血管医学および外科ジャーナル

概要

Klippel Trenaunay Syndrome in a 5 Year Old Boy: A Rare Embryological Vascular Anomaly

Heena Singdia

We present a case history of 5 years old boy, born of a non-consanguineous marriage with uneventful vaginal delivery brought to OPD with complaints of limb length discrepancy where the length and girth of right leg was greater than the left leg, dilated veins over right inguinal area and port wine stain over right half of the body for 4 years, the condition progresses with progressing age of child. There was no history of ulceration, Raynauds phenomenon, paresthesia, difficulty in walking or pedal edema. Baseline investigations, Color Doppler, and X-ray lower limb were normal. Klippel trenaunay syndrome is a vascular malformation disorder comprising a triad of port wine stain, limb hypertrophy and varicosities with a global incidence of 5 cases/100000.