J Korean Orthop Assoc.  1990 Dec;25(6):1793-1798. 10.4055/jkoa.1990.25.6.1793.

Gorham's Syndrom: A Case Report

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.

Keyword

Gorham's syndrome; Massive osteolysis; Lymphangioma

MeSH Terms

Bone Matrix
Dissent and Disputes
Female
Femur
Fibula
Fractures, Spontaneous
Humans
Korea
Lymphangioma
Neck
Osteolysis
Osteolysis, Essential
Pelvis
Tibia
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