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Abernethy Malformation and Hepatocellular Carcinoma: a Serious Consequence of a Rare Disease

2020Journal article Open access
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dc.contributor.authorJaklitsch, M
dc.contributor.authorSobral, M
dc.contributor.authorCarvalho, AM
dc.contributor.authorPinto Marques, H
dc.date.accessioned2022-05-03T15:05:56Z
dc.date.available2022-05-03T15:05:56Z
dc.date.issued2020
dc.description.abstractCongenital portosystemic shunts (CPSS) are a rare vascular consequence of embryogenetic vascular alterations or the persistence of the fetal circulation elements, first described by John Abernethy in 1793 and classified by Morgan and Superina, into complete and partial portosystemic shunts. Its prevalence to this day has not been defined. We present a patient series of a 44-year-old and 47-year-old man and woman, with this rare congenital malformation and underlining hepatocellular carcinoma (HCC) treatment strategies. Over half of the individuals with CPSS have benign or malignant liver tumours, ranging from nodular regenerative hyperplasia, focal nodular hyperplasia, adenomas, HCC and hepatoblastomas. Additionally, it is known that half of individuals with Abernethy malformation type Ib will develop one or multiple types of tumours. There seems to be a direct association with tumorigenesis and CPSS, which is the primary consequence of absent portal flow. Surgery is the treatment of choice, either as a curative resection or orthotopic liver transplantation if recommended as per the criteria, in which replacing the hepatic parenchyma in the setting of an Abernathy malformation will correct the underlining hyper-arterialisation.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationBMJ Case Rep. 2020 Jan 6;13(1):e231843.pt_PT
dc.identifier.doi10.1136/bcr-2019-231843.pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.17/4065
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBMJpt_PT
dc.subjectHCC CHBPTpt_PT
dc.subjectHCC PAT CLINpt_PT
dc.subjectAdultpt_PT
dc.subjectFemalept_PT
dc.subjectMalept_PT
dc.subjectHumanspt_PT
dc.subjectMiddle Agedpt_PT
dc.subjectCarcinoma, Hepatocellular / etiology*pt_PT
dc.subjectCarcinoma, Hepatocellular / surgery*pt_PT
dc.subjectLiver Neoplasms / etiology*pt_PT
dc.subjectLiver Neoplasms / surgerypt_PT
dc.subjectLiver Transplantationpt_PT
dc.subjectPortal Vein / abnormalities*pt_PT
dc.subjectPortal Vein / surgerypt_PT
dc.subjectRare Diseasespt_PT
dc.subjectVascular Malformations / complications*pt_PT
dc.subjectVascular Malformations / surgery*pt_PT
dc.titleAbernethy Malformation and Hepatocellular Carcinoma: a Serious Consequence of a Rare Diseasept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.startPagee231843pt_PT
oaire.citation.titleBMJ Case Reportspt_PT
oaire.citation.volume13pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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