Surgical treatment of pediatric low-grade glioma in developing countries.

Pediatric low-grade gliomas constitute the most common brain tumors worldwide, though with some peculiarities in the presentation and surgical care in different parts of the world. The symptomatology in developing countries is likely to be over longer periods with a tendency to delayed diagnosis due to cultural, religious beliefs, manpower, and infrastructural deficits. Thus, the children present with large tumors with attendant morbidities and an increased risk of mortalities from surgery. Surgery is mainly by "general" neurosurgeons due to the paucity of trained pediatric neurosurgeons. The pre-operative imaging may be limited to anatomic MR imaging, and in some cases, CT scans, without expansive neuropsychological evaluation. The armamentarium available to the neurosurgeon may warrant large openings to access the tumor, and there may be limited possibility for intra-operative mapping of "eloquent" brain functions when this is deemed necessary. Complicating pre-operative acute hydrocephalus can result in two operations that further worsen the catastrophic spending associated with brain tumor surgeries in these climes. While these challenges appear daunting but certainly have not been enough to deter the "can do" spirit of neurosurgeons in developing countries, it is essential to strengthen the training of pediatric neurosurgeons in LMICs and provide a platform for the advocacy of better infrastructure for the surgical management of these tumors.

Defining a Taxonomical Map for Craniosynostoses: An Integrated Nomenclature for Interdisciplinary Workflow and Problem Mapping in Craniosynostoses Management: A Feasibility Study.

Craniosynostoses is a complex clinical and management challenge. This is further complicated by varying phenotypes and specific challenges. Most complex craniosynostoses are approached and managed the same way despite the clinical variability. The goals for craniofacial surgery are common, but the nomenclature based on phenotype, genotype, sutural involvement, or syndromic nature does not shed light on the management workflow. The descriptive diagnosis and terminologies do not highlight the priorities and the operational issues, and most specialists dealing with these conditions remain in their field of tunnelled vision. In this article, the authors propose a concept of categorization based on initial patient presentation. In addition, the etiology and the treatment status, if available, are incorporated along with the presentation. The utility of our proposed categorization is to create a goal-based universal language among various specialists involved.

Split cord malformation associated with congenital dermoid cyst and myeloschisis - case-based literature review on possible embryonic derivation and implications.

Split cord malformation co-existing with either congenital dermoid or myeloschisis has been previously reported. Theories exist which explain the underlying embryopathy behind the occurrence of each of these anomalies in conjunction with split cord malformation. However, the occurrence of all three anomalies in the same patient, to the best of our knowledge, has not yet been reported in the literature. We report two cases on the co-existence of congenital dermoid cyst with both myeloschisis and split cord malformation and review the literature on previous postulations of mechanisms that could possibly explain the simultaneous occurrence of all three distinct anomalies. They involve all three primary germ layers and therefore share a common embryonic origin from the epiblast. We try to explain their co-existence based on the theory of notochordal splitting and endomesechymal tract formation consequent on endodermal-ectodermal adhesion as postulated by Beardmore and Wigglesworth and discuss on implications in terms of treatment paradigms. The unusual combination of all three anomalies in both patients probably highlights a need for further research on their pathogenesis despite these previous theories. These cases demonstrate the fact that currently accepted embryological explanations of most pathologies especially outliers still fall short with much remaining to be understood.

Ventriculoperitoneal shunt infection by Cryptococcus neoformans sensu stricto: Case report and literature review.

