Tag: Cerebral cavernous malformation

Posted on

The role of surveillance MRI scans in patients with sporadic cerebral cavernous malformations

J Neurosurg 144:1017–1023, 2026

This clinical study assesses the diagnostic value of routine versus symptom-driven MRI follow-up in patients with sporadic cerebral cavernous malformations (CCMs), analyzing radiographic changes at the first postdiagnosis scan in a prospective single-center cohort. Results show that new or worsening focal neurological deficit (FND) strongly predicts symptomatic hemorrhage, while routine surveillance rarely detects acute hemorrhage.

Imaging progression—including lesion growth, Zabramski classification changes, T1 hyperintensity, and edema—occurred across indications but had limited impact on management when patients were asymptomatic. The authors recommend symptom-driven MRI, reserving routine surveillance for select cases and early posthemorrhage confirmation.

Objective Evaluate whether routine surveillance MRI vs symptom-driven MRI better detects hemorrhage in patients with sporadic, brain-only cerebral cavernous malformations (CCMs), and what radiographic changes appear at first follow-up.

Cohort/Design Prospective single-center registry analysis of 236 sporadic brain-only CCM patients who had ≥1 follow-up MRI after diagnosis; MRI indications categorized as routine vs symptom-driven (e.g., new/worsening focal neurological deficit [FND], headache, seizure).

Hemorrhage yield Radiographic hemorrhage at first follow-up occurred in 19.1% (13/68) of symptom-driven MRIs vs 1.2% (2/168) of routine/non–CCM-related surveillance MRIs.

Routine progression In routine/non–CCM-related surveillance (n=168), 10.1% showed radiologic progression (growth ≥3 mm, more aggressive Zabramski type, or hemorrhage), but acute hemorrhage was only 1.2%; 88.2% of progression events occurred within 2 years.

Imaging correlates of SH Symptomatic hemorrhage (SH) was associated with lesion growth, moderate-to-severe T1-hyperintensity, and edema at follow-up; baseline lesion size and location did not predict hemorrhage.

Symptom predictors New or worsening FND predicted hemorrhage, while seizure or headache alone did not; in multivariate analysis, only new/worsening FND remained independently associated with hemorrhage (OR 13.73, p<0.001).

Clinical implication Follow-up MRI is most justified when there is new or worsening FND; routine surveillance in asymptomatic patients has limited diagnostic yield but may be reasonable in select situations.

Special cases MRI may still be considered for atypical, prolonged headaches distinct from baseline and not responsive to over-the-counter medication, or for changing/new seizure patterns based on clinical judgment.

Posted on

Magnetic Resonance Imaging-Guided Stereotactic Laser Ablation of Deep Cerebral Cavernous Malformations

Neurosurgery 89:635–644, 2021

Magnetic resonance imaging (MRI)-guided laser interstitial thermal therapy (MRgLITT) has been used successfully to treat epileptogenic cortical cerebral cavernous malformations (CCM). It is unclear whether MRgLITT would be as feasible or safe for deep CCMs

OBJECTIVE: To describe our experience with MRgLITT for symptomatic deep CCMs

METHODS: Patients’records were reviewed retrospectively. MRgLITT was carried out using a commercially available system in an interventional MRI suite with efforts to protect adjacent brain structures. Immediate postoperative imaging was used to judge ablation adequacy. Delayed postoperative MRI was used to measure lesion volume changes during follow-up.

RESULTS: Four patients with CCM in the thalamus, putamen, midbrain, or subthalamus presented with persistent and disabling neurological symptoms. A total of 2 patients presented with disabling headaches and sensory disturbances and 2 with recurrent symptomatic hemorrhages, of which 1 had familial CCM. Patients were considered by vascular neurosurgeons to be poor candidates for open surgery or had refused it. Multiple trajectories were used in most cases. Adverse events included device malfunction with leakage of saline causing transient mass effect in one patient, and asymptomatic tract hemorrhage in another. One patient suffered an expected mild but persistent exacerbation of baseline deficits. All patients showed improvement from a previously aggressive clinical course with lesion volume decreased by 20% to 73% in follow-up.

CONCLUSION: MRgLITT is feasible in the treatment of symptomatic deep CCM but may carry a high risk of complications without the benefit of definitive resection. We recommend cautious patient selection, low laser power settings, and conservative temper- ature monitoring in surrounding brain parenchyma.

