Rendu-Osler-Weber Disease with a
Giant Intracerebral Varix
Secondary to a High-Flow Pial AVF:
Case Report
Masashi Oda, M.D.,* Jun A. Takahashi, M.D.,* Nobuo Hashimoto, M.D.,* and
Tsunemaro Koyama, M.D.†
*Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto,
Japan; and †Ohtsu Municipal Hospital, Ohtsu, Japan

Oda M, Takahashi JA, Hashimoto N, Koyama T. Rendu-OslerWeber disease with a giant intracerebral varix secondary to a
high-flow pial AVF: case report. Surg Neurol 2004;61:353– 6.
BACKGROUND

Intracranial varices are rare and most are associated with
vein of Galen arteriovenous malformations (AVM) or fistulas (AVF).
DESCRIPTION

A 43-year-old left-handed man presented with right hemihypesthesia and spastic gait. Neuroradiological examination revealed a spinal AVF and a giant intracerebral varix
associated with a high-flow pial AVF. He had recurrent
episodes of nasal bleeding, which were also confirmed in
his mother’s medical history, and telangiectases in the tip
of his tongue and fingers. He was diagnosed with RenduOsler-Weber disease. After resection of the spinal AVF
that produced his symptoms, we surgically exposed and
obliterated the giant varix and AVF under intra- and postoperative hypotension and mild barbiturate therapy. The
arteriovenous shunt was completely obliterated without
hyperperfusion of the surrounding brain.
CONCLUSION

This is an extremely rare case of Rendu-Osler-Weber disease with a giant intracerebral varix secondary to a highflow pial AVF that did not involve the vein of Galen. ©
2004 Elsevier Inc. All rights reserved.
KEY WORDS

Giant varix, pial AVF, Rendu-Osler-Weber disease, spinal
AVF.

ntracranial varices are very rare, and most of
them are secondary to high-flow arteriovenous
shunting related to a Galen arteriovenous malformation (AVM) or fistula (AVF) in children and
young adults [7]. We experienced a middle-aged

I

Address reprint requests to: Jun A. Takahashi, M.D., Department of
Neurosurgery, Kyoto University Graduate School of Medicine, 54 ShogoinKawahara-cho, Sakyo-ku, Kyoto 606 – 0857, Japan.
Received December 23, 2002; accepted May 22, 2003.
© 2004 Elsevier Inc. All rights reserved.
360 Park Avenue South, New York, NY 10010 –1710

patient with Rendu-Osler-Weber disease, a rare hereditary disease with a triad of mucocutaneous and
visceral telangiectasia; recurrent nasal bleeding;
and familial autosomal dominant inheritance [3,4].
This patient had a spinal AVF and a giant intracerebral varix secondary to a cerebral AVF, which did
not involve the Galenic venous system.

Case Report
This 43-year-old left-handed man had experienced
recurrent episodes of epistaxis, which were also
confirmed in his mother’s medical history, and telangiectases in the tip of his tongue (Figure 1) and
fingers. One year before his current admission, he
had presented elsewhere with numbness of the
right upper and lower extremities and gait disturbance; he had noticed hypesthesia of his right
lower extremity 4 months earlier.
On admission at Ohtsu Municipal Hospital, a routine craniogram showed an abnormal calcification
around the posterior temporal region. Magnetic resonance images (MRI) revealed coupled lesions of a
spinal AVF (Figure 1) and an intracerebral varix associated with a high-flow pial AVF (Figure 2). C3-T5 laminectomy was performed, exposing a premedullary
AVF fed by the right T7 and right T5 intercostal artery.
The feeders were occluded with clips. After the spinal
AVF was surgically obliterated in Ohtsu Municipal
Hospital, he was admitted to Kyoto University Hospital for treatment of the intracranial lesion.
On admission at Kyoto University Hospital, the
patient had right upper quadrantanopia that was
caused by the cerebral lesions. The following symptoms and signs were due to the spinal lesion itself
and the previous spinal operation; sensory exami0090-3019/04/$–see front matter
doi:10.1016/j.surneu.2003.05.001

354 Surg Neurol
2004;61:353– 6

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Masashi et al

Left: Photograph showing telangiectasis of the tip of the tongue. Right: Photograph illustrating a spinal AV fistula l in
the thoracic spinal cord, which was fed by the right seventh intercostal artery and the left fifth intercostal artery.

nations revealed bilateral hypesthesia and right
hemithermohypesthesia and hemihypalgesia below
the L1 level. He manifested mild motor weakness of

the left lower extremity and hyperreflexia of the
bilateral patellae and Achilles tendons. In addition,
he exhibited bilateral patellar and ankle clonus.

Preoperative angiograms, lateral view. Upper left: Arterial phase. In the right intercarotid artery angiogram, the
angular middle cerebral artery flows into a giant varix. Upper right: Capillary phase. Lower left: Venous phase. The
drainer flows through the varix and into the transverse sinus junction. A preoperative PD-weighted MRI (lower right):
a dilated feeding artery, a varix, and a drainer are demonstrated as signal void.

