Outcome after penetrating keratoplasty in congenital hereditary corneal endothelial dystrophy (CHED). Report on 13 eyes

Background Congenital hereditary endothelial dystrophy (CHED) is a rare bilateral corneal disease. The stromal opacity is supposed to result from terminal misdifferentiation of the endothelial cells. In this study we present the morphological and functional results after penetrating keratoplasty in children with CHED who were operated in our department between 1981 and 1997. Patients and Methods In a retrospective clinical cross-sectional study we looked up case histories of 13 eyes from 8 children (7 female, 1 male) with a mean age of 6.0+/-3.1 years (ranged from 3 to 14 years). In all children penetrating keratoplasty was performed by one surgeon (GOHN), in 3 eyes using nonmechanical excimer laser trephination. The graft-diameter was in 7 eyes 7.0/7.1 mm, in 2 eyes 7.0/7.2 mm, in 2 eyes 6.5/6.6 mm (resp. 6.8 mm), in 2 eyes 6.0/6.1 mm (resp. 6.2 mm). Fixation of grafts was achieved in 2 eyes by single running suture, in 8 eyes by double running suture and in 3 eyes by multiple interrupted sutures. Results During a mean follow-up of 4.0+/-2.4 years visual acuity increased in all patients (from light perception to 6/20 preoperativaly to 2/200 to 14/20 postoperatively). In one patient corneal endothelial-epithelial-decompensation occurred (both eyes unterwent previous antiglaucomatous surgery elsewhere), and in 1 patient loosening of one suture happened after 10 month. No immunological graft reaction occurred during follow-up. After excimer laser trephination (3 eyes from 2 patients) visual acuity and corneal astigmatism after surgery was favorable in comparison to all other patients. Conclusion In children with CHED penetrating keratoplasty results not only in a clear cornea but also in a satisfactory functional outcome. Postoperatively periodical morphological controls and assessment of refraction as well as means to prevent amblyopia are indispensable before age 7.