A 20-year-old patient, diagnosed with posterior polymorphous corneal dystrophy, developed corneal edema for which he underwent Descemet membrane endothelial keratoplasty with a stromal rim (DMEK-S) in the right eye. No intra- or postoperative complications were noted. At the last follow-up 2 years and 9 months after the procedure, the best corrected visual acuity was 1.0 and endothelial cell density declined from 3533 cells/mm 2 to 1012 cells/mm 2 . Despite the endothelial cell loss, DMEK-S appears to be a good alternative to other surgical techniques for the treatment of corneal endotheliopathies, and it may be of benefit to young patients.

Laboratory of the Biology and Pathology of the Eye Institute of Inherited Metabolic Disorders 1st Medical Faculty Charles University Prague and General University Hospital Prague Czech Republic

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Krachmer JH. Posterior polymorphous corneal dystrophy: A disease characterized by epithelial-like endothelial cells which influence management and prognosis. Trans Am Ophthalmol Soc. 1985;83:413–75. PubMed PMC

Jirsova K, Merjava S, Martincova R, Gwilliam R, Ebenezer ND, Liskova P, et al. Immunohistochemical characterization of cytokeratins in the abnormal corneal endothelium of posterior polymorphous corneal dystrophy patients. Exp Eye Res. 2007;84:680–6. PubMed

Bahar I, Kaiserman I, McAllum P, Slomovic A, Rootman D. Comparison of posterior lamellar keratoplasty techniques to penetrating keratoplasty. Ophthalmology. 2008;115:1525–33. PubMed

Ham L, Dapena I, van Luijk C, van der Wees J, Melles GR. Descemet membrane endothelial keratoplasty (DMEK) for Fuchs endothelial dystrophy: Review of the first 50 consecutive cases. Eye (Lond) 2009;23:1990–8. PubMed

Studeny P, Farkas A, Vokrojova M, Liskova P, Jirsova K. Descemet membrane endothelial keratoplasty with a stromal rim (DMEK-S) Br J Ophthalmol. 2010;94:909–14. PubMed

Yellore VS, Papp JC, Sobel E, Khan MA, Rayner SA, Farber DB, et al. Replication and refinement of linkage of posterior polymorphous corneal dystrophy to the posterior polymorphous corneal dystrophy 1 locus on chromosome 20. Genet Med. 2007;9:228–34. PubMed

Autosomal-Dominant Corneal Endothelial Dystrophies CHED1 and PPCD1 Are Allelic Disorders Caused by Non-coding Mutations in the Promoter of OVOL2

Recurrence of posterior polymorphous corneal dystrophy is caused by the overgrowth of the original diseased host endothelium