Central halo choroidal atrophy
Introduction
Introduction to central halo-like choroidal atrophy Central halo-like choroidal atrophy (centralareolarchoroidalatrophy) is an autosomal dominant hereditary disease that is a special type of primary choroidal capillary atrophy, which is mainly characterized by posterior pole round or oval retinal pigment epithelium. Loss of choroidal capillary atrophy, exposure of the choroidal large vessels. basic knowledge The proportion of sickness: 0.003%-0.005% Susceptible people: no specific population Mode of infection: non-infectious complication:
Cause
Central halo-like choroidal atrophy
(1) Causes of the disease
Related to genetic factors.
(two) pathogenesis
Not very clear.
Prevention
Central halo-like choroidal atrophy prevention
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.
Complication
Central halo-like choroidal atrophy complications Complication
Generally no complications.
Symptom
Central halo-like choroidal atrophy symptoms common symptoms night blind visual impairment pigmentation loss fundus changes optic atrophy
The onset of this disease is mild, slow development, early no symptoms, and the age of onset is 20 to 40 years old.
1. Visual function changes visual acuity slowly and progressively. At the time of childhood, there is central visual impairment. At the beginning of middle age, there is obvious visual loss. Some people have symptoms at the age of 50, and there is a central dark spot. Point, dark adaptation function decline, dyslexia and glare, no blind history, but also poor visual acuity at night, cases with poor night vision, ocular electrophysiological examination showed mild abnormal changes in ERG, early normal, as the disease progressed, when the choroid and Secondary retinal pigment epithelium, neuronal epithelial atrophy, ERG can show a significant decrease in RG peak, especially in the 30 ° posterior pole of the macula, according to the degree of RPE involvement, EOG can show normal or mild abnormalities, patients have no obvious color blindness in the early stage, late There may be color blindness, multi-focal ERG 1st and 2nd order response amplitude and latency reduction, Nagasaka et al used multifocal ERG to detect 8 early central halo-shaped choroidal atrophy, and found that the first-order response amplitude decreased in the visible atrophy In the invisible atrophy region, the amplitude is also reduced, and the latency is slightly delayed in many regions; the amplitude of the second-order reaction is low in the posterior pole and the peripheral region; compared with the normal group , Not obvious in all regions 2 and higher order reaction ratio decreases, halo Hartley found that central choroidal atrophy, although the full field ERG examination is normal, but it has been found multifocal ERG abnormalities.
2. In the early stage of fundus changes, the macular degeneration is seen in the macular area, the central concave light is diffused, the macular area is tin foil-like, some of the macular part of the eye has edema and exudate, pigment spots, foveal reflection, and the disease gradually develops. The macular part of the two eyes showed a lesion with a ring-shaped or oval border. The pigmented epithelium and choroidal capillaries disappeared (Fig. 1), and the choroidal vessels were white-lined. This lesion can generally develop to over 50 years old. The choroidal blood vessels in the lesion area can also be occluded. As the disease progresses, the macula gradually forms a hole-like atrophy area with a clear circular mirror boundary. The eyes are basically symmetrical, the lesion is grayish green, and there is bronze-like reflection, which is doped. There are brown-black and yellow-white dots, which can be seen in the choroidal blood vessels and white sclera background. All lesions are confined to the macular area or the macular area. They do not involve the optic disc and the area outside the central area, and there are no abnormalities in the retina and blood vessels.
Examine
Central halo-like choroidal atrophy
Histopathological examination: manifested as fibrous scar formation in the affected area, retinal pigment epithelial cells (RPE), photoreceptor cells and choroidal capillary atrophy, normal retinal choroid in the lesion, macular lesions, choroidal capillaries and retinal neuroepithelial and The pigment epithelium disappeared, large choroidal vessels atrophied, the choroidal retinal macular area with clear boundaries had no vascular wall, and no obvious arteriosclerosis was observed. The avascular region showed atrophy and fibrosis.
1. Fundus fluorescein angiography in the early stage of the disease, due to the retinal pigment epithelium atrophy and pigmentation in the lesion area, it is manifested as a fluorescent spot, the disease progresses to the middle and late stage, choroidal capillary atrophy, manifested as weak choroidal capillaries without perfusion Fluorescence, such as small vessels in the choroid, shrinks, leaving only large choroidal vessels in the lesion, and a strong fluorescent ring appears at the edge due to pigment loss.
2. Indocyanine green angiography is generally weakly fluorescent in the map-like atrophic lesion area, the choroidal large blood vessels are filled with ICG dye, and ICG fluorescence in the choroidal large blood vessels can remain for several minutes due to the high binding of ICG staining to plasma proteins. Similarly, indigo green dyes generally do not leak out of the blood vessels, so the dyeing of the Bruch membrane is not seen.
Diagnosis
Diagnosis and identification of central halo-like choroidal atrophy
diagnosis
In the plaque or posterior pole, it does not develop to the optic disc or around, and the boundary is very clear. The early macular pigment disorder, the foveal reflex disappears, and the salt and pepper is difficult to diagnose. It is generally believed that the disease is pigmented epithelial atrophy, choroid Atrophy is a secondary change that should be differentiated from Stargardt's disease, yolk-like macular degeneration, and congenital macular degeneration caused by toxoplasmosis. This disease is confined to the posterior pole of the macula, and does not develop to the optic disc or surrounding, so it should be Age-related macular degeneration, cone degeneration and other phase identification, and various types of inflammatory retinal choroidal lesions and central choroidal atrophy.
Differential diagnosis
1. Stargardts disease (Stargardts disease) Most of the disease occurs in the 6 to 20 years old, most of them occur before the age of 15 years, the bilateral lesions are symmetrical, mostly elliptical, unclear pigmented epithelial atrophy; late choroidal capillaries Atrophic area, but in the middle, small blood vessels will not shrink, and there are scattered yellow spots on the periphery of the fundus.
2. Cone dystrophy The macular lesions of this disease are often mild, the degree of RPE atrophy is mild, the boundary is unclear, the choroid has no progressive atrophy, and the patient has typical photophobia and nystagmus symptoms.
3. The diagnosis of early central halo-like choroidal atrophy in the macular degeneration of the macular degeneration of the macular degeneration is difficult. It should be differentiated from the disease of dry age-related macular degeneration. The age of onset is relatively late. More than 45 to 60 years old, no family history, the boundary of the lesion is not clear, there are scattered drusen or subretinal neovascularization in the fundus.
4. Whether central choroidal atrophy and central halo-like choroidal atrophy are the same genetic disease remains to be studied. There are two cases of atrophic cases in the literature. The central halo-like choroidal atrophy is characterized by bilateral symmetry and limitation. In the circle of the macula, the choroidal choroid atrophy in the border of the hole, the fundus atrophy around the optic disc, except for the choroidal atrophy around the optic disc, the fundus fluorescein angiography lesions showed a clear fluorescence of the choroidal atrophy.
