Adams-Olıver Syndrome: a Case Report

Abstract

Summary Background: Adams-Oliver Syndrome has been described by Adams and Oliver in 1945. Original definition, along with aplasia cutis congenital syndrome and limb defects, has neurological and cardiological problems. In the first description, genetic defect passes variable autosomal dominant pattern. Afterwards the autosomal recessive and sporadic cases were published. Case Report: 11-year old male patient complained of mobile teeth admitted to our clinic. He was noted to have characteristic view with distal-phalanx and nail hypoplasia of his hand and feet with occipital scalp defect. We consulted to genetics because of these findings and learned that he has the Adams-Oliver Syndrome. The patient has some orofacial manifestations including high-narrow palate, fissured tongue, crowding, dysmorphic facial features, facial asymmetry, deep-philtrum, delayed eruption, class III malocclusion. The extraction of mobile deciduous teeth, restoration of caries and also oral hygiene motivation was made. Then, the patient was referred to orthodontics. Conclusions: Adams-Oliver syndrome represents are rare congenital alteration, insufficiently documented in scientific literature. This shows the need to document news cases.