Imaging of Hereditary Hemorrhagic Telangiectasia
Jeff Jaskolka1,
Louis Wu1,
Raymond P. Chan1 and
Marie E. Faughnan2
1 Department of Medical Imaging, Toronto HHT Centre, St. Michael's Hospital,
University of Toronto, 30 Bond St.,Toronto, ON M5B 1W8, Canada.
2 Department of Medicine, Toronto HHT Centre, St. Michael's Hospital, University
of Toronto, ON M5B 1W8, Canada.
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Fig. 1A. —46-year-old woman with hereditary hemorrhagic telangiectasia
and multiple pulmonary arteriovenous malformations. Posteroanterior chest
radiograph shows well-defined lingular nodule with adjacent tubular opacity
(arrowhead) representing aneurysm and draining vein of pulmonary
arteriovenous malformation.
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Fig. 1B. —46-year-old woman with hereditary hemorrhagic telangiectasia
and multiple pulmonary arteriovenous malformations. Lateral chest radiograph
shows well-defined lingular nodule with adjacent tubular opacity
(arrowhead) representing aneurysm and draining vein of pulmonary
arteriovenous malformation. Second pulmonary arteriovenous malformation
(arrow) is faintly seen at base of right lung.
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Fig. 1C. —46-year-old woman with hereditary hemorrhagic telangiectasia
and multiple pulmonary arteriovenous malformations. Right pulmonary angiogram
shows complex basal pulmonary arteriovenous malformations (arrows).
Note presence of three separate segmental feeding arteries.
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Fig. 2A. —58-year-old woman with hereditary hemorrhagic telangiectasia
that caused decreased exercise tolerance and cyanosis. Unenhanced thoracic CT
scan shows pulmonary arteriovenous malformation (arrow) in right
lower lobe.
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Fig. 2B. —58-year-old woman with hereditary hemorrhagic telangiectasia
that caused decreased exercise tolerance and cyanosis. Unenhanced thoracic CT
scan obtained at lower level than A shows multiple bilateral pulmonary
arteriovenous malformations (arrows).
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Fig. 2C. —58-year-old woman with hereditary hemorrhagic telangiectasia
that caused decreased exercise tolerance and cyanosis. Superselective right
pulmonary angiogram obtained using 5-French catheter shows pulmonary
arteriovenous malformation with simple angioarchitecture in right lower lobe.
Note presence of embolization coils (arrow) from prior treatment of
different arteriovenous malformation.
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Fig. 3A. —44-year-old woman with hereditary hemorrhagic telangiectasia
and lingular pulmonary arteriovenous malformation. Left pulmonary angiogram
shows large solitary lingular pulmonary arteriovenous malformation. Note
feeding artery (white arrowhead), aneurysm sac (arrow), and
draining vein (black arrowhead).
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Fig. 3B. —44-year-old woman with hereditary hemorrhagic telangiectasia
and lingular pulmonary arteriovenous malformation. Angiogram obtained after
coil embolization (arrowhead) shows absence of flow through pulmonary
arteriovenous malformation.
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Fig. 4A. —45-year-old man with hereditary hemorrhagic telangiectasia
and reperfused pulmonary arteriovenous malformation. Initial left pulmonary
angiogram shows large pulmonary arteriovenous malformation in lower lobe.
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Fig. 4B. —45-year-old man with hereditary hemorrhagic telangiectasia
and reperfused pulmonary arteriovenous malformation. Angiogram obtained
immediately after embolization shows coils occluding feeding vessel and no
further opacification of pulmonary arteriovenous malformation.
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Fig. 4C. —45-year-old man with hereditary hemorrhagic telangiectasia
and reperfused pulmonary arteriovenous malformation. Pulmonary angiogram
obtained 2 years after B shows subtle contrast opacification
(arrow) of aneurysm in previously treated pulmonary arteriovenous
malformation.
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Fig. 4D. —45-year-old man with hereditary hemorrhagic telangiectasia
and reperfused pulmonary arteriovenous malformation. Superselective angiogram
more clearly shows aneurysm and draining vein perfusion beyond previously
placed coils.
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Fig. 5A. —58-year-old man with hereditary hemorrhagic telangiectasia
and remote history of stroke, spontaneous hemothorax, and positive findings on
contrast-enhanced echocardiogram for large pulmonary arteriovenous
malformation. Initial four-chamber echocardiogram obtained after contrast
injection of agitated saline shows normal finding of hyperechoic bubbles
(asterisk) in right atrium and ventricle.
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Fig. 5B. —58-year-old man with hereditary hemorrhagic telangiectasia
and remote history of stroke, spontaneous hemothorax, and positive findings on
contrast-enhanced echocardiogram for large pulmonary arteriovenous
malformation. Corresponding delayed echocardiogram shows bubbles resulting
from intrapulmonary shunt in left atrium and ventricle (LV).
