Familial exudative vitreoretinopathy
Introduction
Introduction to familial exudative vitreoretinopathy Familial exudative vitreoretinopathy (familialexudativevitreoretinopathy) at the same time invading both eyes, the severity of the disease on both sides is not necessarily equal, the fundus changes and retinopathy of premature infants are similar, but the disease occurs in full-term neonates, no history of oxygen uptake, Most have a family history of autosomal dominant inheritance, which is different from the latter. basic knowledge Sickness ratio: 0.0001% Susceptible population: newborn Mode of infection: non-infectious Complications: cataract Glaucoma
Cause
Causes of familial exudative vitreoretinopathy
(1) Causes of the disease
The cause of this disease is not fully understood.
(two) pathogenesis
Some people think that it is a variant of retinal blood vessels and vitreous in embryonic period, which is a variant of congenital retinal folds. However, it is also believed that neonates with full-term birth may have individual differences or dysplasia in retinal vascular development, near the retinal serrated margin. There is a vascular zone. At birth, due to the sudden increase in oxygen saturation of fetal hemoglobin, the fetal oxygen partial pressure (fetal PO2) changes sharply into neonatal oxygen partial pressure (neonatal PO2), leading to retinal end Vasoconstriction, obstruction, ischemia, hypoxia, induce abnormal proliferation of peripheral fundus vessels, causing a series of pathological changes such as exudation, hemorrhage, and mechanization, resulting in very similar fundus changes and evolution with retinopathy of prematurity process.
Prevention
Familial exudative vitreoretinopathy 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
Familial exudative vitreoretinopathy complications Complications cataract glaucoma
Can be complicated by cataract, iris atrophy, neovascular glaucoma and other anterior segment lesions.
Symptom
Familial exudative vitreoretinopathy symptoms Common symptoms Intraocular empyema and blind retinal edema
The disease is a chronic progressive disease. The visual impairment is different due to the severity of retinal and vitreous lesions. When traction retinal detachment occurs, it can cause blindness. The development of the lesion is often limited to the young age. If there is no traction after 18 years old. Retinal detachment rarely has a loss of vision, and the incidence of traction retinal detachment is reported to be 21%, most of which are seen before the age of 30.
Some authors have divided the disease into three phases:
1. Phase 1
Indirect ophthalmoscope and scleral compression examination showed that the retinal compression at the peripheral part of the temporal side and the surrounding area were pale, the retinal blood vessels were normal, and the retina did not change.
2. Phase 2
The temporal retina has neovascularization from the equator to the serrated margin, and the retina and its underflow ooze, localized retinal detachment, and the temporal vascular membrane draws the retinal blood vessels, forming a macular degeneration.
3. Phase 3
The lesion developed further, and the traction retinal detachment was found, and there was a large amount of exudation in the retina and subretinal.
Examine
Examination of familial exudative vitreoretinopathy
No special laboratory tests.
FFA showed that there were many branches of retinal vessels, which were densely distributed. They were fan-shaped and suddenly stopped near the equator, and the ends were anastomosed. There was abnormal fluorescein leakage, and there were no perfusion areas in the retinal capillaries around the fundus.
Diagnosis
Diagnosis and diagnosis of familial exudative vitreoretinopathy
There is a family history, no history of premature oxygen inhalation, binocular sputum, vitreous opacity and special ophthalmoscope and FFA, which is an important basis for the diagnosis of this disease.
Need to identify the disease with retinopathy of premature infants and Coats disease, premature retinopathy of premature infants with premature birth, low body weight, oxygen history, no family history, Coats disease without vitreous lesions, no extensive vitreoretinal adhesions, and exudation Not limited to the peripheral fundus, the performance under the ophthalmoscope is very different from the disease.
