Primary neurosurgery for pediatric low-grade gliomas: a prospective multi-institutional study from the Children's Oncology Group
BACKGROUND: Central nervous system neoplasms are the most common solid tumors in children, and more than 40% are low-grade gliomas. Variable locations, extent of resection, postoperative neurodiagnostic evaluation, and histology have confounded therapy and outcome. OBJECTIVES: To investigate disease control and survival after surgery. METHODS: A prospective natural history trial from 1991 to 1996 produced a subset of patients with low-grade gliomas managed by primary surgery and subsequent observation. Patients were evaluable if eligibility, tumor location, and extent of resection were confirmed by pathological diagnosis, preoperative and postoperative imaging, and the surgeon's report. Primary end points were overall survival (OS), progression-free survival (PFS), and postprogression survival. RESULTS: Of 726 patients enrolled, 518 were fully evaluable for analysis. The 5- and 8-year OS rates were 97% +/- 0.8% and 96% +/- 0.9%, respectively, and PFS rates were 80% +/- 1.8% and 78% +/- 2.0%. In univariate analyses, histological type, extent of residual tumor, and disease site were significantly associated with PFS and OS. In multivariate analysis, gross total resection (GTR) without residual disease was the predominant predictor of PFS. In patients with limited residual disease, 56% were free of progression at 5 years. CONCLUSION: GTR should be the goal when it can be achieved with an acceptable functional outcome. The variable rate of progression after incomplete resection highlights the need for new predictors of tumor behavior
Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children
OBJECT: Optimal treatment of primary and recurrent craniopharyngiomas remains controversial. Radical resection and limited resection plus radiation therapy yield similar rates of disease control and overall survival. The data are much less clear for recurrent tumors. The authors report their experience with radical resection of both primary and recurrent craniopharyngiomas in children and compare the outcomes between the 2 groups. METHODS: A retrospective analysis was performed in 86 children younger than 21 years of age who underwent a total of 103 operations for craniopharyngioma between 1986 and 2008; these were performed by the senior author. The goal was resection with curative intent in all patients. Two patients were lost to follow-up and were excluded from analysis. The mean age at the time of surgery was 9.6 years, and the mean follow-up was 9.0 years. RESULTS: All 57 children with primary tumors underwent gross-total resection (GTR). A GTR was achieved in significantly fewer children with recurrent tumors (18 [62%] of 29). There were 3 perioperative deaths (3%). Tumor recurred after GTR in 14 (20%) of 71 patients. Overall survival and progression-free survival were significantly better in patients with primary tumors at time of presentation to the authors' institution. There were no significant differences in the neurological, endocrinological, visual, or functional outcomes between patients with primary and those with recurrent tumors. Factors negatively affecting overall survival and progression-free survival include subtotal resection (recurrent tumors only), tumor size >or= 5 cm, or presence of hydrocephalus or a ventriculoperitoneal shunt. Prior radiation therapy and increasing tumor size were both risk factors for incomplete resection at reoperation. CONCLUSIONS: In the hands of surgeons with experience with craniopharyngiomas, the authors believe that radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity. While GTR does not preclude recurrence and is more difficult to achieve in recurrent tumors, especially large and previously irradiated tumors, radical resection is still possible in patients with recurrent craniopharyngiomas with morbidity similar to that of primary tumors
Role of diffusion tensor imaging in resection of thalamic juvenile pilocytic astrocytoma [Case Report]
