Gorham Disease of Distal Humerus Successfully Treated by Slight Shortening and Pin and Plate Fixation

Keivan Rahbari, Kian Zohrabi, R. Kamrani
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Abstract

Background: Gorham's disease (GD) is one of the scarce and idiopathic skeletal diseases which causes osteolysis following the proliferation of blood vessels. Progressive osteolysis GD of distal humerus with articular involvement and pathologic fracture has not been reported and our case is the first report of this disease involving distal humerus and its joints’ surfaces. Case Report: A 9-year-old boy, case of nonunion of medial condyle of humerus and pathologic fracture of distal humerus after minor trauma, was referred to our clinic and treatment started by casting but due to displacement and nonunion, we decide to operate him. Intraoperative finding was in favour of aneurysmal bone cyst (ABC) near fracture site; therefore, wide resection and fixation by medial tension band wiring (TBW), lateral plating, and fibular allograft application was done (post-operation pathologic result did not show microscopical features of this tumor) and 6 weeks later, he developed stress riser fracture above lateral plate; thus, plate removal was done and severe bone resorption was revealed. Therefore, another specimen was sent for pathology that showed hamartomatous and hemangiomatous lesion of bone. All findings were in favour of GD. He was operated another time and fixed by Persian fixation with small pins and plate, and early plate removal was done for prevention of stress riser fracture. Conclusion: In cases of GD of distal humerus and pathologic fracture, Persian fixation is a good option for fixation and we suggest early device removal for prevention of stress riser fracture.
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肱骨远端Gorham病经轻微缩短及钉钢板固定成功治疗
背景:戈勒姆病(GD)是一种罕见的特发性骨骼疾病,可引起血管增殖后的骨溶解。肱骨远端进行性骨溶解性GD伴关节受累和病理性骨折尚未报道,我们的病例是该疾病涉及肱骨远端及其关节表面的第一例报告。病例报告:一名9岁男孩,肱骨内侧髁骨不连,肱骨远端病理性骨折,轻微创伤后被转诊至我们的诊所,治疗开始于铸造,但由于移位和不连,我们决定对他进行手术。术中发现有利于骨折部位附近的动脉瘤样骨囊肿(ABC);因此,通过内侧张力带布线(TBW)、外侧钢板和同种异体腓骨应用进行了广泛的切除和固定(术后病理结果未显示该肿瘤的显微镜特征),6周后,他出现了外侧钢板上方的应力提升骨折;因此,进行了钢板切除,并显示出严重的骨吸收。因此,另一个标本被送往病理学检查,显示骨骼的错构瘤和血管瘤病变。所有研究结果均支持GD。他再次接受手术,并用小钉和钢板进行波斯固定,并尽早取出钢板以预防应力立管骨折。结论:在肱骨远端GD和病理性骨折的病例中,波斯内固定术是一种很好的固定选择,我们建议尽早取出器械以预防应力性立管骨折。
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审稿时长
12 weeks
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