[The characteristics of retinitis pigmentosa with retinal vascular occlusion].
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.


CONCLUSIONS
Retinitis pigmentosa with retinal vascular occlusion may belonged to a kind of tapeto-retinal dystrophy, vascular progressive obliteration was probably its associated disease.
Wang
Guang-lu
GL
Beijing Tongren Ophthalmic Center, Capital University of Medical Sciences, Beijing 100730, China. continent23@163.com
Lu
Ning
N
Zhang
Feng
F
Peng
Xiao-yan
XY
Li
Yang
Y
Wang
Ming-yang
MY
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D015356:Retinal Artery Occlusion / Q000150:complications / Q000175:diagnosis*; D012174:Retinitis Pigmentosa / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies