Unusual distribution of enamel hypoplasia in an 11-year-old child with Proteus syndrome.

An 11-year-old girl with Proteus syndrome attended our dental department because her teeth were sensitive to cold stimuli and because of the poor appearance of her anterior teeth. Extraoral examination revealed several of the previously reported craniofacial features of Proteus syndrome: facial and skull asymmetry, exostoses of the nasal bridge, and mandibular prognathism. Intraoral examination revealed enamel hypoplasia of primary and permanent teeth. The distribution of hypoplasia was unusual in that only teeth on the right side of the mouth were affected, and also in that only the distal half of the crown of the maxillary right permanent central incisor was affected. Behavioural problems necessitated treatment under general anaesthesia; the affected primary teeth were extracted and the hypoplastic permanent teeth were restored, relieving the patient's symptoms and greatly improving aesthetics.