Abstract

Introduction: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a clinical triad of capillary malformation; varicose veins; and soft tissue and/or bony hypertrophy. This rare syndrome has an estimated incidence of about 2–5/100,000 live births. KTS usually affects the extremities but occasionally can manifest in the craniofacial region, including the oral cavity. Case Presentation: Our patient, a 17-year-old girl, presented with large areas of port wine stain, unilateral hypertrophy of the facial region, and varicose veins. She was diagnosed with KTS. Orthodontic treatment followed by orthognathic surgery was performed to address her complaint of facial asymmetry and poor chewing efficacy. Treatment resulted in significant improvement in her appearance and dental occlusion and our patient was very satisfied with the results. Conclusion: This report illustrates an orthodontic and surgical treatment approach of a case with KTS. Effective management of orthodontic and facial manifestations of KTS considerably enhances the patient’s function, appearance, and overall quality of life.

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