DELAYED DIAGNOSIS AND FATE OF CONGENITAL CHOLESTEATOMA (KERATOMA)

Objective: To analyze clinical presentation, modes of detection, growt h pattern, operative findings, and results of surgery in children 3 ye ars old or older who had extensive congenital cholesteatoma (keratoma) . Design: Survey, case series. Setting: Two academically affiliated me dical centers: a children's hospital and an eye, ear, and throat hospi tal, both located in major metropolitan cities.Patient: Twenty-five ch ildren selected according to specified criteria. Intervention: Tympano mastoid surgery, ie, canal wall up and canal wall down, some with ossi cular reconstructive surgery. Main Outcome Measure: Audiologic assessm ent (speech reception threshold) and recurrence (recidivism) of choles teatoma. Results: Incidence of recidivism, 52%. Hearing maintained wit hin the range of normal to mild hearing impairment postoperatively in 91% of the patients for whom complete data are available. Conclusions: Congenital cholesteatoma may grow for years without causing signs or symptoms and, having grown without early detection, can extend to invo lve the epitympanum and mastoid antrum, cause ossicular erosion, and e ven extend to the middle cranial fossa. To adequately remove a congeni tal cholesteatoma that has gone undetected for many years, exposure of the anterior epitympanum is often necessary and removal of both the b ody of the incus and the head of the malleus often is required. Since congenital cholesteatoma usually develops in a child with a well-pneum atized mastoid that would create a large mastoid bowl if exteriorized, the otologic surgeon is likely to hesitate in using the canal wall do wn mastoidectomy technique. Alternatives to the canal wall down mastoi dectomy technique, which can achieve reasonably good hearing results a nd avoid creation of a large mastoid bowl, include planned two-stage c anal wall up surgery or canal preservation with primary reconstruction and close follow-up with otomicroscopy and serial computed tomographi c scans.