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The portal vein in children: radiological review of congenital anomalies and acquired abnormalities

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Abstract

A variety of portal vein anomalies that occur in children can be identified by US, CT, MRI and portal venography. Although these abnormalities can also occur in adults, there are certain pathological processes and aberrations within the portal system that are specific to children. Knowledge of the embryology and anatomy of the portal vein is of benefit in the understanding of these anomalies. Identifying deviations from normal portal architecture is important in the work-up for surgery such as liver transplantation, and prior to interventional procedures such as stent placement or embolization. The aim of this paper is to summarize the various types of congenital and acquired portal vein abnormalities that occur in children, describe their radiological features and provide images to demonstrate the differences from normal portal venous anatomy.

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References

  1. Sadler T (2000) Cardiovascular system. In: Sadler TW (ed) Langman’s medical embryology, 8th edn. Lippincott Williams & Wilkins, Baltimore Philadelphia, pp 246–248

    Google Scholar 

  2. Niwa T, Aida N, Tachibana K, et al (2002) Congenital absence of the portal vein: clinical and radiological findings. J Comput Assist Tomogr 26:681–686

    Article  PubMed  Google Scholar 

  3. 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 172:227–229

    PubMed  CAS  Google Scholar 

  4. Kondo M, Itoh S, Kunikata T, et al (2001) Time of closure of ductus venosus in term and preterm neonates. Arch Dis Child Fetal Neonatal Ed 85:F57–F59

    Article  PubMed  CAS  Google Scholar 

  5. Patriquin HB, Perreault G, Grignon A, et al (1990) Normal portal venous diameter in children. Pediatr Radiol 20:451–453

    Article  PubMed  CAS  Google Scholar 

  6. Madoff DC, Hicks ME, Vauthey JN, et al (2002) Transhepatic portal vein embolisation: anatomy, indication and technical considerations. Radiographics 22:1063–1076

    PubMed  Google Scholar 

  7. Covey AM, Brody LA, Getrajdman G, et al (2004) Incidence, patterns, and clinical relevance of variant portal vein anatomy. AJR 183:1055–1064

    PubMed  Google Scholar 

  8. Atri M, Bret PM, Fraser-Hill MA (1992) Intrahepatic portal venous variations: prevalence with ultrasound. Radiology 184:157–158

    PubMed  CAS  Google Scholar 

  9. Tan K (1991) Anatomy of the liver and bile ducts. In: Howard ER (ed) Surgery of liver disease in children. Butterworth-Heinemann, Oxford, pp 17–25

    Google Scholar 

  10. Pearson J (1995) The portal venous system. In: Curry R, Tempkin B (eds) Ultrasonography: an introduction to normal structure and functional anatomy. Saunders, Philadelphia, pp 187–195

    Google Scholar 

  11. Carty H, Higham J, Crawford S (2001) The liver, gallbladder, biliary tree. In: Carty H, Higham J, Crawford S (eds) Pediatric ultrasound. Greenwich Medical Media, London, pp 75–76

    Google Scholar 

  12. Abernathy J (1793) Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc Lond 83:59–66

    Article  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  14. Howard E, Davenport M (1997) Congenital extrahepatic portocaval shunts— the Abernathy malformation. J Pediatr Surg 32:494–497

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  16. Shinkai M, Ohhama Y, Nishi T, et al (2001) Congenital absence of the portal vein and the role of liver transplantation in children. J Pediatr Surg 36:1026–1031

    Article  PubMed  CAS  Google Scholar 

  17. De Gaetano AM, Gui B, Macis R, et al (2004) Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: imaging and review of the literature. Abdom Imaging 29:455–459

    Article  PubMed  Google Scholar 

  18. Gallego C, Velasco M, Marcuello P, et al (2002) Congenital and acquired anomalies of the portal system. Radiographics 22:141–159

    PubMed  Google Scholar 

  19. Ozgun B, Warshauer D (1992) Absent medial segment of the left hepatic lobe: CT appearance. J Comput Assist Tomogr 16:666–668

    PubMed  CAS  Google Scholar 

  20. Sato M, Ishida K, Konno H, et al (2000) Congenital absence of the horizontal portion of the left portal vein: ultrasound findings. Eur Radiol 10:362–364