BACKGROUND: Cryptococcal ventriculoperitoneal shunt infection is known to occur due to an underlying infection in the patient rather than by nosocomial transmission of Cryptococcus during shunt placement. A case of chronic hydrocephalus due to cryptococcal meningitis that was misdiagnosed as tuberculous meningitis is described. CASE REPORT: Patient details were extracted from charts and laboratory records. The identification of the isolate was confirmed by PCR-restriction fragment length polymorphism of the orotodine monophosphate pyrophosphorylase (URA5) gene. Antifungal susceptibility was determined using the CLSI M27-A3 broth microdilution method. Besides, a Medline search was performed to review all cases of Cryptococcus ventriculoperitoneal shunt infection. Cryptococcus neoformans sensu stricto (formerly Cryptococcus neoformans var. grubii), mating-type MATα was isolated from the cerebrospinal fluid and external ventricular drain tip. The isolate showed low minimum inhibitory concentrations for voriconazole (0.06mg/l), fluconazole (8mg/l), isavuconazole (<0.015mg/l), posaconazole (<0.03mg/l), amphotericin B (<0.06mg/l) and 5-fluorocytosine (1mg/l). The patient was treated with intravenous amphotericin B deoxycholate, but died of cardiopulmonary arrest on the fifteenth postoperative day. CONCLUSIONS: This report underlines the need to rule out a Cryptococcus infection in those cases of chronic meningitis with hydrocephalus.

Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome.

OBJECTIVE: In this study, the authors aimed to 1) retrospectively analyze the early functional outcomes in a cohort of very young children with craniofacial dysostoses who underwent robot-assisted frontofacial advancement (RAFFA) or robot-assisted midface distraction (RAMD), and 2) analyze the utility of robotic assistance in improving the accuracy and safety of performing transfacial pin insertion for RAFFA or RAMD. METHODS: A retrospective analysis of a cohort of 18 children (age range 1-42 months at presentation), who underwent RAFFA or RAMD from February 2015 to February 2021 in the craniofacial unit at Amrita Institute of Medical Sciences and Research Centre in Kochi, India, was performed. Inclusion criteria were patients who had undergone RAFFA in a single stage or RAMD where the cranial vault had been addressed earlier, had been addressed on follow-up, or had not been addressed and had follow-up of at least 6 months. RESULTS: Overall, 18 children with syndromic craniosynostosis underwent LeFort level III midface distraction, with or without RAFFA, from February 2015 to February 2021 at a single center in India. The patients' ages ranged from 6 to 47 months at the time of the procedure. All patients had significant obstructive sleep apnea (OSA), significant ocular issues, and disturbed sleep as determined by the authors' preoperative protocol. Clinically significant intracranial pressure issues were present in 17 patients. None of the patients had injury due to the transfacial pin trajectory such as globe injury, damage to the tooth buds, or the loss of purchase during the active distraction phase. The mean distraction achieved was 23 mm (range 18-30 mm) (n = 16/18). Of the 18 patients, 10 (56%) had an excellent outcome and 6 (33%) had a satisfactory outcome. In all cases, the degree of OSA had significantly reduced after surgery. Eye closure improved in all patients, and complete closure was seen in 11 patients. On follow-up, the functional gain remained in 14 of 16 patients at the final follow-up visit. The distraction results were stable during the follow-up period (mean 36 months [range 6-72 months]). CONCLUSIONS: The early RAFFA and RAMD protocols investigated in this study gave a significant functional advantage in very young patients with craniofacial dysostoses. The results have demonstrated the accuracy and safety of robotic assistance in performing transfacial pin insertion for RAFFA or RAMD.

Intraoperative Neuromonitoring for Tethered Cord Surgery in Infants: Challenges and Outcome.

OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of intraoperative neuromonitoring (IONM) in surgery for tethered cord in infants. MATERIALS AND METHODS: The study included 87 infants who underwent surgery for closed spinal dysraphism under IONM. Their preoperative neurological and urological statuses were compared with postoperative status clinically. The study design was prospective, and the study's duration was from January 2011 to February 2020. IONM was performed (TcMEP and direct mapping) with an Xltek Protektor 32 IOM system, Natus Neurology/medical Inc., Middleton, USA. Statistical analysis in the form of χ2 is conducted using SPSS. RESULTS: Overall, among 87 patients, clinical improvement was seen in 28 (28/29) patients with motor deficits, 17 (17/24) with bladder deficits, and 18 (18/24) with bowel deficits. The monitorability for motor and sphincter was 97.3% and 90.7%, respectively. The sensitivity of IONM in predicting new motor deficit was 100%, whereas the specificity was 100%. The negative predictive value of predicting motor deficit was 100%, with a diagnostic accuracy of 100%. There were no complications in this cohort related to the IONM. CONCLUSIONS: The study has highlighted that the use of IONM is sensitive in identifying motor injury in infants with reliable outcome correlation. Assessment, monitoring, and outcome correlation of bladder and sphincteric functions are a challenge in this cohort.