Posted on

Assessment and validation of proposed classification tools for brainstem cavernous malformations

J Neurosurg 135:410–416, 2021

Treatment indications for patients with brainstem cavernous malformations (BSCMs) remain difficult and controversial. Some authors have tried to establish classification tools to identify eligible candidates for surgery. Authors of this study aimed to validate the performance and replicability of two proposed BSCM grading systems, the Lawton- Garcia (LG) and the Dammann-Sure (DS) systems.

METHODS For this cross-sectional study, a database was screened for patients with BSCM treated surgically between 2003 and 2019 in the authors’ department. Complete clinical records, preoperative contrast-enhanced MRI, and a postoperative follow-up ≥ 6 months were mandatory for study inclusion. The modified Rankin Scale (mRS) score was determined to quantify neurological function and outcome. Three observers independently determined the LG and the DS score for each patient.

RESULTS A total of 67 patients met selection criteria. Univariate and multivariate analyses identified multiple bleedings (p = 0.02, OR 5.59), lesion diameter (> 20 mm, p = 0.007, OR 5.43), and patient age (> 50 years, p = 0.019, OR 4.26) as predictors of an unfavorable postoperative functional outcome. Both the LG (AUC = 0.72, p = 0.01) and the DS (AUC = 0.78, p < 0.01) scores were robust tools to estimate patient outcome. Subgroup analyses confirmed this observation for both grading systems (LG: p = 0.005, OR 6; DS: p = 0.026, OR 4.5), but the combined use of the two scales enhanced the test performance significantly (p = 0.001, OR 22.5).

CONCLUSIONS Currently available classification systems are appropriate tools to estimate the neurological outcome after BSCM surgery. Future studies are needed to design an advanced scoring system, incorporating items from the LG and the DS score systems.

 

Posted on

Natural history of brainstem cavernous malformations

J Neurosurg 134:917–928, 2021

Given the paucity of data on the natural history of brainstem cavernous malformations (CMs), the authors aimed to evaluate the annual hemorrhage rate and hemorrhagic risk of brainstem CMs.

METHODS Nine hundred seventy-nine patients diagnosed with brainstem CMs were referred to Beijing Tiantan Hospital from 2006 to 2015; 224 patients were excluded according to exclusion criteria, and 47 patients were lost to follow-up. Thus, this prospective observational cohort included 708 cases (324 females). All patients were registered, clinical data were recorded, and follow-up was completed.

RESULTS Six hundred ninety (97.5%) of the 708 patients had a prior hemorrhage, 514 (72.6%) had hemorrhagic presentation, and developmental venous anomaly (DVA) was observed in 241 cases (34.0%). Two hundred thirty-seven prospective hemorrhages occurred in 175 patients (24.7%) during 3400.2 total patient-years, yielding a prospective annual hemorrhage rate of 7.0% (95% CI 6.2%–7.9%), which decreased to 4.7% after the 1st year. Multivariate Cox regression analysis after adjusting for sex and age identified hemorrhagic presentation (HR 1.574, p = 0.022), DVA (HR 1.678, p = 0.001), mRS score ≥ 2 on admission (HR 1.379, p = 0.044), lesion size > 1.5 cm (HR 1.458, p = 0.026), crossing the axial midpoint (HR 1.446, p = 0.029), and superficially seated location (HR 1.307, p = 0.025) as independent adverse factors for prospective hemorrhage, but history of prior hemorrhage was not significant. The annual hemorrhage rates were 8.3% and 4.3% in patients with and without hemorrhagic presentation, respectively; the rate was 9.9%, 6.0%, and 1.0% in patients with ≥ 2, only 1, and 0 prior hemorrhages, respectively; and the rate was 9.2% in patients with both hemorrhagic presentation and focal neurological deficit on admission.

CONCLUSIONS The study reported an annual hemorrhage rate of 7.0% exclusively for brainstem CMs, which significantly increased if patients presented with both hemorrhagic presentation and focal neurological deficit (9.2%), or any other risk factor. Patients with a risk factor for hemorrhage needed close follow-up regardless of the number of prior hemorrhages. It should be noted that the referral bias in this study could have overestimated the annual hemorrhage rate. This study improved the understanding of the natural history of brainstem CMs, and the results are important for helping patients and physicians choose a suitable treatment option based on the risk factors and stratified annual rates. Clinical trial registration no.: ChiCTR-POC-17011575 (http://www.chictr.org.cn/).

