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Central venous access devices for paediatric patients with haemophilia: a single-institution experience
R Titapiwatanakun1, C Moir, R K Pruthi
1Division of Pediatric Hematology and Oncology, Mayo Clinic, Rochester, Minnesota, USA. rodriguez.vilmarie@mayo.edu
Insights
Central venous access devices (CVADs) enable home infusions for children with bleeding disorders. Infections are the main complication, particularly in patients with Factor VIII inhibitors, though overall rates remain low with proper technique.
Area of Science:
- Pediatric Hematology
- Vascular Access Devices
- Bleeding Disorders Management
Background:
- Central venous access devices (CVADs) are crucial for home-based factor concentrate infusions in children with bleeding disorders.
- These infusions support long-term prophylaxis and immune tolerance therapy.
Purpose of the Study:
- To review the outcomes and complications associated with CVAD use in pediatric patients with bleeding disorders.
- To evaluate the safety and efficacy of CVADs in this specific patient population.
Main Methods:
- Retrospective review of 37 CVADs placed in 18 pediatric patients at the Mayo Clinic Comprehensive Hemophilia Center.
- Data collected included patient diagnoses (hemophilia A, von Willebrand disease, hemophilia B), CVAD dwell time, and complications.
Main Results:
- A total of 45,952 CVAD days were recorded, with a median dwell time of 1361 days per device.
- Ten CVAD-related infections occurred (0.22 per 1000 days), with a higher incidence in patients with Factor VIII inhibitors.
- No clinically apparent thrombosis was observed; infection was the most common complication.
Conclusions:
- CVADs are effective for home-based therapy in pediatric bleeding disorders, but infection risk is increased in patients with inhibitors.
- Strict adherence to aseptic technique is crucial for minimizing infection rates.
- The study highlights the safety of CVADs when managed with appropriate protocols, despite the absence of routine thrombosis screening.
Abstract:
Use of a central venous access device (CVAD) can facilitate early introduction of home-based infusion of factor concentrate for long-term prophylaxis or immune tolerance therapy in children with bleeding disorders. The aim was to review outcomes associated with use of CVAD. Retrospective review of paediatric patients with bleeding disorders was observed at the Mayo Clinic Comprehensive Hemophilia Center. Thirty-seven CVAD were placed in 18 patients (haemophilia A [n = 15], type 3 von Willebrand disease [n = 2] and haemophilia B [n = 1]). Follow-up was for 45 952 CVAD days, and median time that CVAD remained in place was 1361 days per device. Factor VIII (FVIII) inhibitors were present in 4 of the 15 patients. Ten CVAD-related infections occurred (median, 672 days; range, 72-1941 days), of which six were in one patient with FVIII inhibitors. Overall infection rate was 0.22 (95% confidence interval [CI], 0.10-0.40) per 1000 CVAD days, with 0.11 infections in patients without FVIII inhibitors compared with a pooled incidence of 0.66 (95% CI, 0.44-0.97) reported in the literature. Indications for removal of 27 CVAD were blockage, change to peripheral venous access, catheter displacement, infection, leak in the port septum, short catheter and skin erosion. No clinically apparent thrombosis or sequelae of thrombosis were observed. Infection is the most common complication associated with CVAD use and is increased in patients who have inhibitors. The low rate of clinically apparent thrombosis reflects our practice of not screening for thrombosis. The low infection rate reflects our practice of using and reinforcing the aseptic technique.
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