Document Type: RESEARCH PAPER

Authors

1 Department of Orthopaedic and Traumatology Dr.Cipto Mangunkusumo General Hospita, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia Integrated Hamophillia TeamDr. Cipto Mangunkusumo General Hospital, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia

2 Department of Orthopaedic and Traumatology Dr.Cipto Mangunkusumo General Hospita, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia

3 Department of Internal Medicine Dr.Cipto Mangunkusumo General Hospital, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia Integrated Hamophillia TeamDr. Cipto Mangunkusumo General Hospital, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia

4 Department of Paediatric Dr.Cipto Mangunkusumo General Hospital, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia Integrated Hamophillia Team, Dr. Cipto Mangunkusumo General Hospital, Faculty of Medicine University of Indonesia, Jakarta, Indonesia

Abstract

Background: Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild
trauma or daily activities. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor,
usually in the muscle adjacent to the bone. We reported haemophilic pseudotumor treated with various surgical
interventions.
Methods: This study was conducted inthe Department of Orthopaedic and Traumatology at a government hospital
over a period of 7 years(2010 –2017). Patients Perioperative management was done in accordance with the Integrated
Hemophilia Team of our institution protocol.Diagnosis and management planning of hemophilic pseudotumor was
confirmed via Integrated HemophiliaTeam meeting. After the surgery, all patients were asked to come for routine
follow up.
Results: We reported six Haemophilia-A patients with pseudotumor in the pelvis, proximal femur and lower
leg. One case in pelvic bone underwent hematoma evacuation, acetabular reconstruction using the Harrington
procedure, and total hip arthroplasty.Two cases, a case in the proximal femur and another case in the distal fibula,
were treated with amputation, other two cases, one was soft tissue psedotumor in the pelvic region and was
treated by hematoma evacuation, and the remaining casewas managed with wide excision and followed by defect
closure.
Conclusion: Surgery is a preferable treatment for pseudotumors that have been present for years.It’s associated
with the best outcomes especially when selected as the primary line ofwith preventable and manageable bleeding
complication. As previously published by many authors, this paper confirms that surgical excision is the treatment of
choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team.
Level of evidence: IV

Keywords

Main Subjects

1. Rodriguez-Merchan EC. Musculoskeletal complications
of hemophilia. HSS J. 2010; 6(1):37-42.
2. World Federation of Hemophilia. 2nd Guidelines for
the management of hemophilia. Montreal: World
Federation of Hemophilia; Blackwell Publishing Ltd;
2005.
3. Ghosh K. Management of haemophilia and its
complications in developing countries. Clin Lab Haem.
2004; 26(4):243-51.
4. Garcia-Perez R, Torres-Salmeron G, Sanchez-Bueno
F, Garcia-Lopez A, Parrilla-Paricio P. Intraabdominal
hemophilic pseudotumor: case report. Rev Esp Enferm
Dig. 2010; 102(4):275-80.
5. Rodriguez-Merchan EC, Goddard NJ. Muscular
bleeding, haematomas, and pseudotumours. In:
Rodriguez-Merchan EC, Goddard NJ, Lee CA, editors.
Musculoskeletal aspects of hemophilia. Oxford:
Blackwell Science; 2000. P. 85-90.
6. Panotopoulos J, Ay C, Trieb K, Funovics PT,
Stockhammer V, Lang S, et al. Surgical treatment
of the haemophilic pseudotumour : a single centre
experience. Int Orthop. 2012; 36(10):2157-62.
7. Duarte ML, Duarte É􀆵 R, Solorzano EB. Hemophilic
pseudotumor – a rare complication. Rev Bras Hematol
Hemoter. 2017; 39(1):84-5.
8. Dagli M, Kutlucan A, Abusoglu S, Basturk A, Sozen M,
Kutlucan L, et al. Evaluation of bone mineral density
(BMD) and indicators of bone turnover in patients with
hemophilia. Bosnian J Basic Med Sci. 2018; 18(2):206.
9. Kamal AF, Sukrisman L, Dilogo IH, Priyamurti H,
Qomaruzzaman MN. Pelvic haemophilic pseudotumour:
a case report. J Orthop Surg. 2014; 22(2):
263-8.
10. Park JS, Ryu KN. Haemophilic pseudotumour
involving the musculoskeletal system: spectrum
of radiologic findings. Am J Roentgenol. 2004;
183(1):55-61.
11. Kamal AF, Pradana AS, Prabowo Y. Bilateral iliopsoas
haemophilic “soft tissue pseudotumours”: a case
report. Inter J Surg Case Rep. 2015; 13(1):19-23.
12. Magallon M, Monteagudo J, Altisent C, Ibanez
A, Rodriguez-Perez A, Riba J, et al. Hemophilic
pseudotumor: multicenter experience over a 25-year
period. Am J Hematol. 1994; 45(2):103-8.
13. He Y, Zhou X, Cui H, Qiu G, Weng X, Zhang B, et al.
Surgical management of haemophilic pseudotumors:
experience in a developing country. J Invest Surg.2017; 32(2):127-36.

