Surgery for Pediatric Cholesteatoma Clinical Presentation

• The hallmark symptom of cholesteatoma is unremitting (or frequently recurrent) painless discharge from the ear (ie, otorrhea).
• Hearing loss is also a common symptom of cholesteatoma. Large cholesteatomas fill the middle ear space with desquamated epithelium and interfere with sound transmission. Associated ossicular damage also can produce or magnify a conductive loss.
• Dizziness is a relatively uncommon symptom of cholesteatoma but occurs if bony erosion produces a labyrinthine fistula or if the cholesteatoma lies directly on the footplate of stapes. Dizziness is a worrisome symptom because it may presage the development of more serious complications.
• Occasionally, cholesteatoma presents initially with the symptoms of CNS complications, sigmoid sinus thrombosis,^[3] epidural abscess, or meningitis.

• Upon physical examination, the most common signs of cholesteatoma are drainage and granulation tissue that are unresponsive to antimicrobial therapy.
• Tympanic membrane (TM) perforation commonly accompanies cholesteatoma and is present in more than 90% of patients.

  • Congenital cholesteatomas are the exception.
  • Occasionally, a cholesteatoma produced by surgical implantation of squamous epithelium presents prior to disruption of the TM.
• Quite frequently, the only finding upon physical examination is a canal filled with mucopus and granulation tissue.

  • Occasionally, the infection can be temporarily avoided with administration of ototopical antibiotic drops.
  • When ototopical therapy is successful, the surgeon often can observe a deep tympanic retraction pocket in the pars flaccida (the portion of the TM superior to the short process of the malleus) or in the posterior quadrant of the TM.
• Very rarely, a cholesteatoma is initially identified on the basis of one of its complications.

  • An infection associated with the cholesteatoma can erupt through the inferior mastoid cortex and present as an abscess in the neck.
  • Occasionally, cholesteatoma initially manifests with the signs of central nervous complications, sigmoid sinus thrombosis, epidural abscess, or meningitis.

The etiology of cholesteatoma varies, and at least 3 types are identified with different pathophysiologies.

• Congenital cholesteatomas are caused by embryonic squamous epithelial rests.^[4]

  • Congenital cholesteatomas are usually found in the anterior mesotympanum or in the peri eustachian tube area.
  • They are most commonly identified in early childhood (ie, age 6 mo to 5 y).
  • As congenital cholesteatomas expand, they can obstruct the eustachian tube, thus producing chronic middle ear fluid and conductive hearing loss.
  • They can also produce conductive hearing loss by expanding posteriorly to encase the ossicular chain.^[5]
  • Unlike other forms of cholesteatoma, congenital cholesteatomas are often identified behind an intact and normal-appearing TM.
• Primary acquired cholesteatomas are caused by TM retraction.

  • The classic primary acquired cholesteatoma is a result of increasingly severe medial retraction of the pars flaccida into the epitympanum.
    • As this process continues, the lateral wall of the epitympanum (ie, scutum) is slowly eroded, producing an enlarging defect in the lateral wall of the epitympanum.
    • The medially retracting TM continues to pass over the heads of the ossicles and into the posterior epitympanum. Ossicular destruction is common.
    • If the cholesteatoma passes posteriorly through the aditus ad antrum into the mastoid, erosion of the tegmen mastoideum with exposure of the dura and/or erosion of the lateral semicircular canal with deafness and vertigo can result.
  • A second type of primary acquired cholesteatoma arises when the posterior quadrant of the TM is retracted into the posterior middle ear.
    • The drum initially adheres to the long process of the incus.
    • As retraction continues medially and posteriorly, squamous epithelium envelops the superstructure of the stapes and retracts into the sinus tympani.
  • Primary acquired cholesteatomas arising from the posterior TM are likely to produce facial nerve injury and destruction of the stapedial superstructure.
• Secondary acquired cholesteatoma is a consequence of injury to the TM.

  • Injury to the TM can be a perforation that occurs as a result of acute otitis media or trauma, or it can be caused by surgical manipulation of the drum.
  • Posterior marginal perforations are most likely to result in cholesteatoma formation.
  • Although considered unlikely to produce cholesteatomas, central perforations occasionally can result in cholesteatoma formation.
  • A procedure as simple as the insertion of tympanostomy tubes can implant squamous epithelium into the middle ear; this ultimately grows to produce a cholesteatoma.
  • Any deep retraction pocket can result in cholesteatoma formation, if the retraction pocket becomes deep enough to trap desquamated epithelium.