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