Cholesteatoma




Keywords

temporal bone, cholesteatoma, middle ear, external auditory canal

 




Introduction


A cholesteatoma is a cystic mass filled with keratin and lined by stratified squamous epithelium. Cholesteatoma is not a neoplasm and can be thought of most simply as skin in the wrong place. Although a cholesteatoma is histologically identical to an epidermoid or epidermal inclusion cyst, the term “cholesteatoma” is preferred for a lesion located in the middle or external ear ( Fig. 42.1 ).




Figure 42.1


Artist’s rendering of sites of cholesteatoma within the middle ear and external ear. Site 1 represents an acquired pars flaccida cholesteatoma in Prussak space. This is the most common site of origin of acquired cholesteatoma. Site 2 represents an acquired pars tensa cholesteatoma. Site 3 represents a congenital cholesteatoma, and site 4 represents a cholesteatoma of the external auditory canal.


Cholesteatomas may be either congenital (~2%) or acquired (~98%). Acquired middle ear cholesteatomas are further subcategorized into those associated with the pars flaccida (more than 80%) of the tympanic membrane and those associated with the pars tensa (<20%). Although the pathogenesis of acquired cholesteatoma remains controversial, the most widely accepted theory is the “retraction pocket theory,” which states that dysfunction of the eustachian tube leads to the buildup of negative pressure in the middle ear such that a segment of the tympanic membrane (most commonly the pars flaccida) is drawn into the middle ear and becomes sequestered, followed by accumulation of the trapped keratin.


Histologically, cholesteatoma is divided into three layers—the cyst contents, the matrix, and the perimatrix. Keratinous debris represents the primary component of the central cyst, and it is the central cystic contents that give the lesion its mass. The central cyst is surrounded by the matrix layer, which consists of stratified squamous epithelium. Although not neoplastic, this squamous epithelium is hyperproliferative, and the keratinized matrix is commonly colonized by bacterial biofilm. The perimatrix is the outermost layer and consists of granulation tissue containing collagen, fibrocytes, and inflammatory cells such as histiocytes and neutrophils.


Osseous erosion ( Fig. 42.2 ) is the hallmark of cholesteatoma and occurs primarily as the result of osteoclastic activity. Additionally, pressure effects promoting osseous remodeling and enzymatic activity of the perimatrix may play a role. Osteoclastic activity is believed to be upregulated by proinflammatory cytokines released by the perimatrix inflammatory cells in response to the presence of bacterial biofilm and/or existing inflammation. In addition to stimulating osteoclastic bone resorption, these cytokines also stimulate further squamous proliferation, feeding a vicious cycle of cholesteatoma enlargement and further osseous erosion.




Figure 42.2


Osteoclastic activity is primarily responsible for the erosive and destructive osseous changes characteristic of cholesteatoma. Osteoclastic activity is stimulated by cytokines, which are upregulated by local inflammation and bacterial colonization. In addition to osteoclastic activity, pressure erosion and perimatrix enzymatic activity may also play a role.


The symptoms and complications of cholesteatoma are primarily related to the associated osseous erosive changes. The ossicles are most commonly eroded, resulting in conductive hearing loss. As the cholesteatoma progresses, the facial nerve canal, tegmen, and lateral semicircular canal may become involved, which can result in facial paralysis, cephalocele, and labyrinthine fistula, respectively.


The treatment for cholesteatoma is complete surgical removal. If any squamous epithelium is left behind or introduced at the time of surgery, the cholesteatoma will recur.




Temporal Evolution: Overview


The skin is continuously regenerating and is estimated to turn over every 40 to 60 days. Normally, the replaced skin cells are shed to the environment. When skin fragments are confined to a space, such as the middle ear, the normal process of regeneration may continue, but the dead cells and keratin cannot be shed. As a result, the natural course of cholesteatoma is to slowly enlarge with time, progressively filling the space in which it is confined. Left untreated, cholesteatoma will fill the middle ear and mastoid in a predictable manner based on (1) the site of origin and (2) the path of least resistance.




Temporal Evolution: in Greater Depth


Pars Flaccida


Pars flaccida cholesteatomas begin in Prussak space ( Fig. 42.3 ), which is bordered by the pars flaccida laterally, the neck of the malleus medially, the lateral process of the malleus inferiorly, the lateral malleal ligament superiorly, and the anterior malleal ligament anteriorly.




