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Claudia L. Craven, Paul Gissen, Rebecca Bower, Laura Lee, Kristian Aquilina, and Dominic N. P. Thompson


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.


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.


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 59.5 (7.5–82.0) for unrevised VADs (n = 17) versus 2 (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).


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.

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The National Traumatic Coma Data Bank

Part 1: Design, purpose, goals, and results

Lawrence F. Marshall, Donald P. Becker, Sharon A. Bowers, Carol Cayard, Howard Eisenberg, Cynthia R. Gross, Robert G. Grossman, John A. Jane, Selma C. Kunitz, Rebecca Rimel, Kamran Tabaddor, and Joseph Warren

✓ This paper describes the pilot phase of the National Traumatic Coma Data Bank, a cooperative effort of six clinical head-injury centers in the United States. Data were collected on 581 hospitalized patients with severe non-penetrating traumatic head injury. Severe head injury was defined on the basis of a Glasgow Coma Scale (GCS) score of 8 or less following nonsurgical resuscitation or deterioration to a GCS score of 8 or less within 48 hours after head injury.

A common data collection protocol, definitions, and data collection instruments were developed and put into use by all centers commencing in June, 1979. Extensive information was collected on pre-hospital, emergency room, intensive care, and recovery phases of patient care. Data were obtained on all patients from the time of injury until the end of the pilot study.

The pilot phase of the Data Bank provides data germane to questions of interest to neurosurgeons and to the lay public. Questions are as diverse as: what is the prognosis of severe brain injury; what is the impact of emergency care; and what is the role of rehabilitation in the recovery of the severely head-injured patient?