Surgery for Pediatric Cholesteatoma Treatment & Management

Medical therapy is not an accepted treatment for cholesteatoma. Patients who refuse surgery or those with a medical condition that makes a general anesthetic too hazardous should have the ear cleaned regularly, usually over 2-3 months. Regular cleaning can help control infection and may slow growth, but it does not stop further expansion or eliminate risk. The mainstay of antimicrobial therapy should be topical, but systemic therapy is occasionally a helpful adjunct.

Surgical therapy consists of removal of the cholesteatoma. As mentioned in History, 2 types of operations are commonly used for the management of cholesteatoma. The open cavity (ie, canal wall down) technique was used exclusively until the House Otologic Group introduced the closed cavity (ie, canal wall up) technique. Various factors are considered in determining which procedure is best in a given circumstance.

Sometimes, this decision cannot be made until the operation has begun and a clear understanding of the extent of disease has been obtained. Open cavity operations have the highest probability of permanently ridding the patient of cholesteatoma. Closed cavity procedures have the advantage of maintaining a normal appearance but have a higher risk of persistent or recurrent cholesteatoma. The risk of persistence is sufficient to cause most surgeons to advise an obligatory "second look" tympanomastoidectomy 6-12 months following the initial operation if a closed cavity procedure is performed.^[6] During the second look, traces of residual disease frequently can be removed prior to the development of either complications or massive recurrence.

• In some circumstances, the surgeon is fairly sure preoperatively which operation (closed or open cavity) to perform, especially in the following situations:^[7]

  • If the patient has had several episodes of the cholesteatoma and wishes to avoid future operations, the open cavity technique is most applicable.
  • For patients who are unwilling or unable to return for a second look procedure, an open cavity operation is safer.
  • A large meatoplasty is simply unacceptable under any circumstance for some patients. These patients can be treated with a closed technique if they understand that disease recurrence is more likely and that they may require multiple serial surgical procedures.The photo exhibits a large meatoplasty performed as part of an open cavity (canal wall down) mastoidectomy. A similar meatoplasty usually is necessary if a clean, dry, problem-free cavity is to be maintained.
• Some congenital anomalies are known to be associated with a lifelong history of eustachian tube dysfunction. In some individuals, previous surgical procedures have irreversibly injured the eustachian tube. In such individuals, an open cavity operation often is best.
• Some intraoperative findings favor an open technique:

  • The sinus tympani is involved.
  • The medial end of the canal wall is involved, with the cholesteatoma wedged laterally between the heads of the ossicles in the epitympanum and medial canal wall.
  • Ostitis or irremovable cholesteatoma in the area around the opening to the Eustachian tube or in the most inferior portions of the middle ear space often calls for a true radical mastoidectomy.
  • Although small defects in the canal wall are readily repaired, larger defects are harder to repair. Converting the operation to an open cavity technique is best.
  • Labyrinthine fistula is present. If the cholesteatoma matrix is left over the fistula so as not to expose it, then the canal must be removed. Otherwise, epithelium is trapped within the mastoid cavity, which simply results in cholesteatoma recurrence. If the cholesteatoma matrix is removed from the fistula, some surgeons are comfortable leaving the canal wall up.
• In the United States, most surgical procedures for cholesteatoma now are performed through an incision behind the ear combined with an incision in the external auditory canal, but the procedure can be performed through an extended incision starting in the canal alone.
• Regardless of which technique is used, all air cells should be removed. If ossicles are involved, they must be removed to avoid recurrence.^[8] The ossicular chain can be reconstructed either at the end of the primary procedure or as part of a secondary operation.
• The TM is usually reconstructed. If a closed cavity technique is used, missing bone should be replaced with a cartilage graft. If an open cavity procedure is selected, a large meatoplasty should be created to allow adequate air circulation into the cavity that remains.

• Consultation with an otolaryngologist is mandatory.
• Rarely, advice from a neurosurgeon is required to help manage an intracranial consultation.