Insular gliomas were traditionally considered a nonsurgical entity due to the high morbidity associated with resection. For the past 20 years, advances in microsurgical and brain mapping techniques have allowed neurosurgeons to resect insular gliomas with acceptable morbidity rates. Maximizing the extent of resection is nowadays the goal of surgery since this has proven to be an independent factor contributing to longer survival. Despite much progress, insular tumors remain a challenge for the neurosurgeon due to the complex anatomy of the region and technical expertise required to minimize morbidity during surgery. Herein, the authors describe the current surgical nuances, based on their experience and a literature review, that will allow the surgeon to achieve a thorough resection while ensuring patient safety. The key factors for successful surgery in the insular region include detailed knowledge of the surgical anatomy, mastery of the nuances of cortical and subcortical mapping methods, and meticulous microsurgical technique.
Roberto Rey-Dios and Aaron A. Cohen-Gadol
Roberto Rey-Dios and Aaron A. Cohen-Gadol
Glossopharyngeal neuralgia (GPN) is an uncommon facial pain syndrome often misdiagnosed as trigeminal neuralgia. The rarity of this condition and its overlap with other cranial nerve hyperactivity syndromes often leads to a significant delay in diagnosis. The surgical procedures with the highest rates of pain relief for GPN are rhizotomy and microvascular decompression (MVD) of cranial nerves IX and X. Neurovascular conflict at the level of the root exit zone of these cranial nerves is believed to be the cause of this pain syndrome in most cases. Vagus nerve rhizotomy is usually reserved for cases in which vascular conflict is not evident. A review of the literature reveals that although the addition of cranial nerve X rhizotomy may improve the chances of long-term pain control, this maneuver also increases the risk of permanent dysphagia and vocal cord paralysis. The risks of this procedure have to be carefully weighed against its benefits. Based on the authors' experience, careful patient selection with a thorough exploratory operation most often leads to identification of the site of vascular conflict, obviating the need for cranial nerve X rhizotomy.
Timothy J. Kovanda, Roberto Rey-Dios, Jared Travnicek, and Aaron A. Cohen-Gadol
Surgical options for pediatric patients with marked dysfunction of a single epileptogenic hemisphere have evolved over time. Complications resulting from highly resective operations such as anatomical hemispherectomy, including superficial siderosis and secondary hydrocephalus, have led to the development of less resective and more disconnective functional hemispherectomy. Functional hemispherectomy has recently given rise to hemispherotomy, the least resective operation primarily aimed at disconnecting the abnormal hemisphere. Hemispherotomy is effective in decreasing seizure frequency and most likely decreases the risk of postoperative complications when compared with its predecessors. Hemispherotomy is a technically challenging operation that requires a thorough understanding of 3D cerebral anatomy to ensure adequate hemispheric disconnection without placing important structures at risk. The details of germane operative anatomy are not currently available because of the difficulty in exposing this operative anatomy adequately in cadavers to prepare detailed instructive illustrations. Using 3D graphic models, the authors have prepared 2D overlay illustrations to discuss the relevant operative nuances for a modified form of this procedure. Through hemispherotomy, experienced surgeons can effectively treat patients with unilateral epileptogenic hemisphere dysfunction while limiting potential complications.
Roberto Jose Diaz, Roberto Rey Dios, Eyas M. Hattab, Kelly Burrell, Patricia Rakopoulos, Nesrin Sabha, Cynthia Hawkins, Gelareh Zadeh, James T. Rutka, and Aaron A. Cohen-Gadol
Intravenous fluorescein sodium has been used during resection of high-grade gliomas to help the surgeon visualize tumor margins. Several studies have reported improved rates of gross-total resection (GTR) using high doses of fluorescein sodium under white light. The recent introduction of a fluorescein-specific camera that allows for high-quality intraoperative imaging and use of very low dose fluorescein has drawn new attention to this fluorophore. However, the ability of fluorescein to specifically stain glioma cells is not yet well understood.
The authors designed an in vitro model to assess fluorescein uptake in normal human astrocytes and U251 malignant glioma cells. An in vivo experiment was also subsequently designed to study fluorescein uptake by intracranial U87 malignant glioma xenografts in male nonobese diabetic/severe combined immunodeficient mice. A genetically induced mouse glioma model was used to adjust for the possible confounding effect of an inflammatory response in the xenograft model. To assess the intraoperative application of this technology, the authors prospectively enrolled 12 patients who underwent fluorescein-guided resection of their high-grade gliomas using low-dose intravenous fluorescein and a microscope-integrated fluorescence module. Intraoperative fluorescent and nonfluorescent specimens at the tumor margins were randomly analyzed for histopathological correlation.
The in vitro and in vivo models suggest that fluorescein demarcation of glioma-invaded brain is the result of distribution of fluorescein into the extracellular space, most likely as a result of an abnormal blood-brain barrier. Glioblastoma tumor cell–specific uptake of fluorescein was not observed, and tumor cells appeared to mostly exclude fluorescein. For the 12 patients who underwent resection of their high-grade gliomas, the histopathological analysis of the resected specimens at the tumor margin confirmed the intraoperative fluorescent findings. Fluorescein fluorescence was highly specific (up to 90.9%) while its sensitivity was 82.2%. False negatives occurred due to lack of fluorescence in areas of diffuse, low-density cellular infiltration. Margins of contrast enhancement based on intraoperative MRI–guided StealthStation neuronavigation correlated well with fluorescent tumor margins. GTR of the contrast-enhancing area as guided by the fluorescent signal was achieved in 100% of cases based on postoperative MRI.
Fluorescein sodium does not appear to selectively accumulate in astrocytoma cells but in extracellular tumor cell-rich locations, suggesting that fluorescein is a marker for areas of compromised blood-brain barrier within high-grade astrocytoma. Fluorescein fluorescence appears to correlate intraoperatively with the areas of MR enhancement, thus representing a practical tool to help the surgeon achieve GTR of the enhancing tumor regions.