Childs Nerv Syst
DOI 10.1007/s00381-015-2663-0

CASE-BASED UPDATE

Epilepsy in a boy with a developmental venous
anomaly—case-based update
Šimić Klarić Andrea & Milić Jakov & Gotovac Nikola

Received: 9 August 2014 / Accepted: 17 February 2015
# Springer-Verlag Berlin Heidelberg 2015

Abstract
Background Developmental venous anomalies (DVAs) are
the most common intracranial vascular malformations of the
brain. They are most commonly asymptomatic and often diagnosed incidentally.
Illustrative case We present a rare case of a 9-year-old boy
with seizures caused by a subcortically located DVA posterior
to the postcentral gyrus of the left brain hemisphere. MRI also
showed an enlarged, microcystically altered pineal gland and
an extracranial cavernous hemangioma. EEG showed left
fronto-centro-parietal spikes that corresponded with the location of the DVA. The spikes were occasionally secondary
generalized. The boy responded well to oxcarbazepine treatment, and no surgical intervention was required. Control MRI
scans showed no progression of the DVA.
Conclusion Epilepsy may be caused by DVA. The treatment
starts with anticonvulsants. The surgical treatment is the therapeutic choice only if seizures are resistant to conservative
therapy because surgery might result in serious ischemic or
hemorrhagic complications because DVAs are considered to
be variations of normal transmedullary veins that are necessary for the drainage of white and gray matter.
Keywords Epilepsy . Developmental venous anomaly .
Venous angioma . Pediatry
Š. K. Andrea (*)
Pediatric Department, General County Hospital, Osječka 106,
Požega 34000, Croatia
e-mail: andrea.simic-klaric@po.t-com.hr
Š. K. Andrea : M. Jakov : G. Nikola
Faculty of Medicine Osijek, Josip Juraj Strossmayer University of
Osijek, J. Huttlera 4, Osijek 31000, Croatia
G. Nikola
Department of Radiology, General County Hospital, Osječka 106,
Požega 34000, Croatia

Introduction
Developmental venous anomalies (DVA), formerly known as
venous angiomas, are clusters of radially aligned veins that
converge into a collecting vein, giving them a characteristic
appearance on MRI and angiography, which can be described
as caput medusae. The pathogenesis of this condition is not
completely understood, although it is thought to be a disorder
of normal embryogenetic processes. Pathohistologically,
DVAs are characterized by mature venous walls with minimal
smooth muscle and elastin and without arterial or capillary elements [1].
With a more frequent use of MRI, this anomaly has shown
to be the most common intracranial vascular malformation,
with an incidence between 0.7 and 2.5 % [1, 2], and is in
two third of the cases present supratentorially [1, 3].

Clinical presentation
Since DVA is a benign vascular lesion, it is usually asymptomatic and diagnosed incidentally on brain MRI scans or in
autopsy [4, 5]. Symptoms are often caused by the
coexisting vascular malformations. Cavernous malformations
(CMs) are present with DVAs in 8 to 33 % of cases,
thus being the most common vascular malformation associated with DVA. Coexisting CMs are often the cause
of intracranial hemorrhages (ICH) in patients with a
DVA [1, 6].
When symptomatic, the most common symptoms of DVAs
include headaches, seizures, and focal neurological deficits
due to acute infarction or intracerebral hemorrhages attributed
to DVA thrombosis [1, 4, 5]. It is important to have in mind,
when classifying a DVA as symptomatic, that associated
causes of the symptoms need to be excluded. Possible causes
may include cortical dysplasia as a cause of epilepsy and

Childs Nerv Syst

associated cavernomas as a cause of hemorrhage. Those seizures for which no other causes have been reported are
thought to be related to reduced blood outflow from the
DVA or to small scale neuronal migration deficits that are
undetectable with neuro-radiological methods [2, 4].

Management of DVA
The treatment of symptomatic DVAs is most commonly
symptomatic and includes a pharmacological approach to
dealing with seizures. It is important to note that DVAs are
considered to be variations of normal transmedullary veins
that are necessary for the drainage of white and gray
matter. Since DVAs are considered to be a variation of
the normal venous system, invasive neurosurgical procedures or stereotactic radiosurgery resulting in excision
or obliteration of the lesion may lead to serious ischemic or
hemorrhagic complications [1, 7]. Because of that, neurosurgical treatment is not the first and the best option for the
majority of these patients.

