J Neurosurg 90:766–769, 1999

Complex right hemisphere developmental venous anomaly
associated with multiple facial hemangiomas
Case report
BEVERLY D. L. AAGAARD, M.D., JOON K. SONG, M.D., JOSEPH M. ESKRIDGE, M.D.,
AND MARC R. MAYBERG, M.D.
Departments of Radiology and Neurosurgery, University of Washington, Seattle, Washington
U Complex developmental venous anomalies (DVAs) represent variations of normal cerebral venous drainage and consist of dilation of the superficial and/or deep venous system. These rare anomalies can occur unilaterally or bilaterally,
supratentorially or infratentorially, focally or they can affect the entire hemisphere. Some DVAs are associated with cervicofacial venous malformations or facial lymphatic malformations. Anomalies of this type are generally clinically silent,
and cerebral dysfunction is usually absent. Symptoms, when they occur, are most commonly headache or mild seizure disorders. The angiographic findings are striking, with well-formed but enlarged transcerebral medullary and deep and/or
superficial cortical veins. Opacification of these venous structures occurs within the same time frame as a normal angiographic venous phase.
The authors report the case of a 33-year-old man in whom a large inoperable arteriovenous malformation had been previously diagnosed and who presented with seizures. Repeated magnetic resonance imaging and angiography demonstrated abnormally dilated transcerebral, superficial, and deep venous structures involving the entire right hemisphere with no
identifiable nidus. Additionally, multiple bilateral benign facial hemangiomas were present in this patient.
It is important to recognize this rare venous appearance as a developmental variant and not mistake it for an arteriovenous malformation or a partially thrombosed vein of Galen malformation. Because these venous anomalies are extreme
variants of the normal venous system, hemorrhage rarely, if ever, occurs and the patient can be reassured that no interventional or surgical therapy is necessary or warranted.

KEY WORDS • complex developmental venous anomaly • facial hemangioma •
venous malformation

C

OMPLEX developmental venous anomalies (DVAs)

represent variations of normal cerebral venous
drainage and consist of dilation of the superficial
and/or deep venous system. Unlike commonly encountered simple venous variants, such as the venous angioma,
complex DVAs are quite rare. The superficial cortical
venous system can be enlarged in DVAs but, unlike the
venous abnormalities seen in patients with Sturge–Weber
disease, is otherwise normally formed. No nidus is present, as in arteriovenous malformations (AVMs), and the
venous structures drain normal brain parenchyma.
We present a case of complete hemispheric complex
DVA in a patient with a mild seizure disorder and multiple facial hemangiomas.
Case Report

History. This patient, an otherwise healthy right-handed
33-year-old man, a professional chef, presented to the
emergency room in May 1997 after two suspected seizures while at work. Each witnessed episode lasted for
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approximately 45 seconds and was accompanied by urinary incontinence but without abnormal motor activity or
fecal incontinence. In the emergency department, the patient’s Glasgow Coma Scale score was determined to be
15, with a nonfocal neurological examination. His vital
signs, including blood pressure, were within normal limits. Computerized tomography (CT) scanning revealed no
evidence of subarachnoid hemorrhage or acute parenchymal abnormalities.
In 1993, the patient had previously undergone angiography for a suspected AVM, which was initiated after CT
and magnetic resonance (MR) imaging studies demonstrated abnormal enlargement of the deep venous system.
Angiography reportedly demonstrated abnormal enlargement of the deep right hemispheric venous system without
an identifiable nidus. Despite the absence of a nidus, a
hemispheric AVM was diagnosed; it was not amenable to
interventional or surgical treatment.
The patient’s history was also notable for a report of
multiple small discrete lesions throughout the face. The
patient related that as a child he could enlarge these facial
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Developmental venous anomalies

FIG. 1. Left: Fast spin–echo T2-weighted axial MR image demonstrating enlargement of the right deep middle cerebral vein (open arrow), basal vein of Rosenthal (solid arrow), and straight sinus (arrowheads). Right: Fast spin–echo
T2-weighted axial image demonstrating enlarged subependymal veins at the right ventricular trigone (arrows).

