A Case of Retinal Astrocytic Hamartoma Causing Blindness in Tuberous Sclerosis.

La, Tae Yoon; Kim, Chul Woo; Lee, Youn Soo; Kim, Min Ho

Case Report

Journal of the Korean Ophthalmological Society 1999;40(5):1421-1426.
Published online May 1, 1999.

A Case of Retinal Astrocytic Hamartoma Causing Blindness in Tuberous Sclerosis.

Tae Yoon La, Chul Woo Kim, Youn Soo Lee, Min Ho Kim

Department of Ophthalmology, Kangnam St. Mary's Hospital, College of Medicine, The Catholic University of Korea.

결절성 경화증에서 시력 상실을 일으킨 망막의 성상세포 과오종 1예

나태윤(Tae Yoon La),김철우(Tae Yoon La),이연수(Youn Soo Lee),김민호(Min Ho Kim)

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.

Key Words: Retinal hamartoma;Tuberous sclerosis