Year 2024 / Volume 116 / Number 1
Editorial
Diagnosis and management of Abernethy syndrome

1-6

DOI: 10.17235/reed.2023.9781/2023

Javier Castro Rodríguez, Manuel Luis Rodríguez Perálvarez, José Luis Montero-Álvarez,

Abstract
Abernethy syndrome (AS or extrahepatic portosystemic shunt) is an uncommon congenital malformation consisting of agenesis or hypoplasia of the portal vein (PV) in such a way that splanchnic venous blood drains directly into the systemic circulation through aberrant communications, resulting in a portosystemic shunt that bypasses the liver AS is an underdiagnosed condition with unknown incidence and complication rate given that symptoms are usually absent. AS identification is increasingly common because of improved imaging techniques, hence prognostic implications and clinical management need be understood. This editorial reviews the natural history of AS and its diagnostic-therapeutic implications, illustrating the process with a series of cases from our institution.
Share Button
New comment
Comments
No comments for this article
References
1. Baiges A, Turon F, Simón-Talero M, et al. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. Hepatology. 2020;71(2):658-69.
2. Kumar P, Bhatia M, Garg A, et al. Abernethy malformation: A comprehensive review. Diagn Interv Radiol. 2022;28(1):21-8.
3. Suárez Sánchez A, Solar García L, García Bernardo CM, et al. Rectorragia como forma de presentación de un caso de síndrome de Abernethy en el adulto. Revista Española de Enfermedades Digestivas. 2018:667-8.
4. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts--the Abernethy malformation. J Pediatr Surg. 1997;32(3):494-7.
5. Benavides de la Rosa F, López de Cenarruzabeitia Í, García-Castaño Gandiaga J, et al. Malformación de Abernethy: shunt portosistémico congénito. Cirugía Española. 2015;93(3):e17.
6. Nam HD. Living-donor liver transplantation for Abernethy malformation - case report and review of literature. Ann Hepatobiliary Pancreat Surg. 242020. p. 203-8.
7. Francois B, Gottrand F, Lachaux A, et al. Outcome of intrahepatic portosystemic shunt diagnosed prenatally. Eur J Pediatr. 2017;176(12):1613-8.
8. Akahoshi T, Nishizaki T, Wakasugi K, et al. Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt: three cases and literature review. Hepatogastroenterology. 2000;47(34):1113-6.
9. Yi JE, Jung HO, Youn HJ, et al. A case of pulmonary arterial hypertension associated with congenital extrahepatic portocaval shunt. J Korean Med Sci. 2014;29(4):604-8.
10. Fu L, Wang Q, Wu J, et al. Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. Eur J Pediatr. 2016;175(2):195-201.
11. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat. 2008;21(2):147-57.
12. Konstas AA, Digumarthy SR, Avery LL, et al. Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol. 2011;80(2):175-81.
13. Ono H, Mawatari H, Mizoguchi N, et al. Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr. 1998;87(6):631-4.
14. Gitzelmann R, Forster I, Willi UV. Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur J Pediatr. 1997;156(9):719-22.
15. Lautz TB, Tantemsapya N, Rowell E, et al. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg. 2011;46(2):308-14.
16. Franchi-Abella S, Branchereau S, Lambert V, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr. 2010;51(3):322-30.
17. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003;33(9):614-20.
18. Guérin F, Blanc T, Gauthier F, et al. Congenital portosystemic vascular malformations. Semin Pediatr Surg. 2012;21(3):233-44.
19. Sokollik C, Bandsma RH, Gana JC, et al. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 2013;56(6):675-81.
20. Lemoine C, Nilsen A, Brandt K, et al. Liver histopathology in patients with hepatic masses and the Abernethy malformation. J Pediatr Surg. 2019;54(2):266-71.
21. Sharma R, Suddle A, Quaglia A, et al. Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int. 2015;14(5):552-7.
22. Benedict M, Rodriguez-Davalos M, Emre S, et al. Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) With Associated Hepatocellular Carcinoma: Case Report and Literature Review. Pediatr Dev Pathol. 2017;20(4):354-62.
23. Sorkin T, Strautnieks S, Foskett P, et al. Multiple β-catenin mutations in hepatocellular lesions arising in Abernethy malformation. Hum Pathol. 2016;53:153-8.
24. Gülşen Z, Yiğit H, Demir P. Multiple regenerative nodular hyperplasia in the left infrarenal vena cava accompanied by Abernethy malformation. Surg Radiol Anat. 2016;38(3):373-8.
25. Özden İ, Yavru A, Güllüoğlu M, et al. Transplantation for Large Liver Tumors in the Setting of Abernethy Malformation. Exp Clin Transplant. 2017;15(Suppl 2):82-5.
Related articles

