Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous...

9
Radiología. 2012;54(5):401---409 www.elsevier.es/rx UPDATE IN RADIOLOGY Cerebral cavernous malformations: Spectrum of neuroradiological findings J.J. Cortés Vela a,, L. Concepción Aramendía b , F. Ballenilla Marco b , J.I. Gallego León b , J. González-Spínola San Gil a a Servicio de Radiodiagnóstico, Hospital General La Mancha Centro, Alcázar de San Juan, Ciudad Real, Spain b Servicio de Radiodiagnóstico, Hospital General de Alicante, Alicante, Spain Received 7 February 2011; accepted 18 September 2011 KEYWORDS Cavernoma; Cavernous angioma; Cavernous haemangioma; Multiple cavernomatosis; Computed tomography; Magnetic resonance; Angiography Abstract Cavernous malformations (cavernomas) are hamartomatous lesions formed by sinu- soidal vascular spaces, with no cerebral parenchyma between them. Seizures are the most usual clinical presentation. They are dynamic lesions, producing changes throughout their evolution. The majority are located in the supratentorial region, but up to 20% of cases they are found in the posterior fossa. In computed tomography (CT) and in magnetic resonance (MR) their typical presentation is as a well defined round or oval lesion, with or without a minimal mass effect or oedema, with little or no contrast enhancement. Their appearance in magnetic resonance imaging (MRI) will depend on the stage of the haemorrhage, a T2 echo gradient being the most sensitive sequence. Angiography do not usually detect cavernomas. However, it may demon- strate a venous developmental anomaly. Cavernomas may present with atypical characteristics, as regards their size, appearance, location and number. © 2011 SERAM. Published by Elsevier España, S.L. All rights reserved. PALABRAS CLAVE Cavernoma; Angioma cavernoso; Hemangioma cavernoso; Cavernomatosis múltiple; Tomografía computarizada; Malformaciones cavernosas intracraneales: espectro de manifestaciones neurorradiológicas Resumen Las malformaciones cavernosas (cavernomas) son lesiones hamartomatosas for- madas por espacios vasculares sinusoidales sin parénquima cerebral entre ellos. Las crisis son su presentación clínica más habitual. Son lesiones dinámicas en las cuales se producen cambios a lo largo del tiempo. La mayoría son de localización supratentorial, pero hasta un 20% de los casos se presentan en la fosa posterior. Tanto en la tomografía computarizada como en la resonancia magnética (RM) su presentación típica es como una lesión redondeada u ovoidea, bien definida, sin o con un mínimo efecto masa o edema, y con poco o ningún realce. Su apariencia Please cite this article as: Cortés Vela JJ, et al. Malformaciones cavernosas intracraneales: espectro de manifestaciones neurorradiológicas. Radiología. 2012. doi:10.1016/j.rx.2011.09.016. Corresponding author. E-mail address: [email protected] (J.J. Cortés Vela). 2173-5107/$ see front matter © 2011 SERAM. Published by Elsevier España, S.L. All rights reserved. Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Transcript of Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous...

Page 1: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

Document down

Radiología. 2012;54(5):401---409

www.elsevier.es/rx

UPDATE IN RADIOLOGY

Cerebral cavernous malformations: Spectrum of neuroradiologicalfindings�

J.J. Cortés Velaa,∗, L. Concepción Aramendíab, F. Ballenilla Marcob, J.I. Gallego Leónb,J. González-Spínola San Gila

a Servicio de Radiodiagnóstico, Hospital General La Mancha Centro, Alcázar de San Juan, Ciudad Real, Spainb Servicio de Radiodiagnóstico, Hospital General de Alicante, Alicante, Spain

Received 7 February 2011; accepted 18 September 2011

KEYWORDSCavernoma;Cavernous angioma;Cavernoushaemangioma;Multiplecavernomatosis;Computedtomography;Magnetic resonance;Angiography

