Central venous access devices in children with congenital coagulation disorders: complications and long-term outcome

Reliable venous access is essential to facilitate the administration of pro phylactic factor concentrate or blood products in children with congenital coagulation disorders and immune tolerance therapy (ITT) regimens in those who develop high responding inhibitors. Poor venous access is even more pro blematic in very young children, the vast majority of whom will require the insertion of central venous access devices (CVADs). Previous studies have suggested that infection rates are low and that there are few long-term com plications associated with CVAD usage. We have reviewed 86 CVADs that have been inserted, since 1988, in 58 children with congenital bleeding disorder s, aged 6 d to 16.5 years, attending Great Ormond Street Hospital, London, and the National Children's Hospital, Dublin. The devices have remained in situ for 2 weeks to 92 months (median 22.5 months). Early (0-2 weeks) compl ications of CVAD insertion included nine bleeding episodes, one extravasati on of factor concentrate, three allergic reactions to factor concentrate an d five catheter infections. Overall, CVAD infection was the commonest probl em encountered, with 52 devices (60%) becoming infected. Twenty-seven CVADs (31%) required removal, Infection rates in children without inhibitors (29 /68) were 1/20 patient-months or 1.6 infections/1000 patient-days, but infe ction rates for those with inhibitors were 1/8.5 patient-months or 4.3/1000 patient-days. Staphylococcus epidermidis was the predominant organism (25/ 52) isolated. Blockage of CVAD (four) and catheter disconnection (four) wer e the most frequently occurring non-infectious long-term complications. Ski n erosion of the port was also seen in three children, in one child at 20 m onths, in one at 29 months and in one at 34 months after insertion. This st udy demonstrates a high CVAD infection rate and highlights the long-term co mplications of CVAD usage.