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Erschienen in: International Ophthalmology 4/2017

22.09.2016 | Original Paper

Clinical characterization of posterior polymorphous corneal dystrophy in patients of Indian ethnicity

verfasst von: Sunita Chaurasia, Rashmi Mittal, G. Bichappa, Muralidhar Ramappa, Somasheila I. Murthy

Erschienen in: International Ophthalmology | Ausgabe 4/2017

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Abstract

Purpose

To characterize the clinical presentation of posterior polymorphous corneal dystrophy (PPCD) in eyes of Indian ethnicity.

Design

Retrospective cohort study from January 1995 to December 2015.

Participants

Patients with the diagnosis of posterior polymorphous corneal dystrophy.

Methods

Medical records of the patients were reviewed for clinical presentation. Histology of corneal specimens of those that underwent keratoplasty was assessed.

Main outcome measures

Descriptive analysis of clinical condition.

Results

Mean age at first evaluation was 32.5 years (range 1–73 years), male:female = 35:18. Majority (44/53; 83 %) of the patients had bilateral involvement. 5/9 (44 %) patients with unilateral presentation were amblyopic in the affected eye. The clinical features documented were vesicles in 94 eyes, band-like pattern in 32 eyes, edema of varying degree in 23 eyes (12 patients, 1 patient was one eyed), and anterior segment changes in 1 eye. 8/45 (17 %) eyes had a regular astigmatism with steep axis >47 D (range 47.2–56.2 D). 16 eyes of 12 patients who had clinically evident corneal edema underwent keratoplasty. Mean age at keratoplasty was 58 years (range 1–73 years). 8 patients had penetrating keratoplasty (PK) and 8 had Descemet stripping endothelial keratoplasty (DSEK). Mean follow-up after keratoplasty was 4.2 years (1 month to 13 years). Except one, all grafts remained clear till the last follow-up. In all specimens, the Descemet membrane was grossly thickened.

