Generic placeholder image

Cardiovascular & Hematological Disorders-Drug Targets

Editor-in-Chief

ISSN (Print): 1871-529X
ISSN (Online): 2212-4063

Review Article

Complications of Muscle Hematomas in Hemophilia

Author(s): E. Carlos Rodriguez-Merchan*

Volume 20, Issue 4, 2020

Page: [242 - 248] Pages: 7

DOI: 10.2174/1871529X20666200415121409

Price: $65

Abstract

Prevention is essential for avoiding the complications of muscle hematomas (compartment syndrome, pseudotumors and peripheral nerve lesions) in hemophilic patients. This is achieved through early diagnosis of muscle hematomas and proper long-term hematological treatment until they have resolved (confirmed by image studies). Ultrasound-guided percutaneous drainage could be beneficial in terms of achieving better and faster symptom relief. Acute compartment syndrome (ACS) requires emergency surgical treatment (decompression fasciotomy). As for pseudotumors, the biopsy will help us confirm the diagnosis and rule out true tumors (chondrosarcoma, liposarcoma, synovial sarcoma) that sometimes mimic hemophilic pseudotumors. Surgical removal of hemophilic pseudotumors is the best solution. As alternatives, there are curettage and filling with cancellous bone and radiotherapy (when surgery is contraindicated). Preoperative arterial embolization (ideally 2 weeks before surgery) helps control intraoperative bleeding during surgery for giant pelvic pseudotumors. Peripheral nerve injuries, which are rare, almost always occur due to compression of hematomas in the vicinity. In most cases, they usually resolve with hematological treatment only. If such treatment fails, surgery would be indicated.

Keywords: Hemophilia, muscle hematomas, pseudotumors, acute compartment syndrome, peripheral nerve injuries, diagnosis, treatment.

