PURPOSE: To report the frequency, outcome, and atypical histology in corneal dystrophies. METHODS: Corneal buttons of patients diagnosed with corneal dystrophy as noted in the records of the ophthalmic pathology register over a period of 6 years were included in this study. The sections from formalin-fixed, paraffin-embedded tissues were reviewed specifically for the type of deposits, associated degenerations such as amyloid and spheroidal deposits, inflammation, and vascularization. Special stains including Masson trichrome, Congo red, and Alcian blue staining were used whenever required. The medical records were evaluated for demographics, clinical presentation, history of consanguinity, family medical history, and clinical outcome of keratoplasty, which was recorded as clear, recurrence of dystrophy, or graft failure. A clinicopathologic correlation was attempted. RESULTS: A total of 144 patients contributed 181 buttons, accounting for 8.1% of keratoplasties performed during the study period. The mean age of the patients was 34 +/- 19 years (range 3-72 years) with a male:female ratio of 1.6 (89):1 (55). Consanguineous parentage was noted in 26% of cases. History of a similar problem in siblings and other family members was elicited in 33 (22%) and 14 (9.7%), respectively. Dystrophies included macular (29.3%), congenital hereditary endothelial dystrophy (34.8%), Fuchs (16.6%), and lattice (15%); the remaining 11% included granular, gelatinous drop-like, Reis-Bucklers, and posterior polymorphous dystrophy. Associated histologic changes were degenerations (15%), vascularization (4%), and inflammation (2%). At a mean follow-up of 42 months, the graft remained clear in 148 eyes (81.7%), failed in 33 eyes (18.2%), and recurred in 5 eyes (2.8%). Graft survival for all dystrophies at the end of 1 year was 94.3 +/- 1.7%, and at the end of 5 years was 74.4 +/- 4.5%. Atypical histologic features did not affect graft survival. CONCLUSION: Consanguineous marriages possibly contributed to the increase in macular dystrophy and CHED in South India. The degenerative changes seen could possibly be related to late presentation or unknown environmental factors and do not have an effect on the ultimate graft outcome.
PURPOSE: To report the frequency, outcome, and atypical histology in corneal dystrophies. METHODS: Corneal buttons of patients diagnosed with corneal dystrophy as noted in the records of the ophthalmic pathology register over a period of 6 years were included in this study. The sections from formalin-fixed, paraffin-embedded tissues were reviewed specifically for the type of deposits, associated degenerations such as amyloid and spheroidal deposits, inflammation, and vascularization. Special stains including Masson trichrome, Congo red, and Alcian blue staining were used whenever required. The medical records were evaluated for demographics, clinical presentation, history of consanguinity, family medical history, and clinical outcome of keratoplasty, which was recorded as clear, recurrence of dystrophy, or graft failure. A clinicopathologic correlation was attempted. RESULTS: A total of 144 patients contributed 181 buttons, accounting for 8.1% of keratoplasties performed during the study period. The mean age of the patients was 34 +/- 19 years (range 3-72 years) with a male:female ratio of 1.6 (89):1 (55). Consanguineous parentage was noted in 26% of cases. History of a similar problem in siblings and other family members was elicited in 33 (22%) and 14 (9.7%), respectively. Dystrophies included macular (29.3%), congenital hereditary endothelial dystrophy (34.8%), Fuchs (16.6%), and lattice (15%); the remaining 11% included granular, gelatinous drop-like, Reis-Bucklers, and posterior polymorphous dystrophy. Associated histologic changes were degenerations (15%), vascularization (4%), and inflammation (2%). At a mean follow-up of 42 months, the graft remained clear in 148 eyes (81.7%), failed in 33 eyes (18.2%), and recurred in 5 eyes (2.8%). Graft survival for all dystrophies at the end of 1 year was 94.3 +/- 1.7%, and at the end of 5 years was 74.4 +/- 4.5%. Atypical histologic features did not affect graft survival. CONCLUSION: Consanguineous marriages possibly contributed to the increase in macular dystrophy and CHED in South India. The degenerative changes seen could possibly be related to late presentation or unknown environmental factors and do not have an effect on the ultimate graft outcome.
Authors: Maryam Ali; VijayKrishna Raghunathan; Jennifer Y Li; Christopher J Murphy; Sara M Thomasy Journal: Exp Eye Res Date: 2016-09-14 Impact factor: 3.467