shotgun retinochoroidopathy
Introduction
Introduction to shotgun-like retinal choroidal lesions Shotgun-like retinal choroidopathy (birdshotretinachoroidopathy) was first officially reported by Ryan and Maumenee in 1980. It is a rare chronic bilateral choroidal retinitis characterized by multiple subretinal creamy lesions and retinal vasculitis. With cystoid macular edema, optic disc edema and vitreous inflammation. basic knowledge The proportion of illness: the incidence rate is about 0.0001% - 0.0002% Susceptible people: no special people Mode of infection: non-infectious Complications: vitreous hemorrhage
Cause
The cause of shotgun-like retinal choroidal lesions
(1) Causes of the disease
The cause is still unclear and is presumed to be an autoimmune ocular inflammatory disease with the HLA-A29 allele.
(two) pathogenesis
The pathogenesis of this disease is not fully understood. Extensive research has found that immune genetic factors play an important role in its pathogenesis. It has a close relationship with HLA-A29 antigen. According to the results reported by several groups of authors, patients with this antigen The positive rate was as high as 61.9%96%, the control group was 5.6%30%, and the relative risk was up to 224. The histological observation of the eyeball revealed that the iris ciliary body had focal lymphocyte and plasma cell infiltration, subretinal and retina. There is a granuloma formation, and there is diffuse infiltration of lymphocytes in the nearby choroid. These results suggest that the autoimmune response (possibly against the retinal S antigen) plays an important role in its occurrence, and some studies suggest that the pineal gland may play a role in this disease. Certainly works.
Prevention
Shotgun-like retinal choroidal lesion prevention
There are no effective preventive measures for this disease. Pay attention to health, do a good job of safety protection, reduce and avoid the irritating and accidental damage of adverse factors, can play a certain preventive role. In addition, early detection, early diagnosis and early treatment are also the key to the prevention and treatment of this disease. In case of onset, active treatment should be actively treated to prevent complications.
Complication
Complications of shotgun-like retinal choroidal lesions Complications, glass volume, optic nerve atrophy
The most common complication of this disease is chronic macular cystic edema, the incidence of up to 50% to 62.6%, is a common cause of central vision loss, this complication is not only helpful for diagnosis, but also an important indication for treatment, retina The incidence of anterior membrane is about 10%, which can lead to a significant decrease in visual acuity. Macular wrinkles can occur in the process of inflammation regression, affecting patients' vision, and can also cause optic disc neovascularization (7%) and subretinal neovascularization (6%). , vitreous hemorrhage, optic atrophy, complicated cataract, iris reddenation, rhegmatogenous retinal detachment.
Symptom
Shotgun-like retinal choroidal lesion symptoms Common symptoms Congestive retinal hemorrhage Dark spot visual impairment Color vision abnormality Eye shadow night blind macular cystic edema Eye purple blood inflammatory cell infiltration
Patients with shotgun-like retinal choroidal lesions usually show varying degrees of visual acuity, often accompanied by premature black shadows, but no other discomfort, visual decline in the early stage of the disease, can be monocular, can almost evolve into both eyes as the disease progresses However, the degree of decline is more asymmetrical in both eyes, and patients often have obvious visual impairment, but the results are only mildly decreased. A few patients have a sense of flash, night blindness or abnormal color vision.
The eye examination showed no anterior segment congestion. The slit lamp examination occasionally showed floating cells in the anterior chamber or fine corneal posterior deposition (KP) and post-iris adhesion. The vitreous had obvious inflammatory cell infiltration during the active period of the disease. Sex cells can sometimes aggregate into a sheep fat-like mass deposited on the surface of the posterior vitreous.
Ophthalmoscopy can be seen in the multiple fundus, creamy light-colored spot lesions, the lesions are round or elliptical, the size is 50 ~ 1500m, the boundaries are not clear, occasionally fusion, radial distribution in the posterior pole to the equatorial fundus More common in the vicinity of the optic disc and below or in the nasal region, often along the choroidal large blood vessels, the center of the macula is not involved, slit lamp plus front mirror or three-sided mirror see the lesion located in the outer layer of the retina, RPE and choroid, lesions There is no obvious change in the surface retina, and there is no pigmentation in the lesion or its edge. Some lesions can fuse with each other. The cystoid macular edema and optic disc edema are common manifestations, and there may be inflammatory changes of the retinal vessels such as venous sheath formation. Arterial constriction, vascular distortion or retinal hemorrhage.
At the fundus fluorescein angiography, abnormal fluorescence patterns such as vascular leakage of the retina and optic disc, coloration of the blood vessel wall and cystoid macular edema are common. Most lesions are obscured in the early stage of angiography, mild fluorescence in the late stage, and some creamy lesions. Although clearly visible under indirect ophthalmoscopy, there is no abnormal change in angiography, so the abnormality of fluorescein angiography is often lighter than that seen under indirect ophthalmoscopy. In contrast, indocyanine green angiography can clearly show Other lesions that could not be seen by the examination, creamy lesions showed clear choroidal non-fluorescent dark spots, a few patients showed fluorescence images of retinal neovascularization, but no capillary perfusion area was present, ERG examination a wave more For normal, the b-wave amplitude decreases and the latency increases.
Examine
Examination of shotgun-like retinal choroidal lesions
1. HLA-A29 antigen typing Most patients are positive for HLA-A29 antigen. If this test is found to be positive, the posterior probability of the disease (the possibility of disease) can be greatly improved.