Abstract Cavernous malformations (cavernomas) are hamartomatous lesions formed by sinu-soidal vascular spaces, with no cerebral parenchyma between them. Seizures are the most usualclinical presentation. They are dynamic lesions, producing changes throughout their evolution.The majority are located in the supratentorial region, but up to 20% of cases they are found inthe posterior fossa. In computed tomography (CT) and in magnetic resonance (MR) their typicalpresentation is as a well defined round or oval lesion, with or without a minimal mass effector oedema, with little or no contrast enhancement. Their appearance in magnetic resonanceimaging (MRI) will depend on the stage of the haemorrhage, a T2 echo gradient being the mostsensitive sequence. Angiography do not usually detect cavernomas. However, it may demon-strate a venous developmental anomaly. Cavernomas may present with atypical characteristics,as regards their size, appearance, location and number.© 2011 SERAM. Published by Elsevier España, S.L. All rights reserved.

PALABRAS CLAVECavernoma;Angioma cavernoso;Hemangioma

Malformaciones cavernosas intracraneales: espectro de manifestacionesneurorradiológicas

Resumen Las malformaciones cavernosas (cavernomas) son lesiones hamartomatosas for-

loaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

cavernoso; madas por espacios vasculares sinusoidales sin parénquima cerebral entre ellos. Las crisis son subitual. Son lesiones dinámicas en las cuales se producen cambios a lo

presentación clínica más ha Cavernomatosis

múltiple;Tomografíacomputarizada;

largo del tiempo. La mayoría son de localización supratentorial, pero hasta un 20% de los casosse presentan en la fosa posterior. Tanto en la tomografía computarizada como en la resonanciamagnética (RM) su presentación típica es como una lesión redondeada u ovoidea, bien definida,sin o con un mínimo efecto masa o edema, y con poco o ningún realce. Su apariencia

� Please cite this article as: Cortés Vela JJ, et al. Malformaciones cavernosas intracraneales: espectro de manifestacionesneurorradiológicas. Radiología. 2012. doi:10.1016/j.rx.2011.09.016.

∗ Corresponding author.E-mail address: [email protected] (J.J. Cortés Vela).

2173-5107/$ – see front matter © 2011 SERAM. Published by Elsevier España, S.L. All rights reserved.

Page 2: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

402 J.J. Cortés Vela et al.

Resonanciamagnética;Arteriografía

en la RM dependerá del estadio de la hemorragia, siendo la secuencia más sensible el eco degradiente T2. El cavernoma no es visible en la arteriografía. No obstante, ésta puede demostraruna anomalía del desarrollo venoso asociada. Los cavernomas pueden presentar característicasatípicas en cuanto a su tamano, apariencia, localización y número.© 2011 SERAM. Publicado por Elsevier España, S.L. Todos los derechos reservados.

I

Chwotvlsemact

dc5sCa

gdacarcaod

FdpTc

btfid(taoihbd

fpTtidTebm(ra

Histological features

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

ntroduction and epidemiology

avernomas are non-encapsulated, well-defined, vascularamartomatous lesions formed by sinusoidal vascular spacesith no cerebral parenchyma between them. They arene of the four major types of vascular malformations ofhe central nervous system, together with developmentalenous anomalies, arteriovenous malformations, and capil-ary telangiectasias.1 The literature offers a wide range ofynonyms of cavernoma, such as cavernous angioma, cav-rnous malformations, and angiographically occult vascularalformation. Although cavernomas were first classified as

rare condition, they are now becoming a more and moreommon finding in neuroimaging studies, particularly afterhe advent of magnetic resonance imaging (MRI).

