Year : 1998 | Volume
: 46 | Issue : 3 | Page : 131--137
Idiopathic central serous chorioretinopathy
D Hussain, JD Gass
Department of Ophthalmology and Visual Sciences, Vanderbilt University School of Medicine, Nashville, USA
Department of Ophthalmology and Visual Sciences, Vanderbilt University School of Medicine, Nashville
Idiopathic central serous chorioretinopathy (ICSC) is usually seen in young males with Type A personality. Clinical evaluation of the macula with fundoscopy and biomicroscopy, coupled with fluorescein angiography establishes the diagnosis. Indocyanine green angiographic studies have reinformed that the basic pathology lies in choriocapillaries and retinal pigment epithelium. Most of the ICSC resolve completely in four months, and some of them could resolve early with direct photocoagulation of the leaking site. Oral steroids have no role, and could even cause an adverse reaction.
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Hussain D, Gass J D. Idiopathic central serous chorioretinopathy.Indian J Ophthalmol 1998;46:131-137
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Hussain D, Gass J D. Idiopathic central serous chorioretinopathy. Indian J Ophthalmol [serial online] 1998 [cited 2020 Jul 16 ];46:131-137
Available from: http://www.ijo.in/text.asp?1998/46/3/131/14961
Idiopathic central serous chorioretinopathy (ICSC) is characterised by accumulation of fluid resulting in circumscribed elevation of retina in the posterior pole. The disease was first recognized by Albrecht von Graefe in 1866 and was named central recurrent retinitis. Since then it has been reported under a variety of names such as idiopathic flat detachment of the macula by Walsh et al, central angiospastic retinopathy by Gifford et al, and central serous retinopathy by Straatsma et al. The condition was named Idiopathic central serous chorioretinopathy by Gass et al in 1967.
This condition affects young individuals between the ages of 20-50 years. The disease is seen predominantly in males as compared to females; the ratio is reported to be 10:1. The frequency of occurrence in the female population is found to double between age 31-40 as compared to ages 21-30 years. This condition is associated with psychological factors; it is found in males with Type A personality, accompanies unusual emotional stress, and occasionally is seen with migraine-type headaches.,
The presenting symptoms of the disease are metamorphopsia, micropsia, a relative positive scotoma, and occasional macropsia. These findings can be demonstrated on the amsler grid. Mild blurring of the vision may occur, which can usually be improved by hyperopic correction. In the acute phase visual acuity of 6/6 to 6/60 has been reported with an average of 6/9. There is a delay in retinal recovery time after exposure to bright light, loss of color saturation and loss of contrast sensitivity. The patient may be asymptomatic when the serous detachment does not extend into the center of the macula.
Ophthalmoscopy reveals a circumscribed round or oval area of retinal elevation in the macula. The foveal reflex may be absent or attenuated. Slitlamp biomicroscopy typically shows serous elevation of the retina; there may be a yellow spot in fovea due to increased visibility of the xanthophyll. The subretinal fluid is usually clear but in 10% of eyes the subretinal space is filled with gray-white serofibrinous exudate [Figure:l]a. These cases are associated with larger retinal detachments. Some chronic cases present with dot-like yellow precipitates at the back of the retina. The underlying retinal pigment epithelial (RPE) detachment that may or may not be visible typically appears round or oval, yellow or yellowish grey. It is best detected in retro illumination. The junction of the detached and attached RPE typically produces a circumscribed halo surrounding the base of the lesion. Fine mottling and occasional clumping of pigment resulting in a triradiate or cruciate pattern is common on the surface of the detached RPE.
The diagnosis of ICSC is confirmed on fluorescein angiography, which demonstrates the site of RPE detachment and the site of leakage of serous fluid from the RPE into the subretinal space. Various patterns of dye leakage are seen. Early phase of the angiogram shows hyperfluorescence corresponding to the size of the RPE detachment ([Figure:1]b [Figure:3]b. In 95% of the cases one or as many as seven areas of leakage of dye are seen from the RPE into the subretinal space. In most cases the dye diffuses slowly to fill in the retinal detachment in 20 to 30 minutes. In 10% of cases the dye leaks and spreads in a smokestack pattern in the subretinal space, probably due to convection currents and higher specific gravity of subretinal exudate [Figure:2]. A majority of the leaking sites are within 1 disc diameter area of the fovea. However, the foveola is affected in less than 10% cases. The incidence of leakage sites is greatest in the upper nasal quadrant, followed by lower nasal quadrant, and the lower temporal quadrant. About 25% of the leaks are located in the papillomacular bundle. Extramacular areas of leakage may be present, so it is important to scan the fundus in the late phase of the angiogram.
