[Orthopedic treatment strategy for hemophilic pseudotumor]

To summarize 10-year orthopedic experiences in the management of hemophilic pseudotumor and to discuss an individualized treatment strategy centered on surgery.

The clinical data of 8 patients with hemophilic pseudotumor between June 2015 and May 2025 were retrospectively analyzed. All patients were male, aged 15-37 years, with a mean age of 27.3 years. Seven patients had hemophilia type A and 1 had hemophilia type B; all had severe hemophilia and no detectable inhibitor. The disease duration ranged from 2 months to 12 years, with a mean of 2.5 years. There were 7 soft-tissue pseudotumors and 1 osseous pseudotumor. Lesions involved the lower leg in 3 cases, the thigh in 1, the hip in 1, both the hip and thigh in 2, and both the knee and thigh in 1. Pseudotumor size ranged from 5 cm×5 cm×4 cm to 32 cm×19 cm×15 cm. Treatment modalities included conservative treatment in 1 case, pseudotumor excision in 5 cases, and transfemoral amputation in 2 cases. Perioperative factor replacement was administered according to hemophilia type and factor activity, followed by wound care, anti-infective treatment when needed, drainage management, and rehabilitation.

For 7 patients undergoing surgery, the operation time was 60-120 minutes (mean, 88.6 minutes), and the intraoperative blood loss was 150-700 mL (mean, 342.9 mL). Six patients received coagulation factor Ⅷ at doses of 25 400-40 000 U (mean, 32 600 U); 1 patient received coagulation factor Ⅸ at a dose of 35 000 U; the length of hospital stay was 15-30 days (mean, 22.1 days). All patients were followed up 2-10 years (mean, 3.9 years.). One patient developed a superficial wound infection, which resolved after sensitive antibiotics and dressing changes. One patient with a lower-leg soft-tissue pseudotumor healed after conservative treatment and had no recurrence. Among the 5 patients treated with pseudotumor excision, 1 patient with an osseous pseudotumor achieved satisfactory wound and bone healing after pseudotumor resection, allogeneic bone grafting, and internal fixation; 3 patients with soft-tissue pseudotumors had no recurrence during follow-up; and 1 patient with a giant hip soft-tissue pseudotumor developed recurrence with ulceration and massive bleeding at 2 years after surgery and eventually died after unsuccessful rescue at another hospital. The 2 patients who underwent amputation achieved wound healing and returned to daily life after prosthetic fitting and rehabilitation.

Surgery remains the main treatment for hemophilic pseudotumors that fail conservative treatment or present with progressive enlargement, ulceration and infection, or compression of vital structures. Adequate perioperative factor coverage, precise preoperative imaging, complete excision whenever feasible, and meticulous elimination of dead space are essential to reduce complications and recurrence.