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Long-term vascular access for infants with moderate to severe osteogenesis imperfecta
Courtney L Devin1, Emily Sagalow1, Annalise Penikis1
1Department of Surgery, Sidney Kimmel Medical College at Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Insights
Port-a-caths provide safe and effective central venous access for infants and children with Osteogenesis Imperfecta (OI), facilitating essential bisphosphonate treatment and minimizing fracture risks during IV access attempts.
Area of Science:
- Pediatric Surgery
- Genetics
- Vascular Access
Background:
- Osteogenesis Imperfecta (OI) is a genetic disorder causing extreme skeletal fragility in children.
- Intravenous bisphosphonate therapy is crucial for managing severe OI but requires reliable vascular access.
- Frequent intravenous (IV) access attempts in fragile pediatric patients with OI can lead to fractures.
Purpose of the Study:
- To evaluate the safety and efficacy of port-a-cath placement in pediatric patients with Osteogenesis Imperfecta.
- To assess the incidence of complications and long-term outcomes associated with port-a-caths in this population.
- To provide data supporting the use of port-a-caths for children with OI requiring long-term IV therapy.
Main Methods:
- Retrospective review of pediatric patients diagnosed with Osteogenesis Imperfecta who underwent port-a-cath placement between 1999 and 2018.
- Analysis of complication rates including infection, thrombosis, skin erosion, and device malfunction.
- Evaluation of the need for reoperation or replacement and duration of device use.
Main Results:
- Port-a-caths were implanted in 17 pediatric patients with OI (median age 8 months).
- Devices remained functional for a median of 53.5 months, with low rates of serious complications (e.g., one infection, one thrombosis).
- Most complications were managed without reoperation, and many devices were electively removed once no longer needed.
Conclusions:
- Port-a-cath placement is a safe and effective procedure for establishing durable central venous access in pediatric patients with Osteogenesis Imperfecta.
- Utilizing port-a-caths facilitates reliable intravenous bisphosphonate delivery, crucial for managing OI.
- This approach significantly reduces the risk of iatrogenic fractures associated with repeated IV access attempts in fragile children.
Purpose:
Osteogenesis imperfecta (OI) is a genetic disorder that causes skeletal fragility. For the most fragile infants and young children with OI, intravenous (IV) bisphosphonate administration is essential, but IV access attempts often cause fractures. Port-a-caths help prevent these events, but some surgeons are hesitant to insert these devices in these infants due to lack of data on their safety.
Methods:
Retrospective study of pediatric patients with OI who underwent port-a-cath placement from 1999 to 2018; incidence of complications such as infection and thrombosis and need for reoperation or replacement are described.
Results:
Port-a-caths were placed in 17 patients with OI (median age, 8 mos [5-23 mos]; median weight, 5.8 kg [3.96-9.08 kg]) and remained in place for a median of 53.5 mos (10-127 mos). One port-a-cath was replaced because of thrombosis. Two port-a-caths were removed because of malfunction, one for skin erosion, and one for infection. In these five cases, replacement was not needed because patients could safely tolerate IV access. Two patients have their port-a-cath in place and the remaining ten patients had theirs removed electively as it was no longer needed.
Conclusion:
Port-a-cath placement in pediatric patients with OI is safe and efficacious for durable central access, enabling reliable IV bisphosphonate delivery and reducing iatrogenic trauma.
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