Cavernous Malformations and Venous Anomalies: Natural History and Surgical Management

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.

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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.

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Natural history and surgical management of spontaneous intracerebral hemorrhage: a systematic review

Journal of Neurosurgical Sciences ◽

10.23736/s0390-5616.20.04940-1 ◽

2021 ◽

Vol 64 (6) ◽

Author(s):

Jonathan RYCHEN ◽

Anthea O’NEILL ◽

Leon T. LAI ◽

David BERVINI

Keyword(s):

Systematic Review ◽

Natural History ◽

Intracerebral Hemorrhage ◽

Surgical Management ◽

Spontaneous Intracerebral Hemorrhage

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Risks of Surgical Management for Cavernous Malformations of the Nervous System

Neurosurgery ◽

10.1097/00006123-199806000-00008 ◽

1998 ◽

Vol 42 (6) ◽

pp. 1227-1227 ◽

Cited By ~ 1

Author(s):

H. Hunt Batjer

Keyword(s):

Nervous System ◽

Surgical Management ◽

Cavernous Malformations

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Cavernous malformations: natural history, diagnosis and treatment

Nature Reviews Neurology ◽

10.1038/nrneurol.2009.177 ◽

2009 ◽

Vol 5 (12) ◽

pp. 659-670 ◽

Cited By ~ 153

Author(s):

Sachin Batra ◽

Doris Lin ◽

Pablo F. Recinos ◽

Jun Zhang ◽

Daniele Rigamonti

Keyword(s):

Natural History ◽

Diagnosis And Treatment ◽

Cavernous Malformations

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Coexistence of intracranial and spinal cavernous malformations: a study of prevalence and natural history

Journal of Neurosurgery ◽

10.3171/jns.2006.104.3.376 ◽

2006 ◽

Vol 104 (3) ◽

pp. 376-381 ◽

Cited By ~ 47

Author(s):

Aaron A. Cohen-Gadol ◽

Jeffrey T. Jacob ◽

Diane A. Edwards ◽

William E. Krauss

Keyword(s):

Family History ◽

Natural History ◽

Mr Imaging ◽

Neurological Deficits ◽

Cavernous Malformations ◽

Recurrent Hemorrhage ◽

Long Term Follow Up ◽

History Of ◽

Clinically Significant

Object The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients’ mean age at presentation was 50 years (range 13–82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

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