Prognostication of the neurological outcome of tethered cord based on intraoperative neuromonitoring findings: how close can we get?

PURPOSE: To evaluate the correlation of intraoperative neuromonitoring (IONM) data in surgery for tethered cord syndrome (TCS) in children to the neurological outcome at 1-year follow-up. METHODS: 208 consecutive patients operated on for TCS, between January 2011 to February 2020, under electrophysiological monitoring in the Division of Paediatric Neurosurgery, AIMS, Kochi, India, were included. Their preoperative neurological, urological and orthopaedic status were compared with the postoperative status at 1 year follow-up. RESULTS: Our study prospectively collected the IONM data and retrospectively correlated it to the children's neurological outcome on follow-up. Out of 208 children, 28% (n = 59/208) had motor, 35% (n = 73/208) had bladder and 26% (n = 54/208) had bowel disturbances. Postsurgery, at one-year follow-up, 91% (n = 52/57) of the patients who had motor deficits had improvement, 82.3% (n = 51/62) of patients who had bladder deficits showed an improvement, and 88.8% (n = 48/54) with bowel deficits showed improvement. The monitorability for motor and sphincter potentials were 99.4% and 89.3%, respectively. Except for four patients (3 with bladder and 1 with motor worsening), all the patients who were monitorable with no deficits remained intact except for four patients, all the patients who had deficits and were monitorable improved after detethering (at 1 year). Clinical worsening corresponded to those who had a drop in amplitude of baseline TcMEP (n = 4). 18 events showed an increase in amplitude compared to the baseline TcMEP. These patients improved clinically on follow-up (at 1 year). CONCLUSIONS: IONM complements the preoperative clinical details in predicting immediate and long-term outcomes.

Indian Society of Pediatric Neurosurgery Consensus Guidelines on Preventing and Managing Shunt Infection: Version 2020-21.

BACKGROUND: Shunt infection is the most significant morbidity associated with shunt surgery. Based on the existing literature for the prevention and management of shunt infection, region and resource-specific recommendations are needed. METHODS: In February 2020, a Guidelines Development Group (GDG) was created by the Indian Society of Paediatric Neurosurgery (IndSPN) to formulate guidelines on shunt infections, which would be relevant to our country and LMIC in general. An initial email survey identified existing practices among the membership of the IndSPN, and eight broad issues pertaining to shunt infection were identified. Next, members of the GDG performed a systematic review of the literature on the prevention and management of shunt infection. Then, through a series of virtual meetings of the GDG over 1 year, evidence from the literature was presented to all the members and consensus was built on different aspects of shunt infection. Finally, the guidelines document was drafted and circulated among the GDG for final approval. Grading of Recommendations Assessment, Development and Evaluation (GRADE) system was used to grade the evidence and strength of recommendation. RESULTS: The guidelines are divided into eight sections. Level I and Level II evidence was available for only five recommendations and led to a moderate level of recommendations. Most of the available evidence was at Level III and below, and hence the level of recommendation was low or very low. A consensus method was used to provide recommendations for several issues. CONCLUSIONS: Although most of the recommendations for the prevention and management of shunt infections are based on a low level of evidence, we believe that this document will provide a useful reference to neurosurgeons not only in India but also in other low and middle income countries. These guidelines need to be updated as and when new evidence emerges.

Four novel mutations in EFNB1 in Indian patients with craniofrontonasal syndrome.

