Tag: Cavernoma

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Which Cerebral Cavernous Malformations are Most Difficult to Dissect From Surrounding Eloquent Brain Tissue?

Neurosurgery 81:498–503, 2017

Cerebral cavernous malformations (CCM) may lead to repetitive intracerebral hemorrhage. In selected cases, a surgical resection is indicated.

OBJECTIVE: To identify magnetic resonance imaging (MRI) features of CCM that correlate with the difficulty of dissection and postoperative outcome.

METHODS: This study prospectively analyzed pre- and postoperative MRI features, intraoperative findings (surgical questionnaire), and postoperative outcome of 41 patients with eloquent CCM. Based on the results of the surgeon’s questionnaire and postoperative MRI findings, all surgical procedureswere dichotomized in a “difficult”(groupA) or “not difficult” (group B) lesion dissection. Based on the correlation of preoperative MRI features with groups A and B, a 3-tiered classification was established and tested for sensitivity and specificity.

RESULTS: In 22 patients, dissection of the lesion was rated difficult. This was significantly correlated with amount of postoperative diffusion restriction on MRI (P=.001) and postoperative outcome(P=.05). Various preoperative MRI featureswere tested for correlation and combined in a 3-tiered classification. Receiver operating characteristics revealed excellent and good results for predicting difficulty of dissection for the different classification types.

CONCLUSION: We provide a meticulous analysis and new classification of preoperative MRI features that seem to be involved in the microsurgical resection of CCM.

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Hemorrhage from cerebral cavernous malformations

J Neurosurg 126:1079–1087, 2017

The aim of this paper is to define an overall cavernous malformation (CM) hemorrhage rate and risk factors for hemorrhage.

METHODS The authors performed a systematic, pooled analysis via the PubMed database through October 2015 using the terms “cavernoma,” “cavernous malformation,” “natural history,” “bleeding,” and “hemorrhage.” English-language studies providing annual rates and/or risk factors for CM hemorrhage were included. Data extraction, performed independently by the authors, included demographic data, hemorrhage rates, and hemorrhage risk factors.

RESULTS Across 12 natural history studies with 1610 patients, the mean age at presentation was 42.7 years old and 52% of patients (95% CI 49%–55%) were female. Presentation modality was seizure in 30% (95% CI 25%–35%), hemorrhage in 26% (95% CI 17%–37%), incidental in 17% (95% CI 9%–31%), and focal deficits only in 16% of cases (95% CI 11%–23%). CM location was lobar in 66% (95% CI 61%–70%), brainstem in 18% (95% CI 13%–24%), deep supratentorial in 8% (95% CI 6%–10%), and cerebellar in 8% (95% CI 5%–11%). Pooling 7 studies that did not assume CM presence since birth, the annual hemorrhage rate was 2.5% per patient-year over 5081.2 patient-years of follow-up (95% CI 1.3%–5.1%). Pooling hazard ratios across 5 studies that evaluated hemorrhage risk factors, prior CM hemorrhage was a significant risk factor for hemorrhage (HR 3.73, 95% CI 1.26–11.1; p = 0.02) while younger age, female sex, deep location, size, multiplicity, and associated developmental venous anomalies (DVAs) were not.

CONCLUSIONS Although limited by the heterogeneity of incorporated reports and selection bias, this study found prior hemorrhage to be a significant risk factor for CM bleeding, while age, sex, CM location, size, multiplicity, and associated DVAs were not. Future natural history studies should compound annual hemorrhage rate with prospective seizure and nonhemorrhagic neurological deficit rates.

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Transulcal parafascicular minimally invasive approach to deep and subcortical cavernomas

J Neurosurg 125:1360–1366, 2016

Cavernomas comprise 8%–15% of intracranial vascular lesions, usually supratentorial in location and superficial. Cavernomas in the thalamus or subcortical white matter represent a unique challenge for surgeons in trying to identify and then use a safe corridor to access and resect the pathology.

Previous authors have described specific open microsurgical corridors based on pathology location, often with technical difficulty and morbidity.

