A Case of Posterior Polymorphous Dystrophy in a Patient with Type I Diabetes

Title A Case of Posterior Polymorphous Dystrophy in a Patient with Type I Diabetes
Author, Co-Author Michael Brayden Lundquist, Robert Cook, Vladimir Yevseyenkov, Wendy Harrison
Topic
Year
2014
Day
Friday
Program Number
145209
Room
Four Seasons Ballroom
Affiliation
Midwestern University Arizona College of Optometry
Abstract Background: Posterior Polymorphous Dystrophy (PPMD) is a well-documented but rare endothelial disorder which is often asymptomatic. It presents with a variety of endothelial changes. Despite our knowledge of the disease, the epidemiology surrounding it is not well understood due to its rare nature. It is autosomal dominant, but it is unknown if other diseases which can affect corneal health have any bearing on its course, which is incredibly variable from patient to patient. It has been associated in research literature with other corneal conditions and autoimmune disorders. New evidence suggests that the genetic changes responsible for PPMD may have systemic effects. Here we report a case of a patient with both type I diabetes (DM) and PPMD.

Case: A 24 year old patient presented for participation in a research study evaluating ocular neural changes in DM.  She was 20/20 OD, OS with no refractive error or visual complaints. Her tests were normal with no diabetic retinopathy, no ERG changes, and a normal macular OCT. A confocal microscopy scan of the cornea concluded the study and noted widespread changes to the endothelium in both eyes, prompting follow up, and the diagnosis of PPMD. Pachymetry readings were 620 microns  OD, 615 microns OS and did not change throughout the day. A slit lamp exam revealed a classic snail track lesion inferior OD.

Conclusion: We believe this is the first case to describe a case of PPMD in a patient with type I DM. We remain concerned about the combined effect they will have on the course of corneal health, particularly in the stroma which could be altered by both disease processes. At this time the patient’s stroma has remained stable and healthy over 6 months of follow up visits with a corneal nerve presentation in the expected range,  but will be followed closely over time for both stromal and endothelial health.
Affiliation of Co-Authors Midwestern University Arizona College of Optometry, Midwestern University Arizona College of Optometry, Midwestern University Arizona College of Optometry
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