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Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings

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Abstract

We describe a 10-year-old girl with congenital absence of the portal vein (CAPV) and multiple hyperplastic nodules in the liver. MRI appearances of the liver lesions and the portocaval anastomosis between the inferior mesenteric vein and internal iliac veins are presented. In addition, the relevance of CAPV and nodular lesions of the liver is reviewed.

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References

  1. Matsuoka Y, Ohtomo K, Okubo T, et al (1992) Congenital absence of the portal vein. Gastrointest Radiol 17:31–33

    Article  PubMed  CAS  Google Scholar 

  2. Grazioli L, Alberti D, Olivetti L, et al (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 10:820–825

    Article  PubMed  CAS  Google Scholar 

  3. Motoori S, Shinozaki M, Goto N, et al (1997) Congenital absence of the portal vein associated with nodular hyperplasia in the liver. J Gastroenterol Hepatol 12:639–643

    PubMed  CAS  Google Scholar 

  4. Komatsu S, Nagino M, Hayakawa N, et al (1995) Congenital absence of portal venous system associated with a large inferior mesenteric-caval shunt: a case report. Hepatogastroenterology 42:286–290

    PubMed  CAS  Google Scholar 

  5. Arana E, Marti-Bonmati L, Martinez V, et al (1997) Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging 22:506–508

    Article  PubMed  CAS  Google Scholar 

  6. Morgan BG, 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 

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

    Article  PubMed  CAS  Google Scholar 

  8. Nakasaki H, Tanaka Y, Ohta M, et al (1989) Congenital absence of the portal vein. Ann Surg 210:190–193

    Article  PubMed  CAS  Google Scholar 

  9. Tanaka Y, Takayanagi M, Shiratori Y, et al (2003) Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol 38:288–294

    Article  PubMed  Google Scholar 

  10. Kim T, Murakami T, Sugihara E, et al (2004) Hepatic nodular lesions associated with abnormal development of the portal vein. AJR 183:1333–1338

    PubMed  Google Scholar 

  11. Bioulac-Sage P, Saric J, Boussarie L, et al (1985) Congenital portocaval shunt in rats: liver adaptation to lack of portal vein—a light and electron microscopic study. Hepatology 5:1183–1189

    Article  PubMed  CAS  Google Scholar 

  12. Ohtomo K, Yashiro N, Yoshikawa K, et al (1985) MR imaging of mesenteric varices. J Comput Assist Tomogr 9:1145–1147

    Article  PubMed  CAS  Google Scholar 

  13. Cordoba J, Blei AT (2003) Hepatic encephalopathy. In: Schiff ER (ed) Schiff’s diseases of the liver. Lippincott Williams & Wilkins, Philadelphia, pp 595–623

    Google Scholar 

  14. Krowka MJ (2003) Pulmonary manifestations of liver disease. In: Schiff ER (ed) Schiff’s diseases of the liver. Lippincott Williams & Wilkins, Philadelphia, pp 543–558

    Google Scholar 

  15. Cheung KM, Lee CY, Wong CT, et al (2005) Congenital absence of portal vein presenting as hepatopulmonary syndrome. J Paediatr Child Health 41:72–75

    Article  PubMed  CAS  Google Scholar 

  16. Alvarez AE, Ribeiro AF, Hessel G, et al (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394

    Article  PubMed  Google Scholar 

  17. Krowka MJ, Porayko MK, Plevak DJ, et al (1997) Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: case reports and literature review. Mayo Clin Proc 72:44–53

    Article  PubMed  CAS  Google Scholar 

  18. Egawa H, Kasahara M, Inomata Y, et al (1999) Long-term outcome of living related liver transplantation for patients with intrapulmonary shunting and strategy for complications. Transplantation 67:712–717

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Mithat Haliloglu.

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Turkbey, B., Karcaaltincaba, M., Demir, H. et al. Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 36, 445–448 (2006). https://doi.org/10.1007/s00247-005-0103-0

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  • DOI: https://doi.org/10.1007/s00247-005-0103-0

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