Pigmented paravenous retinal choroidal atrophy

Introduction to pigmented venous retinal choroidal atrophy Pigmented paravenous retinoic choroidal atrophy (pigmented paravenousretinochoroidalatrophy) is a rare fundus disease, the etiology and pathogenesis of this disease is unknown. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: chronic angle-closure glaucoma

Causes of choroidal venous retinal choroidal atrophy

(1) Causes of the disease

The cause is unknown.

(two) pathogenesis

The pathogenesis of this disease is unclear, but there are various speculations.

1. Inflammatory factors: Brown believes that it is tuberculosis, and the case reported by Ji Xiuxiang has a history of syphilis.

2. Genetic factors: Morgan and Law believe that it is a congenital change, Brognoli believes that RPE congenital hypoplasia, Wave is considered to be caused by degeneration, Skala believes that the disease may be mainly transmitted through the Y chromosome in men, some have been observed and During follow-up, the patient's visual acuity and visual field were changed. It was confirmed that the disease was a chronic progressive retinal choroidal degeneration. Skalka predicted that the disease may be incomplete retinitis pigmentosa according to changes in ERG and EOG. Most authors believe that this disease may have primary damage to RPE first, followed by secondary choroidal atrophy.

3. Vascular factors: Ishida et al believe that the retinal arterial system may cause arterial thinning due to inflammation or degeneration, retinal dystrophy, RPE cell loss and atrophy, microcirculation disorders, pigment particles free, surrounded by retinal veins.

Prevention of pigmented venous retinal choroidal atrophy

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Complications of pigmented venous retinal choroidal atrophy Complications chronic angle-closure glaucoma

It can be combined with central serous retinal choroidal lesions, angle-closure glaucoma, and macular halo-like choroidal atrophy.

Pigmented venous retinal choroidal atrophy Symptoms Common symptoms The fundus changes the primary atrophy of the nighttime blind optic nerve

1. Visual function change: The disease course is slow, the center vision is good, asymptomatic, no night blindness, often found by chance during routine eye examination, generally with slight visual field changes, but the visual acuity is seriously impaired when the lesion is invaded in the late stage, even May lead to blindness, visual field changes have a circular dark point, centripetal contraction, dark spots corresponding to the atrophic lesion area, dark adaptation to both eyes are reduced, EOG is usually abnormal, can be expressed as a decrease in light peak or disappearance of light peak, and ERG from Normal to complete extinction, suggesting that the photoreceptor of the disease is damaged. In progressive patients, EOG can not record light-induced fast shock and slow shock, suggesting that the entire RPE is damaged.

2. Fundus changes: The main manifestation is the gray-white or blue-gray retinal choroidal atrophy around the optic disc, which extends radially from the optic disc to the peripheral portion of the retinal vein. A large number of osteocyte-like pigmentation is visible on the atrophy and vein surfaces on both sides of the vein. The macular area is generally normal. When the lesion is severe, there may be pigmentation changes around the optic disc. The retinal choroid near the affected area of the vein changes atrophy. The small artery and the rest of the retina show normal, and there is no bleeding or exudation in the fundus.

Examination of pigmented venous retinal choroidal atrophy

1. Fundus fluorescein angiography: shows a wide range of venous RPE defects and mild choroidal abnormalities. Early arterial phase in the angiography is developed in the atrophy zone, the edge of the atrophy zone shows strong fluorescence, and the corresponding pigmented area is weakly fluorescent, around the optic disc and the retina. The atrophic areas on both sides showed strong fluorescence in the early stage, and the fluorescence gradually increased later. The pigmentation in the central part of the lesion was weakly fluorescent, and sometimes a few choroidal vessels were seen in the atrophic area, indicating that the small vessels and capillaries in the choroid of this area have been destroyed. The large abandonment zone in the posterior pole is not fluorescent.

2. Electrophysiological examination: Because it is a PR degenerative disease, abnormal changes in ERG and EOG may occur.

Diagnosis and differentiation of pigmented venous retinal choroidal atrophy

diagnosis

It is not difficult to diagnose the slow progress of the lesion and the diagnosis of fluorescein angiography through the special expression of the ocular fundus with bilateral eyes.

Differential diagnosis

1. Primary retinitis pigmentosa: Although the RPE is first damaged, and the choroidal tissue is involved later, there is no night blindness. The fundus lesions are regularly distributed along the venous blood vessels, and the ERG b wave is significantly decreased, even in the severe development period. It has not completely disappeared, the dark adaptation curve is elevated, and the EOG peak is significantly reduced, so it is a specific type of blanket retinal degeneration.

2. choroidal optic atrophy around the optic disc, convoluted choroidal atrophy, choroiditis, central halo-like choroidal atrophy and fundus vascular streaks, the latter degeneration or stripe-like changes around the optic disc wing or radial extension, but with The progression of the retinal vein has nothing to do.

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