Craniofrontonasal syndrome (CFNS) (OMIM #304110) is a very rare, X-linked developmental disorder characterized by facial stigmata, including hypertelorism, frontonasal dysplasia, craniosynostosis, bifid nasal tip, and digital abnormalities. CFNS is caused by mutations in the Ephrin 1 gene (EFNB1) located at Xq13.1, which encodes the transmembrane protein Ephrin B1. Interestingly, heterozygous females are more severely affected than hemizygous males. We report on four individuals from four unrelated Indian families with mild-to-severe CFNS. All patients had variable degrees of hypertelorism and nasal bridge depression, which did not correlate with changes in other tissues. Although patients 3 and 4 showed the most severe facial dysmorphism and syndactyly, there were no structural CNS changes or developmental delay. In contrast, patient 1 displayed agenesis of corpus callosum and developmental delay, although facial and finger abnormalities were milder. Patients 1, 2, and 4 showed different degrees of clefting. DNA sequencing revealed four previously undescribed heterozygous mutations in exons 1 and 2 of EFNB1. Patient 1 carried the second single amino acid deletion reported up to date. The other three affected individuals harbored frameshift mutations, leading to premature termination codons. Our findings broaden the spectrum of EFNB1 mutations and illustrate the absence of an obvious correlation between mutation type, severity, and expression of symptoms.

Expanding the Spectrum of Robotic Assistance in Cranial Neurosurgery.

BACKGROUND: Robotic automation and haptic guidance have multiple applications in neurosurgery. OBJECTIVE: To define the spectrum of cranial procedures potentially benefiting from robotic assistance in a university hospital neurosurgical practice setting. METHODS: Procedures utilizing robotic assistance during a 24-mo period were retrospectively analyzed and classified as stereotactic or endoscopic based on the mode utilized in the ROSA system (Zimmer Biomet, Warsaw, Indiana). Machine log file data were retrospectively analyzed to compare registration accuracy using 3 different methods: (1) facial laser scanning, (2) bone fiduciary, or (3) skin fiduciary. RESULTS: Two hundred seven cranial neurosurgical procedures utilizing robotic assistance were performed in a 24-mo period. One hundred forty-five procedures utilizing the stereotactic mode included 33% stereotactic biopsy, 31% Stereo-EEG electrode insertion, 20% cranial navigation, 7% stereotactic catheter placement, 6% craniofacial stereotactic wire placement, 2% deep brain stimulation lead placement, and 1% stereotactic radiofrequency ablation. Sixty-two procedures utilizing the haptic endoscope guidance mode consisted of 48% transnasal endoscopic, 29% ventriculoscopic, and 23% endoport tubular access. Statistically significant differences in registration accuracies were observed with 0.521 ± 0.135 mm (n = 132) for facial laser scanning, 1.026 ± 0.398 mm for bone fiduciary (n = 22), and 1.750 ± 0.967 mm for skin fiduciary (n = 30; ANOVA, P < .001). CONCLUSION: The combination of accurate, automated stereotaxy with image and haptic guidance can be applied to a wide range of cranial neurosurgical procedures. The facial laser scanning method offered the best registration accuracy for the ROSA system based on our retrospective analysis.

Tailored Strategies to Manage Cerebrospinal Fluid Leaks or Pseudomeningocele After Surgery for Tethered Cord Syndrome.

BACKGROUND: Cerebrospinal fluid (CSF) leaks are a dreaded complication after surgery for tethered cord and are associated with significant patient morbidity. Although many strategies for managing postoperative CSF leaks exist, this problem is still daunting, especially in very young patients. In this study, we compared different management techniques for CSF leaks or significant pseudomeningocele in patients with tethered cord syndrome (TCS). METHODS: We analyzed a cohort of children who underwent surgery for TCS from January 2011 to March 2016 (n = 260) and postoperatively experienced either a CSF leak or significant pseudomeningocele. A subset of patients presented with CSF leak (n = 25). We analyzed patient age, sex, presentation, leak appearance, management, and outcome. The different techniques of management were compared for efficacy and morbidity. RESULTS: The diseases associated with leak formation included lipomyelomeningocele (n = 16), myelocystocele (n = 4), and myelomeningocele (n = 5). Three children also had hydrocephalus. Management techniques included cystoperitoneal shunt (CPS) (n = 15), primary resuturing with local rotation flap of muscle (n = 3), external ventricular drain placement (n = 1), ventriculoperitoneal shunt (n = 3), external ventricular drainage (n = 1), and a combination of techniques (rotation flap with external drain; n = 1). Five patients who underwent primary wound revision experienced a leak and required a secondary intervention, but none of the patients who underwent CPS had any complications. CONCLUSIONS: In carefully selected cases, CPS performed early after CSF leakage is highly successful with low morbidity. The primary closure can be attempted for low-pressure leaks without an associated pseudomeningocele.

