Congenital Cholesteatoma - Endaural Approach Tympanoplasty

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Author: Roxane Machavoine
Published:
Specialties: Neurotology Otology, Pediatric Otolaryngology
Schools: Hôpital Universitaire Necker Enfants Malades, Université Paris Descartes
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Procedure

Introduction:

Cholesteatoma in pediatric population must be divided into two categories: acquired and congenital cholesteatoma.
The first is the consequence of chronic otitis with tympanic retraction. Craniofacial malformations such as clefts or Down syndrome are risk factors of tubal dysfunction and tympanic retraction. These patients must have close otologic follow-up regarding the important risk of cholesteatoma.
Congenital cholesteatoma is a retrotympanic epidermic cyst with a normal eardrum, and without otorrhea, tympanic perforation and otologic surgical past.
The mean age of diagnostic is 5,6 years old.

The case we present is of a 3 years old patient presenting with a retrotympanic white mass, incidental finding during routine otoscopy.
The CT scan reveals an atrial convex opacity of 5mm without any ossicular lysis.
These clinical and radiological data are in favor of a congenital cholesteatoma.
A type II tympanoplasty is performed to remove the cholesteatoma and restore hearing.

Methods:

A two-channels (orbicularis oculi and oris) neuromonitoring of the facial nerve was installed.
Minimal endaural incision and circular external canal incision were performed.
The tympanomeatal flap and annulus were elevated inferiorly and posteriorly to enter in the middle ear.
The antero-superior part of the external canal was notched.
The cholesteatoma extended from the attic to the atrium and supratubal recess. Moreover, the ossicular chain was mobile and undamaged.
Due to the large size of the cholesteatoma, the incudostapedial articulation was dislocated, the chorda tympani was sectioned, and the incus and malleus were removed. The cholesteatoma was partially debulked to facilitate removal.
The stapes was undamaged, the footplate was mobile and the middle ear mucosa was healthy.
Endoscopic examination of the middle ear did not finde any residual of cholesteatoma.
A tragal cartilage graft was harvested and used to reconstruct the superior part of the external canal.
A second cartilage graft was used to between the stapes and the first graft to restore hearing.
The tympanomeatal flap was put back and foam was used to rebuild the anterior tympanomeatal angle.
A pop-oto-wick into a silicon roll and a paraffine tulle were inserted to avoid external canal stenosis.

Results:
The patient was discharged the next day. The paraffin tulle was removed at first clinical control one week from the surgery. Two weeks after the surgery the oto-wick and the silicon roll were removed. No early complications were reported
An audiologic control was scheduled for a three months later.

Conclusion:
Large congenital cholesteatoma often require ossicular and chorda tympani sacrifice for a complete removal to avoid residual.
A double-block tragal cartilage is a simple and efficient ossiculoplasty technique to restore hearing in the pediatric population.

Indications

Cholesteatomas must be removed considering the possible complications as facial paralysis, meningitis, hearing and vestibular loss.

Contraindications

Active otitis externa, acute otitis media and general contraindications.

Instrumentation

Setup

The operation is performed under general anesthesia with the patient in supine position. The head is tilted 20° sideways.
The procedure is undertaken with a surgical microscope except the endaural incision and closure.

Preoperative Workup

General and otologic history is collected including otologic past with neonatal hearing screening, recurrent otitis media and previous otologic surgery.
The clinical exam includes an otoscopy and hearing assessment (pure tone audiometry and speech audiometry).
A CT scan is performed to confirm the clinical suspicion of cholesteatoma, to evaluate the extensions of the mass and to identify key anatomic landmarks (lateral sinus, intrapetrous portion of the internal carotid artery, facial nerve, ossicular chain, inner ear).

Anatomy and Landmarks

Advantages/Disadvantages

Complications/Risks

Tympanoplasty is generally well tolerated and outcomes are simple.
The major surgical risks are lesion of facial nerve, meningeal breach, inner ear traumatism.
A transient vertigo or dysgeusia may happen.
Patients or parents must be advised that after cholesteatoma removal, hearing could be worse than before surgery. Also, a second look can be necessary to remove residual cholesteatoma or to perform an ossiculoplasty.

Disclosure of Conflicts

No disclosure of conflicts.

Acknowledgements

References

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