Anterior chamber dysgenesis with bilateral corneal ectasia [Komplexe Vorderkammerdysgenesie mit Bilateraler Hornhautektasie]

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.