staged procedure was performed in 29
patients, 15 patients (52%) had residual lesions situated mostly on
the oval window, the round window, an exposed facial nerve or an
exposed lateral semicircular canal. The frequency of residual lesions
in patients who presented with extended, multiple cholesteatoma and
those with ossicular malformation was comparable to the frequency of
patients who did not present with these features.
A staging system for
congenital cholesteatoma.Arch
Otolaryngol Head Neck Surg. 2002
Sep;128(9):1009-12.
OBJECTIVE: To develop a
staging system for congenital cholesteatoma in predicting the
likelihood of residual disease. DESIGN: Retrospective analysis of data
from a case series, to identify predictors of residual disease.
SETTING: Tertiary care pediatric hospital. PARTICIPANTS: Children
undergoing surgical removal of congenital cholesteatoma. There were
156 patients, with 160 cholesteatomas; 4 children had bilateral
disease. INTERVENTIONS: Each case was scored as to quadrants of the
middle ear involved, ossicular involvement, and mastoid extension.
MAIN OUTCOME MEASURE: Surgically confirmed residual disease at any
time after the initial procedure. RESULTS: Four stages were defined as
follows: stage I, disease confined to a single quadrant; stage II,
cholesteatoma in multiple quadrants, but without ossicular involvement
or mastoid extension; stage III, ossicular involvement without mastoid
extension; and stage IV, mastoid disease. There was a strong
association between stage and residual disease, ranging from a 13%
risk in stage I to 67% in stage IV. CONCLUSIONS: This simple staging
system may be particularly useful in standardizing the reporting of
congenital cholesteatoma and in adjusting for severity in evaluating
outcomes. It also provides information that is useful in counseling
parents.
Congenital cholesteatoma:
classification, management, and outcome.
Arch Otolaryngol Head Neck Surg. 2002
Jul;128(7):810-4.
OBJECTIVES: To assess
whether a classification system for congenital cholesteatoma (CC) can
be derived from analysis of a large clinical sample of cases and to
assess whether such a classification system is a reliable guide for
surgical intervention, reexploration, and hearing outcome. DESIGN: A
retrospective review of clinical and surgical records of 119 patients
with CC. SETTING: Four tertiary care children's hospitals. PATIENTS:
One hundred nineteen children with CC (age range, 2-14 years).
RESULTS: Congenital cholesteatomas in the anterior mesotympanum were
treated successfully with exploratory tympanotomy. Congenital
cholesteatomas involving the posterior superior quadrant and the attic
usually had concurrent involvement of the incus and stapes and often
required a canal wall up tympanomastoidectomy and a second look for
its control. Congenital cholesteatoma involving the mastoid usually
involved all of the ossicles, was inconsistently controlled with canal
wall up tympanomastoidectomy, and had a poor prognosis for restoration
of conductive hearing loss. The mean +/- SD age of children with CC
was 5.6 +/- 2.8 years, while that of children with acquired
cholesteatoma was 9.7 +/- 3.3 years. CONCLUSIONS: The sequence of
spread of CC, involving 3 sites, suggests a natural classification
system. The CC usually originates in the anterior superior quadrant,
but does not consistently remain there, and may variably occupy the
middle ear and mastoid and result in ossicular destruction and
conductive hearing loss. The location of CC and the involvement of the
ossicles is an accurate predictor of the type of surgery necessary for
its control and for the success of hearing restoration.
The natural history of
congenital cholesteatoma.Arch
Otolaryngol Head Neck Surg. 2002
Jul;128(7):804-9.
OBJECTIVES: To describe the
natural history of congenital cholesteatoma (CC) and to determine
whether such a description provides clues about the origins and end
points of these lesions. DESIGN: A retrospective qualitative analysis
of intraoperative illustrations of 34 consecutive patients with 35 CCs
(1 bilateral). SETTING: Two tertiary care children's hospitals.
PATIENTS: Thirty-four children with CC, mean age, 5.6 years (range,
2-13 years). RESULTS: Congenital cholesteatoma originates generally,
but not universally, in the anterior superior quadrant. The
progression of growth is toward the posterior superior quadrant and
attic and then into the mastoid. Contact with the ossicular chain
generally results in loss of ossicular continuity and in conductive
hearing loss. CONCLUSIONS: Congenital cholesteatoma appears to have a
predictable trajectory of growth, starting as a small pearl in the
middle ear, eventually growing to involve the ossicles and mastoid,
and causing varying degrees of destruction and functional impairment.
The clinical picture of a young child with otorrhea, conductive
hearing loss, tympanic membrane perforation in a nontraditional
location, and a mastoid filled with cholesteatoma may represent the
end point in the natural history of CC, despite the fact that this
type of lesion is outside the accepted definition of CC.
