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Magnetic Resonance Imaging of Mandibular Hemophilic Pseudotumor Associated With Factor IX Deficiency: Report of Case With Review of Literature

2011; Elsevier BV; Volume: 69; Issue: 6 Linguagem: Inglês

10.1016/j.joms.2010.07.005

1531-5053

Srinivasan Kalyanasundaram, Ankur Gadodia, Ashu Seith Bhalla, Ajoy Roy Choudhury, Ongkila Bhutia, Anand Kumar Gupta, Priti Chatterjee, Amit Kumar Dinda,

Myeloproliferative Neoplasms: Diagnosis and Treatment

Hemophilic pseudotumor (HP) is an unusual complication of hemophilia and is characterized by chronic, encapsulated, slow-growing hematomas resulting from recurrent hemorrhages. 1 Resnick D. Bleeding disorders. in: Resnick D. (ed 3). Diagnosis of Bone and Joint Disorders. Volume 4. WB Saunders, Philadelphia, PA1995 Google Scholar HP occurs in about 1% to 2% patients with severe hemophilia and was first reported by Starker 2 Starker L. Knochenusur durch ein hamophiles, subperiostales Hamatom. Mitt Grenzgeb Med Chir. 1918; 31: 381 Google Scholar in 1918. The pseudotumor usually affects the femur, pelvis, tibia, and small bones of the hands and feet. 3 Gilbert M.S. Characterizing the hemophilic pseudotumor. Ann NY Acad Sci. 1975; 240: 311 Crossref PubMed Scopus (59) Google Scholar HP of the mandible is extremely uncommon, with only 15 cases reported to date in English-literature studies 4 Lazarovitis P. Griem M.L. Radiotherapy of hemophilic pseudotumors. Radiology. 1968; 91: 1026 PubMed Google Scholar , 5 Stoneman D.W. Beierl C.D. Pseudotumor of hemophilia in the mandible Report of a case. Oral Surg Oral Med Oral Pathol. 1975; 40: 811 Abstract Full Text PDF PubMed Scopus (18) Google Scholar , 6 Mulkey T.F. Hemophilic pseudotumors of the mandible. J Oral Surg. 1977; 35: 561 PubMed Google Scholar , 7 Martinez-Lage J.L. Sanchez H. Garcia J.S. et al. A pseudo-tumour of the mandible in a haemophiliac patient A case report. J Maxillofac Surg. 1983; 11: 171 Abstract Full Text PDF PubMed Scopus (16) Google Scholar , 8 Brook A.H. Bedi R. Chan Lui W.Y. et al. Hemophilic pseudotumours of the mandible: report of a case in a one year old child. Br J Oral Maxillofac Surg. 1985; 23: 47 Abstract Full Text PDF PubMed Scopus (14) Google Scholar , 9 Bryan G.W. Leibold D.G. Triplett R.G. Hemophilic pseudotumor of the mandible Report of a case. Oral Surg Oral Med Oral Pathol. 1990; 69: 550 Abstract Full Text PDF PubMed Scopus (24) Google Scholar , 10 de Sousa S.O. de Piratininga J. Pinto Júnior D.S. et al. Hemophilic pseudotumor of the jaws: report of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995; 79: 216 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 11 Kale H.A. Rathod K.R. Prasad S.R. et al. Mandibular haemophilic pseudotumour containing a fluid-fluid level. Br J Radiol. 2001; 74: 186 PubMed Google Scholar , 12 Rey E.A. Puia S. Bianco R.P. et al. Haemophilic pseudotumour of the mandible: report of three cases. Int J Oral Maxillofac Surg. 2007; 36: 552 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar , 13 Shi H. Wang S. Wang P. et al. Haemophilic pseudotumour of the mandible. Dentomaxillofac Radiol. 