✓ In 15% of patients with spontaneous subarachnoid hemorrhage (SAH), the source of bleeding cannot be determined despite repeated cerebral angiography. However, some patients diagnosed as having “SAH of unknown cause” actually harbor undetected aneurysms. The authors report six patients with SAH who, despite multiple negative cerebral angiograms, underwent exploratory surgery due to a high clinical and radiographic suspicion for the presence of an aneurysm. Brain computerized tomography (CT) scans revealed blood located mainly in the basal frontal interhemispheric fissure in four patients, in the sylvian fissure in one patient, and in the interpeduncular cistern in one patient. The patients were evaluated as Hunt and Hess Grades I to III, and had undergone at least two high-quality cerebral angiograms that did not reveal an aneurysm. Vasospasm was visualized in two patients. Three patients rebled while in the hospital. Exploratory surgery was performed at an average of 12 days post-SAH. Five aneurysms were discovered at surgery and were successfully clipped. All four patients with interhemispheric blood were found to have an anterior communicating artery (ACoA) aneurysm. The patient with blood in the sylvian fissure was found to have a middle cerebral artery aneurysm. These aneurysms were partially thrombosed. No aneurysm was detected in the patient with interpeduncular SAH, despite extensive basilar artery exploration. Five patients had an excellent outcome and one patient developed diabetes insipidus. These results show that exploratory aneurysm surgery is warranted, despite repeated negative cerebral angiograms, if the patient manifests the classical signs of SAH with CT scans localizing blood to a specific cerebral blood vessel (particularly the ACoA) and if a second SAH is documented at the same site.
Surgery for angiographically occult cerebral aneurysms
Jafar J. Jafar and Howard L. Weiner
A novel computer-assisted volumetric stereotactic approach for resecting tumors of the posterior parahippocampal gyrus
Howard L. Weiner and Patrick J. Kelly
✓ The authors report their experience using a novel surgical approach for resecting tumors located in the posterior parahippocampal gyrus. Prior attempts to resect epileptogenic foci in this location have been limited by a significant risk of injury to lateral temporal lobe cortical and vascular structures. To avoid these potential complications, the authors have used a lateral occipitosubtemporal, computer-assisted stereotactic volumetric approach to resect radiographically defined tumors in seven patients with intraaxial neoplasms of the posteromedial temporal lobe. This series included one female and six male patients, ranging in age from 15 to 67 years, who presented with seizures, visual field loss, or headache. Gross-total resection of three high-grade gliomas, two gangliogliomas, and one mixed glioma was accomplished with no permanent morbidity or operative mortality. The authors conclude that this approach is advantageous for resecting tumors in this location because, by avoiding unnecessary brain resection or retraction, it significantly reduces the risk of injury to lateral temporal lobe structures, helps maintain precise spatial and anatomical orientation for the surgeon, and, like all computer-assisted volumetric approaches, delineates the margin between the tumor and surrounding neural tissue.
Intramedullary spinal epidermoid presenting after thoracic meningocele repair: case report
Bartosz T. Grobelny, Howard L. Weiner, and David H. Harter
A 4-year-old girl with a history of thoracic meningocele repair at the age of 3 months presented with progressive myelopathy. An intramedullary thoracic epidermoid was identified on MRI. The patient underwent excision of the epidermoid and subsequently returned to neurological baseline. This case illustrates the potential for delayed development of intraspinal epidermoid after initial repair of a simple meningocele.
Medulloblastoma: mouse models and novel targeted therapies based on the Sonic hedgehog pathway
Leandro R. Piedimonte, Ian K. Wailes, and Howard L. Weiner
Understanding molecular pathways, signaling cascades, and genetic alterations activated during tumorigenesis is essential for the development of targeted cancer treatments. In children, tumors of the central nervous system are thought to arise from progenitor cells that show considerable temporal and spatial heterogeneity in a developmental environment that is different from that of the adult. Investigating the molecular basis of pediatric tumors is critical because it is likely to generate novel treatments. Animal models have brought many important advances in this field. In this review the authors discuss the mouse models based on the Sonic hedgehog pathway, which have provided a better knowledge of the genetic and molecular alterations of medulloblastoma.
Stereotactic endoscopic resection and surgical management of a subependymal giant cell astrocytoma
Shaun D. Rodgers, Luigi Bassani, Howard L. Weiner, and David H. Harter
Subependymal giant cell astrocytomas (SEGAs) are benign tumors, most commonly associated with tuberous sclerosis complex (TSC). Arising from the lateral ependymal surface, these tumors may obstruct one or both foramina of Monro, resulting in hydrocephalus and often requiring treatment. Although interhemispheric-transcallosal and transcortical-transventricular approaches have been the standard methods for resecting these tumors, advances in neuroendoscopic techniques have expanded SEGAs as a potential target for endoscopic resection.
The authors present a case of an endoscopically resected SEGA with stereotactic guidance in a 4-year-old girl with TSC. A gross-total resection of an enlarging SEGA was achieved. This represents one of the early case reports of endoscopically resected SEGAs. Although recent advances in medical treatment for SEGAs with mammalian target of rapamycin (mTOR) pathway inhibitors have shown promising initial results, the long-term safety and efficacy of mTOR inhibitors has yet to be determined. The propensity of these tumors to cause obstructive hydrocephalus requires that a surgical option remain. Neuroendoscopic approaches may allow a safe and effective technique.