Conclusions

In our study, 12/53 (22.6 %) patients required keratoplasty for visually significant corneal edema. Except one, all were older adults. The patients who needed keratoplasty were bilaterally afflicted and had visually significant cornea edema in both eyes. With a mean follow-up duration of 4.2 years after keratoplasty, no recurrences were noted.
Literatur
1.
Zurück zum Zitat Cibis GW, Krachmer JA, Phelps CD, Weingeist TA (1977) The clinical spectrum of posterior polymorphous dystrophy. Arch Ophthalmol 95(9):1529–1537CrossRefPubMed Cibis GW, Krachmer JA, Phelps CD, Weingeist TA (1977) The clinical spectrum of posterior polymorphous dystrophy. Arch Ophthalmol 95(9):1529–1537CrossRefPubMed
2.
Zurück zum Zitat Heon E et al (1995) Linkage of posterior polymorphous corneal dystrophy to 20q11. Hum Mol Genet 4(3):485–488CrossRefPubMed Heon E et al (1995) Linkage of posterior polymorphous corneal dystrophy to 20q11. Hum Mol Genet 4(3):485–488CrossRefPubMed
3.
Zurück zum Zitat Biswas S et al (2001) Missense mutations in COL8A2, the gene encoding the alpha2 chain of type VIII collagen, cause two forms of corneal endothelial dystrophy. Hum Mol Genet 10(21):2415–2423CrossRefPubMed Biswas S et al (2001) Missense mutations in COL8A2, the gene encoding the alpha2 chain of type VIII collagen, cause two forms of corneal endothelial dystrophy. Hum Mol Genet 10(21):2415–2423CrossRefPubMed
4.
Zurück zum Zitat Shimizu S et al (2004) A locus for posterior polymorphous corneal dystrophy (PPCD3) maps to chromosome10. Am J Med Genet A 130A(4):372–377CrossRefPubMed Shimizu S et al (2004) A locus for posterior polymorphous corneal dystrophy (PPCD3) maps to chromosome10. Am J Med Genet A 130A(4):372–377CrossRefPubMed
5.
Zurück zum Zitat Liskova P, Gwilliam R, Filipec M, Jirsova K, Reinstein Merjava S, Deloukas P et al (2012) High prevalence of posterior polymorphous corneal dystrophy in the Czech Republic; linkage disequilibrium mapping and dating an ancestral mutation. PLoS One 7(9):e45495CrossRefPubMedPubMedCentral Liskova P, Gwilliam R, Filipec M, Jirsova K, Reinstein Merjava S, Deloukas P et al (2012) High prevalence of posterior polymorphous corneal dystrophy in the Czech Republic; linkage disequilibrium mapping and dating an ancestral mutation. PLoS One 7(9):e45495CrossRefPubMedPubMedCentral
6.
Zurück zum Zitat Aldave AJ, Ann LB, Frausto RF et al (2013) Classification of posterior polymorphous corneal dystrophy as a corneal ectatic disorder following confirmation of associated significant corneal steepening. JAMA Ophthalmol 131(12):1583–1590CrossRefPubMedPubMedCentral Aldave AJ, Ann LB, Frausto RF et al (2013) Classification of posterior polymorphous corneal dystrophy as a corneal ectatic disorder following confirmation of associated significant corneal steepening. JAMA Ophthalmol 131(12):1583–1590CrossRefPubMedPubMedCentral
7.
Zurück zum Zitat Shen J, Chixin D, Gu Y (2015) Long-term observation of coexistence of posterior polymorphous corneal dystrophy, resultant high myopia and nonkeratoconic developing corneal astigmatism: a case report of 7-year tracking in a Chinese boy. Medicine (Baltimore) 94(23):e921CrossRef Shen J, Chixin D, Gu Y (2015) Long-term observation of coexistence of posterior polymorphous corneal dystrophy, resultant high myopia and nonkeratoconic developing corneal astigmatism: a case report of 7-year tracking in a Chinese boy. Medicine (Baltimore) 94(23):e921CrossRef
8.
Zurück zum Zitat Teekhasaenee C, Nimmanit S, Wutthiphan S, Vareesangthip K, Laohapand T, Malasitr P, Ritch R (1991) Posterior polymorphous dystrophy and Alport syndrome. Ophthalmology 98(8):1207–1215CrossRefPubMed Teekhasaenee C, Nimmanit S, Wutthiphan S, Vareesangthip K, Laohapand T, Malasitr P, Ritch R (1991) Posterior polymorphous dystrophy and Alport syndrome. Ophthalmology 98(8):1207–1215CrossRefPubMed
9.
Zurück zum Zitat Sella R, Rootman D, Bahar I (2013) Descemet’s stripping automated endothelial keratoplasty for posterior polymorphous corneal dystrophy in an 8-month-old boy. J AAPOS 17(1):94–96CrossRefPubMed Sella R, Rootman D, Bahar I (2013) Descemet’s stripping automated endothelial keratoplasty for posterior polymorphous corneal dystrophy in an 8-month-old boy. J AAPOS 17(1):94–96CrossRefPubMed
10.
Zurück zum Zitat Krachmer JH (1985) Posterior polymorphous corneal dystrophy: a disease characterized by epithelial-like endothelial cells which influence management and prognosis. Trans Am Ophthalmol Soc 83:413–475PubMedPubMedCentral Krachmer JH (1985) Posterior polymorphous corneal dystrophy: a disease characterized by epithelial-like endothelial cells which influence management and prognosis. Trans Am Ophthalmol Soc 83:413–475PubMedPubMedCentral
11.
Zurück zum Zitat DeRespinis PA, Norden RA, Rispoli LC (1996) Posterior polymorphous dystrophy associated with astigmatism and amblyopia in children. J Refract Surg 12(6):709–714PubMed DeRespinis PA, Norden RA, Rispoli LC (1996) Posterior polymorphous dystrophy associated with astigmatism and amblyopia in children. J Refract Surg 12(6):709–714PubMed
12.
Zurück zum Zitat Bozkurt B, Ozkan F, Yilmaz M, Okudan S (2015) Posterior corneal steepening in posterior polymorphous corneal dystrophy. Optom Vis Sci 92(11):414–419CrossRef Bozkurt B, Ozkan F, Yilmaz M, Okudan S (2015) Posterior corneal steepening in posterior polymorphous corneal dystrophy. Optom Vis Sci 92(11):414–419CrossRef
13.
Zurück zum Zitat Raber IM, Fintelmann R, Chhabra S, Ribeiro MP, Eagle RC Jr, Orlin SE (2011) Posterior polymorphous dystrophy associated with non-keratoconic steep corneal curvatures. Cornea 30(10):1120–1124CrossRefPubMed Raber IM, Fintelmann R, Chhabra S, Ribeiro MP, Eagle RC Jr, Orlin SE (2011) Posterior polymorphous dystrophy associated with non-keratoconic steep corneal curvatures. Cornea 30(10):1120–1124CrossRefPubMed
14.
Zurück zum Zitat Pang CJ, Jing Y, Li J, Song XH, Wang LY (2011) Clinical observation of posterior polymorphous corneal dystrophy. Zhonghua Yan Ke Za Zhi 47(1):17–21PubMed Pang CJ, Jing Y, Li J, Song XH, Wang LY (2011) Clinical observation of posterior polymorphous corneal dystrophy. Zhonghua Yan Ke Za Zhi 47(1):17–21PubMed
15.
Zurück zum Zitat Jang MS, Roldan AN, Frausto RF, Aldave AJ (2014) Posterior polymorphous corneal dystrophy 3 is associated with agenesis and hypoplasia of the corpus callosum. Vis Res 100:88–92CrossRefPubMedPubMedCentral Jang MS, Roldan AN, Frausto RF, Aldave AJ (2014) Posterior polymorphous corneal dystrophy 3 is associated with agenesis and hypoplasia of the corpus callosum. Vis Res 100:88–92CrossRefPubMedPubMedCentral
16.
Zurück zum Zitat Nguyen DQ, Hosseini M, Billingsley G, Héon E, Churchill AJ (2010) Clinical phenotype of posterior polymorphous corneal dystrophy in a family with a novel ZEB1 mutation. Acta Ophthalmol 88(6):695–699CrossRefPubMed Nguyen DQ, Hosseini M, Billingsley G, Héon E, Churchill AJ (2010) Clinical phenotype of posterior polymorphous corneal dystrophy in a family with a novel ZEB1 mutation. Acta Ophthalmol 88(6):695–699CrossRefPubMed
Metadaten
Titel
Clinical characterization of posterior polymorphous corneal dystrophy in patients of Indian ethnicity
verfasst von
Sunita Chaurasia
Rashmi Mittal
G. Bichappa
Muralidhar Ramappa
Somasheila I. Murthy
Publikationsdatum
22.09.2016
Verlag
Springer Netherlands
Erschienen in
International Ophthalmology / Ausgabe 4/2017
Print ISSN: 0165-5701
Elektronische ISSN: 1573-2630
DOI
https://doi.org/10.1007/s10792-016-0360-y

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