Abrupt onset of pain and progressive weakness in a young female

Ekaterini Tavernaraki, Chrissafoula Kolofousi, John Kalogeropoulos

Abstract


We report a case of an extremely rare, benign and of unknown etiology syndrome, known as Gorham-Stout syndrome, vanishing bone disease or lymphangiomatosis. It is characterized by the presence of massive osteolysis and intraosseous hyperplasia of small vascular and lymphatic vessels, which leads to destruction and progressive absorption of the osseous matrix.  Although the majority of Gorhanm-Stout syndrome cases takes place during childhood, more than 1/3 appear after the age of 35.  We present a case of a 38 yearold female presenting an abrupt onset of dull aching pain, limitation of motion and progressive weakness. Radiography revealed massive osteolysis of body of both pubic bones and extension to right ischial ramus. Both bone ends where pointing.  CT findings were massive osteolysis of body of both pubic bones and extension to right acetabulum. MRI revealed pathologic signal of affected areas at STIR sequence.

The distinctive histopathological and radiological features led to the final diagnosis.


Keywords


idiopathic osteolysis; CT; MR; vanishing bone

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References


Gorham LW, Wright AW, Shultz HH, et al. Disappearing bones: a rare form of massive osteolysis: report of two cases, one with autopsy findings. Am J Med 1954; 17: 674-682.

Gorham LW, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone): its relation to hemangiomatosis. J Bone Joint Surg [Am] 1955; 37-A: 985-1004.

Choma ND, Biscotti CV, Bauer TW, et al. Gorham’s syndrome: a case report and review of the literature. Am J Med 1987; 83: 1151-1156.

Moller G, Priemel M, Amling M, et al. The Gorham-Stout syndrome (Gorham’s massive osteolysis): a report of six cases with histopathological findings. J Bone Joint Surg [Br] 1999; 81-B: 501-506.

Campbell J, Almond HG, Johnson R. Massive osteolysis of the humerus with spontaneous recovery. J Bone Joint Surg [Br] 1975; 57-B: 238-240.

Hanly JG, Walsh NM, Bresnihan B. Massive osteolysis in the hand and response to radiotherapy. J Rheumatol 1985; 12: 580-582.

Chavanis N, Chaffanjon P, Frey G, et al. Chylothorax complicating Gorham’s disease. Ann Thorac Surg 2001; 72: 937-939.

Collins J. Case 92: Gorham syndrome. Radiology 2006; 238: 1066-1069.

Vinee P, Tanyu O, Hauenstein KH, et al. CT and MRI of Gorham Syndrome. J Comput Assist Tomogr 1994; 18: 985-989.

Dominguez R, Washowich TL. Gorham’s disease or vanishing bone disease: plain film CT, and MRI findings of two cases. Pediatr Radiol 1994; 24: 316-318.


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