An attempt to optimize the outcome of penetrating keratoplasty in congenital aniridia-associated keratopathy (AAK)

C J Farah,B Käsmann-Kellner,F N Fries,B Seitz,L Latta

doi:10.1007/s10792-021-01982-z

C J Farah, B Käsmann-Kellner + Show 3 more

Open Access

https://doi.org/10.1007/s10792-021-01982-z

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Journal: International Ophthalmology	Publication Date: Jul 29, 2021
Citations: 13	License type: open-access

Affiliation: Saarland University

Abstract

PurposeTo propose an optimized microsurgical and medical approach to reduce the risk of complications after penetrating keratoplasty (PKP) in patients with aniridia-associated keratopathy (AAK).MethodsRetrospective observational case series of 25 PKP performed in 16 patients with AAK. Preoperative indications were endothelial decompensation and vascularized scars (68%) or graft failure (32%) due to limbal stem cell deficiency. The optimized approach included a combination of a small corneal graft size (around 7.0 mm), interrupted 10–0nylon sutures, simultaneous AMT as a patch, large bandage contact lens, temporary lateral tarsorrhaphy, postoperative autologous serum eye drops, and systemic immunosuppression. Main outcome measures included: visual acuity, transplant survival, and complications encountered during follow-up of 107 weeks on average.ResultsA complete modified keratoplasty scheme was used in 10 of 25 PKP (group 1), while at least one of the modifications was missing in the other 15 PKP (group 2). After 8 weeks of follow-up, the epithelium was closed in 23 eyes. Visual acuity improved in 19 eyes at 6 months of follow-up, and remained stable in six eyes. None of the eyes showed a decrease in visual acuity. At the last post-operative follow-up, this visual improvement persisted in 14 eyes and graft survival rate after 156 weeks (3 years) was 69% in group 1 versus 44% in group 2 (p = 0.39, log-rank test). Secondary corneal neovascularization (8%), scarring (4%), ulcer (4%), or graft rejection (8%) happened mostly in the second group which was missing at least one of the suggested modifications.ConclusionsPKP in congenital aniridia must be considered as a high-risk keratoplasty. An optimized therapeutic approach seems to be promising in order to reduce the postoperative complication rate in these most difficult eyes.

Highlights

First described as ‘‘congenital irideremia’’ in the nineteenth century, congenital aniridia is a rare (1:60.000–1:90.000) pan-ocular disease that can be differentiated in two major categories depending on the presence of a PAX-6-Gene mutation [1,2,3,4]
penetrating keratoplasty (PKP) in congenital aniridia must be considered as a high-risk keratoplasty
Congenital aniridia is linked to different malformations such as iris, macular or optic nerve hypoplasia, and to deteriorating progressive major ocular dysfunctions such as limbal stem cell deficiency, premature onset of cataract, and secondary glaucoma that can lead to blindness throughout life [2, 3, 6,7,8]

Summary

Introduction

First described as ‘‘congenital irideremia’’ in the nineteenth century, congenital aniridia is a rare (1:60.000–1:90.000) pan-ocular disease that can be differentiated in two major categories depending on the presence of a PAX-6-Gene mutation [1,2,3,4]. Among those with a mutation of the PAX-6-Gene, frequent mutations involve point mutations and deletions. The incidence and severity of aniridiaassociated keratopathy (AAK) increases with age affecting about 20–30% of those patients and leads to corneal opacities, scarring, and vascularization due to a unique form of limbal stem cell deficiency [2, 9,10,11]

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