A new pathogenesis of mesotympanic
(congenital) cholesteatoma. Laryngoscope 2000 Nov;110(11):1890-7.
OBJECTIVES: To introduce a
new, acquired pathogenetic theory of mesotympanic cholesteatoma behind
an intact eardrum in children and to present some doubts on congenital
pathogenesis. STUDY DESIGN: Literature review. METHODS: The incidence
and origination of mesotympanic cholesteatoma in children were
thoroughly analyzed in the world literature and correlated to the
histopathological studies on human middle ear epithelia and to
epidemiological studies on secretory otitis, tubal occlusion, and
acute suppurative otitis media. RESULTS: The new, acquired theory is
based on the fact that that the place of origin of the anterosuperior
mesotympanic cholesteatoma is the area of the malleus handle and
malleus neck, and of the posterosuperior cholesteatoma, the long
process of the incus. During the common pathological conditions there
is a great risk of retractions and adhesions of the eardrum to these
ossicles. After subsequent loosening of the retracted eardrum some
cells of the keratinized squamous epithelium may be left behind and
become included into the tympanic cavity, eventually causing an
inclusion cholesteatoma. Four basic mechanisms of inclusions are
proposed and the presence of great dynamics in middle ear disease in
children, with high incidence of tubal dysfunction, retractions,
secretory otitis, and acute suppurative otitis, is documented, making
the acquired pathogenesis probable. The place of origin does not fit
with the congenital pathogenesis of epithelial formation localized on
the lateral wall of the eustachian tube close to the annulus. The
origination around the malleus and incus fits better with the proposed
acquired pathogenesis. CONCLUSIONS: There are no definitive proofs for
the acquired pathogenesis of the mesotympanic cholesteatoma, nor is
there experimental research to prove or disprove it. Mesotympanic
cholesteatoma, congenital cholesteatoma, acquired pathogenesis of
mesotympanic cholesteatoma, cholesteatoma in children, cholesteatoma
behind intact eardrum.
Congenital cholesteatoma of
the ear in the child. Clinical, follow-up and therapeutic analysis of
a series of 34 cases.
Ann Otolaryngol Chir Cervicofac. 1999
Sep;116(4):218-27.
Long a subject of debate,
congenital cholesteatomas of the middle ear appear to be a specific
clinical entity different from the much more frequent classical
acquired cholesteoma. Characteristic features of congenital
cholesteatomas are young age at diagnosis, typical peroperative
presentation, satisfactory mastoid air cells in almost all cases, and
associated congenital malformations, which may involve the otology
system or not. Diagnosis is a difficult task due to the long latency
period with no clinical manifestations. These congenital
cholesteatomas appear to be more aggressive in a mastoid with
functioning air cells. Thus open excision does not appear to be
appropriate and should be reserved for selected cases. For us, the
closed technique with two procedures is more adapted but requires good
cooperation with the family. The risk of recurrence is however
significant and at least comparable to that of acquired cholesteatomas
in children. Follow-up should be persuade as long as possible.
Functional results have been encouraging even though ossicular
destruction is frequent. The quality of the auditory tube appears to
be a determining factor.
MRI and CT in the
evaluation of acquired and congenital cholesteatomas of the temporal
bone. J
Otolaryngol. 1993 Aug;22(4):239-48.
Acquired
(secondary) cholesteatomas of the middle ear and mastoid are usually a
complication of chronic otomastoiditis and are often accompanied by
infection from the outset, and their contents show evidence of some
inflammatory reaction. Congenital (primary) cholesteatomas of the
temporal bone are due to epithelial rest of embryonal origin. There
are many sites of occurrence of congenital cholesteatomas (epidermoids)
within the temporal bone: (1) middle ear, (2) mastoid, (3) middle ear
and mastoid, (4) petrous bone, (5) the squama, and (6) within the
tympanic membrane. Intradural (cisternal) congenital cholesteatomas
are another type of cholesteatomas that often involve the
cerebellopontine angle (CPA) region and cause varying degrees of
cochlear and vestibular symptoms and signs. In this paper, we stress
the role of magnetic resonance imaging (MRI) and computed tomography
(CT) in the evaluation of acquired and congenital cholesteatomas of
the temporal bone. It is our opinion that CT remains the study of
choice for cholesteatomas of the middle ear cleft. MRI is superior to
CT for the evaluations of infected cholesteatomas, petrous apex, and
CPA cholesteatomas, as well as for the majority evaluation of
cholesteatomatous involvement of the facial nerve, membranous
labyrinth, and intracranial structures.
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