Indocyanine green angiography
ICG dye is advantageous over fluorescein for visualization of the choroidal circulation, as it absorbs and fluoresces in the infrared range and larger ICG molecules that are protein bound do not leak from choriocapillars, therefore allowing better visualization of the choroidal circulation. ICG studies in ICSC, demonstrate choroidal hyperpermeability and dilated choroidal hyperpermeability and dilated choroidal capillaries and venules in the area of RPE leaks seen on fluorescein, multiple areas of occult serous retinal pigment epithelial detachment in the area of ICSC, and multiple area of choroidal ischemia and hyperpermeability seen throughout the fundus, both in the affected and normal eye.
After resolution of the detachment all the fluorescein findings may return to normal. However, mottled hyperfluorescence may be present due to pigment loss in long-standing retinal detachment. In recurrent cases new leakage points are seen within 1 mm of the old leakage in 80% of the cases, and within 0.5 mm of the old leakage in 62% of the cases.
ICSC in women
ICSC may occur in healthy women in the last trimester of uncomplicated pregnancy. This type is associated with serofibrinous subretinal fluid in 90% of the affected women. The detachment resolves in the postpartum phase and there is excellent visual recovery [Figure:3]. Women receiving corticosteroid are predisposed to ICSC with subretinal fibrin.
ICSC in the elderly
The clinical picture is similar to that seen in the younger age group, although a large number present with signs of previous episodes of eccentric detachment. There is some concern in this age group if the leak represents the area of choroidal neovascularization. There is no evidence of any relationship of ICSC and age-related macular degeneration.,
Prolonged and recurrent serous detachments are seen, particularly in Asians and Hispanics. These patients are asymptomatic in the early stage of the disease, due to the extramacular location of the detachment. They often have flask shaped areas of atrophy of the RPE extending inferiorly from the paracentral and peripapillary areas to the equator or ora., Recurrent detachment leads to atrophy of the RPE and bone corpuscular changes, thus causing loss of field and night blindness. Long standing retinal detachment can lead to lipid exudation and macular edema. These patients are prone to recurrent macular detachment and permanent loss of vision.,
Acute bullous retinal detachment
Occasionally cases with ICSC are associated with large areas of serous retinal detachment and multiple large RPE detachments, involving the midperiphery as well as the posterior pole. The areas of retinal detachment may coalesce to form bullous detachment involving the lower half of retina. This condition is likely to occur in patients receiving systemic steroids [Figure:4]. These include patients with ICSC incorrectly diagnosed as having inflammatory disease and patients, often women, with collagen and vascular diseases. The shifting subretinal fluid may be clear or fibrinous. Fluorescein angiography is diagnostic, and demonstrates underlying multiple areas of RPE detachment, the dye streams through breaks in the RPE and fills in the subretinal space. Occasional large areas of underlying RPE rips may be seen.
Associations of ICSC
ICSC has been seen in patients with retinitis pigmentosa,, chorioretinal folds in the macula, patients with Cushing's disease, episcleritis,[29,30] choroiditis, and rarely leprosy.
