Gorham's disease

(also called Gorham's osteolysis, and vanishing bone disease), rare disease of unknown aetiology. Pathologically, there is benign proliferation of vascular channels within bone resulting in osteolysis. The vascular channels, which may be blood or lymphatic, have a single layer of endothelium. Any bone may be affected; the commonest sites are shoulder and pelvic girdles. The disease is virtually always monocentric. Symptoms are variable; weakness, pain or difficulty in walking are the commonest.

Plain films demonstrate progressive, relentless bone destruction without periosteal reaction. In the early stages there are intramedullary and subcortical lucencies resembling osteoporosis. As bone destruction and resorption progress the bone develops a "licked stick of candy" or rotting tooth appearance (Fig.1) with a tapered end. Later still the bone may disappear completely. Radionuclide bone scans demonstrate normal uptake throughout the skeleton. CT and MRI can demonstrate the soft tissue component of the disease. There is usually low signal on T1-weighted images, high signal on T2-weighted images and enhancement after intravenous contrast. Although histologically benign the disease may extend to involve adjacent bones, joints and soft tissues. The disease may arrest spontaneously with fibrous replacement of the vascular component. Complications are variable ranging from minimal disability to death. Pathological fractures, limb shortening or bowing and scoliosis can occur. Involvement of the spine or thorax can result in severe neurologic or pulmonary complications.

JC