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Fuchs' Endothelial Dystrophy Management with Penetrating Fuchs’ Endothelial Dystrophy Management with Penetrating Keratoplasty and Contact Lenses Chirag Patel, OD University of Houston College of Optometry Texas Eye Research and Technology Center Abstract: Fuchs’ endothelial dystrophy (FED) is an autosomal-dominant endothelial disorder characterized by an accelerated loss of corneal endothelial cells leading to corneal edema and impaired visual acuity.1 This report describes pre-surgical and post-surgical management of FED. Case History Demographic: 70 year old white female Chief Complaint: Patient was referred by a corneal specialist (ophthalmology) for contact lens management following penetrating keratoplasty (PKP) OS. She had previously worn a Jupiter scleral contact lens OS, but reverted to an older corneal lens (Rose K) after losing the scleral lens ~7 months prior to this visit. At this visit the patient reported decreased vision OU. Ocular History: o Pseudophakia OU: >15 years ago o FED: diagnosed ~10 years ago o Glaucoma secondary to PKP OS: diagnosed April 2012 Ocular Surgical History: o Cataract surgery w/PCIOL OU: >15 years ago o Photorefractive keratectomy (PRK) OU: >15 years ago (following cataract surgery) o PKP w/ anterior vitrectomy OS: 2010 Medical History: unremarkable Medication History: o Ocular: Latanoprost 0.005% - 1gtt q.h.s. OS, Timolol Maleate 0.5% - 1gtt q.a.m. OS o Systemic: Calcium supplement, Glucosamine Chondroitin Complex, Premarin 25mg , Tylenol 325mg Pertinent Findings Initial Visit: o Entering Visual Acuity w/ Habitual Correction: . OD: 20/40+2 OS: 20/30 o Slit Lamp Findings: . OD: 2+ diffuse guttae located at the corneal endothelium. (-) corneal edema, PCIOL – clear and in good position . OS: Clear and well centered full thickness corneal graft. Trace guttae centrally, 3+ guttae peripherally beyond graft edge. (-) corneal edema, PCIOL – clear and in good position . All other findings were within normal limits o Tonometry (NCT) @ 10:19am: . OD: 25mmHg OS: 13mmHg o Presenting Contact Lens Parameters: . OD: no lens . OS: Rose K-2 IC +1.25 / BC: 7.70 / Dia: 10.4 DVA: 20/25- Harsh central bearing, inferior bearing over graft junction, adequate peripheral and mid peripheral clearance o Contact Lens Trial # 1: . OD: Air Optix N&D Aqua (lotrafilcon A) +0.50 / BC: 8.4 / Dia: 13.8 DVa: 20/30 ORx: +0.25-0.50 X 090 DVa: 20/25 Adequate coverage, centration, and movement . OS: Jupiter Scleral (hexafocon A) -9.25 / BC: 7.03 / Dia: 18.2 ORx: +6.25 sph DVa: 20/20 Acceptable central and peripheral vault, (-) vessel blanching x 360, very large diameter (to inf. fornix) prohibits adequate movement, superior nasal bearing Differential Diagnosis: Primary/Leading: Fuchs’ endothelial dystrophy. Others: Posterior polymorphous dystrophy, Hassall-Henle bodies, congenital hereditary endothelial dystrophy, pseudophakic or aphakic bullous keratopathy, pigment dispersion syndrome, and keratic precipitates secondary to uveitis. Discussion: Signs: The hallmark findings of FED are guttae along Descemet’s membrane (posterior limiting lamina). Guttae are thought to be focal excrescences of abnormal collagen deposited by the endothelium.4 As the disease progresses, patients will develop recurrent epithelial and sub-epithelial bullae. End-stage disease is characterized by severe reduction in visual acuity as the result of the formation of sub-epithelial scar tissue.2 Symptoms: Patients with FED will most commonly complain of a painless reduction in vision, increased glare, and visible halos around lights. These symptoms are particularly severe upon waking in the morning and decrease in severity throughout the day. In more advanced cases, patients may present with recurrent pain caused by the formation of epithelial and sub-epithelial bullae.2 Clinical Staging: o Stage 1: Appearance of corneal guttae on biomicroscopy o Stage 2: Patients experience painless vision loss and increased glare o Stage 3: Formation of epithelial and sub-epithelial bullae o Stage 4: Opacification and vascularization of the cornea FED is an autosomal dominant disorder with incomplete penetrance. There is an apparent predilection toward the female gender with a 4:1 female to male ratio at the time of keratoplasty.3,4 This condition is equally common among whites and blacks, but is rare in individuals of Asian descent.3 Endothelial dysfunction and the resultant stromal