Spina bifida transition care in India: strengths amidst challenges.

In India, adult neurosurgeons are required to care for children regularly because the concept of dedicated pediatric specialty care is not yet entirely established in the subcontinent. Likewise, pediatric neurosurgeons do not exclusively offer their services to the young, but they also provide care to adult patients with neurosurgical disorders. This creates a medical system where the transition between specialties is not often a formal and recognized aspect of neurosurgical care because most neurosurgeons provide care for patients of all ages. Additionally, there are very few teams geared toward caring for conditions in children that merit lifelong medical support, with spina bifida (SB) being one of them. Since there are no focused or structured pediatric programs on a large scale, developing a multidisciplinary clinic for adults becomes challenging. A pragmatic approach using technology-based education, supported by an organized system or a coordinator, may be a new strategy. A new system utilizing telemedicine and smartphones for established patients maybe an alternative option for SB children in India. During virtual video conferences, an established patient may benefit from multispecialty care and education toward a smooth transition that avoids significant issues with time, transportation, or financial constraints. Achieving a seamless transition among allied specialists from the pediatric to adult systems is a utopia. The current system in the subcontinent may be improved, with an opportunity to develop smooth transition care between coordinated specialists (who simultaneously treat children and adults). Learning from various global SB management styles, the Indian transition situation may offer another model in the near future.

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.

Multisuture and Syndromic Craniosynostoses: Simplifying the Complex.

Most complex craniosynostoses are managed the same way as syndromic craniosynostoses (SCs), as these patients often experience similar problems regarding cognition and increased intracranial pressure (ICP). The evaluation and treatment plan for craniosynostoses is complex, and this, additionally, is complicated by the age at presentation. In this article, the authors review the complexity of SCs in the presentation and management. An algorithm is necessary for such multifaceted and multidimensional pathology as craniosynostoses. In most algorithms, posterior calvarial distraction is a consistent early option for complex craniosynostoses presenting early with raised ICP. Addressing the airway early is critical when significant airway issues are there. All other surgical interventions are tailored on the basis of presentation and age.

Avoiding the antenatal counselling faux pas: bridging the gap between prenatal prognostication and postnatal outcome of closed spina bifida.

PURPOSE: Closed spina bifida (CSB) is rare in prenatal literature, and various lesions are grouped under this broad nosological entity CSB, leading to confusing and misleading prognostic conclusions. METHODS: This is a retrospective observational cohort study of prenatally detected CSB cases using two-dimensional ultrasound, complemented by three-dimensional ultrasonography and foetal MRI in indicated cases, from October 2014 to October 2021 in a tertiary-level single centre. RESULTS: The most common upper vertebral level of CSB was lumbar in 66.6% (10/15). The sub-classification of lesions based on prenatal ultrasound showed an agreement in 53% of the cases. Sixty percent had associated abnormalities identified postnatally, the most common being anorectal malformation seen in 33.3%. On postnatal follow-up, 46.6% had bowel incontinence and bladder dysfunction, and 33.3% developed lower limb deformities. CONCLUSIONS: All CSBs do not have a uniformly favourable prognosis. The prognosis of CSB depends on the pathological type, the presence of associated abnormalities and the management.

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.

Controversies in Hydrocephalus: QUO VADIS.

BACKGROUND: Hydrocephalus is a complex issue characterized by increased intracranial pressure secondary to obstruction of cerebrospinal fluid flow and occasionally due to overproduction. As a result, the entity has challenges of different dimensions at the level of understanding and management. METHODS: A literature search, systematic review, and meta-analysis of eligible studies were conducted in the major databases. The literature review included relevant articles on hydrocephalus published until June 1, 2021 (no starting date), databases being the only limitation considering the broadness of the subthemes. Controversies themes were chosen among the literature, not including treatment dilemmas and hydrocephalus research. The further detailed search included these selected themes and an updated literature review on the subjects. RESULTS AND DISCUSSION: Controversies are a hallmark of incomplete science; most complex concepts harbor several debates at various levels. This article reviews controversies in hydrocephalus, offering some updates on popular discussions. It is not meant to be an exposition of the topics themselves but to collect the status quo of unresolved concepts in hydrocephalus. CONCLUSIONS: As with most chronic and complex disorders, hydrocephalus welcomes controversy as a healthy discussion platform to exist until we understand the disorder to its minutest.

Should not we be using aspirin in patients with a ventriculoatrial shunt? Borrowing a leaf from other specialities: a case for surrogate evidence.

