Sympathetic ophthalmia
Introduction
Introduction to sympathetic ophthalmia Sympatheticophthalmia refers to bilateral granulomatous uveitis after penetrating or internal eye surgery. The injured eye is called the induced eye, the uninjured eye is called the sympathetic eye, and the sympathetic ophthalmia is the general term. The incidence of sympathetic ophthalmia accounts for 1.2% of perforating eyeball injuries. The interval between eyeball penetrating injury and sympathetic ophthalmia is mostly between 2 weeks and 1 year, and 2 to 8 weeks is considered the most dangerous. Stage. basic knowledge The proportion of illness: the incidence rate is about 0.005%-0.007% Susceptible people: no specific population Mode of infection: non-infectious Complications: cataract glaucoma edema retinal detachment
Cause
Causes of sympathetic ophthalmia
(1) Causes of the disease
More than 90% are caused by penetrating injury and internal eye surgery, and a few are seen in the malignant melanoma tissue necrosis, perforation of corneal ulcer, ciliary body condensation or photocoagulation.
(two) pathogenesis
There is no unified understanding of its pathogenesis, mainly autoimmune, viral infection or a combination of the two.
Self-immunity
Some studies have suggested that this disease is a delayed type hypersensitivity reaction caused by the pigment protein of uveal melanocytes or some other component of cells. The uveal pigment is a hidden antigen, which destroys the normal structure of uveal membrane due to trauma and other reasons. Lymphatic system intervention, stimulating immune-active cells, producing cellular immune responses, animal experiments have shown that retinal pigment epithelium and purified retinal soluble antigen can also stimulate the same uveal inflammation as the human eye, the disease is immune In the genetic background, the detection rate of HLA-DR4 and HLA-DRW53 was significantly higher in patients than in other populations.
2. Viral infection and virus - autoimmunity
Although most scholars tend to autoimmune theory in recent years, it is not possible to rule out the possibility of viral infection and its effects. Ikui et al. observed 100 specimens of this disease by electron microscopy and found virus-like particles in epithelioid cells. Is a leucovirus, which has the characteristics of invading the uveal membrane. Hager believes that this disease is caused by the same virus as Vogt-Koyanagi-Harada syndrome. The infection is only different. The virus damages the uveal pigment cells, and the pigment is free and giant. After phagocytic treatment, T lymphocytes are activated and become antigens, further forming an antigen-antibody reaction, thereby affecting all organs containing pigments, such as inner ear labyrinth, skin, hair, etc., so this infection factor at least plays an immunoadjuvant role. The eyeball penetrating injury provides an opportunity for the intraocular antigen to reach the local lymph node, so that the intraocular tissue antigen can contact the lymphatic system and cause an autoimmune reaction.
Prevention
Sympathetic ophthalmia prevention
There are many predisposing factors for the occurrence of sympathetic ophthalmia. Reasonable preventive measures can reduce the incidence of this disease. The current preventive measures mainly include the following aspects:
1. Remove the injured eye
There is still much controversy about the role of removing injured eyes in preventing the occurrence of sympathetic ophthalmia. Most of the reports in the literature are non-randomized uncontrolled studies. The conclusions obtained are still unconvincing. The current views are roughly as follows. Kind:
(1) Removal of the injured eye within 2 days after the injury (48h) may have a preventive effect. Although scholars who hold this view cannot present convincing evidence, it seems reasonable, but it is also pointed out that the contents of the eyeball Exterminating surgery seems to have no preventive effect on this disease.
(2) Removal of the injured eye within 2 weeks after the injury may have a preventive effect on sympathetic ophthalmia.
(3) Removal of the injured eye within 2 weeks after the onset of sympathetic ophthalmia has no preventive effect on the occurrence of sympathetic eyes, but may be helpful for visual prognosis.
(4) Removal of the injured eye after the onset of sympathetic ophthalmia may aggravate uveitis in the sympathetic eye.
(5) Removal of the injured eye after sympathetic ophthalmia has no effect on uveitis in the sympathetic eye.
It is generally believed that the eyeball that has no hope of restoring vision and appearance after the eyeball penetrating injury should be removed immediately. For those who have recurrent uvitis after injury, and the visual acuity is reduced to no light, the injured eye should also be removed. Patients with visual acuity (including good light localization) should preserve eyeballs regardless of whether or not sympathetic ophthalmia occurs. Current medical treatment usually allows the patient's inflammation to be fully controlled. After inflammation control, the patient's vision can usually be obtained. Recovery, or management of complications such as cataracts after inflammation control, is expected to improve vision.
2. Handle the wound correctly and timely
Eyeball penetrating injury should be debrided and sutured in time. The wound incarceration of the iris ciliary body is an important factor in inducing sympathetic ophthalmia. Therefore, care should be taken to avoid incarceration of the intraocular tissue in the wound during debridement and suture. It is possible to suture the wound under the microscope to avoid excessive tissue damage. The wound contamination and infection may induce an immune response through the action of an adjuvant, which is a predisposing factor for the occurrence of sympathetic ophthalmia. Therefore, broad-spectrum antibiotics should be used throughout the debridement and suture. And glucocorticoids, reduce the chance of infection after injury and reduce tissue inflammatory response.
3. Avoid repeating internal eye surgery in the same eye as much as possible
Because repeated internal eye surgery (such as multiple retinal reattachment) may lead to sympathetic ophthalmia, endoscopic surgery with no possibility or possibility of restoring vision should be avoided as much as possible. When such surgery is necessary, glucocorticoid therapy should be given before and after surgery, and if necessary, combined with other immunosuppressive drugs.
Therefore, in the case of ocular trauma, in addition to the apparent disregard of function, most of the contents of the eye are lost, should be removed within 2 weeks after the injury to prevent the occurrence of sympathetic ophthalmia, we must try to save the eye that may retain useful vision, in short Eyeball removal surgery should be very cautious after eye trauma.
Complication
Sympathetic ophthalmia complications Complications cataract glaucoma edema retinal detachment
Sympathetic ophthalmia is one of the most serious eye diseases in ophthalmology. Patients often have one eye injury but blind eyes. The complications of sympathetic ophthalmia are closely related to the location of inflammation and the severity, duration, and recurrence of inflammation. Anterior uveitis usually causes complicated cataracts, secondary glaucoma, and inflammation of the posterior segment of the eye can cause subretinal neovascularization, chorioretinal scar formation, macular edema and retinal detachment, recurrent inflammation and chronic inflammation easily cause the above complications .
Symptom
Sympathetic Ocular Symptoms Common Symptoms Eye pain is consistent with pulse... Pigmented eyes, tinnitus, retinal detachment, ciliary congestion, uveitis, photophobia loss, visual distortion
Symptoms of sympathetic ophthalmia after trauma, a few hours, the elderly can reach more than 40 years, 90% occur within 1 year, the most dangerous time is 4 to 8 weeks after the injury, especially the injury and ciliary There is a uveal incarceration in the body or wound, or foreign matter in the eye is more likely to occur.
1. Stimulating eyes:
Wound healing after eyeball injury, or inflammation after healing, refractory ciliary congestion, acute irritation, edema of the posterior fundus, optic disc congestion, corneal KP after corneal, turbid aqueous humor, iris Thick and dark.
2, sympathetic eye:
At first, there were slight symptoms, eye pain, photophobia, tearing, blurred vision, irritating symptoms, mild ciliary congestion, aqueous humor, fine KP, and a inflammatory reaction with the development of the disease. The iris texture was unclear. , pupil diminution and posterior iris adhesion, pupillary margin nodules, pupillary atresia, vitreous opacity, papillary congestion, edema, peripheral choroid visible fine yellow-white like glassy sputum-like lesions, gradually fused and spread, spread throughout the choroid, restored After the period, the pigmentation of the fundus remains, the pigmentation and pigmentation disorder, and the sunset may appear as "sunset red".
After trauma, the uveal inflammation of the injured eye continues or worsens, or relapses after quiescence. After an incubation period, the same side of the eye appears to have inflammation of the same nature, and sympathetic ophthalmia should be considered.
Originating part
According to the origin of sympathetic eye inflammation, it can be divided into two clinical manifestations: the anterior segment of the eyeball and the posterior segment.
1. Anterior segment of the eye
Inflammation begins in the anterior segment and manifests as severe or severe photophobia, tearing, blurred vision, ciliary congestion, tenderness in the ciliary area, KP, Tyndall phenomenon, disappearance of iris texture, dilated pupils, and other iris ciliary bodies. Symptoms and signs of inflammation, if not treated promptly or when treatment is ineffective, may occur after the iris adhesion, pupillary atresia or membrane closure, and even new blood vessels appear on the iris surface and membrane closure.
2. Posterior segment of the eye
Inflammation started in the posterior segment. The patient complained of a significant decrease in visual acuity, accompanied by a sense of flash, small vision or visual distortion, no obvious change in the anterior segment of the eye, or only a few gray-white KP, Tyndall phenomenon was weakly positive, and the vitreous had varying degrees of dusty gray-white opacity. .
