Focal tectal tumors: management and prognosis.

A 7-year review at our institution identified 12 children with midbrain tectal tumors. All presented with signs of increased intracranial pressure, had hydrocephalus on initial imaging, and were treated with ventriculoperitoneal (VP) shunts. Three had clinical and radiographic progression of disease. Two were treated with radiation and chemotherapy, with progression of disease in one. The third received radiation alone. All patients are alive, with a median follow-up of over 4 years. Median progression-free survival is at least 24 months and median total survival is beyond 50 months. The tectal glioma syndrome is a relatively benign variant of the brainstem glioma. The majority of patients may be managed with a VP shunt alone.

Brain and spinal cord hemorrhage in long-term survivors of malignant pediatric brain tumors: a possible late effect of therapy.

Three children with malignant primary CNS tumors treated with craniospinal radiotherapy developed intraparenchymal hemorrhages a median of 5 years following therapy in sites distant from the primary tumor. Radical surgical procedures disclosed fresh and old hematoma, gliosis, and necrosis in all 3 patients and an aggregation of abnormal microscopic blood vessels in two. No tumor was found. All 3 patients remain in long-term (greater than 10 years) continuous remission.

Cervicomedullary tumors in children: a distinct subset of brainstem gliomas.

We reviewed the clinical course of 17 children who underwent surgical resection of an intra-axial cervicomedullary tumor between 1980 and 1992. The clinical symptoms, which reflected medullary dysfunction in nine children and cervical cord deficits in eight, were present for a mean of 2.1 years before diagnosis (range, 2 months to 7.5 years), and for at least 1 year in 80% of the patients. Neurodiagnostic imaging (MRI in 14, CT in 3) showed the tumor epicenter in the medulla in 11 and in the upper cervical cord in six. Surgery was performed for newly diagnosed tumor in 11 children, and for progressive disease in six who had received prior radiotherapy. The surgical resection was gross total in two and partial (60 to 95%) in fifteen. Fifteen patients had low-grade glial tumors (10 astrocytomas, four gangliogliomas, and one mixed glioma), and two had anaplastic gangliogliomas. Four-year progression-free and total survival rates after surgery for patients who had surgery as initial therapy were 70 and 100%; for those who had surgery at the time of progression, these were 41 and 62%. Postsurgical neurologic complications occurred in five children. Four of these children had received prior radiotherapy. Two of them already had severe preoperative deficits and three had moderate deficits that worsened after surgery. Twelve patients with mild deficits were unchanged or improved postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Intramedullary spinal cord tumors in children: localization by intraoperative sonography.

Intraoperative sonography was performed in 20 children in order to localize intramedullary spinal cord tumors. It provided excellent visualization of the extent and consistency of the tumor before surgical opening of the dura mater. The sonographic findings were used in selecting the site and extent of myelotomy. Intraoperative sonography proved to be a valuable adjunct to spinal cord surgery.

MR of spinal cord ganglioglioma.

PURPOSE: Our purpose was to describe the MR imaging features in a series of spinal intramedullary gangliogliomas and to compare these findings with the MR features of intramedullary astrocytomas and ependymomas. METHODS: A retrospective analysis was performed of 76 MR examinations in 27 patients with histologically proved spinal ganglioglioma; these were then compared with imaging findings in a representative sample of histologically proved spinal cord astrocytomas and ependymomas. RESULTS: Statistically significant observations regarding spinal gangliogliomas included young age of the patients (mean, 12 years), long tumor length, presence of tumoral cyst, presence of bone erosion and scoliosis, absence of edema, presence of mixed signal intensity on T1-weighted images, and presence of patchy enhancement and cord surface enhancement. A trend (not statistically significant) was noted for holocord involvement and lack of magnetic susceptibility. CONCLUSION: Spinal ganglioglioma can be strongly suspected if MR images reflect the above criteria; however, the ultimate diagnosis still depends on radical resection and appropriate histopathologic investigation.

Sonography of the spine and spinal cord.

Sonography has proved to be a valuable method for the examination of the spine in children with spinal dysraphism. It facilitates the diagnosis of meningocele, lipoma, and/or spinal cord tethering rapidly and noninvasively. It is suggested that sonography be utilized for screening infants with neurocutaneous signature of spinal dysraphism.

Pseudobulbar palsy after posterior fossa operation in children.

The authors describe their experience with an unusual postoperative syndrome of pseudobulbar palsy occurring a few days after the extirpation of large midline vermian/4th ventricular tumors in children. The patients had a delayed onset of supranuclear cranial nerve palsies associated with emotional incontinence and lability that resolved over several weeks to months. It is postulated that retraction pressure on the medial cerebellum and split vermis is the operative insult responsible for edema that subsequently tracked along fiber pathways in the middle and superior cerebellar peduncles into the upper pons and midbrain. Modifications of operative technique to eliminate the need for retraction are presented.

Chronic headache associated with a functioning shunt: usefulness of pressure monitoring.

Chronic headaches in a shunt-dependent patient with small ventricles has long been treated with little or no regard to intracranial pressure. In this study, pressure monitoring on 12 such patients demonstrated that they fell into three distinct categories: 3 had headaches caused by intracranial hypertension, 2 had headaches from hypotension, and 7 showed no relation of symptoms to pressure. As therapeutic procedures for treating these three categories are entirely different and sometimes opposing, it is clear that intracranial pressure monitoring is essential to successful management of this complaint.

Complications of ventriculoperitoneal shunt procedures or hydrocephalus associated with vein of Galen malformations in childhood.

Twenty children with vein of Galen malformations and hydrocephalus required a ventriculoperitoneal shunt. Shunt placement was associated with a 70% incidence of complications that included status epilepticus in 3 patients, intraventricular hemorrhage in 7 patients, and subdural hematoma or hygroma in 5 patients. Based upon this experience, the authors recommend the use of preoperative prophylactic anticonvulsants and the placement of medium pressure frontal ventriculoperitoneal shunts. This report will address the overall experience with and the basis on which these therapeutic recommendations were made.

Intramedullary spinal cord tumors in neurofibromatosis.

Neurofibromatosis (NF) describes the most commonly inherited disorders affecting the nervous system. These autosomal dominant, neurocutaneous syndromes are associated with multiple tumors of the nervous system, including neurofibromas, schwannomas, meningiomas, and intracranial gliomas. Spinal cord involvement in NF is typically from extramedullary growth of spinal nerve root tumors. Intramedullary spinal cord tumors in NF have been reported as scattered, single cases in literature. However, this association has not been clearly defined as have other nervous system neoplasms that are typically linked with NF. We present a series of nine patients with NF with intramedullary spinal cord tumors who were managed at our institution from 1984 to 1994. The patients' ages ranged from 4 to 31 years. There were seven male patients and two female patients. Three patients had NF-1 (von Recklinghausen's), five patients had NF-2, and one patient had NF, type uncertain. There were three cervicomedullary tumors, two cervical tumors, three cervicothoracic tumors, and one thoracic tumor. A histological examination revealed five ependymomas, three astrocytomas, and one intramedullary schwannoma. Two patients with malignant intramedullary spinal ocrd tumors have died with progressive disease. One other patient has required a subsequent operation for recurrent tumor. The other six patients are doing well, and their prognosis is expected to be related to their systemic disease. We conclude that there is a clinical entity of intramedullary spinal cord tumors associated with NF.