Angiomatosis of cellulose
Hippel-Lindau disease is a retinal tumor, which is often combined with a cerebellum or spinal cord tumor. This syndrome is transmitted by an autosomal dominant type of inheritance. Angiomas( vascular tumors) of the retina exist even from birth, but in most cases begin to manifest from the 2nd or 3rd decade of life. Tumor nodes in the retina often serve as a manifestation of the general process, when, in addition to the nervous system, kidneys, pancreas and liver, and ovaries( in women) are also involved in the pathological process. The vascular tumor forms in the inner part of the retina and, as a result of continuous growth, occupies its entire thickness. On the periphery of the fundus, when examining the ophthalmologist, ampullae enlarged and convoluted vessels in the form of glomeruli are revealed. Such changes in the vessels lead to the appearance of retinal edema, hemorrhages into it. Visual functions are violated depending on the location and prevalence of retinal lesions. The disease progresses continuously, can be complicated by retinal detachment, the development of glaucoma and( or) cataracts. The outcome of the disease is complete blindness, and quite often both eyes are affected.
In the early stages of the treatment of the syndrome is the destruction of the tumor node and vessels feeding this node with a laser or a local action of low temperatures. With the development of glaucoma, as well as in the case of detachment of the retina, surgery is performed. The complex of therapeutic measures include hormones and drugs that protect the blood vessels from additional damage( angioprotectors).
External exudative haemorrhagic retinitis Coats is the formation of vascular tumor-like formation, which is due to congenital anomaly of the retina vessels. The disease occurs in children and young people, mainly in males. In most cases, only one eye captures the pathological process. On the fundus, when examined by an ophthalmologist, extended retinal vessels are identified. In addition, there is a congestion of a large amount of inflammatory fluid in the retina itself or under it. Often reveal hemorrhages and deposits of cholesterol, retinal edema. On the periphery of the fundus, as a result of the intense formation of the inflammatory fluid and the accumulation under the retina, its detachment occurs. The process can capture the entire retina, which is accompanied by damage to the optic nerve. The visual functions are impaired depending on the degree of change in the retina. The extreme degree of impairment of visual functions is the formation of blindness. The treatment does not bring the desired result and in most cases is ineffective. The most serious complications of the disease are hemorrhage in the eye, the development of glaucoma, detachment of the retina.
The degeneration of the retina is for the most part hereditary or congenital. In addition, they are often a symptom of other systemic diseases, for example, with hereditary metabolic disorders, the pathology of the endocrine system, and other signs of retinal dystrophy, depending on the cause, may be manifested as early as the first months of the child's life. A characteristic manifestation of retinal dystrophy is a deterioration of vision in twilight or photophobia in bright light, flashes of light, distortion of the shape of objects, a decrease in central vision and disruption of orientation in space. Dystrophy of the retina is often combined with strabismus and involuntary movements of the eyeballs. The disease continuously progresses slowly, resulting in poor vision or blindness( in the most severe cases).
Retinal pigmentary retinal dystrophy is the most common among all hereditary retina diseases. The disease is manifested by night blindness( gemeralopia) and a progressive narrowing of the field of vision. The earliest sign of retinal pigmentary dystrophy is a disruption of adaptation to vision in the dark, occurring a few years before the appearance of changes on the fundus. On the eye bottom along the course of the vessels of the retina, from the periphery, pigment deposits of a dark brown color appear, which the oculist reveals upon examination. As the disease progresses, the amount and size of pigment deposits increase, they slowly spread and capture the central parts of the retina. Retinal vessels become narrow. The process proceeds from two sides. This disease can be accompanied by the development of cataracts. The field of vision gradually narrows, central vision decreases. Blindness usually occurs between 40-50 years, rarely over the age of 60 years. One-sided defeat is extremely rare.
Treatment consists in the use of neurotrophic drugs, which are used for long-term treatment of the retina and optic nerve. Assign also funds that improve blood circulation in the retina, optic nerve and choroid. These drugs include trental, cavinton, halidor. Recently, a surgical correction of this disease has also been carried out.
Spotted white dystrophy of the retina ( white dot degeneration of the retina) is of a family nature, develops in childhood, progresses slowly. The usual manifestation of the disease is twilight and night blindness. When examined by an ophthalmologist, numerous small whitish, clearly defined pockets on the fundus are revealed. Gradually the lumens of the retina vessels narrow, which leads to a disturbance of the circulation and atrophy of the optic nerve. Treatment is similar to pigmentary retina dystrophy.
Stargardt disease is a juvenile macular degeneration. The yellow spot of the retina is the beginning of the optic nerve, thanks to which the process of perception of various visual images is realized. Stargardt's disease is transmitted by an autosomal recessive type of inheritance and begins to manifest itself at preschool age. In this case, the oculist notes a symmetrical lesion of the yellow spot, which gradually leads to loss of central vision. In the initial stage of the disease, children aged 4-5 years begin to complain of photophobia, eyesight improves in the twilight and becomes worse in the light. At the age of 7-8 years, the decrease in vision is already noticeable, the scotoma appears in the field of vision.
In addition to the above methods for the treatment of retinal dystrophy, treatment with a laser( stimulation) is possible.
Bestial disease is yolk dystrophy of the yellow spot. This disease is transmitted by an autosomal dominant type of inheritance. The first manifestations occur in the preschool age, which is a slight decrease in vision. Upon examination, the oculist reveals the presence in the yellow spot area of a cystic hearth of yellow color, a regular rounded shape that resembles the egg yolk of a raw egg. This cystic formation can burst as a result of excessive accumulation of inflammatory fluid in it. This leads to hemorrhage and swelling of the retina. In the future, the scarring of the affected area of the retina and its atrophy occur, which leads to a decrease in vision.
Treatment consists in the use of drugs that protect the vessels from additional damaging factors( angioprotectors), antioxidants( for example, vitamins A, C, E).
Angioid retinal bands( Grenblad-Strandberg syndrome). The disease is transmitted by autosomal dominant( less often by autosomal recessive) type of inheritance. Most often they are sick with female faces. The first signs of the syndrome appear in the first two decades of life. At the heart of the disease is the damage of the blood vessels due to the generalized destruction of the elastic tissue, accompanied by inflammatory changes, as well as the deposition of a large amount of calcium in the vessel wall. Such people have circulatory disorders in the vessels of the lower extremities, angina pectoris, strokes, bleeding from the gastrointestinal tract. In 50% of patients, retinal lesions are detected in the form of reddish-brown, gray sinuous bands. The appearance of the bands is associated with ruptures of the vascular membrane as a result of the destruction of its elastic layer. Such changes are accompanied by a decrease in central vision.
Treatment: antioxidants( tocopherol, emoxipin), angioprotectors( dicinone) are used. In a number of cases, laser cauterization of angioedema of the retina is carried out.