AJR 2001; 176:919-920
© American Roentgen Ray Society
Osler-Weber-Rendu Disease
Visualizing Portovenous Shunting with Three-Dimensional Sonography
Masayuki Matsuo1,
Masayuki Kanematsu,
Hiroki Kato,
Hiroshi Kondo,
Keiko Sugisaki and
Hiroaki Hoshi
1
All authors: Department of Radiology, Gifu University School of Medicine, 40
Tsukasamachi, Gifu 500-8705, Japan.
Received July 27, 2000;
accepted after revision September 28, 2000.
Address correspondence to M. Kanematsu.
Introduction
Osler-Weber-Rendu disease, also referred to as hereditary hemorrhagic
telangiectasia, is an autosomal dominant disorder characterized with
telangiectasis, aneurysms, and arteriovenous malformations. The reported
prevalence of this disorder is 1-2 cases per 100,000 population. The patient's
skin, mucosa, and blood vessels of the lung, liver, and central nervous system
can be affected [1]. Although
hepatic involvement used to be confirmed by biopsy, necropsy, or angiography
[2], newer, less invasive
techniques such as sonography, CT, and MR imaging have been used to detect
hepatic vascular malformations even in the clinically silent stage of the
disease [3]. More recently,
three-dimensional (3D) sonography has provided a sensitive and noninvasive
method for examining the hepatic vasculature for disease and for confirming
hepatic vascular malformations
[4]. We describe a patient with
Osler-Weber-Rendu disease in whom 3D sonograms reconstructed from power
Doppler sonography were useful in establishing the diagnosis.
Case Report
A 59-year-old woman who complained of upper abdominal pain underwent
contrast-enhanced CT at our hospital and was suspected of having multiple
vascular malformations in the liver. She was hospitalized in our department
for liver examination. She had episodes of multiple petechiae in the tongue,
fingertips, and skin. She had no history of heart failure or hepatic
encephalopathy. Laboratory data showed slight abnormalities in liver function,
an elevated serum ammonia level of 177 mg/dL, and no serologic evidence of
type-B or type-C viral hepatitis.
Triple-phasic contrast-enhanced helical CT (HiSpeed Advantage; General
Electric Medical Systems, Milwaukee, WI) showed multiple patchy early
sinusoidal filling in the liver and early opacified hepatic veins in the
hepatic arterial phase as well as poorly opacified hepatic parenchyma and
dilated portal and hepatic veins in the portal venous phase. However, CT did
not clearly reveal the whole connection of the portovenous shunting.
The patient underwent further transabdominal sonography with gray-scale,
color Doppler, and power Doppler studies (SSD-5500; Aloka, Tokyo, Japan) with
a 3.75-MHz convex transducer. Gray-scale sonography first revealed moderately
dilated hepatic arteries and markedly dilated portal and hepatic veins. The
echogenicity of the hepatic parenchyma was normal. We then performed color
Doppler sonography and confirmed that the right posteroinferior portal vein
and right hepatic vein branches were dilated; these two vessels were connected
in the periphery (Fig. 1A). We
recognized the fistula as portovenous shunting and performed power Doppler
sonography to acquire source images for reconstruction of 3D sonograms. The
acquisition was done through the right hypochondrial area during 10 sec of
breath-holding. The unshaded volume-rendering algorithm was used for the image
reconstruction. The whole connection of portovenous shunting of
Osler-Weber-Rendu disease was revealed by 3D sonography
(Fig. 1B).
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Fig. 1A. —59-year-old woman with Osler-Weber-Rendu disease. Color
Doppler sonogram shows posteroinferior portal vein branch (solid
arrow) as dilated in periphery. Note dilated right hepatic vein branch
(curved arrow). Together these branches form portovenous fistula
(open arrow) in periphery.
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Fig. 1B. —59-year-old woman with Osler-Weber-Rendu disease.