Two different changes are common under ophthalmoscopy: sympathetic disseminated chorioretinitis and sympathetic exudative chorioretinitis.
(1) Sympathetic disseminated choroidoretinitis: There are scattered round yellow-white exudation spots near the equator of the fundus, which is larger than the drusen, but not more than twice the diameter of the primary branch of the central retinal vein. There is no obvious abnormality on the surface and surrounding retina. The exudation spots disappear on their own within 2 to 3 weeks, and new spots appear continuously. During the disappearance process, the edges often see pigmentation halos, and the center may have pigmentation spots.
In the yellow-white spots, FFA angiography was weakly fluorescent at the early stage, followed by fluorescence leakage and strong fluorescent plaques. The late fluorescent spots persisted, suggesting that the tissue was stained. After the oozing spots disappeared, the lesions showed fluorescence, and the pigmentation was fluorescent. .
(2) Sympathetic exudative choroidoretinitis: the initial macular center reflex disappears, the retina is grayish white edema, turbid or radial wrinkles, and then the edema range is rapidly expanded, and the edges may have yellow and white sizes. Exudation of the lesion, optic disc congestion, retinal blood vessels, especially venous filling and distortion, short-term inflammation into the peak, retinal edema, loss of intrinsic transparency into grayish white, retinal detachment visible under the fundus, severe hemispherical bulge, no holes, ie seepage With sexual detachment, as the inflammation gradually subsides, the subretinal fluid gradually absorbs, and it is self-resetting, leaving no traces of yellow-white or linear scars. After retinal edema disappears, the macula has fine pigment spots, and other parts of the fundus are scattered. Different, different sizes of pigmentation and depigmentation, the location is equivalent to the choroidal exudation lesion at the peak of inflammation. In severe cases, the pigmented epithelium and choroidal pigment are destroyed, making the entire fundus appear as the sun sets. Red, called the sunset-like fundus.
During the FFA background fluorescence period, the posterior pole of the fundus including the circumference of the optic disc can be scattered at the fluorescent leakage point, rapidly expanding and enhancing, and fused into a large piece of strong fluorescence. If there is exudative retinal detachment, the dye enters the exudate, and the whole The detachment zone is strongly fluorescent, and after the inflammation subsides, the FFA is a plaque-like fluoroscopy and fluorescent occlusion of the pigment accumulation.
Sympathetic ophthalmia, regardless of its inflammation originating in the anterior or posterior segment of the eyeball, will eventually spread to each other, only the severity is different. In fact, the symptoms of the anterior and posterior segments are not rare at first, only because of the inflammation of the anterior segment. The fundus can't be seen.
In patients with sympathetic ophthalmia, in some cases, hair whitening, hair loss, skin discoloration, tinnitus, deafness and other systemic diseases appear in the late stage of the disease.
Examine
Sympathetic ophthalmia
Mainly: comprehensive ophthalmologic examination including dilated fundus, complete blood count, RPR, FTA-ABS; consider sarcoma, ACE level; chest X-ray to exclude tuberculosis or sarcoma; fundus fluorescein angiography or B Super check, etc.
Histopathological examination:
The pathological examination of irritating eyes and sympathetic eyes, except for the traumatic changes of the irritating eyes, is completely the same, and all have the characteristics of granulomatous uveitis. The whole uveal tissue is thickened by inflammatory cell infiltration, especially the choroid. Significantly, the thickness can reach 2 to 3 times or even 5 to 6 times of normal. The whole uvea has lymphocytes, epithelioid cells and Langerhans giant cells infiltrating. Choroidal lesions begin in the large blood vessel layer, and lymphocyte infiltration occurs around the blood vessels. Forming typical nodules, the center of the nodules are epithelioid cells and giant cells, surrounded by lymphocytes, and some can also see plasma cells, much like tuberculous nodules, but the giant cells phagocytic pigments in this nodule are obvious, and not There is little or no case-like necrosis, and the choriocapillaris layer is less invaded due to lack of pigmentation, and it is inevitable when the lesion develops to a certain extent. The retinal pigment epithelial layer covering it has localized hyperplasia and is flattened. Shaped or nodular bulge, pigment cells grow into fusiform, mixed with epithelioid cells and giant cells, called Dalen-Fuchs nodules, such nodules are not It is unique in the disease, and is also present in Vogt-Koyanagi-Harada syndrome. There are lymphocytes around the retinal vein, and epithelial-like cells infiltrate, that is, sympathetic perivascular inflammation. In the early stage of inflammation, the iris is mainly in the posterior layer, so it is prone to occur. Adhesion, after the cell infiltration is intensified, the tissue is nodular and hypertrophy, the surface is uneven, the ciliary body inflammation starts from the vascular layer, and sometimes the Dalen-Fuchs nodule can be seen. The nodule is transformed by the retinal pigment epithelial cells. Proliferated.