Single-stage deformity correction in children with neurogenic kyphoscoliosis: nuances and a proposal for risk stratification.

OBJECTIVE Neurogenic kyphoscoliosis in pediatric patients has varied causes and diverse management options. The most common management strategy is to stage the orthopedic and neurosurgical aspects of the correction at an interval of 3-6 months from each other. The aim of this study was to report the authors' experience with correction of deformity in children with associated neurological abnormalities requiring intervention as a single-stage surgical treatment. METHODS Of 591 pediatric patients with scoliosis treated at the Amrita Institute of Medical Sciences and Research Centre between January 2001 and January 2011, the authors identified all patients with neurogenic kyphoscoliosis who underwent deformity correction and a neurosurgical procedure in the same surgical session when they were younger than 15 years. Data regarding the demographic details, preoperative Cobb angle/kyphotic angle, surgical details, postoperative complications, cost of the procedure, and long-term neurological outcome were collected for each case and analyzed. Based on the results of this analysis, the authors propose a 2-category risk stratification system for the timing of deformity correction depending on the primary neurogenic etiology. RESULTS Ten cases involving patients with neurogenic kyphoscoliosis requiring both deformity correction and neurosurgical procedure met the criteria and were included in the analysis. All 10 patients were younger than 15 years; their mean age was 10.8 years. The average Cobb angle was 59.5°. Five patients underwent foramen magnum decompression, 5 patients underwent spinal cord detethering, and 1 patient had intraspinal intradural tumor excision. The mean estimated blood loss was 1177 ml (range 700-1550 ml), and the mean operative time was 4.4 hours (range 3-5.5 hours). There were no intraoperative complications. None of the patients had any new postoperative neurological deficits. The mean postoperative Cobb angle of the main thoracic curve was 12.6°. CONCLUSIONS Simultaneous deformity correction along with a definitive neurosurgical procedure in patients with neurogenic kyphoscoliosis can be performed without added morbidity and avoids both the added costs and the risk of complications associated with a second surgery. The risk stratification system proposed in this paper may serve as a guideline for case selection and help to optimize outcome.

Forgotten? Not Yet. Cardiogenic Brain Abscess in Children: A Case Series-Based Review.

BACKGROUND: Brain abscess is a significant cause of morbidity in patients with uncorrected or partially palliated congenital cyanotic heart disease (CCHD). Unfortunately, in the developing world, the majority of patients with CCHD remain either uncorrected or only partially palliated. Furthermore, a risk of this feared complication also exists even among those undergoing staged corrective operations in the interval in between operations. There have been no recent articles in the literature on the outcomes of surgical management of cardiogenic brain abscess in children. In this study, we aimed to describe the clinical and demographic profile of patients with cardiogenic cerebral abscess and to highlight the fact that uncorrected or palliated CCHD continue to be at risk for brain abscess. METHODS: This study was a retrospective analysis of 26 children (age <19 years) being managed for CCHD who were diagnosed with cerebral abscess managed surgically (26 of 39 of cases cerebral abscess in children), at Amrita Institute of Medical Sciences and Research Centre, Kochi, India between December 2000 and January 2014. Data collected retrospectively included demographic information, modes of presentation, diagnosis, location of abscess, details of the underlying heart disease, management of the cerebral abscess, and outcomes of management. RESULTS: The patient cohort comprised 26 patients (16 males and 10 females), with a mean age of 7.19 years (range, 1.5-19 years). Ten of the 26 patients (38%) required reaspiration after the initial surgery. On follow-up, all the patients had improved symptomatically and demonstrated no signs of cerebral abscess. CONCLUSIONS: Cardiogenic origin of cerebral abscess is the most common cause of cerebral abscess in children. Unresolved CCHD is a risk factor for the occurrence, persistence, and recurrence of cerebral abscess.