Since they are normally asymptomatic, their actual inci-ence is not well known. According to series of autopsies,arvernomas occur in about 0.4% of subjects, accounting for---13% of all cerebral vascular malformations, and are onlyecond to venous developmental anomalies in incidence.2

avernomas occur equally in males and females and usuallyppear between the second and fifth decade of life.3

All cavernomas were initially thought to have a con-enital cause. However, it has been shown that they areynamic lesions that undergo changes overtime (de novoppearance, growth, size reduction).4 On some occasions,arcinomas disappear after a hemorrhage.5 Several factorsssociated with de novo formation of carcinoma have beeneported6: previous cranial irradiation, infection of spe-ific viruses, influence of hormones, genetic causes, seeding

long the needle track during biopsy, and venous devel-pmental anomalies.7 The association of cavernoma andevelopmental venous anomaly should be considered since

igure 1 Cavernoma in the left cerebellar hemisphere with assorains into the left transverse sinus. (a) and b) T2-weighted craniallane. Cavernoma in the left cerebellar hemisphere. An associated

1-weighted sequence clearly shows enhancement of the developavernoma after administration of intravenous gadolinium.

Od

oth disorders co-occur in about 30% of cases according tohe medical literature.8 In this respect, it would be very use-ul to complete the radiological studies with the injection ofntravenous contrast material because, unlike cavernoma,evelopmental venous anomaly shows intense enhancementFig. 1). There are also studies reporting the rare associa-ion between cavernoma, developmental venous anomaly,nd capillary telangiectasia as spectrum of one same dis-rder. Capillary telangiectasia is best detected followingntravenous contrast administration.9 Based on a number ofistological and immunohistochemical studies, a theory haseen suggested for cavernoma formation in case of previousevelopmental venous anomaly10,11 (Fig. 2).

Recent genetic studies have provided evidence of dys-unction of specific genes involved in angiogenesis inatients with inherited forms of cerebral cavernomas.hese genes encode proteins that interact at the junc-ion of endothelial cells. These patients would present withncreased vascular permeability caused by the absence orysfunction of the junctions between the endothelial cells.hree genes associated with familial forms of cerebral cav-rnoma have been identified to date. These genes haveeen named after the abbreviation CCM (cerebral cavernousalformations): CCM1 (KRIT1), CCM2 (MGC4607), and CCM3

PDCD10). Of all three, gen CCM3 is associated with a higherisk of hemorrhage, and thus, with appearance of the diseaset an earlier age.12

ciated developmental venous anomaly whose collecting vein MR image and T2 gradient-echo cranial MR image in the axialdevelopmental venous anomaly can be guessed in (a). (c) Axialmental venous anomaly and absence of enhancement of the

n histological examination, cavernomas are composed ofilated vascular channels variable in size lined by a thin and

Page 3: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

Cerebral cavernous malformations: Spectrum of neuroradiological findings 403

Theory of cavernoma formation

Stenosis of collecting vein from the developmental venous anomaly

Increased pressure in the capillary bed

Microhemorrhages and red blood cell extravasation

Fibroblastic and endothelial proliferation

Cavernoma formation

Figure 4 Axial T2-weighted MR image shows a typical caver-n

tcaioib

ntsCta

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Figure 2 Theory suggested for cavernoma formation in thesetting of a previous developmental anomaly.

weak epithelium that lacks elastic and muscular layers pre-disposing to hemorrhage. The channels are surrounded by areactive collagenous matrix. There might be internal calci-fications. The main histologic feature of cavernomas is theabsence of cerebral parenchyma between the interstices ofthe lesions, which distinguishes cavernomas from capillarytelangiectasias.10

Macroscopically, cavernomas are bluish nodules contain-ing areas of bleeding at different stages. Due to recurrentbleeding, carvernomas are surrounded by a pseudocapsuleof gliotic brain that is stained with hemosiderin (Fig. 3). Aswill be discussed below, all these histologic features condi-tion the radiological appearance of cavernomas.

Clinical manifestations

Cavernomas may affect any part of the brain and their clin-ical manifestations mainly depend on their localization.

