[Follow-up of proliferation of juxtapapillary retinal pigment epithelium]

Bengin proliferation of the juxtapapillary retinal pigment epithelium is a rare but important tumor because it may be mistaken for a malignant lesion such as choroidal melanoma.

A 9-year-old male patient consulted our clinic first in 1991 because of a reduction in visual acuity due to a tumor of the optic nerve head in the left eye. Visual acuity was 1.0 in the right eye and 0.2 in the left eye. Slit lamp examination and intraocular pressure were regular in both eyes. Kinetic perimetry showed a enlarged blind spot besides regular isopters. Funduscopy revealed a pigmented, ill-defined prominence of the optic nerve head with a mossy surface. In annual examinations were found a slowly progredient reduction in visual acuity up to 0.04 durning the last visit in January 2000 as well as a enlargement of the blind spot due to an increasing prominence and pigmentation of the optic nerve head. Because of the clincal aspect, the slowly progressive course, the enlargement of the blind spot with deterioration of vision and especially the florescein angiography, which revealed peripapillary capillary alterations with leakage, we diagnosed a proliferation of the juxtapapillary retinal pigment epithelium.

The proliferation of the juxtapapillary retinal pigment epithelium can be differentiated from other pigmented tumors of the optic nerve head by its typical clinical aspect and course and especially by using flourescein angiography.