Figure 42.3


Coronal cone beam computed tomography image demonstrates Prussak space (asterisk) and its borders—the lateral malleolar ligament (curved arrow) , the pars flaccida of the tympanic membrane (straight arrow) , the lateral process (LP) of the malleus, and the neck (N) of the malleus.


Enlarging cholesteatomas follow characteristic paths of least resistance determined by boundary-forming mucosal folds, ossicular suspensory ligaments, and the ossicles themselves ( Fig. 42.4 ). There is no posterior boundary to Prussak space, and enlarging cholesteatomas most commonly first extend posteriorly. Upon reaching the posterolateral wall of the attic, further expansion of the cholesteatoma is deflected superiorly toward the aditus ad antrum and mastoid antrum. As the cholesteatoma fills the aditus ad antrum, the adjacent lateral semicircular canal is at risk. Erosion of the bony covering of the lateral semicircular canal results in a labyrinthine fistula ( Fig. 42.5 ). This complication occurs in 5% to 10% of patients with cholesteatoma and must be recognized and reported by the radiologist, as its presence alters both the operative plan and the prognosis.




Figure 42.4


A 74-year-old female with right conductive hearing loss. The patient declined surgery for right cholesteatoma, instead electing for observation. On the initial study, there is a small amount of soft tissue in Prussak space with associated mild blunting of the scutum (A and C, straight arrows ). The soft tissue passes posteriorly (B, arrow ) and superiorly from Prussak space toward the aditus ad antrum (B, asterisk ). Note the well-aerated anterior epitympanum (A, circle ), facial nerve recess (−) , and sinus tympani (+) . Four years later, there is increased soft tissue within the lateral epitympanum, which now extends posteriorly to partially opacify the facial nerve recess (D). Additionally, the cholesteatoma has passed through the aditus ad antrum to opacify the mastoid antrum (E). Note that the sinus tympani and anterior epitympanum (D) as well as the oval window (D and F, red circle ) are well aerated. Six years after the initial study, there has been a continued progressive increase in cholesteatoma. Note the progressive erosion of the scutum (G and I, curved arrows ) with associated widening of the lateral wall of the attic. Additionally, the anterior epitympanum, facial nerve recess, and sinus tympani (G) as well as the oval window (H and I) are completely opacified.



Figure 42.5


A 37-year-old female with history of previously resected left cholesteatoma now presenting with left otalgia and decreased hearing. As compared to the prior postoperative study, the current coronal noncontrast CT image of the left temporal bone demonstrates increased soft tissue opacification of the attic and mastoid antrum, increased erosion of the scutum (red circle) , and new defects in the lateral semicircular canal (curved arrow) and tegmen tympani (straight arrow) . The new erosive changes are consistent with recurrent cholesteatoma with associated complications.


As the cholesteatoma enlarges, it may extend inferiorly from Prussak space between the tympanic membrane and the posterior malleal fold to reach the posterior mesotympanum, typically filling the oval window niche, round window niche, sinus tympani, and facial recess ( Fig. 42.6 ). As the cholesteatoma fills the oval window, involvement of the adjacent tympanic segment of the facial nerve by cholesteatoma may result in facial paralysis. Additionally, the bony covering of the tympanic segment of the facial nerve is frequently congenitally dehiscent. If such a dehiscence is not recognized and reported prior to surgical removal of an adjacent cholesteatoma, iatrogenic facial paralysis may occur.




Figure 42.6


A 12-year-old male with a history of cholesteatoma status post prior canal wall down tympanomastoidectomy. The axial computed tomography (CT) image from the initial study demonstrates rounded soft tissue filling the left middle ear, sinus tympani (asterisk) , and round window niche (+) ; this tissue was surgically removed and proven to be recurrent cholesteatoma. The postoperative 2-year follow-up axial CT image demonstrates reaccumulation of rounded soft tissue within the left sinus tympani (circle) , which was subsequently removed and also shown to be recurrent cholesteatoma.

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Jun 26, 2019 | Posted by in NEUROLOGICAL IMAGING | Comments Off on Cholesteatoma
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