    Article  PubMed  CAS  Google Scholar 

  21. Radin R, Colletti PM, Ralls PW, et al (1987) Agenesis of the right lobe of the liver. Radiology 164:639–642

    PubMed  CAS  Google Scholar 

  22. Mitchell A, John P, Mayer D, et al (2002) Improved technique of portal vein reconstruction in pediatric liver transplant recipients with portal vein hypoplasia. Transplantation 73:1244–1247

    Article  PubMed  Google Scholar 

  23. Marks C (1969) Developmental basis of the portal venous system. Am J Surg 117:671–681

    Article  PubMed  CAS  Google Scholar 

  24. Park JH, Cha SH, Han JK, et al (1990) Intrahepatic portosystemic venous shunt. AJR 155:527–528

    PubMed  CAS  Google Scholar 

  25. Gallego C, Miralles M, Marin C, et al (2004) Congenital hepatic shunts. Radiographics 24:755–772

    Article  PubMed  Google Scholar 

  26. Kakuno Y, Matsuki M, Kani H, et al (2004) Intrahepatic portosystemic venous shunt: occurrence in a child patient without liver cirrhosis. Radiat Med 22:195–197

    PubMed  Google Scholar 

  27. Santamaria G, Pruna X, Serres X, et al (1996) Congenital intrahepatic portosystemic venous shunt: sonographic and magnetic resonance imaging. Eur Radiol 6:76–78

    Article  PubMed  CAS  Google Scholar 

  28. Kitagawa S, Gleason WA Jr, Northup H, et al (1992) Symptomatic hyperammonaemia caused by a congenital portosystemic shunt. J Pediatr 121:917–919

    Article  PubMed  CAS  Google Scholar 

  29. Kassarjian A, Dubois J, Burrows PE (2002) Angiographic classification of hepatic haemangiomas in infants. Radiology 222:693–698

    Article  PubMed  Google Scholar 

  30. McHugh K, Burrows PE (1992) Infantile hepatic haemangioendotheliomas: significance of portal venous and systemic collateral arterial supply. J Vasc Interv Radiol 3:337–344

    Article  PubMed  CAS  Google Scholar 

  31. Boon LM, Burrows PE, Paltiel HJ, et al (1996) Hepatic vascular anomalies in infancy: a twenty-seven-year experience. J Pediatr 129:346–354

    Article  PubMed  CAS  Google Scholar 

  32. Burrows PE, Dubois J, Kassarjian A (2001) Pediatric hepatic vascular anomalies. Pediatr Radiol 31:533–545

    Article  PubMed  CAS  Google Scholar 

  33. Marchand V, Uflacker R, Baker SS, et al (1999) Congenital arterio-hepatic fistula in a 3-year-old child. J Pediatr Gastroenterol Nutr 28:435–441

    Article  PubMed  CAS  Google Scholar 

  34. Altuntas B, Erden A, Karakurt C, et al (1998) Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm. J Clin Ultrasound 26:357–360

    Article  PubMed  CAS  Google Scholar 

  35. Sachdeva R, Yapor M, Schwersenz A, et al (1993) Massive variceal bleeding caused by a hepatic artery-portal vein fistula: a manifestation of hepatocellular carcinoma in a 12-year-old girl. J Pediatr Gastroenterol Nutr 16:468–471

    Article  PubMed  CAS  Google Scholar 

  36. Bakker J, Robben S, Hazebroek F, et al (1994) Congenital arterioportal fistula of the liver with reversal of flow in the superior mesenteric vein. Pediatr Radiol 24:198–199

    Article  PubMed  CAS  Google Scholar 

  37. Vauthey J, Tomczak R, Helmberger T, et al (1997) The arterioportal fistula syndrome: clinicopathologic features, diagnosis and therapy. Gastroenterology 113:1390–1401

    Article  PubMed  CAS  Google Scholar 

  38. Duff D, Nihill M, McNamara D (1977) Infradiaphragmatic total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J 39:619–626

    Article  PubMed  CAS  Google Scholar 

  39. Benson C, Taylor A, Rossi UG, et al (2005) Three-dimensional anatomy of the great vessels defined by 16-slice multi-detector angiography in neonates, infants, children and adolescents with congenital heart disease (abstract). Pediatr Radiol 35(Suppl 2):S109