2009; 38: 182 Crossref PubMed Scopus (14) Google Scholar , 14 Lee D.W. Jee Y.J. Ryu D.M. et al. Changes in a hemophilic pseudotumor of the mandible that developed over a 20-year period. J Oral Maxillofac Surg. 2009; 67: 1516 Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar (Table 1, Table 2). All the reported cases of mandibular HP, with exception of 1 case 14 Lee D.W. Jee Y.J. Ryu D.M. et al. Changes in a hemophilic pseudotumor of the mandible that developed over a 20-year period. J Oral Maxillofac Surg. 2009; 67: 1516 Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar were due to factor VIII deficiency. To the best of our knowledge, the magnetic resonance imaging findings of mandibular HP have not been previously reported. The purpose of the present study is to report a rare case of mandibular HP in 1 patient with factor IX deficiency (Christmas disease) and describe the magnetic resonance imaging findings. We have also reviewed the previously reported cases of mandibular HP. Table 1Literature Review of Hemophilic Pseudotumor of Mandible No. Reference Investigator Year Age (y) Hemophilia A/B Subtype Trauma/Dental Extraction Severity (Factor Level) Pseudotumor at Other Sites Location 1 4 Lazarovitis P. Griem M.L. Radiotherapy of hemophilic pseudotumors. Radiology. 1968; 91: 1026 PubMed Google Scholar Lazarovitis 1968 11 A Intraosseous No Mild (14%) Yes Angle 2 5 Stoneman D.W. Beierl C.D. Pseudotumor of hemophilia in the mandible Report of a case. Oral Surg Oral Med Oral Pathol. 1975; 40: 811 Abstract Full Text PDF PubMed Scopus (18) Google Scholar Stoneman 1975 13 A Osseous and subperiosteal No Not mentioned No Angle 3 6 Mulkey T.F. Hemophilic pseudotumors of the mandible. J Oral Surg. 1977; 35: 561 PubMed Google Scholar Mulkey 1977 8 A Intraosseous No Severe (≤1%) No Angle 4 6 Mulkey T.F. Hemophilic pseudotumors of the mandible. J Oral Surg. 1977; 35: 561 PubMed Google Scholar Mulkey 1977 7 A Subperiosteal No Severe (not mentioned) No Body 5 6 Mulkey T.F. Hemophilic pseudotumors of the mandible. J Oral Surg. 1977; 35: 561 PubMed Google Scholar Mulkey 1977 16 A Intraosseous No Severe (not mentioned) No Body and ramus 6 7 Martinez-Lage J.L. Sanchez H. Garcia J.S. et al. A pseudo-tumour of the mandible in a haemophiliac patient A case report. J Maxillofac Surg. 1983; 11: 171 Abstract Full Text PDF PubMed Scopus (16) Google Scholar Martinez Lage 1983 10 A Intraosseous 3 mo earlier Mild (23.5%) No Body 7 8 Brook A.H. Bedi R. Chan Lui W.Y. et al. Hemophilic pseudotumours of the mandible: report of a case in a one year old child. Br J Oral Maxillofac Surg. 1985; 23: 47 Abstract Full Text PDF PubMed Scopus (14) Google Scholar Brook 1985 1.5 A Intraosseous 1 yr earlier Severe (1%) No Bilateral 8 9 Bryan G.W. Leibold D.G. Triplett R.G. Hemophilic pseudotumor of the mandible Report of a case. Oral Surg Oral Med Oral Pathol. 1990; 69: 550 Abstract Full Text PDF PubMed Scopus (24) Google Scholar Bryan 1990 2.5 A Intraosseous Incisional biopsy Not mentioned No Ramus 9 10 de Sousa S.O. de Piratininga J. Pinto Júnior D.S. et al. Hemophilic pseudotumor of the jaws: report of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995; 79: 216 Abstract Full Text PDF PubMed Scopus (26) Google Scholar de Sousa 1995 11 A Intraosseous Tooth extraction 6 mo earlier Mild (11.5%) No Bilateral 10 11 Kale H.A. Rathod K.R. Prasad S.R. et al. Mandibular haemophilic pseudotumour containing a fluid-fluid level. Br J Radiol. 