Sclero-cornea and defective mesodermal migration

Abstract

A 5-month-old boy with bilateral sclero-cornea, multiple systemic abnormalities, and normal karyotype had his left eye removed after corneal perforation. Histopathological examination of the enucleated eye revealed an irregular corneal epithelium, absent Bowman's membrane, thickened and vascularized stromal lamellae, absent Descemet's membrane and endothelium, no angle structures, hypoplasia of the iris and ciliary body, and total kerato-iridic adhesions. A profound defect in embryogenesis due to defective mesodermal migration during the earliest phase of mesoderm differentiation is postulated as the causative factor in this case.