Abernethy Malformation in Children with Focal Liver Lesions
D. E. Arutyunyants,
V. V. Khovrin,
T. N. Galyan,
A. S. Kulichkin,
A. V. Filin,
E. Yu. Kryzhanovskaya https://doi.org/10.20862/0042-4676-2021-102-3-166-171
Abstract
Abernethy malformation is a congenital disease characterized by complete or partial underdevelopment of the portal vein, leading to the occurrence of congenital extrahepatic portosystemic shunts. The anomaly with the absence of the portal vein is detected in 80% of cases in childhood. Patients with this pathology are very frequently examined when detecting space-occupying liver lesions and assuming the presence of a vascular abnormality. The paper describes clinical cases of benign focal liver lesions and Abernethy malformation in children.
About the Authors
D. E. Arutyunyants
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Dana E. Arutyunyants, Postgraduate
Abrikosovskiy pereulok, 2, Moscow, 119991
V. V. Khovrin
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Valeriy V. Khovrin, Dr. Med. Sc., Chief Researcher
Abrikosovskiy pereulok, 2, Moscow, 119991
T. N. Galyan
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Tatyana N. Galyan, Cand. Med. Sc., Head of Department of Radiodiagnostics and Computed Tomography
Abrikosovskiy pereulok, 2, Moscow, 119991
A. S. Kulichkin
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Aleksey S. Kulichkin, Radiologist
Abrikosovskiy pereulok, 2, Moscow, 119991
A. V. Filin
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Andrey V. Filin, Dr. Med. Sc., Head of Liver Transplant Department
Abrikosovskiy pereulok, 2, Moscow, 119991
E. Yu. Kryzhanovskaya
Petrovskiy National Research Centre of Surgery
Russian Federation
Russian Federation
Evgeniya Yu. Kryzhanovskaya, Cand. Med. Sc., Senior Researcher, Ultrasound Diagnostician
Abrikosovskiy pereulok, 2, Moscow, 119991
References
1. Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 1994; 29(9): 1239–41. https://doi.org/10.1016/0022-3468(94)90812-5.
2. Ghuman S, Gupta S, Buxi Т, et al. The Abernethy malformation – myriad imaging manifestations of a single entity. Indian J Radiol Imaging. 2016; 26(3): 364–72. https://doi.org/10.4103/0971-3026.190420.
3. Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol.1990; 155(3): 527–8. https://doi.org/10.2214/ajr.155.3.2117349.
4. Baiges A, Turon F, Tasayco S, et al. Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 2020; 71(2): 658–69. https://doi.org/10.1002/hep.30817.
5. Murray C, Yoo S, Babyn P. Congenital extrahepatic portosystemic shunts. Pediatr Radiol. 2003; 33(9): 614–20. https://doi.org/10.1007/s00247-003-1002-x.
6. Barton J, Keller M. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol. 1989; 20(1-2): 113–4. https://doi.org/10.1007/BF02010653.
7. Kawano S, Hasegawa S, Urushihara N, et al. Hepatoblastoma with congenital absence of the portal vein – a case report. Литература [References] Eur J Pediatr Surg. 2007; 17(4): 292–4. https://doi.org/10.1055/s-2007-965448.
8. Pichon N, Maisonnette F, Valleix D, Pichon-Lefièvre F, et al. Hepatocarcinoma with congenital agenesis of the portal vein. Jpn J Clin Oncol 2003; 33(6): 314–6. https://doi.org/10.1093/jjco/hyg050.
9. Lundstedt C, Lindell G, Tranberg K, Svartholm E. Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma. Eur Radiol. 2001; 11(11): 2228–31. https://doi.org/10.1007/s003300100833.
10. Konstas A, Digumarthy S, Avery L, et al.. Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol. 2011; 80(2): 175–81. https://doi.org/10.1016/j.ejrad.2009.12.031.
11. Hu G, Shen L, Yang J, et al. Insight into congenital absence of the portal vein: is it rare? World J Gastroenterol. 2008; 14(39): 5969–79. https://doi.org/10.3748/wjg.14.5969.
12. Schettino G, Fagundes E, Roquete M, et al. Portal vein thrombosis in children and adolescents. J Pediatr (Rio J). 2006; 82(3): 171–8. https://doi.org/10.2223/JPED.1484.
13. Parrón M, Pérez А, Prieto C, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. 2011; 31(3): 707–22. https://doi.org/10.1148/rg.313105070.
Review
For citations:
Arutyunyants D.E., Khovrin V.V., Galyan T.N., Kulichkin A.S., Filin A.V., Kryzhanovskaya E.Yu. Abernethy Malformation in Children with Focal Liver Lesions. Journal of radiology and nuclear medicine. 2021;102(3):166-171. (In Russ.) https://doi.org/10.20862/0042-4676-2021-102-3-166-171
Views:
3894
Email this article 