We experienced a case of open heart surgery for correction of ventricular septal defect and patent ductus arteriosus in five years old boy with hemophilia A. Factor VIII activity in this patient was 0.7% of normal activity. He received 2,000 unit of factor VIII intravenously for loading dose and has been infused 6 unit/kg/hour for 12 hours before the operation. And his factor VIII activity was 68% in the morning of the operation day. During the operation, he received 6 unit/kg/hour of factor VIII. After intracardiac anomaly was repaired and protamine was injected, we administrated 1000 unit of factor VIII. At that time, his factor VIII was 57%. After operation was done, he received 6 unit/kg/hour of factor VIII during stay in ICU and postoperative 14 days. His factor VIII activity was maintained at 36~104% during stay in hospital. So we report a successful case of perioperative management for the open heart surgery in patient with hemophilia A.
Ductus Arteriosus, Patent ; Factor VIII ; Heart Septal Defects, Ventricular ; Heart* ; Hemophilia A* ; Humans ; Male ; Thoracic Surgery*

We experienced a case of open heart surgery for correction of ventricular septal defect and patent ductus arteriosus in five years old boy with hemophilia A. Factor VIII activity in this patient was 0.7% of normal activity. He received 2,000 unit of factor VIII intravenously for loading dose and has been infused 6 unit/kg/hour for 12 hours before the operation. And his factor VIII activity was 68% in the morning of the operation day. During the operation, he received 6 unit/kg/hour of factor VIII. After intracardiac anomaly was repaired and protamine was injected, we administrated 1000 unit of factor VIII. At that time, his factor VIII was 57%. After operation was done, he received 6 unit/kg/hour of factor VIII during stay in ICU and postoperative 14 days. His factor VIII activity was maintained at 36~104% during stay in hospital. So we report a successful case of perioperative management for the open heart surgery in patient with hemophilia A.
Ductus Arteriosus, Patent ; Factor VIII ; Heart Septal Defects, Ventricular ; Heart* ; Hemophilia A* ; Humans ; Male ; Thoracic Surgery*

Aneurysm, False ; surgery ; Factor VIII ; therapeutic use ; Hemophilia A ; drug therapy ; Humans ; Uremia

Humans ; Ankle ; Ankle Joint/surgery* ; Arthritis ; Arthrodesis ; Lower Extremity ; Hemophilia A/complications*

OBJECTIVETo evaluate the short-term effects of ankle joint replacement for the patients with hemophilic arthritis. To find out the main points for improving the joint function and the patients' life quality. To accumulate more experience for clinical treatment.

METHODSThe Kofoed evaluation system and the AOFAS evaluation system were used to follow-up 6 hemophilic patients with ankle arthritis (6 males, ranging in age from 23 to 57 years, with an average of 41.2 years), who were treated with ankle joint replacement from 2004.7 to 2007.7. The scores before operation, and the 6th,12th months after operation were compared.

RESULTSThe Kofoed evaluation system showed the joint replacement significantly improved the scores for relieving pain, joint function, range of motion and the total score (P<0.05). Except the score for relieving pain, the other three also had significant differences between the 6th month and the 12th month after the operation (P<0.05). Compared with each details of the joint function, there was significant improvement before and after the operation (P<0.05). The ankle got more extended range of motion in the 12th month after operation than the 6th month (P<0.05). The AOFAS evaluation system showed the joint replacement significantly improved the scores for relieving pain, joint function, X-ray measurement and the total score (P<0.05). There was no differences between the 6th month after operation and the 12th month in the results for relieving pain and the X-ray measurement (P>0.05). However, the joint function and the total score had significant differences (P<0.05). Compared with each details of the joint function, there was also significant improvement before and after the operation (P< 0.05), and the walk distance, as well as the joint flexion and extension activities, had differences between the 6th month and the 12th month after the operation. Both of the two evaluation system showed the operation could get good curative effects (P< 0.05), and the artificial joint could constantly be in good condition from the 6th month to the 12th month after operation (P> 0.05).

CONCLUSIONIn this short-term observation, the joint replacement can significantly improve the life quality for the hemophilic patients with ankle arthritis.

