Abstract: Gorham disease is a rare syndrome, which is considered as type Ⅳ osteolysis in hardegger classification. The etiology and pathogenesis are unclear. The orthopaedic clinical features of Gorham disease depend on the affected bones. It is characterized by massive osteolysis affecting one or more bones and replaced by bone lymphatic vessels, which may be life-threatening. The diagnosis of the disease is very challenging and is an exclusive diagnosis. In addition, imaging examination is also a necessary condition for diagnosis. The disease is mainly treated by comprehensive treatment, sirolimus can inhibit cell proliferation and angiogenesis; bisphosphonates has anti-osteolytic activity and inhibits osteolysis caused by osteoclasts; radiotherapy may prevent the progression of osteolysis by inhibiting the proliferation of endothelial cells; surgery is mainly used to treat complications. Because the prognosis of chylothorax and other complications is often poor, further research is very necessary to obtain more in-depth insights into the disease and guide the most appropriate clinical treatment strategy.

Key words: Gorham disease, clinical manifestation, diagnosis, treatment