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Introduction: congenital portosystemic venous shunts (CPVS) are rare vascular abnormalities that occur secondary to abnormal development or involution of fetal vasculature. They allow intestinal blood to enter the systemic circulation, bypassing the liver, which in the long term leads to various symptoms and complications. Today, thanks to advanced imaging techniques, the number of reported cases of CPVS is increasing, although for the most part these are single clinical cases or reports summarizing small series of cases. The overall incidence of CPVS is estimated at 1:30 000 births and 1:50 000 for those persisting beyond early childhood.

Material and methods: article consists of 44 foreign literature sources, that  highlight pathogenesis, classification, clinical picture, diagnosis and treatment of CPVS.

Conclusion: early diagnosis and correction of this anomaly using any (endovascular or surgical) occlusion regresses symptoms and prevents long-term complications. At present, given the rarity of this pathology, there is no large statistical analysis and no standards, developed for the management of this category of patients. However, further collection of material, an emphasis on the pathophysiology and anatomy of these lesions, will help to provide more effective care for patients with congenital portosystemic venous shunts.





1.     Kim MJ, Ko JS, Seo JK, et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr. 2012; 171(2): 395-400.

2.     Florio F, Nardella M, Balzano S, et al. Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol. 1998; 21(5): 421-424.

3.     Baiges A, Turon F, Simуn-Talero M, et al. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study. Hepatology. 2020; 71(2): 658-669.

4.     Ольхова Е.Б., Туманян Г.T., Венгерская Г.В. и др. Мальформация Абернети у новорожденных. Эхографическая диагностика. Радиология-практика. 2015; 5(54): 46-58.

Olkhova EB, Tumanyan GT, Hungarian GV, et al. Abernathy malformation in newborns. Echographic diagnostics. Radiology-practice. 2015; 5 (54): 46-58 [In Russ].

5.     Малышева Е.Б., Захарова Е.М., Рыхтик П.И., Жулина Н.И. Мальформация Абернетти - редкая причина гемодинамического цирроза печени. Российский журнал гастроэнтерологии, гепатологии, колопроктологии. Приложение. 2017; 27(1) S49; 48.

Malysheva EB, Zakharova EM, Rykhtik PI, Zhulina NI. Abernetty's malformation is a rare cause of hemodynamic cirrhosis of the liver. Russian journal of gastroenterology, hepatology, coloproctology. Application. 2017; 27(1) S49; 48 [In Russ].

6.     Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc Lond B Biol Sci. 1793; 83: 59-66.

7.     Sokollik C, Bandsma RH, Gana JC, et al. Congenital portosystemic shunt: characterization of a multisystem disease. J. Pediatr. Gastroenterol. Nutr. 2013; 56(6): 675-681.

8.     Guérin F, Blanc T, Gauthier F, et al. Congenital portosystemic vascular malformations. Semin. Pediatr. Surg. 2012; 21(3): 233-244.

9.     Bernard O, Franchi-Abella S, Branchereau S, et al. Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis. 2012; 32(4): 273-287.

10.   Lin ZY, Chen SC, Hsieh MY, et al. Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause. J Clin Ultrasound. 2006; 34(1): 22-26.

11.   Gitzelmann R, Forster I, Willi UV. Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur J Pediatr. 1997; 156: 719-722.

12.   Ponziani FR, Faccia M, Zocco MA, et al. Congenital extrahepatic portosystemic shunt: description of four cases and review of the literature. J Ultrasound. 2019; 22(3): 349-358.

13.   De Paula Oliveira GJ, Ferreira S, Barbosa A. Abernethy Malformation – Congenital Extra-hepatic Portosystemic Shunt Associated with Multiple Liver Adenomatosis: Case Report. Universal Journal of Public Health. 2019; 7(3): 129-137.

14.   Nagata H, Yamamura K, Ikeda K. Balloon-occluded retrograde transvenous obliteration for congenital portosystemic venous shunt: report of two cases. Pediatr Int. 2012; 54(3): 419-421.

15.   Passalacqua M, Lie KT, Yarmohammadi H. Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure. Pediatr Surg Int. 2012; 28(1): 79-83.

16.   Raghuram KA, Bijulal S, Krishnamoorthy KM, Tharakan JA. Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol. 2013; 34(8):1882-5.

17.   DiPaola F, Trout AT, Walther AE, et al. Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. Dig Dis Sci. 2020; 65(4): 1239-1251.

