Faculty Bibliography

Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjogren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjogren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients. One patient experienced skin irritation 15 months after surgery, necessitating pump repositioning. No infection occurred. Our report shows that intrathecal baclofen therapy can have a positive therapeutic effect on spasticity in patients with Sjogren-Larsson syndrome, and therefore may be a promising addition to current treatments.

OBJECTIVE: The need of duraplasty for adequate decompression of Chiari 1 malformation remains highly controversial. Although proponents of dural opening contend that duraplasty increases the likelihood of symptom and syrinx improvement while concurrently decreasing reoperation rates, opponents reference the risks associated with dural opening: CSF leakage, bacterial meningitis, aseptic meningitis, increased bleeding, pseudomeningocele, and hydrocephalus. In this study we retrospectively investigated the outcomes for pediatric patients who underwent surgery for treatment of symptomatic Chiari Malformation. We limited our investigation to patients who were treated with bony decompression and duraplasty in an effort to describe the clinical outcomes and post-operative course associated with this posterior fossa decompression variant. MATERIAL-METHODS: This is a retrospective chart review of all patients who underwent surgical decompression of symptomatic Chiari Malformation with dural opening by a single surgeon at New York University Medical Center between the years of 1985 and 2015. RESULTS:107 patients, median age at surgery 10 years (range: 1-20). Presentation was typical tussive headaches in15.9%, mixed headache in 11.2%, atypical headache in 18.7%, serendipitous finding in 22.4%, scoliosis workup in 22.4%, neurologic deficit in 3.7%, ataxia in 2.8%, other in 3.7%. Focal neurologic deficits at presentation was sensory in19.6%, motor in 6.5%, mixed in 10.3%, CN involvement in 2.8%, none in 57.9% Headache Resolved in 46/48 patients(95.8%). Syrinx resolved in 63.8%, decreased in 28.8%, stable in 5%, progressive in 1.3%. Complications were aseptic meningitis in 15%, Infection in0.9%,dural leaks in0.9% No resurgery in 30 days, total resurgeries: 11 (10.3%). No mortality. CONCLUSIONS: Suboccipital decompression with duraplasty for Chiari 1 malformation can be achieved with a high success rate and a low complication rate in selected patients. Headache resolved in 96% and Syrinx resolved or improved in 93% of the patients. The most common complication was aseptic meningitis

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: Gross-total resection of craniopharyngioma is associated with complications that potentially affect quality of life. This study was designed to investigate the impact of gross-total resection on the long-termquality of life and sexual functioning in adulthood. MATERIAL-METHODS:55 consecutive pediatric patients treated with primary gross-total resection for craniopharyngioma were included in this retrospective fixed cohort study. Amultidimensional questionnaire-based quality of life instrument, the SF36v1 and the Medical Outcomes Study family and sexual functioning scale, was chosen to analyze follow-up data. Additionally, patients were asked to fill out a Questionnaire about medication, visual impairment, education and family life. RESULTS: Of 43 mailed questionnaires 23 were returned (response rate 55%); 17 patients were lost to follow up, 3 individuals were reluctant to participate, 7 patients died and 5 patients were minors at the time of study. The median length of follow up was 19 years (range 10-31). BMI was underweight in 1 patient (4%), normal in 3 patients (13%), overweight in 8 patients (35%), obesity in 4 patients (17%) and severe obesity in 7 patients (31%). 18 out of 19 patients reported about sexual functioning, of whom 53% reported at least 'a little of a problem' in one or more areas of sexual functioning. The mean SF 36v total score was 51.9 for PCS (physical health) and 48.5 for MCS (mental health), no significant difference between the patient cohort and the normal population. No significant correlation between BMI and PCS, but significant correlation between BMI and MCS. 44% Patients reported to have excellent or very good health in general. CONCLUSIONS: In a cohort of adults who underwent gross-total resection for craniopharyngioma in childhood, quality of life scores according to the SF 36 instrument were not different from those in a normal sample population. Sexual dysfunction is slightly more prevalent in this population

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.