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2016 ; 13
(4
): e31116
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Liver Findings in Patients with Hereditary Hemorrhagic Telangiectasia
#MMPMID27895866
Song W
; Zhao D
; Li H
; Ding J
; He N
; Chen Y
Iran J Radiol
2016[Oct]; 13
(4
): e31116
PMID27895866
show ga
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT), also known as
Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular
dysplasia. OBJECTIVES: The aim is to characterize the liver involvement of HHT by
multi-detector helical computed tomography. PATIENTS AND METHODS: Through the use
of multi-detector helical computed tomography, scan data derived from 12 cases of
HHT were retrospectively studied, and an abdominal scan was performed on all of
the patients. RESULTS: (i) Three types of shunting were found in the livers,
including arteriovenous (hepatic artery to hepatic vein) in 6 cases,
arterioportal (hepatic artery to portal vein) in 2 cases, and portal venous
(portal vein to hepatic vein) in 4 cases, (ii) Biliary diseases were detected in
8 cases, including 3 cases of bilomas accompanied by increases of alkaline
phosphatase (mean 271 units, ranging from 152 to 479 units) and arteriovenous
shunts, and the other 5 cases revealed slight biliary dilation with no increase
of alkaline phosphates, but were instead accompanied by arteriovenous shunts (3
cases) and portal venous shunts (2 cases). (iii) Vascular anatomic variants were
observed in 4 cases, including 3 with accompanying arteriovenous shunts and 1
with accompanying arterioportal shunting. CONCLUSION: The involved liver with HHT
typically shows vascular shunting and biliary diseases. Also, arteriovenous
shunts may be vulnerable to biliary diseases.