INTRODUCTION: Ventriculoatrial (VA) shunts are life-saving in circumstances where ventriculoperitoneal shunts (VP) have failed. They are at risk for different complications, and more specific of them are cardiopulmonary complications. Currently, there are no standard recommendations concerning screening for risk factors, prophylaxis, or anticoagulation treatment in patients after VA shunt placement. Our study aims to prospectively study the possible role and efficacy of the use of aspirin to increase the survival of shunts in children with VA shunt and avoid secondary morbidity. In this article, the authors describe the interim results of an ongoing prospective study which supports the use of aspirin for VA shunt. MATERIALS AND METHODS: The study design is prospective. The duration of the study is 2011 onwards and is ongoing. Hospital ethics board clearance and consent from the family were taken before inclusion in the study. All patients who had VA shunt were given a once-a-day low-antiplatelet dose of aspirin 5 mg/kg, from the first postoperative day onwards. Primary endpoints of the study are as follows: (1) major distal end malfunction documented on echocardiography or (2) any cardiac complications associated with the VA shunt catheter. RESULTS: We have 6 patient since march 2011, who are being followed up. None of the shunts had malfunctioned until the reporting. None of the patients had any cardiac issues reported. The patients are to be followed continually. The present follow-up ranges from 2.5 to 10 years. The patient follow-up is being continued. CONCLUSIONS: Aspirin is a drug with well-accepted safety profile, and its use and our preliminary observation and outcome of the use of aspirin in VA shunt are promising.

First report of Vibrio cholerae O9, novel st520, isolated from a child with bacteraemia-associated sepsis.

Vibrios have been identified to cause extra-intestinal complications apart from the occasional cholera-like diarrhoeal outbreaks. The non-O1/O139 Vibrio cholerae strains are ubiquitous in environmental water bodies and hence pose a threat to people even without obvious risk factors. We describe a case of sepsis in a child with spinal dysraphism caused by a V. cholerae O9 strain belonging to a novel sequence type (ST520). The present case highlights the need of considering V. cholerae non-O1/O139 as one of the pathogens while dealing with sepsis cases, and also, the study expounds the importance of proper characterisation of the pathogen for an effective treatment.

Effectiveness of Structured Teaching Program on Knowledge Regarding Home Care Management of Children with Hydrocephalus and Shunt among their Parents.

BACKGROUND: Parents of children with hydrocephalus play a crucial role in recognizing the early signs of shunt-related complications and to prevent morbidity and mortality. AIM: To evaluate the effectiveness of an educational intervention among the parents of children with hydrocephalus and shunt. MATERIALS AND METHODS: Quantitative, quasi-experimental one group pretest-posttest design was used for the study. Twenty parents of children (<5 years) with ventricular shunt were selected by non-probability convenience sampling technique from pediatric neurosurgery outpatient department of tertiary care hospital, Kerala, India. Tool 1 was used to assess the demographic and clinical variables of the sample and the child and tool 2 was the structured knowledge questionnaire on home care management of children with hydrocephalus and shunt. RESULTS: The difference in the mean pretest-posttest knowledge score was statistically significant at P < 0.01. There was a significant association between the mean knowledge score of the sample and the age of the mother, the age of the mother at pregnancy, and the previous knowledge from various sources. CONCLUSION: The study revealed that the parent-centered education was effective in improving the knowledge of parents regarding care of children with hydrocephalus and shunt. It helps them to extend the care of these children from hospital to home and hence improve their quality of life.

A proposal for a tailored protocol for focal suppurative infection of the central nervous system: analysis of an institutional experience in pediatric patients.

OBJECTIVE: The incidence of focal suppurative infections (FSIs) of the brain has significantly decreased owing to the better health and fundamental conditions of the population on the one hand and earlier detection and the availability of more potent antibiotics on the other. Interestingly, the antibiotic protocols have not been well defined in terms of duration despite a prompter diagnosis, definitive management of the etiology, and the advent of various higher-generation antibiotics. In this study, the authors evaluated the current treatment protocol. Their aim was to optimize management protocols for FSIs of the central nervous system based on clinical parameters. METHODS: The study was a retrospective analysis of all children who had undergone surgical management for an FSI at the Division of Paediatric Neurosurgery, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, in the period from January 2001 to February 2018. In addition to demographic characteristics, the details of culture reports and antibiotic regimens were collected. The response to treatment was compared to changes in the infective profile (C-reactive protein) and imaging. Instances of reaspiration were compared with clinical signs, imaging findings, and infective profiles. Treatment response was separated into two groups: responders within or at 2 weeks and responders beyond 2 weeks. The clinical characteristics of these two groups were compared. RESULTS: Forty-eight children were treated in the study period. Nineteen patients benefited from the 2-week (short-term) protocol of intravenous antibiotics. Twenty-nine patients required more than 2 weeks (approximately 4 weeks; long-term protocol) for resolution. Of those requiring more than 2 weeks, 69% had cardiogenic etiology. All patients were followed up with a minimum of 3 weeks of oral antibiotics. In a comparative analysis between short-term and long-term responders, only etiology was significantly different. None of the patients who had the short-term protocol had a recurrence. CONCLUSIONS: A shorter antibiotic protocol can be used successfully in carefully selected patients who are surgically treated and followed up. It is clear that the 2-week intravenous antibiotic protocol is more suitable for immunocompetent patients who have a noncardiogenic etiology.

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.