An Analysis of Outcome of Endoscopic Fenestration of Cavum Septum Pellucidum Cyst - More Grey than Black and White?

BACKGROUND: Cavum septum pellucidum (CSP) and cavum vergae are actually fluid-filled, generally communicating midline cavities located between the third ventricle and corpus callosum. There have been various reports of their association with many behavioral and psychiatric disorders. Infrequently, they have been associated with an obstructive hydrocephalus-like picture. Although the structure and management of CSP has long been known, it has been an enigma as far as functional significance and management indications are concerned. The authors of this article try to analyze the significance of a persistent cavum and involvement of the same entity in varied presentations ranging from an incidental imaging finding to acute hydrocephalus, and propose a possible implication on the present surgical intervention paradigm. PURPOSE: To assess the surgical outcome of fenestration of a CSP cyst. METHODS: Retrospective analysis of 3 patients who underwent endoscopic fenestration for CSP with obstructive hydrocephalus between 2012 and 2014 was done, and data were analyzed for symptomatic clinical improvement in particular behavior. RESULTS: Pre- and postoperative brain MRI showed a significant decrease in the size of the cyst as well as the ventricles. There were no recurrences during follow-up. All of the patients improved. CONCLUSIONS: (1) Endoscopic fenestration of symptomatic CSP cysts is a safe treatment option. (2) Neurocognitive assessment is essential in the evaluation and outcome assessment of CSP.

Extradural temporopolar approach for parahypothalamic hypothalamic hamartoma and use of posterior communicating artery as resection margin pointer.

BACKGROUND: Hypothalamic hamartomas (HH) are malformations originating from the hypothalamus and are associated with seizures, hormonal and behavioral abnormalities. METHOD: Most patients, especially those with a typical syndrome characterized by gelastic seizures, precocious puberty, cognitive decline, and behavior problems, are diagnosed in childhood. Pedunculated and parahypothalamic types of hamartomas are attached to the floor by a narrow or wide peduncle in the absence of distortion of the overlying hypothalamus. This location is most commonly associated with a clinical presentation of precocious puberty, and surgical removal has proved curative in small case series. Enthusiastic resection of hypothalamic lesions are known to produce severe hypothalamic disturbance while under resection might mean inadequate response to surgery. CONCLUSIONS: In this article, the authors describe the use of extradural temporopolar approach to hypothalamic hamartoma as an improvisation to improve access with reduced morbidity and describe a surgical nuance of using posterior communicating artery to determine a safe but maximal resection margin.

Role of endoscopy in recalcitrant intraventricular tuberculoma-innovative novel treatment adjunct.

INTRODUCTION: Intraventricular tuberculomas are rare entities. To the best of our knowledge, only 14 cases have been reported in English literature. Medical management of cerebral tuberculomas is well accepted. Intraventricular tuberculomas may be recalcitrant for unclear reasons. An effective management protocol for this entity is unclear. To the best of our knowledge, the definitive indication, timing, and possible role of surgery in these lesions have not been discussed in literature. MATERIALS AND METHODS: A 27-year-old nursing professional who was undergoing treatment for miliary tuberculosis at another center presented to us in 2008 with right hemiparesis, deteriorating vision, and progressive decline in consciousness. In addition to antituberculous therapy (ATT), she underwent multiple CSF diversion procedures for the obstructive hydrocephalus secondary to a recalcitrant third ventricular tuberculoma. Finally, she underwent endoscopic decompression of the lesion with a very good clinical response at 1-year follow-up. DISCUSSION: We discuss a patient with recalcitrant intraventricular tuberculoma managed using neuroendoscopy along with the standard antituberculous therapy. We also discuss in detail the technique we utilized for endoscopic management of this lesion. CONCLUSION: Being a rare entity, a consensus for management of these lesions is not possible, but we have demonstrated that neuroendoscopic management of these rare entities is an option giving the advantage of definitive diagnosis when required, hastening the resolution, and clearing the CSF pathway.