Objective: To observe clinical features of Retinitis pigmentosa with retinal vascular occlusion and its prognosis.
Methods: To analyze the clinical Data in 18 cases retrospectively using fundus examination, fundus fluorescein angiography, indocyanine green angiography, electroretinogram, visually evoked potential etc. Gene screening was performed in 3 cases.
Results: the major clinical manifestations of the disease were optic atrophy, vascular attenuation to obliteration, widespread retinal pigment epithelium atrophy with depigmentation and/or fine pigment spots, total or nearly total a and b wave were extinct in the examination of electroretinogram. All this manifestations were compatible with that of typical Retinitis Pigmentosa (tapeto-retinal dystrophy). It also had its unique features, such as total or nearly total vascular obliteration, marked optic atrophy in later stage, and choroidal vessels abnormal. Gene mutation was not found in gene encoding area of RHO gene of No: 3 chromosome and of RLBPI gene of No: 15 chromosome. vision loss in this kind retinitis pigmentosa is much faster than that of typical retinitis pigmentosa.
Conclusion: Retinitis pigmentosa with retinal vascular occlusion may belonged to a kind of tapeto-retinal dystrophy, vascular progressive obliteration was probably its associated disease.