Gorham-Stout综合征股骨近端骨溶解行人工全髋关节置换术治疗一例报告  

A case-report of successful treatment with total hip replacement for proximal femoral osteolysis in Gorham-Stout syndrome

作　　者：王广林 郑连杰[1] 杨梁[1] WANG Guang-lin;ZHENG Lian-jie;YANG Liang(Joint Surgery in the Second Affiliated Hospital of Dalian Medical University,Dalian,Liaoning,116021,China)

机构地区：[1]大连医科大学附属第二医院关节与运动医学科,辽宁省116021

出　　处：《中国骨与关节杂志》2023年第10期771-775,共5页Chinese Journal of Bone and Joint

摘　　要：Gorham-Stout综合征(Gorham-Stout syndrome,GSS)又称骨溶解病、特发性大块骨溶解骨病等,特征是骨基质中血管结构的增殖与进行性骨破坏和骨吸收[1],且无新的骨形成[2-3]。到目前为止,所见报道约300例[1]。GSS迄今病因未明。多位学者提出过可能的多因素病理机制。Gorham-Stout syndrome(GSS)is rare in clinic,and what even rarer is that artificial joint replacement after complete dissolution of the right hip joint in Gorham-Stout syndrome.Here,we report a 55-year-old female patient who suffered from lower strength of right lower limb for 2 weeks admitted to our hospital with“right hip osteoarthritis”as primary diagnosis,and then revised diagnosis to Gorham-Stout syndrome combined with the medical history,Radiology and pathological examinations,and in the end an artiflcial hip replacement was performed to help her.Only dislocation complications appeared after the artiflcial hip replacement in early postoperative follow-up,and the operative limb function was good after manual reduction.Patients considering the diagnosis of Gorham-Stout syndrome must exclude tumors,infections,circulatory disorders,osteolytic diseases Hardegger classifledⅠ-Ⅴ,etc.The current etiology of Gorham-Stout syndrome is unknown,but it may be associated with recombination of genes located on chromosomes 7,12,and 20,especially gene fusion genes including the ATG101 gene.The pathological process is a invasive,destroying and aggressive proliferation by non-neoplastic vascular tissue-and which replacing normal bone and even periosseous soft tissue.Vascular endothelial cell growth factor and growth factors related to lymphangysis such as platelet-derived growth factor(PDGF-BB)and osteoclasts are known to be involved in the pathological process in the disease.Treatment of guanzoledronic acid,interferon,sirolimus and other treatments can make good efflcacy for most GSS patients,and as new therapeutic drugs thalidomide and propranolol have achieved good results in some GSS cases.Radiation therapy is effective for GSS treatment.

关 键 词：骨质溶解 原发性 股骨 髋关节 人工关节 Gorham-Stout综合征 

分 类 号：R687.4[医药卫生—骨科学]