Surgical treatment for patients with hemophilic pseudotumor-related femoral fracture: a retrospective study

Keyu Chen,Bin Chen,Zexiong Mao,Yunping Yang,Guiyong Jiang,Jiaxin Lv,Yaowen Xu,Fei Liu

doi:10.1186/s13018-021-02426-1

Abstract

BackgroundHemophilic pseudotumor (HPT)-related fracture is a rare but severe complication in patients with HPTs. These fractures often occur in femurs. There is no consensus on the standard surgical protocol for HPT-related femoral fracture. The present retrospective study evaluated the outcomes of these patients treated with surgical interventions.MethodsTen patients with HPT-related femoral fractures who were treated with 14 surgical procedures due to 11 fractures in our hospital from January 2014 to April 2020 were evaluated retrospectively. Demographic data, fracture location, complications after surgery, and follow-up outcomes were recorded and analyzed. The mean follow-up period was 39.7 months.ResultsThe mean age at surgery was 31 years. Closed reduction external fixation (CREF) was originally performed in 2 patients, open reduction internal fixation (ORIF) was performed in 4 patients, screw fixation alone was performed in 1 patient, brace immobilization was performed in 1 patient, and amputation was performed in 3 patients. Bone union was observed in 5 patients, and an adequate callus was visible in 2 patients. Both patients with CREF had pin infections. Nonunion combined with external fixation (EF) failure occurred in 1 patient, and the plate was broken after ORIF. Three patients underwent autogenous or allogeneic cortical strut grafting. Three patients had HPT recurrence.ConclusionsIt is necessary to perform surgery in patients with HPT-related femoral fractures. Surgical treatments must consider fracture stabilization and HPT resection. Internal fixation is preferable, and EF should only be used for temporary fixation. If the HPT erodes more than one third of the bone diameter, strut grafts are necessary for mechanical stability. Amputation is an appropriate curative method in certain situations.

Highlights

Hemophilia is an X-linked hereditary bleeding disorder caused by a deficiency or lack of coagulation factor VIII (FVIII), which leads to hemophilia A (HA), or factor IX (FIX), which leads to hemophilia B (HB) [1]
Closed reduction external fixation (CREF) was originally performed in 2 patients, open reduction internal fixation (ORIF) was performed in 4 patients, screw fixation alone was performed in 1 patient, brace immobilization was performed in 1 patient, and amputation was performed in 3 patients
Mirels analyzed 78 metastatic long bone lesions from 28 patients, and the results showed that the rate of fracture significantly increased when the size of the lesion was more than two thirds of the bone diameter [23]

Summary

Introduction

Hemophilia is an X-linked hereditary bleeding disorder caused by a deficiency or lack of coagulation factor VIII (FVIII), which leads to hemophilia A (HA), or factor IX (FIX), which leads to hemophilia B (HB) [1]. Pathological fractures are more likely to occur in hemophilia patients with HPTs, and these fractures are caused by minimal trauma or have no obvious cause. The femur is the most susceptible long bone to HPTs, and most HPT-related pathological fractures occur in this bone [9,10,11]. There is no consensus on the standard surgical protocol for HPT-related femoral fracture. The present retrospective study evaluated the outcomes of these patients treated with surgical interventions

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Results

Discussion

Conclusion