Overview of the use of implantable venous access devices in the management of children with inherited bleeding disorders

Abstract

Frequent infusion of factor concentrates may be challenging in young boys with haemophilia, especially if their disease is complicated by inhibitors. A central venous access device (CVAD) is often placed in young patients in need of repeated infusions for prophylaxis or immune tolerance induction. Although user friendly and capable of providing reliable venous access, these devices are associated with a high complication rate over time. In the haemophilia population, major complications include CVAD-associated infections and deep venous thrombosis, which is most often silent. Established risk factors for catheter-related infection include age less than 6 years at the time of CVAD placement and use of an external CVAD when compared with a totally implantable device such as a port. Avoidance of CVAD-related infections is facilitated by strict adherence to aseptic technique. The risk of deep venous thrombosis appears related to the duration for which the catheter is in place, with the risk increasing beyond 4 years. The promotion of a strict clinic policy in which CVADs are left in place for as short a time as possible should decrease the risk of complications. In rare cases where a totally implantable CVAD cannot be placed for technical reasons, an arteriovenous fistula may provide reliable venous access. In all cases, however, venous access via peripheral veins is preferred over CVADs.

Burden of illness and costs among paediatric haemophilia patients with and without central venous access devices treated in US hospitals

“…Risk factors for infection include young age at time of placement and use of external CVADs . Although some studies report younger age at placement as a risk factor for infections, in our study, frequency of infections was higher among the older children.…”

“…Catheters impregnated with antimicrobials have also shown to reduce incidence of infections among adults . In addition, clinic policies that promote use of CVADs for as short as possible might also decrease the risk of complications . Use of arteriovenous fistula may be an option when risk of complications from CVADs is high for certain patients.…”

Central venous access device (CVAD) complications in Haemophilia with inhibitors undergoing immune tolerance induction: Lessons from the international immune tolerance study

“…For the purpose of this analysis, we have included only the 115 patients enrolled and randomized who had actually started ITI (58 LD; 57 HD). The median age of subjects at start of ITI was 15.5 months (IQR [11][12][13][14][15][16][17][18][19][20][21][22][23][24]. The demographics of this group of patients are described in the primary report [19].…”

Treatment of hemophilia: a review of current advances and ongoing issues

“…Totally implantable catheters (ports) are preferred to external CVADs because of the lower risk of complications, especially infection and thrombosis. However, CVADs should not be used routinely in children and, if required, should be removed as soon as feasible 85,86. Indeed, the development of catheter infections may cause dangerous interruptions to prophylaxis, and in particular to ITI.…”