Graphical Abstract

[1]
De la Corte-Rodriguez, H.; Rodriguez-Merchan, E.C. Treatment of muscle haematomas in haemophiliacs with special emphasis on percutaneous drainage. Blood Coagul. Fibrinolysis, 2014, 25(8), 787-794.
[http://dx.doi.org/10.1097/MBC.0000000000000159] [PMID: 24914744]
[2]
Rodriguez-Merchan, E.C. The haemophilic pseudotumour. Haemophilia, 2002, 8(1), 12-16.
[http://dx.doi.org/10.1046/j.1365-2516.2002.00577.x] [PMID: 11886459]
[3]
Rodriguez-Merchan, E.C. Acute compartment syndrome in haemophilia. Blood Coagul. Fibrinolysis, 2013, 24(7), 677-682.
[http://dx.doi.org/10.1097/MBC.0b013e3283631e1a] [PMID: 24064900]
[4]
Rodriguez-Merchan, E.C. Peripheral nerve injuries in haemophilia. Blood Transfus., 2014, 12, s313-s318.
[PMID: 23245720]
[5]
Rodriguez-Merchan, E.C. Musculo-skeletal manifestations of haemophilia. Blood Rev., 2016, 30(5), 401-409.
[http://dx.doi.org/10.1016/j.blre.2016.04.008] [PMID: 27166435]
[6]
Rubinstein, A.J.; Ahmed, I.H.; Vosbikian, M.M. Hand compartment syndrome. Hand Clin., 2018, 34(1), 41-52.
[http://dx.doi.org/10.1016/j.hcl.2017.09.005] [PMID: 29169596]
[7]
Donaldson, J.; Goddard, N. Compartment syndrome in patients with haemophilia. J. Orthop., 2015, 12(4), 237-241.
[http://dx.doi.org/10.1016/j.jor.2015.05.007] [PMID: 26566325]
[8]
Caviglia, H.; Candela, M.; Landro, M.E.; Douglas Price, A.L.; Neme, D.; Galatro, G.A. Haemophilia pseudotumours in patients with inhibitors. Haemophilia, 2015, 21(5), 681-685.
[http://dx.doi.org/10.1111/hae.12632] [PMID: 25688657]
[9]
Rodriguez-Merchan, E.C.; Jimenez-Yuste, V. The role of selective angiographic embolization of the musculo-skeletal system in haemophilia. Haemophilia, 2009, 15(4), 864-868.
[http://dx.doi.org/10.1111/j.1365-2516.2009.02015.x] [PMID: 19320749]
[10]
Espandar, R.; Heidari, P.; Rodriguez-Merchan, E.C. Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization. Haemophilia, 2009, 15(2), 448-457.
[http://dx.doi.org/10.1111/j.1365-2516.2008.01942.x] [PMID: 19175421]
[11]
Rodriguez-Merchan, E.C. Haemophilic cysts (pseudotumours). Haemophilia, 2002, 8(3), 393-401.
[http://dx.doi.org/10.1046/j.1365-2516.2002.00609.x] [PMID: 12010440]
[12]
Valentino, L.A.; Martinowitz, U.; Doolas, A.; Murali, P. Surgical excision of a giant pelvic pseudotumour in a patient with haemophilia A. Haemophilia, 2006, 12(5), 541-544.
[http://dx.doi.org/10.1111/j.1365-2516.2006.01318.x] [PMID: 16919086]
[13]
Rijal, L.; Neogi, D.S.; Ansari, M.T.; Khan, S.A.; Yadav, C.S. Hemophilic pseudotumor--is there a role of radiotherapy? Literature review and a case report. Nepal Med. Coll. J., 2010, 12(3), 193-197.
[PMID: 21446372]
[14]
Hampton, K.K.; Grant, P.J.; Johnston, D.; Prentice, C.R. Pelvic haemophilic pseudotumour occurring in a patient with mild haemophilia: A brief report. Blood Coagul. Fibrinolysis, 1990, 1(6), 747-748.
[PMID: 2133254]
[15]
Marisavljević, D.; Glisić, M.; Elezović, I.; Popović, A.; Rolović, Z. [Successfull extirpation of femoral pseudotumour in a patient with severe haemophilia A and an inhibitor to factor VIII]. Srp. Arh. Celok. Lek., 1991, 119(11-12), 338-342. [Successfull extirpation of femoral pseudotumour in a patient with severe haemophilia A and an inhibitor to factor VIII]. [Article in Serbian].,
[PMID: 17974380]
[16]
Heim, M.; Horoszowski, H.; Schulman, S.; Varon, D.; Barzilai, A.; Engelberg, S.; Martinowitz, U. Multifocal pseudotumour in a single limb. Haemophilia, 1997, 3(1), 50-53.
[http://dx.doi.org/10.1046/j.1365-2516.1997.00072.x] [PMID: 27214620]
[17]
Maliekel, K.; Rana, N.; Green, D. Recombinant factor VIIa in the management of a pseudotumour in acquired haemophilia. Haemophilia, 1997, 3(1), 54-58.
[http://dx.doi.org/10.1046/j.1365-2516.1997.00075.x] [PMID: 27214621]
[18]
