DR. SWETA SINGH

Abstract

Klippel-Trenaunay Syndrome (KTS) is a rare mesodermal phakomatosis with a triad of irregular and asymmetrical capillary port-wine stain and cavernous hemangioma on the trunk or limbs, venous varicosities, and asymmetrical hypertrophy of bone or soft tissue . Sturge-Weber syndrome (SWS) and KTS have overlap in many of the diagnostic criteria ,but there are important differences among these diseases that carry important prognostic and therapeutic implications.We present a case of KTS in 10 year female who had macrocephaly , naevus flammeus on whole face but more on left side , hypertrophied right limb with non healing ulcer, OU Buphthalmos , multiple tram track calcification in left frontal and occipitoparietal cortex with brain atrophy, ipsilateral choroid plexus enlargement with calcification and calvarial thickening.