Hemophilic Pseudotumor

Jensen, Pamela S.

doi:10.1001/archpedi.1975.02120430053015

Cited by 42 publications

(5 citation statements)

References 9 publications

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“…HPT-related fractures are a severe complication of HPTs and may be caused by minimal trauma or have no obvious cause [15]. Jensen et al demonstrated that bone may be affected by HPTs via pressure necrosis, and the femur is the most common site of involvement [11]. HPTs generally progress asymptomatically until pathological bone fracture or neurovascular compression occurs [4].…”

Section: Discussionmentioning

confidence: 99%

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

Chen

Jiang

et al. 2021

J Orthop Surg Res

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Background Hemophilic 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. Methods Ten 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. Results The 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. Conclusions It 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.

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Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Introductionmentioning

confidence: 99%

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

Chen

Jiang

et al. 2021

J Orthop Surg Res

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show abstract

“…HPT-related fractures are a severe complication of HPTs and may be caused by minimal trauma or have no obvious cause [13]. Jensen et al demonstrated that bone may be affected by HPTs through pressure necrosis and he femur is the most common site of involvement [11]. Usually, HPTs progress asymptomatically until pathological bone fractures or neurovascular compression occurs [4].…”

Section: Discussionmentioning

confidence: 99%

“…When combined with HPTs, pathological fractures are more likely to occur, and these fractures are caused by minimal trauma or have no obvious cause. The femur is the most susceptible long bone to HPTs; accordingly, most HPT-related pathological fractures occur in the femur [9][10][11].…”

Section: Introductionmentioning

confidence: 99%

Surgical Treatment for Patients With Hemophilic Pseudotumor-Related Femoral Fracture: A Retrospective Study

Chen

Jiang

et al. 2020

Preprint

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Purpose: Hemophilic 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 aim of this retrospective study is to evaluate the outcome of these patients treated with surgical interventions.Methods: Ten patients with HPT-related femoral fractures who were treated with 12 surgical procedures in our hospital from Jan 2014 to April 2020 were retrospectively evaluated. Demographic data, fracture location, complications after operation and follow-up outcomes were recorded and analyzed with a mean follow-up period of 39.7 months.Results: Bone union was observed in five patients, an adequate callus was visible in two patients, and three patients underwent amputation. Both of 2 patients with closed reduction external fixation (CREF) had pin infection. Nonunion combined with external fixation (EF) failure occurred in one of whom, and the plate was broken after open reduction internal fixation. Three patients underwent autogenous or allogeneic cortical strut grafting. Three patients had HPT recurrence.Conclusion: It is necessary to perform surgery in patients with HPT-related femoral fractures. Surgical treatments must take both fracture stabilization and HPT resection into account. Internal fixation is preferable and EF should be dismissed unless for the purpose of temporary fixation. If the HPT erodes more than one-third of the bone diameter, struct grafts are necessary for mechanical stability. Amputation is a proper curative method in certain situations.

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“…Since the initial report by Starker in 1918 [ 11, over 100 cases of pseudotumors have been reported, involving primarily long bones of the lower extremities and pelvis. Other common sites of involvement have included the calcaneus [2,3], phalanges [4,5], clavicle [ 6 ] , and mandible, [4,7].…”

Section: Introductionmentioning

confidence: 99%

Pseudotumor of hemophilia in the orbit: The role of radiotherapy in management

Meyers

Hakami

1985

American J Hematol

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A case of pseudotumor of hemophilia is presented that occurred in the right orbit of a boy with severe factor VIII deficiency and an inhibitor. After a period of observation and conservative management, low-dose radiotherapy (750 rads) and Proplex was used. Eighteen months after radiotherapy significant healing had occurred. The role of radiotherapy in the treatment of hemophilic pseudotumor is reviewed.

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Hemophilic Pseudotumor

Cited by 42 publications

References 9 publications

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

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

Surgical Treatment for Patients With Hemophilic Pseudotumor-Related Femoral Fracture: A Retrospective Study

Pseudotumor of hemophilia in the orbit: The role of radiotherapy in management

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