There has been great controversy about the etiopathogenesis of ICSC, various theories have been proposed to explain the mechanism of ICSC. The retinal pigment epithelium and Bruch's membrane create a barrier between the choroidal circulation and the retina. Spitznas postulated impairment of few or single retinal pigment epithelial cells that secrete ions in the retinochoroidal direction. This malfunctioning of the RPE pump leads to accumulation of fluid in the subretinal space. Marmor suggested that a focal disruption of the RPE could not cause serous detachment as the neighboring cells would be able to compensate and therefore a diffuse metabolic impairment of the RPE would be the cause of ICSC. Gass and others have postulated focal increase in the permeability of the choriocapillaris, that overwhelms the RPE and causes blister serous elevations of the RPE (RPE detachment). The junction between the attached and detached RPE is stressed mechanically and can cause a rip or decompensation of RPE leading to leakage of electrolytes, protein and water in the subretinal area.[5,12,30] This theory was further supported by indocyanine angiography, which showed choroidal hyperpermeability in the areas of ICSC in all cases studied and pigment epithelial detachment in 75% to 84% all the cases of ICSC.,
Experimental evidence shows that focal damage to choriocapillaris and RPE results in serous detachment in rabbit eyes. Experimental models of ICSC have been produced by intra-scleral injection of indomethacin and repeated injections of epinephrine. These models support the fact that stress plays a role in causing focal permeability change in choriocapillaris.
Diseases that can cause localized detachment should be ruled out. These include optic nerve head pit, choroidal haemangioma, choroidal melanoma, choroidal osteoma, metastatic carcinoma, macular hole with detachment, age-related macular degeneration, malignant hypertension, toxemia of pregnancy, presumed ocular histoplasmosis syndrome, Harada's disease, sympathetic ophthalmia, posterior scleritis, idiopathic uveal effusion syndrome and ocular contusion.
Most ICSC heals spontaneously in 4-8 weeks, with recovery of visual acuity in 6 months. Jalili et al reported that an overall 41% of the eyes with ICSC had a final visual acuity less than 6/6 or a residual change on amsler grid or both. Severe visual loss is reported in 5% of ICSC. Recurrence occurs in one third to one half of all patients, and 10% have three or more recurrences. Half of the recurrences occur within one year but may occur up to 10 years after the first episode. Long-term follow up of ICSC suggests that even single episode may lead to slow progressive disturbance of retinal pigment epithelium at the posterior pole.
The treatment of ICSC is controversial. No medication has been shown to be effective in the management; in fact the use of systemic steroid has resulted in the development of ICSC and worsening of existing ICSC., Argon laser photocoagulation of the RPE underlying the ICSC at a site away from the leakage site on FA (indirect laser photocoagulation) did not alter the course of the disease, but laser application to the site of leakage (direct laser photocoagulation) shortened the duration of ICSC by 2 months and also reduced the recurrence rate., The possible mechanism of laser treatment is debridement of diseased retinal pigment epithelium permitting ingrowth of surrounding healthy RPE and resolution of ICSC. Ficker et al reported long-term results of Argon laser treatment, where it did not affect the final visual outcome at 6-12 years of follow up and also did not affect the recurrence rate or the prevelance of chronic ICSC. Another study reported that treatment did reduce the recurrence rate of ICSC at more than 10 years of follow up. Therefore the treatment is justified only for symptomatic relief by early resolution of the serous detachment.
Since most ICSC resolve spontaneously, immediate photocoagulation is not advisable. The following guidelines have been suggested by Gass:
1. Allow 4 months for spontaneous resolution of the first episode of ICSC.
2. Wait for 6 months or longer before photocoagulation if the RPE leak is less than one fourth disc diameter from the fovea.
3. Allow 1 month for spontaneous resolution in patients with a history of several episodes of detachment in the same eye, if after each episode the patient has regained normal macular function.
4. When the leak is at least one-fourth disc diameter from the center of the fovea, prompt photocoagulation is justified in the following cases. (a) detachment present for 4 months or longer; (b) evidence of permanent loss of acuity or visual field in either eye secondary to previous episodes of ICS; and (c) if for occupational reasons the patient cannot work because of visual dysfunction due to ICSC.
Light to moderate intensity applications of xenon, ruby, argon, krypton or dry laser to the area of RPE leakage or RPE detachment are effective in the resolution of ICSC. It is advisable to choose larger spot size, since the smaller spot size does not seem to influence the rate of recurrence. Complications from the treatment include accidental photocoagulation of macula, fovea distortion, the most significant being a 2-5% risk of development of choroidal neovascularization within several weeks or months after treatment.,, A careful follow up with amsler grid by the patient is recommended for early detection and possible treatment of this complication.
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