Coagulation abnormalities in children undergoing epilepsy surgery
Donato Pacione, Francine Blei, Orrin Devinsky, Howard L. Weiner, and Jonathan Roth
Surgery is increasingly used to treat children with refractory epilepsy. Before surgery, the authors routinely evaluated the coagulation profile to identify coagulation abnormalities not established by personal and family history, physical examination, and routine screening tests.
Thirty-nine consecutive children undergoing testing prior to epilepsy surgery were prospectively evaluated. The authors evaluated a detailed hematological history and an elaborative hematological panel including complete blood count, hepatic panel, anticoagulant levels, coagulation profile (prothrombin time, partial thromboplastin time, international normalized ratio, fibrinogen, thrombin time, von Willebrand antigen, ristocetin cofactor, factor VIII, and individual factor assays when indicated) and platelet aggregation studies (in the presence of adenosine diphosphate, epinephrine, collagen, and ristocetin). Patient variables included tuberous sclerosis complex (TSC), age at epilepsy onset, age at surgery, seizure frequency, number and type of antiepileptic drugs, recent or present ketogenic diet, and use of selective serotonin reuptake inhibitors.
Ten children (25.6%) had either coagulation or platelet function abnormalities. Abnormal coagulation was identified in 5 children, and abnormal platelet function was discovered in 6. A diagnosis of TSC was associated with a platelet function abnormality (p = 0.012), whereas children without TSC had a higher rate of coagulopathy (p = 0.041). None of the other characteristics reached statistical significance. In 2 patients (5.1%) with TSC and platelet aggregation abnormalities, the authors noted normal standard screening laboratory studies and an uneventful detailed personal and family history. One of these 2 patients developed a significant intraoperative bleeding complication.
A preoperative screening with standard laboratory studies and detailed history may not be adequate to fully examine underlying coagulation abnormalities in children with refractory epilepsy. Platelet aggregation studies should be considered in patients with TSC.
Introduction. Evolving perspectives on the intersection between neurosurgery and neurocutaneous disorders
Caitlin E. Hoffman, Ramesh Sharanappa Doddamani, Michael J. Fisher, and Howard L. Weiner
Retracted Tuberous sclerosis complex: molecular pathogenesis and animal models
Leandro R. Piedimonte, Ian K. Wailes, and Howard L. Weiner
Mutations in one of two genes, TSC1 and TSC2, result in a similar disease phenotype by disrupting the normal interaction of their protein products, hamartin and tuberin, which form a functional signaling complex. Disruption of these genes in the brain results in abnormal cellular differentiation, migration, and proliferation, giving rise to the characteristic brain lesions of tuberous sclerosis complex (TSC) called cortical tubers. The most devastating complications of TSC affect the central nervous system and include epilepsy, mental retardation, autism, and glial tumors. Relevant animal models, including conventional and conditional knockout mice, are valuable tools for studying the normal functions of tuberin and hamartin and the way in which disruption of their expression gives rise to the variety of clinical features that characterize TSC. In the future, these animals will be invaluable preclinical models for the development of highly specific and efficacious treatments for children affected with TSC.
Removal of a flanged ventricular catheter: illustrative case
M. Benjamin Larkin, Tyler T. Lazaro, Howard L. Weiner, and William E. Whitehead
Flanged ventricular catheters were created in the 1970s to decrease shunt failure by preventing the holes at the catheter tip from contacting the choroid plexus. However, the flanges on the catheter frequently scarred within and tether to the choroid plexus, resulting in higher rates of intraventricular hemorrhage when removed. Today, flanged catheters are rarely encountered.
The authors describe an illustrative case of a 7-year-old girl recently adopted from another country with a history of myelomeningocele and shunted hydrocephalus. She had been treated with a flanged catheter at birth. She presented with a shunt infection, which required removal of the flanged catheter tethered to the choroid.
The authors illustrate the safe removal of a posterior-entry flanged ventricular catheter tethered to the choroid plexus using monopolar flexible electrocautery. The removal was monitored with a flexible endoscope inserted from an ipsilateral anterior burr hole and was followed by an endoscopic third ventriculostomy.
Symptomatic obstructive hydrocephalus caused by choroid plexus hyperplasia in a pediatric patient: illustrative case
Ana Sofia Alvarez, John P McGinnis, Rajan Patel, and Howard L Weiner
Choroid plexus hyperplasia has been described as a rare cause of communicating hydrocephalus due to cerebrospinal fluid (CSF) overproduction. However, this is the first report of symptomatic obstructive hydrocephalus caused by mechanical obstruction of the aqueduct by a hyperplastic choroid plexus.
A 4-year-old male presented with headaches and intermittent emesis. Magnetic resonance imaging (MRI) of the brain showed abnormal enlargement of the choroid plexus in the lateral ventricles with extension into the third ventricle, resulting in obstruction of the aqueduct of Sylvius, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy (ETV) was chosen as the surgical treatment. During the procedure, high pressure flow of clear CSF was noted. Normal intraventricular anatomy was identified, and no cyst or tumor was found. The postoperative MRI showed a patent third ventriculostomy, without complication, and a significant decrease in supratentorial ventricular size. The patient was discharged 3 days after surgery with a complete resolution of symptoms.
Choroid plexus hyperplasia has the potential to cause obstructive hydrocephalus, and it can be effectively treated with ETV. Our hypothesis is that the change in pressure caused by the procedure may have led to an uncorking of the aqueduct by the hyperplastic choroid plexus, contributing to the observed improvement.