Chiari III malformation is an extremely rare anomaly characterized by low occipital and high cervical encephalocele with herniation of posterior fossa contents, that is, the cerebellum and/or the brainstem, occipital lobe, and fourth ventricle. We report the case of Chairi III malformation in a neonate with associated hypertelorism and microcephaly, discuss the etipopathogenetic and radiological features, and review the pertinent literature. The neonate, a two-day-old child, with Chairi-III malformation had a large low occipital and cervical osseous defect with nearly a nonexistent posterior fossa due to the herniation of the cerebellum and a part of the occipital lobe into the large encephalocele sac. The patient also had associated microcephaly and hypertelorism without hydrocephalus. The existence of significant, viable neural tissue within the encephalocele sac precluded any surgical intervention. In our patient, it is proposed that during early embryogenesis, incomplete distension of the telencephalic and the rhombencephalic ventricles resulted in Chiari III malformation (based upon unifying theory of embryogenesis of McLone and Knepper) with a large encephalocele containing major portions of cerebellum and occipital lobe, and left the chondrocranium without an adequate inductive force of the underlying neural mass. The skull was, therefore, microcephalic and the posterior fossa virtually nonexistent. Hypertelorism also resulted from failure of eyes to completely rotate forward during fetal life owing to the presence of this large posterior encephalocele and the absence of neural cues for the chondrocranial expansion. To the best of the authors' knowledge, the association of Chairi-III malformation with microcephaly and hypertelorism has not been previously reported.

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Ehler-Danlos syndrome (EDS) is heritable connective tissue disorders with varied manifestations whose primary clinical features include soft, hyperextensible skin, dystrophic scarring, easy bruising, and joint hypermobility. Os odontoideum describes a condition in which the dens is separated from the axis body. The exact frequency is unknown. Known cases are either incidentally detected or are diagnosed when patients become symptomatic. Cervical dislocation has been described with type VI lesions but no association with Os odontoideum was found in the English literature. We describe a case of 5-year-old boy a case of Ehler Danlos syndrome presenting with features of cervical dislocation due to Os odontoideum.

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Surgery for Lipomyelomeningocele is complex. A systematic approach and methodology can make the surgery safe and improve the outcomes. The technique is been described.

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Medullobastomas are common tumors in children located usually in cerebellar vermis. In adults also, they present and are likely to be laterally placed. Their incidence in rare sites like cerebellopontine angle is also described. The presentation, neuroradiological appearance, and brief summary of histopathological findings are discussed.

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Out of 2000 clinically suspected cases of bacterial meningitis in children admitted between February 2005 and February 2008, bacteria were isolated in 34 (1.7%) cases. Streptococcus pneumoniae was the commonest isolate (12 cases) followed by Acinetobacter sp. (four cases), Escherichia coli, Enterobacter sp., and Enterococcus sp. (three each). Pseudomonas aeruginosa, Group B Salmonella and Hemophilus influenzae were isolated from one case each. Sphingomonas sp. has emerged as newer pathogen. Multidrug resistance was observed in Acinetobacter sp., E. coli, Enterobacter sp., and methicillin resistant Staphylococcus aerues. Five deaths occurred due to multidrug resistant strains.

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Human tail refers to a congenital cutaneous appendage protruding at the lumbosacral region. This interesting anomaly is often associated with occult spinal dysraphism and presents with treatment dilemma. Management of such lesions must include complete neurological examination and magnetic resonance imaging. Appropriate management including early diagnosis and microsurgical intervention can prevent development or progression of severe neurological deficits in later life. In this article, three cases of human tails are reported along with their successful treatment and review of the literature.

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Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a 3-year-old boy presented with rapidly progressive paraparesis and neurogenic bladder. A thoracolumbar magnetic resonance imaging demonstrated an intrasipnal mass from the T12 to L3 level. An operation was performed with gross total tumor removal. Histologic examination revealed a small round cell tumor and immunohistochemical characteristics of PNET. There was no clinical or radiological evidence for existence of intracranial tumor. The parents denied chemotherapy and radiotherapy. Metastases to lung was noted at 6 months after surgery and died due to respiratory failure at his local hospital. The clinical, imaging, and pathological features are discussed with a review of the literature.