Supratentorial cavernomas are the most common,accounting for approximately 80% of cases. They mainlyaffect the subcortical region and the frontal and tempo-ral lobes are the most commonly affected (Fig. 4). Seizuresare the most frequent clinical manifestation of cavernomas,

generally associated with hemorrhages. However, they canalso be associated with headaches, other focal neurologicaldeficits, and hemorrhages.3,13

ac

Figure 3 Histologic features of the cavernoma. (a) Microscopic histdilated vascular lumina with frequent signs of thrombosis, adjacendepositions. (b) Macro photography of the same lesion shows a bluis

oma located in the subcortical area of the left frontal lobe.

In the posterior fossa, cavernomas mostly affect the pro-uberance and cerebellar hemispheres (Fig. 5) and theirommon manifestations are focal neurological deficits, suchs anomalies in the pairs of cranial nerves and changesn sensitivity and ataxia. The first clinical manifestationsf intraventricular cavernomas (very rare) usually involventracranial hypertension caused by obstruction of the cere-rospinal fluid due to recurrent hemorrhages.14

A number of factors have been reported that modify theatural history and behaviour of these lesions, increasinghe risk of hemorrhage, and thus, conditioning more aggres-ive clinical presentations (multiple lesions, female sex,CM3 genotype, diagnosis before 35 years of age, infraten-orial location, association with a developmental venousnomaly, and lesions >1 cm in diameter15).

According to the series, up to 40% of cavernomas aresymptomatic and incidentally discovered during radiologi-al examinations.2,3,15

ologic section dyed with hematoxylin---eosin showing congestivet cerebral parenchyma with reactive gliosis, and hemosiderinh nodule with hemorrhages at different stages.

Page 4: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

404 J.J. Cortés Vela et al.

Figure 5 (a) Axial T2 gradient-echo MRI sequence shows a cavernoma centered in the protuberance. (b) Coronal T2-weighted MRimage shows a small cavernoma in the left cerebellar hemisphere.

Typical radiological features

Computerized tomography (CT), with or without contrastadministration, detects only 30---50% of lesions, leading tounderdiagnosis of the disease if CT is the only imagingmodality used.16 Cavernomas usually manifest as high-density and well-defined lesions rounded or ovoid in shape,sometimes with internal calcifications. The surroundingbrain parenchyma usually remains normal. There is usuallyminimal or no mass effect on the adjacent structures andno vasogenic edema associated. Mild or no enhancementis observed following intravenous contrast administration17

(Fig. 6).MRI is the primary imaging technique for cavernoma diag-

nosis and evaluation, showing a clearly higher sensitivitythan CT. The appearance of cavernomas varies depend-ing on the stage of the hemorrhage.17 The most commonfinding of uncomplicated cavernomas is known as popcornlesion involving a nucleus with heterogeneous signal inT1- and T2-weighted images (due to thrombosis, hemor-rhage, fibrosis, and calcification) surrounded by a completehemosiderin ring with lower signal intensity in T2-weightedsequences (Fig. 7). The most sensitive sequence to detect

Figure 6 Cavernoma located in the right suprasylvian area,a typical CT image. (a) Unenhanced axial CT. (b) Similar axialimage after contrast injection shows a well-defined, high-density, rounded lesion with no associated edema or mass effectand no enhancement after contrast administration.

cavernomas is T2-weighted gradient-echo sequences.18 Thelesion is said to bloom (it is more clearly visualized) due tomagnetic susceptibility effects caused by hemoglobin degra-dation products. It has recently been demonstrated thatMRI performed with high-field (3T) systems provide bet-ter morphological characterization of these lesions, whilenew susceptibility-weighted imaging (SWI) is more sensitive

Figure 7 Cavernoma located in the right suprasylvian area,a typical MR image (the same case as in Fig. 6). (a) and(b) Axial T1- and T2-weighted RM images, respectively, show thetypical popcorn appearance of a cavernoma with hypointenseperipheral rim in T2-weighted sequence. (c) Note the bloomingin the axial T2-weighted gradient-echo sequence.

