Oral Medicine| Volume 93, ISSUE 3, P276-280, March 2002

Klippel-Trénaunay syndrome (angio osteohypertrophy syndrome): A report of 3 cases


      Klippel-Trénaunay syndrome was first reported in 1900 by Klippel and Trénaunay. The syndrome is characterized by the triad of vascular nevi, venous varicosity, and hyperplasia of soft tissue—and possibly bone—in the affected area. The original description was made with regard to the extremities. The syndrome is diagnosed on the basis of 2 or 3 symptoms. The 3 patients reported here presented with hemangioma and hypertrophy of the jawbone and concomitant malocclusion. There were clinically no obvious varicose veins. It appears that gravity plays an important role in the venous drainage from the head and neck region, and this could be the reason for the rarity of varicose veins in the head and neck region when compared with lower extremities. In addition, 2 of the 3 cases showed exophytic pedunculated growth arising from the gingiva, which has not been reported in any previous case reports. The following report presents the clinical features in the orofacial region and highlights the clinical significance of this syndrome. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:276-80)
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