This series presents 2 cavernomas that were resected using a minimally invasive approach that is less technically demanding and has a good safety profile. The authors report 2 cases of cavernoma: one in the thalamus and brainstem with multiple hemorrhages and the other in eloquent subcortical white matter. These lesions were resected through a transulcal parafascicular approach with a port-based minimally invasive technique.

In this series there was complete resection with no neurological complications. The transulcal parafascicular minimally invasive approach relies on image interpretation and trajectory planning, intraoperative navigation, cortical cannulation and subcortical space access, high-quality optics, and resection as key elements to minimize exposure and retraction and maximize tissue preservation. The authors applied this technique to 2 patients with cavernomas in eloquent locations with excellent outcomes.

 

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Brainstem Cavernous Malformations: Surgical Results in 104 Patients and a Proposed Grading System to Predict Neurological Outcomes

Brainstem Cavernous Malformations- Surgical Results in 104 Patients and a Proposed Grading System to Predict Neurological Outcomes

Neurosurgery 76:265–278, 2015

Once considered inoperable lesions in inviolable territory, brainstem cavernous malformations (BSCM) are now surgically curable with acceptable operative morbidity. Recommending surgery is a difficult decision that would be facilitated by a grading system designed specifically for BSCMs that predicted surgical outcomes.

OBJECTIVE: Informed by our efforts to develop a supplementary grading system for arteriovenous malformations, we hypothesized that a similar system might predict long-term outcomes and guide clinical decision-making.

METHODS: A consecutive, single-surgeon series of 104 patients was used to assess preoperative clinical and imaging predictors of microsurgical outcomes. Univariable logistic regression identified predictors and a multivariable logistic regression model tested the association of the combined predictors with final modified Rankin Scale scores. A grading system assigned points for lesion size, location crossing the brainstem’s midpoint, presence of developmental venous anomaly, age, and time from last hemorrhage to surgery.

RESULTS: Average maximal diameter of BSCMs was 19.5 mm; 50% crossed the axial midpoint; 54.8% had developmental venous anomalies; mean age was 42.1 years; and median time from last hemorrhage to surgery was 60 days. One patient died (0.96%), and 15 patients (14.4%) experienced worsened cranial nerve or motor dysfunction, of which 10 increased their modified Rankin Scale scores (9.6%). BSCM grades ranged from 0 to 7 points and predicted outcomes with high accuracy (receiver operating characteristic = 0.86, 95% confidence interval: 0.78-0.94).

CONCLUSION: Rather than developing a grading system for all cerebral cavernous malformations that is weak with BSCMs, we propose a system for the patients who need it most. The BSCM grading system differentiates patients who might expect favorable surgical outcomes and offers guidance to neurosurgeons forced to select these patients.

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De Novo Formation of a Cavernoma in a Thrombosed Developmental Venous Anomaly

Magnetic Resonance Evolution of De Novo Formation of a Cavernoma in a Thrombosed Developmental Venous Anomaly

Neurosurgery 73:E739–E745, 2013

Mechanisms that lead to de novo formations of nonfamilial-type cavernomas are not well understood. One of the interesting hypotheses is the causative relationship between developmental venous anomaly (DVA) and cavernoma formation. We report a unique case in which serial imaging demonstrated the evolution of de novo formation of a cavernoma in association with a thrombosed DVA. A detailed review of the causal hypothesis between a DVA and cavernoma is also provided.

CLINICAL PRESENTATION: We report a 37-year-old female patient in whom a cavernoma-like lesion arose 1 year after the progressive thrombosis of a medullary (or caput medusa) vein of a DVA. The presence of an acute angulation in the draining vein may have prompted an intrinsic outflow restriction. Possible worsening of venous disequilibrium led to subsequent thrombus progression, venous congestion, and occlusion of the vein with venous dilation and signs of stasis on follow-up magnetic resonance imaging. Finally, this developed into a lobulated lesion with salt-and-pepper appearance at the converging region of medullary tributaries, which typified the classic features of a cavernoma.

