Multiple Cavernous Malformations

OBJECTIVEThe aim of this study was to predict postoperative facial nerve function during pontine cavernous malformation surgery by monitoring facial motor evoked potentials (FMEPs).METHODSFrom 2008 to 2017, 10 patients with pontine cavernous malformations underwent total resection via the trans–fourth ventricle floor approach with FMEP monitoring. House-Brackmann grades and Karnofsky Performance Scale (KPS) scores were obtained pre- and postoperatively. The surgeries were performed using one of 2 safe entry zones into the brainstem: the suprafacial triangle and infrafacial triangle approaches. Six patients underwent the suprafacial triangle approach, and 4 patients underwent the infrafacial triangle approach. A cranial peg screw electrode was used to deliver electrical stimulation for FMEP by a train of 4 or 5 pulse anodal constant current stimulation. FMEP was recorded from needle electrodes on the ipsilateral facial muscles and monitored throughout surgery by using a threshold-level stimulation method.RESULTSFMEPs were recorded and analyzed in 8 patients; they were not recorded in 2 patients who had severe preoperative facial palsy and underwent an infrafacial triangle approach. Warning signs appeared in all patients who underwent the suprafacial triangle approach. However, after temporarily stopping the procedures, FMEP findings during surgery showed recovery of the thresholds. FMEPs in patients who underwent the infrafacial triangle approach were stable during the surgery. House-Brackmann grades were unchanged postoperatively in all patients. Postoperative KPS scores improved in 3 patients, decreased in 1, and remained the same in 6 patients.CONCLUSIONSFMEPs can be used to monitor facial nerve function during surgery for pontine cavernous malformations, especially when the suprafacial triangle approach is performed.

Download Full-text

A Series of 14 Representative Presentations of Cerebral Cavernous Malformations

Interdisciplinary Neurosurgery ◽

10.1016/j.inat.2021.101298 ◽

2021 ◽

pp. 101298

Author(s):

Ryan Hudnall ◽

Eric X. Chen ◽

Patrick J Opperman ◽

Sean Kelly ◽

Justin A. Cramer ◽

...

Keyword(s):

Cerebral Cavernous Malformations ◽

Cavernous Malformations

Download Full-text

Natural history and treatment options of radiation-induced brain cavernomas: a systematic review

Neurosurgical Review ◽

10.1007/s10143-021-01598-y ◽

2021 ◽

Author(s):

Gildas Patet ◽

Andrea Bartoli ◽

Torstein R. Meling

Keyword(s):

Systematic Review ◽

Natural History ◽

Radiation Treatment ◽

Neurological Deficits ◽

Cochrane Library ◽

Average Dose ◽

Cavernous Malformations ◽

Radiation Induced ◽

The Mean

AbstractRadiation-induced cavernous malformations (RICMs) are delayed complications of brain irradiation during childhood. Its natural history is largely unknown and its incidence may be underestimated as RCIMS tend to develop several years following radiation. No clear consensus exists regarding the long-term follow-up or treatment. A systematic review of Embase, Cochrane Library, PubMed, Google Scholar, and Web of Science databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was performed. Based on our inclusion/exclusion criteria, 12 articles were included, totaling 113 children with RICMs, 86 were treated conservatively, and 27 with microsurgery. We were unable to precisely define the incidence and natural history from this data. The mean age at radiation treatment was 7.3 years, with a slight male predominance (54%) and an average dose of 50.0 Gy. The mean time to detection of RICM was 9.2 years after radiation. RICM often developed at distance from the primary lesion, more specifically frontal (35%) and temporal lobe (34%). On average, 2.6 RICMs were discovered per child. Sixty-seven percent were asymptomatic. Twenty-one percent presented signs of hemorrhage. Clinical outcome was favorable in all children except in 2. Follow-up data were lacking in most of the studies. RICM is most often asymptomatic but probably an underestimated complication of cerebral irradiation in the pediatric population. Based on the radiological development of RICMs, many authors suggest a follow-up of at least 15 years. Studies suggest observation for asymptomatic lesions, while surgery is reserved for symptomatic growth, hemorrhage, or focal neurological deficits.

Download Full-text

Treatments and outcomes of untreated cerebral cavernous malformations in China: study protocol of a nationwide multicentre prospective cohort study

BMJ Open ◽

10.1136/bmjopen-2020-037957 ◽

2020 ◽

Vol 10 (10) ◽

pp. e037957

Author(s):

Fuxin Lin ◽

Qiu He ◽

Zhuyu Gao ◽

Lianghong Yu ◽

Dengliang Wang ◽

...