Adult ; Ankle Joint ; physiopathology ; surgery ; Arthritis ; etiology ; physiopathology ; surgery ; Arthroplasty, Replacement ; methods ; Hemophilia A ; complications ; Humans ; Male ; Middle Aged

OBJECTIVETo study the efficacy of total knee anhmplasty (TKA) for the treatment of hemophilic knee arthropathy, and to explore the operative characteristics, the selection of prothesis, the effectiveness and safety of clotting factor replacement treatment.

METHODSFrom January 2008 to June 2010, 10 patients (12 knees)with hemophilic anhropathv underwent TKA. The average age was 33.6 years old (ranged, 17 to 49 years). There was 8 cases of type A hemophilia and 2 cases of type B hemophilia. According to Arnold and Hilgartner classification: 7 knees were IV degree and 5 knees were V degree. The level of VIII factor for replacement treatment was more than 80% on operation day, more than 60% within 3 days after operation, more than 40% from the third day to the second week after operation. Added prothrombin complex concentrate (PCC) to improve the level of IX factor, and the level of IX factor for replacement treatment was more than 40% on operation day, more than 30% within 3 days after operation, more than 20% from the third day to the second week after operation. Functional training was mainly based on continuous passive motion (CPM) device after surgery. Clinical assessment included hospital for special surgery knee score (HSS) and the individual scores (including pain, function, activity, muscle strength, flexion deformity and stability).

RESULTSTen patients (12 knees) were followed-up, and the average duration was 11 months (ranged, 6 to 24 months). The average preoperative HSS score was (44.9 +/- 12.5) (ranged, 29 to 62 scores), whereas the average postoperative HSS score was (84.4 +/- 10.2) (ranged 72 to 96 scores) (P < 0.01). The preoperative individual score including pain, function, activity, muscle strength flexion deformity and stability were significantly improved compared with pre-operation, the differences between them were statistically significant (P < 0.01 ). TKA had the distinct role in relieving pain from preoperative (8.5 +/- 4.1) to postoperative (24.5 +/- 4.4).

CONCLUSIONUnder the strict perioperative coagulation factor replacement therapy, TKA is a safe and an effective treatment for hemophmc anhmpathy of knee joint, whicht can effectively relieve pain and improve joint function.

Adolescent ; Adult ; Arthritis ; surgery ; Arthroplasty, Replacement, Knee ; methods ; Female ; Hemarthrosis ; surgery ; Hemophilia A ; complications ; drug therapy ; Humans ; Male ; Middle Aged

OBJECTIVETo discuss the perioperative management of hemophiliacs A with orthopaedic complications.

METHODSTo regulate the injection of factor VIII concentrate in peroperative period by testing the level of factor VIII:C in 27 cases. The lever of factor VIII:C was improved to 30% - 50% at the day before the operation. To the severe patients, it was maintained at 58.5% - 89.3% during the operation and at 47.0% - 78.4% in postoperation. While to the gentle, it was maintained at 38.5% - 52.5% during the operation and at 29.2% - 52.3% in postoperation. The individualized surgical procedures were carried out, such as arthrocentesis, open knee synovectomy or arthroscopic synovectomy, evacuation or curettage of haematoma, debridements, internal fixation.

RESULTSAll patients were cured in 14 - 105 days by regulating the injection of factor VIII during the peroperative period. The usage of factor VIII was 1 200 - 70 250 IU.

CONCLUSIONWhile the hemophiliacs have orthopaedic complications, it is necessary to promise the factor VIII:C being maintained at the lever of hemostasis during the peroperative period. The hemophiliacs are endurable to the operation.

Adolescent ; Adult ; Factor VIII ; administration & dosage ; Fractures, Bone ; complications ; surgery ; Hemarthrosis ; etiology ; surgery ; Hematoma ; etiology ; surgery ; Hemophilia A ; blood ; complications ; drug therapy ; Humans ; Male ; Perioperative Care ; Postoperative Complications ; blood

