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.
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
Hirayama T, Sabokbar A, Itonaga I, et al: Cellular and humoral mechanisms of
osteoclast formation and bone resorption in Gorham-Stout disease. J Pathol