angiomatosis fiber

August 12, 2017 17:51 | Genetic Diseases

Disease Von Hippel-Lindau is a retinal tumor that is often combined with a tumor of the cerebellum, or spinal cord.This syndrome is transmitted in an autosomal dominant inheritance type.Angioma (vascular tumors) Retinal there since birth, but in most cases begin to appear on the 2nd or 3rd decade of life.The tumor nodes in the retina often serve as a manifestation of the overall process, when, in addition to the nervous system, in the pathological process involved and kidneys, pancreas and liver, ovaries (in women).Vascular tumors formed in the inner portion of the retina as a result of continuous growth takes full thickness.On the periphery of the fundus of the eye when viewed from an ophthalmologist identify ampulovidnye extended and convoluted vessels of the glomeruli.These vascular changes lead to the emergence of retinal edema, hemorrhage into it.Visual functions are violated, depending on the location and extent of retinal lesions.The disease progresses continuously, can be complicated retinal de

tachment, glaucoma and development (or) cataracts.The outcome of the disease is blindness, and often affects both eyes.

the early stages of treatment of the syndrome is the destruction of tumor node and supplying the vessels with a laser unit or a local action of low temperatures.With the development of glaucoma, and in the case of retinal detachment surgery produce.The range of therapeutic measures include hormones and drugs that protect blood vessels from further damage (angioprotectors).

outer exudative hemorrhagic retinitis Coates is the formation of a vascular tumor formation, which is due to a congenital anomaly retinal vessels.The disease occurs in children and young adults, especially in males.In most cases, the pathological process involves only one eye.In the fundus examination with an ophthalmologist revealed dilated retinal vessels.Moreover, there is a large amount of accumulation of inflammatory fluid in itself or underneath the retina.Often reveal hemorrhage and cholesterol deposition, retinal edema.On the periphery of the fundus of the eye as a result of the intensive formation of inflammatory fluid accumulation under the retina and its detachment occurs.The process may take over the entire retina, which is accompanied by lesions of the optic nerve.Visual functions are violated, depending on the extent of retinal changes.The extreme degree of disturbance of visual function is the formation of blindness.Spent treatment does not bring the desired result, and in most cases is ineffective.The most severe complications of the disease are bleeding in the eye, glaucoma, retinal detachment.

dystrophy (degeneration) retina for the most part are hereditary or congenital.In addition, they are often a symptom of other systemic diseases, such as hereditary metabolic disorders, diseases of the endocrine system, and others. Signs of retinal degeneration, depending on the cause, can occur from the first months of life.A typical manifestation of macular degeneration is a deterioration of vision in twilight or photophobia in bright light, flashes of light, distortion of objects, decreased central vision and impaired orientation in space.Macular degeneration is often combined with a squint and involuntary movements of the eyeballs.The disease slowly progresses continuously, leading eventually to blindness, low vision, or (in extreme cases).

pigment retinal dystrophy is the most common of all hereditary retinal diseases.The disease manifests night blindness (day-blindness) and the progressive narrowing of the visual field.The earliest sign of pigmentary retinal dystrophy is a violation of adaptation to the vision in the dark, appearing several years before the appearance of changes in the fundus.In the fundus along the retinal vessels, starting from the periphery, there are deposits of pigment dark brown color that reveals the optometrist at the examination.As the disease progresses increase the number and size of pigment deposits, they are subject to slow and capture the central parts of the retina.retinal vessels become narrow.The process takes place on both sides.This disease may be associated with the development of cataracts.The field of vision gradually narrows, reduces central vision.Blindness usually occurs between 40 to 50 years, rarely older than 60 years.A unilateral lesion is extremely rare.

treatment is the application of neurotrophic drugs that are used for long-term treatment of the retina and optic nerve.Assign also means improving the blood circulation in the retina, optic nerve and choroid.These drugs include Trental, cavinton, Halidorum.In recent years, surgical correction is also carried out for this disease.

Spot white dystrophy of the retina (retinal dystrophy belotochechnaya) runs in families, develops in childhood, progresses slowly.A common manifestation of the disease is the twilight, and night blindness.On examination, the ophthalmologist revealed numerous small whitish, well-defined lesions in the fundus.Gradually gaps retinal vessels narrow, which leads to disruption of blood circulation, and optic nerve atrophy.Treatment is similar to the retinal pigment degeneration.

Stargardt disease - Juvenile macular degeneration.The yellow spot of the retina represents the beginning of the optic nerve, through which the process is carried out and the perception of different visual images.Stargardt disease is transmitted in an autosomal recessive inheritance type and begins to emerge in the preschool years.At the same optometrist notes symmetrical macular lesion that gradually leads to loss of central vision.In the initial stage of the disease in children aged 4-5 years are beginning to complain of photophobia, vision improves at dusk and gets worse in the light.At the age of 7-8 years is already becoming noticeable decrease in vision in the field of view appears scotoma.

addition to the above methods of treatment of retinal dystrophies, perhaps with the help of laser treatment (stimulation).

disease Best - yolk macular degeneration.The disease is transmitted in an autosomal dominant inheritance type.The first manifestations occur in preschool age, that is a slight decrease in vision.On examination, optometrist detects the presence in the field of macular kistoobraznogo hearth yellow, regular round shape, which resembles the appearance of raw egg yolk.This cystic formation may burst as a result of an excessive accumulation of inflammatory fluid in it.This leads to hemorrhage and retinal edema.Further scarring occurs diseased portion of the retina and its atrophy, resulting in a loss of vision.

treatment is the use of drugs that protect blood vessels from other damaging factors (angioprotectors), antioxidants (such as vitamins A, C, E).

angioid band (Grenblad-Strandberg syndrome). disease is transmitted in an autosomal dominant (less in an autosomal recessive) inheritance type.More common in females.The first signs appear syndrome during the first two decades of life.The disease is loss of blood vessels due to the generalized destruction of the elastic tissue, accompanied by inflammatory changes and deposition of large amounts of calcium in the vascular wall.Such individuals having disorders in the circulatory vessels of the lower limb, angina, stroke, bleeding from the gastrointestinal tract.In 50% of patients with human retinal lesions detected as a reddish-brown, gray, winding bands.The appearance of the bands associated with choroidal ruptures as a result of the destruction of its elastic layer.These changes are accompanied by a reduction in central vision.

Treatment: apply antioxidants (tocopherol, emoksipin) angioprotectors (Dicynonum).In some cases, carried out laser cauterization angioid bands.