Successful Surgical Management of Traumatic Intracranial Hemorrhaging After Revascularization Surgery for Moyamoya Vasculopathy: A Case Report and Review of Literature.

BACKGROUND: Traumatic intracranial hemorrhaging associated with revascularization surgery for moyamoya vasculopathy is a potentially devastating problem that requires meticulous management, including surgery. However, only a few studies on this subject have been reported, and the clinical characteristics are poorly understood. We report a case of successful surgical management for a patient with traumatic intracranial hematoma managed with encephalo-duro-arterio-myo-synangiosis (EDAMS). The purpose of this article is to clarify the specific features of clinical scenarios, hemorrhagic sites, and operative techniques by reviewing all published cases. CASE DESCRIPTION: A 10-year-old Japanese girl with a history of EDAMS for quasi-moyamoya disease was referred to our institution after minor head trauma. Cranial computed tomography scans revealed a right intracranial hematoma overlying the temporal muscle flap. After admission, hematoma developed, and emergency hematoma evacuation was performed. Venous hemorrhaging from the fascia of the temporal muscle flap was confirmed. Collaterals from indirect bypass were preserved in the surgery. Postoperative diffusion-weighted imaging revealed no ischemic complications. She immediately recovered and returned to her preinjury baseline. CONCLUSION: In moyamoya vasculopathy, intrinsic collaterals or de novo anastomoses from revascularization surgery are easily injured, even with mild head trauma. Furthermore, the administration of antiplatelets agents increases the risk of hematoma development. Sacrifice of collaterals can lead to acute cerebral infarction. During emergency surgery for traumatic intracranial hematoma, a careful surgical strategy is needed to preserve the collateral supply.

Infected multilocular hydrocephalus treated by rigid and flexible endoscopes with electromagnetic-guided neuronavigation: a case report.

BACKGROUND: Endoscopic surgery assisted by a navigation system has greatly aided treatment of infected multilocular hydrocephalus, especially in children. CASE REPORT: We describe a 2-year-old boy with multilocular hydrocephalus caused by repeated shunt infection, presenting with fever and vomiting. Magnetic resonance images (MRI) showed extraventricular cysts and severe ventricular deformity. There were three ventriculoperitoneal shunts and one residual ventricular catheter. With a flexible endoscope, we fenestrated the wall of extraventricular cysts and removed the residual catheter. We then used a rigid endoscope to fenestrate ventricular components. Both procedures were guided by electromagnetic (EM) navigation, and hydrocephalus was controlled with one ventricular catheter. CONCLUSION: We have successfully treated a case suffered from infected multilocular hydrocephalus in infants using rigid and flexible endoscopes combined with EM navigation.

Thoracic meningocele in lumbo-costo-vertebral syndrome in a child: possible enlargement with repeated motion by anchoring to the diaphragm.

Lumbo-costo-vertebral syndrome (LCVS) is a rare disorder in children that is characterized by hemivertebrae, congenital absence of ribs, meningocele, and hypoplasia of the truncal and abdominal wall presenting as a congenital lumbar hernia. An otherwise healthy 12-month-old girl was referred to the authors' hospital with soft swelling on her left middle back; scoliosis had been present since birth. Imaging revealed a thoracic meningocele, ectopia of the spleen suggesting lumbar hernia, multiple anomalies of the thoracic vertebral columns, and defects of the ribs; thus, LCVS was diagnosed. Surgical observation revealed that the meningocele was firmly anchored to part of the diaphragm, which created stretching tension in the meningocele continuously with exhalation. Once detached, the meningocele shrank spontaneously and never developed again after cauterization. In this case, continuous or pulsatile pressure in the presence of a vertebral defect was thus considered to be an important factor for formation of the thoracic meningocele.

Germinoma in the bilateral basal ganglia presented with cognitive deterioration.

PURPOSE: Here, we report a case of pediatric germinoma located in the bilateral basal ganglia, which presented with severe cognitive deteriorations. CASE REPORT: A 15-year-old boy presented with decreased school performance and mild cognitive disturbances. Magnetic resonance images (MRI) of the brain revealed T2 hyperintensity in the bilateral basal ganglia. The patient was initially observed by a local hospital and had screening for metabolic diseases or inflammatory diseases. Lesions with similar characteristics were also found in the pituitary stalk and infundibulum, and these lesions were enhanced with gadolinium (Gad). MR spectroscopy suggested that these should be neoplastic lesions other than metabolic or inflammatory diseases. Biopsy was performed with ventriculoscope, which proved all lesions of infundibulum, pineal, and basal ganglia were pathologically germinoma. The lesions responded well to the chemotherapy and radiation, and his cognitive function improved significantly. CONCLUSION: A case of germinoma in the bilateral basal ganglia which significantly affect cognitive functions is reported. Differential diagnoses of cognitive symptoms are various, but germinoma could be considered as a possible pathology for it. Early MRI and tumor marker exams are recommended, unless organic brain diseases are completely denied. MR spectroscopy and biopsy with ventriculoscope are useful for diagnosis.

