How do doctors manage Posterior Polymorphous Corneal Dystrophy?

Doctor's Answers 3

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.

Most cases of Posterior Polymorphous Corneal Dystrophy (PPCD) do not result in significant symptoms. However, some cases may develop into other eye conditions like corneal swelling (edema) and glaucoma.

It would be prudent to have your eyes examined on a regular basis to detect these potential complications early as they could benefit from early detection and treatment.

Photo of Dr E-Shawn Goh
Dr E-Shawn Goh

Ophthalmologist

Posterior Polymorphous Corneal Dystrophy (PPD) is one of a number of bilateral inherited progressive disorders of the corneal endothelium (i.e. corneal dystrophy). The range of severity varies widely from young babies needing corneal transplantation at an early age due to corneal opacification, to late deterioration in adulthood and older age that may require corneal transplantation or treatment for glaucoma.

I'm sorry to hear about your recent diagnosis, but am heartened to hear that you say it is a mild case.

I would advise regular follow-up with your ophthalmologist, who will be best able to identify the severity of your condition, and hence be able to indicate a suitable frequency of follow-up for you. During your regular follow-ups, your ophthalmologist will be monitoring you for associated conditions including Alport syndrome or keratoconus, as well as for disease progression including raised intraocular pressure, and progressive corneal decompensation.

At different stages - contact lenses may be used either for optical reasons instead of using spectacles, or be prescribed as a therapeutic lens (if the corneal epithelium breaks down due to progression of the PPD).

Similarly, refractive surgery may be performed in patients with extremely mild disease, bearing in mind that lifelong follow-up would still be necessitated for PPD, and prior refractive surgery may complicate the monitoring and follow-up for complications of PPD.

The use of contact lenses, and refractive surgery are topics that are best discussed in close consultation with your Ophthalmologist.

Best of luck!

ESG

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