Faculty Bibliography

OBJECTIVE:The majority of the 65 million people worldwide with epilepsy live in low- and middle-income countries. Many of these countries have inadequate resources to serve the large patient population affected by epilepsy. Panama is a middle-income country that currently has only 2 facilities that can provide basic epilepsy services and no epilepsy surgery services. To address this need, a group of Panamanian physicians partnered with U.S. epilepsy health care providers to test a hybrid epilepsy surgery program, combining resources and expertise. METHODS:From 2011 to 2017, a multidisciplinary team of neurologists, neurosurgeons, and an electroencephalography (EEG) technician from the United States traveled to Panama 6 times and, in collaboration with the local team, performed surgical procedures for intractable epilepsy at the national children's hospital. Resective surgeries were performed with intraoperative electrocorticography and/or implantation of subdural and depth electrodes and extra-operative monitoring. Cost was calculated using Panama government data. RESULTS:Twenty-seven children with intractable epilepsy were surgically treated. Fifteen children are seizure-free (Engle class I), 11 children are Engel II, and one child is Engel III. No major morbidity or mortality occurred, with only one postoperative infection. The average cost of treatment was calculated at $9850 per patient. SIGNIFICANCE/CONCLUSIONS:This program is a model for creating a multinational and multi-institutional collaboration to provide surgical epilepsy treatment in a middle-income country without an adequate infrastructure. To be successful, this collaboration needed to address medical, technical, and cultural challenges. This partnership helps to alleviate some of the present need for surgical epilepsy services while laying the groundwork for the development of a future local independent epilepsy surgery program.

OBJECTIVE: New onset hydrocephalus following foramen magnum decompression (FMD) for Chiari I malformation (CMI) is rare, and its natural history and pathophysiology is poorly understood. We describe a series of patients who presented with hydrocephalus following FMD for CMI, provide possible explanations of this condition, and outline treatment options. MATERIAL-METHODS: Of patients undergoing FMD for CMI from 6 different tertiary centres in North America, Europe and Middle East, we evaluated patients presenting with new onset hydrocephalus following the FMD. Retrospectively collected data included demographics, clinical and radiological findings of both the CMI and hydrocephalus, time from FMD and hydrocephalus onset, and treatment as well as surgical techniques. RESULTS: None of the patients had obvious symptoms related to idiopathic intracranial hypertension nor hydrocephalus prior to FMD. Of 367 patients who underwent FMD for CMI, 28 (7.6%) subsequently developed symptoms related to hydrocephalus (18 females-64.2%, and 10 males - 35.8%), (7 Le Bonheur Children's Hospital, 6 Tel Aviv Medical Center and Dana Children's Hospital, 6 Cincinnati Children's Hospital Medical Center, 5 Children's National Medical Center, 3 NYU Langone Medical Center, 1 Charite Universitatsmedizin) with a mean age of 11.7 +/- 11.9 years old (range 6 months to 52 years old). Hydrocephalus occurred on average 2.2 +/- 2.6 months after FMD (ranging from 1 week to 8 months). Presenting symptoms of hydrocephalus were headaches (41%), vomiting (24.4%), CSF leak/pseudomeningocele (17%), decreased level of consciousness (7.3%), cranial nerves signs (7.3%) and papilledema (2.4%). 23 patients (82.1%) underwent CSF shunting, 1 patient had an endoscopic third ventriculostomy (3.5%), 3 patients (10.7%) temporary CSF diversion only, and 1 patient (3.5%) acetazolamide. CONCLUSION: Hydrocephalus following FMD for CMI is uncommon. Based on our series and literature review, its incidence is ~7% and most likely will require further surgery. Considering different treatment options, shunting appears to be the favoured option

OBJECTIVE:Duraplasty is one technique successfully used to treat Chiari malformation type I (CM-I). This study describes the timely manner of clinical outcomes and the postoperative course after craniectomy and duraplasty for the treatment of symptomatic CM-I in pediatric children. METHODS:A retrospective chart review was done on 105 consecutive children who underwent surgical decompression of symptomatic CM-I with dural opening by a single surgeon between 1999 and 2015. RESULTS:In 16 of 28 children (57%) with typical Valsalva-related/tussive and mixed headaches, the symptoms resolved before discharge; by 6 months all children were headache free. Two of 28 children (7%) had recurrent headaches 9 months after surgery. In 78 children with syrinx, syrinx resolved or decreased in 68 children (87%), recurred in 8 children (10%), and was stable in 2 children (3%). In 51 children (65%), syrinx resolved or decreased by 3 months and in 62 children (79%) by 6 months. Complications included aseptic meningitis requiring reoperation in 3% and infection in one child (1%). Twelve reoperations occurred, none within the first 30 days. No child had a major morbidity or mortality. CONCLUSIONS:In carefully selected children with CM-I, a high success rate can be achieved with suboccipital decompression and duraplasty. Valsalva-related/tussive headaches resolved in the majority of children at discharge from the hospital; syrinx resolved or decreased in two thirds of the children by 3 months. By 6 months, headaches were resolved in all cases, and syrinx was resolved or decreased in 79% of cases.

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.