CONCLUSION: Compared with other published cases of de novo cavernoma formation in relation to a DVA, our case, for the first time, allows us to witness the temporal evolution from a thrombosed DVA to the birth of a cavernoma around it. This supports the hypothesis that the cavernoma can be an acquired disease that arises from a DVA.

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High-Definition Fiber Tractography of the Human Brain: Neuroanatomical Validation and Neurosurgical Applications

Neurosurgery 71:430–453, 2012

High-definition fiber tracking (HDFT) is a novel combination of processing, reconstruction, and tractography methods that can track white matter fibers from cortex, through complex fiber crossings, to cortical and subcortical targets with subvoxel resolution.

OBJECTIVE: To perform neuroanatomical validation of HDFT and to investigate its neurosurgical applications.

METHODS: Six neurologically healthy adults and 36 patients with brain lesions were studied. Diffusion spectrum imaging data were reconstructed with a Generalized Q-Ball Imaging approach. Fiber dissection studies were performed in 20 human brains, and selected dissection results were compared with tractography.

RESULTS: HDFT provides accurate replication of known neuroanatomical features such as the gyral and sulcal folding patterns, the characteristic shape of the claustrum, the segmentation of the thalamic nuclei, the decussation of the superior cerebellar peduncle, the multiple fiber crossing at the centrum semiovale, the complex angulation of the optic radiations, the terminal arborization of the arcuate tract, and the cortical segmentation of the dorsal Broca area. From a clinical perspective, we show that HDFT provides accurate structural connectivity studies in patients with intracerebral lesions, allowing qualitative and quantitative white matter damage assessment, aiding in understanding lesional patterns of white matter structural injury, and facilitating innovative neurosurgical applications. High-grade gliomas produce significant disruption of fibers, and low-grade gliomas cause fiber displacement. Cavernomas cause both displacement and disruption of fibers.

CONCLUSION: Our HDFT approach provides an accurate reconstruction of white matter fiber tracts with unprecedented detail in both the normal and pathological human brain. Further studies to validate the clinical findings are needed.

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

J Neurosurg 117:295–301, 2012

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.

 

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Petrosal approaches to brainstem cavernous malformations

Neurosurg Focus 33 (2):E10, 2012

Although they provide excellent ventral and lateral exposure of the brainstem, petrosal approaches to brainstem cavernous malformations (CMs) are infrequently reported.

Methods. The authors reviewed their experience with petrosal approaches to brainstem CMs in combination with a comprehensive review of the literature to elucidate resection rates, complication rates, and outcomes.

Results. Including their own results, the authors found 65 cases in 20 reports of brainstem CMs treated with petrosal approaches. The specific approaches were posterior petrosal in 37 cases (57%), anterior petrosal in 17 (26%), extended posterior petrosal in 10 (15%), and a combined petrosal approach in 1 case (2%). For 50 cases in 16 reports with detailed outcome information, the overall complete resection rate was 90%, with early postoperative morbidity reported in 30% of cases and permanent morbidity in 14%. The rate of CSF leakage was 6%.

Conclusions. The versatile petrosal approaches to brainstem CMs are associated with good outcomes and an acceptable morbidity rate. More expansive lesions can be approached using a combination of the standard anterior and posterior petrosal approach, preserving hearing and avoiding the greater complication rates associated with extended posterior petrosal approaches.

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Predictors of seizure freedom in the surgical treatment of supratentorial cavernous malformations

J Neurosurg 115:1169–1174, 2011. DOI: 10.3171/2011.7.JNS11536

Seizures are the most common presenting symptom of supratentorial cerebral cavernous malformations (CCMs) and progress to medically refractory epilepsy in 40% of patients. Predictors of seizure freedom in the resection of CCMs are incompletely understood.

Methods. The authors systematically reviewed the published literature on seizure freedom following the resection of supratentorial CCMs in patients presenting with seizures. Seizure outcomes were stratified across 12 potential prognostic variables. A total of 1226 patients with supratentorial CCMs causing seizures were identified across 31 predominantly retrospective studies; 361 patients had medically refractory epilepsy.