2

Rendu-Osler-Weber Disease with a Pial AVF

Surg Neurol 355
2004;61:353– 6

A postoperative angiogram, lateral view (left): Arterial phase. The varix and AVF are no longer seen. The right
anterior cerebral artery is anterogradely filled from the right internal carotid artery. A postoperative CT image
(right): The drainers are thrombosed.

3

MRI revealed a dilated feeding artery, a varix, and
a dilated drainer as flow-void structures (Figure 2).
A right internal carotid angiogram demonstrated a
parenchymal AVF draining into a giant varix. The
feeding artery was the enlarged angular branch of
the right middle cerebral artery. The dilated draining veins flowed through the varix and drained directly into the left transverse sinus. The lesion did
not involve a vein of Galen. The anterior cerebral
artery was not opacified because of the high-flow
arteriovenous shunt into the varix.
Right temporoparietooccipital craniotomy was
performed, exposing a giant intracerebral varix and
pial AVF under intraoperative hypotension and mild
barbiturate therapy. Tentative clipping of the
feeder just proximal to its entrance into the varix
resulted in its complete collapse. After the enlarged
feeding artery was dissected, a fistula draining into
the varix was confirmed. Complete obliteration of
the varix and AVF and the absence of any other
feeders into the varix were documented on intraoperative angiograms. Although intraoperative monitoring with a laser flowmeter indicated a slight increase in the surface cerebral blood flow, we
confirmed that there was no brain swelling or hyperemic change of the brain surface. We then permanently ligated the feeder and cut it. His arterial
blood pressure was maintained at around 100 mm
Hg during and for 2 days after the operation. He
awoke on the day following the procedure from the
mild barbiturate therapy and demonstrated no neurologic worsening.
Postoperative computed tomography (CT) images
showed a thrombosed drainer, and on angiograms
there was no opacification of the AVF, varix, or right

transverse sinus (Figure 3). The right anterior and
middle cerebral arteries were filled antegradely on
angiograms. The patient was discharged without any
postoperative neurologic deterioration.

Discussion
McCormick [5] classified vascular malformations
into telangiectases, varices, cavernous malformations, venous angiomas, and arteriovenous malformations. Among them, giant intracranial varices are
very rare and most are secondary to vein of Galen
AVM or fistulas [7]. Only a few previous reports
documented cerebral AVF associated with RenduOsler-Weber disease without involvement of the
vein of Galen. Rendu-Osler-Weber disease is a rare
hereditary disease with a triad of mucocutaneous
and visceral telangiectasia and recurrent epistaxis
[1,2– 4]. Our patient had recurrent episodes of nasal
bleeding, which were also confirmed in his mother’s medical history, and telangiectases on the tip of
his tongue and fingers. In addition, he had an intracerebral varix associated with a pial AVF and a
spinal AVM. Therefore, based on his clinical manifestations and family history, we concluded that his
intracranial and spinal vascular lesions constituted
Rendu-Osler-Weber disease [3,4].
The treatment of intracranial high-flow AV shunts
is difficult. Their endovascular occlusion carries the
risk of embolizing the varix and compromising the
venous outlet of the arteriovenous shunt. Reportedly, the strong flow in the arterial feeders washes
away the embolic materials such as detachable balloons and coils [1–3,7], some of which are lost and

356 Surg Neurol
2004;61:353– 6

Masashi et al

migrate into the lung [7]. In addition, the sudden
occlusion of the high-flow feeders may produce significant local cerebrovascular changes. This may
result in acute brain swelling and/or intracerebral
hemorrhage due to the sudden diversion of the
rapid blood flow into vessels in the surrounding
brain that may not be able to contain the pressure
[6,7]. Because multiple feeders are usually involved
in arteriovenous shunts, high-flow AVF should be
occluded in stages. In our case, the high-flow AVF
arose from a single feeder, and we were able to treat
it safely in a one-stage operation.
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3. Kikuchi K, Kowada M, Tomura N, Johkura H. Hereditary hemorrhagic telangiectasia associated with cerebral arteriovenous fistula and multiple cerebral arteriovenous malformations: case report. No Shinkei Geka
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4. Garcia-Monaco R, Taylor W, Rodesch G, et al. Pial
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majority of respondents (65%) said that if they were starting
their practice today, they would not take part in Medicare,
and their predictions about Medicare reflect that. When asked their
“expectations for Medicare-age patients 10 years from now,” 67%
predict more doctors and patients opting out of the system; 58%,
severely rationed care; 36%, complete collapse of the system; and
13%, active euthanasia. Only 9% predict that seniors will receive
the same treatment as they do today.

A

—Journal of American Physicians and Surgeons
Volume 8 Number 4 Winter 2003