Ishiguro, N.; Iwahori, Y.; Kato, T.; Ito, T.; Kojima, T.; Takamatsu, J.; Iwata, H. The surgical treatment of a haemophilic pseudotumour in an extremity: a report of three cases with pathological fractures. Haemophilia, 1998, 4(2), 126-131.
[http://dx.doi.org/10.1046/j.1365-2516.1998.00163.x] [PMID: 9873851]
[19]
Heeg, M.; Smit, W.M.; van der Meer, J.; van Horn, J.R. Excision of a haemophilic pseudotumour of the ilium, complicated by fistulation. Haemophilia, 1998, 4(2), 132-135.
[http://dx.doi.org/10.1046/j.1365-2516.1998.00165.x] [PMID: 9873852]
[20]
Sevilla, J.; Alvarez, M.T.; Hernández, D.; Canales, M.; De Bustos, J.G.; Magallón, M.; Garzón, G.; Hernández-Navarro, F. Therapeutic embolization and surgical excision of haemophilic pseudotumour. Haemophilia, 1999, 5(5), 360-363.
[http://dx.doi.org/10.1046/j.1365-2516.1999.00330.x] [PMID: 10583519]
[21]
Raj, P.; Wilde, J.T.; Oliff, J.; Drake-Lee, A.B. Nasal haemophilic pseudotumour. J. Laryngol. Otol., 1999, 113(10), 924-927.
[http://dx.doi.org/10.1017/S002221510014561X] [PMID: 10664712]
[22]
Heaton, D.C.; Robertson, R.W.; Rothwell, A.G. Iliopsoas haemophiliac pseudotumours with bowel fistulation. Haemophilia, 2000, 6(1), 41-43.
[http://dx.doi.org/10.1046/j.1365-2516.2000.00349.x] [PMID: 10632740]
[23]
Sagarra, M.; Lucas, M.; De La Torre, E.; Almagro, D.; González, R.; García, T.; Menéndez, A.; González, A. Successful surgical treatment of haemophilic pseudotumour, filling the defect with hydroxyapatite. Haemophilia, 2000, 6(1), 55-56.
[http://dx.doi.org/10.1046/j.1365-2516.2000.00344.x] [PMID: 10632744]
[24]
Bellinazzo, P.; Silvello, L.; Caimi, T.M.; Mostarda, G.; Decataldo, F.; Baudo, F. Long-term evaluation of a novel surgical approach to the pseudotumour of the ilium in haemophilia: Exeresis and transposition of the omentum in the residual cavity. Haemophilia, 2000, 6(6), 702-704.
[http://dx.doi.org/10.1046/j.1365-2516.2000.00441.x] [PMID: 11122401]
[25]
Kale, H.A.; Rathod, K.R.; Prasad, S.R.; Madiwale, C.M.; Sheth, R.J. Mandibular haemophilic pseudotumour containing a fluid-fluid level. Br. J. Radiol., 2001, 74(878), 186-188.
[http://dx.doi.org/10.1259/bjr.74.878.740186] [PMID: 11718393]
[26]
Wexler, S.; Edgar, M.; Thomas, A.; Learmonth, I.; Scott, G. Pseudotumour in von Willebrand disease. Haemophilia, 2001, 7(6), 592-594.
[http://dx.doi.org/10.1046/j.1365-2516.2001.00552.x] [PMID: 11851759]
[27]
Gupta, S.; Mohapatra, B.B.; Ghai, S.; Seith, A.; Kashyap, R.; Sharma, R.; Choudhry, V.P. Haemophilic pseudotumour of the paranasal sinuses: Management with radiotherapy and factor replacement therapy. Haemophilia, 2001, 7(6), 595-599.
[http://dx.doi.org/10.1046/j.1365-2516.2001.00566.x] [PMID: 11851760]
[28]
O’Connell, N.; Chen, J.; Byrne, M.; O’Shea, E.; Smyth, H.; Smith, O.P. Massive pseudotumour resection with recombinant factor VIIa (NovoSeven) cover. Br. J. Haematol., 2002, 116(3), 645-648.
[http://dx.doi.org/10.1046/j.0007-1048.2001.03297.x] [PMID: 11849225]
[29]
Stevenson, D.S.; Keast, A.T. An unusual cause of epistaxis: A haemophilic pseudotumour in a non-haemophiliac, arising in a paranasal sinus. J. Laryngol. Otol., 2002, 116(4), 294-295.
[http://dx.doi.org/10.1258/0022215021910573] [PMID: 11945193]
[30]
Eby, C.S.; Caracioni, A.A.; Badar, S.; Joist, J.H. Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease. Haemophilia, 2002, 8(2), 136-141.
[http://dx.doi.org/10.1046/j.1365-2516.2002.00588.x] [PMID: 11952849]
[31]
Keller, A.; Terrier, F.; Schneider, P.A.; Bianchi, S.; Howarth, N.; De Moerloose, P. Pelvic haemophilic pseudotumour: Management of a patient with high level of inhibitors. Skeletal Radiol., 2002, 31(9), 550-553.
[http://dx.doi.org/10.1007/s00256-002-0518-8] [PMID: 12195512]
[32]