lesions by performing Valsalva maneuvers. A biopsy sample from of one of the lesions, obtained in 1990, revealed
benign hemangioma.
Examination. We performed MR imaging after the patient’s seizure, which again revealed a markedly enlarged
right hemispheric venous system with enlargement of the
basal vein of Rosenthal, the vein of Galen, straight sinus,

FIG. 2. Fat-saturated gadolinium-enhanced (0.1 mm /kg) spin–
echo T2-weighted axial MR image obtained at the level of the
mandible demonstrating several discrete enhancing lesions
(arrows) in the facial and oral soft tissues, which are consistent
with hemangiomas.

J. Neurosurg. / Volume 90 / April, 1999

and right transverse and sigmoid sinuses. Enlarged subependymal vessels along the right lateral ventricle were
suspected to represent the nidus (Fig. 1). The brain was
structurally unremarkable, and the parenchyma was normal in appearance.
Additionally, noted on T2-weighted MR imaging were
multiple small (0.5–2 cm) discrete hyperintense lesions
throughout the face, involving the superficial parotid
glands, bilateral masticator spaces, left infratemporal fossa, and right occipital scalp; their appearance on MR imaging was consistent with that of lymphangiomas or hemangiomas (Fig. 2).
A CT scan demonstrated prominent dense calcification
of the basal ganglia, which was unusual for the patient’s
age but was otherwise unremarkable in appearance.
Fluoroscopy demonstrated multiple small rounded calcifications in the soft tissues of the face, ranging in size
from 4 mm to 1 cm and corresponding to the location of
the facial lesions seen on MR imaging.
Right internal carotid artery injection angiography revealed moderately enlarged but otherwise normal-appearing middle cerebral artery branches (Fig. 3). No serpiginous vessels were identified to suggest a nidus. In the
capillary phase, multiple fine radially oriented dilated
transcerebral medullary veins were seen to traverse the
entire right hemisphere, particularly prominent in the region of the right parietal lobe and deep periventricular
white matter. The hyperemia related to these dilated transcerebral veins produced a false pathological blush in the
capillary phase (Fig. 4). Superficial cortical veins, the vein
of Labbé, the internal cerebral veins, the basal vein of
Rosenthal, and the vein of Galen were moderately enlarged. The straight sinus, torcular herophili, and the right
transverse and sigmoid sinuses were markedly enlarged
(Fig. 5).
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B. D. L. Aagaard, et al.

FIG. 3. Lateral digital subtraction right internal carotid artery angiogram demonstrating moderately enlarged but otherwise normalappearing middle cerebral artery branches. No nidus is present.

Right vertebral artery injection angiography demonstrated mild enlargement of the right posterior cerebral
artery and the veins of the right cerebellar hemisphere.
Right external carotid artery, left external carotid artery,
and left internal carotid artery angiograms demonstrated
no additional arterial or venous abnormalities.
Discussion
Complex DVAs represent variations of the normal cerebral venous drainage system. Unlike commonly encountered simple venous variants, such as venous angioma,
complex DVAs are quite rare. As classified by Lasjaunias
and colleagues,4,5 complex DVAs can occur unilaterally or

FIG. 5. Venous-phase angiogram demonstrating enlargement of
the vein of Labbé, internal cerebral vein, basal vein of Rosenthal,
vein of Galen (curved white arrow), straight sinus (straight white
arrow), and torcular herophili (small black arrows).

bilaterally, supratentorially or infratentorially, superficially and/or in deep tissue (Table 1).3,4 Involvement may be
focal or, as in this case, involve the entire hemisphere. In
patients with extensive complex transcerebral medullary
venous anomalies, the enlarged caliber of these small
veins gives rise to the fan-shaped or radially oriented appearance in which the veins converge to drain into either
the cortical or deep venous system. Unlike other venous
anomalies, such as Sturge–Weber syndrome, the superficial cortical venous system is enlarged but otherwise normally formed. No nidus is present, as in AVMs, and no
persistent embryological venous structures remain patent,

FIG. 4. Left: Magnified anteroposterior pillar view in the capillary phase of a right internal carotid artery angiogram
demonstrating multiple fine radially oriented transcerebral veins. Right: The hyperemia resulting from the dilated transcerebral veins produced a false pathological blush in the capillary phase (arrowheads and solid arrows).

