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Open Access 01-03-2018 | Review

Congenital portosystemic venous shunt

Authors: M. Papamichail, M. Pizanias, N. Heaton

Published in: European Journal of Pediatrics | Issue 3/2018

Abstract

Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension.

Conclusion: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment.

What is Known:

• The natural history and basic management of this rare congenital anomaly are presented.

What is New:

• This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

Kim MJ, Ko JS, Seo JK, Yang HR, Chang JY, Kim GB et al (2012) Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 171(2):395–400CrossRefPubMed

Florio F, Nardella M, Balzano S, Giacobbe A, Perri F (1998) Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 21(5):421–424CrossRefPubMed

Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32(4):273–287CrossRefPubMed

Lin ZY, Chen SC, Hsieh MY, Wang CW, Chuang WL, Wang LY (2006) Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause. J Clin Ultrasound 34(1):22–26CrossRefPubMed

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

Account of two instances of uncommon formation, in the viscera of the human body. By Mr. John Abernethy, Assistant Surgeon to St. Bartholomew’s Hospital. Communicated by Sir Joseph Banks, Bart. P. R. S. Author(s): John Abernethy and Joseph Banks Source: Philosophical Transactions of the Royal Society of London, Vol. 83 (1793), pp. 59–66 Published by: Royal Society Stable

Guérin F, Blanc T, Gauthier F, Abella SF, Branchereau S (2012) Congenital portosystemic vascular malformations. Semin Pediatr Surg 21(3):233–244 Review CrossRefPubMed

Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21(2):147–157CrossRefPubMed

Tanoue S, Kiyosue H, Komatsu E, Hori Y, Maeda T, Mori H (2003) Symptomatic intrahepatic portosystemic venous shunt: embolization with an alternative approach. AJR Am J Roentgenol 181(1):71–78CrossRefPubMed

10.

Yu HC, Kim JH, Murakami G, Rodríguez-Vázquez JF, Cho BH (2015) An anomalous portal vein crossing the lesser sac and ending at the upper part of ductus venosus. Anat Cell Biol 48(3):218–221CrossRefPubMedPubMedCentral

11.

Senocak E, Oğuz B, Edgüer T, Cila A (2008) Congenital intrahepatic portosystemic shunt with variant inferior right hepatic vein. Diagn Interv Radiol 14:97–99PubMed

12.

Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M (2011) Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics 31:707–722CrossRefPubMed

13.

Kim IO, Cheon JE, Kim WS, Chung JW, Yeon KM, Yoo SJ et al (2000) Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol 30:336–338CrossRefPubMed

14.

Yoshimoto Y, Shimizu R, Saeki T, Harada T, Sugio Y, Nomura S et al (2004) Patent ductus venosus in children: a case report and review of the literature. J Pediatr Surg 39:E1–E5CrossRefPubMed

15.

Kamimatsuse A, Onitake Y, Kamei N, Tajima G, Sakura N, Sueda T et al (2010) Surgical intervention for patent ductus venosus. Pediatr Surg Int 26:1025–1030CrossRefPubMed

16.

Saito M, Seo Y, Yano Y, Momose K, Hirano H, Yoshida M et al (2013) Successful treatment using coil embolization of a symptomatic intrahepatic portosystemic venous shunt developing through a patent ductus venosus in a noncirrhotic adult. Intern Med 52(5):555–559CrossRefPubMed

17.

Newman B, Feinstein JA, Cohen RA, Feingold B, Kreutzer J, Patel H et al (2010) Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol 40(7):1222–1230CrossRefPubMed

18.

Takahashi S, Yoshida E, Sakanishi Y, Tohyama N, Ayhan A, Ogawa H (2013) Congenital multiple intrahepatic portosystemic shunt: an autopsy case. Int J Clin Exp Pathol 7:425–431PubMedPubMedCentral

19.

Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011 Feb) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46(2):308–314CrossRefPubMed

20.

Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC (2013) Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 56:675–681CrossRefPubMed

21.

Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J et al (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330PubMed

22.

Papamichail M, Ali A, Quaglia A, Karani J, Heaton N (2016) Liver resection for the treatment of a congenital intrahepatic portosystemic venous shunt. Hepatobiliary Pancreat Dis Int. 15(3):329–333CrossRefPubMed

23.

Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528CrossRefPubMed

24.

Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29:1239–1241CrossRefPubMed

25.

Howard ER, Davenport M (1997) Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 32(3):494–497CrossRefPubMed

26.

Kamata S, Kitayama Y, Usui N, Kuroda S, Nose K, Sawai T et al (2000) Patent ductus venosus with a hypoplastic intrahepatic portal system presenting intrapulmonary shunt: a case treated with banding of the ductus venosus. J Pediatr Surg 35(4):655–657CrossRefPubMed

27.

Takehara Y, Mori K, Edagawa T, Sugimoto M, Takehara H, Ito M et al (2004) Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: importance of direct occlusion test of ductus venosus under open laparotomy. Pediatr Int 46(4):484–486CrossRefPubMed

28.