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Page 5: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

Cerebral cavernous malformations: Spectrum of neuroradiological findings 405

Figure 8 Left parietal cavernoma with associated developmental venous anomaly. (a) Coronal T1-weighted MR image afterernomveno

owa

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

administration of intravenous gadolinium showing both the cavarteriography clearly shows the anatomy of the developmental

to detect small lesions than conventional sequences.19

As with CT, the surrounding parenchyma is usually nor-mal and the mass effect, edema, and enhancement

following administration of intravenous gadolinium rarelyoccur.

Arteriography is indicated when there are doubts aboutthe causes of cerebral haemorrhage, when the diagnosis

cCci

Figure 9 Cavernomas with an atypical radiological appearance.occipital cavernoma with vasogenic edema and a mild mass effect oT2-weighted RM image shows a cavernoma in the right hemi-protuberfluid-fluid level inside the cavernoma, loss of the peripheral low-inten(c) and (d) Axial T2-weighted gradient-echo sequence. Intraventriculocated adjacent to the occipital horn of the right lateral ventricle.

a and the enhancing developmental venous anomaly. (b) Theus anomaly, whereas the cavernoma remains occult.

f cavernoma cannot be reliably made using CT or MRI,ith a view to ruling out an arteriovenous malformations the cause of hemorrhage. Arteriography is not indi-

ated when the MRI shows the typical image of cavernoma.avernomas, thrombosed arteriovenous malformations, andapillary telangiectasia make up the group of angiograph-cally occult vascular malformations, whose main common

(A) Axial T2-weighted cranial RM image shows a large rightn the occipital horn of the right lateral ventricle. (b) Coronalance with signs of recent haemorrhage. The image also shows asity rim, vasogenic edema, and mass effect on the IV ventricle.lar haemorrhage caused by previous bleeding of a cavernoma

Page 6: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

4 J.J. Cortés Vela et al.

fbrvMi

A

Ci

wcampois

vtwTctptvt

Figure 10 Axial T2-weighted gradient-echo sequence. Giantcavernoma that takes most of the left occipital lobe. In spite ofio

Fo

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

06

eature is the absence of vascular anomalies due to slowlood flow. This is by far the most common feature.20,21 Arte-iography is very sensitive to demonstrate developmentalenous anomalies associated with cavernomas (Fig. 8), butRI scanning following intravenous contrast administration

s reliable enough to rule it out.

typical radiological features

avernomas may present with atypical radiological featuresn terms of appearance, size, location, and number.17

In case of recent hemorrhage, which usually co-occursith clinical manifestations, cavernomas may lack its typi-al appearance in the different imaging modalities showingtypical characteristics such as associated vasogenic edema,ass effect on adjacent structures, loss of the hypointenseeripheral hemosiderin ring, fluid-fluid level, or presencef perilesional hemorrhage17 (Fig. 9). In these cases, serialmages are useful if the hemorrhage does not undergourgery.22,23

When treating a hemorrhagic lesion in the central ner-ous system with associated edema, Yung et al. demonstratehat the presence of a hyperintense peripheral rim in T1-eighted sequences may be highly suggestive of cavernoma.his rim was observed in 62% of hemorrhages secondary toavernomas, only in 6% of hemorrhages secondary to metas-asis and it was not identified in hemorrhages caused by

rimary tumours or in primary hemorrhages. For this reason,he presence of this rim in a haemorrhage in the central ner-ous system suggests that a cavernoma is highly likely to behe cause of the hemorrhage.24

tc

t

igure 11 Fusiform cavernoma in the cervical spinal cord, whichccult (arrows in d).

ts large size, this cavernoma shows no edema or mass effectn the adjacent structures.

Most cavernomas present with a size <3 cm and many ofhem are millimetric. Nevertheless, large lesions or the so-

alled giant cavernomas may occur25 (Fig. 10).

