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, MD1 Loyola University Medical Center, Maywood IL; 2 Wills Eye Institute, Philadelphia, PA; 3 Northwestern Memorial Hospital, Chicago IL

Introduction

ReferencesMethods

Conclusion

Table 1: Patient Demographics, Surgical Procedure, and Pre- and Post-operative outcome measurements

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.

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.

Results

Figure 4: Slit lamp photo of patient 1 OS preoperatively. Note the apical opacity along with edema

Figure 1: Slit lamp photo of patient 1 OD preoperatively. Note the apical opacity along with edema

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 post-operative 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.

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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.

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.

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

Acknowlegements: The Richard A. Perritt Charitable Foundation

Patient 1 OD Patient 1 OS Patient 2 OS Patient 2 OD Patient 3 OS Patient 4 OD Patient 1 OS Patient 5 OD Patient 6 ODProcedure DSAEK* DSAEK* DSEK DSEK DSAEK CE/IOL PKP* PKP PKP

Patient data Age 41 41 70 70 74 54 41 63 61 Gender F F M M M F F F F

Pre-opBCVA Spectacle correction 20/30 20/40 20/80 20/60 20/60 20/50 20/40 20/400 CF

Keratometry Orbscan Orbscan Tomey TMS4 Tomey TMS4 Pentacam Orbscan Orbscan Tomey TMS4 Tomey TMS4 Average 52.5 56.0 44.0 43.2 48.6 47.4 56.0 53.1 58.2 Inferior steepening (on topography) Yes Yes Yes Yes Yes Yes Yes Yes No (central)

Corneal thickness Ultrasound 610 662 640 696 485 614 662 611 Too thick

Post-opBCVA Spectacle correction 20/25 20/40 20/20 20/25 20/40 (UCVA) 20/25 20/20 20/40 (UCVA) 20/200 (UCVA)

Follow-up duration (months) 14 6 40 18 25 10 10 26 6

Keratometry (Diopters) Visante Visante Tomey TMS4 Tomey TMS4 Pentacam Visante Visante Tomey TMS4 Tomey TMS4 Average 49.9 50.2 42.9 42.3 44.6 47.4 41.9 44.2 41.0 Inferior steepening (on topography) Yes Yes Yes Yes Yes Yes No No No (central) Change in Mean Keratometry 2.6 5.8 1.0 0.9 4.0 0.0 14.2 8.9 17.2

Corneal thickness Ultrasound 548 Not performed 626 675 527 609 526 564 Not performed

Specular microscopy Cell Count 2660 Not performed 1750 1720 Not performed 1828 2994 950 1799 When performed postoperatively (months) 14 37 13 10 10 23 6

DSAEK = Descemet Stripping Automated Endothelial Keratoplasty; DSEK = Descemet Stripping Endothelial KeratoplastyCE/IOL = Cataract Extraction with Intraocular Lens Implant; PKP = Penetrating KeratoplastyBCVA = Best Corrected Visual AcuityUCVA = 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 7: Slit lamp photo of patient 3 demonstratring Vogt’s striae

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

Figure 8: Slit lamp photo of patient 3 showing endothelial guttata with red reflex

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