scholarly journals Remarkable Clinical Improvement Following Microsurgical Resection of Left Lingual Gyrus Cerebral Cavernous Malformation: A Case Report

2020 ◽  
Author(s):  
Samer S. Hoz ◽  
Zahraa F. Al-Sharshahi ◽  
Mustafa M. Altaweel ◽  
Saja A. Albanaa
Keyword(s):  
Occipital Lobe ◽  
Surgical Removal ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Lingual Gyrus ◽  
Microsurgical Resection ◽  
Clonic Seizures ◽  
Magnetic Resonance Imaging Mri

Abstract Introduction Cerebral cavernous malformations (CCMs) are collections of dilated and irregular capillaries in the brain. Cerebral cavernous malformations are predominantly supratentorial; occipital CCMs are rare. Surgical removal is indicated for CCMs with recurrent hemorrhage, refractory seizures, and expanding lesions. Case Description We describe a case of a 15-year-old male who presented with repeated tonic-clonic seizures and right homonymous hemianopia of 3-week duration. Magnetic resonance imaging (MRI) showed a mass located on the left medial occipital lobe, specifically in the left lingual gyrus. The T2-weighted and T2-gradient echo images confirmed the diagnosis of a CCM. Total microscopic resection was achieved. There were no surgical complications. The visual deficit improved, and the patient was seizure-free on subsequent follow-up visits. Conclusion Surgical resection of an occipital CCM resulted in a remarkable improvement in terms of seizures and visual field deficits.

Long-term visual outcome after microsurgical removal of occipital lobe cavernomas

2012 ◽  
Vol 117 (2) ◽  
pp. 295-301 ◽  
Author(s):  
Juri Kivelev ◽  
Elina Koskela ◽  
Kirsi Setälä ◽  
Mika Niemelä ◽  
Juha Hernesniemi
Keyword(s):  
Visual Field ◽  
Visual Fields ◽  
Visual Field Loss ◽  
Occipital Lobe ◽  
Visual Outcome ◽  
Surgical Removal ◽  
Long Term Outcome ◽  
Visual Field Deficit

Object Cavernomas in the occipital lobe are relatively rare. Because of the proximity to the visual cortex and incoming subcortical tracts, microsurgical removal of occipital cavernomas may be associated with a risk of visual field defects. The goal of the study was to analyze long-term outcome after operative treatment of occipital cavernomas with special emphasis on visual outcome. Methods Of the 390 consecutive patients with cavernomas who were treated at Helsinki University Central Hospital between 1980 and 2011, 19 (5%) had occipital cavernomas. Sixteen patients (4%) were surgically treated and are included in this study. The median age was 39 years (range 3–59 years). Seven patients (56%) suffered from hemorrhage preoperatively, 5 (31%) presented with visual field deficits, 11 (69%) suffered from seizures, and 4 (25%) had multiple cavernomas. Surgery was indicated for progressive neurological deterioration. The median follow-up after surgery was 5.25 years (range 0.5–14 years). Results All patients underwent thorough neuroophthalmological assessment to determine visual outcome after surgery. Visual fields were classified as normal, mild homonymous visual field loss (not disturbing the patient, driving allowed), moderate homonymous visual field loss (disturbing the patient, driving prohibited), and severe visual field loss (total homonymous hemianopia or total homonymous quadrantanopia). At the last follow-up, 4 patients (25%) had normal visual fields, 6 (38%) had a mild visual field deficit, 1 (6%) complained of moderate visual field impairment, and 5 (31%) had severe homonymous visual field loss. Cavernomas seated deeper than 2 cm from the pial surface carried a 4.4-fold risk of postoperative visual field deficit relative to superficial ones (p = 0.034). Six (55%) of the 11 patients presenting with seizures were seizure-free postoperatively. Eleven (69%) of 16 patients had no disability during the long-term follow-up. Conclusions Surgical removal of occipital cavernomas may carry a significant risk of postoperative visual field deficit, and the risk is even higher for deeper lesions. Seizure outcome after removal of these cavernomas appeared to be worse than that after removal in other supratentorial locations. This should be taken into account during preoperative planning.