Cavernomas have been reported in many different loca-ions. They may be found in the spinal cord, most commonly

extends from C2 to C5 (a, b, and c) but it is angiographically

Page 7: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

Cerebral cavernous malformations: Spectrum of neuroradiological findings 407

Figure 12 Typical multiple cavernomatosis. (a) T1-weighted sequence. (b) T2-weighted FLAIR sequence. (c) T2-weightedsequence. (d) T2-weighted gradient-echo sequence. All sequences are visualized in the axial plane. Note the superior perfor-mance of T2-weighted gradient-echo sequence to visualize these lesions (including those that had been previously identified and

ary t

ht

M

CmawcsnumTbc

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

many others) due to the magnetic susceptibility artifact second

in the cervical region, usually co-occurring with multiplebrain lesions26 (Fig. 11). Subarachnoid, intraventricular, sub-dural and even extradural lesions as well as lesions in thesinuses of the dura mater have been reported.27

In approximately 15---20% of cases, more than one lesionis diagnosed, a phenomenon which is known as multiplecavernomatosis. These cases are associated with a fam-ily history of cavernoma in over 80% of patients, whereasonly 15% of multiple lesions have been reported in caseswith no family history. Multiple lesions involve a higher riskof bleeding, and thus, they clinically manifest in patientsat an earlier age.28,29 In cases of multiple cavernomas, T2gradient-echo sequences (and more recently, susceptibility-weighted sequences) usually detect a higher number oflesions than the rest of sequences due to the bloomingof small lesions by the magnetic susceptibility effect dueto millimetric hemosiderin depositions18 (Fig. 12). There-fore, when punctuate hemosiderin depositions are seen

in T2 gradient-echo sequences, multiple cavernomatosisshould be included in the differential diagnosis, togetherwith hypertensive angiopathy, amyloid angiopathy, hemor-rhagic metastasis, vasculitis of the central nervous system,

ci

a

o hemoglobin degradation products.

emorrhagic diffuse axonal injury, and radiation-inducedelangiectasia.

anagement

avernomas associated with epilepsy seizures are nor-ally managed with conservative medical treatment of

ntiepileptic drugs. Resection of a cavernoma is indicatedhen seizures cannot be controlled with pharmacologi-al treatment, the adverse effects of the drugs are tooerious, or the patient fails to follow the treatment. Aumber of studies have reported seizure-free rates of 60%sing pharmacological treatment,30 whereas surgical treat-ent provides a seizure-free rate of approximately 80%.31

hose lesions that initially manifest with hemorrhage shoulde managed with conservative treatment. However, surgi-al resection should also be considered if the hemorrhage

auses serious neurological signs or symptoms, or if rebleed-ng occurs.

Surgical treatment is particularly indicated in superficialnd supratentorial lesions that can be easily approached.

Page 8: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

4

Bairt

sbtrprislm

C

Cursgiwamrttt

A

1

C

T

R

1

1

1

1

1

1

1

1

1

1

2

2

2

2

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

08

iopsy can be performed when diagnosis is not entirely reli-ble. It should be noted that resection of the entire lesions mandatory because partial resection entails a higherisk of haemorrhage than that associated with conservativereatment.32

In spite of their difficult surgical approach and risk ofequelae, brainstem lesions and basal ganglia lesions shoulde more aggressively managed since they have a greaterendency toward bleeding and higher sequela and mortalityates. There is controversy as to what is the most appro-riate therapeutic treatment for these lesions. Surgicalesection should be performed. However, the high morbid-ty and mortality rates associated have recently given way totereotactic radiosurgery as an alternative for treatment ofesions with difficult surgical approach, with lower morbidityortality rates.33

onclusions

avernomas are vascular malformations that the radiologistsually faces in daily practice. They present with typicaladiological features, especially on MRI, associated withubacute or chronic blood products. For this reason, T2radient-echo sequences are of great help for carvernomadentification. Cavernomas are quite commonly associatedith developmental venous anomaly, and thus, MRI studiesre necessary for better visualization. However, cavernomasay manifest with atypical features, usually associated with

ecent hemorrhage. In these cases, follow-up is very usefulo confirm diagnosis. Knowledge of these features is crucialo make a correct diagnosis, which enables optimization ofhe different treatment strategies.

