Division of Neurosurgery, Institute of Brain Diseases,
Tohoku University School of Medicine, Sendai,
Japan
Neurosurgery 32; 1028-1031, 1993
ABSTRACT: THE AUTHORS ENCOUNTERED a
rare case of multilobular cavernous angioma arising
from the paraventricular region in an infant. Total
resection of the angioma, which was 8 cm in
diameter, was performed. The patient showed no
neurological deficit. Although 22 cases in infants
have previously been reported, this is the largest
cavernous angioma and it is especially rare because
of its multilobular configuration.
KEY WORDS: Cavernous angioma; Infant;
Magnetic resonance imaging; Multilobular; Xray computed tomography
Acavernous angioma in either an infant or an adult is
a rare vascular anomaly. Takaku et al. (21) reported 2
cases out of 103 brain tumors in infants and Jooma et
al. (9) found 1 in 100 cases. The authors encountered a
rare case with a multilobular configuration and a
diameter of 8 cm. The patient showed no neurological
deficit, despite total resection of this huge angioma.
Although 22 cases of cavernous angioma have
previously been reported (1-5,7-9,11-20,23,24), its
pathophysiology is still unclear. The clinical
pathology, neuroradiological features, and treatment
of this case and 22 other known cases are discussed.
CASE REPORT
The patient was an infant boy, 11 months old, with
an unremarkable family history. There were no
abnormalities during pregnancy or delivery. At the
time of delivery, his head showed a normal
measurement of 33 cm. Several days before
admission, he developed hemiparesis on the left side.
Examination
At the time of admission, his head measured 49
cm, which was still within the normal range.
Neurologically, he was stuporous, and there was
hemiparesis on the left side and conjugate deviation
of the eyeballs to the right side. A plain radiograph of
the head showed enlargement of the fontanel and
diastasis of the sutures. X-ray computed tomography
(X-CT) revealed a huge multilobular mass with a
high-density rim in the right hemisphere. The mass
was surrounded by a broad low-density area. An
enhancement effect was observed in the rim and
septum (Fig. 1). A T1-weighted magnetic resonance

Operation
An osteoplastic craniotomy was performed in the
right parietal region. The mass lesion was dissected
from the surrounding brain tissue under an operative
microscope through a small corticotomy of the
postsensory cortex, with careful preservation of deep
veins. Because of the presence of a demarcated
capsule, dissection of the mass was not difficult and
total resection was done. In the paraventricular
region, the mass adhered to the brain tissue. The
multilobular configuration, which consisted of fresh
to old hemorrhages in the cystic portion and a yellowto-brownish hard capsule, was disclosed in a surgical
specimen. The operative diagnosis was a cavernous
angioma with recurrent hemorrhage.
Postoperative course
X-CT on the second postoperative day showed a
drastic reduction of brain edema. The patient's
hemiparesis and conjugate deviation of the eyeballs
also disappeared postoperatively. During the
following 8 months, a T1-weighted magnetic
resonance image revealed only the small cavity once
occupied by the angioma and a relatively large
subdural space (Fig. 5). The patient showed no
neurological deficit at the time.
Pathological examination
There were blood vessels of varying sizes sealed
by single-layer endothelial cells. Although
proliferating connective tissue intervened between
those blood vessels, no neural tissue was observed.
Bleeding and thrombi, which were fresh or organized,
were also frequently observed (Fig. 6). On the basis
of this histological evidence, a diagnosis of
cavernous angioma was made.
DISCUSSION
Clinicopathology
Including the case presented here, there have been
23 reported cases of cavernous angioma in infants.
There has been a predominance of female infants
among the 22 cases in which the sex has been
mentioned (male, 10; female, 12). The initial
symptoms included enlargement of the head in 10

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AUTHOR(S): Kawagishi, Jun, M.D.;
Suzuki, Michiyasu, M.D.; Kayama, Takamasa, M.D.;
Yoshimoto, Takashi, M.D.

image disclosed a mass with a maximum diameter of
8 cm extending to the pineal region, trigon,
subcortical white matter, and contralateral
hemisphere from the right paraventricular region. The
septum exhibited relatively low-signal intensity,
whereas the inner part of the lobuli showed hyper- to
isosignal intensity (Fig. 2). In a T2-weighted
magnetic resonance image, a multiple hyperintense
mass surrounded by no signal band, which would
indicate the presence of hemosiderin, was observed.
A Niveau formation was also seen (Fig. 3). Cerebral
angiography disclosed no abnormal vessels,
arteriovenous shunting, or pooling of the contrast
media but did reveal a mass sign (Fig. 4). The
character of that mass lesion could not be determined
from the neuroradiological examination; however, an
operation was chosen because of his progressive
symptoms.