Three-dimensional sonogram shows dilated posteroinferior portal vein branch
(solid arrow), right hepatic vein branch (curved arrow), and
exact site of portovenous shunting (open arrow).
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Finally, the patient underwent digital subtraction hepatic angiography with
a femoral Seldinger percutaneous approach. The hepatic arteriograms showed
dilated hepatic arteries, multiple patchy early sinusoidal filling during the
early arterial phase (Fig. 1C), and subtly opacified hepatic veins in the late arterial phase, but they did
not show the portal vein branches. The transarterial portograms revealed
poorly opacified liver parenchyma and early opacified dilated hepatic veins
but failed to reveal the exact site of portovenous shunting
(Fig. 1D). The final diagnosis
of Osler-Weber-Rendu disease was established with the radiologic imaging and
clinical inspection findings. We also confirmed no other organ involvement
caused by the disease.
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Fig. 1D. —59-year-old woman with Osler-Weber-Rendu disease. Digital
subtraction transarterial portogram shows posteroinferior portal vein branch
(large arrow) that is dilated in periphery. Note poor opacification
of hepatic parenchyma and early opacification of hepatic veins (curved
arrows) and right atrium (asterisk).
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Discussion
Osler-Rendu-Weber disease has multiple manifestations. Increased hepatic
blood flow through the arteriovenous or portovenous fistulas in the liver may
cause hepatomegaly, with the patient presenting with right upper quadrant
pain. Left-to-right shunting through the hepatic vascular fistulas may lead to
high-output cardiac failure
[5]. Rarely, hepatic
arterioportal shunting may lead to portal hypertension, with the patient
presenting with gastrointestinal variceal rupture or hepatic encephalopathy
[6].
Researchers making gray-scale sonographic observations in patients with
Osler-Rendu-Weber disease have reported marked dilatation of the hepatic
artery and its branches, a condition that produces images of multiple tubular
structures with echogenic walls within the liver, although gray-scale
sonography has limited capability in revealing vascular malformations
[7]. Previous reports have
described the high sensitivity of hepatic vascular abnormalities with Doppler
sonography, and hepatic vascular involvements in Osler-Weber-Rendu disease can
be detected with Doppler sonography
[8]. Although two-dimensional
(2D) color Doppler sonography helped us to detect portovenous shunting in our
patient, it could not reveal objectively the dimension of this shunting.
Three-dimensional sonography has recently gained much attention in the
field of obstetrics, and abdominal, pelvic, cardiac, ocular, or vascular
applications are expected as well
[4]. Although each sonographer
must become accustomed to operating the 3D sonography system, its
computer-generated image acquisition and display are expected to aid
sonologists in recognizing the dimension of disease in multiple planes,
thereby increasing their certainty of their findings or diagnoses. However, we
used 2D color Doppler sonography instead of 3D sonography as the primary means
of detecting the portovenous shunting because the source image acquisition and
reconstruction took only a few minutes. At present, it may be more practical
to use real-time 2D Doppler sonography for legion detection and 3D sonography
for lesion visualization.
Although 3D CT angiography is another way to reveal vascular malformations,
this method requires a source image acquisition with dedicated methodology for
IV contrast material injection and scanning. Precise extraction of portal and
hepatic veins in the liver—where strong parenchymal enhancement takes
place during the portal venous phase—is often difficult even if a
reconstruction algorithm is used. On the other hand, 3D sonography does not
require contrast administration and offers better differentiation of contrast
enhancement between vessels and liver parenchyma.
In summary, our experience with this patient suggests that Doppler
sonography combined with reconstructed 3D sonography is useful as a practical
imaging technique for the visualization and evaluation of vascular
malformations in Osler-Rendu-Weber disease. The combination of imaging
techniques allows depiction of healthy and diseased vasculature and thus helps
sonologists to recognize the extent of disease in three dimensions and to make
clinical diagnosis with increased confidence.
Acknowledgments
We thank Sachiko Hazeyama of Aloka Company, Japan, for technical
advice.
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Introduction
Case Report