uthorship

1. Responsible for the integrity of the study: JJCV.2. Conception of the study: JJCV, LCA, FBM and JIGL.3. Design of the study: JJCV, LCA, FBM and JIGL.4. Acquisition of data: JJCV.5. Analysis and interpretation of data: JJCV, LCA, FBM and

JIGL.6. Statistical analysis: N/A.7. Bibliographic search: JJCV, LCA, FBM, JIGL and JGSSG.8. Drafting of the paper: JJCV, LCA, FBM, JIGL and JGSSG.9. Critical review with intellectually relevant contrib-

utions: JJCV, LCA, FBM, JIGL and JGSSG0. Approval of the final version: JJCV, LCA, FBM, JIGL and

JGSSG.

onflict of interest

he authors declare not having any conflict of interest.

eferences

1. McCormick WF. The pathology of vascular (‘‘arteriovenous’’)malformations. J Neurosurg. 1966;24:807---16.

2. Robinson JR, Awad IA, Magdinec M, Little JR. Natural history ofthe cavernous angioma. J Neurosurg. 1991;75:707---14.

3. del Curling O, Kelly DL, Elster A. An analysis of the naturalhistory of cavernous angiomas. J Neurosurg. 1991;75:702---8.

2

J.J. Cortés Vela et al.

4. Houtteville JP. Brain cavernoma: a dynamic lesion. Surg Neurol.1997;48:610---4.

5. Pérez López C, Isla Guerrero A, Gómez Sierra A, Budke M,Álvarez Ruiz F, Sarmiento Martínez MA. Tratamiento de la cav-ernomatosis cerebral múltiple. Rev Neurol. 2002;35:407---14.

6. Vajtai I, Varga Z. Origin of the novo central nervous systemcavernomas. J Neurosurg. 1998;88:616---7.

7. Ogilvy CS, Moayeri N, Golden J. Appearance of a cavernoushemangioma in the cerebral cortex after a biopsy of a deeperlesion. Neurosurgery. 1993;33:307---9.

8. Revert Ventura AJ, Martí-Bonmatí L, Poyatos Ruipérez C,Pallardó Calatayud Y, Arana E, Mollá Olmos E. Associationof cavernous and venous angiomas. Rev Neurol. 2007;22:839---45.

9. Abla A, Wait SD, Uschold T, Lekovic GP, Spetzler RF. Develop-mental venous anomaly, cavernous malformation and capillarytelangiectasia: spectrum of a single disease. Acta Neurochir.2008;150:487---9.

0. Dillon WP. Cryptic vascular malformations: controversies interminology, diagnosis, pathophysiology, and treatment. Am JNeuroradiol. 1997;18:1839---46.

1. Sure U, Butz N, Schlegel J. Endothelial proliferation, neoangio-genesis, and potential de novo generation of cerebrovascularmalformations. J Neurosurg. 2001;94:972---7.

2. Yadla S, Jabbour PM, Shenkar R, Shi C, Campbell PG, Awas IA,et al. Cerebral cavernous malformations as a disease of vascu-lar permeability: from bech to bedside with caution. NeurosurgFocus. 2010;29:E4.

3. Lobato RD, Pérez C, Rivas JJ, Cordobés F. Clinical, radiologicaland pathological spectrum of angiographically occult intracra-nial vascular malformations. J Neurosurg. 1988;68:518---31.

4. Iza-Vallejo B, Mateo-Sierra O, Mosqueira-Centurión B, Ruiz-Juretschke F, Carrillo R. Cavernomas cerebrales. Revisión yactualización etiológica, clínica y terapéutica. Rev Neurol.2005;41:725---32.

5. Batra S, Lin D, Recinos PF, Zhang J, Rigamonti D. Cavernousmalformations: natural history, diagnosis and treatment. NatRev Neurol. 2009;5:659---70.

6. Vaquero J, Salazar J, Martínez R, Martínez P, Bravo G. Caver-nomas of the central nervous system: clinical syndromes, CTscan diagnosis and prognosis after surgical treatment. Acta Neu-rochirurg. 1987;85:29---33.

