Case report: Hypodontia and short roots in a child with Fraser syndrome

Abstract

Fraser syndrome is a rare autosomal recessive disorder of which there has only previously been one case reported in the dental literature. The main characteristics are cryptophthalmos, syndactyly and genital abnormalities. Orofacial findings reported are: facial asymmetry, cleft lip and palate, high arched palate, dental crowding, fusion of primary teeth, dental hypoplasia, malocclusion, and supragingival calculus. A 15 year old girl with Fraser syndrome attended Bradford and Airedale salaried dental services complaining of painful mandibular anterior teeth. On examination she presented with hypodontia, shortened roots, and the mandibular anteriors had a titanium trauma splint fixed to reduce the mobility. This had been placed 4 years previously by a paediatric specialist. However oral hygiene was poor around it and therefore the patient had calculus and gingivitis. The splint was removed followed by subgingival scaling under local analgesia, fissure sealants of all posterior teeth, regular oral hygiene instruction and scaling, and occasional use of chlorhexidine gel. She has been reviewed regularly with frequent scalings over two years. This case reports the possibility of hypodontia and short roots being associated with Fraser syndrome.