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Published in:

01-08-2018

Congenital portosystemic shunts: diagnosis and treatment

Authors: Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Branchereau, Danièle Pariente, Florent Guérin, the International Registry of Congenital Portosystemic Shunt members

Published in: Abdominal Radiology | Issue 8/2018

Abstract

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.

Franchi-Abella S, Branchereau S, Lambert V, et al. (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330. https://doi.org/10.1097/MPG.0b013e3181d9cb92 PubMedCrossRef

Blanc T, Guerin F, Franchi-Abella S, et al. (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260:188–198. https://doi.org/10.1097/SLA.0000000000000266 CrossRefPubMed

Mavrides E, Moscoso G, Carvalho JS, et al. (2001) The anatomy of the umbilical, portal and hepatic venous systems in the human fetus at 14-19 weeks of gestation. Ultrasound Obstet Gynecol 18:598–604. https://doi.org/10.1046/j.0960-7692.2001.00581.x CrossRefPubMed

Hikspoors JPJM, Soffers JHM, Mekonen HK, et al. (2015) Development of the human infrahepatic inferior caval and azygos venous systems. J Anat 226:113–125. https://doi.org/10.1111/joa.12266 CrossRefPubMed

Hikspoors JPJM, Peeters MMJP, Kruepunga N, et al. (2017) Human liver segments: role of cryptic liver lobes and vascular physiology in the development of liver veins and left-right asymmetry. Sci Rep 7:17109. https://doi.org/10.1038/s41598-017-16840-1 CrossRefPubMedPubMedCentral

Hikspoors JPJM, Peeters MMJP, Mekonen HK, et al. (2017) The fate of the vitelline and umbilical veins during the development of the human liver. J Anat 231:718–735. https://doi.org/10.1111/joa.12671 CrossRefPubMed

Collardeau-Frachon S, Scoazec J-Y (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627. https://doi.org/10.1002/ar.20679 CrossRef

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

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

10.

Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21:147–157. https://doi.org/10.1002/ca.20574 CrossRefPubMed

11.

Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308–314. https://doi.org/10.1016/j.jpedsurg.2010.11.009 CrossRefPubMed

12.

Kanazawa H, Nosaka S, Miyazaki O, et al. (2015) The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 50:688–695. https://doi.org/10.1016/j.jpedsurg.2015.01.009 CrossRefPubMed

13.

Back SJ, Maya CL, Khwaja A (2017) Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol 47:1069–1078. https://doi.org/10.1007/s00247-017-3869-y CrossRefPubMed

14.

Bernard O, Franchi-Abella S, Branchereau S, et al. (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287. https://doi.org/10.1055/s-0032-1329896 CrossRefPubMed

15.

Cho Y, Tokuhara D, Shimono T, et al. (2014) Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt. Pediatr Res 75:658–662. https://doi.org/10.1038/pr.2014.11 CrossRefPubMed

16.

Shiomi S, Sasaki N, Ikeoka N, et al. (1998) Usefulness of per-rectal portal scintigraphy with Tc-99m pertechnetate for galactosemia in infants. Ann Nucl Med 12:375–378CrossRefPubMed

17.

Uchino T, Matsuda I, Endo F (1999) The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 135:254–256CrossRefPubMed

18.

Chocarro G, Amesty MV, Encinas JL, et al. (2016) Congenital portosystemic shunts: clinic heterogeneity requires an individual management of the patient. Eur J Pediatr Surg 26:74–80. https://doi.org/10.1055/s-0035-1566097 PubMedCrossRef

19.

Achiron R, Kivilevitch Z (2016) Fetal umbilical-portal-systemic venous shunt: in utero classification and clinical significance. Ultrasound Obstet Gynecol 47:739–747. https://doi.org/10.1002/uog.14906 CrossRefPubMed

20.

Loberant N, Herskovits M, Barak M, et al. (1999) Closure of the ductus venosus in premature infants: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography. AJR Am J Roentgenol 172:227–229. https://doi.org/10.2214/ajr.172.1.9888772 CrossRefPubMed

21.

Loberant N, Barak M, Gaitini D, et al. (1992) Closure of the ductus venosus in neonates: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography. AJR Am J Roentgenol 159:1083–1085. https://doi.org/10.2214/ajr.159.5.1414780 CrossRefPubMed

22.

Farrant P, Meire HB, Karani J (1996) Ultrasound diagnosis of portocaval anastomosis in infants—a report of eight cases. Br J Radiol 69:389–393. https://doi.org/10.1259/0007-1285-69-821-389 CrossRefPubMed

23.

Ono H, Mawatari H, Mizoguchi N, et al. (1998) Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr Oslo Nor 87:631–634CrossRef

24.

Francois B, Gottrand F, Lachaux A, et al. (2017) Outcome of intrahepatic portosystemic shunt diagnosed prenatally. Eur J Pediatr . https://doi.org/10.1007/s00431-017-3013-x CrossRefPubMed

25.

