Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: Anatomy and clinical relevance

Arianna Bertocchini, Pierluigi Falappa, Chiara Grimaldi, Giuseppe Bolla, Lidia Monti, Jean De Ville De Goyet

Research output: Contribution to journalArticlepeer-review


Background Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not. Methods All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system. Results Eighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome. Conclusions Wedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic-radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success.

Original languageEnglish
Pages (from-to)1268-1275
Number of pages8
JournalJournal of Pediatric Surgery
Issue number8
Publication statusPublished - 2014


  • Children
  • Extrahepatic portal hypertension
  • Meso-Rex Bypass
  • Wedged hepatic venous portography

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)


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