The clinical profile and the frequency of each dystrophy is given in Table 1. The predominant presenting symptom was decreased vision in 51 (43.22%) eyes, followed by pain in 23 (19.49%), photophobia in 11 (9.32%), and redness in 9 (7.62%) eyes. History of a similar problem in siblings and other members was elicited in 27 (22.88%) and 15 (12.71%) patients, respectively. History of consanguineous marriage was noted in 62% of the study population. During this time period we had 118 patients who were included in the study. Our study period was from January 2017 to December 2019.
We present here a series of corneal dystrophy cases that presented to a tertiary care centre in Bangalore. The observation of atypical presentations, inflammation, and associated spheroidal degeneration was the catalyst for us to review the frequency, distribution, and the outcome of keratoplasty in corneal dystrophies at a tertiary eye care centre in South India. The frequency of dystrophies requiring penetrating keratoplasty in India is 8.4%, 4 and in other series it ranges from 4% to 24%. 1, 3 The phenotypic description, based on clinical and histological findings, provides the most omnibus clinical picture.
But recent studies of genetics of corneal dystrophies have changed the concept of dystrophies. The dystrophies have been classically classified according to the primary layer of involvement into anterior membrane dystrophies (epithelium, epithelial basement membrane, and Bowman layer dystrophies), stromal dystrophies, and endothelial dystrophies (endothelium and Descemet membrane). Some guidelines for a clinical diagnosis of a corneal dystrophy are described, but there are exceptions to these characteristics, such as unilateral presentation in lattice dystrophy, delayed presentation in Fuchs dystrophy and vascularization in gelatinous drop-like keratopathy.
They are generally early onset, axially symmetric, slowly progressive or stationary, free from vascularization, and not usually associated with other systemic conditions. 1 The American Academy of Ophthalmology defines corneal dystrophies as "a group of inherited corneal diseases that are typically bilateral, symmetric, slowly progressive and without relationship to environmental or systemic factors." 2 Also, there is an increased need for thorough work-up before planning intraocular procedures.Ĭorneal dystrophies are defined as primary, inherited, bilateral, heredofamilial disorders of the cornea affecting transparency, leading to varying degrees of visual disturbances. Among these cases, only 2 groups had graft failure, viz, CHED and macular dystrophy.Ĭonclusion: The higher frequency of dystrophies could be due to higher consanguineous marriage, which indicates a need for counselling. The average follow-up of each keratoplasty case was 36☑4 months. Results: During this time period we had 118 patients who were included in the study. Photographic documentation was done for all dystrophy patients. Complete ophthalmological examination was conducted for all patients which included vision (distance & near), slit lamp examination, retinoscopy, auto refractometer readings, dilated fundus examination, pentacam as well as anterior segment OCT.
All patients underwent a thorough evaluation for demographics, clinical presentation, history of consanguinity, family medical history, and clinical outcome of keratoplasty (in those who required it) and a clinicopathologic correlation was attempted. Materials and Methods: All patients who presented to the out-patient department were examined for signs of corneal dystrophy from January 2017 to December 2019. Purpose: To report the frequency of corneal dystrophies.