Abstract:
PURPOSE:To describe the histopathology of the cornea in microphthalmia with linear streaks (MLS) syndrome. METHODS:Two patients with MLS syndrome underwent penetrating keratoplasty. This study describes the histopathology and investigates immunophenotype of the corneal extracellular matrix by using keratan sulfate and collagen type III antibodies. RESULTS:Clinical examination revealed bilateral sclerocornea and characteristic skin changes. By light microscopy, central corneal stroma in both patients showed vascularization and irregular thick collagen lamellae typical of sclerocornea. In addition, corneal thinning, anterior synechiae, and the absence of the Descemet membrane were noted, which was suggestive of Peters anomaly. Diffuse and intense anti-keratan sulfate staining and minimal anti-collagen type III stromal staining were seen in both corneal buttons. CONCLUSIONS:The cornea in MLS may clinically resemble sclerocornea. Histologic features resemble those previously described in sclerocornea and also seen in anterior segment dysgeneses. Keratan sulfate and collagen type III labeling suggests that the corneal extracellular matrix resembled cornea and not sclera.
journal_name
Corneajournal_title
Corneaauthors
Kapur R,Tu EY,Toyran S,Shah P,Vangveeravong S,Lloyd WC 3rd,Edward DPdoi
10.1097/01.ico.0000611352.03660.a7subject
Has Abstractpub_date
2008-07-01 00:00:00pages
734-8issue
6eissn
0277-3740issn
1536-4798pii
00003226-200807000-00022journal_volume
27pub_type
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