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

Daume, L; Renz, C; Trenot, G; Kleinheinz, J

Abstract

Introduction: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital systemic disease characterized by a classic typical triad: cutaneous haemangioma, arterio-venous fistulas or varicosities (or both) and unilateral hypertrophy of hard and soft tissue with different localizations. First described by the French physicians Marcel Klippel and Paul Trenaunay, in 1900, KTWS has a clinically incidence of 2-5/100000. The complete triad (port-wine stains, varicose veins, and soft tissue and/or bony hypertrophy) occurs in almost 2/3 of the patients. Moreover, these features may be present at birth or develops during growing becoming more evident with age. 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. Clinical discussion and 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.