Vinée P, Tanyü M O, Hauenstein K H, Sigmund G, Stöver B, Adler C P
Radiologische Universitätsklinik Freiburg, Abteilung Röntgendiagnostik, Germany.
J Comput Assist Tomogr. 1994 Nov-Dec;18(6):985-9. doi: 10.1097/00004728-199411000-00028.
Gorham syndrome, also known as massive osteolysis or vanishing bone, is a rare disorder (135 cases reported) leading to extensive loss of bony matrix, replaced by proliferating thin-walled vascular channels. Three histologically proven cases of the disease are reported, including the clinical presentation and modern imaging features with CT (with 3D reconstruction) as well as T1- and T2-weighted MRI. Two cases in young women were located in the pelvis with extensive osteolysis reaching to the acetabulum. The third case in a 2-month-old boy is the youngest case ever reported and involved the humerus. The radiological appearance of the disease is discussed and the importance of the modern imaging methods debated.
戈勒姆综合征,也称为大块骨质溶解症或骨质消失症,是一种罕见疾病(已报道135例),可导致骨基质大量丧失,代之以增生的薄壁血管通道。本文报告了3例经组织学证实的该疾病病例,包括临床表现以及CT(三维重建)、T1加权和T2加权MRI等现代影像学特征。2例年轻女性病例位于骨盆,骨质溶解广泛,累及髋臼。第三例为一名2个月大男婴,是有史以来报道的最年轻病例,病变累及肱骨。本文讨论了该疾病的放射学表现,并对现代影像学方法的重要性进行了探讨。