Objective: To evaluate the method of prenatally estimating an appropriate clinical outcome in fetal hydrocephalus. Method: Retrospective study, single institute (Osaka National Hospital). Materials: Hundred and seventeen cases with fetal hydrocephalus treated at Osaka National Hospital from 1992 to 2010 were analysed. Result: Of the 117 cases analysed, 38% are diagnosed as isolated ventriculomegaly (IVM), 51% as other types of malformation (30 cases of myelomeningocele, 4 cases of holoprosencephaly, 4 of Dandy Walker syndrome, 10 of arachnoid cyst and 6 of encephalocele etc.) and 11% as secondary hydrocephalus. They are diagnosed between 17 and 40 weeks of gestation (average 27 weeks), 17% diagnosed between 17 and 21 weeks, 30% between 22 and 27 weeks and 53% after 28 weeks. With the exception of 9 aborted cases and 30 unknown cases too young to be evaluated or lost due to lack of follow-up, final outcome was analyzed in 78 cases. Of these 78 cases, 15% died in utero or after birth, 23% showed severe retardation, 17% moderate retardation, 26% mild retardation, and 19% showed good outcome. Long term consequences were mostly influenced by basic disease and accompanied anomalies. Hydrocephalus associated with arachnoid cyst, atresia of Monro, corpus callosum agenesis and hydrocephalus due to fetal intracranial hemorrhage are categorized in the good outcome group. On the other hand, holoprosencephaly, hydrocephalus associated with encephalocele, syndromic hydrocephalus and hydrocephalus due to fetal virus infection are categorized in the poor outcome group. Conclusion: In order to accurate diagnosis and proper counseling, establishment of diagnosis protocol and treatment policy for fetal hydrocephalus including not only fetal sonography, fetal MRI, TORCH screening test but also chromosomal and gene testing is required.

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Clinical presentation, operative procedures, and surgical results for 101 patients with craniosynostosis (CS) were reported. Patients included 39 single-suture CS, 33 multiple-suture CS, and 29 syndromic CS. Principally early surgery was conducted at the average age of 596 days (median 273 days) with the purpose of decompression for the developing brain and amelioration of craniofacial deformity. Four patients died in the perioperative periods and 7 deceased during the follow-up period. During the average 88-month-follow-up period, 43 patients received secondary surgery and 4 received tertiary surgery. Primary procedure alone was performed on 56 (57%) patients considered to have 'good' result. At the school age, 45 out of 69 patients entered ordinary class and the intelligence quotient of 34 patients ranged from 60 to 126 and averaged at 96.9±18.3. From the author's experience, engaging in the treatment of patients with CS, the higher brain functions including psychosocial development and cognitive function as well as the amelioration of craniofacial deformity should be focused on.

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Neurosurgical long-term management of myelomeningocele (MMC) involves hydrocephalus, Chiari malformation type 2 (CM2), and tethered spinal cord. The rate of hydrocepalus requiring a VP shunt is reported to be declining to about 60% in some series. Shunt insufficiency can develop in more than 90% of those with VP shunts and the life-long management of VP shunt is a mandate. CM2, once regarded as life-threatening when it becomes symptomatic in infancy, also causes chronic respiratory problem for 20-60% of adult patients with MMC. Tethered cord syndrome (TCS) after MMC repair requiring surgical release develops in about 20-30% of patient. TCS often becomes symptomatic from school age to adolescence and careful observation is necessary for those children with MMC. Currently, MMC is no longer a seriously disabling disease as more than half (60-70%) of all patients are expected to return to social activity with some limitation. Finally late deterioration of neurological function as patients survive longer should be considered during the long-term follow-up for MMC patients.

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The treatment for pediatric craniopharyngioma remains challenging and controversial. Although this tumor is histologically benign, the treatments include radical surgery, conservative surgery, radiotherapy, intracystic chemotherapy and multimodality approaches. In addition, the long-term functional outcomes including visual function, endocrine function, cognitive function, hypothalamic function, and quality of life are complex and major problems among survivors. In this paper, we reviewed the recent treatments for pediatric craniopharyngioma and the long-term out-comes after treatment in literatures.

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Though medulloblastoma is the most common malignant brain tumor during childhood, only 80 newly-diagnosed tumors develos every year, as the annual incidence is extremely rare with an occurrence of 0.5 per 100,000 children younger than 15-year-old and of 0.7 per 100,000 for the entire population. Images obtained of medulloblastoma are characterized by a round heterogeously-enhanced mass in or adjacent to the VIth ventricle. Objectives of surgical treatment are the maximum resectioning of the main mass and the relief of the obstructive hydrocephalus. Cerebellar mutism occurs a few days after one fourth of medulloblastoma surgery, and lasts approximately for 50 days followed by subsequent dysarthria. Pathological subtypes include classic medulloblastoma, desmoplastic/nodular medulloblastoma, medulloblastoma with extensive nodularity, large cell/anaplastic medulloblastoma, all corresponding to WHO grade IV. According to age, residual tumor size, and disseminated staging, patients are divided into average-risk group, high-risk group, or baby-medulloblastoma after surgery. Standard treatment in average-risk group includes 23.4-Gy cranio-spinal irradiation (CSI) with posterior boost followed by chemotherapy consisting of CDDP, alkylating agents, and vincristine. Patients in high-risk group receive over 36-Gy CSI with boost radiotherapy to nodular lesions before, concomitantly with, or followed by dose-intensity chemotherapy. In cases with gross total removal, or desmoplastic/nodular pathology, radiotherapy for patients younger than 3-year-old are often delayed until they turn 3-year-old, and are able to survive for long time by appropriate chemotherapy alone. Adolescent survivors with childhood medulloblastoma have a number of late adverse effects regarding another neoplasm, neuro-cognitive function, endocrine activity, cardiovascular organs, and skeletal system. Comprehensive follow-up and support system are mandatory.

