Surgical Management and Outcomes of Patients with Concurrent Fuchs’ Corneal Endothelial

1. 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 Introduction Table 1: Patient Demographics, Surgical Procedure, and Pre- and Post-operative outcome measurements Results 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. 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. 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 Conclusion 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. 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 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 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 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 Methods References 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. 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):229-304. 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):127-35.9(4):229-304. 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 Acknowlegements: The Richard A. Perritt Charitable Foundation