Primary infantile glaucoma in an Australian population

Background: Primary infantile glaucoma presents rarely, but can be responsible for significant visual morbidity. There is little information on the clinical features and visual outcome of a pure population of primary infantile glaucoma, as opposed to a mixed population of primary and secondary glaucoma or combined group of those with trabeculodysgenesis and iridotrabeculodysgenesis. Methods: We conducted a retrospective review of children with primary infantile glaucoma seen in south-eastern Australia between 1980 and 2000, using The Royal Children's Hospital ophthalmic diagnostic coding database. Results: Fifty-one patients with primary infantile glaucoma were identified (83 eyes). This equates to an estimated incidence of approximately 1 in 30 000 births. The mean ± SD age at presentation was 135 ± 84 days. 'Burnt-out' disease (megalocornea without raised intraocular pressure) was diagnosed in 10.8%. Goniotomy was the most commonly performed surgical procedure (69.4% of 72 eyes). Surgical success with one or two goniotomies was achieved in 74% of eyes. Visual outcomes at final review were generally good with 61.8% reading 6/12 or better. There were a disproportionately high number of children having a final recorded acuity of <6/60 in the group diagnosed in the first 3 months of life. Conclusions: Primary infantile glaucoma is a rare ocular condition in this population that presents at a mean age of 4.4 months. Surgical and visual outcomes are generally favourable.