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Rare syndrome affecting bones and characterized by osteolysis; often associated with swelling or diffuse cystic angiomatous proliferation. Associated with massive resorption of bone matrix, often called the vanishing bone disease. Can affect only one bone or may have extension to nearby soft tissue and adjacent bones.

Diffuse Cystic Angiomatosis of Bone; Massive Gorham Osteolysis; Vanishing Bone Disease.

More than 175 cases have been reported in the literature. Both genders seem to be equally affected.

Autosomal dominant.

The monofocal osteolytic lesion may occur at any age, but is more common in the first three decades of life. While the lesion may affect any part of the skeleton, the skull, shoulders and the pelvis are the most frequently affected areas. Spontaneous fractures with absent fracture healing are common. The lesions may be painful to touch and temperature during the acute phase. Small intramedullary lesions of the bones later coalesce to form extensive areas of destruction with involvement of the cortical bone. The affected areas show sclerosis and significant proliferation of vessels (angiomatosis). Severe complications including death have been described, mainly resulting from spinal cord compression and severe chest wall involvement. Progressive deformations and contractures may occur. Laboratory investigations are usually normal. The disease seems to be the result of an increased sensitivity of osteoclast precursors to humoral factors, which promote osteoclast formation and activity and hence bone resorption. Biphosphonates have resulted in clinical improvement in some patients.

They are related to the affected area. If the lesion affects the cervical spine, careful intubation with inline stabilization of the spine is recommended and since neck movement is expected to be limited in these patients, fiberoptic intubation may be the technique of choice. Careful positioning and padding are required to avoid spontaneous fractures.

Succinylcholine should be used only after precurarization to avoid fasciulations that may result in spontaneous fractures.

Bruch-Gerharz D, Gerharz CD, Stege H, et al: Cutaneous vascular malformations in disappearing bone (Gorham-Stout) disease. JAMA 289:1479, 2003.  [PubMed: 12672746]
Hirayama T, Sabokbar A, Itonaga I, et al: Cellular and humoral mechanisms of osteoclast formation and bone resorption in Gorham-Stout disease. J Pathol 195:624, 2001.  [PubMed: 11745700]

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