Spina bifida is one of the most common forms of neurulation defects. The etiology is probably multifactorial, with hereditary, nutritional, and possibly teratogenic factors playing a role. While there is no definitive etiology, it seems clear that the pathological process begins before the 4th week of gestation. Neurulation is normally complete by that time, but it is uncertain whether this disorder represents a failure of neurulation, or a rupture at some point after neurulation has become completed. In this context, the author reviews current views on the embryogenesis of neural tube defects and the possible role of folate to prevent spina bifida aperta.

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Neurosurgical treatment of intracranial hemorrhage in neonates was discussed with a special emphasis on the difference between preterm low birth weight neonates and mature ones. Intracranial hemorrhage in neonates is observed in about 10 to 15% of the preterm low birth weight babies cared for in NICU. It develops more commonly in neonates with a less than 1,500g birth weight. The periventricular germinal matrix layer (GML) develops in the fetus between 24 and 32 weeks' gestation. Because the GML involves not only a rich vascular supply but also hemodynamic weak points, it has been known as a bleeding source of subependymal or intraventricular hemorrhage observed in preterm low birth weight neonates. A poor general condition associated with an immature respiratory function and hemodynamic changes following the birth are also characteristics of the hemorrhage. Intracranial hemorrhage in neonates tends to be serious due to their fragile and immature vascular structure. Management of hydrocephalus following intraventricular hemorrhage in preterm low birth weight baby requires staged planning. It is important to note that less than 50% of neonates with ventriculomegaly following intraventricular hemorrhage develop hydrocephalus, which suggests that posthemorrhagic ventriculomegaly does not necessarily mean hydrocephalus. Once conservative treatment has failed, a ventriculo-subgaleal shunt or a cerebrospinal fluid reservoir is placed prior to the ventriculo-peritoneal (VP) shunt. The recommended body weight to install a VP shunt safely is 2,000 to 2,500g depending on the institutional experience. The prevalence of intracranial hemorrhage in mature babies is regarded as much lower than that in preterm low birth weigt ones. In such cases, contrary to the preterm low birth weight baby, the mature baby often has a background pathology for hemorrhage and a detailed examination to locate the bleeding source and pathology are immperative before surgery. Pathological backgrounds known to cause intracranial hemorrhage in mature babies are organic disease (AVM and other vascular anomalies, angioma), coagulopathy such as hemophilia, vitamin K deficiency, and trauma (mainly birth trauma). Intracranial hemorrhage of unknown cause is not unusual. Surgical treatment for intracranial hemorrhage in neonates should be individualized based on the age, body weight and general condition. It should be remembered prior to surgery that different pathological backgrounds trigger intracranial hemorrhage in neonates with preterm low birth weight and in matured babies.

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Background: Moyamoya disease is a chronic occlusive cerebrovascular disease with unknown etiology, which is one of the most common causes of child-onset stroke in Japan. Surgical revascularizations, both direct and indirect procedures, prevent cerebral ischemic attack by improving cerebral blood flow, while neurological deterioration during the acute stage after revascularization is not rare. The objective of this study was to clarify the concept of revascularization surgery for pediatric moyamoya disease while considering the risk of surgical complications in the acute stage. Patients and methods: The present study includes 19 consecutive patients with moyamoya disease aged from 2 to 14 years old (mean 8.5), who underwent superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis with indirect pial synangiosis for 32 affected hemispheres. Single-photon emission computed tomography (SPECT) was performed 1 and 7 days after surgery in all cases to evaluate hemodynamic alteration after surgery. Long-term outcome was evaluated by the neurological status 3 months after surgery, and the underlying pathology of surgical complications in the acute stage was diagnosed based on SPECT and magnetic resonance findings. Results: In 28 of 32 hemispheres (87.5%), patients showed a complete disappearance of ischemic attack, 4 of 32 hemispheres (12.5%) showed a reduction of ischemic attack, while none showed deterioration of their symptoms (0%). Transient focal neurologic deterioration due to cerebral hyperperfusion was evident in 2 patients (6.3%), and was resolved by blood pressure lowering. One patient developed pseudo-laminar necrosis probably due to a thrombosis one week after surgery (3.1%), which did not affect his long-term outcome. Conclusion: STA-MCA anastomosis with pial synangiosis is a safe and effective treatment for pediatric moyamoya disease. Routine cerebral blood flow measurement in the acute stage is essential to avoid surgical complications including both cerebral ischemia and hyperperfusion.

