Images in Clinical Practice Indian Pediatrics 2005; 42:277 |
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Sclerocornea |
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Sclerocornea, an uncommon developmental abnormality of anterior segment due to mesenchymal dysgenesis presents as a stationary congenital anomaly. It is usually seen as an isolated ocular abnormality involving both eyes. Occurs sporadically but may be familial or autosomal dominant in inheritance. Clinically, most often there is peripheral, white, vascularised 1-2 mm cornea1 rim that blends with sclera obliterating the limbus. The central cornea is generally normal. In total sclerocornea, the entire cornea is involved but the center of the cornea is clearer than periphery, which distinguish it from. Peters’ anomaly where opacity is more at center. The opacification affects the full thickness stroma that limits the visualisation of posterior corneal surface and intraoccular structures. Histology includes disorganised collagenous tissue containing fibrils that is larger than normal. Potentially co-existing abnormalities include shallow anterior chamber, iris abnormalities and microphthalmos. Systemic abnormalities like limb deformities, craniofacial and genitourinary defects can also accompany. In generalised sclerocornea early keratoplasty should be considered in an effort to provide vision. Y. Ramesh Bhat, |