Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: imaging findings.

Buscarini, Elisabetta; Buscarini, L; Civardi, Giuseppe; Arruzzoli, S; Bossalini, G; Piantanida, M.

doi:10.2214/ajr.163.5.7976883

Cited by 106 publications

(92 citation statements)

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“…Another case was reported of a newborn with a shunt with spontaneous resolve in first year of age (8,13). SIPSVS may be demonstrated in any age of life between 20-day-old and late seventies (3,5,12,14,18,19,20,22,24,26,31). Wide range distribution in case reports may make us speculate role of different mechanism in development of SIPSVS.…”

Section: Discussionmentioning

confidence: 94%

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

Filik¹,

Boyacioglu

2006

Acta Med. (Hradec Kralove, Czech Repub.)

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Spontaneous intrahepatic portosystemic venous shunt (SIPSVS) is relatively rare and not well recognized. Herein, we report 75-year-old female of an aneurysmal portosystemic venous shunt detected by colour Doppler ultrasound in check-up examination. A direct vascular communication between left portal vein and middle hepatic vein was confirmed by CT-angiography. The cause of intrahepatic portosystemic venous shunt is disputed. This abnormality, mainly described in cirrhotic liver and rarely in healthy liver, is usually revealed by hepatic encephalopathy or glycoregulation disorders. However, with improvements in imaging the number of reports of SIPSVS identified incidentally in patients without definite symptoms increasing.

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Section: Discussionmentioning

confidence: 94%

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

Filik¹,

Boyacioglu

2006

Acta Med. (Hradec Kralove, Czech Repub.)

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“…1 Hepatic involvement in HHT is probably less common but has been reported. 1,[8][9][10][11][12][13][14] The hepatic vascular malformations of HHT, like those of the central nervous system, can be of several different forms, including large AV fistulas, telangiectasias, and hepatic artery (HA) aneurysms, occasionally in the context of cirrhosis. 8,12 Nineteen cases of hepatic telangiectasias associated with fibrosis and/or cirrhosis were collected by Martini.…”

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confidence: 99%

“…7 Portosystemic shunting in HHT has been delineated anatomically only recently. 8,12 AV shunts between the HA and PV are well described and may result in portal hypertension and gastroesophageal varices. 8,12,20 Common presenting signs include hepatomegaly, right upper quadrant pain, pulsatile mass, and audible bruit.…”

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confidence: 99%

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Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia

et al. 1998

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Experience with hepatic artery embolization for the treatment of symptomatic hepatic arteriovenous malformations (AVMs) in Rendu-OslerWeber disease is limited. We report 2 cases of hepatic AVMs that caused mesenteric angina-like symptoms that were treated with embolization. Both patients developed parenchymal and bile duct necrosis, intrahepatic bilomas, and refractory biliary sepsis, subsequently leading to liver failure. We hypothesize that the pathophysiological cause of biliary necrosis in this setting is similar to that which occurs in the setting of hepatic artery thrombosis of the liver allograft. Progressive liver failure in these patients was treated successfully by liver transplantation. Liver transplantation offers definitive therapy by removing the source of ongoing sepsis, restoring normal liver function, and eliminating the intrahepatic AV shunt.

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“…Abnormality of the common hepatic artery (diameter, flow velocity, tortuosity) may be one of the earliest signs of liver involvement, and an increased diameter appears to be one of the most specific signs of the disease. 10,[16][17][18] We thus considered, in the first group (G1), patients with no abnormality evidenced at DS in the diameter or maximum velocity of the hepatic artery portal and hepatic veins (Table 1); in group 2, we included patients with "early changes" involving only the diameter or maximum velocity of the hepatic artery. Group 3 patients presented signs of significant arteriovenous fistulas, involving either the portal or hepatic veins, or a directly visible intrahepatic vessel suggestive of arteriovenous fistula, and all patients in group 3 had hepatic artery diameter enlargement or high maximum velocity.…”

Section: Interpretation Of the Resultsmentioning

confidence: 99%

Evaluation of previously nonscreened hereditary hemorrhagic telangiectasia patients shows frequent liver involvement and early cardiac consequences

Gincul¹,

Lesca²,

Gelas-Dore³

et al. 2008

Hepatology

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H ereditary hemorragic telangiectasia (HHT) is an autosomal dominant genetic disease driven by mutations in genes such as endoglin (ENG), activin receptor-like kinase type 1 (ACVRL1), and Smad-4, which control the transforming growth factor beta proliferation pathway. 1 HHT patients usually develop a wide range of cutaneous, mucosal, and sometimes visceral arteriovenous malformations. [2][3][4] The symptomatology is dominated by epistaxis and anemia, but potentially life-threatening visceral localizations can be present, such as pulmonary, hepatic, and cerebral vascular malformations. 5 Hepatic involvement in HHT has been described in several studies. 6,7 Characteristic HHT hepatic localization consists of arteriovenous shunting responsible, in severe cases, for high-output cardiac failure (fistulas between the hepatic artery and hepatic veins), ascites (fistulas between the hepatic artery and portal veins), and cholangiopathy with sepsis. Liver transplantation is the main treatment option at these advanced stages. Previous, mostly small series have shown Doppler sonography (DS) abnormalities in 30% to 70% of HHT patients. 8,9 Early and advanced DS findings include (1) enlargement, increased flow velocity, and tortuosity of hepatic artery branches; (2) increased diameter and flow velocity in he-

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Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: imaging findings.

Cited by 106 publications

References 0 publications

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia

Evaluation of previously nonscreened hereditary hemorrhagic telangiectasia patients shows frequent liver involvement and early cardiac consequences

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