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.

Dorsal bony spur in pediatric split cord malformations: eight-year experience from a tertiary care hospital.

INTRODUCTION: Dorsal spurs in Type I split cord malformations (SCM-I) are infrequent findings. The pathogenesis of the same is debatable. The objective of this study is to analyze our experience with SCM-I patients having dorsal bony spurs. METHODOLOGY: Retrospective analysis of SCM patients operated from 2010 to 2017 was performed. Their demographic profile, clinic-radiological features, operative findings, and outcome following surgery were recorded. RESULTS: Twenty-four cases of Type I SCM harboring dorsal bony spurs were identified with mean age of 4.96 years. The commonest split site was lumbar, documented in 62.5%. Scoliosis was observed in 58.3%. Pre-operative neurological deficits were seen in 66.6% cases with asymmetric weakness of limbs seen in 16.6%. There was no new neurological deficit observed post-operatively. CONCLUSIONS: This is the largest series of dorsal spurs occurring in SCM, reported in literature so far. Meticulous pre-operative evaluation and imaging are important to identify dorsal spurs for appropriate management and good clinical outcome. Differentiating dorsal spur from ventral spur is important as it has a bearing on surgical approach.

Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS).

PURPOSE: The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. METHODS: We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC z-score, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. RESULTS: Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up. CONCLUSION: ETV and shunting led to improvements in HC centile, z-score, and FOR measurements during long-term follow-up of infants with hydrocephalus secondary to aqueductal stenosis. Head size did not significantly differ between the treatment groups during follow-up, however ventricle size was greater in those undergoing ETV when measured at 1 and 3 years following treatment.

Microvascular decompression versus stereotactic radiosurgery as primary treatment modality for trigeminal neuralgia: A systematic review and meta-analysis of prospective comparative trials.

OBJECTIVE: The current opinion among neurosurgeons regarding the selection between microvascular decompression (MVD) and gamma knife radiosurgery for trigeminal neuralgia is not based on clear evidence. In this meta-analysis, we have attempted to synthesize the findings of the prospective trials comparing the efficacy and complications of the two procedures as primary treatment modality for medically refractory trigeminal neuralgia. MATERIALS AND METHODS: The authors performed a systematic review of PubMed for manuscripts comparing the efficacy or complications of MVD and stereotactic radiosurgery for medically refractory trigeminal neuralgia. The data of the identified studies was pooled and a meta-analysis was done. RESULTS: Five prospective studies fulfilling the eligibility criteria were identified. The mean age of the patients subjected to gamma knife therapy (GKT) was more than those who underwent MVD. The initial success rate in the pooled data with MVD was 96% (95% confidence interval [C.I.] 93.3%-98.6%) as compared to GKT which was 71.8% (95% C.I. 64.9%-78.7%) with the ratio of 1.309 (95% C.I. 1.217-1.409; P= <0.001). This superiority was sustained till the last follow up available in all the studies. Out of the complications common to both procedures, MVD had a lower rate of facial numbness, with a risk ratio of 0.481 (95% C.I. 0.297-0.778); and dysesthetic pain, with a risk ratio of 0.470 (95% C.I. 0.172-1.286). CONCLUSIONS: MVD seems to be more efficacious than GKT as a first line treatment for trigeminal neuralgia immediately as well as on a long term basis. However, the dilemma regarding the choice of treatment to be adopted still remains for special subgroups of patients, like the elderly patients and those in whom no vascular compression has been found during surgery. Further studies are needed for elucidating the unequivocal treatment plan under these circumstances.

Standardization of the technique of silicon injection of human cadaveric heads for opacification of cerebral vasculature in Indian conditions.

A surgeon's understanding of the surgical anatomy can be greatly enhanced by the dissection of preserved cadaveric specimens. A reliable and inexpensive biological model for testing and standardization of dye injection concentrations is proposed utilizing the goat's head as a biological model. The first phase was concerned with standardization of the dye by titrating its concentration and injecting various amounts into cerebral vessels of a goat's head until an optimal concentration had been ascertained. In the second phase, this optimum concentration of the dye was injected into four human cadaveric heads following the same technique standardized using the goat's head. Upon dissecting the four cadaveric human heads which were injected with silicon dyes and preserved in 10% formalin, the vessels were all well-opacified and the brain was of near normal consistency and good for dissection, without showing any features of putrefaction. The goat model, having similar color, texture, and the handling as the cadaveric head, offers an opportunity to test indigenously manufactured polymerizing dyes in the future. This biological model, therefore, has the potential to considerably reduce the cost of cadaver preparation.

