Transcript Document

Extra-cerebral Intracranial Cavernous
Malformations: MRI findings in 27
patients.
Mauricio Castillo, MD, FACR,
Hortensia Alvarez, MD
University of North Carolina at Chapel Hill
Abstract
• Objective: to review imaging/clinical findings
in patients with extra-cerebral intracranial
cavernous malformations (CM).
• From our TF database we extracted 28
patients with extra-cerebral intracranial CM.
All had MRI with at least pre-contrast T1, T2
images & post Gd T1.
• 13/27 patients underwent resection with
confirmation of diagnosis.
Findings
• Patients age: 1 week to 55 years
• Gender: 22 males, 6 females
• CM size: 1-12 cm
– Largest ones in children and those in trigones of
lateral ventricles
• Associated vascular anomalies:
– DVAs in 4 patients
– Veno-sinus malformation in 1 patient
Findings
• Locations:
– Intraventricular: 13
• 5 in 3rd ventricle, 1 in 4th, 7 in lateral
ventricles
• 2 patients had multiple cerebral CM
syndrome
–
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–
–
–
Dural (convexities): 7
Middle fossae: 2
Falx cerebri: 4
Cavernous sinus: 1
IAC: 1
Most common locations of CM in our
patients.
Symptoms and Management
• Most common symptoms:
– Hydrocephalus: 6
– Seizures: 8
– Headaches: 2
– Others: 11
• Surgical resection for all those that produced
seizures & 2 with focal neurological deficits.
• Resection or biopsy for all patients with
hydrocephalus.
CM, General Facts
• Occur in 0.5% of the population, account for 15%
of all cerebral vascular malformations.
• 80% supratentorial, 20% infratentorial, 15% of all
CM are extra-axial.
• 50-75% of patients are eventually symptomatic:
•
•
•
•
40-70% have seizures,
35-50% have focal deficits,
10-30% have headaches,
Hemorrhage is rare (more common in children).
• Solitary lesions do not exclude familial syndrome.
CM- Treatment
• Surgery reserved for patients with seizures/focal
deficits unresponsive to other therapies & those
with CM in accessible regions.
• Re-operations are rare.
• Gamma-knife/radiosurgery while effective
increases risk of hemorrhage.
• Aspirin, NSAD, heparin, warfarin may increase
risk of bleeding.
MRI-Pathology-Clinical Correlations
Type
MRI
Histology
Clinical
1
T1 & T2 bright core
Subacute blood
Generally symptomatic,
may re-bleed
2
T1 & T2 complex core,
hypointense halo
Bleeds & thromboses
May be either symptomatic
or asymptomatic
3
T1 & T2 hypointense
core & halo
Chronic bleeds
May be either symptomatic
or asymptomatic
4
T1 hypointense core, T2
hyperintense/complex
core, low signal halo
Vascular channels &
stroma, no
acute/subacute
bleeds
May be either symptomatic
or asymptomatic
5
Punctate hypointense
lesions on SWI/GRE
Tiny CM or capillary
telangiectasia
Asymptomatic, true new
lesions
Adapted & modified from Zabramski & de Souza.
Histopathology. CM are vascular
malformations consisting of closely
clustered, enlarged capillary
channels (caverns) with a single
layer of endothelium without
normal mature vessel wall elements
or intervening brain. Thrombosis &
intra- and extra-lesional
hemorrhage may be present.
Edema may surround lesions with
recent hemorrhage.
Intraventricular-ependymal
• Comprise 2.5% of all cerebral CM.
• Diagnosis is difficult by imaging & thus resection
may be indicated; ependymal siderosis may
suggest correct diagnosis.
• Of about 60 cases of intraventricular CMs
reported in the literature, 17 occurred in trigones.
• Trigone CM may be large (especially in children).
4th ventricular type 2 CM in a 43-year-man. Axial T2 & FLAIR images show rounded,
centrally complex & peripherally low signal lesion (arrows) in obex of 4th ventricle
which resulted in hydrocephalus. The mass was surgically resected.
Right trigone type 2 CM in a 40-year-old man. Axial T1 post Gd (left), pre Gd
parasagittal T1 (center) & axial T2 (right) show complex appearing mass which
was biopsied but not resected. Note large subependymal component of
associated DVA (arrow). Smaller CM is present anterior to frontal horn of right
lateral ventricle.
1-week-old male with intraventricular type 2 CM. A large complex mass is located in
the right trigone. Note large medial DVA (right parasagittal images). The mass was
surgically resected.
3 patients, 20-year-old female (left), 32-year-old male (center) & 29-year-old female with 3rd
ventricular CM (arrows). Masses in first 2 patients resulted in hydrocephalus & were
surgically resected. CM have complex appearances (type 2), center patient shows left
periventricular edema.
28-year-old male shows 3rd ventricular type 2 CM with obstruction of the
foramina of Monro & dilatation of the lateral ventricles.