Fig. 1 EEG showing fronto-centro-parietal spikes on the left hemisphere

Illustrative case
A 9-year-old boy was admitted to our hospital following a
seizure. In the morning, after acquiring little sleep caused by
excitement because of the trip he was to attend that morning,
while driving in the car, he suddenly complained of being hot,
vertiginous, and experiencing a frontal headache. His parents
said he was distant, unresponsive, disoriented, and looked to
the side for 15 min. The patient experienced no vomiting or
foaming of the mouth. He did not recall the drive to the hospital. One month prior to the incident, a similar episode occurred in school.
The family history is negative on epilepsy and other neurological diseases, but a few members from the mother’s side
of the family had arrhythmias. Perinatal history was
uneventful. In the neonatal period, a brain ultrasound
showed grades I–II intraventricular hemorrhage. The child’s
psychomotor development was normal, with mild articulation
disorders.
Physical examination at the time of admission showed
no pathology. In the neurological examination, the

Childs Nerv Syst
Fig. 2 MRI showing DVA.
Magnetic resonance of the brain
showing DVA

patient was unstable while performing the Romberg’s
test. Electroencephalogram (EEG) showed left frontocentro-parietal spikes, which occasionally were secondary generalized (Fig. 1).
Brain magnetic resonance imaging (MRI) 3T showed numerous serpiginous hypointensive signals in the superficial cortex posterior to the left postcentral gyrus (Figs. 2, 3, and 4). The
findings are consistent not only with an aggregation of blood
vessels but also with traces of calcification and superficial
hemosiderosis as a sign of previous hemorrhage. Discrete glial
changes were present on the surrounding gyruses. The pineal
gland was enlarged, dimensions 12×6 mm, microcystically

altered, but with no compression to the mesencephalic aqueduct. An extracranial cavernous hemangioma in the left frontal
region was also noticed.
Digital subtraction angiography (DSA) showed a developmental venous anomaly with hypertrophic cortical veins
forming a caput medusae and calcifications. Conclusively,
neuroimaging showed a developmental venous anomaly in
the left centro-parietal part of the brain as well as an extracranial cavernous hemangioma in the left frontal region.
Transcranial color Doppler (TCD) of the circle of Willis
showed an asymmetry with an increased mean flow velocity
in the left anterior and posterior cerebral artery. Mixed scotomas

Fig. 3 DSA showing DVA. Digital subtraction angiography of the brain
showing DVA

Fig. 4 DSA showing DVA. Digital subtraction angiography of the brain
showing DVA

Childs Nerv Syst

in the visual field were also observed. Visual evoked
potentials were normal. Heart ultrasound and Holter
24-h EKG were normal.
In the follow-up, the boy experienced two more seizures
with similar characteristics. He has been treated with
oxcarbazepine 30 mg/kg. No seizures occurred in the last year.
Control MRI scans after a year and 2 years showed no progression of the anomaly.

Discussion
The cases of epilepsy related to DVA were not often described
in children. Our patient exhibited seizures which manifested
with fronto-centro-parietal spikes on EEG. Focal
neuroelectrical surges may be a result of underlying brain
malformations. MRI and DSA showed a DVA which may be
the cause of seizures because of the position consistent with
the abnormal EEG result. The lesion may be associated with a
gliotic scar or it may become hemosiderin-laden and may
contribute to the etiology of epilepsy [1].
Although the DVAs are rarely symptomatic and are usually
an incidental finding, when symptomatic, the most common
symptoms are headaches, seizures, and hemorrhages [1]. No
other changes, such as brain tumors or focal dysplasia, were
detected in the centro-parietal region of our patient. However,
a microcystically altered pineal gland, 12×6 mm, was noticed.
Cysts in the pineal gland are rarely symptomatic when smaller
than 10 mm [8], but those over 10 mm may result in
symptoms which may include paroxysmal generalized
seizures which manifest as a spike-wave complex of
3 Hz or more [9]. The diagnostical findings in our patient suggest that the DVA is the most probable cause
of the seizures.
However, some authors disagree with the idea of DVAs as
the reason for epilepsy [10, 11]. Striano et al. found that seizures occur only in a small percentage of patients with DVA.
Those having epilepsy present with complex partial seizures,
as it was the case with our patient.
DVAs are considered to be a variation of the normal venous
system, so the surgical excision is, therefore, a risk factor for
the development of serious postoperative complications,
including venous infarction and cerebral edema,
resulting from the brain being deprived of normal

venous drainage [1, 6]. So far, our patient responded
well to anticonvulsant treatment.

Conclusion
Epilepsy may be caused by developmental venous anomalies.
The treatment of DVA-related epilepsy starts with anticonvulsants. The surgical treatment is the therapeutic choice only if
seizures are resistant to conservative therapy because surgery
might result in serious ischemic or hemorrhagic complications.

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