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Complex anterior chamber dysgenesis with bilateral corneal ectasia
Authors:M Busin  D Meller  A Cusumano
Affiliation:Universit?ts-Augenklinik Bonn.
Abstract:BACKGROUND: Sclerocornea is a congenital, uni- or bilateral, non-progressive, non-inflammatory malformation characterized by histological changes of the cornea resembling scleral tissue. CASE REPORT: We report the case of a 2-month-old child with bilateral dysplasia of the anterior segment including: 1. extreme corneal ectasia; 2. vascularized, opacified microcornea; 3. presence of rudimental limbus; 4. anterior scleral dysplasia. The axial length measured by means of ultrasonography (A-scan) was about 24 mm in both eyes. A penetrating keratoplasty was first performed in the right eye and one week later in the left eye. Intraoperatively an advanced iris atrophy with extensive anterior synechiae, as well as a localized anterior subcapsular cataract were seen bilaterally. A pale optic disk with a maximally enlarged optic cup could be seen only in the left eye. CONCLUSION: Although the histologic examination was compatible with the diagnosis of sclerocornea, the presence of atypical clinical findings previously unreported does not allow a definite classification of the case reported herein.
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