Osler-Weber-Rendu disease with multiple intrahepatic arteriovenous fistulas

Danchin, N.; Thisse, J.Y; Neimann, J. L.; Faivre, G

doi:10.1016/0002-8703(83)90253-3

Cited by 34 publications

(9 citation statements)

References 6 publications

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“…The incidence of liver involvement in HHT is unknown, but it appears the lesion is uncommon, with fewer than 80 cases reported in the literature. 7,9,11,[13][14][15][20][21][22][23][24][25][26][27][28][29] From the literature, the most common indication for therapeutic intervention for symptomatic hepatic involvement is highoutput cardiac failure. Conversely, mesenteric angina was the indication for treatment of hepatic AVMs in a single case in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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

et al. 1998

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“…Over 50 cases of HHT presenting as hepatic arteriovenous fistulae and/or telangiectasia have been reported [55][56][57], although the true incidence may be much higher. Chronic liver disease is present in up to 30% of patients with HHT [30].…”

Section: Hepatic Manifestationsmentioning

confidence: 99%

“…Portosystemic encephalopathy should be managed in the standard fashion. Portal hypertension can be controlled by propranolol, but in severe cases might require embolization or surgical ligation of the shunt [56,69,70]. Variceal bleeding is managed in the standard fashion acutely but may require hepatic artery embolization or ligation for long-term control [62].…”

Section: Hepatic Manifestationsmentioning

confidence: 99%

Gastrointestinal and Hepatic Manifestations of Hereditary Hemorrhagic Telangiectasia

Sharma

Howden

1998

Dig Dis

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Hereditary hemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber (ORW) disease is an autosomal-dominant disorder that frequently presents with gastrointestinal bleeding which may be a diagnostic and therapeutic challenge. Hepatic involvement in this disease is increasingly recognized and poses another therapeutic challenge. With advances in genetic screening and diagnostic procedures, and the increasing awareness of the condition by physicians and patients, this disease is being diagnosed more often. This article reviews the available literature on gastrointestinal and hepatic manifestations of HHT, and the various diagnostic and therapeutic modalities available for its management.

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“…Immunosuppressive therapy was Discussion M. Osler is an autosomal dominant disorder [lo] characterized by the classical triad of mucocutaneous and visceral teleangiectasia, recurrent epistaxis, and a positive family history. Liver involvement with typical arteriovenous fistulas and teleangiectasias has been reported in more than 50 cases [5,101. In a few cases, these pathological features were also associated with fibrosis and cirrhosis [8].…”

Section: Case Reportmentioning

confidence: 99%

Effective therapy for hepatic M. Osier with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation

Neumann¹,

Knoop²,

Langrehr³

et al. 1998

Transplant Int

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Hereditary hemorrhagic teleangiectasia, or M. Osler (OslerWeber-Rendu disease), is an autosoma1 dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal varices grade IV. The patient underwent liver transplantation and posttransplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, however, is recommended only for patients with normal liver function and carries a considerable risk of bile duct necrosis.

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Osler-Weber-Rendu disease with multiple intrahepatic arteriovenous fistulas

Cited by 34 publications

References 6 publications

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

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

Gastrointestinal and Hepatic Manifestations of Hereditary Hemorrhagic Telangiectasia

Effective therapy for hepatic M. Osier with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation

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