Gorham syndrome

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[edit] Discussion of Gorham syndrome

  • Gorham syndrome is also known as Gorham-Stout syndrome, massive osteolysis, idiopathic osteolysis, disappearing bone disease, phantom bone disease, vanishing bone disease, spontaneous absorption of bone, progressive atrophy of bone, or hemangiomatosis or lymphangiomatosis of bone.
  • Gorham syndrome is a rare disorder with approx 200 reported cases.
  • This disease may occur at any age; however, it is most often recognized in children and young adults.
  • There is no sex predilection or inheritance pattern.
  • 57% of patients have a history of trauma.

  • Massive and progressive osteolysis is caused by the proliferation of abnormal thin-walled endothelial-lined capillaries of vascular or lymphatic origin.
  • The mechanism of bone resorption is unknown.
  • The skeletal distribution of the disease has been widespread, but the upper arm or shoulder girdle (26%) and mandible (15%) have been favored sites.
  • Skeletal involvement is virtually always monocentric, and involvement of adjacent bones occurs by means of direct spread.

[edit] Imaging Findings for Gorham syndrome

  • Radiographically, the earliest changes are foci of intramedullary and subcortical lucency resembling osteoporosis.
  • Concentric reduction results in tapering of involved long bones.
  • This is followed by complete resorption of the involved bone in severe cases.
  • A typical and notable finding is the lack of sclerosis or osteoblastic reaction.

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Gorham syndrome