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Cavernous angioma


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Cavernous angioma

Incidence of Occurrence and Symptoms

Cavernous Angioma, also known as cerebral cavernous malformation (CCM), cavernous haemangioma, and cavernoma, is a vascular disorder of the central nervous system that may appear either sporadically or exhibit autosomal dominant inheritance. The incidence in the general population is between 0.1-0.5%, and clinical symptoms typically appear between 30 to 50 years of age. Once thought to be strictly congenital, these vascular lesions have been found to occur de novo.

This disease is characterized by grossly dilated blood vessels with a single layer of endothelium and an absence of neuronal tissue within the lesions. These thinly-walled vessels resemble sinusoidal cavities filled with stagnant blood. Blood vessels in patients with CCM can range from a few millimeters to several centimeters in diameter. CCM lesions commonly resemble raspberries in external structure.

Many patients live their whole life without knowing they have a cerebral cavernous malformation. Other patients can have severe symptoms like seizures, headaches, paralysis, bleeding in the brain (cerebral hemorrhage), and even death. The nature and severity of the symptoms depend on the lesion's location in the brain. Approximately 70% of these lesions occur in the supratentorial region of the brain; the remaining 30% occur in the infratentorial region.

Symptoms and Diagnosis

Clinical symptoms of this disease include recurrent headaches, focal neurological deficits, hemorrahagic stroke, and seizures, but CCM can also be asymptomatic. Diagnosis is most commonly made by magnetic resonance imaging MRI, but not all MRI exams are created equal. It's paramount that the patient request a gradient-echo MRI (aka T2-Flair) in order to unmask small or punctate lesions which may otherwise remain undetected. Sometimes quiescent CCMs can be revealed as incidental findings during MRI exams ordered for other reasons.

Sometimes the lesion appearance imaged by MRI remains inconclusive. Consequently neurosurgeons will order a cerebral angiogram or magnetic resonance angiogram (MRA). Since CCMs are low flow lesions (they are hooked into the venous side of the circulatory system), they will be angiographically occult (invisible). If a lesion is discernable via angiogram in the same location as in the MRI, then an arteriovenous malformation (AVM) becomes the primary concern.

CCMs & Venous Angiomas

Not infrequently a CCM is accompanied by a venous angioma, also known as a developmental venous anomaly (DVA). These lesions appear either as enhancing linear blood vessels or caput medusae--a radial orientation of small vessels that resemble the hair of Medusa from Greek Mythology. These lesions are thought to represent developmental anomalies of normal venous drainage. These lesions should not be removed, as reports of venous infarcts have been reported. When found in association with a CCM that needs resection, great care should be taken not to disrupt the angioma.


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Predictive factors for intracerebral hemorrhage in patients with cavernous angiomas
From Neurological Research, 4/1/05 by Cantu, Carlos

Objectives: Prediction of intracerebral hemorrhage (ICH) in patients with cavernous angiomas is not totally elucidated. The aims of our study were to determine the rate of cerebral hemorrhage, its associated factors, and the clinical outcome in patients with cavernous angiomas in a Hispanic population.

Methods: We studied 133 patients with cavernous angiomas. The patients were classified into two groups depending on whether they presented an ICH. A comparative analysis of demographics and clinical data, neuroimaging characteristics, and prognosis was carried out in patients with and without hemorrhage. The hemorrhage rate (expressed as the percentage per patient per year) was also estimated.

Results: Seventy-eight patients (59%) had hemorrhage. Non-lobar location of angiomas was associated with hemorrhage [OR 4.82 (CI 95% 2.17-10.73; p=

Conclusions: The non-lobar location of cavernous angiomas gives a higher risk of hemorrhage in our Mexican mestizo population, without the hemorrhage being related to either age or sex. [Neurol Res 2005; 27: 314-318]

Keywords: Cavernous angiomas; intracerebral hemorrhage; cerebrovascular malformations


Cerebral cavernous angiomas (CCA) represent 9% of all the types of brain vascular malformations. The prevalence is 0.4-0.8% of the general population1,2, although little is known about CCA in Hispanic populations. CCA consist of sinusoidal vascular channels lined by a single layer of endothelium without a complete vascular wall3. Magnetic resonance imaging (MRI) shows reticular lesions with a hypointense halo, and can be associated with acute bleeding, which exhibits a hyperintense signal or chronic bleeding, which exhibits a hypointense signal4-7.

