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Clinical and ultrastructural feature of a cornea with pellucid marginal degeneration
Author(s) -
AKHTAR S,
KIRAT O,
KATAN H,
ALMUBRAD T
Publication year - 2011
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2011.413.x
Subject(s) - cornea , hemidesmosome , ultrastructure , keratoconus , collagen fibril , ectasia , stroma , medicine , ophthalmology , pinhole (optics) , anatomy , pathology , optics , immunohistochemistry , physics
Purpose Pellucid marginal degeneration (PMD) of the cornea is a rare ectatic disorder which typically affects the inferior peripheral cornea in a crescentic fashion. We report clinical, histological and ultrastructural PMD cornea. Methods A 57 year old female was diagnosed with PMD and keratoconus in the right eye, and PMD in the left eye. Uncorrected vision was: OD 5/200 with pinhole 20/400; OS 20/100 with the pinhole to 20/70. The patient underwent lamellar keratoplasty in the right eye and the excised cornea was processed for light and electron microscopiy. Results Four months post‐operatively uncorrected vision in the right eye was 20/160 improving with the pinhole to 20/60. Ultrastructure of the peripheral and central parts of the cornea was similar to each other. The epithelium was irregular and most of the basal epithelial cells were vacuolated. Hemidesmosomes were broken at various places. Bowman’s layer was degenerated and absent or replaced by collagenous panus. Lamellae in the anterior and middle stroma were thin and undulating. Numerous microfilaments were aggregated at the inter‐lamellar junction. The collagen fibrils (CF) were running in random directions in the anterior and middle stroma. Conclusion Our observation of disorganisation and degeneration of CF suggestes that PMD could be related to malfunctioning in the synthesis of CF due to a disorder in keratocan, a luecine rich proteoglycan. Cross linking treatment should be considered for treatment in early stages of the disease.