THE FINAL PICTURE

Few diseases lead to such visual loss, discomfort and disfigurement. Lagrange referred to the final stage of a hydrophthalmic eye as completely “ désorganisé, disgracieux et douloureux ”.

A visit to a Blind Asylum and a search of its records afford ample evidence of this statement.

With head hung, watery eyes and every effort made to avoid light the victim seeks refuge in the dark. Relief may follow only bilateral enucleation.

One finds enormous eyes, infected and bleary, with lids that give very incomplete protection. The bluish sclera may be staphylomatous and the cornea so opaque that the iris is obscured. From the presence of retinal detachment and vitreous shrinkage there may be a greyish yellow reflex. The tension may be low from ciliary atrophy and phthisis bulbi or raised from an intra-ocular haemorrhage due to rupture of a branch of a long posterior ciliary artery.

A permanent cure. When one remembers the deep-seated cause of hypertension in the majority of cases of congenital glaucoma it is not surprising that complete and permanent cures are very rarely known. Even if the operation is performed in the earliest stages there is little chance of such a cure. However, since Seefelder's day the methods of treatment have improved so much that his views may be considered as unduly pessimistic. He wrote: “ I intentionally do not use the term ‘permanent cure ’, since I know of no case of operated hydrophthalmia where undiminished sight has been retained till later life. In my opinion there can be no definite point of time at which we can decide that a hydrophthalmia is permanently cured. Such observations as that of our case 20 must be highly disconcerting. Here, ten years after dismissal from the clinic, there was vision of 6/30 corrected with normal tension—definitely a gratifying result— while four years later the vision had inexplicably sunk to 6/100 and could not be improved by any correction.”

The oldest person with congenital glaucoma in Jaensch's own experience was a man aged forty-four years. He had been able to see until he was twelve when a detachment appeared in one eye. The other failed when he was thirty-six and both eyes developed phthisis bulbi. In his summary Jaensch wrote: “Thus hydrophthalmia is prognostically very unfavourable and therapeutically very difficult to influence”.