Keyword(s):

Informed Consent ◽

Natural History ◽

Study Protocol ◽

Treated Group ◽

Secondary Outcome ◽

Long Term Outcomes ◽

Cavernous Malformations ◽

Electronic Data Capture System

IntroductionThe treatment decision and long-term outcomes of previously untreated cerebral cavernous malformation (U-CCM) are still controversial. Therefore, we are conducting a nationwide multicentre prospective registry study in China to determine the natural history and effect of surgical treatment on long-term outcomes in Chinese people with U-CCM.Methods and analysisThis study was started on 1 January 2018 and is currently ongoing. It is a cohort follow-up study across a 5-year period. Patients will be followed up for at least 3 years after inception. Patients with U-CCM will be enrolled from 24 Grade III, level A hospitals distributed all over China. The cohort size is estimated to be 1200 patients. Patients are registered in surgically treated group and conservatively treated group. Clinical characteristics, radiology information and laboratory data are prospectively collected using an electronic case report form through an electronic data capture system. The primary outcome of this study is poor clinical outcome at the last follow-up (modified Rankin Scale score >2 lasting at least 1 year). The secondary outcome includes symptomatic haemorrhage, drug refractory epilepsy, focal neurological deficits, morbidity and all-cause mortality during follow-up. Univariate and multivariate regression analysis will be performed to determine the risk factors for poor outcomes in all patients, and to estimate the effect of surgery. Life tables, Kaplan-Meier estimates, log-rank test and proportional hazards Cox regression will be used to analyse the follow-up data of conservatively treated patients to determine the natural history of U-CCM. Initial presentation and location of U-CCM are prespecified subgroup factors.Ethics and disseminationThe study protocol and informed consent form have been reviewed and approved by the Research Ethical Committee of First Affiliated Hospital of Fujian Medical University (FAHFMU-2018-003).Written informed consent will be obtained from each adult participant or from the guardian of each paediatric participant. The final results will be published in peer-reviewed journals.Trial registration numberNCT03467295.

Download Full-text

Unexpected Binding of Tozuleristide “Tumor Paint” to Cerebral Vascular Malformations: A Potentially Novel Application of Fluorescence-Guided Surgery

Neurosurgery ◽

10.1093/neuros/nyab106 ◽

2021 ◽

Author(s):

Andrew J Kobets ◽

David Nauen ◽

Amy Lee ◽

Alan R Cohen

Keyword(s):

Near Infrared ◽

Vascular Malformations ◽

Cns Tumors ◽

Matrix Metalloproteinase 2 ◽

Cerebrovascular Lesions ◽

Cavernous Malformations ◽

Guided Surgery ◽

Fluorescence Guided Surgery ◽

Cerebral Vascular Malformations ◽

Cerebral Vascular

Abstract BACKGROUND Fluorescence-guided surgery (FGS) is under investigation as a means to improve the extent of resection for primary central nervous system (CNS) tumors. Tozuleristide, known also as “Tumor Paint,” is an investigational tumor-targeting agent covalently conjugated to a derivative of the fluorescent dye indocyanine green. OBJECTIVE To report the finding of avid intraoperative fluorescence of tozuleristide on cerebral vascular malformations. METHODS Our institution is participating in a phase 2/3 study of intraoperative near-infrared fluorescence detection of pediatric primary CNS tumors in patients receiving intravenous tozuleristide and imaged with the Canvas system. Our site enrolled 2 patients with intracranial lesions, suspected preoperatively of possibly being gliomas that proved to be cavernous vascular malformations after resection. RESULTS Each lesion had a dark blue mulberry appearance and each fluoresced avidly with tozuleristide. Each was completely resected, and the patients recovered without deficit. Pathological assessment showed cavernous angioma for both cases. Tozuleristide fluorescence is postulated to result from binding to matrix metalloproteinase-2 and annexin A2, and literature review demonstrates expression of both these ligands on multiple cerebrovascular lesions, including cavernous malformations. CONCLUSION This finding deserves further investigation to determine if tozuleristide “Tumor Paint” may have a wider role in the identification of non-neoplastic intracranial pathologies.

Download Full-text

Mortality in Patients with Brainstem Cavernous Malformations

Cerebrovascular Diseases ◽

10.1159/000516909 ◽

2021 ◽

pp. 1-7

Author(s):

Julia Velz ◽

Marian Christoph Neidert ◽

Yang Yang ◽

Kevin Akeret ◽

Peter Nakaji ◽

...

Keyword(s):

Mortality Rate ◽

Literature Review ◽

Systematic Literature Review ◽

Postoperative Mortality ◽

Mortality Rates ◽

Cavernous Malformations ◽

Patient Cohort ◽

Actual Risk ◽

Single Center Study

Objective: Brainstem cavernous malformations (BSCM)-associated mortality has been reported up to 20% in patients managed conservatively, whereas postoperative mortality rates range from 0 to 1.9%. Our aim was to analyze the actual risk and causes of BSCM-associated mortality in patients managed conservatively and surgically based on our own patient cohort and a systematic literature review. Methods: Observational, retrospective single-center study encompassing all patients with BSCM that presented to our institution between 2006 and 2018. In addition, a systematic review was performed on all studies encompassing patients with BSCM managed conservatively and surgically. Results: Of 118 patients, 54 were treated conservatively (961.0 person years follow-up in total). No BSCM-associated mortality was observed in our conservatively as well as surgically managed patient cohort. Our systematic literature review and analysis revealed an overall BSCM-associated mortality rate of 2.3% (95% CI: 1.6–3.3) in 22 studies comprising 1,251 patients managed conservatively and of 1.3% (95% CI: 0.9–1.7) in 99 studies comprising 3,275 patients with BSCM treated surgically. Conclusion: The BSCM-associated mortality rate in patients managed conservatively is almost as low as in patients treated surgically and much lower than in frequently cited reports, most probably due to the good selection nowadays in regard to surgery.

Download Full-text