Letter

A case of complex hemangioendothelioma of the liver in an infant

DOI: 10.17235/reed.2023.9949/2023

Letter

Amyloidosis: a rare cause of severe acute liver failure

DOI: 10.17235/reed.2023.9598/2023

Letter

Icteric syndrome secondary to polycystic liver disease

DOI: 10.17235/reed.2023.9590/2023

Letter

Liver disorders and celiac disease

DOI: 10.17235/reed.2023.9516/2023

Letter

Budd-Chiari syndrome in Behçet’s disease

DOI: 10.17235/reed.2022.9267/2022

Editorial

Drug induced liver injury by immunotherapy

DOI: 10.17235/reed.2022.9179/2022

Letter

Epithelioid angiomyolipoma of the liver: an incidental diagnosis

DOI: 10.17235/reed.2022.8895/2022

Letter

Intra-abdominal epithelioid angiosarcoma

DOI: 10.17235/reed.2022.8878/2022

Review

Immunotherapy in hepatocellular carcinoma

DOI: 10.17235/reed.2022.8876/2022

Digestive Diseases Image

Liver abscess due to parvimonas micra

DOI: 10.17235/reed.2022.8751/2022

Digestive Diseases Image

Posterior reversible encephalopathy syndrome (PRES) in post liver transplantation

DOI: 10.17235/reed.2022.8694/2022

Editorial

Acute pancreatitis: an opportunity for gastroenterology hospitalists?

DOI: 10.17235/reed.2022.8573/2022

Letter

Gastric ulcer with liver penetration

DOI: 10.17235/reed.2022.8528/2021

Letter

Ertapenem neurotoxicity in liver transplantation

DOI: 10.17235/reed.2021.8469/2021

Digestive Diseases Image

Ischemic cholangiopathy following hepatic artery pseudoaneurysm with thrombosis

DOI: 10.17235/reed.2021.8395/2021

Digestive Diseases Image

Giant cystic hepatocarcinoma in the absence of cirrhosis

DOI: 10.17235/reed.2021.8313/2021

Letter

Unexpected diagnosis for nodular hepatic lesions

DOI: 10.17235/reed.2021.8230/2021

Letter

Clip migration after cholecystectomy

DOI: 10.17235/reed.2021.8156/2021

Letter

EBVMCU in a liver-transplant patient

DOI: 10.17235/reed.2021.8064/2021

Review

A review of the diagnosis and management of liver hydatid cyst

DOI: 10.17235/reed.2021.7896/2021

Letter

COVID-19 and the liver: the chicken or the egg dilemma

DOI: 10.17235/reed.2021.7861/2021

Digestive Diseases Image

Percutaneous treatment of a splenorenal shunt with an atrial septal closure device