The male to female ratio is 1:1 occurring mainly between 20 and 40 years of age8-10. Clinical presentation includes chronic headache, focal neurological signs, seizures, or cerebral hemorrhage3,8-11. Some factors have been associated with a risk of hemorrhage including age, female sex, and infratentorial localization of the CCA. However, predictive factors of hemorrhage in the CCA are not totally elucidated10,12-19. Another factor that is difficult to determine is the hemorrhage rate in patients with CCA, due to the different methods used in the literature. It has been reported to be between 0.25 and 6.5% per patient per year in prospective analysis3,8-11,20. Assuming that CCA are congenital lesions, a retrospective hemorrhage rate has been estimated as from 0.5 to 2.3% per patient per year8,9,20.

The objectives of our study were: (1) to determine the rate of intracerebral hemorrhage (ICH) in Hispanic patients with CCA, (2) to establish the functional prognosis of ICH in this group of patients, and (3) to determine the factors associated with the development of ICH.


Since 1988 all patients that have had a stroke are prospectively evaluated in our neurologic referral center. The information from every patient is coded and included in a computerized database. This information includes demographic data, vascular risk factors, diagnostic testing results, the description of the mechanisms and causes of the stroke, and the clinical outcome. From the stroke records available at the National Institute of Neurology and Neurosurgery, we identified 145 patients with CCA. We excluded 12 patients, eight because of lack of follow-up and four because an MRI study was not available. All 133 patients with MRI evidence of CCA were included in the study. Patients had MRI scans performed with 0.5-Tesla magnets and spin echo T1-weighted images and T2-weighted images were reviewed. Additionally, 108 (81.2%) had a cerebral CT scan, 63 (47.4%) had a digital subtraction cerebral angiography, and 21 (15.8%) had surgery for resection of the angioma with pathologic confirmation of the diagnosis.

From each patient the following data was also obtained: age at diagnosis of CCA, family history of cerebral hemorrhage and epilepsy, clinical presentation of the CCA, and its morphologic characteristics (number, site, co-existence with venous angiomas). The localization of CCA was divided into supratentorial, infratentorial, and spinal cord locations. The prognosis was analysed after 6 months of follow-up using the Glasgow Outcome Scale, dichotomized into good prognosis (grade 5, good recovery) and bad prognosis (grades 1-4, including moderate and severe disability, persistent vegetative state, and death)21.

The patients were classified into two groups depending on whether they presented with ICH or not. Patients were considered as having ICH when they had neurological symptoms compatible with acute hemorrhage attributable to CCA that were documented with an MRI study.

The ICH rate was calculated assuming that cavernous angiomas are congenital lesions. A retrospective rate was used to determine the frequency of ICH9. Results were expressed as a percentage per patient per year considering the age of the patient at the time of the ICH. If there was no ICH, the age at last medical visit or the time of surgery of those undergoing resection of the CCA were considered to calculate the IHC rate in our population.

A comparative analysis of the variables examined was carried out between patients with and without ICH. The groups were compared using the chi-squared test for categorical variables and the Student's t-test for continuous variables. At entry, multiple logistic regression analysis was performed to determine the association of multiple risk factors (p


From 133 patients, 67 (50.4%) were male and 66 (49.6%) female, with a mean age of 34.3 ± 14.6 years. All patients were Mexican mestizos (a mixture of native American Indians and Spaniards). The average follow-up was 5 years giving a total of 4561 patients per year. In 78 patients (58.65%) we documented an ICH and the remaining 55 patients (41.35%) presented other neurological manifestations or were found incidentally.

