Intraosseous venous malformation of the maxilla after enucleation of a hemophilic pseudotumor: A case report

BACKGROUND: Hemophilic pseudotumor (HP) is a rare complication in patients with hemophilia. The lesion most frequently occurs in the long bones, pelvis, small bones of the hands and feet, or rarely in the maxillofacial region. Postoperative changes in HP are seldom arrested, whereas angiogenesis characterized by disturbed wound healing in HP may cause vascular malformations. CASE SUMMARY: We report the case of an 11-year-old boy who was affected by maxillary intraosseous venous malformation. Enucleation of an HP without factor replacement was performed initially on the right side of the maxilla 3 years ago. The patient was referred to us because of painless swelling in the same location. Factor replacement and subtotal maxillectomy were performed. Pathological examinations revealed intraosseous venous malformation. CONCLUSION: This study is the first to document the development of intraosseous venous malformation after enucleation of an HP in the maxillofacial region. Angiogenesis characterized by disturbed wound healing in patients with hemophilia may be pivotal in the pathogenesis of this condition.