To review the clinical features and current understanding of spina bifida with an emphasis on the Indian Scenario. Selected articles and current English language texts were reviewed. The authors experience was also reviewed and analysed. Spina bifida is a common congenital anomaly encompassing a wide spectrum of neural tube defects.It is broadly classified as spina bifida aperta and occulta. With the prenatal screening, the incidence of aperta is gradually declining, whereas the detection of occulta has increased with the advent of magnetic resonance imaging. Over the years, the understanding of pathophysiology has made a significant changein the management of these anomalies. Early detection and complete correction can significantly reduce the neurological disability. This article is an overview of spina bifida with a special emphasis on Indian scenario.

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Central nervous system tuberculosis in children presents commonly as tubercular meningitis, post-tubercular meningitis hydrocephalus, and much more rarely as space-occupying lesions known as tuberculomas. The occurrence of this condition, though previously reported only in the developing world, is now frequently reported in human immunodeficiency virus positive migrants in the western world. The exact pathogenesis of this condition is still incompletely understood, and the mainstay of treatment is chemotherapeutic regimes. Neurosurgical intervention is rarely necessary, and is confined to cases of hydrocephalus after tubercular meningitis and to large tubeculomas with space-occupying effects.

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Background: Split cord malformation (SCM) is a rare condition. With decreasing incidence of neural tube defect (NTD) in the West, the reports of SCM are getting lesser and lesser. However, in India, spinal dysraphism is still a major problem encountered by the neurosurgeons. Objective: Our aim was to analyze 300 patients of SCM for their clinical features, radiological findings and outcome of surgery, which can throw light on the subject to others, who have less scope of finding these cases frequently. Materials and Methods: Over a 16-year period, we encountered 300 cases of SCM at AIIMS. Over the same period, more than 1500 cases of NTD were managed. SCM was noticed in 20% of cases with NTD. Skin stigmata were noted in two-third of the cases, and scoliosis and foot deformity were observed in 50% and 48% cases, respectively. Motor and sensory deficits were observed in 80% and 70% cases, respectively. Commonest site affected was lumbar or dorsolumbar (55% and 23%, respectively). In 3% cases, it was cervical in location. Magnetic resonance imaging (MRI) scan revealed a large number of anomalies like lipoma, neuroenteric cyst, thick filum and dermoid or epidermoid cysts. All the patients were surgically treated. In type I, bony spurs were excised, and in type II, bands tethering the cord were released. Associated anomalies were managed in the same sitting. Patients were followed up from 3 months to 3 years. Results: Overall improvement was noticed in 50% and stabilization in 44% cases and deterioration of neurological status was recorded in 6% cases. However, 50% of those who deteriorated improved to preop status prior to discharge, 7-10 days following surgery. Conclusions: SCM is rare and not many large series are available. We operated 300 cases and noticed a large number of associated anomalies and also multilevel and multisite splits. Improvement or stabilization was noted in 94% and deterioration in 6% cases. We recommended prophylactic surgery for our asymptomatic patients.

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Aim: Medulloblastoma is one of the most common posterior fossa tumors in childhood. The treatment-related side effects as well as predictive outcome still remain as a major challenge. The improved understanding of the disease and advances in molecular biology is changing the treatment paradigms from Chang's staging system to molecular risk stratification. However, surgery still remains as an important mainstay of therapy and is formidable. The role of radical surgery has always been a crucial factor in the outcome of these patients, the best survival being reported in patients who had total excision of the tumor and with no metastasis. Patient and Methods: An analysis of 365 patients (age<18 years) of medulloblastoma who underwent treatment at the Seth G.S. Medical College and King Edward VII Memorial hospital (KEM), Mumbai over a 25- year period (1985-2000 and 2001-2010) is presented. The clinical profile, radiological features, pathology and surgical nuances are discussed. Results: The most common age group affected was between 3 and 12 years. 75.3% presented with headaches, vomiting and 63.2% with papilledema. Sitting position was used in majority of cases. A total of 8 patients underwent shunting; all of them were in the postoperative period (5.19%). 92.2% (142 cases) had classical medulloblastoma, 5.1% (8 cases) had desmoplastic variant, 1.9% (3 cases) had anaplastic changes and 0.6% (1 case) had glial differentiation. The 5-year and 10-year progression free survival rate was 73 and 41% for average risk disease while for high risk disease rate it was 34%. The mortality rate was 2%. The quality of life was enhanced in patients who survived 5-10 years after treatment. Conclusion: Surgery for medulloblastoma is formidable. The option of sitting position for medulloblastoma surgery is still viable. A vigilant neuroanesthesiologist and a safe surgery are necessary to achieve a good postoperative result. Radiological characteristics are helpful adjuncts for determining effective surgical strategy. Permanent CSF drainage can be avoided in majority of patients and can be definitively considered in progressive symptomatic hydrocephalus. A safe maximal resection and a good Karnofsky score are paramount to ensure compliance with adjuvant therapy and contribute to an overall survival advantage.

