I. Case History

 45 year old Caucasian male  Referral to the clinic for scleral fitting of the right eye after tectonic lamellar keratoplasty for corneal perforation secondary to peripheral ulcerative  Ocular history: Peripheral Ulcerative Keratitis, OU; Suspect, OU; Post-corneal transplant, OD; Irregular , OU.  Medical History: Depression, former smoker  Medications: Latanoprost 0.005% QD OU; Timolol Maleate 0.5% BID OU; Prednisolone Acetate 1% QD OU

II. Pertinent findings

 Visual Acuities (VA) with correction: OD 20/300-; OS 20/40.  Refraction: o OD: +1.25 -1.25 x 015 VA: 20/300 o OS: +3.00 -0.50 x 010 VA: 20/20-  Intraocular Pressures (IOP) o OD: 14 mmHg o OS: 28 mmHg . With compliant latanoprost and timolol treatment  Anterior Segment: o OD: . Nasal pinguecula and 1+ conjunctival injection . Inferior tectonic lamellar graft from 3-9 o’clock arcing inferior to the with sutures intact temporal and nasal, significant stromal scarring . Diffuse pigment on endothelium o OS: . Nasal pinguecula and 1+ conjunctival injection . s/p temporal removal . Stromal scarring and peripheral thinning inferior

 Topography via Pentacam o OD: K’s 35.6/[email protected] . 16.4 Diopters of Irregular Corneal Astigmatism o OD HVID: 11.9 mm

III. Differential diagnosis

 Terrian’s Marginal Degeneration o Presents as bilateral marginal furrow o Non-ulcerative peripheral thinning beginning superiorly and progressing o (-) epithelial defect, pain, discomfort, redness, inflammation o Typically begins superior  Pellucid Marginal Degeneration o Typically inferior non-ulcerative peripheral thinning o (-) epithelial defect, pain, discomfort, redness, inflammation o “crab claw” or “kissing dove” corneal topography

IV. Diagnosis and discussion

 Peripheral Ulcerative Keratitis (PUK) o Characterized by crescent shaped lesion of the juxtalimbal corneal stroma. o Lesion typically shows stromal thinning and inflammation with an overlying epithelial defect. o Incidence of 3 cases per million per year. o Typically present with pain, redness, tearing, , and decreased vision. o Progressive thinning may lead to corneal perforation. o The tendency for peripheral location is due to characteristics of the limbal , which provides access for circulating immune complexes to the peripheral cornea via the capillary network.1 o Typically associated with autoimmune conditions in 50% of cases such as rheumatoid arthritis, Systemic Lupus Erythematosus (SLE), Wegener’s Granulomatosus, etc.1 o Mooren’s Ulcer – PUK with unknown etiology and no related autoimmune condition underlying the ulceration  Tectonic Lamellar Keratoplasty o A tectonic graft only replaces the area of cornea that is damaged o Lamellar keratoplasty removes the corneal stroma down to Descemet’s membrane and replaces it with tissues from a donor cornea, leaving the host endothelium intact. o Lamellar keratoplasty is advantageous over penetrating keratoplasty (PKP) in cases of PUK o More host tissue and increase corneal thickness to help prevent repeat corneal perforations.2 o PKP has increased rejection rate due to inflamed limbal and conjunctival blood vessels surrounding area of ulceration1

V. Treatment, management

 The patient was fit with ZenLens Oblate Design OD o Power: +1.00D o Base Curve: 7.9 mm o Diameter: 17.0 mm o VA: 20/20 o Central vault approximately 250 um, full limbal clearance 360, aligned edge  Fit with Air Optix Aqua, OS o Patient’s habitual lens, did not want a scleral lenses OU o Power: +3.00D o VA: 20/20-  Patient was extremely satisfied with the comfort of his scleral lens and improved vision from 20/300 to 20/20.  Lens was evaluated and found to be acceptable at follow up appointments.  Continue Latanoprost 0.005% QD, Timolol Maleate 0.5% BID, and Prednisolone Acetate 1% QD.  Follow up 3 months for IOP check and visual fields for glaucoma suspect.  Return to corneal specialty annually as directed for post-corneal transplant care.  (1) Yagci, A. (2012). Update on peripheral ulcerative keratitis. Clinical (Auckland, N.Z.), 6, 747–754. http://doi.org/10.2147/OPTH.S24947  (2) Bessant, D. "LAMELLAR KERATOPLASTY IN THE MANAGEMENT OF INFLAMMATORY CORNEAL ULCERATION AND PERFORATION." Eye 8 (1994): 22-28. Web.

VI. Conclusion

 When fitting a scleral lens over a corneal transplant, fitting with an oblate scleral lens design will provide the best outcome  Utilizing corneal and scleral topography to map corneal astigmatism, K’s, HVID, and scleral toricity help to improve scleral lens fittings.  Maintain an open communication with the corneal surgeon to provide optimal patient care for post-transplant patients.