Faculty Bibliography

OBJECT: Gross-total resection (GTR) of craniopharyngioma is associated with high rates of complications that potentially affect quality of life (QoL). This study investigated the impact of GTR on the long-term QoL and sexual functioning in young adults. METHODS: 81 pediatric patients treated with primary GTR of craniopharyngioma were included in this retrospective cohort study. The Quality of Life Questionnaire SF36v1 and the Medical Outcomes Study family and sexual functioning scale were used to analyze follow-up data. RESULTS: 22 patients consented and completed the questionnaires. The median time of follow-up was 19 years (range 10-26). 55% of the patients reported to have excellent or very good health in general. The mean SF 36v total score was 51.63 for PCS and 49.26 for MCS. There was no significant difference between the patient cohort and the normal population. Twenty-one out of 22 subjects reported about sexual functioning, of whom 25% of women and 54% of men reported at least 'a little of a problem' in one or more areas of sexual functioning. Body mass index (BMI) values were: 14% normal, 41% overweight, 36% obese and 9% morbidly obese. Preoperative hypothalamic involvement and retrochiasmatic location of the tumor was significantly correlated with BMI. CONCLUSIONS: Young adults with childhood-onset craniopharyngioma report QoL and sexual functioning similar to that of the normal population. Overweight and obesity are more prevalent in the study population. Retrochiasmatic location of the tumor and hypothalamic involvement on the preoperative imaging correlate with higher BMI in long-term follow-up

Optic pathway gliomas (OPGs) are relatively common and benign lesions in children; however, in adults these lesions are nearly always malignant and hold a very poor prognosis. In this report the authors present the case of an adult patient with a benign OPG who underwent subtotal resection without adjuvant therapy and has had no tumor progression for more than 20 years. A 50-year-old woman presented with a 2-year history of personality changes, weight gain, and a few months of visual disturbances. Ophthalmological evaluation showed incomplete right homonymous hemianopsia. MRI demonstrated a 2.5 × 2.5 × 2.5-cm enhancing left-sided lesion involving the hypothalamus with extension into the suprasellar cistern, extending along the left optic tract and anterior to the level of the optic chiasm. A biopsy procedure revealed a juvenile pilocytic astrocytoma. A subtotal resection of approximately 80% of the tumor was performed. Postoperatively, the patient experienced complete resolution of her personality changes, and her weight decreased back to baseline. Ophthalmological examination showed increased right homonymous hemianopsia. In the years following her surgery, there was a spontaneous decrease in tumor size without adjuvant therapy. The patient continues to have an excellent quality of life despite a visual field defect, and no further tumor growth has been observed.

BACKGROUND: Hydrocephalus in patients with neurofibromatosis type I (NF1) is usually obstructive, and may arise secondary to tumoral or non-tumoral causes. The treatment of hydrocephalus in these patients is often challenging due to combined pathologies and unique anatomical changes. The use of endoscopic third ventriculostomy (ETV) as treatment has rarely been described in this group. In this study we aimed to characterize the indications, considerations and outcome of ETV in patients with NF1 gathered in a multicenter international cohort. METHODS: Five centers participated in this retrospective study. Following an IRB approval, data and images were collected. Inclusion criteria were patients of all ages with NF1 who underwent an ETV for the treatment of obstructive hydrocephalus. Exclusion criteria were patients who had no post-operative radiological or clinical follow up. ETV failure was defined as recurrent clinical or radiological signs of hydrocephalus. RESULTS: Forty-two patients were included in this study. Common etiologies for hydrocephalus were aqueductal/tectal tumor (31%), aqueductal web (26%), and aqueductal stenosis due to neurofibromatosis related changes (14%). Ten patients had a preoperative diagnosis of optic pathway glioma. Six patients were defined as ETV failure within 1 month, 3 more within 9 months and 1 more within 4 years. ETV was defined as successful in 32 patients (76%) with a mean follow up of 59.4 +/- 50.9 months (4 months - 15 years). CONCLUSION: ETV is a safe treatment for selected patients with NF1 and obstructive hydrocephalus. Individual anatomical and pathological aspects should be taken into consideration.

New developments in diagnostic methods, technical improvements in the surgical field, and a better understanding of the effects of epilepsy on the developing brain are contributing to the general observation that more children with epilepsy are being treated surgically. Malformations of cortical development are the most common cause of seizures in pediatric surgical candidates, and the best predictor of seizure freedom after surgery appears to be the complete removal of the epileptogenic lesion. To achieve this goal in challenging cases, such as magnetic resonance imaging-negative or multifocal lesions, a staged approach with pre- and/or post-resective invasive electroencephalography monitoring has increasingly been used at a number of centers. As the experience with this approach has grown, and the risks and benefits are better understood, a larger number of patients with epilepsy have been identified as potential surgical candidates. In this review, a number of the recent developments in pediatric epilepsy surgery are discussed.

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: 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: 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: 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