Outer exudative retinopathy
Introduction
Introduction to exudative retinopathy of the outer layer External exudative retinopathy (external exudative retinopathy) or outer hemorrhagic retinopathy (external hemorrhagicretinopathy), also known as Coats disease, retinal telangiectasia (retinaltelangiectasis), mostly found in male adolescents, fewer women, a few occur in adults, Usually infringe on one eye, occasionally on both sides. The course of the disease is slow and progressive. It is not easy to detect early, until the vision is significantly reduced, and it is noticed when there is white diarrhea or disuse. basic knowledge The proportion of illness: 0.3% Susceptible people: mostly seen in male teenagers Mode of infection: non-infectious Complications: glaucoma edema, vitreous hemorrhagic vitreoretinopathy, iridocyclitis, cataract
Cause
The cause of exudative retinopathy of the outer layer
(1) Causes of the disease
The cause is unknown. Some people think that the disease may be inflammation, but the source of inflammation has not been determined. Some people also think that it is related to syphilis, tuberculosis, toxoplasmosis and other inflammations, but most patients can not find the source of infection, adult patients The cause is more complicated. In addition to congenital vascular abnormalities, there may be other reasons. For example, some patients have uveitis and cholesterol is high and the disease is suspected. Inflammation may be the cause of this disease. Some people also found this disease. The secretion of steroids exceeds normal, and the glucose tolerance curve is prolonged, indicating that the adrenal cortex is hyperactive, so it is thought that this disease may be related to endocrine disorders and metabolic disorders.
The disease is a retinal vascular abnormality, the vascular endothelial cell barrier function is lost, so that a large amount of plasma oozes out of the retinal neuroepithelial layer, causing retinopathy of extensive retinal detachment, but this retinal vascular abnormality is congenital or acquired. Whether it is primary or secondary, so far, there is no conclusion.
In the literature, most of the histological examinations of this disease are advanced cases. Trapathi and Ashton (1971) have performed electron microscopy on the tissue samples of an early typical case, and observed that the vascular endothelial cells have vacuoles, degeneration, and severe cases. The endothelial cell layer completely disappeared, and the wall of the tube was composed only of plasma and fibrous layers, and the periphery was glial.
In the late cases, under the light microscope examination, the retina is widely detached; the retina is degenerated, atrophied, and the structure is completely destroyed; the deep retina has cysts of different sizes, and the subretinal and detached retina are filled with hemorrhage and protein exudate; There are also scented plaques, pigment free, varying amounts of foam cells and cholesterol crystals, retinal vasodilation, thickened wall, hyaline degeneration, PAS staining showing thickening of the subendothelial membrane, endothelial cell hyperplasia, When the blood vessels are occluded, the internal cells may be detached or even disappeared. The blood overflows, the blood vessels only have contours, and there are new blood vessels in the inner and outer layers of the retina. There are lymphocytes around the blood vessels, infiltration of inflammatory cells such as large mononuclear cells, and protein in the vitreous. Sexual exudate and a small amount of lymphocytes, large mononuclear cells, hemorrhage and cholesterol crystal voids can also be seen in some specimens, obvious chronic inflammatory cell infiltration near the choroid and retinal adhesions and mechanized connective tissue mass, connective tissue The mass is located between the choroids of the retina and varies in size. The retinal pigment epithelium proliferates and enters the connective tissue mass. Some patients may have calcification or ossification, sometimes you can still see giant cell foreign body reaction and cholesterol crystal voids.
In cases with secondary glaucoma, the anterior ocular anterior adhesion of the iris can be seen on the anterior segment of the eyeball; the iris membrane covers the fibrous vascular membrane: edema of the basal layer of the corneal epithelium.
(two) pathogenesis
It is unknown to date that most authors believe that the Coats disease in children and adolescents is caused by congenital small blood vessel abnormalities. Even if there are no obvious vascular abnormalities in the fundus, fluorescein angiography or histopathological examination can reveal changes in blood vessels, telangiectasia and small. Arterial, venular lesions, glassy changes in the vessel wall, mucopolysaccharide deposition under endothelial cells, thickening of the wall, narrowing of the lumen, slow blood flow, vascular occlusion, impaired vascular wall barrier, aneurysm Formed with microangioma, causing oozing and bleeding of the slurry, and massive exudation.
Prevention
Outer exudative retinopathy prevention
The early stage of the disease is not easy to detect. Most patients have a significant loss of vision, jaundice or exotropia, or even secondary glaucoma. It is a pity that it is too late. Therefore, regular checkups should be conducted for children and adolescents. Vision, especially dilated examination of the fundus, for early detection and early treatment, the current consensus is that early argon laser treatment is effective, and some cases can maintain better vision.
Complication
Outer exudative retinopathy complications Complications glaucoma edema glass volume blood proliferative vitreoretinopathy iridocyclitis cataract
In cases with secondary glaucoma, the anterior ocular anterior adhesion of the iris can be seen on the anterior ocular tissue specimen; the iris membrane covers the fibrous vascular membrane: basal edema of the corneal epithelium, vitreous hemorrhage and proliferative vitreoretinopathy, and the iris can be combined in the late stage. Stomatitis and complicated cataracts, secondary glaucoma, can cause eyeball atrophy.
