TY - JOUR
T1 - Internal cerebral veins varix due to midline arteriovenous malformation: how much is the risk of bleeding?
AU - Sturiale, Cl
AU - Puca, Alfredo
AU - Albanese, Alessio
AU - Marchese, Enrico
AU - Maira, Giulio
PY - 2011
Y1 - 2011
N2 - Springer-Verlag 2011
Sir,
We report a case of 40-year-old sportsman admitted to our
department for a generalized seizure associated with
bladder and bowel incontinence occurred during exercise.
The patient quickly regained consciousness without postcritical
deficits. Neurological examination appeared completely
normal. A head computer tomography (CT) scan
showed a midline arteriovenous malformation (AVM)
without the evidence of bleeding (Fig. 1a, b). A digital
subtraction angiography revealed a dysplastic nidus fed by
branches of bilateral internal–external carotid and vertebrobasilar
arteries, and draining both in the superficial and
deep venous system, which showed varicose dilatation with
Galen’s vein stenosis (Fig. 1c–g).
Cerebral varix is defined as an ectasia which doubles
vein diameter. It can be due to an arteriovenous shunt and
appears more common in patients with direct fistulas, than
in cerebral AVM with dysplastic nidi [1]. Differently from
those associated with Galen’s vein fistulae, huge intracranial
varix associated with cerebral AVMs were seldom
reported. Headache, bruit, focal mass effect and hydrocephalus
are common presentation symptoms [2]. Intracranial
haemorrhage has been instead less often reported at
clinical onset [3]. In the most of the cases of giant varix,
direct arteriovenous communications in the context of
nidus were observed, especially between feeding arteries
and the varicose veins [4]. This discrete angiographic
finding separate these vascular malformations from the
classical and more common AVMs in which an abnormal
vascular network is identified between the arterial feeders
and draining veins [4]. Venous hypertension with retrograde
drainage often results in focal neurological deficits
and seizures [1]. Although it is commonly assumed that
venous ectasia increases the bleeding risk, varix haemorrhagic
presentation has been rarely reported [4]. It is may
be due to the progressive thickening of the arterialized
veins wall, with intimal proliferation of synthetic smoothmuscle
cells and disappearance of the internal elastic
lamina, that make arterialized veins not greatly prone to
rupture [1, 4].
On the other hand, instead, the presence of venous varix
on the draining veins has been recognized as a factor
increasing the risk of haemorrhage in dural arteriovenous
fistulae [5].
In the case reported here, the presence of multiple
feeders causing high flow shunt rate, direct drainage in a
cisternal venous system, and an impaired venous outlet due
to the Galen’s vein stenosis, may have contributed to the
varix formation. Prolonged sporting activity, instead, has
not been a factor influencing AVM rupture.
AB - Springer-Verlag 2011
Sir,
We report a case of 40-year-old sportsman admitted to our
department for a generalized seizure associated with
bladder and bowel incontinence occurred during exercise.
The patient quickly regained consciousness without postcritical
deficits. Neurological examination appeared completely
normal. A head computer tomography (CT) scan
showed a midline arteriovenous malformation (AVM)
without the evidence of bleeding (Fig. 1a, b). A digital
subtraction angiography revealed a dysplastic nidus fed by
branches of bilateral internal–external carotid and vertebrobasilar
arteries, and draining both in the superficial and
deep venous system, which showed varicose dilatation with
Galen’s vein stenosis (Fig. 1c–g).
Cerebral varix is defined as an ectasia which doubles
vein diameter. It can be due to an arteriovenous shunt and
appears more common in patients with direct fistulas, than
in cerebral AVM with dysplastic nidi [1]. Differently from
those associated with Galen’s vein fistulae, huge intracranial
varix associated with cerebral AVMs were seldom
reported. Headache, bruit, focal mass effect and hydrocephalus
are common presentation symptoms [2]. Intracranial
haemorrhage has been instead less often reported at
clinical onset [3]. In the most of the cases of giant varix,
direct arteriovenous communications in the context of
nidus were observed, especially between feeding arteries
and the varicose veins [4]. This discrete angiographic
finding separate these vascular malformations from the
classical and more common AVMs in which an abnormal
vascular network is identified between the arterial feeders
and draining veins [4]. Venous hypertension with retrograde
drainage often results in focal neurological deficits
and seizures [1]. Although it is commonly assumed that
venous ectasia increases the bleeding risk, varix haemorrhagic
presentation has been rarely reported [4]. It is may
be due to the progressive thickening of the arterialized
veins wall, with intimal proliferation of synthetic smoothmuscle
cells and disappearance of the internal elastic
lamina, that make arterialized veins not greatly prone to
rupture [1, 4].
On the other hand, instead, the presence of venous varix
on the draining veins has been recognized as a factor
increasing the risk of haemorrhage in dural arteriovenous
fistulae [5].
In the case reported here, the presence of multiple
feeders causing high flow shunt rate, direct drainage in a
cisternal venous system, and an impaired venous outlet due
to the Galen’s vein stenosis, may have contributed to the
varix formation. Prolonged sporting activity, instead, has
not been a factor influencing AVM rupture.
KW - Adult
KW - Angiography, Digital Subtraction
KW - Carotid Artery Diseases
KW - Humans
KW - Intracranial Arteriovenous Malformations
KW - Male
KW - Tomography, X-Ray Computed
KW - Varicose Veins
KW - Adult
KW - Angiography, Digital Subtraction
KW - Carotid Artery Diseases
KW - Humans
KW - Intracranial Arteriovenous Malformations
KW - Male
KW - Tomography, X-Ray Computed
KW - Varicose Veins
UR - http://hdl.handle.net/10807/3364
U2 - 10.1007/s10072-011-0784-0
DO - 10.1007/s10072-011-0784-0
M3 - Article
SN - 1590-3478
VL - 32
SP - 1253
EP - 1254
JO - Neurological Sciences
JF - Neurological Sciences
ER -