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Clinical Findings and Surgical Outcomes of Two Patients with Concurrent Fuch’s Endothelial Dystrophy and Keratoconus
Allison Landes, MD; Charles Bouchard, MD; Samir Vira, MD
Loyola University Chicago Stritch School of Medicine
Introduction
Cases of concurrent Fuch’s endothelial
dystrophy and keratoconus have been
reported in the literature1-6. Most of these
cases are bilateral with only two reported
unilateral cases1,2. It has been suggested that
the corneal edema in Fuch’s might be
masked by the corneal thinning in
keratoconus; likewise, the thin cornea one
would expect to find in keratoconus could be
falsely thickened due to the corneal edema of
Fuch’s3. This feature may lead to an
underestimation of the disease severity. It is
critical to identify both entities when they
exist concurrently in order to choose the most
appropriate surgical procedure when
indicated and to avoid an unexpected
surgical outcome. LASIK should be avoided
in all patients with keratoconus and cataract
extraction may lead to corneal
decompensation in Fuch’s. Several of the
previously reported combined cases were
treated with penetrating keratoplasty4,5. Here
we report 4 eyes (2 patients) with concurrent
bilateral Fuch’s and keratoconus. One eye
underwent cataract extraction and two eyes
underwent Descemet’s stripping endothelial
keratoplasty (DSEK). To our knowledge,
these are the first reported combined cases
that have undergone DSEK. Our hypothesis
is that the endothelial graft may slow the
progression of keratoconus over time.
Methods
We reviewed the clinical findings and surgical
outcomes of 2 patients with Fuch’s endothelial
dystrophy and keratoconus. Clinical
examination, corneal topography, central
corneal thickness (CCT), and endothelial cell
density were evaluated as well as surgical
outcomes.
Acknowledgements:
This work was supported by the
Richard A. Perritt Charitable Foundation.
Results
Case 1 (Pt #1, OS): A 41 year old woman presented to
the Loyola Cornea Service with mild bilateral apical
scarring and corneal topography consistent with
keratoconus. The left eye had a mean keratometry of
56.0D. She also had confluent corneal guttata, stromal
edema, and mild microcystic edema consistent with
Fuch’s dystrophy. CCT was 662 OS. Her best corrected
visual acuity OS was 20/40-2 with spectacles and 20/30-1
with a rigid gas permeable contact lens (RGP). She was
contact lens intolerant and desired improvement in her
spectacle vision, so she elected to have a DSEK
procedure OS. Postoperatively, she developed an
inferotemporal sectoral graft separation. Rebubbling on
post-operative day 16 resulted in only mild improvement
in the separation. Two weeks after the rebubbling, she
was scheduled for a PKP. However, when the patient
was brought under the operating microscope, the
procedure was aborted due to significant improvement of
the graft separation. Eight weeks after the rebubbling the
separation had resolved and her best corrected visual
acuity was 20/40+2 with glasses and 20/30-1 with a RGP.
Mean keratometry at 6 months postoperative was
50.21D.
Case 2 (Pt #1 OD): In the right eye, this same patient
had a mean keratometry of 52.5D, CCT 610, and a best
corrected visual acuity 20/40-2 with spectacles and
20/25-1 with a RGP. She was contact lens intolerant OD,
so she underwent DSEK OD 5 months after DSEK OS.
Postoperative day one the graft was centered with
peripheral detachments inferiorly and superiorly. One
week later she was 20/40 uncorrected and the graft was
only mildly separated inferotemporally. She was
subsequently lost to follow-up and presented 2 months
later with graft rejection OU and vision 20/100 OU.
Topical prednisolone was restarted q1-2 hours. Two days
later her vision improved to 20/50 OD and 20/60 OS and
she was also started on an oral prednisone taper. Four
days later vision remained stable and specular
microscopy revealed and endothelial cell count density of
2646/mm2 OD, unobtainable OS.
Case 3 (Pt #2, OD): A 54 year old female presented
with exam findings and corneal topography consistent
with keratoconus OD worse than OS. Mean
keratometry was 47.4D OD and 45.5D OS. She had
confluent corneal guttata and CCT measurements of
614 OD and 595 OS. She also had a visually
significant cataract OD. Her best corrected visual
acuity OD was 20/40 with spectacles and 20/30- with a
RGP. She underwent uneventful cataract extraction
with posterior chamber lens implant OD. At 4 months
postoperative, her best corrected spectacle vision was
20/25-2, mean keratometry was 47.17D, CCT was
609, and specular microscopy revealed an endothelial
cell density of 929/mm2.
Pre and Postoperative Values
Pre-op BCVA
Pre-op Topography (mean
keratometry)
Pre-op CCT
Procedure
Post-op BCVA
Post-op Topography (mean
keratometry)
Post-op CCT
Post-op Specular Microscopy
Case 1 (Pt #1, OS)
20/40-2 (glasses)
20/30-1 (RGP)
56.0D (Orbscan)
Case 2 (Pt #1, OD)
20/40-2 (glasses)
20/25-1 (RGP)
52.5D (Orbscan)
Case 3 (Pt #2, OD)
20/40 (glasses)
20/30- (RGP)
47.4D (Orbscan)
662
DSEK and rebubble
20/40+2 (glasses)
20/30-1 (RGP)
(4 months)
50.21D (Visante)
(6 months)
610
DSEK
Unobtainable due to rejection,
20/40 sc
614
CE/IOL
20/25-2 (glasses)
(1 month)
unobtainable
unobtainable
2646/mm2
(2.5 months)
47.17D (Visante)
(4 months)
609 (5 months)
929/mm2
(5 months)
Pt #1 Pre-op
Pt #1 Post-op OD, 1 week
Pt #1 Post-op OS, 1 and 6 mo.
Pt #2 Topography, POM #4
Conclusion
Patients with Fuch’s dystrophy and
keratoconus have been previously
described. We report the first case of DSEK
in the management of endothelial
dysfunction in this patient population.
Adherence of the graft may be problematic
due to the steep posterior corneal surface in
keratoconus. If DSEK is performed for visual
loss associated with Fuch’s dystrophy prior
to visually significant apical corneal scarring
from keratoconus, the progression of the
keratoconus might be stabilized. Long term
follow up is planned to better assess this
hypothesis.
References
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