Stewart JK, Kuo WT, Hovsepian DM, Hofmann LV, Bonham CA, Sze DY. Portal venous remodeling after endovascular reduction of pediatric autogenous portosystemic shunts. J Vasc Interv Radiol 2011;22(8):1199–1205

29.

Kanazawa H, Nosaka S, Miyazaki O, Sakamoto S, Fukuda A, Shigeta T et al (2015) The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 50(4):688–695 ReviewCrossRefPubMed

30.

Kobayashi N, Niwa T, Kirikoshi H, Fujita K, Yoneda M, Saito S et al (2010) Clinical classification of congenital extrahepatic portosystemic shunts. Hepatol Res 40(6):585–593CrossRefPubMed

31.

Blanc T, Guerin F, Franchi-Abella S, Jacquemin E, Pariente D, Soubrane O et al (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260(1):188–198CrossRefPubMed

32.

Grimaldi C, Monti L, Falappa P, d’Ambrosio G, Manca A, de Ville de Goyet J (2012) Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 47:e27–e31CrossRefPubMed

33.

Delle Chiaie L, Neuberger P, Von Kalle T (2008) Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol 32:233–235CrossRefPubMed

34.

Nagasaka H, Miida T, Yorifuji T, Hirano K, Inui A, Fujisawa T et al (2013) Metabolic improvements in intrahepatic porto-systemic venous shunt presenting various metabolic abnormalities by 4-phenylacetate. Clin Chim Acta 419:52–56CrossRefPubMed

35.

Yamagami T, Nakamura T, Tokiwa K, Ohno K, Itoh H, Maeda T (2000) Intrahepatic portosystemic venous shunt associated with biliary atresia: case report. Pediatr Radiol 30:489–491CrossRefPubMed

36.

Fu L, Wang Q, Wu J, Guo Y, Huang M, Liu T et al (2016 Feb) Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. Eur J Pediatr 175(2):195–201CrossRefPubMed

37.

Ohno T, Muneuchi J, Ihara K, Yuge T, Kanaya Y, Yamaki S et al (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–e899CrossRefPubMed

38.

Knirsch W, Benz DC, Bühr P, Quandt D, Weber R, Kellenberger C et al (2016) Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv 87(7):1281–1292CrossRefPubMed

39.

Pupulim LF, Vullierme MP, Paradis V, Valla D, Terraz S, Vilgrain V (2013) Congenital portosystemic shunts associated with liver tumours. Clin Radiol 68:e362–e369CrossRefPubMed

40.

Kawano S, Hasegawa S, Urushihara N, Okazaki T, Yoshida A, Kusafuka J et al (2007) Hepatoblastoma with congenital absence of the portal vein: a case report. Eur J Pediatr Surg 17(4):292–294CrossRefPubMed

41.

Lautz TB, Shah SA, Superina RA (2016) Hepatoblastoma in children with congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 62(4):542–545CrossRefPubMed

42.

Konstas AA, Digumarthy SR, Avery LL, Wallace KL, Lisovsky M, Misdraji J et al (2011) Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol 80:175–181CrossRefPubMed

43.

Murray CP, Yoo SJ, Babyn PS (2003) Congenital extrahepatic portosystemic shunts. Pediatr Radiol 33:614–620CrossRefPubMed

44.

Sorkin T, Strautnieks S, Foskett P, Peddu P, Thompson RJ, Heaton N et al (2016) Multiple β-catenin mutations in hepatocellular lesions arising in Abernethy malformation. Hum Pathol 53:153–158CrossRefPubMed

45.

Gong Y, Zhu H, Chen J, Chen Q, Ji M, Pa M et al (2015) Congenital portosystemic shunts with and without gastrointestinal bleeding—case series. Pediatr Radiol 45(13):1964–1971CrossRefPubMed

46.

Singh K, Kapoor A, Kapoor A, Gupta K, Mahajan G (2006) Congenital intrahepatic portosystemic shunt—an incidental rare anomaly. Indian J Pediatr 73:1122–1123CrossRefPubMed

47.

Torigoe M, Maeshima K, Takeshita Y (2013) Congenital intrahepatic portosystemic venous shunt presenting with paraparesis as the initial symptom. Intern Med 52:2439–2442CrossRefPubMed

48.

da Rocha AJ, Braga FT, da Silva CJ, Maia AC Jr, Mourão GS, Gagliardi RJ (2004) Reversal of parkinsonism and portosystemic encephalopathy following embolization of a congenital intrahepatic venous shunt: brain MR imaging and 1H spectroscopic findings. AJNR Am J Neuroradiol 25:1247–1250PubMed

49.

Lee SH, Lee DG (2015) Macroscopic hematuria caused by congenital portosystemic shunt and concomitant nutcracker syndrome. Pediatr Int 57(3):e84–e86CrossRefPubMed

50.

Grazioli L, Alberti D, Olivetti L, Rigamonti W, Codazzi F, Matricardi L et al (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 10(5):820–825CrossRefPubMed

51.

Caiulo VA, Presta G, Latini G, Mattioli G, Jasonni V (2001) Diagnosis and follow-up of congenital intrahepatic portosystemic venous shunt by ultrasounds. Acta Paediatr 90:1209–1210CrossRefPubMed

52.

