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Year : 1987  |  Volume : 35  |  Issue : 2  |  Page : 60

Macular diseases and RPE-The dark twin to the fore?

Dr R.P Centre, A.I.I.M.S, New Delhi-29, India

Correspondence Address:
Rajvardhan Azad
Dr R.P Centre, A.I.I.M.S, New Delhi-29
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Source of Support: None, Conflict of Interest: None

PMID: 3450619

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How to cite this article:
Azad R. Macular diseases and RPE-The dark twin to the fore?. Indian J Ophthalmol 1987;35:60

How to cite this URL:
Azad R. Macular diseases and RPE-The dark twin to the fore?. Indian J Ophthalmol [serial online] 1987 [cited 2023 Dec 8];35:60. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1987/35/2/60/26213

The macula because of its specialised anatomical configuration and physiological functions of utmost importance enjoys a vital and privileged status in the human retina Therefore involvement of macula in a disease process means a distru­bance so annoying or even devastating, that it mandates early ophthalmic consultation. The majority of macular disorders are of unknown aetiology and it has been cutomary to blame either the choriocapillaries or Bruch's membrane or RPE In recent years there has been a shift in the concept and more and more investigations point to the prime role of RPE in most of the macular disorders Its derangements may be classified as primary or secondary. For example drusen, fundus favimaculatus and vitelliform degenerations are the diseases in which RPE is primarily involved Whereas the RPE is not the site of initial or primary affection in presumed histoplasmosis syndrome, where, the initial choroiditis causes secondary damage to the RPE, by gradual breakdown and vascularistion of the barrier between choroid and retina, resulting in the severe disciform lesions

Senile macular degeneration is yet another instance where the primary seat of involvement is believed to be in the Bruch's membrane or the choriocapillaries or both The RPE transport and barrier functions are affected later. Early involve­ment of the RPE in heredodegenerative disorders and various pigmentary disorders is a reasonable speculation but the biochemical nature of the lesions remain unknown

Choroideraemia, Angioid streaks and Geographic Helicoid peripapillary choroidopathy are geneti­cally determined diseases where the RPE is involved either simultaneously with or indepen­dently from the adjacent layers of the choroid & the retina Myopic maculopathy where breaks in the Bruch's membrane in the macula result in SRNV and haemorrhage, atrophy of the RPE remains an important finding.

Of the inflammatory conditions AMPPE and central serous retinopathy deserve special men­tion. AMPPE was described by Gass & coworkers to define a self limiting entity, where yellowish speck like lesions are seen involving the posterior pole. Unfortunately these lesions have been con­fused with disseminated choroidits and CSR The typical clinical and fluorescein picture and the good visual prognosis should clinch the diagnosis in most of these cases.

The prognosis in most of the degenerative disor­ders remain guarded because of unknown aetio­logy and uncertain treatment available Recent studies have established that SMD with SRNV can be halted with laser photocoagulation and this has been a vital contribution in the management of SMD. CSR generally has a good prognosis and when indicated or desired an attack of CSR can be shortened with laser therapy. But in many other dystrophic macular lesions no treatment is avail­able. It is hoped that with the identification of possible biochemical errors in certain maculo­pathies a more rational approach will be possible (remember essential gyrate atrophy). In the meanwhile, the importance of genetic counselling in hereditarily transmitted macular disorders needs re-emphasis


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