TY - JOUR
T1 - Case report
T2 - Resection and reconstruction of a massive femoral hemophilic pseudotumor
AU - Buchowski, Jacob M.
AU - Cascio, Brett M.
AU - Streiff, Michael B.
AU - Frassica, Frank J
PY - 2005/1
Y1 - 2005/1
N2 - Hemophilic pseudotumor is a rare, but well-known, complication of hemophilia. We describe a 50-year-old man with mild hemophilia A, but with no previous need for Factor VIII supplementation, who presented with a pathologic fracture of the right femoral neck and shaft caused by a large hemophilic pseudotumor. Initial nonoperative therapy with factor replacement and skeletal traction resulted in radiographic evidence of fracture healing, but the patient's pain persisted. Therefore, he had a radical resection of his hemophilic pseudotumor (soft tissue component and entire femur), and reconstruction with a custom total femoral replacement. Six months after resection, the patient returned to full-time employment. Although pseudotumor formation is a well-recognized complication of hemophilia, the pseudotumor in our study is one of the largest yet described. More importantly, to our knowledge this is the first report of a pseudotumor treated by radical resection and reconstruction with a custom femoral prosthesis. We think that radical resection and reconstruction with a custom total femoral prosthesis is a valuable alternative to amputation in massive pseudotumors of the femur and soft tissues of the thigh.
AB - Hemophilic pseudotumor is a rare, but well-known, complication of hemophilia. We describe a 50-year-old man with mild hemophilia A, but with no previous need for Factor VIII supplementation, who presented with a pathologic fracture of the right femoral neck and shaft caused by a large hemophilic pseudotumor. Initial nonoperative therapy with factor replacement and skeletal traction resulted in radiographic evidence of fracture healing, but the patient's pain persisted. Therefore, he had a radical resection of his hemophilic pseudotumor (soft tissue component and entire femur), and reconstruction with a custom total femoral replacement. Six months after resection, the patient returned to full-time employment. Although pseudotumor formation is a well-recognized complication of hemophilia, the pseudotumor in our study is one of the largest yet described. More importantly, to our knowledge this is the first report of a pseudotumor treated by radical resection and reconstruction with a custom femoral prosthesis. We think that radical resection and reconstruction with a custom total femoral prosthesis is a valuable alternative to amputation in massive pseudotumors of the femur and soft tissues of the thigh.
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U2 - 10.1097/01.blo.0000137545.37824.b0
DO - 10.1097/01.blo.0000137545.37824.b0
M3 - Article
C2 - 15662330
AN - SCOPUS:13744263846
SN - 0009-921X
VL - 430
SP - 237
EP - 242
JO - Clinical orthopaedics and related research
JF - Clinical orthopaedics and related research
ER -