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Fig. 6. —36-year-old previously healthy man with right cerebellar
infarct. Subsequent investigation confirmed presence of pulmonary
arteriovenous malformation. Unenhanced axial CT scan obtained 2 days after
onset of symptoms shows diffuse hypoattenuation of right cerebellar hemisphere
due to infarction. Note hydrocephalus due to compression of fourth
ventricle.
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Fig. 7. —30-year-old man with hereditary hemorrhagic telangiectasia
and pulmonary arteriovenous malformation complicated by cerebral abscess.
Enhanced axial CT scan shows ring-enhancing abscess in left frontal lobe with
vasogenic edema and mild subfalcine herniation.
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Fig. 8A. —25-year-old man with hereditary hemorrhagic telangiectasia
and large cerebral arteriovenous malformation. Axial fast spin-echo
T2-weighted image shows cerebral arteriovenous malformation (arrow)
with heterogeneous signal intensity in left frontoparietal region. Note
prominent tubular flow void (arrowhead) anterior to malformation
corresponding to draining vein.
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Fig. 8B. —25-year-old man with hereditary hemorrhagic telangiectasia
and large cerebral arteriovenous malformation. Lateral projection of left
internal carotid angiogram shows large aneurysm sac (white arrow)
with enlarged, tortuous feeding arteries (white arrowheads) and
shunting into dilated cortical veins (black arrowheads) and superior
sagittal sinus (black arrow).
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Fig. 9A. —9-year-old boy presenting with subarachnoid hemorrhage
secondary to spinal arteriovenous malformation. Hereditary hemorrhagic
telangiectasia was subsequently diagnosed. Sagittal spin-echo T1-weighted
image shows numerous serpiginous flow voids (arrow) posterior to
spinal cord.
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Fig. 9B. —9-year-old boy presenting with subarachnoid hemorrhage
secondary to spinal arteriovenous malformation. Hereditary hemorrhagic
telangiectasia was subsequently diagnosed. Anterior intercostal angiogram
obtained at level of T10 vertebra confirms presence of spinal arteriovenous
malformation (arrow) draining into dilated, tortuous medullary veins
seen on MR image (A).
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Fig. 10A. —61-year-old man with hereditary hemorrhagic
telangiectasia–related liver disease. Sonogram obtained through right
lobe of liver shows markedly enlarged and tortuous hepatic artery
(arrow).
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Fig. 10B. —61-year-old man with hereditary hemorrhagic
telangiectasia–related liver disease. Pulsed Doppler sonogram shows
increased velocity and flow volume in hepatic artery. Spectral analysis shows
aberrant waveform consistent with decreased peripheral resistance.
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Fig. 10C. —61-year-old man with hereditary hemorrhagic
telangiectasia–related liver disease. Hepatic angiogram confirms
dilated, tortuous hepatic artery and diffuse telangiectases.
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Fig. 11A. —68-year-old man with hereditary hemorrhagic telangiectasia
and duodenal arteriovenous malformation. Early image from superior mesenteric
angiography shows dilated, tortuous pancreaticoduodenal arteries
(arrow) supplying duodenal arteriovenous malformation.
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Fig. 11B. —68-year-old man with hereditary hemorrhagic telangiectasia
and duodenal arteriovenous malformation. Later image from same examination as
A shows dilated, tortuous pancreaticoduodenal arteries (arrow)
supplying duodenal arteriovenous malformation. Note early filling of vein
(arrowhead) resulting from arteriovenous shunting.
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Fig. 12A. —68-year-old woman with hereditary hemorrhagic telangiectasia
and anemia related to gastrointestinal involvement. Contrast-enhanced CT scan
obtained at level of pancreas shows multiple ill-defined, hyperattenuating
foci (arrows) within pancreatic parenchyma.
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Fig. 12B. —68-year-old woman with hereditary hemorrhagic telangiectasia
and anemia related to gastrointestinal involvement. Splenic angiogram confirms
presence of multiple pancreatic arteriovenous malformations
(arrows).
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Fig. 12C. —68-year-old woman with hereditary hemorrhagic telangiectasia
and anemia related to gastrointestinal involvement. Inferior mesenteric
angiogram shows colonic arteriovenous malformation (arrow) arising
from branch of sigmoid artery.
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Fig. 12D. —68-year-old woman with hereditary hemorrhagic telangiectasia
and anemia related to gastrointestinal involvement. Superior mesenteric
angiogram shows arteriovenous malformations at hepatic flexure supplied by
branch of right colic artery.
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Copyright © 2004 by the American Roentgen Ray Society.
