CHORIORETINAL COLOBOMAS IN A PEDIATRIC POPULATION

Objective: The purpose of the study was to determine the prevalence of retinal detachment and associated anomalies in pediatric patients wit h chorioretinal colobomas, Design: Observational case series, Particip ants: Forty-eight patients (86 eyes) of patients with chorioretinal co lobomas followed in two academic pediatric eye clinics were reviewed. Main Outcome Measures: The frequency of associated systemic abnormalit ies and the prevalence of retinal or choroidal detachment during the f ollow-up period.Results: Forty-eight patients (86 eyes) were identifie d; ocular involvement ranged from small colobomas with isolated chorio retinal involvement to large colobomas affecting the iris, choroid, re tina, and optic nerve. The mean ages at initial and most recent examin ations were 27 and 100 months, respectively. Follow-up averaged 6 year s, 1 month. Six retinal detachments were found in four patients. Patie nt ages at the time of detection of retinal detachment were 5 months, 29 months, 10 years, and 15 years. A choroidal detachment was found in a child at age 9 years. The combined prevalence of retinal or choroid al detachment was 10.4% of patients and 8.1% of affected eyes, Thirtee n eyes were microphthalmic, and six had microcornea. Eighteen patients (38%) had other systemic abnormalities. Conclusions: The 8.1% prevale nce of retinal or choroidal detachment among eyes in our series was mu ch smaller than previously cited estimates of 23% to 43%. The higher f igures probably reflect a referral bias toward patients with vitreoret inal problems and an older patient population. Because of the acknowle dged difficulty of managing retinal detachments in patients with chori oretinal colobomas, emphasis should be directed toward early detection and possible prophylactic therapy. The high proportion of patients wh o have associated systemic abnormalities indicates the need for thorou gh evaluation of these children by other pediatric specialists.