A Case of Retinal Astrocytic Hamartoma Causing Blindness in Tuberous Sclerosis

ABSTRACT

Tuberous sclerosis is a inherited systemic disorder which is well known as classic triad of epilepsy, adenoma sebaceum and mental retardation. Fifty to eighty-seven percent of patients with this disorder manifest retinal hamartoma in eyeball, but this tumor is known to be never malignant and rarely cause blindness. This is a very unusual case that retinal hamartoma of 5-year old boy with tuberous sclerosis was complicated with neovascular glaucoma, vitreous hemorrhage, and cataract, and that very severe ocular pain and visual loss developed. We resected the tumor through pars plana vitrectomy but the tumor recurred in 3 weeks, and the affected eye was finally enucleated. However, there were no malignant findings and only benign findings of hamartoma which is composed of astrocyte with long process, oval nuclei and eosinophilic cytoplasm on histologic examination. So we report this unusual case with review of available literature.