How do doctors manage Posterior Polymorphous Corneal Dystrophy?

Doctor's Answer

Hi Mei

Corneal dystrophies are by definition genetic conditions of the cornea (the clear window at the front of the eye), with no associated inflammation. One of these kinds is called 'Posterior Polymorphous Corneal Dystrophy' (PPMD).

This condition affects the back layer of the cornea-the endothelium and Descemet membrane. This layer is important in maintaining transparency of the cornea by pumping fluid out of the cornea. The cornea can become swollen and cloudy if this layer 'decompensates' or stops performing its function adequately.

The condition can behave very differently in different patients.

In most patients, the changes are non-progressive or only very slowly progressive. Very uncommonly the cornea may become hazy due to the decompensation as mentioned above.

As is the case with most genetic conditions, there is no medical treatment for this condition. Most patients also do not feel any problem ie they are 'asymptomatic'. Therefore the management is usually just observation, perhaps on an annual basis/yearly checkup.

In this day and age, PPMD should not lead to blindness. If the cornea does become hazy and vision becomes blurred over time, there are surgeries such as DSAEK or DMEK which are very specialized techniques to transplant healthy functioning endothelial cells and replace the abnormal PPMD cells. Thereafter the cornea clears up and vision is restored.

If glaucoma does appear, that is also treated as usual with eyedrops initially, and less commonly surgery can be considered if the eyedrops are not sufficient for controlling eye pressure. But to recap, most PPMD patients go through life without needing these complicated management methods and surgeries.

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