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The present pediatric traumatic brain injury guidelines outline several important topics of treatment and provide a detaileb, evidence-based system for management of children with severe TBI, although there are limitations due to the lack of strong evidence or proven efficacy for obtaining better functional outcome of the patient. Especially, securing the airway and maintaining proper breathing are paramount in the first step of the pediatric TBI management in order to prevent any extension of secondary brain injury. Breakthrough discoveries in the treatment of pediatric TBI can be expected, along with the development of therapeutic interventions by focusing on the patient's high cognitive function and diagnostic imaging studies in the future.

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The revised organ transplantation law went: into effect from July, 2010 in Japan. We discuss problems with the organ procurement system from the view point of donor hospitals, especially for children. Doctors working in donor hospitals have to differentiate their brain death diagnosis between children and adults to exclude child abuse, hypotension, and/or hypothermia. The heavy burden for donor hospitals and organ procurement from a child donor are discussed. Organ donation takes much time and requires many specialists. For example, it takes more than 45 hours from the diagnosis of clinical brain death to the end of organ harvesting. To reduce the heavy burden for donor hospitals, a support system needs to be established for the donor hospital and the donor family. The organ procurement procedure is strictly regulated by the law and legal guidelines; we propose that the new framework is redesigned rapidly within the new law and the guidelines.

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[in Japanese]

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We report the case of a 69-year-old woman with severe brain injury who expressed her wish to donate her kidneys and corneas before she died due to cardiac arrest. She was transferred to our hospital while she was in a deep coma. Her consciousness level was 3, according to the Glasgow Coma Scale, and she had fixed dilated pupils. Her brain computed tomography (CT) scans showed a massive subdural hematoma. Emergency surgical intervention failed to improve her neurological findings. Further aggressive treatment was abandoned and conservative treatment was administered. On the sixth postoperative day, her family requested organ donation according to the patient's will. She did not meet the criteria of brain death, and thus, organ retrieval was planned after cardiac arrest. Coordinators and a surgical team stayed at our hospital preparing for organ transplantation for 2 days, before she died of cardiac arrest. Eleven minutes after her death due to cardiac arrest was confirmed, a surgery to harvest her kidneys was initiated. We were later informed that both of her kidneys had been transplanted, and that they were functioning well. Since the implementation of the revised act on organ transplantation in July 2010, the number of organs retrieved from patients experiencing brain death has been increasing. Organ procurement after death due to cardiac arrest is somewhat different from that after brain death and is a serious issue that needs to be addressed.

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We present an extremely rare case of primary sellar neuroblastoma. A 75-year-old woman presented with progressive visual disturbance and bitemporal hemianopsia. Magnetic resonance imaging (MRI) revealed a huge parasellar enhanced lesion that had enlarged in the course of a year. She underwent surgical resection via the endoscopic transsphenoidal approach followed by conventional radiotherapy (55Gy) and carboplatin-based chemotherapy. The tumor decreased in size in response to adjuvant therapies. We found only 9 earlier cases of primary sellar neuroblastoma in the literature. Preoperative diagnosis is difficult because primary sellar neuroblastomas have neither specific radiographic findings nor symptoms. Standard treatment has not been established to date. Although primary sellar neuroblastoma is extremely rare, it should be considered as a differential diagnosis in patients with huge, rapidly-growing parasellar tumors.

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We reported an atypical PA case in adult. A 27-year-old woman presented with uncontrollable epileptic seizures and fast clinical deterioration. Magnetic resonance imaging (MRI) disclosed T2-hyperintense lesion with a marginal rim located in the left superior temporal gyrus. Resective surgery was performed and histopathological examination revealed a mixture of the atypical features, including biphasic compact and spongy pattern with interspersed eosinophilic granular bodies, as well as ganglioglioma-like and oligodendroglioma-like components. Based on the morphological findings and IDH-1 status the diagnosis of atypical PA was confirmed. Detailed information on the clinical presentation, radiological characteristics, histopathological pattern, and surgical strategy is reported.

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