Guideline for management and treatment of fetal and congenital hydrocephalus: Center Of Excellence-Fetal and Congenital Hydrocephalus Top 10 Japan Guideline 2011.

INTRODUCTION: Hydrocephalus does not indicate a single clinical entity, but includes a variety of clinicopathological conditions caused by excessive cerebrospinal fluid (CSF) based on the disturbed circulation. Recent progress in prenatal neuroimagings such as MRI and ultrasound echoencephalography on fetus enables to understand clinicopathological conditions of CSF circulation disorder in conjunction with morphological changes in the central nervous system properly. It has been revealed that the CSF dynamics develop in the theory of evolution from the immature brain, as in the animals with the minor CSF pathway predominance, towards matured adult human brain together with the completion of the major CSF pathway: the "Evolution Theory in CSF Dynamics". Now, we can analyze CSF circulation dynamically and also analyze the flow velocity and direction of CSF movement. CENTER OF EXCELLENCE-FETAL HYDROCEPHALUS TOP 10 JAPAN: Along with this technical improvement, the standards of clinicopathological evaluation of hydrocephalus as well as the classification and concept of hydrocephalus shall undergo a major upgrade. Based on such remarkable improvement in the recent practical diagnostic evaluation of fetal hydrocephalus, it is now required to update the guideline for management and treatment of fetal and congenital hydrocephalus, and a nationwide study group; Center of Excellence-Fetal Hydrocephalus Top 10 Japan, was organized in 2008 in Japan. The retrospective analysis of 333 cases of congenital hydrocephalus indicated a fact that 43% of these cases were diagnosed prenatally, and the majority of cases were treated in these top 10 institutes in Japan. Now, congenital hydrocephalus diagnosed immediately after birth is regarded as to be based on embryonic stage; brain disorder in patients with congenital hydrocephalus should be considered in conjunction with neuronal mature process of embryonic stage. The fact is supported by the current trends in hydrocephalus research represented by "Perspective Classification of Congenital Hydrocephalus" and "Multi-categorical Hydrocephalus Classification". The ultimate goal of hydrocephalus treatment remains achieving arrested hydrocephalus by shunt surgeries. In the future, to achieve arrested hydrocephalus, minimum quantity of CSF to be drained should be elucidated. Consideration for accurate operative indication of ETV along with new neuroendoscopic device development and analysis of CSF circulation is expected in the future. The data in this prospective multicenter analysis in this guideline are credited in Oxford Evidence level 2b (Grade II).

[The evaluation of preoperative and postoperative frontal lobe functions in three operative cases of meningioma].

We report three cases of frontal meningioma with their pre- and post-operative evaluations of higher brain functions, especially of frontal lobe functions. All of the cases showed the improvement of the frontal lobe functions after the tumor removal. The evaluations of frontal lobe functions in benign brain tumors such as a meningioma are reported only in a few cases. The evaluations of frontal lobe functions in the operative cases of benign brain tumors provide many interesting and valuable informations about frontal lobe functions. So we must be more interest in evaluations in higher brain functions and accumulate cases for the further analysis of higher brain functions.

Parry-Romberg syndrome with a giant internal carotid artery aneurysm.

BACKGROUND: Parry-Romberg syndrome or progressive facial hemiatrophy is a rare clinical entity of an unknown etiology. We present the case of a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. CASE DESCRIPTION: The case was a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. Its association was extremely rare. Our report and some other reports of Parry-Romberg syndrome with cerebral aneurysms suggest the hypothesis that Parry-Romberg syndrome could be related to some inflammatory causes such as an autoimmune disease. CONCLUSION: We present the case of a 56-year-old woman with Parry-Romberg syndrome and a giant internal carotid artery aneurysm. Its association is extremely rare and will support the hypothesis that Parry-Romberg syndrome could be related to some inflammatory causes such as an autoimmune disease.

[A case report of the chondroma arising from the falx cerebri].

We have reported a case of chondroma arising from the falx cerebri in a 60-year-old female with no neurological symptom. The tumor was revealed as a round mass, attached to the falx cerebri, with marginally scattered calcification on CT scan and with slight enhancement on MRI images. And cerebral angiogram presented a mass with no tumor stain on the external carotid angiography against our preoperative diagnosis of falx meningioma. A subtotal removal of the mass was performed with the diagnosis of chondroma on pathological examination. The chondroma arising from the falx cerebri is extremely rare with only 15 reported cases in the literature. We have presented the first case in Japan and reviewed the previous reports.