Yin De-Long, Lin Jia-Min, Li Yuan-Hui, Chen Peng, Zeng Mian-Dong
Department of Orthopedic, The Third Affiliated Hospital of Guangzhou Medical University, Guangzhou 510150, Guangdong Province, China.
Department of Orthopedic, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China.
World J Clin Cases. 2023 Apr 26;11(12):2788-2795. doi: 10.12998/wjcc.v11.i12.2788.
Hemophilia A is a rare inherited bleeding disorder caused by mutations in the factor VIII gene. This clotting factor plays an intrinsic role in the blood coagulation pathway. Patients with hemophilia may develop orthopedic manifestations such as hemarthrosis, but multiple malunion of fractures over the knee is rare and difficult to treat.
We report a patient with hemophilia A who developed severe knee osteoarthritis along with fracture malunion and nonunion. Total knee replacement was performed using a custom-made modular hinged knee prosthesis (cemented) equipped with extended distal and proximal stems. At 3 years' follow-up, the patient exhibited excellent clinical function and remained satisfied with the surgical outcome. Surgical intervention was accompanied by rigorous coagulation factor replacement.
This case highlights various unique scenarios specific to individuals with hemophilia and fracture deformity.
甲型血友病是一种由凝血因子 VIII 基因突变引起的罕见遗传性出血性疾病。这种凝血因子在血液凝固途径中发挥内在作用。血友病患者可能会出现诸如关节积血等骨科表现,但膝关节以上骨折多处畸形愈合罕见且难以治疗。
我们报告一名甲型血友病患者,其出现了严重的膝关节骨关节炎以及骨折畸形愈合和不愈合。使用定制的模块化铰链式膝关节假体(骨水泥固定)进行了全膝关节置换,该假体配备了延长的远端和近端柄。随访 3 年时,患者临床功能极佳,对手术结果仍感满意。手术干预同时进行了严格的凝血因子替代治疗。
本病例突出了血友病患者和骨折畸形患者所特有的各种独特情况。