title: The classification based on intrahepatic portal system for congenital portosystemic shunts.
abstract: OBJECTIVE
  Liver transplantation was previously indicated as a curative operation for congenital absence of portal vein. Recent advances in radiological interventional techniques can precisely visualize the architecture of the intrahepatic portal system (IHPS). Therefore, the therapeutic approach for congenital portosystemic shunt (CPS) needs to be reevaluated from a viewpoint of radiological appearances. The aim of this study was to propose the IHPS classification which could explain the pathophysiological characteristics and play a complementary role of a therapeutic approach and management for CPS.
  
  
  METHODS
  Nineteen patients with CPS were retrospectively reviewed. The median age at diagnosis was 6.8 years old. Eighteen of these patients underwent angiography with a shunt occlusion test and were classified based of the severity of the hypoplasia of IHPS.
  
  
  RESULTS
  The eighteen cases who could undergo the shunt occlusion test were classified into mild (n=7), moderate (n=6) and severe types (n=5) according to the IHPS classification. The IHPS classification correlated with the portal venous pressure under shunt occlusion, the histopathological findings, postoperative portal venous flow and liver regeneration. Shunt closure resulted in dramatic improvement in the laboratory data and subclinical encephalopathy. Two patients with the severe type suffered from sepsis associated with portal hypertension after treatment, and from the portal flow steal phenomenon because of the development of unexpected collateral vessels. The patients with the severe type had a high risk of postoperative complications after shunt closure in one step, even if the PVP was relatively low during the shunt occlusion test.
  
  
  CONCLUSIONS
  The IHPS could be visualized by the shunt occlusion test. The IHPS classification reflected the clinicopathological features of CPS, and was useful to determine the therapeutic approach and management for CPS.
authors.affiliation: Transplantation Center, National Center for Child Health & Development, Tokyo, Japan. Electronic address: kanazawa-hr@ncchd.go.jp.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanazawa
authors.affiliation: Department of Radiology, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Nosaka
authors.affiliation: Department of Radiology, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Osamu
authors.initials: O
authors.lastname: Miyazaki
authors.affiliation: Transplantation Center, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.affiliation: Transplantation Center, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.affiliation: Transplantation Center, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Takanobu
authors.initials: T
authors.lastname: Shigeta
authors.affiliation: Department of Clinical Pathology, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.affiliation: Transplantation Center, National Center for Child Health & Development, Tokyo, Japan.
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
mesh_terms: D006503:Hepatic Veins / Q000002:abnormalities*; D006801:Humans; D011168:Portal System / Q000002:abnormalities*; D011169:Portal Vein / Q000002:abnormalities*; D054079:Vascular Malformations / Q000145:classification*
keywords: Abernethy malformation; Congenital absence of the portal vein; Congenital portosystemic shunt; Liver transplantation; Shunt occlusion test