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Use of central venous catheters in children with severe congenital coagulopathy
R J Liesner1, A J Vora, I M Hann
1Haemophilia Comprehensive Care Centre, Great Ormond Street Hospital for Children NHS Trust, London.
Insights
Port-a-cath devices in children with severe congenital coagulopathy facilitate essential treatments like prophylaxis and immunotolerance. Despite complications such as infection and hematoma, the benefits are considered substantial by clinicians and parents.
Area of Science:
- Pediatric Hematology
- Vascular Access Devices
- Coagulation Disorders
Background:
- Severe congenital coagulopathy presents significant challenges in managing factor replacement therapy.
- Central venous access is crucial for effective prophylaxis, domiciliary treatment, and immunotolerance induction in these patients.
Purpose of the Study:
- To evaluate the safety and efficacy of port-a-cath device insertion in children with severe congenital coagulopathy.
- To assess complication rates and long-term outcomes associated with port-a-cath use in this pediatric population.
Main Methods:
- Retrospective review of 27 port-a-cath insertions in 23 children with severe congenital coagulopathy across two UK centers.
- Analysis of peri- and post-operative management, complication rates (hematoma, infection, extravasation), and long-term follow-up data.
Main Results:
- Hemostasis was achieved in most patients. Post-operative complication rate was 27%, including port-site hematoma and infection. A total of 13 infections occurred in 10 patients, with 6 requiring device removal.
- Children with factor VIII inhibitors experienced specific treatment challenges, including the use of porcine factor VIII or recombinant factor VIIa.
Conclusions:
- Port-a-cath insertion is a valuable tool for managing severe congenital coagulopathy, enabling critical therapies.
- While complications exist, the perceived benefits of port-a-cath use outweigh the risks for both clinicians and parents involved in patient care.
Abstract:
From two U.K. centres 23 children with severe congenital coagulopathy had a total of 27 port-a-cath devices inserted to facilitate factor VIII or IX prophylaxis (eight patients), domiciliary therapy (seven patients), immunotolerance (four patients), or a combination thereof (four patients). Six children had a factor VIII inhibitor at the time of insertion. The mean age at operation was 30 months, with a range of 9-76 months. The cumulative length of follow-up is 639 months with a mean of 27.8 months and a range of 5-79 months. Haemostasis was achieved peri- and post-operatively with high-purity concentrate in the majority of patients without an inhibitor. All those with an inhibitor had porcine factor VIII, except one who had recombinant factor VIIa. The post-operative complication rate was 27% (6/23): three had a port-site haematoma (one required removal and replacement), two had post-operative infection, and one had swelling caused by extravasation. To date there have been 13 documented infections in 10/23 patients (five with inhibitor): a rate of 0.24 per follow-up year or 0.67 per 1000 patient-days. Six were caused by Gram-positive and seven by Gram-negative organisms. Six infections could not be eradicated by antibiotics and the port-a-cath system had to be removed; in three it was replaced by a second port-a-cath. Although there are risks involved in the use of port-a-caths in this population, both clinicians and parents involved in the care of these children believe that the benefits are considerable and the potential hazards are acceptable.