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Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
09:37

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats

Published on: August 1, 2018

Coexistent idiopathic retroperitoneal and mediastinal fibrosis presenting with portal hypertension

E F van Bommel1, N D Bouvy, E Liem

  • 1Department of Internal Medicine, University Hospital Dijkzigt, Rotterdam, Netherlands.

The Netherlands Journal of Medicine
|May 1, 1994
PubMed

Insights

A rare case of idiopathic fibrosis caused portal hypertension by affecting the portal venous system. This finding highlights the diverse ways this systemic fibrotic disorder can manifest.

Area of Science:

  • Gastroenterology and Hepatology
  • Pathology

Background:

  • Idiopathic retroperitoneal and mediastinal fibrosis are rare conditions.
  • These fibrotic processes can affect various organs and systems.
  • Systemic idiopathic fibrosis is a recognized but heterogeneously presenting disorder.

Observation:

  • A unique case of coexistent idiopathic retroperitoneal and mediastinal fibrosis is presented.
  • The patient exhibited symptoms of portal hypertension.
  • The cause of portal hypertension was identified as fibrotic entrapment of the portal venous system.

Findings:

  • The fibrotic process extended to encase the portal venous system, leading to obstruction.
  • This case demonstrates a novel presentation of idiopathic fibrosis.
  • The anatomical involvement highlights the systemic nature of the idiopathic fibrosis.

Implications:

  • This case expands the known clinical spectrum of idiopathic fibrosis.
  • It underscores the importance of considering systemic idiopathic fibrosis in patients with unexplained portal hypertension.
  • Further research into the pathogenesis and management of idiopathic fibrosis is warranted.

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