The natural history of intracranial cavernous malformations

Gross, Bradley A.; Lin, Ning; Du, Rose; Day, Arthur L.

doi:10.3171/2011.3.focus1165

Cited by 271 publications

(262 citation statements)

References 31 publications

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“…13,70 Among the general population, the reported prevalence of CNS cavernous malformations is 0.4%-0.6%. 16,25,31,48,52,56 These lesions most often occupy the intracranial (mainly supratentorial) compartment. 10,16,25,33,52,66 By contrast, spinal cord cavernous malformations are very rare entities, accounting for 5%-12% of intraspinal vascular malformations.…”

mentioning

confidence: 99%

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

Badhiwala¹,

Farrokhyar

Alhazzani

et al. 2014

SPI

115

118

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Object Information pertaining to the natural history of intramedullary spinal cord cavernous malformations (ISCCMs) and patient outcomes after surgery is scarce. To evaluate factors associated with favorable outcomes for patients with surgically and conservatively managed ISCCMs, the authors performed a systematic review and metaanalysis of the literature. In addition, they included their single-center series of ISCCMs. Methods The authors searched MEDLINE, EMBASE, CINAHL, Google Scholar, and The Cochrane Library for studies published through June 2013 that reported cases of ISCCMs. Data from all eligible studies were used to examine the epidemiology, clinical features, and neurological outcomes of patients with surgically managed and conservatively treated ISCCMs. To evaluate several variables as predictors of favorable neurological outcomes, the authors conducted a meta-analysis of individual patient data and performed univariate and multivariate logistic regression analyses. Variables included patient age, patient sex, lesion spinal level, lesion size, cerebral cavernomas, family history of cavernous malformations, clinical course, presenting symptoms, treatment strategy (operative or conservative), symptom duration, surgical approach, spinal location, and extent of resection. In addition, they performed a meta-analysis to determine a pooled estimate of the annual hemorrhage rate of ISCCMs. Results Eligibility criteria were met by 40 studies, totaling 632 patients, including the authors' institutional series of 24 patients. Mean patient age was 39.1 years (range 2–80 years), and the male-to-female ratio was 1.1:1. Spinal levels of cavernomas were cervical (38%), cervicothoracic (2.4%), thoracic (55.2%), thoracolumbar (0.6%), lumbar (2.1%), and conus medullaris (1.7%). Average cavernoma size was 9.2 mm. Associated cerebral cavernomas occurred in 16.5% of patients, and a family history of cavernous malformation was found for 11.9% of evaluated patients. Clinical course was acute with stepwise progression for 45.4% of patients and slowly progressive for 54.6%. Symptoms were motor (60.5%), sensory (57.8%), pain (33.8%), bladder and/or bowel (23.6%), respiratory distress (0.5%), or absent (asymptomatic; 0.9%). The calculated pooled annual rate of hemorrhage was 2.1% (95% CI 1.3%–3.3%). Most (89.9%) patients underwent resection, and 10.1% underwent conservative management (observation). Outcomes were better for those who underwent resection than for those who underwent conservative management (OR 2.79, 95% CI 1.46–5.33, p = 0.002). A positive correlation with improved neurological outcomes was found for resection within 3 months of symptom onset (OR 2.11, 95% CI 1.31–3.41, p = 0.002), hemilaminectomy approach (OR 3.20, 95% CI 1.16–8.86, p = 0.03), and gross-total resection (OR 3.61, 95% CI 1.24–10.52, p = 0.02). Better outcomes were predicted by an acute clinical course (OR 1.72, 95% CI 1.10–2.68, p = 0.02) and motor symptoms (OR 1.76, 95% CI 1.08–2.86, p = 0.02); poor neurological recovery was predicted by sensory symptoms (OR 0.58, 95% CI 0.35–0.98, p = 0.04). Rates of neurological improvement after resection were no higher for patients with superficial ISCCMs than for those with deep-seated ISCCMs (OR 1.36, 95% CI 0.71–2.60, p = 0.36). Conclusions Intramedullary spinal cord cavernous malformations tend to be clinically progressive. The authors' findings support an operative management plan for patients with a symptomatic ISCCM. Surgical goals include gross-total resection through a more minimally invasive hemilaminectomy approach within 3 months of presentation.