Posted on

Contralateral posterior interhemispheric approach to deep medial parietooccipital vascular malformations

J Neurosurg 129:198–204, 2018

Deep medial parietooccipital arteriovenous malformations (AVMs) and cerebral cavernous malformations (CCMs) are traditionally resected through an ipsilateral posterior interhemispheric approach (IPIA), which creates a deep, perpendicular perspective with limited access to the lateral margins of the lesion. The contralateral posterior interhemispheric approach (CPIA) flips the positioning, with the midline positioned horizontally for retraction due to gravity, but with the AVM on the upper side and the approach from the contralateral, lower side. The aim of this paper was to analyze whether the perpendicular angle of attack that is used in IPIA would convert to a parallel angle of attack with the CPIA, with less retraction, improved working angles, and no significant increase in risk.

METHODS A retrospective review of pre- and postoperative clinical and radiographic data was performed in 8 patients who underwent a CPIA.

RESULTS Three AVMs and 5 CCMs were resected using the CPIA, with an average nidus size of 2.3 cm and CCM diameter of 1.7 cm. All lesions were resected completely, as confirmed on postoperative catheter angiography or MRI. All patients had good neurological outcomes, with either stable or improved modified Rankin Scale scores at last follow-up.

CONCLUSIONS The CPIA is a safe alternative approach to the IPIA for deep medial parietooccipital vascular malformations that extend 2 cm or more off the midline. Contralaterality and retraction due to gravity optimize the interhemispheric corridor, the surgical trajectory to the lesion, and the visualization of the lateral margin, without resection or retraction of adjacent normal cortex. Although the falx is a physical barrier to accessing the lesion, it stabilizes the ipsilateral hemisphere while gravity delivers the dissected lesion through the transfalcine window. Patient positioning, CSF drainage, venous preservation, and meticulous dissection of the deep margins are critical to the safety of this approach.

Posted on

Anterior subtemporal approach for posterolateral brainstem cavernomas

Acta Neurochir (2012) 154:2009–2016

The neuronavigation-assisted anterior subtemporal approach is proposed in this article as an alternative to surgery of posterolateral brainstem cavernomas. Brainstem cavernomas represent a neurosurgical challenge because of the high morbidity and mortality rate related to their surgical removal. Several nerve nuclei, ascending and descending fibers make this region at high risk of serious postoperative deficits.

Methods Between 1998 and 2010, 24 patients underwent surgical removal of brainstem cavernomas in our institution. Ten of these patients presented a cavernous malformation in the posterolateral region of the brainstem and underwent surgical removal by means of a neuronavigation-assisted anterior subtemporal approach.

Results Lesion removal was complete for all patients. There were no cases of surgery-related death. Neurological status improved or remained unchanged after surgery in all cases. All patients presented good outcomes at 12 to 154 months’ follow-up (mean 70 months; GOS05 in 8/10 patients, 4 in 2/10 patients; mRS00–1 in all patients). Only one patient presented transient confusion, aphasia and seizures related to temporal lobe swelling, which resolved completely within a few days. One patient developed cranial nerve III palsy and left hemiparesis with gradual recovery.

Conclusions This approach represents a valid alternative to the “more classical” approaches for the surgery of posterolateral cavernomas of the pontomesenchephalic junction reaching the tentorial incisura, reducing the risk of damaging the vein of Labbé, temporal lobe swelling, cerebellar swelling, ophtalmoparesis, fourth ventricle cranial nerve nuclei lesions. Skeletonization of sigmoidal sinus provides with good outcomes, low morbidity and mortality.

Posted on

An analysis of flow dynamics in cerebral cavernous malformation and orbital cavernous angioma using indocyanine green videoangiography

Acta Neurochir (2012) 154:1169–1175 DOI 10.1007/s00701-012-1354-9

Cerebral cavernous malformations (CCMs) are known to be vascular anomalies with low perfusion because of being angiographically occult. We attempted direct visualization of blood flow within CCMs and orbital cavernous angiomas (CAs), and analyzed flow dynamics using indocyanine green videoangiography (ICG-VAG).

Methods This series included seven CCMs and two orbital CAs. ICG-VAG was performed to visualize blood flow of the lesions before resection. Time to peak of staining was evaluated by reviewing recorded ICG-VAG.

Results In five of seven CCMs, stain was identified. CCMs were seen as avascular areas in both arterial and venous phases, and were stained gradually. Stain was maximized late after venous phase. The orbital CAs were also stained lately, but more intensely than CCMs.

Conclusions The present study directly demonstrated slow and low perfusion within CCM and orbital CA using ICGVAG. On the basis of characteristic flow dynamics of CCMs, intraoperative ICG-VAG provides useful information in microsurgical resection.

Proudly powered by WordPress