edema are caused by a reduction of Na+, K+ - ATPase pump activity.3,5 A mutation has been identified in the gene coding for the α2 chain of type VIII collagen in patients with FED.3 FED is a common indication for keratoplasty and accounted for approximately 15% of all PKP performed in the US in 2000.3 Treatment, Management: Treatment specific to this patient: o This patient displays bilateral signs of FED with asymmetric progression. Due to advanced corneal disease, this patient was previously treated with PKP in the left eye. Signs and symptoms at our initial examination indicate stage 2 disease in the right eye. o Contact Lens: . OD: Dispense trial lens: Air Optix N&D Aqua (lotrafilcon A) +0.50 / BC: 8.4 / Dia: 13.8 Patient fit with high Dk lens for daily wear to maximize oxygen supply to anterior cornea and reduce the likelihood of corneal hypoxia secondary to contact lens wear. OS: Order trial lens: Jupiter Scleral (hexafocon A) -3.00 / BC: 7.03 / Dia: 17.4 OAD was reduced to 17.4mm to improve movement and OZD was increased to reduce peripheral bearing. Patient is scheduled for a follow-up visit once trial lens arrives for OS. o Glaucoma Suspect OD / Glaucoma OS : . Patient is currently being managed for this condition by referring ophthalmologist. Advised pt. to return to ophthalmologist for re-evaluation OD and IOP check OU. Recommended treatment: o Treatment of FED is most commonly supportive and focused on delaying the need for keratoplasty.3 o Supportive management includes topical use of hypertonic solutions to dehydrate the corneal stroma and decreasing ambient humidity.3 o Episodes of pain from bullae formation and rupture may be treated with hypertonics, bandage soft contacts, and anterior stromal puncture.3 o Keratoplasty is considered in cases that show inadequate treatment with supportive therapy. In patients over 60 years old with lens changes, keratoplasty should be done in conjunction with cataract extraction.3 . Two types of keratoplasty: PKP is the conventional method of corneal transplant which involves removing all layers of the central cornea and replacing them with healthy donor tissue.3 Posterior lamellar keratoplasty involves replacing only the diseased endothelium, Descemet’s membrane, and the deep stroma with healthy donor tissue. Advantages of this relatively new procedure over PKP include unaltered corneal topography, a faster recovery period, more predictable post-surgical refractive error, and an overall increase in the structural integrity of the globe.6 Recurrence of the disease in transplanted tissue is less than 10%.4 o Contact lens management . When fitting contacts, the primary consideration should be to maximize oxygen flow to the cornea.4 . In stage 1 and 2 disease, high Dk rigid gas permeable (RGP) or silicone hydrogel lenses are both viable options.4 . In stage 3, the formation of bullous keratopathy eliminates the viability rigid corneal contact lenses.4 . Treatment following full thickness corneal graft commonly requires large diameter corneal, mini-scleral, or scleral lenses. Conclusions . Clinical Pearls o FED does not necessarily exclude a patient from contact lens wear. o When managing a patient, it is important to consider the specific needs of each eye individually. o If a patient reports their quality of life has become adversely affected, consider surgical intervention. References 1. Shousha, M.A., Perez, V.L., Wang, J., Ide, T., Jiao, S., Chen, Q., Chang, V., et al. (2010). Use of Ultra-High- Resolution Optical Coherence Tomography to Detect In Vivo Characteristics of Descemet's Membrane in Fuchs' Dystrophy. Ophthalmology, 117 (6), pp. 1220-1227. 2. Berger, S.T., McDermott, M.L., Atluri, H.K.S. (2009). Fuchs’ Dystrophy. In: Ophthalmology (eds Yanoff, M., Duker, J), Mosby, St. Louis, MO., pp. 312-314 3. Adamis, A.P., Filatov, V., Tripathi, B.J., Tripathi, R.C. (1993). Fuchs' endothelial dystrophy of the cornea. Survey of Ophthalmology, 38 (2), pp. 149-168. 4. Bergmanson, J.P.G., Sheldon, T.M., Goosey, J.D. (1999). Fuchs’ endothelial dystrophy: a fresh look at an aging disease. Ophthalmic Physiol Opt. 19 (3), pp. 210-221. 5. Elhalis, H., Azizi, B., Jurkunas, U.V. (2010). Fuchs endothelial corneal dystrophy. Ocular Surface, 8 (4), pp. 173-184. 6. Mau, K. (2009). What DSAEK is going on? An alternative to penetrating keratoplasty for endothelial dysfunction. Optometry, 80, pp. 513-523 .
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