Table 1 shows the demographic data, clinical history, and clinical manifestations for both groups. Differences in age were not observed, but there was a higher frequency of males in the ICH group (55.1 versus 43.6%) without reaching statistical significance. There was a trend for systemic arterial hypertension in the ICH group (p=0.07). We also documented a higher frequency of family history of epilepsy in the non-ICH group (27.3 versus 10.3%) with an odds ratio for ICH of 0.31 (CI 95% 0.1-0.7; p=0.01). As expected, headache and focal neurological signs were more frequent in the ICH group. The association between focal signs and ICH was strong [OR 7.56 (CI 95% 3.3-16.9; p

Regarding the morphological characteristics, most cavernous angiomas were single and the coexistence with venous angiomas was low and without influence in the development of ICH (Table 2). The risk of hemorrhage was lower in patients with CCA in the lobar localization compared with those in other sites [OR 0.20 (CI 95% 0.10-0.44; p

The multiple logistic regression model showed that the only factor associated with the development of ICH was the non-lobar localization (basal ganglia, posterior fossa, and spinal cord) of CCA [OR 4.82 (CI 95% 2.17-10.73; p=

The clinical outcome of patients with CCA was usually good with an excellent recovery in 107 patients (80.5%). There were no patients with persistent vegetative state or deaths but 26 patients (19.5%) had a moderate or severe disability. As noted in Figure 1, the prognosis was poor in 29.5% of the ICH group as compared with 5.5% of the non-ICH group [OR 7.25 (CI 95% 2.05-25.59) (p=0.001)].

The hemorrhage rate in the 133 patients with CCA was 1.71% per patient per year, and varied according to the localization of the angiomas (Table 3). We found a high frequency of ICH from the CCA in the following localizations: deep hemispheric 2.82%, cerebellum 2.39%, brainstem 2.33%, and spinal cord 2.31% per patient per year. Conversely, the hemorrhage rate per patient per year in the lobar localization was only 1.22%.


In the present series an ICH occurred in 59% of the patients with CCA in our Hispanic population. In the logistic regression model, the only factor associated with cerebral hemorrhage was the location of the lesions. The frequency of hemorrhage was higher when the location of the CCA was infratentorial (88%) and deep hemispheric (77%) than when it was lobar (39.7%). Although supratentorial CCA are more common than infratentorial ones (65-85 versus 20-35%)12,22-25, the risk of hemorrhage is higher in infratentorial cavernous angiomas as was attested in our study.

Robinson et al.10 and Moriarity et al.22 reported a link between hemorrhage and female sex whereas Kupersmith et al.23 showed a predominance in males. Conversely, our study and most of the series do not show any link between gender and ICH. It has also been reported that clinical onset with epilepsy occurs at a younger age than clinical presentation with focal neurological signs10,16,18,19. In our series there was no difference with respect to age at clinical presentation between groups with and without ICH.

The hemorrhage rate in patients with CCA has been difficult to establish. The prospective rate of hemorrhage has been estimated by Moriarity et al.22 as 3.1% per patient per year and in familial cavernous angiomas Zabramski et al.11 reported a bleeding rate of 6.5% per patient per year. A retrospective rate of hemorrhage has also been calculated assuming that CCA are congenital. Based on this, Curling et al.8 estimated a frequency of 0.25% per patient per year, Kondziolka et al.9 1.3% per patient per year, and Kim et al.20 2.3% per patient per year. Using this method we found a global rate of 1.71% per patient per year, but this result was influenced by the location of the lesions. The rate of hemorrhage was as low as 1.22% per patient per year in lobar angiomas and as high as 2.33, 2.39, and 2.82% per patient per year in patients with brainstem, cerebellum, and deep hemispheric angiomas, respectively. Using this method Fritchi et al.25 and Kondziolka et al.9 found a frequency of hemorrhage in brainstem cavernous angiomas of 2.75 and 2.4% per patient per year, respectively, which yields results that are very similar to our study. There are few reports concerning spinal cord cavernous angiomas26-31. We had five cases representing 3.8% of the total and also had a similar hemorrhagic rate for posterior fossa cavernous angiomas (2.31% per patient per year).

