Surgical Management of Haemophilic Pseudotumors: Experience in a Developing Country

ABSTRACT

Aim: Hemophilic pseudotumors result from repeated episodes of bleeding into bone, subperiosteum, and soft tissue. Since clotting factors became available, uncontrolled perioperative bleeding is a less significant problem for surgeons in developed countries. However, they are more difficult to come by in China. Additionally, patients often have to undergo surgery for giant masses and suffer complications. We wanted to present our experience in the surgical management of hemophilic pseudotumors over a 40-year period. Methods: We retrospectively reviewed 429 hemorrhagic coagulopathy patients between 1983 and 2015. Diagnosis of hemophilic pseudotumor was confirmed following clinical, radiological, and pathological criteria. The data were recorded and analyzed: type and severity of hemophilia, presence of inhibitor, etiological antecedent, localization of pseudotumors, clinical signs, surgical management and outcomes. Results: Eighteen pseudotumor patients underwent surgical treatment. All of them were male, with mean age of 34.3 years. Fifteen patients had hemophilia A and three patients had hemophilia B. There were twelve proximal and two distal pseudotumor patients. The mean follow-up was 51.9 months. For pseudotumors in the extremities, complete surgical resection was achieved. For four patients with pelvic or abdominal pseudotumors, complete surgical resection was only achieved in two patients because of preventing potential vital organs injuries. Delayed healing of the incision, allergic reactions, and ureteral injury were the major complications. Conclusion: Surgery is an alternative method with safety and efficacy. Careful and individual treatment is required by the hematologist, orthopedic surgeon and other members of the team who collaborate and participate in hemophilic surgery.

Keywords:

• hemophilia
• pseudotumor
• surgery
• management
• pharmacological regimen
• developing country

DECLARATION OF INTERESTS

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

FUNDING

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

ETHICAL STATEMENT

All experimental protocols were approved by the Institutional Review Board of Peking Union Medical College Hospital (No.S-K188).