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Surgical Management and Outcomes of Patients with Concurrent Fuchs’ Corneal Endothelial
Dystrophy and Keratoconus – A Multi-Center Case Series
Samir Vira1,2, MD, Usiwoma Abugo1, BS, Brian Sperling2, Sadeer Hannush2, MD, Surendra Basti3, MD, Charles Bouchard1, MD
1 Loyola University Medical Center, Maywood IL; 2 Wills Eye Institute, Philadelphia, PA; 3 Northwestern Memorial Hospital, Chicago IL
Table 1: Patient Demographics, Surgical Procedure, and Pre- and Postoperative outcome measurements
Introduction
Cases of concurrent Fuch’s endothelial
dystrophy (FED) and keratoconus (KCN)
have been reported in the literature1-7.
Most of these cases are bilateral with
only two reported unilateral cases1,2. It
has been suggested that the corneal
edema in FED might be masked by the
corneal thinning in KCN; likewise, the
thin cornea one would expect to find in
KCN could be falsely thickened due to
the corneal edema of FED3. 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.
Several of the previously reported
combined cases were treated with
penetrating keratoplasty4,5. This is the
first multi-center case series that
includes patients with concurrent disease
who underwent Descemet stripping
endothelial keratoplasty (DSEK),
penetrating keratoplasty (PKP) or
cataract extraction with intraocular lens
implant (CE-IOL). We present the
surgical management and outcomes of
patients with bilateral FED and KCN.
Methods
This is a retrospective case series of 9
eyes of 6 patients with combined FED
and KCN who underwent DSEK, PKP,
or CE-IOL with one of 3 surgeons at 3
different centers. Clinical information
collected included the following:
corneal topography, central corneal
thickness, and endothelial cell density
from specular microscopy. Visual
outcomes and change in keratometric
measurements were evaluated.
Acknowlegements: The Richard A. Perritt
Charitable Foundation
Patient 1 OD
DSAEK*
Patient 1 OS
DSAEK*
Patient 2 OS
DSEK
Patient 2 OD
DSEK
Patient 3 OS
DSAEK
Patient 4 OD
CE/IOL
Patient 1 OS
PKP*
Patient 5 OD
PKP
Patient 6 OD
PKP
41
F
41
F
70
M
70
M
74
M
54
F
41
F
63
F
61
F
20/30
20/40
20/80
20/60
20/60
20/50
20/40
20/400
CF
Orbscan
52.5
Yes
Orbscan
56.0
Yes
Tomey TMS4
44.0
Yes
Tomey TMS4
43.2
Yes
Pentacam
48.6
Yes
Orbscan
47.4
Yes
Orbscan
56.0
Yes
Tomey TMS4
53.1
Yes
Tomey TMS4
58.2
No (central)
Corneal thickness
Ultrasound
610
662
640
696
485
614
662
611
Too thick
Post-op
BCVA
Spectacle correction
20/25
20/40
20/20
20/25
20/40 (UCVA)
20/25
20/20
14
6
40
18
25
10
10
26
6
Visante
49.9
Yes
2.6
Visante
50.2
Yes
5.8
Tomey TMS4
42.9
Yes
1.0
Tomey TMS4
42.3
Yes
0.9
Pentacam
44.6
Yes
4.0
Visante
47.4
Yes
0.0
Visante
41.9
No
14.2
Tomey TMS4
44.2
No
8.9
Tomey TMS4
41.0
No (central)
17.2
Procedure
Patient data
Age
Gender
Pre-op
BCVA
Spectacle correction
Figure 1: Slit lamp photo of patient 1 OD
preoperatively. Note the apical opacity along
with edema
Keratometry
Average
Inferior steepening (on topography)
Follow-up duration (months)
Keratometry (Diopters)
Average
Inferior steepening (on topography)
Change in Mean Keratometry
Figure 2: Slit lamp photo of patient 1 OD
postoperatively at month 14. Note the apical
opacity and edema have resolved in the
DSAEK graft
Results
Figure 4: Slit lamp photo of patient 1 OS
preoperatively. Note the apical opacity along
with edema
20/40 (UCVA) 20/200 (UCVA)
Corneal thickness
Ultrasound
548
Not performed
626
675
527
609
526
564
Not performed
Specular microscopy
Cell Count
When performed postoperatively (months)
2660
14
Not performed
1750
37
1720
13
Not performed
1828
10
2994
10
950
23
1799
6
DSAEK = Descemet Stripping Automated Endothelial Keratoplasty; DSEK = Descemet Stripping Endothelial Keratoplasty
CE/IOL = Cataract Extraction with Intraocular Lens Implant; PKP = Penetrating Keratoplasty
BCVA = Best Corrected Visual Acuity
UCVA = Uncorrected Visual Acuity
*Patient 1 required rebubbling of dislocated graft at Post-op week 2. The patient then developed bilateral graft rejection (Post-op month 2 for OD, Post-op month 10 for OS) due to poor compliance. The
rejection episode resolved in OD. However, the edema
Figure 3: Pre- and post-operative (month 14)
topography of patient 1 after DSAEK. Note the
improvement in keratometry readings but no
change in inferior steepening
All patients had slit lamp and corneal topography
findings consistent with combined FED and KCN.
