Outcome of Surgical Management of Hemophilic Pseudotumor: Review of 10 Cases from Single-Center

Abstract

Objective: To evaluate the operative methods and clinical results of surgical treatment in a case series of 10 patients with hemophilic pseudotumors.

Methods: Ten patients with hemophilic pseudotumors who received surgical resection treatment in our hospital from October 2017 to June 2020 were retrospectively reviewed. All patients were hemophilia A (factor VIII deficiency).The age range was 20-51 years. Preoperative imaging examination revealed the size of irregular mass from 8.2 cm× 3.3 cm× 2.3 cm to 22.3 cm× 15.5 cm× 17.0 cm. With the supplementary of recombinant coagulation factor VIII, five cases received complete resection; one received resection and skin grafting; one received cytoreduction surgery as the pseudotumor closing to iliac vessel and nerve; three cases received complete resection and construction as bone destruction. The perioperative variables were recorded and all the patients were followed in the outpatient clinic. Clinical and radiological assessments were conducted.

Results: In these patients, the average intraoperative blood loss volume was 783.1 mL (range, 240-2100 mL). Six patients received blood transfusion during perioperative period. The average duration of surgery was 140.7 min (range, 110-240 min). All wounds healed smoothly and there was no infection or chronic sinus formation. The average length of hospital stay was 16.3 days (range, 12-25 days). There is no iatrogenic vascular nerve injury in our series. Complete follow-up was performed in all patients. Mean follow-up duration was 14.2 months (range, 6-26 months). One patient with pseudotumor in the thigh had a recurrence 1 year after operation, then secondary operation was performed. In three cases who received complete resection and construction, patient 8 obtained bone graft and late fixation. X-ray examination showed bone formation in the lesion at the 2-year follow-ups after operation. Patient 9 underwent knee replacement, his left knee showed flexion deformity in preoparation. At the last follow-up, range of motion was improved from 0° to 40° compared with preoperative status. Patient 10 had pseudotumor in the distal femur, received long bone graft and intramedullary nail fixation.

Conclusions: Surgical resection for hemophilic pseudotumors is an effective and safe method. The choice of surgical procedure must be individualized according to the localization and progress of pseudotumor.

Keywords: Hemophilia; Hemophilic pseudotumors; Surgical management; Surgical resection.