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Central venous access devices in children with congenital coagulation disorders: complications and long-term outcome
1National Paediatric Centre for Inherited Coagulation Disorders, National Children's Hospital, Tallaght, Dublin 24, Ireland.
Insights
Central venous access devices (CVADs) are crucial for children with bleeding disorders, but this study found a high rate of CVAD infections and long-term complications, necessitating careful management.
Area of Science:
- Pediatric Hematology
- Vascular Access Devices
- Infectious Diseases
Background:
- Reliable venous access is critical for managing congenital coagulation disorders and immune tolerance therapy (ITT) in children.
- Central venous access devices (CVADs) are often necessary for young children with poor venous access.
- Previous research suggested low complication rates for CVADs.
Purpose of the Study:
- To evaluate the complication rates, particularly infections, associated with CVADs in children with congenital bleeding disorders.
- To assess the long-term outcomes and challenges of CVAD usage in this pediatric population.
Main Methods:
- Retrospective review of 86 CVADs inserted in 58 children with congenital bleeding disorders (aged 6 days to 16.5 years).
- Data collected on device duration, complications (early and late), and causative organisms.
- Analysis of infection rates stratified by inhibitor status.
Main Results:
- Overall, 60% of CVADs (52/86) became infected, with Staphylococcus epidermidis being the most common organism.
- Infection rates were higher in children with inhibitors (4.3/1000 patient-days) compared to those without (1.6/1000 patient-days).
- Significant long-term complications included CVAD blockage (4), disconnection (4), and skin erosion (3).
Conclusions:
- This study highlights a high incidence of CVAD infections and significant long-term complications in children with congenital bleeding disorders.
- The findings underscore the need for vigilant monitoring and management strategies to mitigate risks associated with CVADs in this vulnerable population.
Abstract:
Reliable venous access is essential to facilitate the administration of prophylactic 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 problematic 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 complications associated with CVAD usage. We have reviewed 86 CVADs that have been inserted, since 1988, in 58 children with congenital bleeding disorders, 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) complications of CVAD insertion included nine bleeding episodes, one extravasation of factor concentrate, three allergic reactions to factor concentrate and five catheter infections. Overall, CVAD infection was the commonest problem 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 infection 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) were the most frequently occurring non-infectious long-term complications. Skin erosion of the port was also seen in three children, in one child at 20 months, in one at 29 months and in one at 34 months after insertion. This study demonstrates a high CVAD infection rate and highlights the long-term complications of CVAD usage.