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Background: Anterior encephaloceles are rare conditions. Except for a few places from South East Asia, no large series has been published in the World literature. Materials and Methods: At AIIMS, we have managed 133 cases over a 40-year-period from 1971 to 2010. Frontoethmoidal type was the most frequent, noticed in 104 patients, followed by nasopharyngeal nasal in 12 and orbital encephaloceles in 6 patients. Observation: Ten patients were adults over the age of 18 years and 15 patients were between 5 and 18 years of age. Swelling over the nose was reported in all 104 patients with frontoethmoid type. In nasopharyngeal type, patients presented with respiratory problem. Patients with orbital mass had proptosis, on the side of encephalocele. Computed tomography (CT)/Magnetic resonance imaging (MRI) was performed in 127 patients, which was able to delineate the bone defect and associated brain anomalies. All the patients were subjected to repair of encephalocele. Patients with hypertelorism required orbital osteotomies and correction of deformity. Outcome: There were four deaths, all prior to 2000. No death was encountered in the last 10 years. CSF leak was the commonest postoperative complication, noticed in 24 patients. Overall cosmetic outcome was good.

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Hydrocephalus is a common clinical problem seen in pediatric neurosurgical practice. Hydrocephalus involves dilatation of the cerebral ventricular system with corresponding, compressive effects on the parenchyma. It can be communicative or obstructive types. Congenital, acquired, infective, and secondary hydrocephalus have different clinical features with different modality of treatments. Ventriculoperitoneal shunt is the gold standard of treatment. Endoscopic 3 ^rd ventriculostomy is rapidly gaining prominence as an alternative. Various kinds of hydrocephalus, their pathophysiology, treatment and complications are reviewed.

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Objective: To study the clinical features and treatment outcome of pediatric patients with bony craniovertebral abnormalities. Materials and Methods: The authors studied 189 consecutive cases of pediatric bony craniovertebral junction abnormalities operated between 2001 and March, 2010. Results: The pathologies were developmental (n = 162), traumatic ( n = 18) and tuberculous (n = 9). Surgical procedures included transoral decompression (n = 118), occipitocervical fusion (OCF, n = 139), C 1 -C 2 fusion (n = 45), and posterior fossa decompression (n = 5). Methods for OCF included contoured stainless steel rods (n = 86), titanium lateral mass screws and plates (n = 47) and steel wires (n = 6). Constructs of all patients of posterior fixation with contoured rods and wires or lateral mass screw and rod who could be followed up were either stable/fused or were fused and stable. No implant failure was noticed among these two surgical procedures. However, 6 patients with C 1-C 2 fusion had broken wires on follow-up requiring repeat posterior fixation. Good neurological outcome was observed even in poor-grade patients. No significant effect on the curvature or growth of the spine was observed at follow-up. Conclusions: Pediatric craniovertebral junction anomalies can be managed successfully with good outcomes using a low cost contoured rod and wires.

