Object: Endoscopic removal of intraventricular brain tumors is well established for cystic tumors such as colloid cysts. Aspiration followed by removal or ablation of the membranous wall is possible given the constituent features of these tumors. It is generally expected that endoscopic removal of solid brain tumors from the intraventricular compartment would impose additional technical demands. In this paper, the feasibility and safety of endoscopic removal of solid intraventricular brain tumors is evaluated. Methods: Eighty-one patients who underwent endoscopic management of an intraventricular brain tumor were identified from a prospective database. Of these patients, seven underwent attempted endoscopic surgical removal of a solid primary brain tumor. Patient selection, surgical technique, procedure-related morbidity, and extent of removal were reviewed. Five patients underwent complete resection of a solid intraventricular brain tumor, a treatment option that was based on intraoperative assessment and confirmed by postoperative imaging. No patient experienced any procedure-related morbidity. Of the individuals in whom a total endoscopic resection was successful, there has been no symptomatic or radiological evidence of recurrence (mean follow up 20 months). Maximum tumor diameter ranged from 0.5 to 1.8 cm for patients who underwent complete resection, whereas maximum tumor diameter measured 2.4 and 2.5 cm in the two patients in whom a subtotal excision was performed. Conclusions: In select patients, complete endoscopic removal of solid intraventricular brain tumors is possible and safe. Factors that influence the ability of a surgeon to perform a complete endoscopic resection include tumor size, composition, and vascularity. The procedure requires careful patient selection, the use of refined endoscopic instrumentation, and a disciplined surgical technique.

Object: Endoscopic fenestration has been recognized as an accepted treatment choice for patients with symptomatic arachnoid cysts. The success of this procedure, however, is greatly influenced by individual cyst anatomy and location as well as the endoscopic technique used. This review was conducted to assess what variables influence the treatment success for different categories of arachnoid cysts. Methods: Thirty-three consecutive patients who underwent endoscopic fenestration for treatment of an intracranial arachnoid cyst were identified from a prospective database. The surgical indications and techniques were reviewed, and surgical success rates and patient outcomes were assessed. Specific examples of each cyst category are included to illustrate the technical aspects of endoscopic cyst fenestration. Endoscopic fenestration of arachnoid cysts was successful when judged by cyst decompression, and symptom resolution was noted in 32 (97%) of 33 cases. The one patient with short-term treatment failure underwent a successful repetition of the operation. There were no surgery-related morbidities or deaths. Conclusions: Arachnoid cysts are a relatively benign pathological entity that can be managed by performing endoscopically guided cyst wall fenestrations into the ventricular system or cerebrospinal fluid–containing cisterns. Proper patient selection, preoperative planning of endoscope trajectory, use of frameless navigation, and advances in endoscope lens technology and light intensity combine to make this a safe procedure with excellent outcomes.

Administration of an adeno-associated virus coding for the CLN2 gene into the brain parenchyma slows the progression of CLN2 disease in children.

The authors conducted a phase I study of late infantile neuronal ceroid lipofuscinosis using an adenoassociated virus serotype 2 (AAV2) vector containing the deficient CLN2 gene (AAV2_CUhCLN2). The operative technique, radiographic changes, and surgical complications are presented. Ten patients with late infantile neuronal ceroid lipofuscinosis disease each underwent infusion of AAV2_CUhCLN2 (3 × 10¹² particle units) into 12 distinct cerebral locations (2 depths/bur hole, 75 minutes/infusion, and 2 μl/minute). Innovative surgical techniques were developed to overcome several obstacles for which little or no established techniques were available. Successful infusion relied on preoperative stereotactic planning to optimize a parenchymal target and diffuse administration. Six entry sites, each having 2 depths of injections, were used to reduce operative time and enhance distribution. A low-profile rigid fixation system with 6 integrated holding arms was utilized to perform simultaneous infusions within a practical time frame. Dural sealant with generous irrigation was used to avoid CSF egress with possible subdural hemorrhage or altered stereotactic registration. Radiographically demonstrated changes were seen in 39 (65%) of 60 injection sites, confirming localization and infusion. There were no radiographically or clinically defined complications. The neurosurgical considerations and results of this study are presented to offer guidance and a basis for the design of future gene therapy or other clinical trials in children that utilize direct therapeutic delivery.

The successful treatment of children with a primary CNS germ cell tumor can be greatly influenced by the neurosurgeon involved in the diagnostic and therapeutic care of these children. Variability in surgical philosophies no doubt exists due to the relatively infrequent incidence of these tumors, a lack of consensus regarding diagnostic and therapeutic approaches, and the advent of recent surgical innovations. Many of these issues were discussed at the Second International Symposium on Central Nervous System Germ Cell Tumors through presented abstracts and invited presentations. The neurosurgical aspects of these proceedings are summarized here in an effort to present the agreed-upon and debated issues that may confront the pediatric neurosurgeon.