{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T11:03:01Z","timestamp":1789815781552,"version":"4.0.1"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T00:00:00Z","timestamp":1789689600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T00:00:00Z","timestamp":1789689600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Childs Nerv Syst"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Cerliponase alfa enzyme replacement therapy has transformed the management of neuronal ceroid lipofuscinosis type 2 (CLN2) disease but requires lifelong cerebrospinal fluid (CSF) access for repeated intraventricular infusions. Conventional scalp-based ventricular reservoirs are associated with complications including infection, mechanical deterioration, and device revision related to repeated puncture. Chest port\u2013mediated ventricular access has emerged as a potential alternative; however, detailed operative descriptions remain limited. We describe operative considerations and stepwise technique for establishing ventricular access using a Rickham reservoir connected via shunt tubing to a subcutaneous chest port system for repeated intraventricular cerliponase alfa infusion. Illustrative clinical experience demonstrates durable long-term use of this configuration. Key technical elements include neuronavigation-guided ventricular catheter placement, creation of a subcutaneous or subfascial chest pocket depending on patient body habitus, incorporation of strain-relief loops to reduce catheter tension, and systematic testing of the completed construct. The resulting system allows reliable ventricular access via a chest port while preserving ventricular catheter integrity. Chest port\u2013mediated ventricular access represents a feasible and durable strategy for long-term intraventricular therapy in CLN2 disease. Dissemination of operative technique may facilitate broader adoption and support reliable delivery of enzyme replacement therapy for patients requiring lifelong treatment.<\/jats:p>","DOI":"10.1007\/s00381-026-07463-x","type":"journal-article","created":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T21:59:25Z","timestamp":1789768765000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Conversion of a ventricular rickham reservoir to a subcutaneous chest port for repeated intraventricular enzyme replacement therapy: technical note and surgical considerations"],"prefix":"10.1007","volume":"42","author":[{"given":"Mahie","family":"Gopalka","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samiya","family":"Manocha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Romanski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin","family":"Hoeman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Boop","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandi K.","family":"Lam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,18]]},"reference":[{"key":"7463_CR1","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1080\/21678707.2020.1856654","volume":"8","author":"SC Aylward","year":"2020","unstructured":"Aylward SC, Pindrik J, Abreu NJ, Cherny WB, O\u2019Neal M, de Los Reyes E (2020) Cerliponase alfa for CLN2 disease, a promising therapy. Expert Opin Orphan Drugs 8:445\u2013454","journal-title":"Expert Opin Orphan Drugs"},{"key":"7463_CR2","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1023\/A:1005564509027","volume":"22","author":"MJ Bennett","year":"1999","unstructured":"Bennett MJ, Hofmann SL (1999) The neuronal ceroid-lipofuscinoses (Batten disease): a new class of lysosomal storage diseases. J Inherit Metab Dis 22:535\u2013544","journal-title":"J Inherit Metab Dis"},{"key":"7463_CR3","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1007\/s00381-025-06822-4","volume":"41","author":"S Boop","year":"2025","unstructured":"Boop S, Nistal D, Barrios-Anderson A, Cherny WB, Chang IJ, Shelkowitz E, Kho T, Goldstein HE, Hauptman J (2025) Novel surgical approach for intraventricular cerliponase alfa enzyme replacement therapy via central venous access device (CVAD) port in neuronal ceroid lipofuscinosis type 2 (CLN2) disease. 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Pediatr Neurol 110:64\u201370","journal-title":"Pediatr Neurol"},{"key":"7463_CR6","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.ejpn.2020.12.002","volume":"30","author":"B Estublier","year":"2021","unstructured":"Estublier B, Cano A, Hoebeke C, Pichard S, Scavarda D, Desguerre I, Auvin S, Chabrol B (2021) Cerliponase alfa changes the natural history of children with neuronal ceroid lipofuscinosis type 2: the first French cohort. Eur J Paediatr Neurol 30:17\u201321","journal-title":"Eur J Paediatr Neurol"},{"key":"7463_CR7","doi-asserted-by":"publisher","DOI":"10.3390\/children13030365","author":"M Gopalka","year":"2026","unstructured":"Gopalka M, Patel J, Votoupal M, Lam S (2026) Patient and family perspective on transition from ventricular access device to chest-sited port for intracerebroventricular infusion in CLN2 disease. 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