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Hepatic arteriovenous malformation

MedGen UID:
101044
Concept ID:
C0520557
Congenital Abnormality
Synonym: Liver arteriovenous malformation
SNOMED CT: Arteriovenous malformation of liver (84150000); Hepatic arteriovenous malformation (84150000)
 
HPO: HP:0006574

Definition

A benign vascular lesion characterized by the presence of a complex network of communicating arterial and venous vascular structures in the liver. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatic arteriovenous malformation

Conditions with this feature

Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome
MedGen UID:
331400
Concept ID:
C1832942
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Telangiectasia, hereditary hemorrhagic, type 2
MedGen UID:
324960
Concept ID:
C1838163
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Telangiectasia, hereditary hemorrhagic, type 1
MedGen UID:
1643786
Concept ID:
C4551861
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.

Professional guidelines

PubMed

Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Letteboer TG, Mager JJ, Snijder RJ, Koeleman BP, Lindhout D, Ploos van Amstel JK, Westermann CJ
J Med Genet 2006 Apr;43(4):371-7. Epub 2005 Sep 9 doi: 10.1136/jmg.2005.035451. PMID: 16155196Free PMC Article

Recent clinical studies

Etiology

Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Giraud S, Bardel C, Dupuis-Girod S, Carette MF, Gilbert-Dussardier B, Riviere S, Saurin JC, Eyries M, Patri S, Decullier E, Calender A, Lesca G
Orphanet J Rare Dis 2020 Sep 22;15(1):254. doi: 10.1186/s13023-020-01533-2. PMID: 32962750Free PMC Article
Schwarze V, Marschner C, Negrão de Figueiredo G, Rübenthaler J, Clevert DA
Ultraschall Med 2020 Feb;41(1):29-35. Epub 2019 Jul 30 doi: 10.1055/a-0973-8517. PMID: 31362328
Zanjani KS, Mazloumi M, Zeinaloo A, Hedayati M, Khalilzadeh O, Yazdi HR
Diagn Interv Radiol 2012 Mar-Apr;18(2):231-5. Epub 2011 Oct 14 doi: 10.4261/1305-3825.DIR.4418-11.0. PMID: 21996781
Nishimori A, Okajima H, Okumura K, Yoneyama T, Takeichi T, Asonuma K, Ikeda O, Inomata Y
J Hepatobiliary Pancreat Surg 2009;16(3):382-5. Epub 2009 Feb 10 doi: 10.1007/s00534-008-0032-z. PMID: 19209381

Diagnosis

Duhaut L, Eyries M, Lewin M, Ciacio O, Kounis I, Cherqui D, Antonini T, Duclos-Vallée JC, Feray C, Samuel D, Guettier C, Coilly A
Hepatology 2021 Aug;74(2):1121-1123. Epub 2021 Jun 15 doi: 10.1002/hep.31694. PMID: 34132417
Agha HM, Zakaria R, Mostafa FA, Hamza H
Tex Heart Inst J 2015 Apr;42(2):184-7. Epub 2015 Apr 1 doi: 10.14503/THIJ-13-3660. PMID: 25873838Free PMC Article
Zanjani KS, Mazloumi M, Zeinaloo A, Hedayati M, Khalilzadeh O, Yazdi HR
Diagn Interv Radiol 2012 Mar-Apr;18(2):231-5. Epub 2011 Oct 14 doi: 10.4261/1305-3825.DIR.4418-11.0. PMID: 21996781
Thatrimontrichai A, Chanvitan P, Janjindamai W, Dissaneevate S, Kritsaneepaiboon S, Hongsakul K
Indian J Pediatr 2012 May;79(5):673-5. Epub 2011 Jul 6 doi: 10.1007/s12098-011-0512-z. PMID: 21732018
Peng YF, Chen LK, Chou YH, Chang FC, Hwang SJ
Zhonghua Yi Xue Za Zhi (Taipei) 2002 Dec;65(12):614-8. PMID: 12636208

Therapy

Schwarze V, Marschner C, Negrão de Figueiredo G, Rübenthaler J, Clevert DA
Ultraschall Med 2020 Feb;41(1):29-35. Epub 2019 Jul 30 doi: 10.1055/a-0973-8517. PMID: 31362328
Agha HM, Zakaria R, Mostafa FA, Hamza H
Tex Heart Inst J 2015 Apr;42(2):184-7. Epub 2015 Apr 1 doi: 10.14503/THIJ-13-3660. PMID: 25873838Free PMC Article
Bolus C, Yamada R, Alshora S, Hannegan C, Anderson B
J Radiol Case Rep 2014 Aug;8(8):33-42. Epub 2014 Aug 31 doi: 10.3941/jrcr.v8i8.2171. PMID: 25426243Free PMC Article
Hisamatsu K, Ueeda M, Ando M, Koike K, Matsuo N, Matsu-ura K, Ueda H, Hirohata M, Imai M
Intern Med 1999 Dec;38(12):962-8. doi: 10.2169/internalmedicine.38.962. PMID: 10628935
Mejides AA, Adra AM, O'Sullivan MJ, Nicholas MC
Obstet Gynecol 1995 May;85(5 Pt 2):850-3. doi: 10.1016/0029-7844(94)00227-5. PMID: 7724135

Prognosis

Ford TJ, Fong MW, Cheah BC, Alexopolous C
J R Coll Physicians Edinb 2014;44(2):126-9. doi: 10.4997/JRCPE.2014.207. PMID: 24999773
Zanjani KS, Mazloumi M, Zeinaloo A, Hedayati M, Khalilzadeh O, Yazdi HR
Diagn Interv Radiol 2012 Mar-Apr;18(2):231-5. Epub 2011 Oct 14 doi: 10.4261/1305-3825.DIR.4418-11.0. PMID: 21996781
Thatrimontrichai A, Chanvitan P, Janjindamai W, Dissaneevate S, Kritsaneepaiboon S, Hongsakul K
Indian J Pediatr 2012 May;79(5):673-5. Epub 2011 Jul 6 doi: 10.1007/s12098-011-0512-z. PMID: 21732018
Hisamatsu K, Ueeda M, Ando M, Koike K, Matsuo N, Matsu-ura K, Ueda H, Hirohata M, Imai M
Intern Med 1999 Dec;38(12):962-8. doi: 10.2169/internalmedicine.38.962. PMID: 10628935
Chen CH, Yang PM, Sheu JC, Huang GT, Tsang YM, Lee PH, Chen DS
Hepatogastroenterology 1999 Jan-Feb;46(25):443-7. PMID: 10228838

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