Abstract 159: Exceptional Aggressiveness of CCM3 Phenotype

Stroke ◽  
2014 ◽  
Vol 45 (suppl_1) ◽  
Author(s):  
Tania J Rebeiz ◽  
Abdul Ghani Mikati ◽  
Darlene Simkhin ◽  
Cornelia Lee ◽  
Amy Akers ◽  
...  
Keyword(s):  
Spontaneous Mutation ◽  
Mutation Screening ◽  
High Sensitivity ◽  
Skin Lesions ◽  
Cerebral Cavernous Malformations ◽  
Cognitive Disability ◽  
Cavernous Malformations ◽  
Patient Advocacy Group ◽  
Sporadic Cases

Introduction: Familial forms of cerebral cavernous malformations (CCM) account for about 1/3 of cases, involving autosomal dominant inheritance at 1 of 3 gene loci. Few studies have examined any special features of the rarest cases with CCM3 (PDCD10 ) mutation at q3, constituting <15 % of probands genotyped by sequential mutation screening, and <2% of CCM cases at large. Hypothesis: We hypothesize that CCM3 cases have unique phenotypic features not recognized in the more common CCM1 and 2 families, or in sporadic cases. Methods: Twelve probands including 17 subjects with confirmed CCM3 mutations were prospectively enrolled through systematic facilitated referral by the patient advocacy group Angioma Alliance. Clinical features were catalogued, including high sensitivity susceptibility weighted imaging (SWI). Rates of overt hemorrhage were determined based on adjudicated criteria. Comparisons were made to systematic literature review of natural history data on non-CCM3 cases. Results: The first overt hemorrhage occurred most often in the 1st decade of life (mean age 5.8). Nine of 17 subjects (52%) suffered 30 overt hemorrhages, with an estimated incidence of 6.7 % /patient/year based on exposure risk since birth, and 17% /patient/year based on risk since first symptom onset. Lesion burden on SWI was exceptionally high, >100 lesions in 28%, and > 20 lesions in 72% of cases, respectively. Adjusted bleed rate was <0.5% /lesion/year. New SWI lesions formed at a rate of 2.7/patient/year in prospective follow-up, and 1.8/patient/year based on years since birth. Scoliosis was found in 47% (an association not recognized previously), skin lesions in 29.4%, and brain tumors in 29.4% of cases, respectively. Cognitive disability affected 47% of cases, mostly in association with high lesion burden. Six of 15 cases with parental screening (40%) represented a spontaneous mutation. Conclusion: CCM3 is exceptionally aggressive compared to other familial and sporadic CCM. High risks of bleeding and cognitive disability mostly reflect severe lesion burden early in life, rather than a higher risk per lesion. These results will inform the design of clinical trials, urgently needed to address this unique CCM cohort.

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

2006 ◽  
Vol 104 (3) ◽  
pp. 376-381 ◽  
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.

scholarly journals Supratentorial cerebral cavernous malformations: clinical, surgical, and genetic involvement

2006 ◽  
Vol 21 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Vincenzo Antonio D'Angelo ◽  
Costanzo De Bonis ◽  
Rosina Amoroso ◽  
Alessandro Calì ◽  
Leonardo D'Agruma ◽  
...  
Keyword(s):  
Mutational Analysis ◽  
Family Members ◽  
Neurological Deficits ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Psychological Burden ◽  
Management Algorithm ◽  
Screening Protocol

Object Although there is general agreement on the methods of treatment for symptomatic supratentorial cerebral cavernous malformations (CMs) located in noneloquent areas, some controversy exists regarding the management of cerebral CMs that are asymptomatic and/or located in eloquent or deep areas. Moreover, recent advances in genetic findings could influence both standard clinical management and the follow-up strategy in affected individuals. Thus, the objective of this study was to develop, based on the authors' experience and a literature review, a management algorithm to deal with supratentorial cerebral CMs. Methods The authors retrospectively reviewed the clinical data related to 118 patients who underwent surgery for symptomatic supratentorial cerebral CMs at their institution. Twenty-eight of 118 patients harbored multiple lesions, and nine of these 28 patients had a clinically positive familial history. Genetic investigations were performed in 89 patients (75%). Conclusions Surgery for supratentorial cerebral CMs in noneloquent locations is safe and curative. In cerebral CMs located in deep and eloquent areas and with symptoms including progressive neurological deficits, evidence of hemorrhage, and uncontrolled seizures, surgical treatment according to an integrated plan based on frameless stereotactic guidance and functional magnetic resonance imaging is recommended and results in acceptably low morbidity. The data support the need for long-term imaging follow up in all patients, careful preoperative vascular studies to detect associated venous anomalies, and the importance of genetic mutational analysis. The DNA screening protocol will change the care of family members of patients with familial forms of cerebral CMs, because affected asymptomatic family members may benefit by early detection of lesions. At the same time, the exclusion of family members who are not carriers of the mutation as members of the population at risk reduces the economic and psychological burden of clinical and instrumental monitoring.

