Comparison of perioperative practices for placement of central venous access devices (CVAD) in children with haemophilia

Abstract Background In children with haemophilia (CwH), central venous access devices (CVADs) are frequently placed to aid in the delivery of factor concentrates. In those who develop inhibitors, CVADs also allow for easy venous access and facilitation of immune tolerance therapy. Aim In this study, we compare perioperative practices for CVAD placement in children with haemophilia to assess similarities and differences in practices across centres in two countries (Singapore and Canada). Methods Retrospective chart review was conducted involving CwH (with and without inhibitors) who underwent CVAD placement from January 2007 to September 2017 at two centres in Singapore and at one centre in Hamilton, Canada. Data obtained included demographics, operative details, preoperative investigations, perioperative factor replacement, use of bypassing agents, antibiotic and antifibrinolytic use, length of stay, complications and need for CVAD revision. Results Twenty-one CwH were included in the data analysis. Amongst those without inhibitors, the mean preoperative factor dose was 50.0 IU/kg (SD=7.6) in Singapore, and 72.4 IU/kg (SD=12.5) in Hamilton (p=0.002); mean total factor use in the perioperative period was 425.0IU/kg (SD=114.9) in Singapore and 646.8IU/kg (SD=118.1) in Hamilton (p=0.004); mean duration of clotting factor replacement was 5.3 days (SD=0.9) in Singapore and 6.9 days (SD=0.7) in Hamilton (p=0.004). Amongst those with inhibitors, the mean preoperative dose of rFVIIa was 160.5 mcg/kg (SD=99.9) in Singapore and 88.2 mcg/kg (SD=3.8) in Hamilton (p=0.244); mean total rFVIIa used from surgery to discharge was 3,008.0 mcg/kg (SD=2305.9) in Singapore and 2,640.2 mcg/kg (SD=134.1) in Hamilton (p=0.842); mean duration of rFVIIa cover was 5.3 days (SD=1.7) in Singapore and 9.5 days (SD=2.1) in Hamilton (p=0.054). None of the CwH without inhibitors developed postoperative complications, compared to 57% in those with inhibitors (p=0.006). Conclusion Amongst CwH without inhibitors, significant variations were seen in perioperative factor replacement. Amongst those with inhibitors, there were also differences in perioperative practices across centres, although not statistically significant. Across centres, CwH with inhibitors were found to have more postoperative complications.