Multiple cavernous angiomas are reported in 18-21% of the cases13,20. Although it could be expected for patients with multiple CCA to be more prone to bleeding, in our study they were present in 13% of cases and with no increase in the risk of ICH. The coexistence of multiple vascular malformations is also important; the most common association is that with venous angiomas reported in 13-33%13,32-39. Tropper et al.14 reported this coexistence in 18% but noted that hemorrhage was linked in 41.7% of patients with this association. We found cavernous and venous angiomas coexistence in only 12 patients (9%) and ICH occurred in eight of them (66.7%) as reported also by Abdulrauf et al.32 However, the estimated risk for hemorrhage was only 1.4 (p=0.76) probably due to the low prevalence of the association or due to sample size.

An interesting finding in our study was that a family history of epilepsy occurred in 17% of our patients, suggesting familial forms of cavernous angiomas, as has been described with high frequency in the Mexican-American population40. It is worth mentioning that a family history of epilepsy was associated with a low frequency of hemorrhage, suggesting that familiar CA could have a lower tendency to bleed, although this observation needs to be corroborated in future studies.

In conclusion, the main finding in our study was that a non-lobar location of cavernous angioma gives a higher risk of hemorrhage in our Mexican mestizo population, without the hemorrhage being related to either age or sex. The rate of hemorrhage was as low as 1.22% per patient per year in lobar angiomas and as high as 2.3-2.8% per patient per year in patients with brainstem, cerebellum, deep hemispheric, and spinal cord angiomas.


1 Sarwar M, McCormick WF. Intracerebral venous angioma: Case report and review. Arch Neurol 1978; 35: 323-325

2 Otten P, Pizzolato CP, Rilliet B, et al. 131 cases of cavernous angioma (cavernotnas) of the CNS, discovered by retrospective analysis of 24 535 autopsies. Neurochirugie 1989; 35: 82-83

3 Moriarity JL, Clatterbuck RE, Rigamonti D. The natural history of cavernous malformations. Neurosurg Clin North Am 1999; 10: 411-417

4 Lee BC, Herzberg L, Zimmerman RD, et al. MRI imaging of cerebral vascular malformations. AJNR Am J Neuroradiol 1985; 6: 863-870

5 Rigamonti D, Drayer BP, Johnson PC, et al. The MRI appearance of cavernous malformations (angiomas). J Neurosurg 1987; 67: 518-524

6 Tomlinson FH, Houser OW, Scheithauer BW, et al. Angiographically occult vascular malformations: A correlative study of features on magnetic resonance imaging and histological examination. Neurosurgery 1994; 34: 792-799

7 Hallam DK, Russell EJ. Imaging of angiographically occult cerebral vascular malformations. Neuroimag Clin North Am 1998; 8: 323-347

8 Del Curling O Jr, Kelly DL Jr, Elster AD, et al. An analysis of the natural history of cavernous angiomas. J Neurosurg 1991; 75: 702-708

9 Kondziolka D, Lunsford LD, Kestle JRW. The natural history of cerebral cavernous malformation. J Neurosurg 1995; 83: 820-824

10 Robinson JR, Awad IA, Little JR. Natural history of cavernous angioma. J Neurosurg 1991; 75: 709-714

11 Zabramski JM, Wascher TM, Spetzler RF, et al. The natural history of familial cavernous malformations: Results of an ongoing study. J Neumsurg 1994; 80: 422-432

12 Barker FC, Amin-Hanjani S, Butler WE, et al. Temporal clustering of hemorrhages from untreated cavernous malformations of the central nervous system. Neurosurgery 2001; 49: 15-25

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19 Robinson J, Awad IA. Clinical spectrum and natural course. In: Awad IA, Barrow DL, eds. Cavernous Malformations. Park Ridge: AANS, 1993: pp. 25-36

20 Kim DS, Park YG, Choi JU, ef al. An analysis of the natural history of cavernous malformations. Surg Neurol 1997; 48: 9-17

21 Jannetta B, Teasdale G, Braakman R, et al. Prognosis of patients with severe head injury. Neurosurgery 1979; 4: 283-289

22 Moriarity JL, Wetzel MB, Clatterbuck RE, et al. The natural history of cavernous malformations: A prospective study of 68 patients. Neurosurgery 1 999; 44: 1166-1171

23 Kupersmith MJ, Kalish H, Epstein F, et al. Natural history of brainstem cavernous malformations. Neurosurgery 2001; 48: 47-54

