Central venous access devices in children with hemophilia: an update.

The most frequent indication for placement of a central venous access device in hemophiliacs is in very young boys (ages 1-2 years) with severe hemophilia who are started on a program of long-term factor prophylaxis designed to eliminate target joint bleeding and the development of chronic musculoskeletal disease. Although expensive, this strategy is extremely successful. It involves intravenous infusion of 25-40 factor units per kg on alternate days (minimum 3 times a week) for boys with severe hemophilia A, and twice a week for boys with severe hemophilia B. To facilitate this prophylaxis regimen some hemophilia clinics routinely recommend placement of a central venous access device; others, more concerned about associated complications such as sepsis, stress the importance of using peripheral veins wherever possible, with central access devices reserved for occasional, selected cases only. A decision to use such a device should only be made after discussion of the risks/benefits with parents (or guardians) and with patients if of an appropriate age. If such a system is to be used, we recommend that a totally implantable device (Port-A-Cath) be placed because of the lower risk of infection, and because totally implantable devices allow children to take part in activities such as swimming. Important complications include catheter-related sepsis, which may occur in 25% or more of devices over time and, much less frequently, catheter-related deep vein thrombosis.