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Developmental venous anomalies
TABLE 1
Classification of DVAs
superficial
supratentorial
infratentorial
mixed
deep
supratentorial
infratentorial
mixed
complex (combined superficial & deep)
supratentorial
infratentorial
unilat
bilat
associated dural anomalies
sinus pericranii
congenital vascular malformations (arterial,
arteriovenous, venous [cavernomas])
acquired anomalies (ectasia)

Conclusions
The complex DVA represented by this case is a variant
form of normal venous drainage. Anomalies of this type
are usually clinically silent and cerebral dysfunction is
usually absent. As presented, the angiographic findings
are striking and may affect a portion of the brain or the
entire hemisphere, as seen in this case. Because these
venous anomalies are extreme variants of normal anatomy
and do not represent arteriovenous or partially thrombosed vein of Galen malformations, hemorrhage is not a
clinical concern and no surgical or interventional therapy
is necessary.
Acknowledgments
The authors thank Janet Schukar and Paul Schwartz for their
assistance with photography.
References

such as the midline prosencephalic vein seen in vein of
Galen malformations.7 The mild enlargement sometimes
seen in ipsilateral arteries with complex DVAs likely reflects the increased flow in the venous system.
These anomalies are usually clinically silent and are an
incidental finding. The brain parenchyma between the dilated venous channels appears structurally normal, and
cerebral dysfunction is generally absent. Symptoms, when
they occur, are generally headache or mild seizure disorders and are thought to reflect altered venous flow in
these less flexible or less adaptable anomalous veins.5 In
patients exhibiting cervicofacial venous malformations,
20% will have concurrent cerebral DVAs.1 Developmental
venous anomalies have also been reported in patients with
facial lymphatic malformations, although the developmental association is unclear.4
The incidence of hemorrhage in patients with DVAs has
been controversial in the past. In general, DVAs either
simple, such as the venous angioma, or complex are rarely, if ever, associated with hemorrhage, unlike entities
such as cavernous venous malformations or AVMs.2,6,8,9
Cavernous venous malformations associated with venous
variants likely represent the true cause of hemorrhage.2
It is important to recognize that this pattern of venous
enlargement and drainage represents a venous anomaly,
not a malformation. Because hemorrhage is not a clinical
concern and the venous structures drain normal brain
parenchyma, no further therapy, surgical or interventional,
is necessary. Failure to recognize this entity correctly can
lead to needless anxiety for the patient and inappropriate,
potentially injurious treatment.

J. Neurosurg. / Volume 90 / April, 1999

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Rev 9:233–242, 1986
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241–251, 1968
7. Mullan S, Mojtahedi S, Johnson DL, et al: Embryological basis
of some aspects of cerebral vascular fistulas and malformations.
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8. Rigamonti D, Drayer BP, Johnson PC, et al: The MRI appearance of cavernous malformations (angiomas). J Neurosurg 67:
518–524, 1987
9. Rigamonti D, Spetzler RF, Drayer BP, et al: Appearance of venous malformations on magnetic resonance imaging. J Neurosurg 69:535–539, 1988
Manuscript received March 31, 1998.
Accepted in final form November 12, 1998.
Address for Dr. Aagaard: Columbia University, New York,
New York.
Address for Dr. Mayberg: Cleveland Clinic Foundation, Cleveland, Ohio.
Address reprint requests to: Joseph M. Eskridge, M.D., Department of Neurosurgery, University of Washington Medical Center,
Box 356470, 1959 Northeast Pacific, Seattle, Washington 98195.

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