Enlarging pediatric ectopic Rathke's cleft cyst in the prepontine cistern: case report.

Rathke's cleft cyst is a cystic disease that occurs in the sella turcica or, occasionally, in the suprasellar area. An ectopic Rathke's cleft cyst is extremely rare, and its nature is less well understood. The authors report the case of a 14-year-old girl who presented with a growing cystic lesion in the prepontine cistern, immediately behind the dorsum sellae. Preoperative imaging and intraoperative investigation showed part of the cyst wall continuing into the dorsum sellae, to the pituitary gland. The cisternal portion of the cyst wall was totally resected via a right subtemporal approach. Histopathological examination of the cyst wall showed a monolayer of ciliated cells, identical to those of Rathke's cleft cyst. To the best of the authors' knowledge, this represents the first pediatric case of Rathke's cleft cyst occurring in the prepontine cistern.

A case of metastatic brain tumor causing multifocal cerebral embolism.

The patient was a 72-year-old woman who had previously undergone treatment for femoral chondrosarcoma (histologically rated as myxofibrosarcoma). She suddenly developed left homonymous hemianopsia and was diagnosed with cerebral embolism. Because she had atrial fibrillation, we treated her for cardiogenic cerebral embolism. About 3 months later, however, she developed left hemiplegia, and head magnetic resonance imaging revealed multiple tumorous lesions affecting the previously detected infracted area and several new areas. We assumed that a tumor embolus had caused cerebral embolism, which resulted in growth of the tumor from the embolus and formation of a metastatic brain tumor. The metastatic foci formed from the tumor embolus were visualized by diagnostic imaging, and histological examination of the resected tumor confirmed that the brain tumor had occluded the brain vessel (tumorigenic cerebral embolism). No such case has been reported to date, and this case seems to be important.

Management of intraventricular hemorrhage in preterm infants with low birth weight.

The management of posthemorrhagic hydrocephalus is difficult and not well standardized. We evaluated our management protocol for infants with intraventricular and/or periventricular hemorrhage (IVH and PVH, respectively). There were four deaths and two significant treatment-related complications in our series. We also observed two cases of isolated ventricle in patients treated with reservoir placement. After evaluating our series, we modified our protocol from reservoir placement to either cerebrospinal fluid (CSF) drainage or ventriculosubgaleal shunt directly. We will reevaluate this new protocol in the near future.

Spontaneous regression of a spinal extradural arteriovenous fistula after delivery by cesarean section.

A 23-year-old Japanese woman presented with a newly developed spinal extradural arteriovenous fistula (AVF) during pregnancy. She had been followed up for a suspected spinal cavernous angioma and became unable to walk during the 29th week of her pregnancy. Magnetic resonance (MR) imaging showed a spinal extradural AVF at the T3 to T4 levels compressing the spinal cord. After delivery by cesarean section, her neurological symptoms gradually began to resolve, and she was able to resume walking without assistance. MR imaging confirmed spontaneous regression of the AVF. This case suggests that exacerbated neurological symptoms and AVF growth triggered by pregnancy can improve after delivery without interventional treatment. Careful follow up of neurological findings is required to prevent unnecessary interventional procedures in pregnant women with spinal AVF.

Embryopathogenetic surgicoanatomical classification of dysraphism and surgical outcome of spinal lipoma: a nationwide multicenter cooperative study in Japan.

OBJECT: The natural history of asymptomatic spinal lipoma in infancy remains unclear, and the indication for the prophylactic untethering operation is still debatable. To address this question, a multicenter cooperative study for the treatment of spinal lipoma was performed by the 7 most active institutions in neurosurgical care for spina bifida in Japan between 2001 and 2005. METHODS: Patients were classified using the embryopathogenetic surgicoanatomical classification. Their neurosurgical postoperative course was analyzed using the Spina Bifida Neurological Scale. Among 261 patients, 159 were asymptomatic and 102 were symptomatic. RESULTS: Of the 136 patients for whom prophylactic surgeries were performed, 135 remained asymptomatic and only 1 (0.4%) of the 261 patients presented with mild sensory disturbance. Mild foot deformity was identified in 1 (4.3%) of 23 conservatively observed patients. Of 100 symptomatic patients, deterioration after surgery was seen in 6%, and improvement in 44%. Complete resolution of symptoms was seen in only 14.2%. Filar types for patients > 3 years old improved in Spina Bifida Neurological Scale scores from 12.3 to 14.0. The mean age of symptomatic patients with lipomyelomeningocele was the youngest of all (1.3 years), which indicates lipomyelomeningocele may deteriorate in early infancy. Improvements from surgery were seen for all types of lipoma except the caudal type, presenting at an older mean age (15 years). CONCLUSIONS: A low rate of postsurgical worsening indicates that surgeries for asymptomatic and symptomatic lipomas are safe. Surgeries done after the onset of symptoms seldom cure the patients. These two results support early untethering for any kind of lipoma; however, further study of the natural history is required.

