Central venous access devices in haemophilia

Abstract

Central venous access devices (CVADs) can facilitate repeated and/or urgent administration of coagulation factors in haemophilic patients. We conducted a systematic review and meta-analysis of complication rates and risk factors for poor outcome. Forty-eight studies with a total of 2704 patients and 2973 CVADs were included. The primary indications for CVADs were immune tolerance therapy (34.9% of patients), difficult venous access (31.8%) and prophylaxis (29.1%). Fully implanted CVADs were employed in 77.4% of cases and external CVADs in 22.6%. A total of 1190 infections were reported, and the pooled incidence of infection was 0.66 per 1000 CVAD days [confidence interval (CI), 0.44-0.97 per 1000 CVAD days]. Among patients developing infection, the pooled time to first infection was 295 days (CI, 181-479 days). Presence of inhibitors was an independent risk factor for infection with an incidence rate ratio (IRR) of 1.67 (CI, 1.15-2.43). Infection was less likely in patients >6 years of age (IRR, 0.46; CI, 0.27-0.79) and recipients of fully implanted CVADs (IRR, 0.31; CI, 0.12-0.86). Available information on thrombosis was limited, with only 55 cases being reported. Eventually, 31.3% of CVADs were removed, and infection was the reason for removal in 69.9% of cases and thrombosis in 4.1%. The pooled time period CVADs remained indwelling prior to removal or the expiration of the study observation period was 578 days per CVAD (CI, 456-733 days per CVAD). CVADs can confer major benefits in patients with haemophilia requiring long-term venous access, and serious complications are rare.

Environmental risk factors for inhibitor development in children with haemophilia A: a case–control study

“…Invasive procedures during the antenatal/perinatal period and FVIII infusions during vaccinations or infections were evaluated for the first time in our series, showing no role on inhibitor development. Since catheter-related infections were included in the analysis, our results indirectly suggest that infections associated with the use of central venous catheters in haemophilic children (Valentino et al, 2004) are not associated with a higher inhibitor risk. FVIII treatment for severe bleeding or surgery has been suspected to influence inhibitor formation because extensive tissue damage and inflammation may trigger an antibody response against extravascular FVIII (Oldenburg et al, 2004).…”

Optimizing management of immune tolerance induction in patients with severe haemophilia A and inhibitors: towards evidence‐based approaches

“…Infections represent the major complication associated with CVAD use in haemophilia and the most common cause for their removal, with higher risk for external devices than subcutaneous ports (Ljung, 2007). This complication is more frequent in inhibitor patients (van Dijk et al, 2004;Valentino et al, 2004;Mancuso et al, 2008). A preliminary report from the I-ITI study showed that CVAD infections occurred frequently during ITI.…”

Haemophilia in the first years of life

“…However, some studies have shown a much higher frequency of infections, 1-2/ 1000 catheter-days [54,55]. In a meta-analysis of 48 studies with 2704 patients and 2973 central venous access devices (of which only 77% were implanted), the average frequency of infection was 0.66 per 1000 catheter-days [56]. It is well known that implantable devices, such as the Port-A-Cath, have a lower infection rate compared with that of external catheters.…”