A prospective clinical trial of implantable central venous access in children with haemophilia

To assess the risks associated with the use of central venous ports in children with haemophilia, 15 HIV-negative patients were prospectively evaluated. Port insertion was required for immune tolerance in two inhibitor patients and continuous prophylaxis in 13 patients with severe factor VIII deficiency, for whom surgery was covered with recombinant factor VIII (rFVIII), then given daily at home until day 6. One inhibitor patient (titre 7BU/ml) received high-dose rFVIII by continuous infusion until day 3, followed by an immune tolerance treatment scheme; the other (titre 12 BU/ml) was given recombinant activated factor VII by continuous infusion until day 7. After training on the use of the port, all patients continued their infusion programme at home. All ports remained in place for a median period of 413d (range 125-509). The median number of entries into the port was 184 (range 53-567). Port-site haematoma and infection occurred in one patient on day 7 when an inhibitor became detectable (titre 12 BU/ml). An infectious complication occurred in another patient after 310d. The port infection rate was 0-42 per 1000 patient-days (0.33 per 1000 entries into the port). This protocol for port placement with short hospitalization appears feasible and safe.