How do doctors manage Posterior Polymorphous Corneal Dystrophy?

Doctor's Answer

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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