{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T02:25:48Z","timestamp":1794018348884,"version":"4.8.0"},"reference-count":23,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,1,1]]},"abstract":"<jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a rare autosomal recessive disease caused by tripeptidyl peptidase 1 enzyme deficiency. At the authors\u2019 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\u2019s lifetime. In this study, the authors audited the longevity and survival of these VADs and examined the causes of device failure.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      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\n                      <jats:italic>Propionibacterium<\/jats:italic>\n                      and\n                      <jats:italic>Staphylococcus<\/jats:italic>\n                      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\u201382.0) for unrevised VADs (n = 17) versus 29.0 (6\u201387.5) 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).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/2021.7.peds21129","type":"journal-article","created":{"date-parts":[[2021,10,8]],"date-time":"2021-10-08T08:42:15Z","timestamp":1633682535000},"page":"115-121","source":"Crossref","is-referenced-by-count":6,"title":["A survival analysis of ventricular access devices for delivery of cerliponase alfa"],"prefix":"10.3171","volume":"29","author":[{"given":"Claudia L.","family":"Craven","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Gissen","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom;"},{"name":"Department of Paediatric Metabolic Diseases, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; and"},{"name":"UCL Institute of Child Health, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Bower","sequence":"additional","affiliation":[{"name":"Department of Paediatric Metabolic Diseases, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Paediatric Metabolic Diseases, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristian","family":"Aquilina","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom;"},{"name":"UCL Institute of Child Health, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic N. P.","family":"Thompson","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom;"},{"name":"UCL Institute of Child Health, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"issue":"8","key":"b1","series-title":"Lancet Child Adolesc Health","first-page":"582","article-title":"Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease: an observational cohort study","volume":"2","author":"Nickel M","year":"2018","unstructured":"Nickel M, Simonati A, Jacoby D, Lezius S, Kilian D, Van de Graaf B,  Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease: an observational cohort study. Lancet Child Adolesc Health. 2018;2(8):582\u2013590."},{"issue":"5","key":"b2","series-title":"Arch Dis Child","first-page":"361","article-title":"The epidemiology of progressive intellectual and neurological deterioration in childhood","volume":"95","author":"Verity C","year":"2010","unstructured":"Verity C, Winstone AM, Stellitano L, Will R, Nicoll A. The epidemiology of progressive intellectual and neurological deterioration in childhood. Arch Dis Child. 2010;95(5):361\u2013364."},{"issue":"20","key":"b3","series-title":"N Engl J Med","first-page":"1898","article-title":"Study of intraventricular cerliponase alfa for CLN2 disease","volume":"378","author":"Schulz A","year":"2018","unstructured":"Schulz A, Ajayi T, Specchio N, de Los Reyes E, Gissen P, Ballon D,  Study of intraventricular cerliponase alfa for CLN2 disease. N Engl J Med. 2018;378(20):1898\u20131907."},{"issue":"11","key":"b4","series-title":"Drugs","first-page":"1247","article-title":"Cerliponase alfa: first global approval","volume":"77","author":"Markham A","year":"2017","unstructured":"Markham A. Cerliponase alfa: first global approval. Drugs. 2017;77(11):1247\u20131249."},{"issue":"5","key":"b5","series-title":"J Child Neurol","first-page":"348","article-title":"Review of cerliponase alfa: recombinant human enzyme replacement therapy for late-infantile neuronal ceroid lipofuscinosis type 2","volume":"35","author":"Lewis G","year":"2020","unstructured":"Lewis G, Morrill AM, Conway-Allen SL, Kim B. Review of cerliponase alfa: recombinant human enzyme replacement therapy for late-infantile neuronal ceroid lipofuscinosis type 2. 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Brain Sci. 2020;10(2):119."}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/29\/1\/article-p115.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/29\/1\/article-p115.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/29\/1\/article-p115.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T01:58:37Z","timestamp":1794016717000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/29\/1\/article-p115.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,1]]},"references-count":23,"journal-issue":{"issue":"1"},"URL":"https:\/\/doi.org\/10.3171\/2021.7.peds21129","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,1]]}}}