Symptom
Symptoms of exudative retinopathy of the outer layer Common symptoms Uveitis retinal detachment microcirculatory disorder visual impairment macular cystic edema
Early non-conscious symptoms, because most of them are monocular, and occur in children and adolescents, they are often not detected by the patient until the visual acuity is significantly reduced or the pupil has yellow-white reflex, or the extraocular tilt begins to attract attention. The typical change of the fundus is the retina. Exudation and vascular abnormalities, early optic disc is normal, macular can be normal or invaded, lesions can begin to appear anywhere in the fundus, but the temporal side, especially around the optic disc and the macula are the most common, can be limited to one or two quadrants, Or throughout the fundus, exudation is white or yellowish white, punctate or fused into a sheet, often arranged in a strip of uneven borders, or in a semi-circular or circular shape, called circinate retinopathy Exudation is located behind the retinal blood vessels in the deep layer of the retina, and can partially cover the blood vessels. Some exudation ridges can be up to several diopter, so it is also called outer exudative retinopathy, and is often spotted in the vicinity of exudation. Cholesterol crystal corpuscles and punctate and flaky hemorrhage, even pigmentation, marked abnormalities in the lesions, arteries and veins can be damaged, especially small arteries Obviously, the lesion mainly occurs in the second or third branch of the blood vessel, the vasodilation is distorted, the wall of the tube is tumor-like, fusiform or lenticular hemangioma, or arranged in a beaded shape, and the blood vessel can also be in the shape of a wreath, spiral or New Zealand. The knot is distorted, and may be accompanied by new blood vessels and intervascular communication branches. The lesions located in the vicinity of the macula may invade the macula, produce macular edema, or have a starburst-like exudation, and the severe macular forms a scar.
Examine
Examination of outer exudative retinopathy
1. Blood cholesterol check: Blood cholesterol test can be performed for adult patients.
2. Glucose tolerance test.
3. Pathological examination:
Regardless of young or elderly patients, the pathological changes are basically the same, retinal vasodilation, thickening of the vessel wall, hyaline degeneration, chronic inflammatory cell infiltration around the blood vessels, mainly lymphocytes and large mononuclear cells, vascular endothelial cell hyperplasia, The blood vessels are narrowed or even occluded, and there are PAS-positive mucopolysaccharide deposits under the intima. Some vascular endothelial cells fall off or even disappear, lose barrier function, and blood spills. In some cases, new blood vessels may form, and early retinal edema may occur. Protein exudate and hemorrhage are located in the outer layer of the retina, causing partial or total retinal detachment. The detached subretinal is filled with protein exudate and hemorrhage. There are a large number of foam cells and cholesterol crystal voids and macrophages that phagocytose lipids. The retinal pigment epithelium is still normal, the choroid may have chronic inflammatory cell infiltration, and the late retinal exudation is replaced by connective tissue, located in the retina and between the retina and the choroid. There are cavities in the scar tissue, which are filled with various debris. , such as residual pigment after bleeding, glassy substance, giant cells, cholesterol Or other crystalline, calcium tablets, etc., retinal pigment epithelial hyperplasia, degeneration and loss, and finally a large number of blood vessels and retinal degeneration, arteriosclerosis, retina replaced completely the proliferation of fibroblast and glial tissue.
Diagnosis
Diagnosis and diagnosis of exudative retinopathy of outer layer
The disease is distinguished from retinoblastoma, retinopathy of prematurity, metastatic endophthalmitis, and other eye diseases that occur in childhood and have chalkiness. Among them, the identification of retinoblastoma is particularly important if retinoblasts are used. Misdiagnosis of Coats disease can delay the treatment of retinoblastoma and endanger the lives of children.
1. Retinoblastoma: It is more likely to be confused with Coats disease, and there are often those who remove the eyeball because Coats disease mistakenly believes in the tumor.
2. Retinopathy of prematurity: white pupils should be differentiated from Coats disease in the late stage, but this disease is mostly caused by both eyes. It is more common in premature infants who have received oxygen therapy.
3. Metastatic endophthalmitis: often secondary to acute infections of the whole body, especially lung infections. There are often different degrees of inflammation in the anterior segment of the eye, such as the posterior corneal wall, positive flash of aqueous humor, narrowing of the pupil, and other signs of uveitis. .
4. Age-related macular degeneration: elderly patients with a small number of Coats disease should be differentiated from macular degeneration, the latter lesions are always in the macular area, there may be repeated bleeding and large exudation, but the retinal blood vessels have no tumor-like changes.
5. Diabetic retinopathy: Sometimes there is ring exudation and microangioma, but diabetic patients often have bilateral eye disease, and have a history, symptoms and signs of systemic diabetes.
In addition, central retinal vein occlusion, diabetic retinopathy, etc., can sometimes be seen under the examination of the Coats disease (large yellow-white exudation, microangioma, cholesterol crystallization), is called Coats disease-like reaction .