Takedani, H.; Mikami, S.; Kawasaki, N.; Abe, Y.; Arai, M.; Naka, H.; Yoshioka, A. Excision of pseudotumour in a patient with haemophilia A and inhibitor managed with recombinant factor VIIa. Haemophilia, 2004, 10(2), 179-182.
[http://dx.doi.org/10.1111/j.1365-2516.2004.00784.x] [PMID: 14962208]
[33]
Libby, E.N.; White, G.C. Cranial pseudotumour in haemophilia. Haemophilia, 2004, 10(2), 186-188.
[http://dx.doi.org/10.1046/j.1365-2516.2003.00848.x] [PMID: 14962210]
[34]
O’Dowd, M.; Geoghegan, T.; Munk, P.L.; McAuley, G.; Torreggiani, W.C. Haemophilic pseudotumour presenting with large bowel obstruction. Australas. Radiol., 2006, 50(4), 386-388.
[http://dx.doi.org/10.1111/j.1440-1673.2006.01607.x] [PMID: 16884430]
[35]
Ahuja, S.P.; Sidonio, R., Jr; Raj, A.B.; Bertolone, S.J.; Silverman, C.; Antekeier, D.P.; Fallat, M.E. Successful combination therapy of a proximal haemophilic pseudotumour with surgery, radiation and embolization in a child with mild haemophilia A. Haemophilia, 2007, 13(2), 209-212.
[http://dx.doi.org/10.1111/j.1365-2516.2006.01425.x] [PMID: 17286777]
[36]
Rey, E.A.; Puia, S.; Bianco, R.P.; Pinto, M.T. Haemophilic pseudotumour of the mandible: Report of three cases. Int. J. Oral Maxillofac. Surg., 2007, 36(6), 552-555.
[http://dx.doi.org/10.1016/j.ijom.2006.10.002] [PMID: 17467240]
[37]
Al Saadi, A.S.; Al Wadan, A.H.; El Hamarneh, S.A.; Emad, M.E. Life-threatening biopsy of an iliopsoas pseudotumour in a patient with haemophilia: A case report. J. Med. Case Reports, 2008, 2, 135.
[http://dx.doi.org/10.1186/1752-1947-2-135]] [PMID: 18447900]
[38]
D’Young, A.I. Conservative physiotherapeutic management of chronic haematomata and haemophilic pseudotumours: Case study and comparison to historical management. Haemophilia, 2009, 15(1), 253-260.
[http://dx.doi.org/10.1111/j.1365-2516.2008.01855.x] [PMID: 18783440]
[39]
Toepfer, A.; Diehl, P.; Gradinger, R.; Rudert, M. Haemophilic pseudotumour of the distal femur - a case report and characterisation of this entity. Z. Orthop. Unfall., 2008, 146(5), 651-654.Article in German.
[http://dx.doi.org/10.1055/s-2008-1038837] [PMID: 18846494]
[40]
Shi, H.; Wang, S.; Wang, P.; Yu, Q. Haemophilic pseudotumour of the mandible. Dentomaxillofac. Radiol., 2009, 38(3), 182-184.
[http://dx.doi.org/10.1259/dmfr/28176102] [PMID: 19225091]
[41]
Bernstaedt, M.; Kunze, C.; Brandt, J.; Hasenmueller, S.; Oldenburg, J.; Koerholz, D.; Mauz-Koerholz, C. Haemophiliac pseudotumour: two case reports of patients with moderate haemophilia A. Klin. Padiatr., 2009, 221(3), 172-173.
[http://dx.doi.org/10.1055/s-0029-1220704] [PMID: 19437366]
[42]
Petratos, D.V.; Pergantou, H.; Matsinos, G.S.; Anastasopoulos, J.N.; Platokouki, H. Intraosseous pseudotumour of the talus in a child with severe haemophilia. J. Pediatr. Orthop. B, 2009, 18(6), 357-361.
[http://dx.doi.org/10.1097/BPB.0b013e32832f07d4] [PMID: 19593217]
[43]
Yoshitake, Y.; Nakayama, H.; Takamune, Y.; Yasunaga, M.; Hiraki, A.; Shinohara, M. Haemophilic pseudotumour of the mandible in a 5-year-old patient. Int. J. Oral Maxillofac. Surg., 2011, 40(1), 120-123.
[http://dx.doi.org/10.1016/j.ijom.2010.05.013] [PMID: 20709496]
[44]
Liu, B.; Liu, L.; Feng, Y.; Li, L. A case report on the surgical treatment of the huge inflammatory pseudotumor in the AIDS patient with hemophilic. Case Rep. Pathol., 2011, 2011798649
[http://dx.doi.org/10.1155/2011/798649]] [PMID: 22937392]
[45]
Yang, B.T.; Wang, Y.Z.; Wang, X.Y.; Wang, Z.C. Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature. Br. J. Radiol., 2012, 85(1016), 1107-1111.
[http://dx.doi.org/10.1259/bjr/12938443] [PMID: 22253339]
[46]
Panotopoulos, J.; Ay, C.; Trieb, K.; Funovics, P.T.; Stockhammer, V.; Lang, S.; Holinka, J.; Windhager, R.; Pabinger, I.; Wanivenhaus, H.A. Surgical treatment of the haemophilic pseudotumour: A single centre experience. Int. Orthop., 2012, 36(10), 2157-2162.