7. Rivera PP, Willinsky RA, Porter PJ. Intracranial cavernous mal-formations. Neuroimag Clin North Am. 2003;13:27---40.

8. Brunereau L, Labaug P, Tournier-Lasserve E, Laberge S, Levy C,Houtteville JP. Familial form of intracranial cavernous angioma:MR imaging findings in 51 families. Radiology. 2000;214:209---16.

9. Campbell PG, Jabbour P, Yadla S, Awad IA. Emerging clini-cal imaging techniques for cerebral cavernous malformations:a systematic review. Neurosurg Focus. 2010;29:E6.

0. Numaguchi Y, Fukui M, Miyake E, Kishikawa T, Ikeda J, MatsuuraK, et al. Angiographic manifestations of intracerebral cavernoushemangioma. Neuroradiology. 1977;14:113---6.

1. Tomlinson F, Houser OW, Scheithauer BW. Angiographicallyoccult vascular malformations: a correlative study of featureson magnetic resonance imaging and histological examination.Neurosurgery. 1994;34:92---9.

2. Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J,Spetzler RF. The MR appearance of cavernous malformations(angiomas). J Neurosurg. 1987;67:518---24.

3. Willinsky R, Harper W, Wallace MC, Kucharczyk W, MontaneraW, Mikulis D, et al. Follow-up MR of intracranial cavernomas:the relationship between haemorrhagic events and morphology.Interv Neuroradiol. 1996;2:127---35.

4. Yun TJ, Na DG, Kwon BJ, Rho HG, Park SH, Suh YL, et al. AT1 hyperintense perilesional signal aids in the differentiationof a cavernous angioma from other hemorrhagic masses. Am JNeuroradiol. 2008;29:494---500.

Page 9: Cerebral cavernous malformations: Spectrum of neuroradiological … · 2015. 2. 18. · previous cranial irradiation, infection of spe-cific viruses, influence of hormones, genetic

ogic

3

3

3

1220---8.

Document downloaded from http://www.elsevier.es, day 22/01/2015. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Cerebral cavernous malformations: Spectrum of neuroradiol

25. Kan P, Tubay M, Osborn A, Blaser S, Couldwell WT. Radiographicfeatures of tumefactive giant cavernous angiomas. Acta Neu-rochir. 2008;150:49---55.

26. Toldo I, Drigo P, Mammi I, Marini V, Carollo C. Vertebral andspinal cavernous angiomas associated with familial cerebralcavernous malformation. Surg Neurol. 2009;71:167---71.

27. Kivelev J, Niemelä M, Kivisaari R, Hernesniemi J. Intraventric-ular cerebral cavernomas: a series of 12 patients and review ofthe literature. J Neurosurg. 2010;112:140---9.

28. Gómez-Sierra A, Rodríguez de Lope A, Mateo-Sierra O. Caver-

nomatosis cerebral familiar. Neurocirugía. 1998;9:312---5.

29. Labauge P, Brunereau L, Laberge S, Houtteville JP. Prospectivefollow-up of 33 asymptomatic patients with familial cerebralcavernous malformations. Neurology. 2001;57:1825---8.

3

al findings 409

0. Noto S. Management of patients with cavernous angiomaspresenting epileptic seizures. Surg Neurol. 2005;64:495---8.

1. Ferroli P. Cerebral cavernomas and seizures: a retrospectivestudy on 163 patients who underwent pure lesionectomy. NeurolSci. 2006;26:390---4.

2. Amin-Hanjani S, Ogilvy CS, Ojemann RG, Crowell RM.Risks of surgical management for cavernous malfor-mations of the nervous system. Neurosurgery. 1998;42:

3. Hasegawa T, McInerney J, Kondziolka D. Long-term results afterstereotactic radiosurgery for patients with cavernous malfor-mations. Neurosurgery. 2002;50:1190---7.