Contrast venography in young Haemophiliacs with implantable central venous access devices

Abstract

To assess the risk of deep vein thrombosis in haemophiliacs with long-term central venous catheters, we studied haemophiliacs followed at our centre with implantable venous access devices (ports) in place for > 6 months. Medical records were reviewed for a history of catheter-related complications. Each patient was examined for physical stigmata of thrombosis. Patency of the vessels was evaluated by contrast venography. Of 21 males with ports, 19 had factor VIII deficiency and two factor IX deficiency. Nineteen ports were evaluable (i.e. were in place for > 6 months). Seventeen patients have their original ports in place; two ports were replaced for mechanical dysfunction (1) and recurrent infection (1). Difficulty withdrawing or infusing occurred with three ports, two of which were cleared with urokinase. Physical examination was normal on all 19 patients. Venograms were performed in 13 of 19 patients. Parents of the remaining six patients refused venography because of the need for peripheral venipuncture. One patient had a small nonocclusive thrombus on the same side as his functioning catheter, and another had minimal narrowing of the subclavian vein at the site of a prior catheter. The overall prevalence of clinically relevant upper venous system thrombosis identifiable by contrast venography was zero (95% CI, 0-23%). We conclude that haemophiliacs do not have as high a risk of thrombosis as other populations of patients with central venous catheters. The theoretical risk of thrombosis should not preclude use of central venous catheters in patients with haemophilia.

Catheter-related deep venous thrombosis in children with hemophilia

“…Although we initially reported that catheter-related thrombosis is rare in hemophilia patients, 14 with longer follow-up we now realize that DVT is indeed common. Approximately half of our patients with CVCs in place for more than 1 year now have evidence of DVT.…”

“…14 None had definitive radiographic evidence of DVT in the upper venous system (the subclavian, brachiocephalic, or jugular veins, or the superior vena cava) despite having had catheters in place for 10 to 60 months (mean, 23 months). However, a recent abstract 15 and several case studies 16,17 indicate that thrombosis does occur in patients with hemophilia.…”

“…We used contrast venography to identify thrombi because it is the "gold standard" for detection of upper venous system thrombosis. 8,14 Patients, materials, and methods…”

“…In particular, we attempted to re-evaluate each of the 13 patients whom we had previously studied. 14 The potential risks of radiation exposure and contrast injection were discussed with the patients and their parents. No parent declined to have his or her child evaluated, and informed consent was obtained in each case.…”

Haemophilia in the first years of life

“…This is in most instances harmless but may theoretically progress to a micro-thrombosis without clinical significance or a radiologically evident thrombosis with or without clinical symptoms. During the first 10 years of Port-A-Cath usage in haemophilia, there were few reports of thromboses and many publications did not report any thromboses despite long follow-up periods [50,55,59,60]. Most of these studies were retrospective and venography was usually not routinely performed.…”