Birth prevalence of visually significant infantile cataract in a defined U.S. population

PURPOSE: To determine the birth prevalence of visually significant infantile cataract, using population-based comprehensive medical record retrieval, in a defined US population. DESIGN: Retrospective, population-based, medical record retrieval. METHODS: We reviewed records of all pediatric patients 0-17 years) coded as cataract during a 20-year period (1978 to 1997) using the resources of the Rochester Epidemiology Project. "Infantile cataract" was defined as a cataract diagnosed within the first year of life. "Possible infantile cataract" was defined as a cataract, diagnosed after the first year in a child born in Olmsted County, where there was no evidence of an acquired traumatic, acquired systemic, or acquired ocular etiology. Visually insignificant cataracts were excluded. RESULTS: Ten incident cases of visually significant infantile cataract were identified during the 20-year study period, yielding a birth prevalence of 3.0 per 10,000 live births (95% CI: 1.5-5.6 per 10,000). Five additional cases of visually significant "possible infantile cataract" were identified, diagnosed between the ages of 2 and 8 years. Inclusion of these "possible infantile cataracts" would result in an estimate of overall birth prevalence for visually significant infantile cataract of 4.5 per 10,000 live births (95% CI: 2.5-7.5 per 10,000). CONCLUSIONS: Using population-based medical record retrieval methods, we estimate the birth prevalence of visually significant infantile cataract to be 3.o to 4.5 per 10,000. Infantile cataracts are an important cause of visual impairment in children and these data are useful in planning clinical trials and allocating health care resources.