Results. Seventy-five percent of the patients were seizure free after microsurgical lesion removal, whereas 25% continued to have seizures. All patients had had preoperative seizures and > 6 months of postoperative follow-up. Modifiable predictors of postoperative seizure freedom included gross-total resection (OR 36.6, 95% CI 8.5–157.5) and surgery within 1 year of symptom onset (OR 1.83, 95% CI 1.30–2.58). Additional prognostic indicators of a favorable outcome were a CCM size < 1.5 cm (OR 15.4, 95% CI 5.2–45.4), the absence of multiple CCMs (OR 2.02, 95% CI 1.13–3.60), medically controlled seizures (OR 2.38, 95% CI 1.29–4.39), and the lack of secondarily generalized seizures (OR 3.33, 95% CI 2.09–5.30). Other factors, including extended resection of the hemosiderin ring, were not significantly predictive.

Conclusions. In the surgical treatment of supratentorial CCMs, gross-total resection and early operative intervention may improve seizure outcome. While surgery should not be considered the first-line treatment for CCM-related epilepsy, it is important to understand the variables associated with seizure freedom in CCM resection given the considerable morbidity and diminished quality of life associated with epilepsy.

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A Proposed Grading System of Brain and Spinal Cavernomas

Neurosurgery 69:807–814, 2011 DOI: 10.1227/NEU.0b013e31821ffbb5
Most cavernomas in the central nervous system are characterized by a benign natural course. Progressive symptoms warrant surgical removal. In the literature, the factors affecting long-term postoperative outcome are not statistically well confirmed.
OBJECTIVE: To perform a multifactorial analysis of risk factors on a large patient series and to use the results to propose a simple grading scale to predict outcome.
METHODS: We studied 303 consecutive patients with cavernomas treated surgically at our department from 1980 to 2009. Follow-up assessment was performed on average 5.7 years postoperatively (range, 0.2-36 years). The main outcome measure was the patients’ condition at the last follow-up on Glasgow Outcome Scale. For statistical analysis, the outcome measure was dichotomized to favorable (Glasgow Outcome Scale 5) and unfavorable (Glasgow Outcome Scale 1-4). Binary logistic regression analysis was used to estimate the effect of age, sex, seizures, preexisting neurological deficits, hemorrhage, and size and location of cavernoma on long-term outcome.
RESULTS: Infratentorial, basal ganglia, or spinal location and preexisting neurological deficit were the only independent risk factors for unfavorable outcome, with relative risks of 2.7 (P = .008) and 3.2 (P = .002), respectively. We formulated a grading system based on a score of 1 to 3. When applied to our series, the proposed grading system strongly correlated with outcome (P < .001, Pearson x2 test). The risk for long-term unfavorable outcome was 13%, 22%, and 55% for grades 1 through 3, respectively.
CONCLUSION: The proposed grading system showed a convincing correlation with postoperative outcome in surgically treated cavernoma patients.
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The natural history of intracranial cavernous malformations

Neurosurg Focus 30 (6):E24, 2011, DOI: 10.3171/2011.3.FOCUS1165

Literature reports on the natural history of cerebral cavernous malformations (CMs) are numerous, with considerable variability in lesion epidemiology, hemorrhage rates, and risk factors for hemorrhage.

In this review, the authors performed a meta-analysis of 11 natural history studies. The overall male-to-female ratio was 1:1, and the mean age at presentation was 30.6 years. Overall, 37% of patients presented with seizures, 36% with hemorrhage, 23% with headaches, 22% with focal neurological deficits, and 10% were asymptomatic. Some patients had more than one symptom. Seizure presentation was most prevalent among supratentorial CMs, while focal neurological deficits were common in patients with infratentorial CMs. By location, CMs were in the cerebral hemispheres (66%), brainstem (18%), basal ganglia or thalamus (8%), cerebellum (6%), and other (2.5% [combined supra- and infratentorial, callosal or insular]). Overall, 19% of patients harbored multiple intracranial CMs, and 9% had radiographically apparent associated developmental venous anomalies. An overall annual hemorrhage rate of 2.4% per patient-year (range 1.6%–3.1%) was identified across 3 studies.