Microvascular decompression for hemifacial spasm: A systematic review of vascular pathology, long term treatment efficacy and safety.

INTRODUCTION: Hemifacial spasm (HS) is a rare disorder caused by the compression of facial nerve root exit zone (REZ) at the brainstem by a vascular loop. Microvascular decompression (MVD) is a popular treatment modality for HS. OBJECTIVE: The purpose of this study was to determine the long-term efficacy and safety of MVD for HS by assessing the effect of the procedure from the literature published over the last 25 years. MATERIALS AND METHODS: A systematic data review from 1992 to 2015 using specific eligibility criteria yielded 27 studies on MVD for HS, the data of which were pooled and subjected to a meta-analysis. RESULTS: The pooled odds ratio (OR) revealed by the meta-analysis showed that anterior inferior cerebellar artery was the most common offending vessel in 37.8% (95% confidence interval [CI]: 27.8-47.7%) of the patients. Complete resolution of HS was seen in 88.5% (95% CI: 86.7-90.4%) of the patients after a long-term follow up. The complication rate was low following MVD, the most common being temporary facial paresis in 5.9% (95% CI: 4.3-7.5%) of patients. CONCLUSIONS: MVD is a safe and effective treatment for HS with long-term benefits and a low complication rate.

Pressure ulcer in patients with severe traumatic brain injury: significant factors and association with neurological outcome.

AIMS AND OBJECTIVES: To assess the factors associated with development of pressure ulcer in patients with severe traumatic brain injury (TBI) and study its prognostic significance with respect to neurological outcome at three months. BACKGROUND: Patients with severe TBI are prone to develop pressure ulcer due to various factors, which have never been studied in detail. DESIGN: Prospective longitudinal study. METHODS: Eighty-nine patients of TBI in age group 20-60 years admitted with Glasgow Coma Scale (GCS) 4-8 without serious systemic disorder were enrolled for the study. Patient characteristics, haemoglobin, serum albumin levels at admission and their weekly changes till 21 days were noted along with daily assessment for presence of pressure ulcer. Mortality was assessed at 21 days and neurological outcome at three months through telephonic interview. RESULTS: Of 89 patients studied, pressure ulcer was observed in 6 (7%) and 14 (16%) at the end of two and three weeks, respectively. Pressure ulcer in univariate analysis was significantly associated with poorer GCS (p = 0·05), delayed enteral feeding (p = 0·005) and fall in haemoglobin at two weeks (p = 0·005). Only the latter two were found significant in multivariate analysis. Age, gender, surgical intervention, tracheostomy, prolonged fever and change in albumin had no significant association with pressure ulcer development. Presence of pressure ulcer was significantly associated with mortality at 21 days (p = 0·006) and unfavourable neurological outcome at three months (p = 0·01). CONCLUSIONS: The significant factors influencing pressure ulcer development in patients with TBI were delayed enteral feeding and fall in haemoglobin. Pressure ulcer had significant association with mortality at 21 days and recovery status at three months. RELEVANCE TO CLINICAL PRACTICE: Early nutritional supplementation and monitoring of haemoglobin should be an important part of nursing care interventions for patients at increased risk of developing pressure ulcer.

Large pontine tubercular abscess treated surgically.

Tuberculosis of the central nervous system (CNS) is a life threatening condition with 50% mortality in advanced disease and serious neurological deficits in those who survive. Tuberculous abscess is a rare manifestation of CNS tuberculosis, brainstem involvement being even rarer. The management of these conditions poses a great challenge to the treating physician. We report a case of large tubercular abscess of pons which increased in size on anti-tubercular treatment, but showed excellent improvement following craniotomy and aspiration.

A rare case of ventricular diverticulum in a child with occipital encephalocele.

Ventricular diverticula are dilatations of the ventricular system caused by stretching and protrusion of the ventricular wall as a result of severe chronic obstructive hydrocephalus. We report a case of lateral ventricular diverticulum arising from the occipital horn in a patient with occipital encephalocele. To the best of our knowledge, this is a first case of ventricular diverticulum in a patient with occipital encephalocele.

Craniovertebral Junction Anomalies: An Overlooked Cause of 'Posterior Circulation Stroke'.

