Case Report: Fuchs Dystrophy after Corneal Transplantation

Case Report

J Ophthalmol & Vis Sci. 2023; 8(1): 1073.

Case Report: Fuchs Dystrophy after Corneal Transplantation

Leite J*, Neves MM, Gomes M and Oliveira L

Department of Ophthalmology, Centro Hospitalar Universitário do Porto (CHUPorto), Portugal

*Corresponding author: João Leite Department of Ophthalmology, Centro Hospitalar Universitário do Porto (CHUPorto), Largo do Prof. Abel Salazar, Oporto, 4099-001 Oporto, Portugal

Received: December 19, 2022; Accepted: January 24, 2023; Published: January 31, 2023

Abstract

Fuchs Endothelial Corneal Dystrophy (FECD) consists of a progressive loss of corneal endothelial cells, with subsequent development of subepithelial connective tissue objectively leading to severely impaired vision. Generally, the time interval between the asymptomatic stage, with only the presence of guttates on biomicroscopy, to the most advanced stage, associated with chronic edema that causes corneal opacity and scarring, can vary between 20 to 30 years of evolution. Eye bank donor cornea selection includes FECD screening based on macroscopic and specular microscopyfindings. However, this screening may not be sensible enough to detect the disease in its earlier stages. We report 2 cases of FECD in corneal grafts in which the donor corneas eye bank screening was negative for FECD. These two cases highlight the importance of a meticulous evaluation of donor corneas candidates for corneal transplant. Traditional evaluation was not sensible enough to detect early phases of FECD. Additional screening methods, such as genetic testing, would improve the accuracy to detect FECD and other diseases present in donor corneas used in corneal transplants.

Keywords: Fuchs Dystrophy; Corneal Transplant; Penetrating Keratoplasty; Descemet Stripping Automated Endothelial Keratoplasty; Eye Bank

Abbreviations: FECD: Fuchs Endothelial Corneal Dystrophy; IC3D: International Classification of Corneal Dystrophies; LE: Left Eye; PK: Penetrating Keratoplasty; BCVA: Best Corrected Visual Acuity; DSAEK: Descemet Stripping Automated Endothelial Keratoplasty; EK: Endothelial Keratoplasty; DMEK: Descemet Membrane Endothelial Keratoplasty; hCECs: Human Corneal Endothelial Cells

Introduction

Fuchs Endothelial Corneal Dystrophy (FECD) is an age-related disorder that affect especially women [1], first described in 1910 by Ernst Fuchs as “corneal epithelial dystrophy” [2]. However, it was quickly discovered that the endothelium was the cause of this dystrophy with morphological and functional abnormalities [2,3]. According to the International Classification of Corneal Dystrophies (IC3D), FECD is categorized into 2 forms [2,4,5]: a rare, early-onset form and a more common late-onset form and both subtypes appear to have similar time to progression from disease onset to corneal decompensation [1,4]. Clinically FECD presents in four stages: in the first stage of the disease, the patient is asymptomatic but with central irregularly scattered guttae; in second stage, blurred vision, glare, and colored halos develop around the lights as stromal and epithelial edema develops. The patient may notice an improvement in symptoms over the course of the day due to increased tear evaporation, resulting in increased tear osmolality; the third stage is characterized by the development of subepithelial connective tissue objectively and although the patient is less complaining due to the reduction in edema, there is a decrease in visual acuity; the fourth stage is characterized by chronic edema that causes corneal opacity and scarring, leading to severely impaired vision and pain [2,3,6].

This article presents 2 cases of FECD that occur in eyes previously submitted to corneal transplantation.

Case Report 1

We report the case of an 82-year-old woman, pseudophakic, with a history of Left Eye (LE) Penetrating Keratoplasty (PK) due to FECD in 2007. In 2017, a new PK was performed due to graft failure (diffuse corneal opacification). Two years after the second PK, in 2019, the Best Corrected Visual Acuity (BCVA) was 20/40. In 2021, the patient started complains of slightly reduced vision in her LE (20/50), with episodic vision fluctuations and glare. Slit-lamp examination revealed changes in endothelial specular reflection, with small dark spots and a “beaten metal” pattern (Figure 1). Specular microscopy (Figure 2a) confirmed the diagnosis of FECD, with the presence of typical endothelial guttate, irregular warts and cells varying in size and shape. The preoperative evaluation of the donor cornea (50 years old; endothelial cell count: 2463 cells/mm2.) used in the 2017 corneal transplant (Figure 2b) showed a normal slit lamp examination and specular microscopy evaluation revealed only a few small black spots, interpreted as artifacts as they didn’t seem to be guttatas. Overall, there were not clear signs suggesting pathological endothelial changes contraindicating the use of that cornea in a transplant.