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Case Reports
. 2024 Jun;17(3):477-483.
doi: 10.1007/s12328-024-01923-0. Epub 2024 Mar 4.

Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage

Satoru Hagiwara  1 , Toru Takase  2 , Itsuki Oda  3 , Yoriaki Komeda  4 , Naoshi Nishida  4 , Akihiro Yoshida  4 , Tomoki Yamamoto  4 , Takuya Matsubara  4 , Masatoshi Kudo  4
Affiliations
Case Reports

Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage

Satoru Hagiwara et al. Clin J Gastroenterol. 2024 Jun.

Abstract

A 53-year-old woman was diagnosed with liver dysfunction in August 20XX. Computed tomography (CT) revealed multiple hepatic AV shunts, and she was placed under observation. In March 20XX + 3, she developed back pain, and CT performed during an emergency hospital visit showed evidence of intrahepatic bile duct dilatation. She was referred to our gastroenterology department in May 20XX + 3. We conducted investigations on suspicion of hereditary hemorrhagic telangiectasia (HHT) with hepatic AV shunting based on contrast-enhanced CT performed at another hospital. HHT is generally discovered due to epistaxis, but there are also cases where it is diagnosed during examination of liver damage.

Keywords: Hepatic arteriovenous shunt; Hereditary hemorrhagic telangiectasia; Liver damage.

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Conflict of interest statement

Masatoshi Kudo received patent royalties from Chugai Pharmaceutical Co., Ltd. Masatoshi Kudo received scholarship grants from Chugai Pharmaceutical Co., Ltd., TAIHO Pharmaceutical Co., Ltd., Otsuka Pharmaceutical Co., Ltd., GE Healthcare Japan Corporation and Eisai Co., Ltd.

Figures

Fig. 1
Fig. 1
Abdominal ultrasonography (May 20XX + 3) a In the B-mode, arteriovenous anastomosis is observed in the right lobe of the liver. b Arteriovenous anastomoses are also observed on color Doppler imaging. cDilated and tortuous arteries are observed. Moreover, the right hepatic vein (RHV) appears significantly expanded by 12 mm and the middle hepatic vein (MHV) by 12.4 mm. d Contrast-enhanced ultrasound shows a bile duct dilated to 8 mm in liver S6.
Fig. 2
Fig. 2
Contrast-enhanced computed tomography (June 20XX + 3) a Marked intrahepatic dilation of the hepatic vein (HV) and inferior vena cava (IVC) is observed during the arterial phase. b Dilated and tortuous arteries are observed in both lobes of the liver. c In the equilibrium phase, dilated bile ducts can be seen in both lobes of the liver. d No arteriovenous malformations were found in the lungs.
Fig. 3
Fig. 3
Upper endoscopy (May 20XX + 3) Scattered telangiectasia is evident mainly in the gastric antrum, in addition to the gastric mucosal findings associated with HHT.
Fig. 4
Fig. 4
Head MRA examination (June 20XX + 3) a High-signal intensity is observed in the bilateral globus pallidus on T1-weighted imaging. b MRA shows decreased signal intensity in the left internal carotid artery (C3), suggesting the possibility of stenosis. MRA magnetic resonance angiography
See this image and copyright information in PMC

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References

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