Introduction And Importance: Hemophilic pseudotumor is a rare and serious complication of hemophilia, often occurring in the long bones and muscle tissue of the extremities, with an incidence of about 1-2%. However, there is no effective surgical treatment for massive hemophilia pseudotumors of the extremities. Therefore, we would like to report this case to provide new ideas and methods for the treatment of massive hemophilic pseudotumors of the extremities through resection and total femoral prosthesis reconstruction.
Case Presentation: After admission, the patient first underwent a month-long coagulation factor replacement therapy to maintain the patient's factor IX level at approximately 100%. Then, extensive resection of hemophilic pseudotumor and total femoral prosthesis reconstruction was performed in collaboration with several departments, and we collected the patient's preoperative, intraoperative and postoperative data for analysis.
Clinical Discussion: After coagulation factor replacement therapy the patient's factor IX level was significantly increased and approached 100%. The surgical procedure was uneventful, and postoperative X-ray suggested a well-positioned prosthesis with postoperative pathological features consistent with a hemophilic pseudotumor. The patient was able to move around with the assistance of a walker 14 days after surgery, and there was no recurrence after one year of follow-up.
Conclusion: For treat the massive hemophilic pseudotumors of the extremities, extensive resection and prosthetic replacement with coagulation factor replacement therapy is a safe and effective treatment that can significantly improve the prognosis and quality of life of patients.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8980738 | PMC |
http://dx.doi.org/10.1016/j.ijscr.2022.106986 | DOI Listing |
Rev Bras Ortop (Sao Paulo)
November 2024
Departamento de Cirurgia Ortopédica, Fundación Cardioinfantil, Instituto de Cardiología, Bogotá, Colômbia.
Patients with hemophilia disease have a high risk of hemorrhage. Most hemorrhages can occur in the musculoskeletal system, presenting as hematomas, or, in rare occasions, as hemophilic pseudotumors, an uncommon pathology that are often misdiagnosed as musculoskeletal tumors because of their clinical behavior and characteristics on diagnostic imaging. Despite many treatment options, surgical excision is the treatment of choice.
View Article and Find Full Text PDFSpec Care Dentist
November 2024
Department of Medicine & In-charge Hemophilia Day Care Centre, Lok Nayak Hospital and Maulana Azad Medical College, New Delhi, India.
Aim: Hemophilic pseudotumor (HP) is a very rare complication of hemophilia seen in only 1-2% of the cases. Although it is much more common in long bones, pelvis and small bones of hands and feet and very rarely involving jaw bones.
Method And Result: In the present case, the presence of a rare hemophilic pseudotumor of the mandible with the positive history of Hemophilia B justifies that the history, clinical and radiological examinations were sufficient to arrive at conclusive diagnosis precluding invasive diagnostic procedures such as biopsy hence avoiding the risk of hemorrhage, infection, or fistula.
Skeletal Radiol
July 2024
University of Texas Medical Branch, Galveston, USA.
Hemophilic pseudotumor (HP) is a rarely encountered cystic mass that forms as a result of repeated bleeding from extra-articular soft tissues. HP cases have been previously documented in several locations in the body, most commonly in the femur and pelvis. To date, no upper extremity case involving the bilateral forearms has been reported.
View Article and Find Full Text PDFJ Clin Med
April 2024
Center for Bleeding Disorders, Careggi University Hospital, 50139 Florence, Italy.
Patients with Haemophilia (PWH) need orthopaedic treatments and often they undergo surgery. Classically, PWH with inhibitors have to face such procedures earlier than other patients. Major orthopaedic surgery is not easy and complications are frequent.
View Article and Find Full Text PDFKey Clinical Message: Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding. Timely initiation of factor VIII replacement is crucial.
Abstract: Hemophilic pseudotumors are rare complications occurring in individuals with severe hemophilia, characterized by progressive cystic swellings in muscles and/or bones due to recurrent bleeding.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!