Limbal Stem Cell Deficiency: Demographics and Clinical Characteristics of a Large Retrospective Series at a Single Tertiary Referral Center.

PURPOSE: The aim of this study was to characterize a large cohort of patients presenting to a single referral center for limbal stem cell deficiency (LSCD). METHODS: A retrospective chart review of all patients with a clinical diagnosis of LSCD from 2002 to 2015 was performed. Demographics, etiology, previous ocular surgeries, visual acuity, and treatment were assessed. RESULTS: Seven hundred thirty-eight eyes of 434 patients (51.4% male subjects) were diagnosed with LSCD. The mean presenting age was 42.9 years, 70% presented with bilateral disease, and overall vision was poor. The most common etiologies were congenital aniridia (30.9%), chemical or thermal injuries (20.6%), contact lens (16.8%), Stevens-Johnson syndrome (SJS, 10.4%), and iatrogenic (7.3%). Congenital aniridia had a significantly increased association with glaucoma or ocular hypertension (P < 0.0001). Chemical or thermal injuries (P = 0.0007), SJS (P < 0.0001), and mucous membrane pemphigoid (P < 0.0001) had a significantly increased association with eyelid pathology. The mean logMAR best corrected visual acuity (analysis excluded eyes with light perception and no light perception) at presentation was 1.145 (∼20/280). Keratoplasty performed (before presentation at our center) without first addressing the LSCD was seen in 80 eyes; all keratoplasties failed due to recurrence of the LSCD. CONCLUSIONS: Patients presenting with LSCD were on average middle aged without sex predominance. LSCD was most commonly bilateral, comprised a wide range of etiologies, and decreased vision substantially. Ocular comorbidities may need to be treated before treating the LSCD surgically. Finally, not addressing the LSCD (primary issue) first can result in keratoplasty failure.

Donor-Derived Conjunctival-Limbal Melanoma After a Keratolimbal Allograft.

PURPOSE: To report a single case of donor-derived conjunctival-limbal melanoma that occurred after a keratolimbal allograft (KLAL). METHODS: Case report and literature review. RESULTS: A 56-year-old white woman with a history of bilateral limbal stem cell deficiency developed a donor-related melanoma after a KLAL. Three months after undergoing an uncomplicated KLAL, the patient presented with hemorrhagic nodules within her conjunctiva and transplanted tissue. Excisional biopsy was performed, and the pathology results revealed melanoma cells. Although the donor of the KLAL had a history of metastatic melanoma, the ocular tissue was in compliance with all eye bank requirements for donation. After discovery of the tumor, the patient's systemic immunosuppression was stopped. Within 1 week, the patient demonstrated a dramatic improvement in the size of the lesion. One month after the initial biopsy, the KLAL tissue was excised, and a pathology report revealed that there were no viable tumor cells on the ocular surface. As the limbal stem cell deficiency recurred, the eye underwent placement of a Boston type 1 keratoprosthesis. CONCLUSIONS: We present a case of conjunctival-limbal melanoma after a KLAL from a donor who had a history of metastatic melanoma. In response to this case, the US eye banking guidelines were amended to include stricter parameters for vascularized ocular tissue transplantation.

Outcomes of Keratoplasty After Ocular Surface Stem Cell Transplantation.

PURPOSE: To investigate the long-term outcomes of penetrating keratoplasty (PK) and deep anterior lamellar keratoplasty (DALK) after ocular surface stem cell transplantation (OSST). METHODS: An observational retrospective review was performed on all patients who underwent OSST followed by either PK or DALK with a minimum of 6-month follow-up. RESULTS: One hundred two eyes fulfilled the inclusion criteria. The mean time to keratoplasty after OSST was 19 months, and the mean follow-up was 57 months after keratoplasty. A Kaplan-Meier analysis demonstrated 92% graft survival at year 1, 77% at year 2, 62% at year 3, 55% at year 4, and 54% at year 5. During the study period, a total of 44 grafts failed. The mean time to graft failure was 32 months. Preoperative median best-corrected visual acuity for all eyes was 20/550, which improved to 20/100 (P < 0.001) at 1 year postoperatively, 20/60 (P < 0.001) at 2 years, and 20/100 (P < 0.001) at last follow-up. CONCLUSIONS: Without addressing the underlying stem cell deficiency, keratoplasty in patients with total limbal stem cell deficiency will ultimately fail in all cases. OSST with either subsequent PK or DALK provides successful visual outcomes with an acceptable complication profile.

Rate of Rejection After Descemet Stripping Automated Endothelial Keratoplasty in Fuchs Dystrophy: Three-Year Follow-up.

PURPOSE: To evaluate the rate of rejection after Descemet stripping automated endothelial keratoplasty (DSAEK) in patients with Fuchs endothelial dystrophy (ED) and to analyze potential risk factors for rejection. METHODS: An observational retrospective chart review of 400 patients with Fuchs ED was performed. The primary outcome was the rate of endothelial graft rejection. Secondary outcomes included graft clarity after rejection, medications at the time of rejection, and preoperative graft thickness. RESULTS: A total of 400 consecutive eyes with at least 1 year of follow-up were evaluated with an average follow-up of 38 months (range 12-95 months). The cumulative rate of rejection at 1 year was 1%; 2 years, 2.9%; and 3 years, 4.8%. The greatest number of rejection episodes occurred between postoperative months 19 and 30 (N = 7). There was a statistically significant association of developing a rejection episode when a patient was off of topical steroids (P = 0.001, odds ratio = 6.25, 95% confidence interval = 2.03-19.3) or had a donor graft thickness greater than 145 µm (P = 0.011, odds ratio = 5.03, 95% confidence interval, 1.28-23.1). CONCLUSIONS: Corneal graft rejection is an uncommon but important complication after DSAEK. This study reports a lower rejection rate in Fuchs ED than that reported in previous studies. Risk factors for developing a rejection episode include discontinuation of topical steroids and a graft thickness greater than 145 µm. Maintaining patients on indefinite topical corticosteroid therapy and using thinner donor tissue will likely result in an even lower rate of rejection.