Central venous catheters (CVCs) are a common adjunct to hemophilia therapy,
but the risk of CVC-related deep venous thrombosis (DVT) in hemophiliacs i
s not well defined. In a previous study, 13 patients with CVCs had no radio
graphic evidence of DVT. However, recent abstracts and case studies demonst
rate that DVT does occur. Therefore, this study sought to determine the fre
quency of DVT in children with hemophilia and long-term CVCs and to correla
te venographic findings with clinical features. All hemophilia patients wit
h tunneled subclavian CVCs in place for 12 months or more were candidates f
or evaluation. Patients were examined for physical signs of DVT and questio
ned about catheter dysfunction. Contrast venograms were obtained to identif
y DVT. Fifteen boys with severe hemophilia were evaluated, including 9 from
the initially studied group of 13. Eight patients had evidence of DVT, 5 o
f whom previously had normal venograms. Five of 15 patients had clinical pr
oblems related to the CVC, all of whom had DVT. Four of 15 patients had sug
gestive physical signs; 3 had DVT. The mean duration of catheter placement
for all patients was 57.5 months (range, 12-102 months). For patients with
DVT, the mean duration was 66.6 +/- 7.5 months, compared to 49.5 +/- 7.2 mo
nths for patients without DVT (P = .06). No patient whose CVC was in place
fewer than 48 months had an abnormal venogram. Many hemophilia patients wit
h CVCs develop DVT of the upper venous system, and the risk increases with
duration of catheter placement. (C) 2001 by The American Society of Hematol
ogy.