DOI: 10.17235/reed.2020.7701/2020

Digestive Diseases Image

Inflammatory pseudotumor-like follicular dendritic cell sarcoma of the liver

DOI: 10.17235/reed.2020.7602/2020

Review

Physical frailty in liver transplantation

DOI: 10.17235/reed.2020.7448/2020

Letter

Acute hepatitis due to olmesartan: an uncommon entity

DOI: 10.17235/reed.2020.7236/2020

Letter

Management of hepatic artery aneurysms

DOI: 10.17235/reed.2020.7219/2020

Digestive Diseases Image

Epithelioid hemangioendothelioma: a rare cause of a liver tumor

DOI: 10.17235/reed.2020.7127/2020

Review

Pyroptosis in liver disease

DOI: 10.17235/reed.2020.7034/2020

Letter

Biopsy for hepatocellular carcinoma: only occasionally

DOI: 10.17235/reed.2020.6935/2020

Original

A new pattern in hepatitis A virus infection in an urban population

DOI: 10.17235/reed.2020.6526/2019

Case Report

Ectopic hepatocellular carcinoma arising from the peritoneum

DOI: 10.17235/reed.2019.6408/2019

Letter

Hepatic epithelioid angiomyolipoma: contribution of a new case

DOI: 10.17235/reed.2019.6388/2019

Digestive Diseases Image

Concurrent hepatic and renal angiomyolipomas in tuberous sclerosis complex

DOI: 10.17235/reed.2020.6376/2019

Review

Psoriasis and fatty liver: a harmful synergy

DOI: 10.17235/reed.2019.6263/2019

Letter

A giant liver abscess due to Fasciola hepatica infection

DOI: 10.17235/reed.2019.6136/2018

Letter

Giant hepatobiliary cystadenoma: clinic-pathological findings

DOI: 10.17235/reed.2018.5414/2017

Letter to the Editor

Post-transplant lymphoproliferative disease after liver transplantation

DOI: 10.17235/reed.2017.5387/2017

Letter to the Editor

Complicated peribiliary cysts in patient without any prior liver disease

DOI: 10.17235/reed.2017.5350/2017

Letter to the Editor

Liver metastasis from colorectal cancer 12 years after liver transplantation

DOI: 10.17235/reed.2017.4507/2016

Case Report

The clinical extremes of autoimmune cholangitis

DOI: 10.17235/reed.2017.4167/2015

Case Report

Glycogenic hepatopathy in young adults: a case series

DOI: 10.17235/reed.2016.3934/2015

Letter to the Editor

Severe acute liver failure and thyrotoxicosis: an unusual association

DOI: 10.17235/reed.2015.3607/2014

Letter to the Editor

Leiomyosarcoma of the ascending colon: a rare tumor with poor prognosis

DOI: 10.17235/reed.2015.3606/2014

Letter to the Editor

PITAVASTATIN: OTHER STATIN TO BE USED AND MONITORED

DOI: 10.17235/reed.2015.3565/2014

Letter to the Editor

Liver abscess by Eikenella Corrodens

DOI: 10.17235/reed.2015.3613/2014

Letter to the Editor

Leiomyoma of the round ligament of the liver: Report of one case

DOI: 10.17235/reed.2015.3725/2014

Citation tools
Castro Rodríguez J, Rodríguez Perálvarez M, Montero-Álvarez J. Diagnosis and management of Abernethy syndrome. 9781/2023


Download to a citation manager

Download the citation for this article by clicking on one of the following citation managers:

Metrics
This article has received 662 visits.
This article has been downloaded 207 times.

Statistics from Dimensions


Statistics from Plum Analytics

Publication history

Received: 12/06/2023

Accepted: 15/06/2023

Online First: 31/07/2023

Published: 11/01/2024

Article Online First time: 49 days

Article editing time: 213 days


Share
This article hasn't been rated yet.
Reader rating:
Valora este artículo:




Asociación Española de Ecografía Digestiva Sociedad Española de Endoscopia Digestiva Sociedad Española de Patología Digestiva
The Spanish Journal of Gastroenterology is the official organ of the Sociedad Española de Patología Digestiva, the Sociedad Española de Endoscopia Digestiva and the Asociación Española de Ecografía Digestiva
Cookie policy Privacy Policy Legal Notice © Copyright 2023 y Creative Commons. The Spanish Journal of Gastroenterology