Search

Resting-state functional MRI (rs-fMRI) identifies resting-state networks (RSN) in the human brain by analyzing the connectivity of anatomically remote neuronal populations with synchronous low-frequency fluctuation in blood oxygen level-dependent (BOLD) signal. Network analysis has informed the understanding of functional brain organization and is beginning to reveal the impact that neurological disorders such as epilepsy may have on the developing cerebral cortex. Among children undergoing epilepsy surgery, mapping the brain networks supporting language, sensorimotor and visual function is a critical part of the preoperative evaluation. However, task-based functional mapping techniques are particularly difficult in immature patients and those with severe impairment. Functional mapping of RSN is a promising tool that may help circumvent the challenges of adequate cooperation and limited abilities of developmentally disabled children to perform age-appropriate functions. We discuss the current methodology of rs-fMRI in the pediatric population, review the literature of rs-fMRI in pediatric epilepsy and present our experience of using rs-fMRI for functional network mapping in children undergoing epilepsy surgery.

In the pediatric population, a strong correlation between multilevel laminectomy and postlaminectomy spinal deformities, predominantly kyphosis, has been demonstrated. This has been observed mainly in the cervical and thoracic spine. Therefore, laminoplasty was proposed as an alternative to laminectomy with a reduced incidence of spinal deformity and need for stabilization and fusion. Moreover, to the best of our knowledge, postlaminoplasty kyphosis of the thoracic spine has not been previously described in the literature. We report the case of a pediatric patient who rapidly developed severe thoracic kyphosis 3 months after resection of a symptomatic extradural spinal arachnoid cyst and multilevel en bloc thoracic laminoplasty and review the relevant literature.

Little to no pediatric or neurosurgical literature has been published about the complications of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood. The interventional neuroradiology literature, however, suggests that ventriculoperitoneal shunting as first-line treatment for hydrocephalus in children with vein of Galen malformations is fraught with short- and long-term dangers, including status epilepticus, intraventricular hemorrhage, subdural hematoma and hygroma, venous infarction, malignant dystrophic calcification, and worsening developmental delay. We present a single pediatric case where a ventriculoperitoneal shunt procedure for symptomatic hydrocephalus seemed to be the major contributing factor to the rapid neurological deterioration and eventual death of an infant with a vein of Galen malformation. Based on this experience and our review of the literature, we suggest the use of endovascular embolization of the vein of Galen malformation to reestablish a balance in hydrovenous dynamics as first-line treatment rather than directly addressing hydrocephalus with CSF diversion. The ventriculoperitoneal shunt procedure should be reserved for cases with symptomatic hydrocephalus in which the patient is a poor candidate for embolization, or for cases where endovascular therapy has already been maximized. The role of endoscopy in the treatment of hydrocephalus associated with vein of Galen malformations is not clear.

Malignant rhabdoid tumors of the spine are rare pediatric neoplasms that have a poor prognosis. The histological, ultrastructural, and immunohistochemical features are essential elements used in their diagnosis. We report the case of a malignant rhabdoid tumor of the cervical spine in a 13-month-old infant. Tumor cells were vimentin positive with prominent nucleoli indented by eosinophilic cytoplasmic inclusions containing intermediate filaments. INI1 immunostaining was negative. This patient was operated on twice for symptomatic spinal cord compression. Despite chemotherapy, she developed worsening leptomeningeal dissemination, lower cranial nerve dysfunction, and hydrocephalus that did not respond to CSF diversion. She died 4 months after initial diagnosis. We review the literature on spinal malignant rhabdoid tumor and discuss the nomenclature, pathology, radiology, treatment, and outcomes of this rare entity.

Optimal treatment for hydrocephalus related to Dandy-Walker syndrome (DWS) remains elusive. Patients with DWS-related hydrocephalus often require combinations of shunting systems to effectively drain both the supratentorial ventricles and posterior fossa cyst. We describe an endoscopic technique, whereby a frontally placed, single-catheter shunting system effectively drained the supratentorial and infratentorial compartments. This reduces the complexity and potential risk associated with the combined shunting systems required by so many with DWS-related hydrocephalus.

Introduction: Elevation and repair of an open depressed skull fracture is often thought of as an emergency procedure. Common indications for emergent elevation of a depressed skull fracture have been dural tear, seizure, gross contamination or mass effect from bone or a sizable underlying intracerebral hematoma. As treatment of head injury moves towards management of cerebral perfusion pressure (CPP) rather than intracranial pressure (ICP), we sought a way to maximize CPP in the initial treatment of head-injured patients with depressed skull fractures that would eventually require surgery by delaying surgery, when possible, until after the initial period of elevated ICP. Methods: Over a 12-month period, 7 patients (all male, ages 1–15 years) were admitted to our institution with the diagnosis of open depressed skull fracture without significant mass effect requiring urgent decompression. All had significant head trauma with altered mental status and a Glasgow Coma Score of 3–12. Patients were treated with antibiotic prophylaxis (nafcillin, ceftriaxone, metronidazole), seizure prophylaxis (phenytoin) and underwent CPP management in an intensive care unit setting as indicated by intracranial pressure monitoring or clinical assessment. Length of medical management of CPP ranged from 4 to 12 days. Upon stabilization of CPP, patients were operated for repair of their dural, bone and scalp injuries. Results: All 7 patients treated in the above manner suffered no ill effects from their delayed surgery: there was no meningitis, no late seizures, and no cerebrospinal fluid leak. Complications attributable to delay were not present at follow-up ranging from 12 to 24 months. Conclusions: We have delayed surgery for repair of open depressed skull fractures in order to maximize medical management of CPP in the setting of acute trauma. Among other considerations, the risk of intraoperative hypotension occurring at a time of acutely raised ICP was avoided by this delay. We conclude that there is a role, in this specifically defined subset of head trauma patients, for delayed surgical repair of open depressed skull fractures.