    Google Scholar 

  40. Marks C (1974) Surgical implications of portal venous system malformation. Ann R Coll Surg Engl 55:299–306

    PubMed  CAS  Google Scholar 

  41. Choi S, Park W (1995) Preduodenal portal vein: a cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg 30:1521–1522

    Article  PubMed  CAS  Google Scholar 

  42. Fernandes E, Burton E, Hixson S, et al (1990) Preduodenal portal vein: surgery and radiological appearance. J Pediatr Surg 25:1270–1272

    Article  PubMed  CAS  Google Scholar 

  43. Snavely JG, Breakell ES (1954) Fatal hemorrhage from esophageal varices due to malformations and congenital stenoses in the portal venous system. Am J Med 16:459–464

    Article  PubMed  CAS  Google Scholar 

  44. Khan MH, Yaqub N, Ashraf M (2004) Complete liver duplication with right central diaphragmatic defect. J Coll Physicians Surg Pak 14:504–505

    PubMed  Google Scholar 

  45. Wolfe JN, Evans WA (1955) Gas in the portal vein of the liver in infants: a roentgenographic demonstration with post-mortem anatomical correlation. AJR 74:486–489

    CAS  Google Scholar 

  46. Sebastià C, Quiroga S, Espin E, et al (2000) Portomesenteric vein gas: pathological mechanisms, CT findings and prognosis. Radiographics 20:1213–1224

    PubMed  Google Scholar 

  47. Buonomo C (1999) The radiology of necrotising enterocolitis. Radiol Clin North Am 37:1187–1196

    Article  PubMed  CAS  Google Scholar 

  48. Chevallier P, Peten E, Souci J, et al (2002) Detection of portal venous gas on sonography, but not on CT. Eur Radiol 12:1175–1178

    Article  PubMed  Google Scholar 

  49. Gürakan F, Eren M, Koçak N, et al (2004) Extrahepatic portal vein thrombosis in children. Etiology and long-term follow up. J Clin Gastroenterol 38:368–372

    Article  PubMed  Google Scholar 

  50. Sobhonslidsuk A, Reddy K (2002) Portal vein thrombosis: a concise review. Am J Gastroenterol 97:535–539

    Article  PubMed  Google Scholar 

  51. De Gaetano AM, Lafortune M, Patriquin H, et al (1995) Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR 165:1151–1155

    PubMed  Google Scholar 

  52. Thomas TV (1967) Aneurysm of the portal vein: report of two cases, one resulting in thrombosis and spontaneous rupture. Surgery 61:550–555

    PubMed  CAS  Google Scholar 

  53. Gallagher DM, Leiman S, Hux C (1993) In utero diagnosis of a portal vein aneurysm. J Clin Ultrasound 21:147–151

    Article  PubMed  CAS  Google Scholar 

  54. Schild H, Schweden F, Braun B, et al (1982) Aneurysm of the superior mesenteric vein. Radiology 145:641–642

    PubMed  CAS  Google Scholar 

  55. Blasbalg R, Yamada R, Tieferes D (2000) Extrahepatic portal vein aneurysms. AJR 174:877

    PubMed  CAS  Google Scholar 

  56. Marwan IK, Fawzy TI, Egawa H, et al (1999) Innovative techniques for and results of portal vein reconstruction in living-related liver transplantation. Surgery 125:265–270

    PubMed  CAS  Google Scholar 

  57. Buell JF, Funaki B, Cronin DC, et al (2002) Long-term venous complications after full-size and segmental pediatric liver transplantation. Ann Surg 236:658–666

    Article  PubMed  Google Scholar 

  58. Kirsch JP, Howard TK, Klintmalm GB, et al (1990) Problematic vascular reconstruction in liver transplantation. Part II. Portovenous conduits. Surgery 107:544–548

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Radiology, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK

    Jonathan A. G. Corness, Kieran McHugh & Derek J. Roebuck

  2. Cardiothoracic Unit, Institute of Child Health, London, UK

    Andrew M. Taylor

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  1. Jonathan A. G. Corness
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  2. Kieran McHugh
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  3. Derek J. Roebuck
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  4. Andrew M. Taylor
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Correspondence to Kieran McHugh.

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Corness, J.A.G., McHugh, K., Roebuck, D.J. et al. The portal vein in children: radiological review of congenital anomalies and acquired abnormalities. Pediatr Radiol 36, 87–96 (2006). https://doi.org/10.1007/s00247-005-0010-4

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