scholarly journals Radiation diagnostics of cerebral cavernous malformations

2021 ◽  
Vol 2 (1) ◽  
pp. 39-48
Author(s):  
Elena N. Girya ◽  
Valentin E. Sinitsyn ◽  
Alexey S. Tokarev
Keyword(s):  
Pulse Sequences ◽  
Vascular Pathology ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Imaging Sequence ◽  
Magnetic Resonance Imaging Mri ◽  
The Moment ◽  
Ct And Mri ◽  
Selection Of

Cerebral cavernous malformations are a fairly common vascular pathology at the moment, with the number of detected cases increasing dramatically in recent years. This is because modern neuroimaging methods such as computed tomography (CT) and magnetic resonance imaging (MRI) have been introduced into clinical practice and are widely available. Prior to the advent of CT and MRI technologies, it was extremely difficult to diagnose this pathology, and the diagnosis was usually made intraoperatively or based on autopsy data. Further, the literature review is devoted to the radiological diagnosis of cerebral cavernous malformations (CM). The role of neuroimaging methods in the diagnosis of cavernous malformations, as well as the use of MRI for CM visualization, was analyzed. The advantages of MRI over other neuroimaging methods for this pathology have been demonstrated. Pulse sequences of MRI and signaling characteristics of various foci were characterized, depending on the morphological substrate. The significance of the susceptibility-weighted imaging sequence was also evaluated for the detection of multifocal lesions in cases of familial CM. The study of the main pulse sequences of MRI for visualization of CM will improve the protocol algorithm for the timely diagnosis of this pathology and the selection of therapeutic approach.

scholarly journals Diagnosis and Management of Wooden Foreign Bodies in the Orbit: A Case Report

2021 ◽  
Vol 17 (2) ◽  
pp. 158-161
Author(s):  
Hyo Bong Kim ◽  
Hoon Kim ◽  
Soo Yeon Lim ◽  
In Chang Koh
Keyword(s):  
Magnetic Resonance Imaging ◽  
Optic Nerve ◽  
Foreign Bodies ◽  
Surgical Removal ◽  
Resonance Imaging ◽  
Upper Eyelid ◽  
Diagnosis And Management ◽  
Optic Nerve Damage ◽  
Magnetic Resonance Imaging Mri

The diagnosis and management of intraorbital foreign bodies are challenging for surgeons. Foreign bodies made of wood and those located close to the optic nerve are especially difficult to manage. Herein, we report our experience with the diagnosis and management of intraorbital wooden foreign bodies utilizing magnetic resonance imaging (MRI). A 50-year-old man presented to the emergency room with a laceration on his left upper eyelid. No foreign bodies were visible through the laceration, and computed tomography findings showed only evidence of cellulitis with abscess formation. Despite drainage and antibiotics, the cellulitis worsened; subsequently, we found multiple wooden foreign bodies on MRI. Surgical removal was performed, excluding those in the intraconal space to avoid optic nerve damage. The patient recovered well and has not experienced any ophthalmic complications at 1 year of follow-up.

Natural Course of Cerebral Cavernous Malformations in Children: A Five-Year Follow-Up Study

Stroke ◽  
2021 ◽  
Author(s):  
Alejandro N. Santos ◽  
Laurèl Rauschenbach ◽  
Dino Saban ◽  
Bixia Chen ◽  
Annika Herten ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Brain Stem ◽  
Odds Ratio ◽  
Pediatric Patients ◽  
Cox Regression ◽  
Cerebral Cavernous Malformations ◽  
Multivariate Logistic Regression ◽  
Cavernous Malformations ◽  
History Of