2. Other laboratory tests According to clinical needs, some laboratory tests on syphilis and tuberculosis can be performed to eliminate these diseases, and serum angiotensin-converting enzyme and lysozyme can be used to help differential diagnosis of sarcoma-like diseases. , liver and kidney function and other aspects of the examination, to help clinically select drugs.
3. Fluorescein fundus angiography The creamy changes in the fundus can be expressed in fluorescein fundus angiography:
1 There can be no abnormal changes in angiography. There are two possibilities in this case. One is that the lesion is very early; the second is that the lesion is located in the outer layer of the choroid, and the choroidal capillaries are not affected.
2 Early angiography showed a small range of weak fluorescence in some creamy lesions, which may be related to changes in retinal pigment epithelium and mild choroidal capillary perfusion abnormalities at the lesion site.
3 fluorescein fundus angiography early creamy lesions showed weak fluorescence, choroidal vessels can be seen through atrophic or depigmented retinal pigment epithelium and non-perfused choroidal capillaries, corresponding to creamy lesions Strongly fluorescent lesions (Fig. 1), which are common changes in creamy lesions, especially in the middle and late stages.
Retinal vasculitis may vary greatly between patients and in the same patient. The most common changes are focal venous and capillary leaks in the posterior pole, diffuse vascular leakage and along the retina Strong fluorescence of blood vessels, some patients may have leakage of the entire retinal capillary bed on both sides.
Other common changes include optic disc strong fluorescence and cystoid macular edema, and diffuse retinal pigment epithelial atrophy can be found.
4. Indocyanine green angiography can detect a large number of weak fluorescent choroidal lesions in the middle of indocyanine green angiography. Weak fluorescence may reflect choroidal capillary hypoperfusion. These lesions are consistent with the seen shotgun-like lesions, but the angiography shows The number far exceeds the number of lesions found in clinically and fluorescein fundus angiography, suggesting that it is a diffuse inflammatory disease that is often in the vicinity of large and medium-sized blood vessels, but the choroidal blood vessels themselves are absent. Involved, some strong fluorescent spots can be seen in the later stage of angiography.
5. Electrophysiological examination of the retinal current map shows that the a wave is normal, the b wave is reduced, and the latency is prolonged. This change indicates that the inner layer of the retina is involved, and the photoreceptor-retinal pigment epithelium-choroid complex is not affected or slightly affected. The functional damage of the patient is mainly caused by retinal vasculitis instead of choroidal inflammation. Other abnormalities have disappeared from the oscillation potential to the inability to record changes in the retinal current map. It is also found that the dark adaptation threshold is increased, the visual evoked potential amplitude is decreased, and the response is delayed.
6. Other examinations Visual examination of the patient can reveal multiple visual field defects, such as narrowing of the peripheral visual field, central dark spot or paracentral dark spot; patients may also have acquired color vision abnormalities, mostly blue-yellow, some patients also It can be of two types: blue-yellow and red-green.
Diagnosis
Diagnosis and identification of retinal choroidal lesions
diagnosis
The diagnosis of this disease is mainly based on the typical clinical manifestations, that is, the characteristic multiple creamy lesions. This lesion is more clear under the absence of red light and indirect ophthalmoscopy, without vitreous changes of the snow levee-like, macular Cystic edema and mild anterior segment inflammatory response generally do not require laboratory and auxiliary examinations, but these tests are helpful for differential diagnosis. The diagnostic points of shotgun-like retinal choroidal lesions are as follows:
1. The general characteristics of Caucasian; the age is more than 30 years old, the average age is 50 years old; the incidence of both eyes.
2. Eye performance:
(1) Characteristic manifestations: multiple creamy lesions after the equatorial region, 50 ~ 1500m size; cystoid macular edema; retinal vasculitis.
(2) Other manifestations: optic neuritis; optic atrophy; acute anterior ischemic optic neuropathy; mild non-granulomatous iridocyclitis; subretinal neovascularization.
3. Auxiliary inspection
(1) HLA-A29 antigen is positive.
(2) fluorescein fundus angiography: weak fluorescence in the early stage of the lesion, strong fluorescence in the later stage; retinal vascular leakage; cystoid edema of the macula.
(3) Indocyanine green angiography: corresponding to weak fluorescent dark spots at the creamy lesion; the number of lesions was found to be much more than the number of clinical and fluorescein fundus angiography; weakly fluorescent lesions were located near the large and middle vessels; Weak fluorescent dark spots.
(4) Electroretinogram, electro-oculogram and dark adaptation.
Differential diagnosis
1. The vitreous cell infiltration of uveitis in the middle is sometimes confused with the shotgun-like retinal choroidal lesion, but the former lacks the creamy fundus of the fundus, while the latter does not have the exudation of the surrounding snow-like dyke.
2. The inflammatory manifestations of Harada's eyes are similar to this disease, but there are many serous retinal detachments without cream spots, and obvious changes of chorioretinal atrophy can be seen after subretinal fluid absorption.
3. Sympathetic ophthalmia Vitreous inflammation and yellow-white lesions in the fundus are similar to this disease, but there are many traumatic or surgical history in the contralateral eye.
4. Acute posterior multifocal squamous pigment epithelial lesions and multifocal choroiditis with total uveitis may also have choroidal lesions with vitreous inflammation, but the fundus atrophy lesions are often accompanied by pigmentation changes.
5. Intraocular lymphoma can present with uveitis, but the lesions are different from the disease.
