Abstract
Abstract
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–mediated 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–mediated 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.