2001; 74: 186 PubMed Google Scholar Kale 2001 17 A Intraosseous No Severe (≤1%) No Body 11 12 Rey E.A. Puia S. Bianco R.P. et al. Haemophilic pseudotumour of the mandible: report of three cases. Int J Oral Maxillofac Surg. 2007; 36: 552 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Rey 2007 7 A Intraosseous 7 mo earlier Mild (20%) No Midline 12 12 Rey E.A. Puia S. Bianco R.P. et al. Haemophilic pseudotumour of the mandible: report of three cases. Int J Oral Maxillofac Surg. 2007; 36: 552 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Rey 2007 14 A Intraosseous Tooth extraction 3 d earlier Mild (11%) No Body 13 12 Rey E.A. Puia S. Bianco R.P. et al. Haemophilic pseudotumour of the mandible: report of three cases. Int J Oral Maxillofac Surg. 2007; 36: 552 Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Rey 2007 12 A Intraosseous No Mild (9.5%) Yes Body 14 13 Shi H. Wang S. Wang P. et al. Haemophilic pseudotumour of the mandible. Dentomaxillofac Radiol. 2009; 38: 182 Crossref PubMed Scopus (14) Google Scholar Shi 2009 9 A Osseous and subperiosteal 4 yr earlier Mild (14.3%) No Body and ramus 15 14 Lee D.W. Jee Y.J. Ryu D.M. et al. Changes in a hemophilic pseudotumor of the mandible that developed over a 20-year period. J Oral Maxillofac Surg. 2009; 67: 1516 Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar Lee 2009 34 B Intraosseous 20 yr earlier Severe (0.3%) No Body 16 Our case 7 B Osseous and subperiosteal 1 yr ago Moderate (6%) No Ramus, condyle and coronoid Srinivasan et al. MRI of Mandibular Hemophilic Pseudotumor. J Oral Maxillofac Surg 2011. Open table in a new tab Table 2Findings and Treatment No. Imaging Done Lesion Characteristics Periosteal Reaction Soft Tissue Treatment 1 OPG Unilocular radiolucent Not mentioned Not mentioned Radiotherapy 2 OPG Well-defined, unilocular radiolucent, trabeculated Yes Yes Curettage 3 OPG Septated radiolucency with tooth extrusion No No Curettage 4 OPG/occlusal Subperiosteal lesion with trabeculations Periosteal elevation Yes Aspiration 5 OPG Two separate radiolucent areas No No EACA 6 Radiography Radiolucency surrounded by radiopaque zone No No Enucleation 7 Radiography Loculated lucencies with cortical breach No No Factor VIII 8 OPG Well-defined, unilocular radiolucent No No Curettage 9 OPG Multilocular radiolucencies in bilateral bodies No No Curettage 10 OPG/CT Expansile lytic lesion with fluid-fluid level No No Immobilization 11 OPG Radiolucent lesion with calcifications No No Curettage 12 OPG Radiolucent lesion surrounding the alveolus No No Enucleation 13 OPG Radiolucent cyst with displacement of tooth No No Resection 14 OPG/CT Ill-defined multilocular radiolucent expansile Periosteal struts Yes Resection 15 OPG/CT Radiopaque bony expansion with a cystic cavity No No Resection Our Case OPG/CT/MRI Multilocular expansile lytic lesion YES YES Resection Abbreviations: OPG, orthpantomography; CT, computed tomography; EACA, epsilon-aminocaproic acid; MRI, magnetic resonance imaging. Srinivasan et al. MRI of Mandibular Hemophilic Pseudotumor. J Oral Maxillofac Surg 2011. Open table in a new tab Srinivasan et al. MRI of Mandibular Hemophilic Pseudotumor. J Oral Maxillofac Surg 2011. Abbreviations: OPG, orthpantomography; CT, computed tomography; EACA, epsilon-aminocaproic acid; MRI, magnetic resonance imaging. Srinivasan et al. MRI of Mandibular Hemophilic Pseudotumor. J Oral Maxillofac Surg 2011.