An immunohistochemical and electron microscopic study of atypical teratoid/rhabdoid tumor.

We report two infant cases with atypical teratoid/rhabdoid tumor (AT/RT) located in the cerebellar vermis and spinal cord. MRI showed the tumors were isointense on T1-weighted images and mixed intensity of isointense and slight high intensity on T2-weighted images. Postcontrast MRI demonstrated clear margin of tumor and heterogeneous strong enhancement. It was difficult to differentiate the tumor from medulloblastoma by hematoxylin and eosin staining. However, immunohistochemical staining showed that these tumor cells react positively for cytokeratin, smooth muscle actin (SMA), and epithelial membrane antigen (EMA) and helped us with the differentiation. Electron microscopic study has confirmed the presence of mesenchymal components, such as filaments and desmosome junctions in the rhabdoid cells, but no neuronal components. The tumors rapidly increased in size, showing high MIB-1 index, and the prognosis was gave.

Traumatic subarachnoid hemorrhage associated with acupuncture.

Subarachnoid hemorrhage as a complication of acupuncture has been reported in only a few cases. We report another case and discuss the implications of subarachnoid hemorrhage following acupuncture. Although acupuncture has traditionally been thought to be relatively safe, physicians should be aware that it may be a cause of intracranial hemorrhage.

The intracranial pressure of the patients with mild form of craniosynostosis.

INTRODUCTION: While raised intracranial pressure (ICP) is a well recognized complication affecting children with syndromic craniosynostosis, certain percentage of the patients with non-syndromic craniosynostosis may have some problems related to increased ICP, such as developmental delay or visual problem. However, it is still not clear how many percent of and/or which types of craniosynostosis patients suffer from those symptoms, especially in older patients. OBJECTIVE: The aim of this study was to examine the ICP of older children with mild form of craniosynostosis to determine if any of them should be surgically treated. MATERIALS AND METHODS: We measured ICP before making the decision for surgical intervention. RESULTS: Twenty-five of thirty-six patients had raised ICP in our series. DISCUSSION: All of the 25 patients were treated surgically and followed-up after more than 1 year. All patients improved in some degree. Further investigations should be performed to determine what is the threshold for raised ICP in children.

A case of petrous bone myxoid chondrosarcoma associated with cerebellar hemorrhage.

We report a rare case in which a myxoid chondrosarcoma originated from the petrous bone and invaded the cerebellar hemisphere with hemorrhage. Neuroimaging showed the characteristic feature of multiple small cysts along the solid tumor, and the cystic formation was confirmed as a mucoid secretion by Alcian blue staining and electron microscopic examination. This tumor recurred following partial removal and stereotaxic radiosurgery.

Solitary myofibromatosis of the skull: a case report and review of literature.

CASE REPORT: We present a case of solitary myofibromatosis of the skull in a 4-year-old girl. Surgery was performed and the final diagnosis of myofibromatosis was made histopathologically. DISCUSSION: Solitary myofibromatosis of the skull is rare and we found approximately 20 reported cases in the English-language literature. We reviewed eight well-described cases. CONCLUSION: Neuroradiologically, common features such as a lytic lesion with a sclerotic rim on roentgenogram and intra-diploic lesion with periosteal new bone formation both in the outer and inner table of the skull on computed assisted tomography are noticed.

Expression of Rb2/p130 protein correlates with the degree of malignancy in gliomas.

It has been reported that there is an inverse correlation between the immunohistochemical expression of Rb2/p130, a member of the retinoblastoma gene family, and the degree of malignancy in at least some histological types. In order to investigate the expression of this protein in gliomas, we evaluated 58 samples from patients with resected gliomas. We focused on the relationship between the degree of malignancy of the glioma and the immunohistochemical detection of Rb2/p130. Expression of Rb2/p130 was observed in 38 glioma specimens (65.5%), including a high expression level in low-grade glioma specimens (> 30% positive cells in 84% of tumors) and a low expression level in high-grade glioma specimens (> 30% positive cells in 12% of tumors). The most aggressive of the gliomas exhibited very low to undetectable levels of Rb2/p130. Moreover, we observed an inverse correlation between Rb2/p130 expression and the degree of malignancy. These findings suggest that the differentiation of gliomas might be partially mediated by the Rb2/p130 gene, and that Rb2/ p130 expression can additionally be an indicator of a better prognosis in patients with gliomas.

A case of primary Ewing's sarcoma of the occipital bone presenting with obstructive hydrocephalus.

BACKGROUND: Ewing's sarcomas account for approximately 10% of primary malignant bone tumors. While most of the primary Ewing's sarcomas occur in the long bones, pelvis, or ribs, approximately 1-6% of these sarcomas arise in the skull. We found approximately 50 cases of Ewing's sarcoma of the calvarium reported in our search of the literature. Of these, 36 cases were reported in detail. CASE REPORT AND DISCUSSION: In this article we describe a rare case of primary Ewing's sarcoma of the occipital bone presenting with acute obstructive hydrocephalus and review the literature.