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

Summary. Reliable venous access is essential to facilitatethe administration of prophylactic factor concentrate orblood products in children with congenital coagulationdisorders and immune tolerance therapy (ITT) regimens inthose who develop high responding inhibitors. Poor venousaccess is even more problematic in very young children,the vast majority of whom will require the insertion ofcentral venous access devices (CVADs). Previous studieshave suggested that infection rates are low and that thereare few long-term complications associated with CVADusage. We have reviewed 86 CVADs that have beeninserted, since 1988, in 58 children with congenitalbleeding disorders, aged 6 d to 16·5 years, attendingGreat Ormond Street Hospital, London, and the NationalChildren’s Hospital, Dublin. The devices have remained insitu for 2 weeks to 92 months (median 22·5 months).Early (0–2 weeks) complications of CVAD insertionincluded nine bleeding episodes, one extravasation offactor concentrate, three allergic reactions to factorconcentrate and five catheter infections. Overall, CVADinfection was the commonest problem encountered, with52 devices (60%) becoming infected. Twenty-seven CVADs(31%) required removal. Infection rates in children with-out inhibitors (29/68) were 1/20 patient–months or 1·6infections/1000 patient–days, but infection rates for thosewith inhibitors were 1/8·5 patient–months or 4·3/1000patient–days. Staphylococcus epidermidis was the predomi-nant organism (25/52) isolated. Blockage of CVAD (four)and catheter disconnection (four) were the most frequentlyoccurring non-infectious long-term complications. Skinerosion of the port was also seen in three children, inone child at 20 months, in one at 29 months and in oneat 34 months after insertion. This study demonstrates ahigh CVAD infection rate and highlights the long-termcomplications of CVAD usage.Keywords: PortaCath, haemophilia, inhibitors, prophylaxis,infection.Recurrent haemarthroses are the major cause of morbidityin children with severe (level ,0·01 iu/ml) factor VIII/IXdeficiency. Prophylactic factor concentrates given on aregular basis significantly reduce the incidence of haemar-throsis (Nilsson et al, 1992, Liesner et al, 1996) and, whencommenced early in life, long-term joint damage can beavoided (Petrini et al, 1991) or, in some cases, reversed(Liesner et al, 1996). Thus, the World Health Organization/World Federation of Haemophilia now recommend thatprophylaxis be commenced between the ages of 1 and2 years in those with severe haemophilia. The intravenousadministration of factor concentrate twice or three times perweek is fraught with difficulty in the majority of youngchildren when only using peripheral veins. Similarly,immune tolerance therapy (ITT) using large doses of factorconcentrate twice a day for 12–24 months for childrenwith inhibitors to factor VIII/IX is almost impossible withoutthe use of central venous access devices (CVADs). Childrenwith other congenital coagulation disorders also require aCVAD to facilitate home treatment programmes or bloodproduct administration. These devices can be fully implant-able (PortaCath; Deltac, USA) or partly externalized (singleor double lumen Quintan catheters). The use of a port ispreferable to an external device because it causes fewerlimitations to the child’s lifestyle, and it has been suggestedthat there is a lower risk of infection (Blanchette et al, 1997;Warrier et al, 1997)However, despite the obvious attractions of these devices,their routine use for prophylaxis and ITT has not gaineduniversal acceptance because of the potential risks of haemor-rhage (Liesner et al, 1995), thrombosis (Vidler et al, 1999)and infection (Blanchette et al, 1996; Ragni et al, 1997).