DEPARTMENT OF OPHTHALMOLOGY

FACULTY OF MEDICINE SRIWIJAYA UNIVERSITY

RSUP MOHAMMAD HOESIN
PALEMBANG

Case Report : Management of Fuchs Endothelial Corneal Dystrophy

Presenter : Rezandi Aziztama
Supervisor : Anang Tribowo, MD

Has been reviewed and approved by:

Anang Tribowo, MD

Wednesday, 22nd June 2022

 MANAGEMENT OF FUCHS ENDOTHELIAL
CORNEAL DYSTROPHY: A CASE
REPORT

Abstract
Introduction: Corneal dystrophy is a corneal disorder caused by genetic
disorders which generally defined as a bilateral, symmetrical, congenital
condition that appears to have little or no association with environmental or
systemic factors. Fuchs endothelial corneal dystrophy (FECD) is the most
common of the corneal dystrophies. The clinical manifestations of each
type of corneal dystrophy vary greatly, the therapy given also varies
depending on clinical symptoms.
Purpose: To report a case of a patient with Fuchs Endothelial Corneal
Dystrophy who was treated with Keratoplasty accompanied by injection of
anti-VEGF.
Case Report: A woman, 50 years old, came for treatment at the RSMH eye
polyclinic with a chief complaint white spot appeared on the right eye.
Then about 4 months ago the same complaint appeared in the left eye.
Complaints are accompanied by discomfort such as a lump, sometimes
watery and glare in both eyes. Complaints are not accompanied by red
eyes. The patient also complained of blurry vision more or less 2 years
ago. The visual acuity of the right eye was 1/300 and the left eye was
6/18. On ophthalmological examination, the cornea appeared cloudy and
there was edema with endothelial dystrophy and neovascularization OD,
for the right eye, examination of the anterior chamber, difficult to assess
iris, pupil and lens and ROFD (-). It also found there’s endothelial
dystrophy and neovascularization OS. The patient was diagnosed with
Fuchs endothelial corneal dystrophy (FECD). The patient was treated with
keratoplasty in the right eye and subconjunctival injection of anti-VEGF in
the left eye. The main goal of therapy in this case is to reduce
inflammatory infection and improve vision. Anti-VEGF has been shown to
reduce corneal neovascularization and the prognosis for graft survival is
good.
Conclusion: A case of Fuchs Endothelial Corneal Dystrophy was reported in
a female patient with complaints of white spots appearing on both eyes.
The management in this case was in the form of keratoplasty and
subconjunctival anti-VEGF injection. The prognosis for graft survival is
good, especially if the procedure is performed before vascularization
occurs.

Keywords: corneal dystrophy, Fuchs Endothelial Corneal Dystrophy, anti-

VEGF, keratoplasty

I. Introduction

1
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Corneal dystrophy is a corneal disorder caused by genetic disorders.

Corneal dystrophy is generally defined as a bilateral, symmetrical,
congenital condition that appears to have little or no association with
environmental or systemic factors. The term dystrophy is actually a term
that is less precise to use in corneal dystrophy, it would be better to use
the term genetic disorder of the cornea. However, because this
inappropriate use has been used for nearly ten years in the literature
discussing corneal dystrophies, the International Committee for the
Classification of Corneal Dystrophies (IC3D) has decided to continue using
the term dystrophy for this disorder.
Fuchs endothelial corneal dystrophy (FECD) is the most common of
the corneal dystrophies. Globally in 2012, 39% of corneal transplants were
to correct FECD. 5 However, corneal dystrophy is a rare disease. In the
United States, less than 0.01% of Americans have CD. Another study
showed that approximately 530 persons per million in the United States
have endothelial-type CD, and 5–10 persons per million have granular,
macular or lattice-type CD. 6 Only four families are reported to have
CSCD.7
The clinical manifestations of each type of corneal dystrophy vary
greatly, from having no symptoms to severe visual impairment. The
therapy given also varies depending on clinical symptoms, adequate
classification based on histological findings, the extent and depth of the
corneal lesions that occur. The purpose of this case report is to report a
case of a patient with Fuchs Endothelial Corneal Dystrophy who was
treated with Keratoplasty accompanied by injection of anti-VEGF.

