Clinical Study On The Characteristics And Perioperative Management Of Hemophilia Pseudotumor

Hemophilia is a group of hemorrhagic diseases with hereditary coagulation dysfunction,which can be divided into hemophilia A and hemophilia B due to their common characteristics such as impaired production of active thrombin,prolonged coagulation time and bleeding tendency after minor trauma.Hemophilia A accounted for 80%to 90%of hemophilia patients,hemophilia B accounted for 15%to 20%Joint or muscle is the most common bleeding site of hemophilia.Acute bleeding of musculoskeletal system or chronic complications caused by bleeding(such as chronic hemophilia osteoarthropathy,hemophilia pseudotumor of soft tissue and bone,pathological fracture,etc.)'as well as injuries of musculoskeletal system caused by trauma often need orthopedic treatment.Hemophilia pseudotumor,also known as hemophilia blood cyst,is a rare complication of hemophilia,the incidence of about 1%?2%.The essence of this disease is repeated bleeding of extra-articular bone or soft tissue,leading to intraventricular hematocele,continuous enlargement of hematoma,compression and destruction of adjacent soft tissue.At present,the pathogenesis of hemophilia pseudotumor is not completely clear.Jensen et al.believe that the pathogenesis may be soft tissue hematoma oppresses adjacent bone tissue,causing damage and defect;Subperiosteal hemorrhage hyperplasia,organization,oppression of bone,degeneration,necrosis;Cortical and marrow internal hemorrhage,secondary compression of bone tissue,resulting in bone cortical thinning,hematoma puncture of the cortex thereby forming a soft tissue mass.Hemophilia pseudotumor is a rare disease,and currently there is a lack of standard diagnosis and treatment.Treatment of the disease depends on the location,size,growth rate,and impact on the surrounding tissue of the pseudotumor,and each case requires specific analysis before a treatment can be determined.Based on the existing problems of hemophilia pseudotumor,retrospective follow-up was conducted on the patients hospitalized with pseudotumor in our hospital,to discuss the characteristics of the formation of pseudotumor,and to discuss the perioperative management,so as to provide a reference for the prevention and treatment of clinical pseudotumor.The clinical data of hemophilia pseudotumor patients admitted to the department of orthopaedics of trauma hospital of south China hospital from January 2012 to January 2019 were retrospectively analyzed.Epidemiological data,natural course of hemophilia pseudotumor,perioperative management,postoperative complications and postoperative psychological and life quality of patients were included in the statistics.A total of 25 patients were included in this study,and the average age of the patients at the time of surgery was 34.1±11.0 years old(11~54 years old).Surgery including 8 cases of pure hemophilia false tumor resection,4 cases of hemophilia false tumor excision of joint plate internal fixation plus allograft bone graft,1 case of hemophilia false tumor excision of + autogenous fibular graft joint plate fixation,1 case of hemophilia false tumor excision ofjoint plate internal fixation plus autogenous iliac bone graft and 3 cases of open reduction and plate internal fixation of fracture + hemophilia false tumor resection,1 case of hemophilia false tumor excision with pelvic reconstruction,7 cases of different height of lower limb amputation.Through multidisciplinary team collaboration,perioperative patients with hemophilia sex pseudotumor ? preoperative coagulat:ion factor activity(F ?:C):113.2±17.3%(89.6%-153.9%),?(F ?:C):92.2±21.1%(72.6%-129.10/%).The average length of stay was 23.3±5.0 days(15-32 days).Intraoperative and postoperative complications included intraoperative transfusion allergy in 1 case,postoperative hematoma in 1 case,hemophilia pseudotumor recurrence in 1 case,fracture nonunion in 1 case,delayed wound healing in 1 case.The mean follow-up time was 33.2±22.4 months(ranging from 6 to 82 months).Through this study,recurrent spontaneous hemorrhage of skeletal muscle tissue can form hemophilia pseudotumor in a small number of hemophilia patients.The formation of pseudotumor may be related to the bleeding site,bleeding frequency,bleeding amount and treatment plan.Hemophilia pseudotumor has a senous impact on the life and quality of life of patients.Currently,the factor replacement scheme and perioperative management method adopted in this study can ensure the safety and feasibility of hemophilia pseudotumor surgery,'and help patients to restore part of the quality of life.Currently,orthopaedic surgery for hemophilia pseudotumor is still difficult,and multidisciplinary team cooperation is needed to ensure the optimal treatment for patients.