Golewale N, Paltiel HJ, Fishman SJ, Alomari AI (2010) Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach. J Pediatr Surg 45:1676–1681. https://doi.org/10.1016/j.jpedsurg.2010.03.009 CrossRefPubMed

26.

Tanya Chun P, Chun T, Files M, et al. (2013) Percutaneous embolization of congenital portosystemic venous fistula in an infant with down syndrome. Case Rep Vasc Med 2013:127023. https://doi.org/10.1155/2013/127023 PubMedPubMedCentralCrossRef

27.

Yamaguchi H, Kosugiyama K, Honda S, et al. (2016) Down syndrome with patent ductus venosus and hepato-biliary-pancreatic abnormalities. Indian J Pediatr 83:78–80. https://doi.org/10.1007/s12098-015-1797-0 CrossRefPubMed

28.

Pérez-García C, Martín YR, Del Hoyo AA, et al. (2017) Adams-Oliver syndrome with unusual central nervous system findings and an extrahepatic portosystemic shunt. Pediatr Rep 9:7211. https://doi.org/10.4081/pr.2017.7211 CrossRefPubMedPubMedCentral

29.

Sokollik C, Bandsma RHJ, Gana JC, et al. (2013) Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 56:675–681. https://doi.org/10.1097/MPG.0b013e31828b3750 CrossRefPubMed

30.

Uchino T, Endo F, Ikeda S, et al. (1996) Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus. Gastroenterology 110:1964–1968CrossRefPubMed

31.

Shinkai M, Ohhama Y, Nishi T, et al. (2001) Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 36:1026–1031. https://doi.org/10.1053/jpsu.2001.24731 CrossRefPubMed

32.

Cho Y, Shimono T, Morikawa H, et al. (2014) Hepatic focal nodular hyperplasia with congenital portosystemic shunt. Pediatr Int 56:e102–105. https://doi.org/10.1111/ped.12456 CrossRefPubMed

33.

Gordon-Burroughs S, Balogh J, Weiner MA, et al. (2014) Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 46:2418–2421. https://doi.org/10.1016/j.transproceed.2014.04.012 CrossRefPubMed

34.

Sanada Y, Mizuta K, Niki T, et al. (2015) Hepatocellular nodules resulting from congenital extrahepatic portosystemic shunts can differentiate into potentially malignant hepatocellular adenomas. J Hepato Biliary Pancreat Sci 22:746–756. https://doi.org/10.1002/jhbp.277 CrossRef

35.

Benedict M, Rodriguez-Davalos M, Emre S, et al. (2017) Congenital extrahepatic portosystemic shunt (abernethy malformation type Ib) with associated hepatocellular carcinoma: case report and literature review. Pediatr Dev Pathol 20:354–362. https://doi.org/10.1177/1093526616686458 CrossRefPubMed

36.

Lautz TB, Shah SA, Superina RA (2016) Hepatoblastoma in children with congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 62:542–545. https://doi.org/10.1097/MPG.0000000000001012 CrossRefPubMed

37.

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

38.

Takahashi S, Yoshida E, Sakanishi Y, et al. (2014) Congenital multiple intrahepatic portosystemic shunt: an autopsy case. Int J Clin Exp Pathol 7:425–431PubMed

39.

Nardone R, Taylor AC, Höller Y, et al. (2016) Minimal hepatic encephalopathy: a review. Neurosci Res 111:1–12. https://doi.org/10.1016/j.neures.2016.04.009 CrossRefPubMed

40.

Matsuura T, Takahashi Y, Yanagi Y, et al. (2016) Surgical strategy according to the anatomical types of congenital portosystemic shunts in children. J Pediatr Surg 51:2099–2104. https://doi.org/10.1016/j.jpedsurg.2016.09.046 CrossRefPubMed

41.

Edula RGR, Pyrsopoulos NT (2015) New methods of testing and brain imaging in hepatic encephalopathy: a review. Clin Liver Dis 19:449–459. https://doi.org/10.1016/j.cld.2015.04.001 CrossRefPubMed

42.

Kobayashi D, Edwards HD, Singh J, et al. (2011) Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: a cause of sudden death in an infant. Pediatr Pulmonol 46:1041–1044. https://doi.org/10.1002/ppul.21463 CrossRefPubMed

43.

Lin W-C, Hsu T-W, Chen C-L, et al. (2014) Reestablishing brain networks in patients without overt hepatic encephalopathy after liver transplantation. J Cereb Blood Flow Metab 34:1877–1886. https://doi.org/10.1038/jcbfm.2014.143 CrossRefPubMedPubMedCentral

44.

da Rocha AJ, Braga FT, da Silva CJ, et al. (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

45.

Ohno T, Muneuchi J, Ihara K, et al. (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–899. https://doi.org/10.1542/peds.2006-3411 CrossRefPubMed

46.

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

47.

Fu L, Wang Q, Wu J, et al. (2016) Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome. Eur J Pediatr 175:195–201. https://doi.org/10.1007/s00431-015-2623-4 CrossRefPubMed

48.

