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A cholesteatoma is a benign skin tumor, usually occurring in the middle ear and mastoid spaces. Sometimes it can be difficult to diagnose early through the hand-held otoscope. The characteristic glistening white growths can appear as vague, irregular, unremarkable whitish structures that resemble tympanosclerosis cholesteatoma, colesteatoma, cholestetoma.
A cholesteatoma is a hamartomatous- like, keratin-producing squamous epithelial lesion that is constructed very much like an onion, in layers. The bulk of these tumors consists of desquamated skin deposited into a core of shed dead cells called the matrix. The outer shell or surface is the only viable tissue and may represent only 1% to 2% of the tumor.
The danger associated with these lesions arises from their location and biologic activity. These epithelial skin structures are found misplaced in the mucosally lined middle ear, mastoid spaces, subcutaneous spaces of the tympanic membrane, or the canal wall. Part of their destructive nature is due to the powerful lytic lysozymes in the matrix that can dissolve soft tissues and bone. Adjacent tissue also is destroyed by direct pressure of the
There are at least two major types of cholesteatomas based on their etiology: congenital and acquired. Congenital cholesteatomas can be defined as an embryonic nest of epithelial tissue in an ear that has no tympanic perforation or history of chronic ear disease. They usually appear as a small
Congenital cholesteatomas commonly are thought to arise from nests of embryonic epithelial tissue, but data also support an alternative theory. Amniotic fluid that contains viable shed epithelial cells from the skin can reflux from the nasopharynx during fetal swallowing and up the eustachian tube into the middle ear.
Acquired cholesteatomas usually break through the tympanic membrane and present as white debris or with drainage in the ear canal. They are subdivided into three subtypes, based on their origins from retraction pockets, chronic infection, or
Retraction pockets are the most common mechanism for the formation of cholesteatoma (Figs. 1 (Figure Not Available) , 2 (Figure Not Available) , and 3) . They arise following prolonged eustachian tube dysfunction when the weakest part of the tympanic membrane, the pars flaccida, is sucked into the middle ear and eventually buds off as an autonomous ball of squamous epithelial tissue. The mechanism for chronic inflammation causing
Congenital cholesteatomas can present as only a small white ghost of a lesion, usually behind the midanterior inferior quadrant of the intact tympanic membrane of a smaller child. These frequently are asymptomatic. Acquired cholesteatomas have a classic recurrent draining nature that subsides or disappears during a course of antibiotics and resumes at the conclusion of therapy. They may leave a trail of white debris or present as an angry-looking, red, bloody, granulomatous polyp when there is secondary infection.
An otolaryngologist should be consulted if a cholesteatoma is suspected or if there is an unusual lesion in a child's ear. Surgery always is required and usually entails drilling out the lateral wall of the mastoid cavity of the skull behind the ear canal. Usually a new tympanic membrane must be applied, but the damaged ossicles typically are not repaired or replaced until a second-look operation is performed 6 to 8 months later. This surgery is performed to look for recurrence, the rate of which can be as high as 50%, even after good treatment. The type of operation is tailored to the extent
At best, a 50% cure rate is achieved with appropriate treatment, but in some cases, the tumor is so extensive that externalizing it in a radical mastoidectomy is the only possible approach. In these patients, the posterior canal wall, tympanic membrane, and ossicles all are removed to provide convenient periodic access to the area for removal of the cholesteatoma debris so it will not expand and extend intracranially. Although cure may be difficult to achieve, a