Figure 4

Radiographic and oro-dental features of Family 2. CT scanning identified (A) intracranial calcifications and (B) probably vascular calcifications in patient VI-1. Radiographs show (C) undermineralised long bones, (D) carpal bones and phalanges (E) and a mild radius bowing in patient VI-2. (F) Dental radiograph showing an absence of density differences between enamel and dentin, incomplete root formation and enlarged pulp chambers. Apical radiolucencies associated with permanent teeth are also present. (G & H) Affected family members presented permanent erupted teeth with yellow discoloration, hypomineralised and hypoplastic enamel. Severe delays in permanent tooth eruption were observed in both siblings.