Erschienen in:
01.03.2012 | Case Report
A case report of Gorham–Stout syndrome remission
verfasst von:
Toru Shimizu, Kaori Sato, Tomomi Yoshida, Atsushi Takahashi, Takashi Yanagawa, Naoki Wada, Makoto Sohmiya, Kenji Shirakura, Hideomi Watanabe
Erschienen in:
Journal of Orthopaedic Science
|
Ausgabe 2/2012
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Excerpt
Gorham–Stout syndrome (GSS) is a rare disorder characterized by spontaneous bone resorption with rapid progression, occasionally after minor trauma. It is also known as massive osteolysis or vanishing bone disease [
1‐
3]. Some patients present with relatively abrupt pain and have swelling in the affected extremity, whereas others present with a history of insidious onset of pain, limitation of motion, and progressive weakness in the involved limb. The affected bone disappears completely [
4]. The degree of osseous deformity in patients with GSS becomes severe. Paraplegia occurs in patients with spinal cord compression caused by vertebral involvement. Spinal involvement increases mortality to over 30%, because of complications such as spinal cord compression [
5]. Thoracic cage, pulmonary, or pleural involvement leads to compromise of respiratory function and consequent death [
3,
6]. Visceral complication can occur when the disease invades the surrounding tissues [
7]. Recently, interferon and bisphosphonates have been reported to have remissive effects, although the mechanisms are unclear [
8,
9]. Understanding the mechanisms that contribute to spontaneous or drug-induced remission is important in the control of this severe condition. …