A
B
C
D
27-year-old man. Coronal T1 post Gd (A), axial T1 post Gd (B), FLAIR (C) & T2 (D) images
show a CM in the frontal horn of the left lateral ventricle. This type 2 CM shows minimal
contrast enhancement & a peripheral rim of low signal.
45-year-old man. Axial FLAIR & T2 images show CM in the
subependymal region of the left frontal horn of the lateral
ventricle. This type 2 CM shows a halo of low T2 signal.
Two males with familial cerebral CM syndrome. In both patients
(35 [left]& 26 [above[ years-old) MR images show one CM in
the frontal horns of the right lateral ventricles each as well as
multiple cerebral CV. Type 2 CM.
A
B
C
D
6-year-old boy with right trigone type 4 CM on T1 pre Gd & T2 images (A,B). SWI (C)
shows DVA adjacent to right frontal horn & post Gd sagittal T1 image (D) shows a
malformation of the superior sagittal sinus with a persistent & large falcine sinus.
Dural CM
• Only few cases reported, generally indistinguishable
from meningioma by MRI:
– May show dural “tails” of contrast enhancement,
– High T2 signal suggests diagnosis of CM.
• Most occur in middle age (45-65 years) women.
• Most in falx cerebri, lateral cavernous sinus walls,
tentorium & falx cerebelli.
– ICA CM are extremely unusual, imaging diagnosis is
difficult in small ones
• Those in middle cranial fossa may erode bone.
• Highly vascularized & bloody at surgery.
2 patients with dural-based type 2 CM. MR images shows CM in the right
middle cranial fossae (left: 33-year-old man, right: 28-year-old woman). Both
were resected as they resulted in seizures.
3 male patients (27-year-old, 45 years old, 39-year-old, left-to-right). MR images show
variable appearance of peripherally located CM which were resected due to seizures. All
3 were extra-axial & arose from the meninges. Center is type 3, other two are type 2.
Type 2 CM in a 21-year-old male with chronic intractable epilepsy.
Extra axial location was confirmed at surgery.
CT & MR images in a 35-year-old man with seizures show a
mostly T1 hypointense, T2 bright & homogeneously contrast
enhancing mass in the right parietal region. Pathology
confirmed diagnosis of type 5 CM. (from: Vogler & Castillo. AJNR 1995; 16: 773)
Falx CM
2 patients with anterior falcine type 2 CM resected due to seizures. Both
show hypointense T2 halo (left: 43-year-old man, right: 51-year-old man)
18-month-old boy with seizures. T1 post Gd & T2 images show
complex type 2 CM in left medial occipital region. At surgery,
the mass was extra-axial & adherent to the falx.
55-year-old female with breast cancer. MR images show left para-falcine mass thought
to be a meningioma & followed for 11 years. The mass showed minimal growth during
the last year and was thus resected. The type 5 CM was adherent to the falx and extraaxial. Note high T2 signal (arrow).
Cavernous Sinus CM
45-year-old woman with a left CN III palsy. Top row: MR images show an enhancing
mass (arrows) centered in the left cavernous sinus. Note high T2 signal. Bottom row:
coronal dynamic contrast enhanced T1 images show peripheral filling in of the mass
typical of a CM in this region. Type 5 CM.
ICA CM
25-year-old female with hearing loss. T2 (left), T2* (center) and T1 post contrast (right)
images show the typical appearance of a cavernoma in the CPA cistern extending into the IAC.
Chronic blood products are seen in the cerebellum. Type 2 lesion.
Courtesy A. Pajon, Bogota, Colombia
Conclusions
• In our series, CM were type 2 (n=22), type 4 (n=4) &
type 5 (n=2).
• 26 had MRI features that suggested or made the
correct diagnosis.
• All dural-based CM were symptomatic & resected (50%
of all CM were symptomatic).
• Patients age & lesion sizes varied widely, most CM
occurred in males.
• Associated venous anomalies were less common than
reported with intracerebral CM (14% vs. 20-30%).
• Exact prevalence of extra-cerebral, intra-cranial CM
could not be established as this was a retrospective
review.
References
• Morrison L, Akers A. Cerebral cavernous malformation,
familial. GeneReviews. 2011.
http://www.ncbi.nlm.nih.gov/books/NBK1293
• Zabramski JM, Wascher TM, Spetzler RF, Johnson B et al.
The natural history of familial cavernous malformations:
results of an ongoing study. J Neurosurg. 1994;80:422–32
• de Souza JM, Domingues RC, Cruz LC, Domingues FS et al.
Susceptibility-weighted imaging for the evaluation of
patients with familial cerebral cavernous malformations: a
comparison with t2-weighted fast spin-echo and gradientecho sequences. AJNR Am J Neuroradiol. 2008;29:154–8.
• Sabat SB. Intraventricular cavernous malformation with
superficial siderosis. Arch Neurol 2010; 67: 638-9
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