Tapetoretinalny dystrophies of a retina

Description:

Tapetoretinalny dystrophies (Latin tapete carpet, cover + late lat. retina mesh cover; synonym: tapetoretinalny degenerations, tapetoretinalny an abiotrophy) — hereditary diseases of a retina which general symptom is pathological change of its pigmental epithelium; are characterized by the progressing decrease in visual function up to a blindness.

Depending on localization of pathological process in peripheral or central department of a retina distinguish peripheral and central Etc. Carry pigmental dystrophy of a retina to peripheral — the most common form from all Etc. and also white-dotted dystrophy of a retina (including a white-dotted eyeground), a zheltopyatnisty eyeground, a kongenitalny stationary night blindness. The central tapetoretinalny dystrophies include macular dystrophies — Shtargardt's dystrophy, dystrophy Vera and Best's dystrophy.

Symptoms of Tapetoretinalny dystrophies of a retina:

Peripheral tapetoretinalny dystrophies.
The earliest change in a retina is destruction of cells of a neuroepithelium, and first of all sticks, up to their disappearance. The changes arising at first in peripheral department of a retina extend then in the central department. In rare instances preferential central department of a retina (the central form of pigmental dystrophy of a retina) or the limited sektoroobrazny site of a retina (a sectoral form) is surprised. Against the background of disappearance of cells of a neuroepithelium the quantity of glial cells and fibers which fill the released space increases. In cells of a pigmental epithelium phagosomas disappear. Pigment cells migrate in inside layers of a retina. Due to the proliferation of glial fabric and migration of pigment cells blood circulation in a capillary network is broken.

As a rule, both eyes are surprised. The first symptom of pigmental dystrophy of a retina — decrease in sight in the dark (hemeralopia), there are defects of a field of vision later, visual acuity decreases, the eyeground changes. Characteristic defect of a field of vision is ring-shaped scotoma which when progressing a disease extends both to the center, and to the periphery and leads to concentric narrowing of a field of vision (a tubular field of vision). At the same time, despite rather high central sight which can remain many years sharp disturbance of orientation leads to an invalidism. At the central and sectoral form of pigmental dystrophy of a retina reveal respectively the central and sectoral scotoma.

On an eyeground usually find pigmental deposits, narrowing of arterioles of a retina, a wax-like atrophy of an optic disk Pigmental (deposits are located, as a rule, on the periphery of a retina (often around veins) and have an appearance of the typical pigmental centers (so-called bone little bodies) or глыбок, mosaics, spots. At the central form of pigmental dystrophy they are localized preferential in the central department of a retina, at a sectoral form the sektoroobrazny arrangement of a pigment on an eyeground is noted. In rare instances pigmental deposits in a retina can be absent (a pigmental retinitis without pigment). In a late stage of a disease zones of an atrophy of a choriocapillary layer of an idiovascular cover of an eyeglobe come to light. In some cases pigmental dystrophy of a retina is followed by development of the complicated back cortical cataract, glaucoma, short-sightedness. The disease slowly progresses, coming to the end in most cases with a blindness.
The Zheltopyatnisty eyeground — a disease with an unspecified mode of inheritance, is shown at the age of 10 — 25 years. In deep layers of a retina in the field of a back pole of the eyeball the yellow or yellow-white spots found, as a rule, are formed it is accidental at an eyeground research. Visual functions usually are not broken, except for cases of defeat of macular area of a retina when visual acuity of the ERG decreases is not changed. Treatment is same, as at pigmental dystrophy of a retina.

The Kongenitalny stationary night blindness is inherited on autosomal dominantly, autosomal recessively to type, can be linked to a floor. It is often combined with short-sightedness. The main symptom is orientation disturbance in the dark. Visual acuity remains normal or goes down (0,7 — 0,4). The field of vision is not changed a thicket, in certain cases slightly narrowed. The eyeground, as a rule, without pathology, only at a combination to short-sightedness is absent a foveolyarny reflex and the small depigmentation of a retina is noted. Treatment is same, as at pigmental dystrophy of a retina.

The central tapetoretinalny dystrophies are characterized by pathological changes in the central part of an eyeground (fig. 2), the progressing decrease in visual acuity, in a late stage emergence of the central scotoma. At Best's dystrophy visual acuity, despite rough changes in macular area, can remain a long time high and decreases only at formation of the atrophic center in the field of a macula lutea. Central Etc. lead to decrease in the central sight. Peripheral sight and dark adaptation at central Etc. are not broken. Treatment is same, as at peripheral tapetoretinalny dystrophies.

Reasons of Tapetoretinalny dystrophies of a retina:

Peripheral tapetoretinalny dystrophies. Pigmental dystrophy of a retina (a pigmental retinitis, a pigmental degeneration of a retina, a pigmental retinal abiotrophy, palochkokolbochkovy dystrophy) is inherited on autosomal recessively, can be inherited on autosomal dominantly, in some cases linked to a floor, to type.

Can be both an independent disease, and one of manifestations of other hereditary diseases — an amaurotic idiocy, Laurence — Muna — Grain stillage — Bidlya of a syndrome, and also the syndrome described by Asher (S.N. Usher), which is characterized by a combination of pigmental dystrophy of a retina to a hearing impairment that is caused by simultaneous defeat of a pigmental epithelium of a retina and the epithelium of kortiyevy body having homogeny.
White-dotted dystrophy of a retina is inherited usually on autosomal recessively type. It is shown at youthful age by a hemeralopia, the progressing narrowing of a field of vision, anomaly of color sensation. On an eyeground reveal multiple white spots and in some cases pigmental deposits, narrowing of arteries, blanching of an optic disk, to the ERG it is usually not changed. The disease progresses with age. Treatment is same, as at pigmental dystrophy of a retina.

Treatment of Tapetoretinalny dystrophies of a retina:

Peripheral tapetoretinalny dystrophies. At treatment appoint intramusculary Riboflavinum mononucleotide or ATP, it is long — vasodilators, and also drugs a part of which are ribonucleotides