Outcomes of Descemet-Stripping Automated Endothelial Keratoplasty in Congenital Hereditary Endothelial Dystrophy

Abstract

Objectives: To evaluate the outcomes and complications of Descemet-stripping automated endothelial keratoplasty (DSAEK) in children with congenital hereditary endothelial dystrophy (CHED). Methods: Eighteen eyes from 10 children with CHED who underwent standard phakic DSAEK were enrolled in this retrospective interventional case series. Medical records including slit-lamp biomicroscopy, cycloplegic refraction, visual acuity, and measurements of central corneal thickness, corneal topography, and endothelial cell density were evaluated. All complications were recorded. Results: The mean age of cases was 8.1±4.2 (range: 3–16) years at the time of surgery. Descemet-stripping automated endothelial keratoplasty was performed successfully in all eyes, which survived in 16/18 (88.9%) of cases during a median follow-up period of 38 (range 19–64) months. In patients with clear graft, best-corrected visual acuity was improved to a range of 20/100 to 20/40 and improved to fix and follow in one younger child. Endothelial cell density decreased by an average of 42.1%, comparing baseline and 6-month follow-up and was relatively stable thereafter. A marked flattening of the anterior corneal curvature (mean change of anterior K reading: +2.56±3.14 diopters) was observed after DSAEK. The only major complication in patients with clear graft was intraocular pressure elevation in 61.1% of the eyes, which was easily controlled with one antiglaucoma medication. None of the eyes developed cataract. Conclusion: Descemet-stripping automated endothelial keratoplasty is a safe and successful procedure in children with an acceptable graft survival in a mid-term follow-up period. A marked hyperopic shift could occur after DSAEK in children with CHED.

Microscope Integrated Optical Coherence Tomography Guided Descemet Stripping Automated Endothelial Keratoplasty in Congenital Hereditary Endothelial Dystrophy

“…The endothelial loss at the final follow-up compared to the baseline was 36.2% (range, 14.7 to 49.3%), which was comparable to other studies in the literature. 2 , 18 , 36 , 37 Thirty-five eyes (35/39; 89.7%) developed clear cornea at the four-week follow-up and all the eyes had clear cornea at six week follow-up period in our study which was X relatively long compared to other studies. 2 This could again be attributed to the relatively thicker baseline corneas in our eyes compared to the other studies.…”

“…of Eyes Pre-Operative Visual Acuity CCT No. of Regrafts Graft Survival Rate Graft Clarity Post-Operative Visual Acuity Post-Operative Refraction ECD Loss Post-Operative Complications Our study 9.87±8.2y (8m-42y) 17.3m (8–58m) 48 eyes of 29 patients (39-DSAEK; 9-PKP) 1.71±0.66 (0.6–2.7) 1100±174 µ 3/39 in DSAEK; 3/9 in PKP 36/39 (92.3%) among primary DSAEK 36 had clear grafts 3 failed grafts 0.76±0.19 36.2% (14.7 to 49.3%) 1 eye- pupillary block 3 eyes- failed graft in primary DSAEK Mohebbi et al 18 8.16±4.2y (3–16y) 38m (19–64) m 18 (all DSAEK) 20/400 to 20/63 in 12 eyes FF in 3 eyes No FF in 3 eyes 1- re-DSAEK 16/18 eyes (88.9%) 15 clear centred graft at last f/u 1 faintly hazy graft 2 failed graft 20/100 to 20/40 FF in young +2.5763.3 DS (at last f/u) 42.1% (at 6mo) Busin glide (35.7± 12.9%) Taco 60/40 technique (46%±14.8%; 1 case of pupillary block on POD1 Raised IOP (61.1%) 1 graft failure at 9 months due to traumatic wound dehiscence 1 graft failure at 30 months Yang et al 34 4.3±4.0y (6m-13y) 6.5±3.6y (child) 0.78±0.18y (infant) 4±1.9y (2.5–8.5 y) 30 (19-child; 11-infant) all DSAEK LogMAR 1.03±0.25 (FF to LogMAR 1.03) (child) no FF (infant) 1 (to PK) All cleared at POW1 till 1y LogMAR 0.54±0.20 (child) LogMAR 0.32±0.11 (infant) NA 31.21±9.17% (child) Not co-operative...…”

“…In a series of 18 eyes of 10 CHED patients with a median follow-up of 38 months demonstrated favourable outcomes of DSAEK in CHED patients. 18 DMEK could be challenging especially in pediatric eyes, however, results are encouraging with favourable outcomes. 19 Saad et al 20 performed DMEK in 14 eyes of 8 CHED patients and reported good visual outcomes.…”

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

“…[ 18 ] Since the first published series of DSAEK in CHED by Busin et al , several publications have shown that DSAEK is a safe and effective surgery in providing rapid restoration of corneal clarity with fewer complications compared to PKP. [ 19 20 21 22 23 24 25 26 27 28 29 30 ]…”

“…[ 18 19 20 21 22 23 24 25 26 27 28 29 30 ] Donor tissue preparation: The donor lenticules can be either prepared preoperatively by trained technicians and appropriately stored (pre-cut donor lenticules) or they can be cut at the time of the surgery either by manual dissection or by using an automated lamellar therapeutic keratoplasty (ALTK) system. [ 22 26 28 29 ] Manual dissection can be done using Melles blunt dissectors and a Moria or Barron artificial anterior chamber. [ 17 20 21 23 25 30 ] A graduated diamond knife can help decide the depth of dissection depending on the donor thickness.…”