Fundus examination:
It is most meaningful to have Dalen-Fuchs nodules and sunset-like fundus changes in injured or non-surgical eyes.
Fluorescein fundus angiography:
It is helpful for diagnosis. The most common and typical manifestation of the acute phase of the disease is multiple strong fluorescent dot-like leakage at the level of retinal pigment epithelium in the venous phase. In the later stage, these strong fluorescent spots can be fused into flake strong fluorescence. (Fig. 1), in patients with severe inflammation, a lake-like change can be formed. In the chronic phase of recurrent or recurrent uveitis, the most common change is the appearance of multiple weak fluorescence areas in the early stage of angiography, and later staining. The location of fluorescence changes is consistent with the distribution of clinically seen Dalen-Fuchs nodules. Another common manifestation is that the optic disc can show fluorescence leakage at an early stage. In some patients, retinal fluorescein leakage, blood vessels can still be found. Wall staining, etc. changes.
Indocyanine green angiography is also helpful for diagnosis. In the active phase of the disease, multiple weak fluorescent dark areas can be found, but the distribution is not as regular as the weak fluorescent dark area of Vogt-Koyanagihara disease, in recurrent or chronic inflammation. The patient can see the weak fluorescent dark areas corresponding to the Dalen-Fuchs nodules seen under the ophthalmoscope.
Type B ultrasonic inspection:
Choroidal thickening can be found, and this change supports the diagnosis of sympathetic ophthalmia.
Diagnosis
Diagnosis of sympathetic ophthalmia
Strictly speaking, the most accurate diagnosis of sympathetic ophthalmia must be based on histopathology, but this disease is a serious eye disease that can cause blindness in both eyes. For timely rescue, early diagnosis is very important, and can not wait for the results of pathological examination. Delayed treatment, in addition, when the stimulating eye still retains certain vision, it can not rush to remove the eyeball, so when the penetrating traumatic eye inflammation persists, if the healthy eye has symptoms such as photophobia, tearing, visual fatigue, etc., it should be checked immediately, such as Slit lamp microscope and ophthalmoscope without any positive signs, for sympathetic stimulation, no special pathological significance, but should also be reviewed several times a day or several times a day, especially within 8 weeks after injury, more need to be highly vigilant sympathetic Ocular inflammation, on the other hand, if a anterior or posterior segment of uveal inflammation is found, a clinical diagnosis of sympathetic ophthalmia can be made.
diagnosis
1. Have a history of eyeball penetrating injury and inflammatory reaction in both eyes.
2. When the sympathetic eye appears in the KP anterior chamber and the anterior vitreous has floating matter and flashing, the occurrence of sympathetic ophthalmia can be considered.
3. After removing the irritating eye that has been blind, it can be further diagnosed by pathological examination.
Differential diagnosis
1. For those who have a history of trauma and another eye with irritation, try to eliminate the primary lesion.
2, exclude crystal uveitis, uveal encephalitis (VKH): they have common points that are difficult to identify, but also have their own characteristics.
3. Identification with Behcet's syndrome (behcet's disease).
4, lens allergic ophthalmia (phacoallergic ophthalmia) more common in cataract surgery or lens traumatic capsule rupture, some cases can cause uveitis, it is very confused with this disease, but the former in the other eye inflammation, The inflammation of the surgical eye is completely or substantially static, and the disease is the opposite. The inflammation of the uninjured eye (sympathetic eye) occurs when the inflammation of the traumatic eye (irritating eye) continues or increases; in addition, the lens allergic ophthalmia is the lens The protein is allergic and the lens remains in the eye. However, the identification of the two is not only clinically very difficult, but also histopathologically difficult to distinguish. For example, some specimens can see granulomatous grapes of sympathetic ophthalmia. A typical change in membranous inflammation, granulomatous inflammation around the lens cortex of the lens allergic ophthalmitis can be seen. It has been suggested that the lens protein reaction can induce sympathetic ophthalmia, suggesting that the lens protein and the retina have common antigenicity.
