OUTCOME AFTER PENETRATING KERATOPLASTY IN CONGENITAL HEREDITARY CORNEAL ENDOTHELIAL DYSTROPHY (CHED) - REPORT ON 13 EYES

Background Congenital hereditary endothelial dystrophy (CHED) is a rar e bilateral corneal disease. The stromal opacity is supposed to result from terminal misdifferentiation of the endothelial cells. In this st udy we present the morphological and functional results after penetrat ing keratoplasty in children with CHED who were operated in our depart ment between 1981 and 1997. Patients and Methods In a retrospective cl inical cross-sectional study we looked up case histories of 13 eyes fr om 8 children (7 female, 1 male) with a mean age of 6.0+/-3.1 years (r anged from 3 to 14 years). In all children penetrating keratoplasty wa s performed by one surgeon (GOHN), in 3 eyes using nonmechanical excim er 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 s ingle 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 per ception to 6/20 preoperativaly to 2/200 to 14/20 postoperatively). In one patient corneal endothelial-epithelial-decompensation occurred (bo th eyes unterwent previous antiglaucomatous surgery elsewhere), and in 1 patient loosening of one suture happened after 10 month. No immunol ogical graft reaction occurred during follow-up. After excimer laser t rephination (3 eyes from 2 patients) visual acuity and corneal astigma tism 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 re fraction as well as means to prevent amblyopia are indispensable befor e age 7.