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Penetrating Keratoplasty in Congenital Hereditary Endothelial Dystrophy

Özdemir, Burak MD; Kubaloğlu, Anil MD; Koytak, Arif MD; Coskun, Erol MD; Çinar, Yasin MD; Sari, Esin Sogutlu MD; Özertürk, Yusuf MD

doi: 10.1097/ICO.0b013e31823d03af
Clinical Science

Purpose To compare the results of penetrating keratoplasty (PK) in a series of congenital hereditary endothelial dystrophy (CHED) patients operated at amblyogenic years and at later ages.

Methods Records of 65 eyes of 35 CHED patients who underwent PK were reviewed retrospectively. Visual results and graft clarity rates were compared between patients operated after (group 1) and before (group 2) 12 years of age. Results were also compared between eyes with and without nystagmus.

Results Group 1 consisted of 47 eyes (24 patients), whereas group 2 consisted of 18 eyes (11 patients). Thirteen patients (54%) in group 1 and 10 patients (91%) in group 2 had nystagmus. Mean ages at surgery were 26.4 and 7.6 years in group 1 and group 2, respectively. Mean follow-up period was 101.0 months in group 1 and 59.7 months in group 2. Group 1 cases had better graft clarity rates than group 2 cases (P = 0.023). Postoperative best-corrected visual acuities (BCVAs) were 20/80 or better in 39 eyes of group 1 (83%) and in 5 eyes of group 2 (28%). Visual outcomes were found significantly better in group 1 cases (P < 0.001). In group 1, 76% of eyes with nystagmus and 91% of eyes without nystagmus had BCVAs of 20/80 or better. In group 1, both preoperative and postoperative BCVAs were found significantly better in eyes without nystagmus (P < 0.001 and P = 0.002, respectively).

Conclusions Delayed keratoplasty seems to offer better graft outcomes and visual prognosis to CHED patients, even in the presence of nystagmus.

*Kartal Training and Education Hospital, Second Eye Clinic, Istanbul, Turkey

Bezmialem Vakif University, Faculty of Medicine, Istanbul, Turkey

Department of Ophthalmology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey

§Department of Ophthalmology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey

Nusaybin State Hospital, Mardin, Turkey

Reprints: Burak Özdemir, Kartal Training and Education Hospital, II Eye Clinic, Cevizli/Kartal, Istanbul 34650, Turkey (e-mail:

The authors state that they have no financial or conflicts of interest to disclose.

Received February 21, 2010

Accepted August 24, 2011

Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.