ArticleBMJ case reports2020
Abernethy malformation and hepatocellular carcinoma: a serious consequence of a rare disease.
Article in BMJ case reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 11 citations in OpenAlex.
- Liver tumors associated with congenital portosystemic shunts - report of 3 cases and literature review.Abdominal radiology (New York) · 2026Review
- Interventional, surgical, and clinical management of Abernethy malformations: A case series and novel anatomic variant.Radiology case reports · 2026Article
- Hepatocellular Carcinoma Arising From Well-Differentiated Hepatocellular Neoplasm of Uncertain Malignant Potential in a Patient With Abernethy Malformation.Case reports in surgery · 2026Article
- Recapitulating Liver Embryology-Lessons to Be Learned for Liver Diseases.Journal of developmental biology · 2025Review
- Liver Transplantation as a Treatment for Unresectable Hepatic Adenoma in a Patient With Abernethy Syndrome.Cureus · 2024Article
- Article
- Regenerative nodules as harbinger of Abernethy malformation.BMJ case reports · 2023Article
- Involution of Hepatocellular Neoplasm after Embolization of a Portosystemic Vascular Shunt in an Adult with Abernethy Type II Malformation.Journal of vascular and interventional radiology : JVIR · 2021Article
- An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia.Radiology case reports · 2020Article
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Congenital 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.
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