척추측만증을 동반한 Klippel-Treauary-Weber 증후군

Abstract

Klippel-Trenaunay-Weber syndrome is a rare congenital anomaly, characterized by triad of hemihypertrophy, multiple cutaneous hemangioma and arteriovenous malformation. However there can be many other manifestations like polydactly, atypical venous distributions, invasion of hemangioma into the internal organ, etc. We report a 7 year old female, known as Klippel-Trenaunay-Weber syndrome. She had a big soft mass on her entire back since her birth. She had vertebral scoliosis with pelvic tilting and leg length discrepancy. Due to a big lipoma of her back, neither bracing nor surgical intervention about scoliosis were possible. We applied functional foot orthosis for conservative management of scoliosis. She showed remarkable improvement at angle of scoliosis.