An Attempt to Optimize the Outcome of Penetrating Keratoplasty in Congenital Aniridia-associated Keratopathy (AAK)

Overview

Journal Int Ophthalmol

Specialty Ophthalmology

Date 2021 Jul 29

PMID 34324101

Citations 6

Authors

C J Farah

F N Fries

L Latta

B Kasmann-Kellner

B Seitz

Affiliations

Soon will be listed here.

Abstract

Purpose: To propose an optimized microsurgical and medical approach to reduce the risk of complications after penetrating keratoplasty (PKP) in patients with aniridia-associated keratopathy (AAK).

Methods: Retrospective 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.

Results: A 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.

Conclusions: PKP 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.

Citing Articles

The Triple Procedure in Patients with Congenital Aniridia.

Wowra B, Lach-Wojnarowicz O, Wysocka-Kosmulska M, Dobrowolski D, Wylegala E J Clin Med. 2024; 13(21).

PMID: 39518758 PMC: 11547142. DOI: 10.3390/jcm13216619.

[Phototherapeutic keratectomy for treatment of infectious keratitis in a patient with PAX6-related aniridia].

Hoxha Z, Flockerzi E, Szentmary N, Fries F, Seitz B Ophthalmologie. 2024; 121(11):916-919.

PMID: 39283354 DOI: 10.1007/s00347-024-02104-7.

Superficial Keratectomy Alone versus in Combination with Amniotic Membrane Transplantation in Aniridia-Associated Keratopathy and a Short-Term Clinical Outcome.

Wowra B, Wysocka-Kosmulska M, Dobrowolski D, Wylegala E J Clin Med. 2024; 13(11).

PMID: 38892970 PMC: 11173058. DOI: 10.3390/jcm13113258.

[Modern corneal diagnostics as the key for the correct classification of the disease and optimal treatment decisions].

Berger T, Flockerzi E, Daas L, Hamon L, Khattabi Z, Berger M Ophthalmologie. 2023; 120(12):1238-1250.

PMID: 37707672 DOI: 10.1007/s00347-023-01919-0.

A Cross-sectional Analysis of 556 Eyes Entering the Homburg Aniridia Centre.

Fries F, Naray A, Munteanu C, Stachon T, Lagali N, Seitz B Klin Monbl Augenheilkd. 2023; 241(3):275-282.

PMID: 37647922 PMC: 10954372. DOI: 10.1055/a-2065-8405.

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