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confidence: 99%

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

Badhiwala¹,

Farrokhyar

Alhazzani

et al. 2014

SPI

115

118

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“…The frequently observed coexistence of CM indicates the existence of a form occurring between the stages of capillary and venous malformation. 8,11,12 This entity should be managed in time to avoid severe sequelae as reported in previous studies.…”

Section: Discussionmentioning

confidence: 99%

“…12 The unique characteristics of the giant BCTs in our series add more support to this theory. 6,8,11 Small BCT and CM might be the two ends of the same pathological entity, with giant or large BCT as the bridging developmental stage. Moreover, it has been suggested that either BCT or CM could develop as a consequence of dynamic change in the blood flow and then develop into the other type of lesion (i.e., CM or BCT).…”

Section: Diagnosis Of Bctsmentioning

confidence: 99%

Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases

Yu¹,

Sun²,

You

et al. 2016

JNS

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B rain capillary telangiectasia (BCT) is a rare vascular malformation with a prevalence of 0.4%-0.7% based on autopsy and MRI diagnosis. 12 Most BCTs are small in size and clinically silent with a predilection of pons and basal ganglia. Clinical data of large and symptomatic BCTs are rare. Only 10 symptomatic cases with 3 diffuse BCTs have been reported in the English-language literature of more than 200 BCTs. 12,20,24,27,29 Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological deficits and even death if left untreated. 2,12,13,21,22 Therefore, large BCTs should be managed with special caution. Due to their rarity, the diagnosis of large BCTs has been difficult. 29,30 Large BCTs have different radiological characteristics, histopathological characteristics, and clinical courses from small BCTs. 8,12,25 Standard management is undefined for large or giant BCTs. 7,12,20,24,27,29 To our knowledge, the current study presents the largest case series of large BCTs ever reported. We analyzed the radiological findings, histopathological characteristics, clinical presentation, and management in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8-8 cm). Coexisting cavernous malformations (CMs) were confirmed in 3 cases. Resections were performed in all patients to control seizures and other recurrent neurological events.abbreviatioNs BCT = brain capillary telangiectasia; CM = cavernous malformation; DSA = digital subtraction angiography; ECoG = electrocorticography; EEG = electroencephalography. Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left untreated. Therefore, large BCTs should be managed with special caution. Because of the lack of reports, diagnosis of large BCTs has been difficult. Strategies of management are undefined for large or giant BCTs. The current study presents 5 cases of giant and large BCTs. To the authors' knowledge, this is the largest series of this disease ever reported. Radiological findings, histopathological characteristics, clinical presentations, and surgical management were analyzed in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8-8 cm). Four patients presented with focal or generalized seizures, and 1 patient presented with transient vision loss attributed to the lesions. Gross-total resection of the lesion was achieved in all patients. After surgery, the 4 patients with seizures were symptom free for follow-up periods varying from more than 1 to 5 years with no additional neurological deficits. The unique location, radiological characteristics, and clinical course suggest that giant BCTs could be a different entity from small BCTs. Surgery might be a good option for treatment of patients with intractable neurological symptoms, especially in those with surgically acce...

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“…Cavernous malformations are common cerebrovascular abnormalities, affecting 0.4-0.8% of the general population 13,14,15 . Pathologically, CCM are defined by clusters of dilated capillary cavities, in back-to-back disposition, lined by a single layer of endothelium, lacking smooth muscle and no intervening brain parenchyma 16 ( Figure 1).…”

Section: Familial Cerebral Cavernous Malformations (Fccm)mentioning

confidence: 99%

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management

Gasparetto

Alves-Leon

Domingues

et al. 2016

Arq. Neuro-Psiquiatr.

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Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.

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The natural history of intracranial cavernous malformations

Cited by 271 publications

References 31 publications

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases

Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management

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