Background: Vertebral artery dissection (VAD) is a treatable cause of vertebrobasilar ischemic stroke and can be spontaneous or more commonly traumatic. Craniovertebral junction (CVJ) anomalies are a rare and often overlooked cause of VAD. Objective: The objective of this study was to study cases where CVJ anomaly presented as posterior circulation infarct and to conduct a relevant literature review. Materials and Methods: The medical records of seven patients who were managed for posterior circulation infarct associated with CVJ anomaly at our center from January 2009 through August 2013 were reviewed. PubMed and MEDLINE databases were also searched for similar cases, and the published case reports/series were reviewed. Results: Seven patients met our inclusion criteria and were included in the study. The mean age was 17.4 years (range: 10-35 years). All the patients were males. The most common symptoms were headache, vomiting, and gait ataxia. Slurring of speech was seen in one patient. One patient had repeated episodes of gait ataxia with left-sided weakness with complete recovery in between the episodes. One patient presented in unconscious state. Four patients complained of vertigo. The median duration of symptoms was 7 days (range: 3 days-12 months). Conclusions: CVJ anomalies can present as posterior circulation infarct. One must evaluate all patients with posterior circulation stroke, especially young patients, for possible CVJ anomalies. Dynamic lateral cervical spine X-ray is an important tool to diagnose AAD. CVJ anomalies represent a treatable cause of VAD.

Association of matrix metalloproteinase-1 gene polymorphism with glioblastoma multiforme in a northern Indian population.

Matrix metalloproteinase-1 (MMP-1) is known to be involved in the pathogenesis of glioma. It damages the extra-cellular matrix to produce invasiveness in cancer tissue, and hence has a direct effect in cancer invasion. The study aims to explore the association of single nucleotide polymorphism of -1607 MMP-1 gene with susceptibility to glioblastoma multiforme (GBM) in northern Indian subjects. One hundred and ten GBM patients and 150 healthy controls were included in this study. 1607 MMP-1 gene was studied by PCR-RFLP; different genotypes being combinations of 1G and 2G allele (1G/1G, 1G/2G and 2G/2G). 2G/2G genotype was significantly associated with GBM patients (OR, 2.24; P = 0.016; 95% CI, 1.16-4.30) as compared to controls. Prevalence of the 2G allele of -1607 MMP-1 polymorphism was significantly greater in GBM patients as compared to controls (62.3 vs 48.3%, OR, 1.76; P = 0.002; 95% CI, 1.23-2.52). This study suggests that the 2G/2G genotype and 2G allele of -1607 MMP-1 polymorphism are associated with an increased susceptibility for developing GBM.

Delayed bilateral thalamic bleeding post-ventriculoperitoneal shunt.

Ventriculoperitoneal shunt is one of the most common procedure done by neurosurgeon worldwide. We present a rare case of delayed intracerebral bleed post VP shunt and discuss the possible causes of intracerebral hemorrhage post ventriculoperitoneal shunt and bilateral thalamic bleed. The presumed cause in our case was shunt induced disseminated coagulation profile.

Craniosynostosis associated with neural tube defects: is there a causal association?

The majority of primary craniosynostosis cases are sporadic. Very few articles in the literature have described cases of primary craniosynostosis associated with neural tube defects (NTDs). This co-occurrence has been seen by most authors as just a coincidence. The authors report a clinical series of 4 patients of primary craniosynostosis associated with NTDs treated at their center. Among these 4 cases, 2 had lumbosacral myelomeningocele, 1 frontoethmoidal encephalocele and 1 had occipital encephalocele. Although the co-occurrence of craniosynostosis and NTD is said to be rare, there seems to exist a justified underlying explanation for the same. The NTD causes a decrease in intracranial pressure due to egress of cerebrospinal fluid in the malformed sac that results in a deficient cerebral impulse for cranial growth that might stimulate premature sutural fusion. Thus, all patients with NTDs should be thoroughly evaluated for this association and for possible surgical management.

Chiari III malformation with proatlas abnormality.

Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. They are characterized by an occipital or cervical encephalocele, along with anomalies commonly seen with the type II malformation. Chiari III in association with proatlas abnormality is very rare. Here we describe a patient with Chiari III malformation associated with occipital condyle hypoplasia.

Successful surgical excision of a nondysraphic holodorsal intramedullary lipoma in a 14-month-old child.

INTRODUCTION: Nondysraphic intramedullary lipomas of the spinal cord are rare lesions. They are most commonly seen in the second or third decade of life. Their occurrence in the pediatric age group is even rarer. CASE REPORT: The authors describe a 14-month-old child who presented with delayed motor milestones involving the bilateral lower limbs. The child was unable to sit or stand. MRI revealed a dorsally located intramedullary lipoma extending from C7 to D12. The child underwent C7-L1 laminotomy and gross total excision of the lipoma. Postoperatively, the child improved neurologically and attained normal power in the bilateral lower limbs. DISCUSSION: Very few cases of extensive intramedullary lipomas involving the dorsal cord have previously been reported. Only two cases of pediatric extensive dorsal lipomas have been mentioned to date. The authors describe successful surgical excision of a holodorsal intramedullary lipoma in a 14-month-old child. To the best of the authors' knowledge, this is the first case being reported of a holodorsal intramedullary lipoma in the youngest patient.

