[Long-term clinical course and changes in the choroidal circulation in the foveal region in a case of juvenile retinitis pigmentosa with bilateral cystoid macular edema].

PURPOSE: To report the long-term clinical course of a case of juvenile retinitis pigmentosa (RP) with bilateral cystoid macular edema (CME) which was subjected to sequential investigation with laser Doppler flowmetry (LDF) to evaluate the choroidal blood flow (CBF) in the foveal region. CASE: A 17-year-old man with RP was referred for a full examination. His best-corrected visual acuity (VA) was 1.0 in the right eye and 0.9 in the left eye. At the initial examination, retinal pigment epithelium degeneration and obvious CME were noted in the both eyes. LDF revealed a CBF of 19.8 (a. u.) in the right eye and 14.2 (a. u.) in the left. Three years and 6 months later, the VA decreased to 0.8 in the right eye and 0.4 in the left, and the CBF decreased to about 50% of the initial values in either eye. Vitrectomy was performed for bilateral CME. 6 years and 2 months after the initial visit, the VA decreased to 0.1 in the right eye and 0.05 in the left eye, showing a marked decrease of the CBF to 2.5 (a. u.) in the right eye and 1.6 (a. u.) in the left eye, about 12% of the initial values. CONCLUSION: We describe a case of bilateral CME due to RP in which sequential LDF was performed. We theorize that the worsening of VA and the decrease of CBF advanced simultaneously and gradually in our patient.