title: Surgical management of hemophilic pseudotumor complicated by destructive osteoarthropathy.
abstract: Hemophilic pseudotumor gradually erodes bone and induces fracture or deformity, causing joint dysfunction or destructive osteoarthropathy. Reports about surgery for hemophilic pseudotumor complicated by destructive osteoarthropathy are scarce. The object of this study was to evaluate the results and complications of surgical management for patients of pseudotumor complicated by destructive osteoarthropathy. We retrospectively reviewed records from July 1996 to July 2013, and found eight patients with pseudotumor complicated by destructive osteoarthropathy. We recorded their demographic data, time of surgery, amount of blood loss and transfusion, bone union, and complications. Seven patients were diagnosed with hemophilia A and one with hemophilia B. The mean age at surgery was 31.9 ± 8.3 years. Two of the eight underwent excision of the pseudotumor and metallic fixation, one had amputation, and five underwent autogenous or exogenous bone grafting and fixation with an absorbable screw. The median operating time was 170 min (135-315 min). The median amount of intraoperative blood loss was 1350 ml (100-4000 ml). The amount of red blood cells, plasma, and whole blood transfusion after surgery were 0-24 units, 0-2000 ml, and 0-4600 ml, respectively. After a median follow-up of 75 months, the numbers of pseudotumor recurrence, fracture nonunion, coagulation factor inhibitor formation, and wound complications were one, one, two, and four, respectively. Surgery is an effective treatment for hemophilic pseudotumor complicated by destructive osteoarthropathy. However, the incidences of wound infection, coagulation factor inhibitor formation, hemophilic pseudotumor recurrence, and fracture nonunion are high.
authors: Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
authors: Jiliang
authors: J
authors: Zhai
authors: Xisheng
authors: X
authors: Weng
authors: Baozhong
authors: B
authors: Zhang
authors: Hui-ming
authors: HM
authors: Peng
authors: Yan-yan
authors: YY
authors: Bian
authors: Lei
authors: L
authors: Zhou
mesh_terms: D000328:Adult; D001847:Bone Diseases / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D001842:Bone and Bones / Q000473:pathology / Q000601:surgery; D005260:Female; D006467:Hemophilia A / Q000150:complications*; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
references: Radiographics. 2003 Jul-Aug;23(4):852-6
references: 12853660
references: Haemophilia. 2002 Jan;8(1):12-6
references: 11886459
references: Lancet. 1989 Dec 2;2(8675):1333-4
references: 2574277
references: Haemophilia. 1999 Sep;5(5):360-3
references: 10583519
references: Haemophilia. 2006 Sep;12(5):541-4
references: 16919086
references: Int Orthop. 2012 Oct;36(10):2157-62
references: 22752668
references: Skeletal Radiol. 2002 Sep;31(9):550-3
references: 12195512
references: Haemophilia. 2014 Jan;20(1):e58-62
references: 24354486
references: Haemophilia. 2002 Mar;8(2):104-11
references: 11952845
references: Haemophilia. 2009 Mar;15(2):448-57
references: 19175421
references: Am J Hematol. 1994 Feb;45(2):103-8
references: 8141115
references: Prog Clin Biol Res. 1990;324:257-62
references: 2308969
references: Haemophilia. 1998 Mar;4(2):132-5
references: 9873852
references: Haemophilia. 2009 Jan;15(1):253-60
references: 18783440
references: Haemophilia. 2000 Jan;6(1):55-6
references: 10632744
references: Haemophilia. 2002 May;8(3):393-401
references: 12010440