Gorham's Syndrom: A Case Report / 대한정형외과학회잡지

ABSTRACT

Gorham's syndrome (Gorham's disease, Massive osteolysis, Disappearing bone disease, phantom bone) is a rare disorder characterized by histologically benign proliferation of thin-walled vascular channels originating in bone associated with extensive lysis of regional bone matrix. It starts in bone, but it may secondarily involve soft tissues and adjacent bones. Although the osteolysis usually arrests spontaneously, its biologic behavior is unpredictable. The etiolgy is still in dispute and the treatment presents many problems. The author's case was a 20-year old girl, who had pathologic fracture in neck and supracondylar area of left femur with osteolysis of left pelvis, femur, and proximal fibula and tibia. She also had lymphangioma involving bones and adjacent soft tissues. To our knowledge, there is no report on Gorham's syndrome in Korea. The authors report a case of Gorham's syndrome with review of literatures.