• Type II Abernethy Malformation with Hepatopulmonary Syndrome: Single-Stage Endovascular Occlusion of an SMV–IVC Communication — A Case Report
  • Mapping before closure
  • What this case illustrates
  • Read and share

§Type II Abernethy Malformation with Hepatopulmonary Syndrome: Single-Stage Endovascular Occlusion of an SMV–IVC Communication — A Case Report

New SJORANM case report | Published 8 November 2026 | Volume 36, Issue 2, pages 1-10

Authors: Rajakumar Ramar, Babu Peter Sathyanathan, Shivashankar Kumaresan, Kirubakaran Balu, Benin Reejan, Sibi Elango.

Institution: Barnard Institute of Radiology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, India.

§Mapping before closure

A 12-year-old girl with progressive exertional dyspnoea, cyanosis and hepatopulmonary syndrome was found to have a Type II Abernethy malformation: a side-to-side communication between the superior mesenteric vein (SMV) and infrarenal inferior vena cava (IVC), with patent intrahepatic portal branches.

Cross-sectional vascular imaging defined the anatomy. During temporary balloon occlusion, portal pressure rose from 7 to 17 mmHg, and angiography demonstrated intrahepatic portal filling. The team proceeded with single-stage closure using a 12-mm Amplatzer Vascular Plug II and adjunctive gelfoam embolisation of collateral channels.

At 3 months, room-air oxygen saturation increased from 74% to 94%, central cyanosis had resolved, serum ammonia decreased from 103 to 46.8 micromol/L, and arterial blood gases had normalised.

§What this case illustrates

The report highlights the role of vascular mapping and temporary balloon occlusion assessment before endovascular closure of a congenital portosystemic shunt. This is a single-patient case report, not evidence of general treatment efficacy or a universal closure threshold.

Important limitations: Follow-up was limited to 3 months. The authors report an unresolved discrepancy between baseline pulse oximetry and arterial oxygen measurements. Systemic venous pressure was not recorded; the reported portal pressure rise is not a portosystemic gradient. Longer-term outcomes remain unknown.

§Read and share

Read the open-access article: https://doi.org/10.59667/sjoranm.v36i2.14

Journal article | Source PDF

This announcement is an editorial summary, not a reproduction of the full article or medical advice. The original article remains authoritative.

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