Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia

García–Tsao, Guadalupe; Korzenik, Joshua R.; Young, Lawrence H.; Henderson, Katharine J.; Jain, Dhanpat; Byrd, Boyd; Pollak, Jeffrey; White, Robert I.

doi:10.1056/nejm200009283431305

Cited by 362 publications

(323 citation statements)

References 44 publications

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“…An alternative hypothesis is the presence of nodular transformation and hyperplasia leading to ''pseudo-cirrhosis''. 42 It is thought that there is enlargement of HAVMs during pregnancy similar to that occurring in other organ systems. 14,43 Two case reports have been published of women presenting with hepatic AVMs and bile duct ischemia during pregnancy.…”

Section: Gastrointestinal Avmsmentioning

confidence: 99%

Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

Lomax

Edgcombe

2009

Can J Anesth/J Can Anesth

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Purpose To review the effects of hereditary hemorrhagic telangiectasia (HHT) in the parturient and the anesthetic management of such patients during pregnancy and delivery. Source A literature search was performed using Medline and EMBASE databases. Bibliographies of retrieved articles were searched for additional sources. Principal findings Hereditary hemorrhagic telangiectasia affects 1 in 5000-8000 people. It is a genetic condition in which vascular dysplasia affects many organs particularly the pulmonary, cerebral, gastrointestinal, and spinal vasculature. A large proportion of women with HHT have uneventful pregnancies. However, women can present in pregnancy with clinically silent but potentially life-threatening features of the disorder including fatal hemorrhage from ruptured arteriovenous malformations (AVMs), systemic emboli, and high output cardiac failure secondary to arteriovenous shunting. Literature on the anesthetic management of HHT in pregnancy is limited. Both general and regional anesthetic techniques have been successfully performed in these patients, but are reliant on identifying the presence of specific AVMs; avoidance of cardiovascular instability; and prophylaxis against systemic emboli secondary to pulmonary AVM shunting. The presence of spinal AVMs is considered a relative contraindication to regional techniques. As with other systemic AVMs, these can develop and increase in size during pregnancy with implications for the timing of screening and surveillance. Conclusions An understanding of the presence and potential development of life-threatening AVMs during pregnancy is imperative for anesthesiologists caring for parturients with HHT. Even in the asymptomatic patient, a high index of suspicion should be maintained, screening performed where possible and anesthetic technique adapted accordingly. RésuméObjectif Passer en revue les effets de la te´langiectasie he´morragique he´re´ditaire (THH) chez les parturientes et la prise en charge de telles patientes pendant leur grossesse et l'accouchement. Source Une recherche de la litte´rature

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Section: Gastrointestinal Avmsmentioning

confidence: 99%

Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

Lomax

Edgcombe

2009

Can J Anesth/J Can Anesth

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“…Treatment of hepatic malformations is conservative 14 . Experience with surgical ligation is limited and data on embolization, although limited, show mortality in about 25% of the cases 14 .…”

Section: Treatmentmentioning

confidence: 99%

Síndrome de Rendu-Osler-Weber: relato de caso e revisão de literatura

Juares

Dell’Aringa

Nardi

et al. 2008

Rev. Bras. Otorrinolaringol.

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Heredi tary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease is a rare fibrovascular dysplasia that makes vascular walls vulnerable to trauma and rupture, causing skin and mucosa bleeding. It is of dominant autosomal inheritance, characterized by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations and positive family history. Epistaxis is often the first and foremost manifestation. It's associated to arteriovenous malformations in several organs. There are possible hematologic, neurologic, pulmonary, dermatologic and gastrointestinal complications. Treatment is supportive and helps prevent complications. This study is a case report of a patient with this syndrome who came to the ENT Outpatient Ward of the Faculdade de Medicina de Marília; and we have done a bibliographic review of the disease's etiopathogenesis, clinical manifestations and clinical-surgical treatment options.

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“…Arteriovenous shunting may cause biliary ischemia with subsequent bile duct necrosis and sclerosing cholangitis, while arterioportal shunting may result in symptomatic portal hypertension. 2,3 Hepatic vascular lesions in HHT range from tiny telangiectases to large confluent vascular masses and marked dilatation of the hepatic artery. Perfusional changes within the liver may lead to nodular regenerative hyperplasia and focal nodular hyperplasia.…”

mentioning

confidence: 99%

“…Liver biopsy is not indicated in patients with HHT suspected of having hepatic involvement because of a potential risk for bleeding. [1][2][3] Treatment is symptomatic relief of the portal hypertension, transarterial embolization of liver arteriovenous malformations in the setting of high-output cardiac failure, and medical therapy for cholangitis. 4 Liver transplantation is indicated in patients with chronic biliary disease and in those with intractable high-output cardiac failure or refractory portal hypertension.…”

mentioning

confidence: 99%