[http://dx.doi.org/10.1007/s00264-012-1593-7] [PMID: 22752668]
[47]
Serban, M.; Mihailov, D.; Savescu, D.; Bordos, D.; Talpos Niculescu, S.; Jinca, C.; Lăcătuşu, A.; Siebeck, M.; Schramm, W. Long-term outcome of an unusual haemophilic pseudotumour. Hamostaseologie, 2012, 32, S43-S44.
[http://dx.doi.org/10.1055/s-0037-1619774] [PMID: 22960649]
[48]
Lim, M.Y.; Nielsen, B.; Ma, A.; Key, N.S. Clinical features and management of haemophilic pseudotumours: A single US centre experience over a 30-year period. Haemophilia, 2014, 20(1), e58-e62.
[http://dx.doi.org/10.1111/hae.12295] [PMID: 24354486]
[49]
Low, S.F.; Sridharan, R.; Ngiu, C.S.; Haflah, N.H. Osseous haemophilic pseudotumour and concurrent primary hyperparathyroidism: A diagnostic conundrum. BMJ Case Rep., 2014, 2014bcr2013203282
[http://dx.doi.org/10.1136/bcr-2013-203282]] [PMID: 24729114]
[50]
Kamal, A.F.; Sukrisman, L.; Dilogo, I.H.; Priyamurti, H.; Qomaruzzaman, M.N. Pelvic haemophilic pseudotumour: A case report. J. Orthop. Surg. (Hong Kong), 2014, 22(2), 263-268.
[http://dx.doi.org/10.1177/230949901402200232] [PMID: 25163970]
[51]
Purkait, R.; Mukherji, A.; Dolai, T.K.; Bhadra, R. Intraosseous pseudotumour in a child with mild hemophilia B: report of a rare case and brief review of literature. Indian J. Hematol. Blood Transfus., 2014, 30, 366-368.
[http://dx.doi.org/10.1007/s12288-014-0414-0] [PMID: 25332621]
[52]
Dutt, K.; Agarwal, P.N.; Singh, R.; Tomar, V.S. Haemophilic pseudotumour: Surgical management of a rare case. Indian J. Surg., 2015, 77(1), 62-64.
[http://dx.doi.org/10.1007/s12262-013-1016-z] [PMID: 25829715]
[53]
Kwon, A.Y.; Huh, K.H.; Yi, W.J.; Symkhampha, K.; Heo, M.S.; Lee, S.S.; Choi, S.C. Haemophilic pseudotumour in two parts of the maxilla: Case report. Dentomaxillofac. Radiol., 2016, 45(6)20150440
[http://dx.doi.org/10.1259/dmfr.20150440]] [PMID: 27359071]
[54]
Kumar, S.; Charllu, A.P.; Chacko, R.; Porinchu, J. Spontaneous bone regeneration in a large haemophilic pseudotumour of mandible. BMJ Case Rep., 2018.bcr2018226088
[http://dx.doi.org/10.1136/bcr-2018-226088]
[55]
Rodriguez Merchan, E.C. The haemophilic pseudotumour. Int. Orthop., 1995, 19(4), 255-260.
[http://dx.doi.org/10.1007/BF00185235] [PMID: 8557426]
[56]
Mann, H.A.; Hilton, A.; Goddard, N.J.; Smith, M.A.; Holloway, B.; Lee, C.A. Synovial sarcoma mimicking haemophilic pseudotumour. Sarcoma, 2006, 2006, 27212.
[http://dx.doi.org/10.1155/SRCM/2006/27212] [PMID: 17251656]
[57]
Allen, D.J.; Goddard, N.J.; Mann, H.A.; Rodriguez-Merchan, E.C. Primary malignancies mistaken for pseudotumours in haemophilic patients. Haemophilia, 2007, 13(4), 383-386.
[http://dx.doi.org/10.1111/j.1365-2516.2007.01438.x] [PMID: 17610552]
[58]
Rodriguez-Merchan, E.C.; Gomez-Cardero, P. Pathological fracture of a true tumour mimicking a haemophilic pseudotumor. Haemophilia, 2005, 11(2), 188-190.
[http://dx.doi.org/10.1111/j.1365-2516.2005.01073.x] [PMID: 15810925]
[59]
Fernandez-Delgado, J.; Rodriguez-Merchan, E.C.; Martinez-Mendez, J.R. First giant gluteal neurofibroma reported in the literature in a person with haemophilia and its high risk of massive bleeding to death. Haemophilia, 2005, 11(4), 419-421.
[http://dx.doi.org/10.1111/j.1365-2516.2005.01105.x] [PMID: 16011600]
[60]
Large, D.F.; Ludlam, C.A.; Macnicol, M.F. Common peroneal nerve entrapment in a hemophiliac. Clin. Orthop. Relat. Res., 1983, (181), 165-166.
[http://dx.doi.org/10.1097/00003086-198312000-00025] [PMID: 6641048]
[61]
Kaymak, B.; Ozçakar, L.; Cetin, A.; Erol, K.; Birsin Ozçakar, Z. Concomitant compression of median and ulnar nerves in a hemophiliac patient: A case report. Joint Bone Spine, 2002, 69(6), 611-613.
[http://dx.doi.org/10.1016/S1297-319X(02)00460-8] [PMID: 12537271]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy