Faculty Bibliography

OBJECTIVE: Pediatric optic pathway gliomas (OPGs) are often considered benign, but can have detrimental effects on the quality of life, impair vision and are a potentially lethal disease. The aim of this study is to report the characteristics and outcomes of surgically treated OPGs and to identify candidates for different treatment strategies. MATERIAL-METHODS: Retrospective chart review of consecutive pediatric patients with surgically treated OPGs by a single surgeon at our institution from 1985-2015. Three treatment pathways were defined: surgery without planned adjuvant therapy (1), surgery with planned adjuvant therapy (2) and patients with prior treatment (3). RESULTS:88 patients - 49 male and 39 female - were included in analysis. 8 patients had NF1. Pathology revealed pilocytic astrocytoma (85.2%), pilomyxoid astrocytoma (8%), and pilocytic/pilomyxoid astrocytoma (5.7%). Radiologic location of the tumor was: hypothalamic (87%), and involvement of only chiasmand/or tract in (13%).Median age at diagnosiswas 4 years, median age at surgery was 6 years, and median time from diagnosis to surgery was 1 year. At the time of the study: Pathway 1: 37 patients; median PFS 84 +/- 33.4; median OS of 118 months (range: 24 - 337 months); OS rate 68%. Pathway 2: 9 patients; median PFS 45 +/- 10.9 months; median OS of 127 months (range: 23 - 368 months); OS rate 67%. Pathway 3: 42 patients; median PFS 74 +/- 13.3 months; median OS of 69 months (range: 7 - 356) months; OS rate 76%.. CONCLUSIONS: The role of surgery in the treatment of pediatric OPGs depends on patient characteristics and tumor biology. With the adequate therapeutic strategy, long-term PFS and OS can be achieved

OBJECTIVE: Neuroendoscopy is increasingly being used in the management of intraventricular brain tumors. The role of endoscopy in diagnostic biopsy is well established. Expansion of these techniques may allow for definitive resection of intraventricular tumors. We report the feasibility and outcomes of endoscopic resection of select intraventricular tumors in children. METHODS: The clinical characteristics of 11 children with solid intraventricular tumors who underwent tumor resection were reviewed. Twelve procedures were performed. RESULTS: Gross-total resection was achieved in 11 of 12 cases (92%). Maximal tumor diameter ranged from 9 to 26 mm (mean, 16.6 mm). Pathologic results included subependymal giant cell astrocytomas, ependymomas, nongerminomatous germ cell tumors, and pilocytic astrocytomas. Mean follow-up was 35 months (range, 10-109 months). All patients returned to their neurologic baselines after surgery. Local tumor recurrence occurred in 1 patient and distant recurrence in another. In 1 patient, a transitory intraoperative increase of intracranial pressure without clinical implications occurred. There was no permanent morbidity or mortality in this series. Hydrocephalus was present preoperatively in 5 cases and was treated either with tumor removal alone or with an additional endoscopic third ventriculostomy. No patient required a ventriculoperitoneal shunt. CONCLUSIONS: Neuroendoscopic gross-total resection of solid intraventricular tumors is a safe and efficacious procedure in carefully selected pediatric patients.

Objective: We report the feasibility and outcomes of endoscopic resection of select intraventricular tumors in children. Methods: The clinical characteristics of 11 children with solid intraventricular tumors who underwent tumor resection were reviewed. 12 procedures were performed. Results: Gross total resection was achieved in 11 of 12 cases (92%). Maximal diameter ranged from 9-26 mm (mean 16.6 mm). Pathology included subependymal giant cell astrocytomas (SEGA), ependymomas, non-germinomatous germ cell tumor (NGGCT) and pilocytic astrocytoma. Mean follow-up was 35 months (range 10-109 months). All patients returned to their neurological baseline following surgery. Local tumor recurrence occurred in one patient and distant recurrence in another. Complications occurred in one patient, no permanent morbidity or mortality occurred. Hydrocephalus was present preoperatively in 5 cases and was treated with tumor removal alone or with additional endoscopic third ventriculostomy. No patient required a ventriculoperitoneal shunt. Conclusion: Neuroendoscopic gross-total resection of solid intraventricular tumors is a safe procedure in carefully selected pediatric patients

OBJECT: Presently, the best available treatment for intramedullary spinal cord tumors (IMSCTs) in children is microsurgery with the objective of maximal tumor removal and minimal neurological morbidity. The latter has become manageable with the development and standard use of intraoperative neurophysiological monitoring. Traditionally, the perioperative neurological evaluation is based on surgical or spinal cord injury scores focusing on sensorimotor function. Little is known about the quality of life after such operations; therefore, this study was designed to investigate the impact of surgery for IMSCTs on the quality of life in children. METHODS: Twelve consecutive pediatric patients treated for IMSCT were included in this retrospective fixed cohort study. A multidimensional questionnaire-based quality of life instrument, the Pediatric Quality of Life Questionnaire version 4 (PedsQL 4.0), was chosen to analyze follow-up data. This validated instrument particularly allows for a comparison between a patient cohort and a healthy pediatric sample population. RESULTS: Of 11 mailed questionnaires (1 patient had died of progressive disease), 10 were returned, resulting in a response rate of 91%. There were 8 low-grade lesions (5 pilocytic astrocytomas, 1 ganglioglioma, 1 hemangioblastoma, and 1 cavernoma) and 4 high-grade lesions (2 anaplastic gangliogliomas, 1 glioblastoma, and 1 glioneuronal tumor). The mean age at diagnosis was 7.5 years, the mean follow-up was 4.2 years, and 83% of the patients were male. Total resection was achieved in 5 patients and subtotal resection in 7. Four patients had undergone 2 or more resections. The 4 patients with high-grade tumors and 2 with incompletely resected low-grade tumors underwent adjuvant treatment (2 chemotherapy and 4 both radiotherapy and chemotherapy). The mean modified McCormick Scale score at the time of diagnosis was 1.7; at the time of follow-up, 1.5. The mean PedsQL 4.0 total score in the low-grade group was 78.5; in the high-grade group, 82.6. There was no significant difference in PedsQL 4.0 scores between the patient cohort and the normal population. CONCLUSIONS: In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population.

BACKGROUND: We are in an aging population and many elderly people are prone to falling and suffering an acute traumatic subdural hematoma (aSDH). Yet, the operative treatment of patients older than 65 years of age for aSDH remains controversial, and very limited data exists with regard to expected outcome in this elderly patient group. METHODS: We retrospectively analyzed 37 consecutive patients (all >65 years) who underwent craniotomy for aSDH in our department between January 1, 2002 and December 31, 2007. RESULTS: Thirty-seven consecutive patients (54% women, 46% men) were treated for aSDH by means of craniotomy and duraplasty. Median age was 73 years (interquartile range, 10 years). Thirty patients (81%) had significant comorbidities and 43% of patients were treated by anticoagulation or thrombocyte aggregation inhibitors. Median initial Glasgow coma scale score was 8 (interquartile range, 7), and 51% had pupillary abnormalities. Perioperative morbidity occurred in 12 of 37 patients (32%), and 13 patients died in the postoperative period (35%). Overall outcome according to Glasgow outcome scale (GOS) was favorable (GOS, 4 and 5) in 15 of 37 patients (41%); severely disabled (GOS, 3) in 8 of 37 (22%), and unfavorable (GOS, 1 and 2) in 14 of 37 (38%). CONCLUSIONS: Craniotomy for patients older than 65 years of age remains controversial, and our case series seems to support the notion that surgical treatment is associated with significant postoperative morbidity, mortality, and adverse outcome. However, selected patients benefit from an intervention, with a good outcome in 41% of patients.