We outline the standard treatment for pediatric hydrocephalus based on US guidelines published in the Journal of Neurosurgery : Pediatrics in November 2014. The following nine clinical issues are addressed in these guidelines : 1) management of posthemorrhagic hydrocephalus in premature infants, 2) endoscopic computer-assisted electromagnetic navigation and ultrasonography as technical adjuvants for shunt placement, 3) cerebrospinal fluid shunt or endoscopic third ventriculostomy, 4) effect of valve type on cerebrospinal fluid shunt efficacy, 5) preoperative antibiotics for shunt surgery in children with hydrocephalus, 6) antibiotic-impregnated shunt systems versus conventional shunts in children, 7) management of cerebrospinal fluid shunt infection, 8) effect of ventricular catheter entry point and position, and 9) change in ventricle size as a measurement of effective hydrocephalus treatment. A systematic literature review has also been conducted on these issues and reported evidence-based guidelines, and their recommendations are introduced.

  Surgical treatment for juvenile Moyamoya disease (MMD) has been successful in stroke prevention. A previous study demonstrated lower perioperative complications as well as favorable neovascularization when employing combined direct and indirect revascularization compared to the indirect procedure alone.

  Since cognition is a major determinant of QOL, optimal brain development may be equally as important as normalization of the cerebral hemodynamics in juvenile MMD. To perform surgical intervention in a timely manner, establishing an optimal neuroimaging marker is necessary to identify patients with reversible cognitive impairments.

  A novel neuroimaging technique allows tracking of the brain development trajectory as well as ischemic brain injury as a part of routine clinical investigations. Diffusion tensor imaging as well as its extension, kurtosis imaging, can detect covert white matter injuries particularly in the superior longitudinal fasciculus. If structural connectivity is impaired in the dorsolateral prefrontal, this could be responsible for impaired executive function. Advanced neuroimaging techniques enable us to explore chronic ischemic injuries, allowing treatment which can potentially lead to optimal brain development in juvenile moyamoya disease.

  Recent advances in imaging and molecular diagnostic technology have increased the rates of the prenatal diagnosis of neurosurgical central nervous system abnormalities. However, the prognosis of the patients who were diagnosed prenatally are not well known. We report our experience of 62 patients who were available for follow-up study, and 18 aborted cases. The mean diagnosis time of born cases was 29.8 weeks (18 weeks-39 weeks), and aborted cases was 18.2 weeks (13 weeks-24 weeks). The mean age of born cases was 7.3 years (0-25 years), and the born cases included 23 myelomeningocele, 9 isolated ventriculomegaly, and 8 fetal intraventricular hemorrhage. Among aborted cases, there was 7 myelomeningocele, and 6 genetic abnormalities such as hereditary hydrocephalus. 11 cases of the born cases died, and the surviving cases differed in prognosis depending on the disease. We assessed DQ/IQ of the surviving patients older than 2 years old (n=54). 26 survivnig cases were normal (DQ, IQ>80), 8 cases were with mild disability (60-79), 3 cases with moderate disability (40-59), and 3 cases with severe disability (<39). Of prenatally diagnosed cases, the prognosis varied significantly depending on the disease, however nearly half of cases that were able to follow up had normal development. Improved accuracy of the diagnosis was thought to be necessary for prenatal diagnosis.

  Cervical laminoplasty as a posterior decompression procedure instead of laminectomy had been firstly reported in Japan in 1970’s and 1980’s around the world. And its procedure has been developed and devised for a long time in Japan. Cervical laminoplasty has been mainly applied for narrow spinal canal due to cervical degenerative disorders including well-known cervical spondylotic myelopathy (CSM) and ossified posterior longitudinal ligament (OPLL) and so on.

  Various procedures of cervical laminoplasty recently have been consolidated into main two procedures, open-door laminoplasty and double open-door laminoplasty.

  In this paper, I would like to focus on its developed history and consolidated stream using our original illustrations for each cervical laminoplasty.

  Surgical removal of giant (over 40 mm) vestibular schwannomas (VSs) is associated with higher rates of postoperative complications than smaller tumors. Especially, the cystic VSs carry different risks of postoperative complications when compared to solid VSs. The transmastoid approaches, including the retro- and translabyrinthine approaches have several advantages in that these approaches are less likely to cause damage to the cranial nerves, cerebellum, and brainstem during surgery. They provide not only a straight corridor to the cerebellopontine angle (CP angle), but also the shortest access to the internal auditory canal and jugular foramen. However, the operative field is deep and narrow, and the operative view is unfamiliar compared to that in the lateral suboccipital approach (retrosigmoid approach). We have used combined transmastoid approaches that provide multidirectional operative corridors to the CP angle enables the surgeon to safely perform radical resection of giant cystic VSs. These surgical approaches can be divided into several variations. Each variation consists of a transmastoid approach (retro- or translabyrinthine approach) and additional subtemporal or suboccipital craniotomies with or without tentorial incision.

  Thus, transmastoid approaches can be combined with other procedures to enhance the exposure and resectability of giant cystic VSs. These combined transmastoid approaches provide successful resection of the lesion with low morbidity and minimum brain retraction.

  The presentation of intracranial tumors during pregnancy is relatively uncommon. We report a case of cerebellar hemangioblatoma presenting with brainstem compression and obstructive hydrocephalus during the third trimester of pregnancy.

  A 29-year-old woman visited our hospital presenting with headache and nausea. Magnetic resonance imaging revealed a well-circumscribed mass in the left cerebellar hemisphere with diffuse brain edema, brainstem compression and hydrocephalus. The tumor was strongly enhanced by gadolinium. Vertebral angiography demonstrated a tumor stain supplied by the left posterior cerebellar artery and superior cerebellar artery, draining into transverse sinus.

  We delivered the baby by performing cesarean section, and then removed the tumor by suboccipital and occipital transtentorial approach. The tumor was accompanied by a high-flow arteriovenous shunt. After clipping off the feeding arteries, we detached the tumor from the cerebellum and achieved total en bloc resection. Histopathological examination confirmed the diagnosis of hemangioblastoma. The postoperative course was uneventful, and the patient was discharged one month after the operation with her baby.

  Previous reports suggest that the presentation of cerebral tumors during pregnancy is related to the increased circulating blood volume and serum estrogen concentration, leading to an increase in the tumor volume and the aggravation of the brain edema. Since we can expect tumor shrinkage after child delivery due to the normalization of the circulating blood volume and hormonal changes, elective tumor removal after delivering the baby can be a treatment option. However, another reported case shows neurological deterioration after child delivery ; the optimal course of treatment for cerebral tumors during pregnancy is still controversial. We must plan the treatment strategy based on the condition of each patient.