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Object: This study evaluated the long-term outcome of 108 consecutive patients to establish an optimal treatment strategy for respective subgroups of the newly diagnosed intracranial germ cell tumors (GCTs). Methods: A retrospective review of medical records from the authors' department for the duration of April 1989 through March 2007 identified 108 patients with newly diagnosed intracranial GCTs. The diagnosis was germinoma in 83 patients, and nongerminomatous GCT (NGGCT) in 25 patients. Long-term quality of life (QOL) was also evaluated in patients with germinoma. Results: Reading patients with germinoma, the 10-year overall and progression-free survival (PFS) rates at a median follow-up period of 99 months were 86 and 74%, respectively. Patients treated with chemotherapy only demonstrated a decline PFS rate, and patients treated with chemotherapy followed by reduced-dose radiation therapy to the whole ventricle, whole brain, or craniospinal axis indicated significantly improved PFS than patients treated with only radiation or reduced-dose radiation therapy to the focal fields. In the QOL study, the academic outcome for germinoma patients appeared to be better than anticipated. However, neurocognitive defunctionalization was observed at the 10-year follow-up for patients treated with whole brain irradiation. Nongerminomatous GCT patients were categorized into good, intermediate, and poor prognosis groups as proposed by the Japanese Pediatric Brain Tumor Study Group. In the good and intermediate prognosis groups, the 10-year overall survived and PFS rates were 100 and 93%, respectively. In the poor prognosis group, the 3-year overall survived and PFS rates were 56 and 29%, respectively. All patients with NGGCTs, in whom the lesions on MR imaging disappeared after combination therapies consisting of resection, radiation therapy, and chemotherapy, remained alive. Conclusions: Chemotherapy followed by reduced-dose radiation therapy covering the whole ventricle improves the prognosis in patients with germinoma. Combination of radiation therapy, chemotherapy, and radical resection as initial or salvage treatment achieved excellent tumor control in the intermediate prognosis NGGCT group. The outcomes were still dismal for the poor prognosis NGGCT group, so initial therapy should target complete disappearance of all lesions on MR imaging.

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Spontaneous dissecting aneurysms are recognized to be uncommon We assessed the clinical manifestation of dissecting cerebral aneurysms in the posterior fossa, and especially investigated the difference between the precedent headache and nuchal pain associated with subarachnoid hemorrhage (SAH) and that associated with infarction. Medical records and neuroimaging scans of 57 consecutive patients over 8 years with dissecting aneurysms in the posterior circulation were investigated. The incidence of sideration as an initial symptom (symptom that necessitated the visit to our hospital) and the incidence and characteristics of headache and nuchal pain were reviewed. Among 57 consecutive patients, twelve patients (21%) presented with SAH, 19 (33%) with infarction, and 23 patients (40%) presented with only headache and nuchal pain. In comparison with the values reported in the literature, the number of mildly symptomatic patients diagnosed with dissecting aneurysms without SAH or infarction has increased. This increase can be attributed to an increased awareness about dissecting aneurysms and the improvements in diagnostic technology. Precedent headache and nuchal pain occurred in 65% patients with SAH or infarction. Six of the 12 patients (50%) with SAH experienced precedent headache and nuchal pain, and 5 patients (83%) had sudden-onset pain. Fourteen of the 19 patients (74%) with infarction experienced precedent headache and nuchal pain, whereas 4 patients (29%) had sudden-onset pain that gradually progressed to headache and nuchal pain in most cases. Patients who had only headache and nuchal pain were younger than those with SAH or infarct. Further, patients with precedent headache and nuchal pain were younger than those without precedent headache and nuhcal pain. I assumed that the difference in the incidence of precedent headache and nuchal pain in patients with SAH and infarct can be accounted for by 2 factors: the localization pattern of the substance P fiber in the intracranial arterial wall and the direction of the growing pseudolumen. A number of patients were undiagnosed when they visited the clinic with precedent headache and nuchal pain only, and these patients showed clinical exacerbation later. Persistent unilateral headache and nuchal pain without sudden-onset is possibly caused by a dissecting aneurysm. Early diagnosis of dissecting aneurysms in patients presenting with precedent headache and nuchal pain is important for preventing adverse effects such as SAH or infarct.

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Surgery for tumors located at the cranio-cervical junction is challenging. Chordomas are one of the tumors for which treatment is difficult due to its deep location and high rate of recurrence. As the survival rate is significantly higher after total tomor resection than after subtotal or partial resection, the surgical approach should be selected carefully based on the tumor location and extension. We encountered two patients with cranio-cervical junction chordomas. In the first patient, the tumor was located at the mid and lower clivus, which was partially destroyed. Surgery using a Le Fort I maxillotomy was performed, and the tumor was almost totally removed. In the other patient, we performed surgery for the tumor that extended from the lower clivus to the C_2 level via the extreme lateral transcondylar approach with transposition of the vertebral artery followed by C_0-C_4 fixation, which resulted in near total removal. For the purpose of maximum removal of cranio-cervical junction chordomas, we need to carefully select the best surgical approach.

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A 56-year-old woman with an unruptured aneurysm located at the bifurcation of the left middle cerebral artery underwent craniotomy. The aneurysm was torn during dissecting the left anterior temporal artery (ATA) which was adhering to the dome of the aneurysm. Thus, we cut the left ATA just proximal and distal to the adhering portion and anastomosed the artery in an end-to-end fashion. Subsequently, the aneurysm was clipped. This procedure may be useful when dissecting an artery adhering to an aneurysm is difficult.

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