18.   Ogul H, Bayraktutan U, Yalcin A, et al. Congenital absence of the portal vein in a patient with multiple vascular anomalies. Surg Radiol Anat. 2013; 35(6): 529-534.

19.   Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system forportasystemic vascular anomalies. J Pediatr Surg. 1994; 29(9):1239-1241.

20.   Glonnegger H, Schulze M, Kathemann S, et al. Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt. Front Pediatr. 2020; 8: 501.

21.   Raskin NH, Price JB, Fishman RA. Portal-systemic encephalopathy due to congenital intrahepatic shunts. The New England Journal of Medicine. 1964; 270: 225-229.

22.   Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. Am J Roentgenol. 1990; 155: 527-528.

23.   Senocak E, Oğuz B, Edgьer T, Cila A. Congenital intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol. 2008; 14: 97-99.

24.   Niwa T, Aida N, Tachibana K, et al. Congenital absence of the portal vein: clinical and radiologic findings. J Comput Assist Tomogr. 2002; 26(5): 681-6.

25.   Kobayashi N, Niwa T, Kirikoshi H, et al. Clinical classification of congenital extrahepatic portosystemic shunts. Hepatol Res. 2010; 40(6): 585-93.

26.   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): 354-362.

27.   Kroencke T, Murnauer M, Jordan FA, et al. Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation). Cardiovasc Intervent Radiol. 2018; 41(8): 1285-1290.

28.   Alonso-Gamarra E, Parrón M, Pérez A, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 2011; 31(3): 707-722.

29.   Brasoveanu V, Ionescu MI, Grigorie R, et al. Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review. Am J Case Rep. 2015; 16: 637-644.

30.   Duprey J, Gouin B, Benazet MF, le Gal J. Glucose intolerance and post-stimulative hypoglycaemia secondary to congenital intra-hepatic porto-caval anastomosis. Annales de Medecine Interne. 1985; 136(8): 655-658.

31.   Watanabe A. Portal-systemic encephalopathy in non-chirrotic patients: classification of clinical types, diagnosis and treatment. Journal of Gastroenterology and Hepatology. 2000; 15(9): 969-979.

32.   Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatric Radiology. 2003; 33(9): 614-620.

33.   Nishimura Y, Tajima G, Dwi Bahagia A, et al. Differential diagnosis of neonatal mild hypergalactosaemia detected by mass screening: clinical significance of portal vein imaging. Journal of Inherited Metabolic Disease. 2004; 27(1): 11-18.

34.   Eroglu Y, Donaldson J, Sorensen LG, et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. Journal of Pediatric Gastroenterology and Nutrition. 2004; 39(4): 410-417.

35.   Emre S, Amon R, Cohen E, et al. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transplantation. 2007; 13(12): 1662-1668.

36.   Kim MJ, Ko JS, Seo JK, et al. Clinical features of congenital portosystemic shunt in children. European Journal of Pediatrics. 2012; 171(2): 395-400.

37.   Timpanaro T, Passanisi S, Sauna A, et al. Congenital portosystemic shunt: our experience. Case Rep Pediatr. 2015; 691618.

38.   Chocarro G, Amesty MV, Encinas JL, et al. Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient. Eur J Pediatr Surg. 2016; 26(1): 74-80.

39.   Achiron R, Kivilevitch Z. Fetal umbilical-portal-systemic venous shunt: in utero classification and clinical significance. Ultrasound Obstet Gynecol. 2016; 47: 739-747.

40.   Franchi-Abella S, Gonzales E, Ackermann O, et al. Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY). 2018; 43(8): 2023-2036.

41.   Musa J, Madani K, Saliaj K, et al. Asymptomatic presentation of a congenital malformation of the portal vein with portosystemic shunt. Radiol Case Rep. 2020; 15(10): 2009-2014.

42.   Back SJ, Maya CL, Khwaja A. Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol. 2017; 47: 1069-1078.

43.   Nam HD. Living-donor liver transplantation for Abernethy malformation - case report and review of literature. Ann Hepatobiliary Pancreat Surg. 2020; 24(2): 203-208.

44.   Papamichail M, Pizanias M, Heaton N. Congenital portosystemic venous shunt. Eur J Pediatr. 2018; 177(3): 285-294. (АНГИОЛОГИЯ.ру) - портал о диагностике и лечении заболеваний сосудистой системы