Knirsch W, Benz DC, Bühr P, et al. (2016) Catheter interventional treatment of congenital portosystemic venous shunts in childhood. Catheter Cardiovasc Interv 87:1281–1292. https://doi.org/10.1002/ccd.26362 CrossRefPubMed

49.

Layangool T, Kojaranjit V, Promphan W, et al. (2014) An unusual cause of progressive cyanosis post Fontan operation: congenital extra-hepatic porto-systemic shunt. J Med Assoc Thail Chotmaihet Thangphaet 97(Suppl 6):S176–181

50.

Raghuram KA, Bijulal S, Krishnamoorthy KM, Tharakan JA (2013) Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol 34:1882–1885. https://doi.org/10.1007/s00246-012-0428-z CrossRefPubMed

51.

Newman B, Feinstein JA, Cohen RA, et al. (2010) Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol 40:1222–1230. https://doi.org/10.1007/s00247-009-1508-y CrossRefPubMed

52.

Grimon G, André L, Bernard O, et al. (1994) Early radionuclide detection of intrapulmonary shunts in children with liver disease. J Nucl Med 35:1328–1332PubMed

53.

Alvarez AE, Ribeiro AF, Hessel G, et al. (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394. https://doi.org/10.1002/ppul.10182 CrossRefPubMed

54.

Ohnishi Y, Ueda M, Doi H, et al. (2005) Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess. J Pediatr Surg 40:e1–3. https://doi.org/10.1016/j.jpedsurg.2005.02.011 CrossRefPubMed

55.

Bellah RD, Hayek J, Teele RL (1989) Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 20:115–117CrossRefPubMed

56.

McKie PM, McCully RB, Kamath PS, et al. (2012) Amelioration of high cardiac output and pulmonary hypertension by occlusion of congenital porto-systemic shunt. Circulation 126:2533–2534. https://doi.org/10.1161/CIRCULATIONAHA.112.106609 CrossRefPubMed

57.

Sumida W, Kaneko K, Ogura Y, et al. (2006) Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure. J Pediatr Surg 41:e9–e12. https://doi.org/10.1016/j.jpedsurg.2006.07.014 CrossRefPubMed

58.

Karashima S, Hattori S, Nakazato H, et al. (2000) Membranoproliferative glomerulonephritis in congenital portosystemic shunt without liver cirrhosis. Clin Nephrol 53:206–211PubMed

59.

Lee SH, Lee D-G (2015) Macroscopic hematuria caused by congenital portosystemic shunt and concomitant nutcracker syndrome. Pediatr Int 57:e84–86. https://doi.org/10.1111/ped.12671 CrossRefPubMed

60.

Gong Y, Zhu H, Chen J, et al. (2015) Congenital portosystemic shunts with and without gastrointestinal bleeding—case series. Pediatr Radiol 45:1964–1971. https://doi.org/10.1007/s00247-015-3417-6 CrossRefPubMed

61.

Bas S, Guran T, Atay Z, et al. (2015) Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation) in children. Horm Res Paediatr 83:282–287. https://doi.org/10.1159/000369395 CrossRefPubMed

62.

Satoh M, Yokoya S, Hachiya Y, et al. (2001) Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 160:307–311CrossRefPubMed

63.

Senniappan S, Pitt K, Shah P, et al. (2015) Postprandial hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt. Horm Res Paediatr 83:217–220. https://doi.org/10.1159/000369014 CrossRefPubMed

64.

Knisely AS (2014) Patent ductus venosus and acute liver failure in the neonate: consider neonatal hemochromatosis with liver scarring. Liver Transplant 20:124. https://doi.org/10.1002/lt.23768 CrossRef

65.

Scalabre A, Gorincour G, Hery G, et al. (2012) Evolution of congenital malformations of the umbilical-portal-hepatic venous system. J Pediatr Surg 47:1490–1495. https://doi.org/10.1016/j.jpedsurg.2012.02.022 CrossRefPubMed

66.

Matsuura T, Yanagi Y, Saeki I, et al. (2011) Outcome of modified portal vein anastomosis for recipients with portal vein thrombosis or stenosis before living donor liver transplantation. J Pediatr Surg 46:2291–2295. https://doi.org/10.1016/j.jpedsurg.2011.09.015 CrossRefPubMed

67.

Kuo MD, Miller FJ, Lavine JE, et al. (2010) Exploiting phenotypic plasticity for the treatment of hepatopulmonary shunting in Abernethy malformation. J Vasc Interv Radiol JVIR 21:917–922. https://doi.org/10.1016/j.jvir.2010.01.038 CrossRefPubMed

Title: Congenital portosystemic shunts: diagnosis and treatment
Authors: Stéphanie Franchi-Abella
Emmanuel Gonzales
Oanez Ackermann
Sophie Branchereau
Danièle Pariente
Florent Guérin
the International Registry of Congenital Portosystemic Shunt members
Publication date: 01-08-2018
Publisher: Springer US
Published in: Abdominal Radiology / Issue 8/2018
Print ISSN: 2366-004X
Electronic ISSN: 2366-0058
DOI: https://doi.org/10.1007/s00261-018-1619-8

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