A review of techniques and outcomes of endothelial keratoplasty in congenital hereditary endothelial dystrophy

Abstract

Congenital hereditary endothelial dystrophy affects the Descemet membrane and endothelium, resulting in corneal decompensation. Penetrating keratoplasty (PKP) has been the gold-standard surgical management until recently; however, at present, endothelial keratoplasty (DSEK/DSAEK/n-DSEK: Descemet-stripping or non-Descemet stripping endothelial keratoplasty and DMEK/n-DMEK: Descemet membrane endothelial keratoplasty) is being preferred due to lesser intraoperative and postoperative complications, early visual recovery, and comparable visual outcomes. Endothelial keratoplasty (EK) can be challenging, especially in pediatric eyes with CHED due to smaller eyeballs, shallow anterior chambers, phakic status, and poor intraoperative visibility due to thick and hazy corneas. A total of 198 articles matched our search strategy. After screening for duplication and going through the titles and abstracts, 12 relevant original articles, one case series, and six case reports were included in this review. Various surgical modifications have to be adopted in comparison to adult eyes to overcome the aforementioned difficulties. Regardless, studies have shown favorable visual outcomes with better graft survival and fewer complications in eyes that underwent EK compared to PKP. Hence, timely surgical intervention and strict amblyopia management can result in better final visual outcomes. The purpose of this review is to summarize various intraoperative difficulties and the surgical modifications required, different surgical techniques, visual and graft-related outcomes, and various complications of EK in CHED eyes.

Keywords: Congenital hereditary endothelial dystrophy; Descemet membrane endothelial keratoplasty; Descemet stripping automated endothelial keratoplasty; endothelial keratoplasty.

Figure 1

Descemet stripping endothelial keratoplasty in CHED eye: (a) Injection of Trypan blue dye 0.06% into the anterior chamber for better visualization of Descemet membrane during scoring by using a reverse Terry–Sinskey hook; (b) A metallic instrument such as the crescent blade is inserted beneath the detached DM after descemetorhexis to highlight the stained DM for better visualization; (c) Wounds for insertion of donor tissue are shifted superiorly from their regular 9- and 3-o’clock positions such that during graft delivery, forceps does not pass across the pupil and the iris protects the underlying crystalline lens from any possible contact with the instrument; (d) Air injection to maintain 75% air volume in the anterior chamber and visualization of double ring sign, suggesting the correct graft orientation