Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review

J Pediatr Surg. 2012 Feb;47(2):e27-31. doi: 10.1016/j.jpedsurg.2011.10.079.

Abstract

Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Balloon Occlusion
  • Child
  • Diagnosis, Differential
  • Dyspnea / etiology
  • Focal Nodular Hyperplasia / diagnosis
  • Hepatic Veins / abnormalities*
  • Hepatic Veins / diagnostic imaging
  • Hepatic Veins / surgery
  • Hepatopulmonary Syndrome / etiology
  • Humans
  • Hypoxia / etiology
  • Incidental Findings
  • Liver / blood supply
  • Liver / diagnostic imaging
  • Liver / pathology
  • Liver Circulation
  • Magnetic Resonance Imaging
  • Male
  • Portal Vein / abnormalities*
  • Portal Vein / diagnostic imaging
  • Portal Vein / surgery
  • Pulmonary Fibrosis / diagnosis
  • Radiography
  • Splanchnic Circulation
  • Ultrasonography, Doppler, Color
  • Vascular Fistula / congenital*
  • Vascular Fistula / diagnostic imaging
  • Vascular Fistula / surgery