Four of the six patients were female with an
average age of 59 years. Follow-up for patients
ranged from 6 to 40 months (median of 14
months). Five eyes underwent DSEK; three eyes
underwent PKP; one eye had CE/IOL. In patients
who underwent DSEK, best corrected visual
acuity (BCVA) was 20/40 or better in all eyes.
Mean keratometry measurements improved in all
cases; however, topography still demonstrated
inferior steepening in each case. In patients who
underwent PKP, visual acuity improved in all
cases. The patient who underwent CE/IOL had
BCVA of 20/25 with unchanged keratometry
measurements.
Conclusion
Figure 5: Slit lamp photo of patient 1 OS
postoperatively at month 6. Note that the
apical opacity and edema has resolved in
the DSAEK graft.
Figure 6: Pre- and post-operative (month 6)
topography of patient 1 after DSAEK. Note the
improvement in keratometry readings but no
change in inferior steepening
Patients with FED and KCN have been previously
reported with most of them managed with PKP.
We present 5 eyes of 3 patients who were
managed with DSEK for the FED.
Topographically, the characteristic inferior
steepening of KCN in these cases did not change;
however, all DSEK cases had flatter postoperative keratometry measurements with
improvement in visual acuity. If DSEK is
performed for FED prior to visually significant
apical corneal scarring from KCN, the progression
of the KCN might be stabilized by the DSEK
procedure. Long term follow-up is planned to
better assess this hypothesis.
References
Figure 8: Slit lamp photo of patient 3
showing endothelial guttata with red reflex
Figure 7: Slit lamp photo of patient 3
demonstratring Vogt’s striae
1. Martone G, Tommasi C, Traversi C, Balestrazzi A, Berni E, Nuti E,
Tosi GM. Unilateral corneal endothelial dystrophy and anterior
keratoconus. Eur J Ophthalmol 2007; 17(3):430-2.
2. Darlington JK, Mannis MJ, Segal WA. Anterior keratoconus
associated with unilateral cornea guttata. Cornea 2001; 20(8):881-4.
3. Jurkunas U, Azar DT. Potential complications of ocular surgery in
patients with coexistent keratoconus and Fuch’s endothelial dystrophy.
Ophthalmology 2006; 113:2187-97.
4. Lipman RM, Rubenstein JB, Torczynski E. Keratoconus and Fuch’s
corneal endothelial dystrophy in a patient and her family. Arch
Ophthalmol 1990; 108(7):993-5.
5. Salouti R, Nowroozzadeh M, Zamani M, Ghoreyshi M. Combined
anterior keratoconus and Fuch’s endothelial dystrophy: a report of two
cases. Clin Exp Optom 2010; 93(4):268-270.
6. Orlin SE, Raber IM, Eagle RC Jr, Scheie HG. Keratoconus
associated with corneal endothelial dystrophy. Cornea 1990; 9(4):229304.
7. Cremona FA, Ghosheh FR, Rapuano CJ, Eagle RC Jr,
Hammersmith KM, Laibson PR, Ayres BD, Cohen EJ. Keratoconus
associated with other corneal dystrophies. Cornea 2009 Feb; 28(2):12735. 9(4):229-304.