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Objective: The objective was to assess the outcome of selective motor fasciculotomy in relieving upper limb harmful resistant spasticity and thereby to improve motor functions in persons with cerebral palsy. Materials and Methods: Twenty people having cerebral palsy (12 females and 8 males) with age ranging from 5 to 35 (mean 12.85) years with upper limb resistant spasticity due to spastic hemiplegia (n=7), triplegia (n=6), and quadriplegia (n=7) were assessed using Modified Ashworth Scale, Selective Voluntary Control Grade, Wee FIM Scale and hand function evaluation. Selective motor fasciculotomy was performed on the musculocutaneous nerve (n=13) for elbow flexors spasticity, median nerve (n=24) for pronators and radial wrist flexors spasticity and ulnar nerve (n=3) for ulnar wrist flexors spasticity. Pre- and post-op therapeutic exercises were performed. Results: Statistical analysis using the Wilcoxon Signed Ranks test showed significant reduction in spasticity and improvement in selective voluntary control, hand functions (grasp to hold a 2 inch rod), and Wee FIM (self-care domain in particular). There was no recurrence in spasticity and complications following surgery. Conclusions: The selective motor fasciculotomy of musculocutaneous, median, and ulnar nerves significantly reduces spasticity in the affected muscle groups and thereby improves the self-care (motor) functions in selected people with cerebral palsy who have harmful resistant spasticity without any organic shortening of the muscles. The procedure is safe and the spasticity does not recur.

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Pediatric brain tumors have always been challenging as well as intriguing in their anatomical, surgical, and postsurgical management-related issues. They are a heterogeneous set of pathologies involving different age groups in childhood and also differ widely from their adult counterparts as far as adjuvant therapies are concerned. Though neurosurgeons across the world are radical in surgery for most of the pediatric tumors, it can often be at the cost of future quality of life in suprasellar tumors. As the time has gone by, the pendulum has swung toward rather conservative and maximal safe surgical resections with adjuvant therapies coming to the forefront. Hence, the aim is to achieve a good quality of life for these children along with a control of tumor growth (rather than cure) and to again tackle the tumors, if required, once these children reach adolescence or adulthood. We have reviewed the literature for different pediatric suprasellar tumors and discussed their current management giving our perspective with illustrative cases.

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Study Design: A retrospective, case study of 64 pediatric patients who underwent spinal surgery for Pott's spine and have minimum 6 months follow-up. Objective: The aim of this study was to evaluate the surgical management and outcome of 64 pediatric Pott's spine cases operated by single surgeon over last 8 years, with a minimum follow-up of 6 months. The prognostic significance and the outcome of the demographic factors, location of the disease, and its surgical approach were also evaluated in these patients. Materials and Methods: The data collected retrospectively from the institute's record case sheets of the 64 pediatric patients with Pott's disease of the spine, operated between 2002 and 2010 in the Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, were analyzed. The study population consisted of 40 male and 24 female pediatric patients. Clinical findings included back pain, paraparesis, kyphosis, fever, sensory disturbance, and bowel and bladder dysfunction. Results: The most common region of Pott's disease was the thoracic region [21/64 (33%)] followed by craniovertebral junction (CVJ) [17/64 (27%)]. At presentation, all of the patients had neurological features of spinal cord compression. All patients were treated with antituberculous chemotherapy which was continued after the surgical intervention also. Transthoracic surgical approach [18/64 (28%)] was the most frequently required surgery followed by transoral decompression with posterior fusion [12/64 (19%)] depending on the site of disease and compression of neuraxis. Initially, more than two-third of the patients were in poor grade of Kumar and Kalra scoring as well as modified Kumar and Kalra scoring, which were reduced to about one fifth after the surgical intervention, and the outcome was good as the condition of 46 patients (72%) improved significantly. Conclusion: Currently, treatment of spinal tubercular infections requires a multidisciplinary team that includes infectious diseases experts, neuroradiologists, and spine surgeons. The key to successful management is early detection and timely and judicious surgical intervention, the decision of which needs to be taken in view of clinicoradiological compression of the spinal cord and nerve roots, age of the patient and responsiveness of ATT.

