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


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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


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