Blood-aqueous barrier function in a patient with combined hamartomas of the retina and retinal pigment epithelium.

Abstract

Clinical data showing breakdown in the blood-aqueous barrier in patients with combined hamartomas of the retina and retinal pigment epithelium are very limited. We used a laser flare-cell meter to examine blood-aqueous barrier function in a 35-year-old male patient who had typical combined hamartomas of the retina and retinal pigment epithelium characterized by an ill-defined, slightly elevated, grey-white epipapillary lesion with overlying glial proliferation and surrounding vascular tortuosity. Fluorescein angiography showed early hypofluorescence corresponding to the pigmented area with speckled hyperfluorescence in the lesion. Prominent retinal vascular tortuosity and late dye leakage were noted in the late phase. Visual evoked potential demonstrated poor waveform. Laser photometry showed an increase in the aqueous flare intensity in the affected eye, as compared to the results in the unaffected eye and to data from normal subjects. The increase in the aqueous flare intensity in this patient suggests that the function of the blood-aqueous barrier may be affected in combined hamartomas of the retina and retinal pigment epithelium.