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Epilepsy is a common clinical entity in neurology clinics. The understanding of the genetics of epilepsy has undergone a sea change prompting re-classification by the International league against epilepsy recently. The prevalence rates of epilepsy in India are similar to those of developed nations. However, the large treatment gap is a major challenge to our public health system. Perinatal injuries are a major causative factor in pediatric group. We have discussed a few common etiologies such as neurocysticercosis and newer genetic epilepsy syndromes. We have also briefly touched upon the Indian experience in pediatric epilepsy surgery.

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Brain tumours form the most common type of solid tumour in children and more that 50% of these are infratentorial. Cerebellar astrocytomas and brain stem gliomas are the commonest posterior fossa glial tumours in children. Cerebellar astrocytomas represent up to 10% of all primary brain tumours and up to 25% of posterior fossa tumors in children, with Low grade gliomas forming the commonest of the cerebellar gliomas. They commonly present with symptoms and signs of raised intracranial pressure due to obstructive hydrocephalus. Radiologically they may be solid or cystic with or without a mural nodule. Surgical excision is the mainstay of treatment and forms the most consistent factor influencing progression free and long term survival. While majority of the tumours are pilocytic astrocytomas, they may also be fibrillary astrocytomas or even high grade tumours. Tumour histology does not appear to be an independent factor in the prognosis of these children, and therefore no palliative treatment after surgery is advocated. Brain stem gliomas account for approximately 10% of all pediatric brain tumours. Cranial nerve signs, ataxia and cerebellar signs with or without symptoms and signs of raised intracranial pressure are classically described symptoms and signs. Radiographic findings and clinical correlates can be used to categorize brain stem tumours into four types: diffuse, focal, exophytic and cervicomedullary. Histologically most brain stem gliomas are fibrillary astrocytomas. Diffuse brain stem gliomas are the most commonly seen tumour in the brain stem. These lesions are malignant high grade fibrillary astrocytomas. Focal tumours of the brain stem are demarcated lesions generally less than 2 cms in size, without associated edema. Most commonly seen in the midbrain or medulla, they form a heterogeneous pathological group, showing indolent growth except when the lesion is a PNET. Dorsally exophytic tumours lie in the fourth ventricle, while cervicomedullary lesions are similar to spinal intramedullary tumours. Expanding lesions are the only lesions amenable for excision while infiltrative and ventral lesions are not.

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The development of pediatric neurosurgery in this country actually began with holding of the 17 ^th Annual Conference of the International Society for Pediatric Neurosurgery (ISPN) in 1989 in Mumbai. It subsequently led to the formation in 1990 of the Indian Society for Pediatric Neurosurgery (IndSPN), which is responsible for organizing regular annual conferences and CMEs in cooperation with the ISPN. The first three international CME programs were arranged in 1992, 1994 and 1995, followed by the next three courses from 1998 to 2000. Subsequently, five more such programs were organized in 2002, 2004, 2007, 2009, and 2010. The official publication, Journal of Pediatric Neurosciences (JPN), which was started in 2006, has also made good progress in the last 5 years. In the last 20 years, the IndSPN has made striking progress in the development and growth of pediatric neurosurgery in the country and has successfully managed to bring this subspeciality to an international level. The IndSPN now has about 150 active members and quite a few of them practice mainly pediatric neurosurgery. In large teaching institutions, pediatric neurosurgery has been accepted as a subspeciality with a designated neurosurgeon taking care of the pediatric patients. This augers well for the subspeciality as persons with greater interest and expertise will be tackling its problems. Some of these institutions have recently started or are in the process of starting the 1-year fellowship training program in pediatric neurosurgery.

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Modern epilepsy started in India in 1995 at Sri Chitra Tirunal Institute of Medical Science and Technology, Trivandrum and at All India Institute of Medical Sciences, New Delhi. At both centres the attempt was to get the program going with patients having surgically remediable epilepsy syndromes -who could be evaluated with non invasive investigations. The mainstay of the evaluation was a good quality epilepsy specific MRI and video EEG coupled with a SPECT study and a neuropsychological evaluation. Concordance of the focus on all investigations was critical to a good outcome. There were several problems on the way - but they were managed keeping in consideration our local needs and requirements. Intraoperative electocorticography was done and good outcomes attained. The critical determinants of success were the formation of a team with various interdisciplinary specialists and a strong will to succeed.

