Posterior Polymorphous Dystrophy: An Unusual Presentation
Case Report of PPD
Vol. 14 No. 1 : 2019 (191-196)
Abstract
Posterior Polymorphous Dystrophy (PPD) is a rare, innocuous and asymptomatic condition in which corneal endothelial cells display characteristics similar to epithelium. It is often bilateral and frequently asymmetric. We report a case of a 10-year-old girl with a family history of glaucoma who presented with right eye blurring of vision since few years. She had frequent spectacle-prescription changing due to unimproved visual acuity. Cycloplegic refraction revealed high astigmatism and moderate amblyopia over the right eye. Her best corrected vision was 6/9 for the right and left eyes. Slit-lamp examination showed a vesicular-like lesion at the periphery of corneal endothelial layers in both eyes. Endothelial cell density was much reduced on the right eye compared to the left, with more severe astigmatism. Other ocular examinations were unremarkable. Patient was instructed to patch her better left eye periodically. Although patients with Posterior Polymorphous Dystrophy (PPD) present at the age of adulthood, the age at diagnosis is highly variable. It is rare and inherited through an autosomal dominant pattern. PPD may rarely lead to astigmatism, by which was present in the patient due to the features of non-keratoconic and keratoconic cornea on the right and left eye, respectively. Endothelial cell counts were reduced more on the right eye compared to the left, which later may worsen her visual acuity thus indicating corneal transplant in the future. Patient also had a strong family history of glaucoma. Hence, screening of associated disease of corneal endothelial dystrophy in the family may be necessary.
Keywords :
amblyopia,
astigmatism,
cornea,
Correspondance Address
Prof. Madya Dr. Jemaima Che Hamzah. Department of Opththalmology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia. Tel: +603-91455981/5982 Email: jemaima@ppukm.ukm.edu.my
References
Ahn, Y.J., Choi, S.I., Yum, H.R., Shin, S.Y., Park, S.H. 2017. Clinical features in children with posterior polymorphous corneal dystrophy. Optom Vis Sci 94(4): 476-81.
Aldave, A.J., Ann, L.B., Frausto, R.F., Nguyen, C.K., Yu, F., Raber, I.M. 2013. Classification of posterior polymorphous corneal dystrophy as a corneal ectatic disorder following confirmation of associated significant corneal steepening. JAMA Ophthalmol 131(12): 1583-90.
Blair, S.D., Seabrooks, D., Shields, W.J., Pillai, S., Cavanagh, H.D. 1992. Bilateral progressive essential iris atrophy and keratoconus with coincident features of posterior polymorphous dystrophy: a case report and proposed pathogenesis. Cornea 11(3): 255-61.
Cibis, G.W., Krachmer, J.A., Phelps, C.D., Weingeist, T.A. 1977. The clinical spectrum of posterior polymorphous dystrophy. Arch Ophthalmol 95(9): 1529-37.
Cremona, F.A., Ghosheh, F.R., Rapuano, C.J., Eagle, R.C., Hammersmith, K.M., Laibson, P.R., Ayres, B.D., Cohen, E.J. 2009. Keratoconus associated with other corneal dystrophies. Cornea 28(2): 127-35.
DeRespinis, P.A., Norden, R.A., Rispoli, L.C. 1996. Posterior polymorphous dystrophy associated with astigmatism and amblyopia in children. J Refract Surg 12(6): 709-14.
Heon, E., Greenberg, A., Kopp, K.K., Rootman, D., Vincent, A.L., Billingsley, G., Priston, M., Dorval, K.M., Chow, R.L., McInnes, R.R., Heathcote, G., Westall, C., Sutpin, J.E., Semina, E., Bremner, R., Stone, E.M. 2002. VSX1: a gene for posterior polymorphous dystrophy and keratoconus. Hum Mol Genet 11(9): 1029-36.
Jeon, H.S., Hyon, J.Y. 2017. Unilateral posterior polymorphous corneal dystrophy presented as anisometropic astigmatism: 3 case reports. Case Rep Ophthalmol 8(1): 250-8.
Kiel, J. 1999. Posterior polymorphous dystrophy. Clin Exp Optom 82(1): 20-2.
Raber, I.M., Fintelmann, R., Chhabra, S., Ribeiro, M.P.F., Eagle Jr, R.C., Orlin, S.E. 2011. Posterior polymorphous dystrophy associated with nonkeratoconic steep corneal curvatures. Cornea 30(10): 1120-4.
Rodrigues, M.M., Phelps, C.D., Krachmer, J.H., Cibis, G.W., Weingeist, T.A. 1980. Glaucoma due to endothelialization of the anterior chamber angle: a comparison of posterior polymorphous dystrophy of the cornea and Chandler's syndrome. Arch Ophthalmol 98(4): 688-96.
Teekhasaenee, C., Nimmanit, S., Wijtthiphan, S., Vareesangthip, K., Laohapand, T., Malasitr, P., Ritch, R. 1991. Posterior polymorphous dystrophy and Alport syndrome. Ophthalmology 98(8): 1207-15.
Wallace, D.K., Pediatric Eye Disease Investigator Group. 2006. A randomized trial to evaluate 2 hours of daily patching for strabismic and anisometropic amblyopia in children. Ophthalmology 113(6): 904-12.
