Klippel-Trenaunay-Weber syndrome: oro-dental manifestations and management

Abstract

Background: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital systemic disease characterized by cutaneous haemangioma, arterio-venous fistulas or varicosities (or both) and unilateral hypertrophy of hard and soft tissue with different localizations. Typical orofacial manifestations include facial asymmetry, jaw enlargement and malocclusion. Case presentation: A previously diagnosed 55-year-old male patient was referred to the Department of Cranio-Maxillofacial Surgery presenting with extended mandibular ramus exostosis on the left side and concomitant malocclusion. Surgical removal of the hypertrophic bone was performed due to progressive mouth opening restriction, limited oral hygiene ability and increased mandible deviation. Bleeding complications and wound healing disorders were not observed. Consistent mouth opening training resulted in an improvement in mouth opening. Conclusion: It is suggested to consider KTWS as one of the differential diagnoses if vascular syndromes in the head and neck region occur. Special attention must be given to dental treatment due to eventual excessive haemorrhage that might occur after oral surgical procedures.