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Background: Diverse tumors in the posterior third ventricular region (TPTVR) frequently occur in children. A decade's experience with pediatric TPTVR is presented, focusing on the Indian perspective. Materials and Methods: 25 children (age range: 3-18 years; mean age: 13.32 years; presentation range: 7 days-2.5 years) had clinico-radiological assessment with contrast computed tomography (CT) and magnetic resonance imaging (MRI). The ventricular/lumbar cerebrospinal fluid (CSF) alpha feto protein (AFP)/beta human chorionic gonadotrophin (HCG) estimation was done when radiological suspicion of a germ cell tumor was present. Extent of resection was deemed partial when some tumor mass remained at the end of surgery, near total when <10% was retained over vital neurovascular structures, and total when complete resection was attained. Results: Operations included infratentorial supracerebellar approach (n = 12), occipito-transtentorial approach (n = 2), endoscopic biopsy and third ventriculostomy (n = 1), frontal parasagittal craniotomy, interhemispheric transcallosal subchoroidal approach (n = 2), middle temporal gyrus transcortical transventricular approach (n = 1), fronto-temporo-zygomatic combined transylvian and subtemporal approach (n = 1) and right ventriculoperitoneal shunt and stereotactic biopsy (n = 1). Only CSF diversion was performed for five patients with a small TPTVR. CSF diversion was required in 12 (48%) patients. Tumor pathology included pinealoblastoma (n = 4; one with pineocytic differentiation), nongerminomatous germ cell tumor (NGGCT; n = 3), germinoma (n = 3), pilocytic astrocytoma (n = 2), epidermoid (n = 3) and primitive neuroectodermal tumor (PNET), fibrillary astrocytoma, glioblastoma, teratoma, and meningioma (n = 1, respectively). A patient with neurocysticercosis was diagnosed solely on MRI (four did not undergo biopsy). Fractionated radiotherapy was administered in 13 patients with primary pineal tumors, PNET, NGGCT, fibrillary astrocytoma and glioblastoma. Extent of excision was total in 10 (40%), near total in 5 (20%), partial in 3 (12%) and a biopsy in 2 (8%) patients. Conclusions: Histopathologic characterization of TPTVR is essential prior to their further management. Benign lesions often have a good prognosis following gross total surgical resection. Pure germinomas are highly susceptible to radiotherapy. NGGCTs often have malignant components that require adjuvant therapy following surgery. The advancements in microsurgical techniques have led to gratifying perioperative results in these deep-seated lesions.

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Intramedullary tumors of the spinal cord account for 35-40% of intraspinal tumors in children. The biological behavior of these tumors is of slow progression, and hence aggressive surgery has been advocated. Surgical adjuncts include use of intraoperative neurophysiological monitoring, preoperative ultrasound, microsurgical techniques and ultrasonic suction devices. Osteoplastic laminoplasty approaches avoid post-laminectomy deformities in younger children. Postoperative radiotherapy and more recently chemotherapy regimes have been proposed for incompletely resected tumors.

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Pediatric aneurysms are different from adult aneurysms - they are more rare, are giant and in the posterior circulation more frequently than in adults and may be associated with congenital disorders. Infectious and traumatic aneursyms are also seen more frequently. Vein of Galen malformations are even rarer entities. They may be of choroidal or mural type. Based on the degree of AV shunting they may present with failure to thrive, with hydrocephalus or in severe cases with heart failure. The only possible treatment is by endovascular techniques - both transarterial and transvenous routes are employed. Rarely transtorcular approach is needed. These cases should be managed by an experienced neurointerventionist.

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Craniostenosis is a common problem in the pediatric neurosurgery departments. The management of this problem is still evolving. Some misconceptions exist regarding this condition particularly regarding the indications for surgery. The author started performing this surgery nearly two decades ago. The experience gained over time as well as the problems encountered are discussed.

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