Background and Purpose: The purpose of this study was to investigate the natural course of cerebral cavernous malformations (CCM) in the pediatric population, with special emphasis on the risk of first and recurrent bleeding over a 5-year period. Methods: Our institutional database was screened for patients with CCM treated between 2003 and 2020. Patients ≤18 years of age with complete magnetic resonance imaging data set, clinical baseline characteristics, and ≥1 follow-up examination were included. Surgically treated individuals were censored after CCM removal. We assessed the impact of various parameters on first or recurrent intracerebral hemorrhage (ICH) at diagnosis using univariate and multivariate logistic regression adjusted for age and sex. Kaplan-Meier and Cox regression analyses were performed to determine the cumulative 5-year risk for (re)hemorrhage. Results: One hundred twenty-nine pediatric patients with CCM were analyzed. Univariate logistic regression identified brain stem CCM (odds ratio, 3.15 [95% CI, 1.15−8.63], P =0.026) and familial history of CCM (odds ratio, 2.47 [95% CI, 1.04−5.86], P= 0.041) as statistically significant predictors of ICH at diagnosis. Multivariate logistic regression confirmed this correlation (odds ratio, 3.62 [95% CI, 1.18−8.99], P= 0.022 and odds ratio, 2.53 [95% CI, 1.07−5.98], P =0.035, respectively). Cox regression analysis identified ICH as mode of presentation (hazard ratio, 14.01 [95% CI, 1.80−110.39], P= 0.012) as an independent predictor for rehemorrhage during the 5-year follow-up. The cumulative 5-year risk of (re)bleeding was 15.9% (95% CI, 10.2%−23.6%) for the entire cohort, 30.2% (20.2%−42.3%) for pediatric patients with ICH at diagnosis, and 29.5% (95% CI, 13.9%−51.1%) for children with brain stem CCM. Conclusions: Pediatric patients with brain stem CCM and familial history of CCM have a higher risk of ICH as mode of presentation. During untreated 5-year follow-up, they revealed a similar risk of (re)hemorrhage compared to adult patients. The probability of (re)bleeding increases over time, especially in cases with ICH at presentation or brain stem localization.

scholarly journals Antithrombotic medication and bleeding risk in patients with cerebral cavernous malformations: a cohort study

2019 ◽  
Vol 130 (6) ◽  
pp. 1922-1930
Author(s):  
David Bervini ◽  
Christian Jaeggi ◽  
Pasquale Mordasini ◽  
Philippe Schucht ◽  
Andreas Raabe
Keyword(s):  
Bleeding Risk ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Vascular Abnormalities ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Hemorrhagic Risk ◽  
Risk Of Hemorrhage ◽  
Antithrombotic Medication

OBJECTIVECerebral cavernous malformations (CCMs) are frequently diagnosed vascular abnormalities. The hemorrhagic risk associated with the use of long-term antithrombotic medication (ATM) in patients with CCMs is a matter of controversy. The aim of this study was to determine the hemorrhagic risk associated with ATM use in patients diagnosed with one or more CCMs.METHODSDemographic, clinical, treatment, and ATM-related information on patients diagnosed with one or more CCMs at a single institution over more than 34 years was retrospectively recorded. Univariate and multivariate descriptive and survival analyses were used to assess potential risk factors associated with CCM-related hemorrhage at presentation and during follow-up (first or subsequent hemorrhage).RESULTSA total of 408 patients were included in the analysis and 492 CCMs were followed up after diagnosis, for a total of 1616 lesion-years. Thirty-seven (7.5%) CCMs bled during follow-up, leading to an overall annual rate of CCM-related symptomatic hemorrhage of 2.3% (95% CI 1.7%–3.2%). Eighty-two patients harboring 91 CCMs (16.8%) were on ATM. When stratified for ATM, the annual rates of hemorrhage were 0.7% (95% CI < 0.01% to 4.2%) for the lesions in patients on ATM versus 2.5% (95% CI 1.8%–3.4%) for those not on ATM. ATM was not found to be associated with either an increased risk of CCM-related hemorrhage at presentation (p = 0.355) or an increased risk of CCM-related hemorrhage (first or subsequent hemorrhage) in multivariate descriptive (p = 0.912) and survival (p = 0.145) analyses.CONCLUSIONSThe use of ATM does not seem to be associated with an increased risk of hemorrhage in patients diagnosed with CCMs.

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