Cho Y, Tokuhara D, Shimono T, Yamamoto A, Higashiyama S, Kotani K et al (2014) Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt. Pediatr Res 75(5):658–662CrossRefPubMed

53.

Córdoba J (2011) New assessment of hepatic encephalopathy. J Hepatol 54:1030–1040CrossRefPubMed

54.

Sharma R, Suddle A, Quaglia A, Peddu P, Karani J, Satyadas T et al (2015) Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int 14(5):552–557CrossRefPubMed

55.

Lisovsky M, Konstas AA, Misdraji J (2011) Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation. Am J Surg Pathol 35(9):1381–1390CrossRefPubMed

56.

Barton JW 3rd, Keller MS (1989) Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 20:113–114CrossRefPubMed

57.

Turkbey B, Karcaaltincaba M, Demir H, Akcoren Z, Yuce A, Haliloglu M (2006) Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 36:445–448CrossRefPubMed

58.

Palvanov A, Marder RL, Siegel D (2016 Sep) Asymptomatic intrahepatic portosystemic venous shunt: to treat or not to treat? Int J Angiol 25(3):193–198PubMed

59.

Paganelli M, Lipsich JE, Sciveres M, Alvarez F (2015) Predisposing factors for spontaneous closure of congenital portosystemic shunts. J Pediatr 167(4):931–935 e12CrossRefPubMed

60.

Yoshimatsu R, Takeuchi Y, Morishita H, Iida N, Okabe H, Yamagami T et al (2006) Successful embolisation of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes. Br J Radiol 79:e162–e165CrossRefPubMed

61.

Wu L, Zhao L, Lu Y, He L, Hu X (2016) Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 46(4):541–547CrossRefPubMed

62.

Suzuki K, Shimohira M, Hashizume T, Suzuki Y, Shibamoto Y (2013) Dual microcather-dual detachable coil technique in embolization for a congenital intrahepatic portosystemic venous shunt (IPSVS). Minim Invasive Ther Allied Technol 22:316–318CrossRefPubMed

63.

Power AH, Bjarnason H (2012) Large spontaneous intrahepatic portal-systemic venous shunt treated with coil and Amplatzer vascular plug embolization. Perspect Vasc Surg Endovasc Ther 24(2):90–94CrossRefPubMed

64.

Chandrasekharan R, Pullara SK, Thomas T, Kader NP, Moorthy S (2016) Congenital intrahepatic portosystemic shunts: imaging findings and endovascular management. Indian J Radiol Imaging 26(1):92–94CrossRefPubMedPubMedCentral

65.

Guneyli S, Cinar C, Bozkaya H, Parildar M, Oran I, Akin Y (2012) Successful transcatheter closure of a congenital high-flow portosystemic venous shunt with the Amplatzer vascular plug II. Perspect Vasc Surg Endovasc Ther 24(4):202–205CrossRefPubMed

66.

Lee SA, Lee YS, Lee KS, Jeon GS (2010) Congenital intrahepatic portosystemic venous shunt and liver mass in a child patient: successful endovascular treatment with an amplatzer vascular plug (AVP). Korean J Radiol 11(5):583–586CrossRefPubMedPubMedCentral

67.

Sanada Y, Urahashi T, Ihara Y, Wakiya T, Okada N, Yamada N et al (2012 Mar) The role of operative intervention in management of congenital extrahepatic portosystemic shunt. Surgery 151(3):404–411CrossRefPubMed

68.

Matsuura T, Takahashi Y, Yanagi Y, Yoshimaru K, Yamamura K, Morihana E et al (2016) Surgical strategy according to the anatomical types of congenital portosystemic shunts in children. J Pediatr Surg 51(12):2099–2104CrossRefPubMed

69.

Kimura T, Soh H, Hasegawa T, Sasaki T, Kuroda S, Yuri E et al (2004) Laparoscopic correction of congenital portosystemic shunt in children. Surg Laparosc Endosc Percutan Tech 14(5):285–288CrossRefPubMed

70.

Gordon-Burroughs S, Balogh J, Weiner MA, Monsour HP Jr, Schwartz MR, Gaber AO et al (2014) Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 46(7):2418–2421CrossRefPubMed

71.

Sakamoto S, Shigeta T, Fukuda A, Tanaka H, Nakazawa A, Nosaka S et al (2012) The role of liver transplantation for congenital extrahepatic portosystemic shunt. Transplantation 93(12):1282–1287CrossRefPubMed

72.

Sakamoto S, Kasahara M, Shigeta T, Fukuda A, Kakiuchi T, Miyasaka M et al (2011) Living donor liver transplantation for multiple intrahepatic portosystemic shunts after involution of infantile hepatic hemangiomas. J Pediatr Surg 46(6):1288–1291CrossRefPubMed

Title: Congenital portosystemic venous shunt
Authors: M. Papamichail
M. Pizanias
N. Heaton
Publication date: 01-03-2018
Publisher: Springer Berlin Heidelberg
Published in: European Journal of Pediatrics / Issue 3/2018
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-017-3058-x

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