Achmad Fauzi Kamal 1,2,3,4,5 , Agus Waryudi 1,2,3,4,5 , Aryadi Kurniawan 1,2,3,4,5 , Anna Mira Lubis 1,2,3,4,5 , Djayadiman Gatot 1,2,3,4,5 Show Affiliations »
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. 2019 © BY THE ARCHIVES OF BONE AND JOINT SURGERY.
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. 2019 © BY THE ARCHIVES OF BONE AND JOINT SURGERY.
Entities: Chemical
Keywords:
Hemophilia type-A; emophilic pseudotumor; urgical treatment
Year: 2019
PMID： 31970256 PMCID： PMC6935531
Source DB: PubMed Journal: Arch Bone Jt Surg ISSN： 2345-461X