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Ear Pathology Online

Pathology of Congenital Cholesteatoma

Dr Sampurna Roy MD    


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Congenital cholesteatoma is a rare entity. The aetiopathogenesis of this lesion is still unknown.

An embryologic origin is hypothesized when cholesteatoma develops in patients without previous history of otitis.

Acquired Cholesteatoma

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.

The primary site of congenital cholesteatoma was classified into 3 types;

1) anterior-superior quadrant,

2) posterior-superior quadrant, and

3) epitympanic.

According to some studies the cases were scored as to quadrants of the middle ear involved, ossicular involvement, and mastoid extension.

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.

Two important operative findings:

- 1. the tympanic membrane manifested neither retraction, perforation, nor granulation.

- 2. the tympanic membrane was not continuous with the cholesteatoma.

Gross: Most common presentation was that of an asymptomatic white mass behind a normal intact tympanic membrane.

Computed tomography (CT) scan was useful in documenting extension beyond the mesotympanum.

These spherical, whitish lesions/cystic structures measure 3 mm or more in diameter.

The lesions may enlarge to become indistinguishable from acquired cholesteatomas.

Histopathological features: Temporal bone histopathological studies of some cases of congenital cholesteatoma demonstrated two distinct pathological types of congenital cholesteatoma.

- A "closed" keratotic cyst in the anterior mesotympanum, which is easily removed, and

- An "open" infiltrative type in which there is no containment of the keratotic debris and the cholesteatoma matrix is in direct continuity with middle ear mucosa. Surgical extirpation of the "open" type is difficult and more likely to be associated with residual disease.

Congenital cholesteatomas show a thinner and flatter matrix than acquired cholesteatomas, probably because the former are most frequently 'closed' and therefore subject to pressure effects from the keratin within the cyst. 'Open' forms also occur in smaller numbers.

It is possible that a screening program for congenital cholesteatoma in infants might reduce the incidence of the severe, extended form of the disease.

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.

Characteristic features of congenital cholesteatomas:

The patients 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.

MRI can be of particular help in distinguishing congenital cholesteatoma from cholesterol granuloma.


Further reading

Clinical features, presenting symptoms, and surgical results of congenital cholesteatoma based on Potsic's staging system.

Congenital middle ear cholesteatoma: experience from 26 surgical cases.

A staging system for congenital cholesteatoma.

Clinical study of congenital cholesteatoma of the middle ear

Occult contralateral congenital cholesteatoma: is the epidermoid formation theory enough?

Clinical evaluation of congenital cholesteatoma of the middle ear.

Congenital cholesteatoma: classification, management, and outcome.

The natural history of congenital cholesteatoma.

A new pathogenesis of mesotympanic (congenital) cholesteatoma.

Congenital cholesteatoma of the ear in the child. Clinical, follow-up and therapeutic analysis of a series of 34 cases.

Congenital cholesteatoma of the tympanic membrane.

Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report.

Congenital cholesteatomata with other anomalies.


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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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