OBJECT: The choice of surgical approach during resection of a thalamic juvenile pilocytic astrocytoma (JPA) is dictated by the location of the displaced normal thalamus and posterior limb of the internal capsule (PLIC). Diffusion tensor (DT) imaging and white matter tractography can identify the location of the PLIC in relation to the tumor and may be useful in planning the operative trajectory. METHODS: Diffusion tensor imaging was used to localize the PLIC on preoperative MR imaging in 6 children undergoing resection of thalamic JPAs. After review of the standard T2-weighted MR imaging sequences, the anticipated position of the PLIC was determined. This result was compared with the location of the PLIC determined by a blinded radiologist with the use of DT imaging. The utility of DT imaging in determining the surgical approach to a thalamic JPA, degree of resection, and neurological outcomes were all evaluated. RESULTS: Diffusion tensor imaging confirmed the expected location of the PLIC as approximated on conventional T2-weighted images in all 6 cases. In 1 patient in particular, unexpected medial deviation of the PLIC was identified, and this proved useful in tailoring the approach to a more lateral trajectory. Gross-total resection of all cystic and solid tumor components was confirmed on postoperative imaging in all cases. All patients experienced mild to moderate worsening of neurological status immediately following resection, but 4 of 6 patients were back to their preoperative baseline at 6-month follow-up. CONCLUSIONS: Diffusion tensor imaging and white matter tractography successfully identified the white matter fibers emanating from the precentral gyrus within the PLIC in children with thalamic JPAs prior to surgery. Diffusion tensor imaging served as a valuable tool for stereotactic planning of operative approaches to thalamic JPAs. Localizing the position of the PLIC helped minimize potential neurological morbidity and facilitated gross-total resection
Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study
OBJECTIVE:Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3Â months. METHODS:To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3Â months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications. RESULTS:Sixty-four patients underwent 69Â surgeries before the age of 3Â months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41Â months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, pÂ <Â .0001). Outcome was not significantly associated with the type of surgery (hemispheric or more limited resections). SIGNIFICANCE/CONCLUSIONS:Epilepsy surgery during the first few months of life is associated with excellent seizure control, and when performed by highly experienced teams, is not associated with more permanent morbidity than surgery in older infants. Thus surgical treatment should not be postponed to treat DRE in very young infants based on their age.
Radiologic response to MEK inhibition in a patient with a WNT-activated craniopharyngioma [Letter]
Extended lumbar drainage in idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis of diagnostic test accuracy
BACKGROUND:When appropriately selected, a high proportion of patients with suspected idiopathic normal pressure hydrocephalus (iNPH) will respond to cerebrospinal fluid diversion with a shunt. Extended lumbar drainage (ELD) is regarded as the most accurate test for this condition, however, varying estimates of its accuracy are found in the current literature. Here, we review the literature in order to provide summary estimates of sensitivity, specificity, positive- and negative predictive value for this test through meta-analysis of suitably rigorous studies. METHODS:Studies involving a population of NPH patients with predominantly idiopathic aetiology (>80%) in which the intention of the study was to shunt patients regardless of the outcome of ELD were included in the review. Various literature databases were searched to identify diagnostic test accuracy studies addressing ELD in the diagnosis of iNPH. Those studies passing screening and eligibility were assessed using the QUADAS-2 tool and data extracted for bivariate random effects meta-analysis. RESULTS:Four small studies were identified. They showed disparate results concerning diagnostic test accuracy. The summary estimates for sensitivity and specificity were 94% (CI 41-100%) and 85% (CI 33-100%), respectively. The summary estimates of positive and negative predictive value were both 90% (CIs 65-100% and 48-100%, respectively). CONCLUSION/CONCLUSIONS:Large, rigorous studies addressing the diagnostic accuracy of ELD are lacking, and little robust evidence exists to support the use of ELD in diagnostic algorithms for iNPH. Therefore, a large cohort study, or ideally an RCT, is needed to determine best practice in selecting patients for shunt surgery.
