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

Published on: August 1, 2018

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Idiopathic noncirrhotic portal hypertension

M Isabel Fiel1, Thomas D Schiano2

  • 1Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, Box 1194, The Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029, United States.

Seminars in Diagnostic Pathology
|August 14, 2019
PubMed

Insights

Idiopathic noncirrhotic portal hypertension presents diverse histological findings, challenging pathologists. This review clarifies definitions, clinical aspects, and diagnostic pearls for this condition.

Area of Science:

  • Hepatology
  • Pathology
  • Gastroenterology

Background:

  • Portal hypertension commonly stems from cirrhosis, but noncirrhotic causes exist.
  • Liver biopsy is crucial for diagnosing many noncirrhotic portal hypertension etiologies.
  • Idiopathic noncirrhotic portal hypertension (INPH) is increasingly recognized, posing diagnostic challenges due to varied histology and definitions.

Purpose of the Study:

  • To review the clinical features of idiopathic noncirrhotic portal hypertension.
  • To outline the diverse histological findings associated with INPH.
  • To provide guidance for pathologists on diagnosing INPH, including common pitfalls.

Main Methods:

  • Literature review of clinical and histopathological studies on noncirrhotic portal hypertension.
  • Analysis of diagnostic criteria and histological features of INPH.
  • Synthesis of practical advice for pathologists.

Main Results:

  • INPH has varied histopathological presentations, often subtle and overlapping.
  • Accurate diagnosis requires careful integration of clinical and histological data.
  • Specific histological clues and potential diagnostic traps are identified.

Conclusions:

  • Idiopathic noncirrhotic portal hypertension diagnosis is complex, requiring pathologist expertise.
  • Understanding diverse histological patterns is key to differentiating INPH from other conditions.
  • This review offers a structured approach to aid pathologists in diagnosing INPH.

Keywords:
Hepatoportal sclerosisIdiopathic portal hypertensionNodular regenerative hyperplasiaNodular transformationNoncirrhotic portal fibrosisObliterative portal venopathy

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