ANTERIOR-CHAMBER DYSGENESIS WITH BILATERA L CORNEAL ECTASIA

Background Sclerocornea is a congenital, uni- or bilateral, non-progre ssive, non-inflammatory malformation characterized by histological cha nges of the cornea resembling scleral tissue. Case Report We report th e case of a 2-month-old child with bilateral dysplasia of the anterior segment including: 1. extreme corneal ectasia; 2. vascularized, opaci fied microcornea; 3. presence of rudimental limbus; 4. anterior sclera l dysplasia. The axial length measured by means of ultrasonography (A- scan) was about 24 mm in both eyes. A penetrating keratoplasty was fir st performed in the right eye and one week later in the left eye. Intr aoperatively an advanced iris atrophy with extensive anterior synechia e, as well as a localized anterior subcapsular cataract were seen bila terally. A pale optic disk with a maximally enlarged optic cup could b e seen only in the left eye. Conclusion Although the histologic examin ation was compatible with the diagnosis of sclerocornea, the presence of atypical clinical findings previously unreported does not allow a d efinite classification of the case reported herein.