BACKGROUND: Total knee arthroplasty (TKA) can reduce severe joint pain and improve functional disability in hemophilia. However, the long-term outcomes have rarely been reported in China. Therefore, this study aimed to evaluate the long-term outcomes and complications of TKA in Chinese patients with hemophilic arthropathy. METHODS: We retrospectively reviewed patients with hemophilia who underwent TKA between 2003 and 2020, with at least 10 years of follow-up. The clinical results, patellar scores, patients' overall satisfaction ratings, and radiological findings were evaluated. Revision surgery for implants during the follow-up period was recorded. RESULTS: Twenty-six patients with 36 TKAs were successfully followed up for an average of 12.4 years. Their Hospital for Special Surgery Knee Score improved from an average of 45.8 to 85.9. The average flexion contracture statistically significantly decreased from 18.1° to 4.2°. The range of motion (ROM) improved from 60.6° to 84.8°. All the patients accepted patelloplasty, and the patients' patellar score improved from 7.8 preoperatively to 24.9 at the last follow-up. There was no statistically significant difference in clinical outcomes between the unilateral and bilateral procedures, except for a better ROM at follow-up in the unilateral group. Mild and enduring anterior knee pain was reported in seven knees (19%). The annual bleeding event was 2.7 times/year at the last follow-up. A total of 25 patients with 35 TKAs were satisfied with the procedure (97%). Revision surgery was performed in seven knees, with 10- and 15-year prosthesis survival rates of 85.8% and 75.7%, respectively. CONCLUSIONS TKA is an effective procedure for patients with end-stage hemophilic arthropathy, which relieves pain, improves knee functions, decreases flexion contracture, and provides a high rate of satisfaction after more than ten years of follow-up.
Humans ; Arthroplasty, Replacement, Knee/methods* ; Hemophilia A/surgery* ; Follow-Up Studies ; Retrospective Studies ; Treatment Outcome ; Knee Joint/surgery* ; Range of Motion, Articular ; Arthritis/complications* ; Pain ; Contracture/surgery* ; Surgeons ; Knee Prosthesis

OBJECTIVETo evaluate the clinical results and improve perioperative management in patients with hemophilic arthropathy.

METHODSFrom May 2002 to March 2007, 20 patients with hemophilic arthropathy were admitted. Fourteen patients (20 knees) were performed total knee arthroplasty, and 6 patients (8 hips) were performed total hip arthroplasty, while 2 patients were performed ankle arthrodesis. The average preoperative HSS score, hip Harris score and ankle AOFAS score were 83, 89 and 78 points respectively.

RESULTSAll patients had been followed up, and the mean time was 27.3 months. The average postoperative knee HSS score increased to 83 points, and hip Harris score increased to 89 points, while the AOFAS score increased to 78 points. Delayed bleeding and deep venous thrombosis and late infection with loose prosthesis occurred in one case respectively.

CONCLUSIONSArthroplasty and arthrodesis are promising for severe hemophilic arthropathy to obtain pain relief and functional improvement Correct perioperative management is the key to successful treatment in hemophilic arthropathy.

Adolescent ; Adult ; Arthritis ; etiology ; surgery ; Arthrodesis ; Arthroplasty, Replacement ; Blood Coagulation Factors ; therapeutic use ; Follow-Up Studies ; Hemophilia A ; complications ; Hemophilia B ; complications ; Humans ; Male ; Middle Aged ; Perioperative Care ; Treatment Outcome

OBJECTIVETo evaluate the amount of blood loss and the efficacy of clotting factor in controlling blood loss during total knee arthroplasty.

METHODSThe medical documents of 18 patients with haemophilic arthritis (HA) secondary to haemophilia A and 19 patients with osteoarthritis (OA) were retrospectively reviewed. Demographic data,functional and hematological test results,the amount of blood loss and transfusion,and complications were analyzed.

RESULTSThe median amounts of total and external blood loss were 2240 ml(1892-3415 ml) and 1326 ml(934-2256 ml)in the HA group, which were significant higher than those in the OA group [1746 ml(1259-2246 ml)and 846 ml (504-1217 ml), respectively]. The median amounts of external blood loss in the two groups were 680 ml(370-1330 ml)and 730 ml(200-1190 ml)and there was no significant difference(p=0.620). Moreover, more patients in the HA group required blood transfusion (84.2% vs. 47.4%), and more red cells were transfused per patient in the HA group (2.3 U vs. 0 U).

CONCLUSIONSThe total blood loss and hidden blood loss are higher in the HA patients than in OA patients during total knee arthroplasty, although the external blood loss is basically the same. Management with more clotting factor may decrease the blood loss in HA patients.

Adolescent ; Adult ; Arthritis ; etiology ; surgery ; Arthroplasty, Replacement, Knee ; Hemophilia A ; complications ; Humans ; Male ; Middle Aged ; Osteoarthritis, Knee ; surgery ; Postoperative Hemorrhage ; Retrospective Studies ; Young Adult