Purpose: To report the occurrence of unilateral iris neovascularization in children secondary to medulloepithelioma.
Methods: Presenting features and the clinical course of patients confirmed to have medulloepithelioma were reviewed.
Results: Seven patients with medulloepithelioma had iris neovascularization during the clinical course. Associated cataract and lens coloboma occurred in two and three cases, respectively. In six (86%) cases, no apparent cause for iris neovascularization could be detected at presentation. Two cases had tube shunts for management of neovascular glaucoma before medulloepithelioma was recognized. All cases eventually required enucleation.
Conclusion: Presence of iris neovascularization is an early manifestation of medulloepithelioma. Children with iris neovascularization of unknown cause should be evaluated to exclude underlying medulloepithelioma.