Surgical Treatment for Hemophilic Pseudotumor: Twenty-three Cases with an Average Follow-up of 5 Years.

Abstract

Hemophilic pseudotumor (HPT) is a rare disease with many challenges. Only a few reports on surgical treatment for HPT have been published. The cases of 23 patients with HPT who had surgical treatment from July 1996 to December 2014 were retrospectively reviewed. Demographic data, blood loss and transfusion during surgery, outcomes, and complications after surgery were analyzed. Eleven patients underwent HPT resection; 4 underwent HPT excision, allograft transplantation, and absorbable screw fixation; 3 had HPT resection and metallic internal fixation; 2 had HPT resection, autogenous fibular grafting, and absorbable screw fixation; 2 underwent curettage and bone-grafting; and 1 patient received above-the-knee amputation. The average age (and standard deviation) of the patients at the time of surgery was 31.9 ± 12.8 years (range, 6 to 54 years) with an average follow-up of 5.3 ± 4.7 years (range, 1.1 to 19.6 years). The median duration of the surgery was 157 minutes (range, 90 to 315 minutes). The median amount of blood loss during surgery was 800 mL (range, 100 to 4,000 mL). Three patients (13%) had a postoperative infection, 2 (8.7%) had recurrence of HPT, and another 2 patients had fracture nonunion. Surgical treatment of HPT with a modified protocol of coagulation factor replacement is safe and effective. It should be recommended for patients with HPT who have progressive enlargement of the mass, recurrent and massive bleeding, spontaneous perforation, bone erosion, or compression of surrounding tissues or who have had failure of conservative treatment. Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.