congenital retinoschisis
Introduction
Introduction to congenital retinoschisis There are different names in this literature, such as sex-linked hereditary revenile retinopathy (sex-linked hereditary juvenile retineschisis), congenital vitreous vascular glaucoma (congenitarvascularveils in thevitreous), juvenile retinoschisis (juvenileretinoschisis), etc. In the nerve fiber layer, the anterior wall of the bulge is the inner membrane of the retina and a layer of nerve fibers at a partial level. Congenital retinoschisis is a sexually linked genetic disease. Its causes are as follows: one is the congenital anomaly of the innermost layer of the retina, especially the inner end of Müller cells attached to the inner limiting membrane. There is some genetic defect; the other is vitreous Abnormal cortex, retinal caused by its traction, congenital onset, around 10 years old due to monocular or binocular vision, strabismus, vitreous hemorrhage, and can be found in a family survey because of a proband, only found in male children, Females are rare, mostly for both eyes, and the cleft palate is symmetrical. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: retinal detachment
Cause
Congenital retinoschisis
Hereditary factors (56%):
Congenital retinoschisis is a sexually linked genetic disease. Its causes are as follows: one is the congenital anomaly of the innermost layer of the retina, especially the inner end of Müller cells attached to the inner limiting membrane. There is some genetic defect; the other is vitreous Abnormal cortex, retinal caused by its traction, congenital onset, around 10 years old due to monocular or binocular vision, strabismus, vitreous hemorrhage, and can be found in a family survey because of a proband, only found in male children, Females are rare, mostly for both eyes, and the cleft palate is symmetrical.
Prevention
Congenital retinal cleft palate prevention
The disease is generally not carried out or is very slow, mainly between 5 and 10 years old, and stops development after 20 years of age. Pay attention to proper rest, do not master the combination of movement and rest, rest well, is conducive to the recovery of fatigue; exercise can enhance physical strength, enhance disease resistance, the combination of the two, can better recover.
Complication
Congenital retinal palpebral complications Complications retinal detachment from vitreous
Peripheral retinal palpebral fissure occurs in 50% of patients, and retinal detachment, vitreous hemorrhage and pigmentation can also occur.
Symptom
Congenital retinoschisis symptoms common symptoms visual impairment visual field vision visual field vision changes
The splitting occurs in the lower fundus, especially under the ankle. It is a huge cystic ridge from the equator to the far periphery. It is a separate inner layer of the retina. The rear edge forms a clear convex surface and the front edge cannot reach the serrated edge. The anterior sputum is thin, translucent, and the retinal blood vessels are accompanied by it. The blood vessels are often white-lined or have parallel white sheaths. The retina often has degeneration and pigmentation. After the anterior wall is ruptured, multiple circular or elliptical large holes are in the cystic At the posterior wall (ie, the outer layer of the neuroepithelial layer), there are snow-like white spots, and sometimes retinal vascular projections are visible.
The cleft palate occurs in the macula. It is called macular retinoschisis. It has visual impairment at the beginning. It can be seen in the macula. It has a stellate pigmentation and develops from the center fossa. Radial cystic wrinkles gradually merge into a doughnut-like retinal palpebral fissure.
Congenital retinal cleft palate occurs in the peripheral or macular area, visual acuity is significantly damaged, more than half is less than 0.3, the macular part of the visual acuity is more poor, the visual field is the absolute defect in the corresponding cleft palate, ERGa wave is normal, b wave declines.
Examine
Examination of congenital retinoschisis
1. Ask about family history.
2. Dilated, iris top pressure check the fundus, the examination should be carried out in the dark room, the patient takes more seats, the examiner can sit or stand. When checking the right eye, the examiner is located on the right side of the patient, with the right hand holding the mirror and the right eye; when checking the left eye, it is located on the left side of the patient, with the left hand holding the mirror and the left eye. Exclude the outer retinal tears or detachment.
Diagnosis
Diagnosis and diagnosis of congenital retinoschisis
Differential diagnosis
1. Age-related degenerative retinoschisis.
2. Rhegmatogenous retinal detachment. The incidence of rhegmatogenous retinal detachment depends on three factors, namely retinal tears, vitreous liquefaction and a sufficient pulling force to separate the retina from the pigment epithelium, where retinal tears are the key. There are often retinal vitreous degeneration between the retinal tears, lattice-like degeneration and cystic degeneration around the retina; vitreous liquefaction, atrophy and contraction cause posterior vitreous detachment. Degeneration of the retina and vitreous is associated with age, heredity, myopia, and trauma.
