Proteus Syndrome: A Rare Entity

Abstract

ABSTRACT The Proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report, we describe a case of an 18-year-old male patient with Proteus syndrome, who had pronounced hemifacial hypertrophy, exostoses in the left parietal region, and enlargement of the inferior alveolar nerve and mandibular canal in the affected region. The dental development of the affected left mandible and maxilla was characterized by extremely premature development and eruption of the permanent teeth. The proposed multidisciplinary management of the patient and a review of literature of the Proteus patients, who exhibited manifestation in the craniofacial region is discussed in this report.