II. Case Report

A woman, 50 years old, came for treatment at the RSMH eye

polyclinic subdivision IIM RSUP Dr. Mohammad Hoesin Palembang. The
patient complained about 6 months ago a white spot appeared on the
right eye. Then about 4 months ago the same complaint appeared in the
patient's left eye. Complaints are accompanied by discomfort such as a
lump, sometimes watery and glare in both eyes. There are no red eyes.
The patient also complained of blurry vision more or less 2 years ago.
 3

There was no history of complaints like this before. The longer the white
spots spread and the complaints did not improve, then the patient went to
the clinic and was referred to RSMH. The patient only had a history of
wearing glasses. There was no family history of malignancies and similar
conditions. The general physical examination of the patient was
unremarkable. The visual acuity of the right eye was 1/300 and the left
eye was 6/18. On ophthalmological examination, the cornea appeared
cloudy and there was edema with endothelial dystrophy and
neovascularization OD, for the right eye, examination of the anterior
chamber, difficult to assess iris, pupil and lens and ROFD (-). It also
found there’s Cloudiness at 11-4 o'clock, size 7x5 mm, endothelial
dystrophy and neovascularization OS. The other anterior segment
examinations of the eyes were within normal limits. B-scan
ultrasonography of the right eye and left eye were within normal limits.

Figure 1. B-scan ultrasonography of the right eye and left eye

within normal limits
Source: RSUP Mohammad Hoesin Palembang

The patient was diagnosed with endothelial dystrophy by the results of

the ophthalmological examination which showed the presence of
endothelial dystrophy. Clinically, the diagnosis is more towards Fuchs
endothelial corneal dystrophy (FECD). In these patients, genetic
examination could not be performed due to limited facilities.
The management of the patient was keratoplasty in the right eye and
subconjunctival injection of anti-VEGF in the left eye. She was then
administered mefenamic acid 500 mg three times a day,
methilprednisolone three times a day, cefixime 100mg two times a day and
Dexamethasone, neomycin sulfate & polymixin B sulfate six times daily for
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the right eye, Sodium chloride & potassium chloride six times daily for the
left eye.
Nine days post keratoplasty and subconjunctival anti-VEGF injection
follow-up showed the visual acuity of the right eye was 4/60 and left eye
6/12. There are still endothelial dystrophy and neovascularization on the
left eye. The posterior segment examination showed RFODS (+). Anti-
VEGF has been shown to reduce corneal neovascularization and the
prognosis for graft survival is good.

III. Discussion
It was reported that a woman, 50 years old, came for treatment at
the RSMH eye polyclinic. From the history of the disease, it was found
that the patient complained about 6 months ago that a white spot
appeared on the right eye. Then about 4 months ago the same complaint
appeared in the patient's left eye. Complaints are accompanied by
discomfort such as a lump, sometimes watery and glare in both eyes.
Complaints are not accompanied by red eyes. The patient also
complained of blurry vision more or less 2 years ago. There was no
history of complaints like this before. The longer the white spots spread
and the complaints did not improve, then the patient went to the clinic and
was referred to RSMH. The patient only had a history of wearing glasses.
The patient denied a family history of the same disease.On
ophthalmological examination, the cornea appeared cloudy and there was
edema with endothelial dystrophy and neovascularization OD, for the right
eye, examination of the anterior chamber, difficult to assess iris, pupil and
lens and ROFD (-). It also found there’s Cloudiness at 11-4 o'clock, size
7x5 mm, endothelial dystrophy and neovascularization OS.

Corneal dystrophy is generally defined as a bilateral, symmetrical,

congenital condition that appears to have little or no association with
environmental or systemic factors. The term dystrophy is actually a term
that is less precise to use in corneal dystrophy, it would be better to use
the term genetic disorder of the cornea. However, because this
inappropriate use has been used for nearly ten years in the literature
 5