Hydrocephalus Research.

OBJECTIVE: This paper highlights the hydrocephalus research efforts undertaken at AIIMS, New Delhi, supporting progress in the field. MATERIAL: Over a period of three decades, basic research, clinical investigations, and multicentric studies were undertaken. This report will review the work mainly to emphasize the need for future generations to pursue further research. Studies that impacted hydrocephalus care (mainly in India) are described, and some of these findings may be useful in other resource-challenged situations. RESULTS: Investigative studies on the effect of shunting on brainstem auditory evoked responses (BAER), transcranial Doppler (TCD), and CT-SPECT were published offering management options for patients. Participation in the International Infant Hydrocephalus Study (IIHS) study offered opportunities to compare our approaches and develop modifications in patient care. This effort proved shunting was equal or better for young children with congenital aqueductal stenosis. Shunt infection protocols and changes made in a systematic manner helped develop local protocols to reduce postoperative shunt infections. CONCLUSIONS: Hydrocephalus research over three decades at AIIMS, New Delhi was productive and educational, confirming that locally performed investigative work can help in decision making. Further studies and active participation in international efforts are necessary to advance the field.

Calvarial tuberculosis: Uncommon manifestation of common disease--a series of 21 cases.

OBJECTIVE: Tuberculosis is an endemic disease in developing countries like India. It can involve almost any part of human body. One such, albeit rare, is the extra pulmonary manifestation - tuberculosis of calvaria. There have been many isolated case reports and short series published on this unusual presentation. The aim of this presentation is to acquaint the reader with the varied presentation; to emphasise the role of prompt surgical debridement; as also to stress the role of complete anti-tubercular drug therapy in the management of this rare disease. METHODS: A retrospective analysis of a total of 21 patients over a period of 10 years period (July 1995 to June 2005). All patients were screened for the evidence of primary pulmonary tuberculosis. As part of their radiological workup, they were subjected to either a CT scan-head or MRI-brain. All patients were surgically managed at the institute. ATT was prescribed for 18 months. Follow-up period ranged from 6 months to 8 years. RESULTS: All patients improved following the completion of therapy with no evidence of recurrence. CONCLUSION: Calvarial tuberculosis is endemic in developing countries. This disease mainly affects the young population. With the rising incidence of AIDS, neurosurgeons all over the world are more likely to be exposed to this disease. CT-head and MRI-brain is the mainstay for radiological diagnosis. Definitive diagnosis rests on a biopsy report. Surgical debridement followed by anti-tubercular therapy for 18 months is the mainstay of the management of this unusual presentation.

Frequent loss of heterozygosity and altered expression of the candidate tumor suppressor gene 'FAT' in human astrocytic tumors.

BACKGROUND: We had earlier used the comparison of RAPD (Random Amplification of Polymorphic DNA) DNA fingerprinting profiles of tumor and corresponding normal DNA to identify genetic alterations in primary human glial tumors. This has the advantage that DNA fingerprinting identifies the genetic alterations in a manner not biased for locus. METHODS: In this study we used RAPD-PCR to identify novel genomic alterations in the astrocytic tumors of WHO grade II (Low Grade Diffuse Astrocytoma) and WHO Grade IV (Glioblastoma Multiforme). Loss of heterozygosity (LOH) of the altered region was studied by microsatellite and Single Nucleotide Polymorphism (SNP) markers. Expression study of the gene identified at the altered locus was done by semi-quantitative reverse-transcriptase-PCR (RT-PCR). RESULTS: Bands consistently altered in the RAPD profile of tumor DNA in a significant proportion of tumors were identified. One such 500 bp band, that was absent in the RAPD profile of 33% (4/12) of the grade II astrocytic tumors, was selected for further study. Its sequence corresponded with a region of FAT, a putative tumor suppressor gene initially identified in Drosophila. Fifty percent of a set of 40 tumors, both grade II and IV, were shown to have Loss of Heterozygosity (LOH) at this locus by microsatellite (intragenic) and by SNP markers. Semi-quantitative RT-PCR showed low FAT mRNA levels in a major subset of tumors. CONCLUSION: These results point to a role of the FAT in astrocytic tumorigenesis and demonstrate the use of RAPD analysis in identifying specific alterations in astrocytic tumors.