|Year : 2000 | Volume
| Issue : 4 | Page : 315-7
Retinal pigment epithelial tears associated with idiopathic central serous chorioretinopathy
MP Shanmugam, M Bhende
Medical Research Foundation, Chennai, India
M P Shanmugam
Medical Research Foundation, Chennai
Source of Support: None, Conflict of Interest: None
Keywords: Adult, Choroid Diseases, complications, diagnosis, Diagnosis, Differential, Edema, Fluorescein Angiography, Fundus Oculi, Humans, Laser Coagulation,
|How to cite this article:|
Shanmugam M P, Bhende M. Retinal pigment epithelial tears associated with idiopathic central serous chorioretinopathy. Indian J Ophthalmol 2000;48:315
Two patients with idiopathic central serous chorioretinopathy with retinal pigment epithelial tears are described.
Indian J Ophthalmol 2000;48:315-17
Retinal pigment epithelial (RPE) tears were first described by Hoskins et al and are most often reported in age-related macular degeneration. They are also known to occur in trauma, histoplasmosis, choroidal effusion, laser photocoagulation, retinal detachments, chorioretinal scars, metastatic carcinoma of choroid, and carotid-cavernous fistula. There are reports of RPE tears in idiopathic central serous chorioretinopathy (ICSC). [2,3] We report two patients of ICSC who presented with RPE tears and exudative retinal detachment.
| Case report 1|| |
A 40-year-old diabetic patient presented with sudden decrease in his right eye vision. His left eye was normal. He had 6/60;N36 vision in the right eye and 6/6 in the left eye. Fundus examination showed an inferior retinal detachment extending from the arcades to the pars plana. RPE tears were noted temporal to the fovea [Figure:1a]. Small multiple RPE detachments were seen in the posterior pole. No evidence of inflammation or choroidal neovascular membrane was seen.
Fundus fluorescein angiography (FFA), showed hyperfluorescence in the area of the tear, beginning in the choroidal phase and increasing in intensity with the progression of the angiogram; but this did not leak the dye. The edges of the tear showed blocked fluorescence [Figure:1b]. There was minimal dye leakage from the RPE detachments in the late phase of the angiogram. The leaking RPE detachments were photocoagulated and the tear was not treated. The patient's vision improved to 6/9;N12 three months later. The retinal detachment decreased and the tears were covered with glial tissue. Eight months after treatment, he had 6/6;N6 vision and reattached retina in his right eye.
| Case report 2|| |
A 47-year-old hypertensive male presented with sudden loss of left eye vision for 2 months. He had 6/6; N6 vision in right eye and 6/36, N36 in the left eye. Anterior segment of both eyes showed early posterior subcapsular cataract. The right eye fundus showed an RPE detachment without sensory retinal detachment superotemporal to the fovea. The left eye fundus showed a large RPE tear superotemporal to the fovea [Figure:2a] and inferior exudative retinal detachment. Two small pigment epithelial detachments were seen along the inferotemporal arcade. The RPE tear showed typical features on fundus flourescein angiography and did not leak fluorescein [Figure:2b]. The RPE detachments showed late leakage and were photocoagulated. Two months later, the patient's vision improved to 6/24; N 12 with reattachment of the retina. The RPE tear was covered with glial tissue. Pigmentary alterations at the fovea were the cause for poor visual recovery.
| Discussion|| |
A Medline search yielded only two reports of RPE tears in association with ICSC in literature. [2,3] Most patients with ICSC develop one or more serous detachments of the RPE in the macular or paramacular region. These RPE detachments in ICSC are typically small, but in some patients, they may become larger and be surrounded by a reddish or salmon-pink halo caused by a marginal serous separation of the retina. These large detachments may be prone to tear formation.
The pathogenesis of ICSC has been postulated to be due to focal increase in the permeability of the choriocapillaries leading to the damage of overlying RPE, focal loss of RPE attachment to Bruch's membrane and movement of plasma proteins and water into the subretinal space. Spitznas has suggested that the reversal of the direction of ion secretion by focally damaged RPE allows water to move towards the retina rather than the choroid. This increased fluid beneath the pigment epithelium in ICSC causes tangential stress leading to the formation of a tear. RPE tears usually occur at the edge of the RPE detachment, scrolling away from that edge.
Acute RPE tears lead to leakage of subpigment epithelial detachment fluid into the sub retinal space causing decrease in vision. In the presence of a RPE tear, fluid can escape from the choriocapillaries and cause a bullous detachment, as seen in both our patients. The RPE tear has been postulated to compromise the function of the remaining RPE cells thereby allowing continuous fluid leakage. As a repair mechanism, a layer of hypopigmented RPE cells covers the defect, thereby blocking further ingress of fluid. In addition, partial closure of the choriocapillaries may take place, further decreasing the fluid leakage. Hence these self-sealing RPE tears need not be treated with laser photocoagulation. However, any associated CSR leaks as in our patients, will need laser photocoagulation.
RPE tears are repaired by three mechanisms. In a majority of cases, the tear is covered by a layer of fibrous tissue. In a few eyes, the inner surface of the Bruch's membrane remains relatively unaltered, and in some, the pigment epithelium resembling normal RPE covers the defect. In both cases described here, fibrous scarring was seen over the tear on follow up.
It is important to differentiate these patients from those with RPE tears associated with age-related macular degeneration since patients with RPE tear and ICSC have relatively better visual prognosis. An important differentiating feature is that while RPE tears due to ICSC are usually associated with large, inferior retinal detachments, it is uncommon in patients with ARMD, though Silvestri et al have reported one such case. Additionally, patients with RPE tears associated with ICSC usually do not show other evidence of choroidal neovascularisation such as subretinal exudation or haemorrhage. Presence of multiple RPE detachments and CSR type of leaks on FFA also aid in the diagnosis of ICSC.
| References|| |
Hoskin A, Bird AC, Sehmi K. Tears of detached retinal pigment epithelium. Br J Ophthalmol
Menerath JM, Bacin F, Al-Odeh A, Dalers H. Giant peripheral tear of the retinal pigment epithelium. J Fr Ophthalmol
Gass JDM, Little H. Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy. Ophthalmology
Spitznas M: Pathogenesis of central serous retinopathy; A new working hypothesis. Graefes Arch Clin Exp Ophthalmol
Silvestri G, Johnston PB. Clinical evidence of the fluid dynamics in a giant tear of the retinal pigment epithelium. Retina
Machemer R, Heriot W. Retinal pigment epithelial tears through the fovea with preservation of good visual acuity. Arch Ophthalmol
Chuang EL, Bird AC. Repair of tears of the retinal pigment epithelium. Eye
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]