24 Maraire JN, Awad IA. Intracranial cavernous malformations: Lesion behavior and management strategies. Neurosurgery 1995; 37: 591-605

25 Fritschi JA, Reulen HJ, Spetzler RF, et al. Cavernous malformations of the brain stem: A review of 139 cases. Acta Neurochir (Wein) 1994; 130: 35-46

26 Anson JA, Spetzler RF. Surgical resection of intramedullary spinal cord cavernous malformations. J Neurosurg 1993; 78: 446-451

27 Furuya K, Sasaki T, Suzuki I, et al. Intramedullary angiographically occult vascular malformations of spinl cord. Neurosurgery 1996; 39: 1123-1130

28 Harrison MJ, Eisenberg MB, Ullman JS, et al. Symptomatic cavernous malformations affecting the spine and spinal cord. Neurosurgery 1995; 37: 195-204

29 Ogilvy CS, Louis DN, Ojemann RC. Intramedullary cavernous angiomas of the spinal cord: Clinical presentation, pathological features, and surgical management. Neurosurgery 1992; 31: 219-229

30 McCormick PC, Michelsen WJ, Post KD, et al. Cavernous malformations of the spinal cord. Neurosurgery 1988; 23: 459-463

31 Cosgrove GR, Bertrand C, Fontaine S, et al. Cavernous angiomas of the spinal cord. J Neurosurg 1988; 68: 31-36

32 Abdulrauf SI, Kaynar MY, Awad IA. A comparison of the clinical profile of cavernous malformations with and without associated venous malformations. Neurosurgery 1999; 44: 41-47

33 Wilms C, Demaerel P, Robberecht W. Coincidence of developmental venous anomalies and other brain lesions: A clinical study. Eur Radiol 1995; 5: 495-500

34 Rigamonti D, Spetzler RF. The association of venous and cavernous malformations. Acta Neurochir (Wien) 1988; 92: 100-105

35 Ostertun B, Solymosi L. Magnetic resonance angiography of cerebral developmental venous anomalies: Its role in differential diagnosis. Neuroradiology 1993; 35: 97-104

36 Huber C, Henkes H, Hermes M. Regional association of developmental venous anomaly with angiographically occult vascular malformations. Neuroradiology 1996; 6: 30-37

37 Awad IA, Robinson JR Jr, Mohanty S, et al. Mixed vascular malformations of the brain: Clinical and pathogenic considerations. Neurosurgery 1993; 33: 179-188

38 Wilms G, Bleus E, Demaerel P, et al. Simultaneous occurrence of developmental venous anomalies and cavernous angiomas. AJNR Am J Neuroradiol 1994; 15: 1247-1254

39 Steno J, Bizik I, Lampert M. Concurrent cavernous and venous cerebral angiomas. Bratisl Lek Listy 1999; 100: 317-320

40 Rigamonti D, Hadley MN, Drayer BP, et al. Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med 1988; 319: 343-347

Carlos Cantu*[dagger], Luis Murillo-Bonilla*[double dagger], Antonio Arauz*, Jesús Higuera*[dagger], Joel Padilla* and Fernando Barinagarrementeria*§

* Stroke Clinic, Instituto Nacional de Neurologia y Neurocirugía 'Manuel Velasco Suarez', Insurgentes Sur 3877, Colonia La Fama, Delegación Tlalpan, Mexico, D.F. CP 14269

Present addresses: [dagger] Stroke Clinic, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico. [double dagger] Hospital Angeles del Pedregal, Mexico City, Mexico. § Hospital Angeles, Queretaro, Mexico

Correspondence and reprint requests to: Luis Murillo-Bonilla, MD, CIEVAC: Centra Integral de Enfermedad Vascular Cerebral, Hospital Angeles del Pedregal, Camino Sta. Teresa No. 1055, Consultorio 1025, Colonia Héroes de Padierna, Delegatión Tlalpan, México, D.F. 10700. lluismurillo@avantel.netl Accepted for publication August 2004

Copyright Maney Publishing Apr 2005
Provided by ProQuest Information and Learning Company. All rights Reserved

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