discussing corneal dystrophies, the International Committee for the

Classification of Corneal Dystrophies (IC3D) has decided to continue
using the term dystrophy for this disorder. 1-4 IC3D issued a new
classification consisting of 4 categories, namely:
• Category 1 (K1): clearly defined corneal dystrophy, the affected gene
has been found, the mutation has been specifically identified.
• Category 2 (K2): dystrophy that has been clearly defined, the location
of the affected chromosome locus is known, but the specific gene that
is affected cannot be identified.
• Category 3 (K3): dystrophy that has been clearly defined clinically, but
the location of the affected chromosomal locus is unknown
• Category 4 (K4): this category is reserved for corneal dystrophies that
are newly suspected, or have previously been documented as corneal
dystrophies, although there is no research evidence to prove this.
In these patients, genetic examination could not be performed due to limited
facilities. Therefore, the possible diagnosis based on the IC3D
classification is in categories 3 and 4. Then, judging by the results of the
ophthalmological examination which showed the presence of endothelial
dystrophy, the diagnosis was narrower in endothelial dystrophy. In the
Descemet Membrane and Corneal Endothelial Dystrophy Group, there
are 3 possible diagnoses, namely Fuchs endothelial corneal dystrophy
(K1, K2, or K3), Posterior Polymorphous corneal dystrophy (K1 or K2),
Hereditary congenital corneal dystrophy 1 (K2) or Hereditary congenital
corneal dystrophy 2 (K1).
Fuchs endothelial corneal dystrophy (FECD) is the most common
among the corneal dystrophies. 7FECD can be divided into early-onset
(manifests in the 3rd decade of life) and late-onset (manifests in the 5th
decade of life, on average). Both early and late forms have a female
predominance at a ratio of 2.5-3:1.3. 15 Meanwhile, the prevalence and
incidence of CHED are currently unknown. Cases that have been
identified are mainly among children with a family history of corneal
dystrophy and blood parents from Saudi Arabia, Pakistan, India, and
Ireland. 11 Posterior polymorphic dystrophy (PPCD) is considered
extremely rare. Because many patients are asymptomatic, the exact
 6

incidence of PPCD is unknown. PPCD affects men and women equally.

From an epidemiological point of view, the diagnosis is more towards
Fuchs endothelial corneal dystrophy (FECD).
The dystrophy in CHED is usually non-progressive with
asymmetrical corneal opacities and edema that ranges from a diffuse
haze to a ground-glass appearance; focal gray spots are occasionally
seen. Corneal thickening (2–3 times normal) occurs, with rare
subepithelial band keratopathy and increased IOP. Blurred vision and
nystagmus occur with minimal or no epiphora or photophobia. 2 In FECD,
clinical conditions related to the severity of corneal damage are divided
into 4 stages where the final stage is a condition of neovascularization.
Cornea guttata is first seen in the center and then spreads to the
periphery (stage 1). In some patients, the guttata cornea develops, and
the disease never progresses beyond this stage. Cornea guttata may
become confluent and appear to be "beaten metal". The guttate cornea
in late-onset FECD is larger than that seen in early-onset FECD. Stage 2
is characterized by endothelial decompensation and stromal edema. As
the disease progresses, Descemet's membrane may thicken, and
stromal edema may worsen, leading to epithelial bullae and bullous
keratopathy (stage 3). The central corneal thickness may be closer to 1
mm (0.50–0.60 mm is usually considered normal). Subepithelial fibrosis,
scarring, and superficial peripheral vascularity from chronic edema occur
in late-stage disease (stage 4). 2 Close examination of the posterior
surface of the cornea geographic, discrete or wide bands of endothelium
with scalloped edges. Clinically, the diagnosis is more towards Fuchs
endothelial corneal dystrophy (FECD).
The management of the patient was keratoplasty in the right eye
and subconjunctival injection of anti-VEGF in the left eye. The main goal
of therapy in this case is to reduce inflammatory infection and improve
vision. Keratoplasty is a surgical procedure for replacing the cornea, in
this case penetrating keratoplasty (full-thickness), which is the
replacement of all layers of the cornea. The indications for keratoplasty
depend on the visual requirements and the expected long-term benefits
of surgery. Common indications for optical keratoplasty include bullous
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keratoplasty, corneal clouding after infectious keratitis, trauma, graft

failure, corneal endothelial and stromal dystrophy, corneal degeneration,
congenital corneal opacities, and keratoconus. visual function as well as
increased visual acuity are still a consideration.
The prognosis for graft survival is good, especially if the procedure
is performed before vascularization occurs. 2 Inhibition of VEGF has been
shown to reduce corneal neovascularization and improve corneal graft
survival in animal models. Bevacizumab also inhibits corneal
neovascularization in humans. Although the results of previous studies
were statistically significant, the regression of corneal neovascularization
was only partial.25

IV. Conclusion

A case of Fuchs Endothelial Corneal Dystrophy was reported in a

female patient with complaints of white spots appearing on both eyes.
The management in this case was in the form of keratoplasty and
subconjunctival anti-VEGF injection. Anti-VEGF has been shown to
reduce corneal neovascularization and the prognosis for graft survival is
good, especially if the procedure is performed before vascularization
occurs.
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