Deep venous thrombosis screening in patients with inherited bleeding disorders and central venous catheters.

Children with inherited bleeding disorders often require central venous catheters (CVCs). Although CVCs are known to be complicated by deep venous thrombosis (DVT), little is known about the timeline of DVT development or risk of post-thrombotic syndrome (PTS). The aim of this study was to determine the timeline and confirm the incidence of thrombosis in patients with bleeding disorders who have CVCs. In 2002, we instituted a screening programme to monitor for CVC-related complications in children with haemophilia and von Willebrand disease. This is a retrospective review of this cohort. All children with CVC followed up between 1 January 2000 and 1 June 2009 were evaluated for DVT every 24 months with contrast venography and Doppler sonography. An institutional PTS severity scale was utilized at each visit. Thirty-six patients had 37 CVCs placed. Thirty patients had imaging studies, with DVT observed in 14 (47%). Most cases of DVT were diagnosed at the first venogram (median CVC duration 26 months). There were no abnormal ultrasound results. Sixteen patients (44%) had clinical findings consistent with PTS, including 10 (71%) with an abnormal venogram. Dilated chest wall veins appeared to be more strongly associated with underlying DVT (positive predictive value of 0.8) than arm circumference discrepancy. Successful transition to use of peripheral veins occurred at a median of 11 months after abnormal venograms. CVC-related DVT is common in children with inherited bleeding disorders and likely occurs earlier than previously thought. Clinical signs of PTS are also common, but long-term sequelae and severity of PTS are not known.