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A survival analysis of ventricular access devices for delivery of cerliponase alfa
- Source :
- Journal of Neurosurgery: Pediatrics. 29:115-121
- Publication Year :
- 2022
- Publisher :
- Journal of Neurosurgery Publishing Group (JNSPG), 2022.
-
Abstract
- OBJECTIVE Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a rare autosomal recessive disease caused by tripeptidyl peptidase 1 enzyme deficiency. At the authors’ center, the medication cerliponase alfa is administered every 2 weeks via the intracerebroventricular (ICV) route. This requires the placement of a ventricular access device (VAD) or reservoir and frequent percutaneous punctures of this device over the child’s lifetime. In this study, the authors audited the longevity and survival of these VADs and examined the causes of device failure. METHODS A single-center survival analysis of VAD insertions and revisions (January 2014 through June 2020) was conducted. All children received cerliponase alfa infusions through a VAD. Patient characteristics and complications were determined from a prospectively maintained surgical database and patient records. For the VAD survival analysis, the defined endpoint was when the device was removed or changed. Reservoir survival was assessed using Kaplan-Meier curves and the log-rank (Cox-Mantel) test. RESULTS A total of 17 patients had VADs inserted for drug delivery; median (range) age at first surgery was 4 years 4 months (1 year 8 months to 15 years). Twenty-six VAD operations (17 primary insertions and 9 revisions) were required among these 17 patients. Twelve VAD operations had an associated complication, including CSF infection (n = 6) with Propionibacterium and Staphylococcus species being the most prevalent organisms, significant surgical site swelling preventing infusion (n = 3), leakage/wound breakdown (n = 2), and catheter obstruction (n = 1). There were no complications or deaths associated with VAD insertion. The median (interquartile range) number of punctures was 12.0 (7.5–82.0) for unrevised VADs (n = 17) versus 29.0 (6–87.5) for revised VADs (n = 9) (p = 0.70). The median survival was 301 days for revisional reservoirs (n = 9) versus 2317 days for primary inserted reservoirs (n = 17) (p = 0.019). CONCLUSIONS In the context of the current interest in intrathecal drug delivery for rare metabolic disorders, the need for VADs is likely to increase. Auditing the medium- to long-term outcomes associated with these devices will hopefully result in their wider application and may have potential implications on the development of new VAD technologies. These results could also be used to counsel parents prior to commencement of therapy and VAD implantation.
- Subjects :
- Male
Reoperation
medicine.medical_specialty
Percutaneous
Adolescent
Context (language use)
Cerliponase alfa
Catheters, Indwelling
Neuronal Ceroid-Lipofuscinoses
Interquartile range
medicine
Humans
Child
Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
Device failure
Survival analysis
business.industry
Infant
General Medicine
Recombinant Proteins
Surgery
Infusions, Intraventricular
Child, Preschool
Female
Neurosurgery
Complication
business
Subjects
Details
- ISSN :
- 19330715 and 19330707
- Volume :
- 29
- Database :
- OpenAIRE
- Journal :
- Journal of Neurosurgery: Pediatrics
- Accession number :
- edsair.doi.dedup.....7ffdcbedc119eced8c84aea73b77f1ea