Klippel Trenaunay syndrome: An illustrative report of a rare case

Abstract

Klippel Trenaunay syndrome is a triad of congenital anomalies characterized by nevus flammeus, varicosities, and unilateral bony and soft tissue hypertrophy. This syndrome was first reported in 1900 by Klippel and Trenaunay. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Some authors have reported lesions resembling pyogenic granulomas to be a consistent feature of KTS. Presented here is an illustrative report of Klippel Trenaunay syndrome in an 18 year old female patient showing all the characteristic findings. Interestingly, increased appearance of nutrient canals in the affected maxillary quadrant is seen in our case which seems to be reported for the first time in the literature.