idiopathic polypoid choroidal vasculopathy
Introduction
Introduction to idiopathic polypoid choroidal vasculopathy Lawrence Yanmuzzi first described idiopathic polypoidal choroidal vasculopathy (IPCV) at a meeting of the American Yellow Spot Society in Miami in 1982. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: retinal detachment
Cause
Idiopathic polypoid choroidal vasculopathy
(1) Causes of the disease
It is not clear that IPCV is more common in black women, Asian men and older people in their 60s. Therefore, age factors and ethnic factors may be related to the development of IPCV. In systemic diseases, Ross et al observed 2 cases of fundus. Hypertensive black women with retinal aneurysm (RMA) and IPCV changes, suggesting that RMA and hypertensive retinopathy cause retinal vascular changes, aneurysm dilatation and local vasoconstriction may be characteristic of choroid of IPCV There is a correlation between the formation of vascular lesions, but most scholars currently believe that IPCV has no obvious relationship with hypertension, diabetes, cardiovascular disease and other systemic diseases, and there is no clear evidence that IPCV is associated with smoking, with hypertension, diabetes, In patients with cardiovascular disease, IPCV may have an increased risk of recurrent bleeding in the fundus. According to solitary choroidal hemangioma, congenital choroidal vascular dysplasia, IPCV may also be associated with congenital choroidal vascular dysplasia.
(two) pathogenesis
The pathogenesis of IPCV is unclear, and there is no large sample of epidemiological data to show the risk factors for IPCV.
Prevention
Idiopathic polypoid choroidal angiopathy prevention
Prevention: According to clinical experience, certain measures can be taken to prevent the increase of intraocular pressure and glaucoma after retinal detachment surgery, such as scleral cerclage and external compression tightness, to avoid post-location; use minimum required amount, low concentration during surgery Inflated gas, such as 18% to 20% SF6 or 12% to 16% C3F8; master the injection amount of silicone oil; minimize the range of retinal condensation and photocoagulation. Active and reasonable anti-inflammatory treatment after surgery, pay attention to monitoring intraocular pressure from the first day after surgery. If necessary, patients with these risk factors may be treated with prophylactic topical or systemic hypotensive medication to avoid further increase in intraocular pressure and further visual impairment caused by glaucoma.
Complication
Idiopathic polypoid choroidal vasculopathy Complications
Retinal detachment is more common.
Symptom
Idiopathic polypoid choroidal vasculopathy symptoms Common symptoms Visual field of vision changes visual deformity Retinal hemorrhage nodules
1. Symptoms may have no obvious symptoms. Some patients may accidentally find it when the fundus examination is performed. If the lesion is located at or near the center of the macula, the visual acuity may gradually decrease. If the vitreous hemorrhage occurs, the visual acuity may suddenly decrease seriously, and the lesion may involve the macular area. The person may have visual distortion.
2. Most of the patients seen in the fundus can see multifocal yellow-white exudation in the fundus. Some patients can see multiple fundus or single orange-red lesions in the fundus. Some patients can see the orange-red lesions in the deep posterior ligament, and the deep fundus is often seen in the fundus. And (or) pre-retinal hemorrhage, one or more hemorrhagic or serous pigment epithelial detachment (PED) are common, lesions are more common in the posterior pole, near the macula and near the optic disc, there are reports in the surrounding Some patients have vitreous hemorrhage, and the fundus cannot be plucked. If the lesion is severely prolonged, serious mechanized membrane formation can be seen in the fundus. In 20 eyes of 16 eyes of Peking Union Medical College Hospital, there are hard exudates in the fundus accounted for 80%. It can be seen that 75% of the patients with orange-red lesions in the fundus, 5 eyes with obvious localized retinal detachment, 80% of the large deep hemorrhage can be seen, 70% of the serous or hemorrhagic PED can be seen, and the formation of IPCV mechanical membrane may be due to Repeated hemorrhage and exudation of an aneurysmal-like lesion of IPCV leads to secondary retinal or choroidal neovascularization.
Examine
Examination of idiopathic polypoid choroidal vasculopathy
No special laboratory tests.
1. Fundus fluorescein angiography (FFA) varies according to the condition. Generally, there is fluorescein leakage in the visible lesion area and accumulation of fluorescein in the late stage of angiography. If deep hemorrhage, fluorescent masking is observed, serous Pigment epithelial detachment is a clear fluorescence with clear boundaries. Hemorrhagic pigment epithelial detachment often shows obvious fluorescence patterns of sluice hemorrhage (Fig. 1C). Some authors report abnormal choroidal branch vascular network by FFA examination, especially when this abnormality occurs. The branch vessel network has small diameter and RPE atrophy, and it is easier to find when the barrier effect is weakened. In short, the FFA morphology of IPCV has no obvious specificity, and its fluorescence characteristics are similar to the occult choroidal neovascularization of wet AMD.
2. Indocyanine green angiography (ICGA) ICGA is critical for the diagnosis of IPCV. Because IPCV is a choroidal vascular abnormality, it can only be clearly shown by ICGA. The typical ICGA features of IPCV are: 1 choroidal abnormal branch vascular network The abnormal vascular network has a larger vessel diameter than the choroidal neovascularization of AMD, which can be fan-shaped or radial, often showing nourishment of blood vessels; 2 an angioma-like dilated nodule can be seen at the end of the abnormal vascular network. Polypoid-like structures, which may be single or multiple in series, with serous or hemorrhagic PED often seen at the margins of polypoid-like lesions, which is a significant imaging feature for diagnosing the disease (Fig. 1E), sometimes due to This abnormal blood vessel is very small, or due to bleeding, exudation, pigment epithelium detachment and other reasons, but not typical choroidal abnormalities of the branch vascular network, there are individual cases due to scarring of the lesion or due to hemorrhagic pigment epithelial detachment at the edge This hemangio-like dilated structure is not covered or masked (Fig. 1F). Most of the polypoid lesions are located near the macular area, and Sho et al report polypoids. By varying the macula is located in 85% of cases reported Uyama the like, polypoid lesions were macula is located as high as 94%.
3. Optical coherence tomography (OCT) examines the corresponding part of the nodular-like strong fluorescence seen by ICGA. OCT shows a dome-shaped bulge under the pigmented epithelium. The subretinal orange-red lesion shows a high elevation on the OCT, and the slurry The retinal pigment epithelial detachment is different. The latter is a flattened bulge. The abnormal blood vessels are located between the Bruch's membrane and the retinal pigment epithelium. Therefore, IPCV is presumed to be a special type of AMD.
Diagnosis
Diagnosis and differentiation of idiopathic polypoid choroidal vasculopathy
The disease is mainly based on ICGA results, that is, abnormal choroidal branch vascular network and aneurysmal dilated lesion structure to make a diagnosis, in addition to the fundus orange red polypoid lesions and multifocal recurrent serous or hemorrhagic pigment epithelial detachment is also diagnosed An important reference for the disease, but recently many authors do not emphasize the need to find abnormal choroidal branch vascular network in ICGA. It is believed that as long as the nodular shape is seen in the late stage of ICGA angiography, the angioma-like strong fluorescence structure can make IPCV. The diagnosis can be diagnosed if the patient's ICGA shows a typical vascular network with a typical choroidal abnormality, and a fundus examination reveals a polypoid lesion and/or serous or hemorrhagic pigment epithelial detachment.