Prior hemorrhage and female sex were risk factors for bleeding, while CM size and multiplicity did not affect hemorrhage rates. Although not impacting the hemorrhage rate itself, deep location was a risk factor for increased clinical aggressiveness.

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Advances in the Treatment and Outcome of Brainstem Cavernous Malformation Surgery: A Single-Center Case Series of 300 Surgically Treated Patients

Neurosurgery 68:403–415, 2011 DOI: 10.1227/NEU.0b013e3181ff9cde

Brainstem cavernous malformations (BSCMs) are relatively uncommon, low-flow vascular lesions. Because of their relative rarity, relatively little data on their natural history and on the efficacy and durability of their treatment.

OBJECTIVE: To evaluate the long-term durability of surgical treatment of BSCMs and to document patient outcomes and clinical complications.

METHODS: The charts of all patients undergoing surgical treatment of BSCM between 1985 and 2009 were reviewed retrospectively. The study population consisted of 300 patients who had surgery for BSCM. Forty patients were under 19 years of age at surgery; pediatric BSCMs have been reported separately. Patient demographics, lesion characteristics, surgical approaches, and patient outcomes were examined.

RESULTS: The study population consisted of 260 adult patients with a female-to-male ratio of 1.5 and mean age of 41.8 years. Of the 260 patients, 252 presented with a clinical or radiographic history of hemorrhage. The mean follow-up in 240 patients was 51 months. The mean Glasgow Outcome Scale on admission, at discharge, and at last follow-up was 4.4, 4.2, and 4.6. Postoperatively, 137 patients (53%) developed new or worsening neurological symptoms. Permanent new deficits remained in 93 patients 3(36%). There were perioperative complications in 74 patients (28%); tracheostomy, feeding tube placement, and cerebrospinal fluid leakage were most common. Eighteen patients (6.9%) experienced 20 rehemorrhages. Twelve patients required reoperation for residual/recurrent BSCM. The overall annual risk of postoperative rehemorrhage was 2%/patient.

CONCLUSION: Although BSCM surgery has significant associated risks, including perioperative complications, new neurological deficits, and death, most patients have favorable outcomes. Overall, surgery markedly improved the risk of rehemorrhage and related symptoms and should be considered in patients with accessible lesions.

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Epileptogenicity of Cavernomas Depends on (Archi-) Cortical Localization

Neurosurgery 67:918–924, 2010 DOI: 10.1227/NEU.0b013e3181eb5032

Patients with cerebral cavernomas have an estimated risk of the development of epilepsy of 1.5% to 2.4% per patient-year.

OBJECTIVE: To clarify the predictive value of different risk factors for epilepsy in patients with supratentorial cavernomas.

METHODS: We retrospectively analyzed data of 109 patients with supratentorial cavernomas. The correlation of epilepsy with the variables of single or multiple cavernomas, sex, age, side, cortical involvement, mesiotemporal archicortical vs neocortical involvement, lobar location of neocortical cavernomas, the presence of a hemosiderin rim and of edema, and the maximal diameters of cavernoma, hemosiderin rim, and edema, if present, were calculated using univariate and multivariate penalized likelihood logistic regression models.

RESULTS: Cortical involvement was the most relevant risk factor for epilepsy (P , .0001). No patient with a subcortical cavernoma presented with epilepsy. Epilepsy was more common in patients with mesiotemporal archicortical cavernomas than in patients with neocortical cavernomas (P = .02), whereas the lobar location of neocortical cavernomas was not significantly associated with the risk of the development of epilepsy. In the multivariate analysis, a greater diameter of the cavernoma, the absence of edema, and localization in the left hemisphere were also associated with the occurrence of epilepsy (P , .05).

CONCLUSION: The epileptogenicity of supratentorial cavernomas depends on cortical, especially mesiotemporal archicortical, involvement. Exclusively subcortical cavernomas are highly unlikely to cause epilepsy. This information is helpful in counseling patients with cavernomas regarding their risk of epileptic seizures and in patients with multiple cavernomas and epilepsy to generate a valid hypothesis of which cavernoma may cause epilepsy.

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