Also known as the vanishing bone disease, ghost bone, for the first time by Gorham of the system description, definition, so it is also call
Also known as the vanishing bone disease, ghost bone, for the first time by Gorham of the system description, definition, so it is also called Gorham disease. Clinical is extremely rare, the disease has a certain self limited. Etiology: it is not clear about the specific causes and pathogenesis of the disease. Preliminary studies suggest that with the following factors: 1, hemangioma, lymphangioma doctrine is the doctrine that hemangioma and lymphangioma in bone growth, leading to bone destruction and absorption, part of the pathology confirmed the presence of vascular tumor; 2, metabolic disorder theory that is local generation of Xie Yichang, leading to bone destruction, the absorption, but the lack of laboratory evidence. Pathology: mainly osteolysis, including bone atrophy, little Liang Xishu, disappeared, local bone resorption by fibrous connective tissue, hyperplasia of capillaries, the blood sinus Jicheng plexus vascular replacement, and vascular dilation significantly. Vascular endothelial cell growth is not active, no cell atypia. There were no lymphocytes and other inflammatory cells infiltration, no periosteal reaction, no new bone formation. Clinical manifestations: the disease is more common in children and young people, the body can appear in multiple bones, and the following jaw, ribs, femur and other most common. In the early stage of the disease, there is no obvious clinical symptoms. With the development of the disease, the local osteolysis is obvious, and local pain and tenderness may appear. Imaging findings: mainly X examination. The change of local osteoporosis bone, irregular radiolucent area, with the progression of the disease, increase bone radiolucent area, bone destruction, absorption, but no local periosteal reaction, no new bone formation, with pathological fracture change. Diagnosis and differential diagnosis: according to the patient's medical history, signs, combined with special X-ray examination can be clear. But the need to identify with the following diseases: 1, simple changes in systemic bone osteoma, patients without systemic symptoms, local to pathological fracture, X-ray examination showed a cystic destruction, a boundary, cortical bone integrity, a periosteal reaction can be identified. 2, malignant tumor not only local bone destruction, soft tissue swelling, X-ray examination showed periosteal reaction, new bone formation, pathological diagnosis. Treatment and prognosis: as the disease has a certain self limiting, for those who are not obvious symptoms can be observed and treated, pay attention to protection, to prevent the occurrence of pathological fractures. Some scholars have put forward local radiotherapy, but the effect is not good; other hormone treatment, calcium treatment, A-2b interferon and treatment of Gorham disease. For long bone obvious bone resorption, some scholars with surgical curettage, bone grafting and bone resection, artificial limbs, but there is an obvious recurrence.