Gorham’s disease

Gorham’s disease also known as Gorham vanishing bone disease and phantom bone disease,is a very rare skeletal condition of uncertain etiology, characterized by the uncontrolled proliferation of distended, thin-walled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas and/or fibrosis.

In 1983 Heffez and colleaguespublished a case report in which they suggested eight criteria for a definitive diagnosis of Gorham’s disease:

In the early stages of the disease x-rays reveal changes resembling patchy osteoporosis. As the disease progresses bone deformity occurs with further loss of bone mass and, in the tubular bones (the long bones of the arms and legs), a concentric shrinkage is often seen which has been described as having a “sucked candy” appearance. Once the cortex (the outer shell) of the bone has been disrupted, vascular channels may invade adjacent soft tissues and joints. Eventually, complete or near-complete resorption of the bone occurs and may extend to adjacent bones, though spontaneous arrest of bone loss has been reported on occasion. Throughout this process, as the bone is destroyed it is replaced by angiomatous and/or fibrous tissue.

Dr. Tushar Mehta

MBBS , DNB (Ortho), MNAMS, PGDCR, http://www.facebook.com/orthopaedicsplus.in