Posterior Fossa Society Consensus Meeting 2018: a synopsis
PURPOSE/OBJECTIVE:The Posterior Fossa Society (PFS) was founded 4Â years ago to systematically gather and exchange information on the post-operative (CMS) and cerebellar cognitive affective syndrome (CCAS). With its consensus meetings, the PFS orchestrates research studies in the field of cerebellar injury and progresses the knowledge of post-operative pediatric cerebellar mutism syndrome (CMS). In this article, we captured the 3-day program of presentations, group discussions, interactive workshops, and dialogue, highlighting the key topic areas of CMS and its research advances. METHODS:This synopsis is based on the third consensus meeting which was held in Reykjavik, Iceland, in August 2018. RESULTS:Three working groups have been defined to drive the future research priorities on post-surgical CMS: (i) refining definition and symptoms scoring of CMS; (ii) understanding the pathogenesis and enhancing risk-stratification strategies; and (iii) developing rehabilitation approaches and protocols. CONCLUSIONS:The third consensus meeting highlighted a unanimous desire for data-driven information to advance the knowledge and guide future research efforts. The PFS constitutes an established and expanding network of multi-disciplinary expertise that can facilitate the development of collaborative studies and produce official guidelines on the topic.
Introduction to the cerebellar mutism syndrome
Treatment Options for Hydrocephalus Following Foramen Magnum Decompression for Chiari I Malformation: A Multicenter Study
BACKGROUND:New-onset hydrocephalus following foramen magnum decompression (FMD) for Chiari I malformation (CM-I) is rare; its natural history and pathophysiology are poorly understood. OBJECTIVE:To describe a series of patients who developed hydrocephalus following FMD for CM-I, provide possible explanations of this phenomenon, and outline treatment options. METHODS:Out of patients undergoing FMD for CM-I from 6 different tertiary centers, we evaluated patients presenting with new-onset hydrocephalus following FMD. The retrospectively collected data included demographics, clinical, and radiological findings of the CM-I and hydrocephalus patients. Time from FMD and hydrocephalus onset, treatment, and surgical techniques were assessed. RESULTS:Of 549 patients who underwent FMD for CM-I, 28 (5.1%) subsequently developed hydrocephalus (18 females, 10 males), with a mean age of 11.7Â Â±Â 11.9 yr (range 6 mo to 52 yr). Hydrocephalus occurred on average 2.2Â Â±Â 2.6 mo after FMD (range 1 wk to 8 mo). Four patients did not have a violation of the arachnoid during the FMD surgery. Main presenting symptoms of hydrocephalus were headaches (17, 41%), vomiting (10, 24.4%), and cerebrospinal fluid (CSF) leak or pseudomeningocele (7, 17%).Overall, 23 patients (82.1%) underwent CSF shunting, 1 patient (3.5%) had an endoscopic third ventriculostomy, 3 patients (10.7%) temporary CSF diversion only, and 1 patient (3.5%) was treated with acetazolamide. CONCLUSION/CONCLUSIONS:Hydrocephalus following FMD for CM-I is uncommon, but important. Based on our series and literature review, its incidence is about 5% to 7% and most likely will require further surgery. Shunting appears to be the favored treatment option.
Quality of life, hypothalamic obesity, and sexual function in adulthood two decades after primary gross-total resection for childhood craniopharyngioma
PURPOSE/OBJECTIVE:All treatments for childhood craniopharyngioma are associated with complications that potentially affect quality of life. This study was designed to investigate the impact of gross total resection on long-term quality of life and sexual functioning in adulthood. METHODS:Adults treated with primary gross total resection for childhood craniopharyngioma and â‰¥â€‰10Â years of follow-up were included in this retrospective cohort study. The Short Form 36 Health Survey Questionnaire Version 2 (SF-36v2), Medical Outcomes Study (MOS) sexual functioning survey, and a sociodemographic/health questionnaire were administered. RESULTS:). Preoperative hypothalamic involvement correlated with a significantly higher BMI, although the proportion of participants with class 3 obesity (BMIâ€‰â‰¥â€‰40) did not differ significantly from that of the general population (9% and 7%, respectively). CONCLUSIONS:Young adults with gross total resection of childhood craniopharyngioma report similar quality of life and sexual functioning compared to the general population, but appear to be less sexually active. Hypothalamic involvement on preoperative imaging was associated with a higher BMI in long-term follow-up.