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Spina bifida is a common neural tube defect. The relative deficiency of folic acid (FA) in mother during periconceptional period is an important factor. We measured the serum FA levels in spina bifida babies and their mothers and compared the result with the FA level in normal babies. The blood samples were collected at the time of admission to hospital. Enquiry made about the age, sex, and birth order, problem in siblings and FA supplementation and detailed examination done to access the severity of the problem. Folic acid estimation was done by enzyme-linked immunosorbent assay method. Antenatal FA supplementation was found in 40% of cases and 80% of control group. In the study group, the mean FA level in babies and mothers of FA supplemented group was 21.73 ± 5.64 ng/dl and 15.02 ± 3.02 ng/dl and in non-supplemented group were 7.41 ± 1.48 ng/dl and 4.12 ± 0.81 ng/dl, respectively. In control group mean FA level in babies and mothers of FA supplemented group were 16.88 ± 3.2 ng/dl and 14.70 ± 2.30 ng/dl and in nonsupplemented group were 14.33 ± 1.74 ng/dl and 10.75 ± 1.34 ng/dl, respectively. The serum FA levels in spina bifida babies as well as in their mothers were significantly low as compared to control group. This study suggests that the low serum FA levels in a lady at the time of pregnancy may be a causative factor in the pathogenesis of spina bifida and low levels in affected babies.

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A rare complication of ventriculoperitoneal (VP) shunt is presented. A 6-year-old boy presented with a tube coming out of the mouth. He had a VP shunt done earlier. Clinical features and imaging studies showed that the peritoneal end had perforated the stomach and then migrated to and excited from the mouth. The shunt was removed and he made an uneventful recovery. Though migration of the peritoneal end of the shunt tube into various organs is known, only five cases have been reported in the English literature of a shunt tube coming out of the mouth and this is the sixth. The management of this very rare problem is discussed and the literature reviewed.

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To report a case of unilateral optic nerve aplasia with unusual presentation and to review the literature. Clinical and histopathological features of a 36-week-old gestation male infant were described with review of all cases of optic nerve aplasia in the literature. The unusual findings in this case are the systemic congenital anomalies. Twenty-nine out of forty-two cases reported in the literature are true cases of optic nerve aplasia; the others probably represent optic nerve hypoplasia. Out of four cases reported as bilateral, only one case could possibly represent bilateral optic nerve aplasia. Optic nerve aplasia is very rare anomaly. It is overdiagnosed entity in the literature. Bilateral cases are exceedingly rare.

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Astroblastoma is thought to arise from astroblast - an intermittent cell between spongioblast and astrocytes and is a rare tumor in the pediatric age group. The histological appearance are characterized by radiating arrangement of spindle-shaped tumor cells forming perivascular pseudorosettes, and being very similar to that seen in ependymomas, makes differentiation difficult. The authors report the case of a 5-year-old girl who had a large frontal tumor, which was thought to be an astroblastoma preoperatively, based on the unique magnetic resonance imaging features these tumors have. The histological findings, in association with the preoperative radiology confirmed the diagnosis of astroblastoma. The authors conclude that the MR imaging of astroblastomas is extremely helpful in differentiating from ependymomas and is used for preparing the histopathology reports in these patients.

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Hydatid disease is caused by the infestation of the larvae of Taenia echinococcus. The definitive hosts of echinococcus are various carnivores, the common being the dog. All mammals (more often being sheep and cattle) are intermediate hosts. Humans are infected through the falco-oral route by the ingestion of food or milk contaminated by dog faeces that contain the ova of the parasite or by direct contact with dogs. We are reporting three cases of craniospinal hydatid cyst. First case of large intracranial hydatid cyst in a 9-year-old male child presented with holocranial headache with diminution of vision and right hemiparesis. Second case of a 34-year-old female presented with weakness, tingling, and pain right upper limb for 4 months with painless swelling right supraclavicular region. Third case of a 8-year-old child presented with low backache and paraparesis with acute retention of urine for 3 months. All the patients of craniospinal hydatidosis were managed in our department surgically.

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Ectopic lacrimal gland tissue (ELGT) of the orbit is seen rarely. A 5-year-old child with an ELGT of the right orbit causing to unilateral proptosis is presented. Computerized tomography and magnetic resonance imaging studies showed an irregular, soft-tissue in the right orbit, without bony erosion. The lesion was excised subtotally by right frontal transcranial approach.

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