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Neurosurgery 1992-98
June 1993, Volume 32, Number 6
1028 Huge Multilobular Cavernous Angioma in an
Infant: Case Report
Case Report

subsequent bleeding and rapid growth under
conservative therapy.
Received, April 23, 1992.
Accepted, January 6, 1993.
Reprint requests: Michiyasu Suzuki, M.D.,
Division of Neurosurgery, Institute of Brain Diseases,
Tohoku University School of Medicine, Seiryo-machi
1-1, Aoba-ku, Sendai, Japan 980.

1.

2.

3.

4.

5.
Neuroradiology
X-CT examination was performed in 16 cases.
Fifteen cases (96%) showed a high-density area on
plain X-CT, and 9 cases (56%) showed an enhanced
effect with contrast media. However, the obvious
multilobular configuration in X-CT found in our case
has never been described in any previous reports.
Cyst formation was also a rare pathological finding,
and there were only four cases with such formation.
Twelve patients underwent cerebral angiography,
which disclosed an avascular mass (seven patients or
58%) and abnormal staining (two patients or 17%).
Magnetic resonance imaging was performed in only
one other patient besides ours, and it revealed
multicystic formation.
Treatment and outcome
Total resection of the angioma was performed in 17
patients. Thirteen patients (76%) were free of
symptoms, and in two other patients, the symptoms
improved. There are several possible reasons for the
excellent outcome of total resection: 1) A cavernous
angioma is easy to dissect without brain damage
because of its clear demarcation and few feeders and
drainers; 2) developing brains have more plasticity.
In infants, the natural history of cavernous angioma is
still unclear and operative indications have not been
clearly determined. Nevertheless, it should be
stressed that total resection is recommended,
regardless of the size or preoperative neurological
deficit, if the patient is currently symptomatic, either
because of a mass effect, hemorrhage or seizure, or
evidence of having bled in the past. The outcome of
total resection is excellent, and there are risks of

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cases (44%), epilepsy in 6 (26%), bleeding in 4
(17%), and other symptoms in 3 (13%). Recently, an
epileptic attack in a patient with cavernous angioma
has been considered as a sign of bleeding. However,
in adult cases, a higher incidence of bleeding (50 to
70%) has been reported as an initial symptom (6,10,24).
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arisen from the paraventricular region. The maximum
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COMMENTS
Kawagishi and colleagues have presented a
remarkable case of a very large cavernous angioma in
an 11-month-old child with a hemiparesis. They
stated that their operation was performed through a
"small corticotomy," but they do not mention its size
or exactly how the mass was managed. I assume that
the mass was removed piecemeal, that much of it
represented clot and granulation tissue, and that
bleeding was very easily handled--as is the case in
most of the malformations that reach this size. The
authors show us a single postoperative scan, which
looks remarkably good, and they state that the patient

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Figure 3. T2-weighted magnetic resonance image
(axial view). No signal rim surrounded multiple
hyperintense areas (arrowheads) in this T2-weighted
image. Niveau was also noted inside (arrow).

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Figure 2. T1-weighted magnetic resonance image. A,
gadolinium-enhanced axial view. B, gadoliniumenhanced coronal view. C, plain sagittal view. A
multilobular tumor with mosaic intensity was
observed, extending from the right paraventricular
region to the trigon, pineal region, and subcortical
white matter.

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Figure 1. X-CT, axial plain. Left, plain. A huge
multilobular tumor with a high-density rim
surrounded by a broad low-density area was seen in
the right hemisphere. Right, enhanced. An
enhancement effect was also noted in the rim
(arrowheads).

Figure 6. Photomicrograph of a surgical specimen
(hematoxylin and eosin, ×20). There were many
dilated vessels with lumen of varying sizes. These
were covered with a single-layered endothelium.
Bleeding (arrowheads) and thrombosed vessels
(arrow) were also noted. Proliferating connective
tissue intervened between those vessels, but no neural
tissue was observed.

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Figure 5. T1-weighted magnetic resonance image, 8
months after surgery. Only a small cavity (arrow)
was seen.

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Figure 4. Right carotid angiogram. Left, lateral view;
Right, anteroposterior view. An obvious mass sign
was observed, but there were no abnormal vessels,
pooling, or arteriovenous shunting.