title: Resection and reconstruction of a massive femoral hemophilic pseudotumor.
abstract: 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.
authors.affiliation: Department of Orthopaedic Surgery, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Jacob M
authors.initials: JM
authors.lastname: Buchowski
authors.forename: Brett M
authors.initials: BM
authors.lastname: Cascio
authors.forename: Michael B
authors.initials: MB
authors.lastname: Streiff
authors.forename: Frank J
authors.initials: FJ
authors.lastname: Frassica
mesh_terms: D001847:Bone Diseases / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D005269:Femur / Q000000981:diagnostic imaging / Q000601:surgery*; D006406:Hematoma / Q000175:diagnosis* / Q000209:etiology* / Q000601:surgery; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D019637:Orthopedic Procedures / Q000295:instrumentation / Q000379:methods; D011474:Prosthesis Design; D011859:Radiography; D019651:Plastic Surgery Procedures / Q000295:instrumentation / Q000379:methods; D016896:Treatment Outcome