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Cholangiopathy in children with extrahepatic portal venous obstruction

Moinak Sen Sarma1, Surender Kumar Yachha1, Praveer Rai2

  • 1Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India.

Journal of Hepato-Biliary-Pancreatic Sciences
|September 28, 2018
PubMed

Insights

Most children with extrahepatic portal venous obstruction (EHPVO) have asymptomatic portal cavernoma cholangiopathy (PCC). Routine evaluation with magnetic resonance cholangiography-portovenography (MRC-MRPV) is recommended for early detection.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Diagnostic Imaging

Background:

  • Portal cavernoma cholangiopathy (PCC) is a complex biliary complication of extrahepatic portal venous obstruction (EHPVO).
  • Comprehensive studies on PCC, particularly in pediatric populations, are limited.
  • This study focuses on evaluating PCC in children using advanced imaging techniques.

Purpose of the Study:

  • To evaluate portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal venous obstruction (EHPVO).
  • To assess the utility of magnetic resonance cholangiography-portovenography (MRC-MRPV) and endoscopic ultrasonography (EUS) in diagnosing and characterizing PCC in this cohort.

Main Methods:

  • Prospective cross-sectional study involving children diagnosed with EHPVO.
  • Utilized magnetic resonance cholangiography-portovenography (MRC-MRPV) and radial array endoscopic ultrasonography (EUS).
  • Classified PCC as asymptomatic, symptomatic, or absent, and graded biliary changes using the modified Llop scale.

Main Results:

  • 92% of children with EHPVO exhibited PCC, predominantly asymptomatic (85%).
  • Significant correlation observed between age at study, disease duration, and PCC severity.
  • Superior mesenteric vein non-patency was a strong predictor of biliary changes on MRC and EUS.

Conclusions:

  • The majority of pediatric EHPVO cases present with asymptomatic PCC, necessitating routine evaluation via MRC-MRPV.
  • Combined MRC-MRPV and EUS effectively characterize PCC and associated biliary changes.
  • Superior mesenteric vein involvement significantly exacerbates cholangiopathy in EHPVO patients.
Abstract

Keywords:
BiliaryCholangiographyEndosonographyPortovenographyShunt

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