OBJECTIVE: : Bleeding complications either spontaneously or post-operatively are very common in patients with haemophilia. Sometimes these bleeding complications remain unresponsive despite being on high dose of clotting factor replacement. The aim was to assess the role of endovascular embolization in patients with haemophilia in (a) treating haemorrhagic complications due to local causes refractory to clotting factors substitution and (b) reducing intraoperative blood loss in elective pseudotumour surgery. METHODS: : 10 patients seen between January 2000 and April 2015 with severe haemophilia A or B who had unexplained profuse persistent bleeding or required large pseudotumour excision and were taken up for digital subtraction angiography and embolization were included in the study. Data of all these patients were reviewed using the computerized hospital information system and picture archiving and communication system. Details including indications for the procedure, patient preparation for the procedure, imaging findings, details of angiography with intervention, if any, and outcome as well as follow-up data were analyzed. RESULTS: : In 6 of these 10 cases, bleeding was spontaneous, in 2 cases due to trivial fall and in 2 cases due to post-operative bleeding. Angiography in these patients revealed vascular blush, abnormal hypervascularity or active extravasation. In all 10 patients, an embolization procedure was performed, with bleeding controlled in 8 patients. There were no procedure-related complications during the procedure, post-procedure bleeding or haematoma at the site of arterial access. One patient had recurrence of bleeding for whom surgical exploration was required, and one patient had significant bleeding intraoperatively which was controlled with high-dose clotting factors, blood transfusion and fresh frozen plasma intraoperatively. CONCLUSION: : Endovascular embolization is a safe, effective and cost-saving procedure in arresting bleeding in selected patients with severe haemophilia who are unresponsive to adequate clotting factor replacement and where local vascular causes could be contributing to the bleeding. Pre-operative embolization is also a good procedure to reduce intraoperative blood loss in patients with large pseudotumours. ADVANCES IN KNOWLEDGE:: Angiography and embolization in patients with haemophilia is technically challenging and should be performed by highly skilled interventional radiologists, which limits its wider use and familiarity among multidisciplinary teams managing haemophilia. By bringing the knowledge of this effective treatment to the specialist groups who care for patients with haemophilia, its wider application may be possible which can save life and/or reduce morbidity.
OBJECTIVE: : Bleeding complications either spontaneously or post-operatively are very common in patients with haemophilia. Sometimes these bleeding complications remain unresponsive despite being on high dose of clotting factor replacement. The aim was to assess the role of endovascular embolization in patients with haemophilia in (a) treating haemorrhagic complications due to local causes refractory to clotting factors substitution and (b) reducing intraoperative blood loss in elective pseudotumour surgery. METHODS: : 10 patients seen between January 2000 and April 2015 with severe haemophilia A or B who had unexplained profuse persistent bleeding or required large pseudotumour excision and were taken up for digital subtraction angiography and embolization were included in the study. Data of all these patients were reviewed using the computerized hospital information system and picture archiving and communication system. Details including indications for the procedure, patient preparation for the procedure, imaging findings, details of angiography with intervention, if any, and outcome as well as follow-up data were analyzed. RESULTS: : In 6 of these 10 cases, bleeding was spontaneous, in 2 cases due to trivial fall and in 2 cases due to post-operative bleeding. Angiography in these patients revealed vascular blush, abnormal hypervascularity or active extravasation. In all 10 patients, an embolization procedure was performed, with bleeding controlled in 8 patients. There were no procedure-related complications during the procedure, post-procedure bleeding or haematoma at the site of arterial access. One patient had recurrence of bleeding for whom surgical exploration was required, and one patient had significant bleeding intraoperatively which was controlled with high-dose clotting factors, blood transfusion and fresh frozen plasma intraoperatively. CONCLUSION: : Endovascular embolization is a safe, effective and cost-saving procedure in arresting bleeding in selected patients with severe haemophilia who are unresponsive to adequate clotting factor replacement and where local vascular causes could be contributing to the bleeding. Pre-operative embolization is also a good procedure to reduce intraoperative blood loss in patients with large pseudotumours. ADVANCES IN KNOWLEDGE:: Angiography and embolization in patients with haemophilia is technically challenging and should be performed by highly skilled interventional radiologists, which limits its wider use and familiarity among multidisciplinary teams managing haemophilia. By bringing the knowledge of this effective treatment to the specialist groups who care for patients with haemophilia, its wider application may be possible which can save life and/or reduce morbidity.
Authors: Eveline P Mauser-Bunschoten; Jacco A C Zijl; Willem Mali; A Cees van Rinsum; H Marijke van den Berg; Goris Roosendaal Journal: Blood Date: 2004-12-21 Impact factor: 22.113
Authors: R Kulkarni; J M Soucie; J Lusher; R Presley; A Shapiro; J Gill; M Manco-Johnson; M Koerper; P Mathew; T Abshire; D Dimichele; K Hoots; R Janco; D Nugent; S Geraghty; B Evatt Journal: Haemophilia Date: 2009-07-22 Impact factor: 4.287