Biometric and magnetic resonance imaging assessment of dentofacial abnormalities in a case of Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome is characterized by cutaneous hemangioma, arteriovenous fistulas or varicosities (or both), and unilateral hypertrophy of hard and soft tissues with different localization. Klippel-Trenaunay-Weber syndrome represents a syndrome of interest for those in the fields of oral and maxillofacial radiology and pathology because of the high incidence of compromise of the orofacial area in affected patients. The patient reported here presents with all of the 3 characteristic signs of the syndrome. The somatic and facial hypertrophy is contralateral to angiomata. As revealed through the use of magnetic resonance imaging, the primary component of facial hypertrophy is adipic subcutaneous tissue. Statistical analysis of the dental dimensions revealed that teeth on the hypertrophic side are significantly larger than normal.