14. Kerr R. Imaging of musculoskeletal complications
of hemophilia. Semin Musculoskelet Radiol. 2003;
7(2):127-36.
15. Maclachlan J, Gough-Palmer A, Hargunani R, Farrant
J, Holloway B. Haemophilia imaging: a review. Skelet
Radiol. 2009; 38(10):949-57.
16. Pettersson H, Ahlberg A. Computed tomography in
hemophilic pseudotumor. Acta Radiol Diagn (Stockh).
1982; 23(5):453-7.
17. Espandar R, Heidari P, Rodriguez-Merchan EC.
Management of haemophilic pseudotumours with
special emphasis on radiotherapy and arterial
embolization. Haemophilia. 2009; 15(2):448-57.
18. Lim MY, Nielsen B, Ma A, Key NS. Clinical features and
management of hemophilic pseudotumors: a single US
centre experience over a 30-year period. Hemophilia.
2014; 20(1):e58-62.
19. 19. Rodriguez-Merchan EC. What´s new in orthopedic
surgery for people with hemophilia. Arch Bone J Surg.
2018; 6(3):157-60.
20. 20. Keller A, Terrier F, Schneider PA, Bianchi S, Howarth
N, De Moerloose P. Pelvic hemophilic pseudotumor:
management of a patient with high level of inhibitors.
Skeletal Radiol. 2002; 31(5):550-3.
21. Meer JV, Horn JV. Excision of a haeophilic pseudotumour
of the ilium, complicated by fistulation. Haemophilia.
1998; 4(2):132-5.
22. Dagli M, Kutlucan A, Abusoglu S, Basturk A, Sozen M,
Kutlucan L, et al. Evaluation of bone mineral density
(BMD) and indicators of bone turnover in patients with
hemophilia. Bosn J Basic Med Sci. 2018; 18(2):206-10.
23. Kang JO, Cho YJ, Yoo MC, Hong SE. Hemophilic
pseudotumor of the ulna treated with low dose
radiation therapy: a case report. J Korean Med Sci.
2000; 15(5):601-3.
24. Rodriguez-Merchan EC. Haemophilic cysts (pseudotumours).
Haemophilia. 2002; 8(3):393-401.
25. Rodriguez-Merchan EC. The haemophilic
pseudotumour. Haemophilia. 2002; 8(1):12-6.
26. Ozbek N, Unsal M, Kara A, Gumruk F, Gurgey A.
Treatment of hemophilic pseudotumor with lowdoseradiotherapy.
Turk J Pediatr. 1996; 38(1):91-4.
27. Issaivanan M, Shrikande MP, Mahapatra M, Choudhry
VP. Management of hemophilic pseudotumor of
thumb in a child. J Pediatr Hematol Oncol. 2004;
26(2):128-32.

28. Pisco JM, Garcia VL, Martins JM, Mascarenhas
AM. Hemophilic pseudotumor treated with
transcatheterarterial embolization: case report.
Angiology. 1990; 41(12):1070-4.
29. Bellinazzo P, Silvello L, Caimi MT, Baudo F, deCataldo F.
Novel surgical approach to pseudotumour of ilium in
haemophilia. Lancet. 1989; 2(8675):1333-4.