Abernethy Malformation and the Spectrum of Imaging Findings: A Case Report

A Sunnapwar,K Gangadhar,Kn Chintapalli,Sv Nair

doi:10.3126/njr.v3i2.9622

Abstract

Congenital extrahepatic porto systemic shunt (CEPS) is a rare condition in which the porto mesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. It is commonly associated with multiple congenital anomalies. A diagnosis of Abernethy malformation is made by non-invasive cross-sectional imaging techniques such as Doppler ultrasound, CT or MRI, which show the shunt and intrahepatic portal vein branches. We report a case presenting with psychological issues, systemic hypertension, upper extremity deformities, liver lesions and portal hypertension. Usually the patients are diagnosed in childhood, in our case the patient is a middle aged woman. This case report shows the importance of imaging in diagnosis of this condition and presentation of the patient with portal hypertension which is rare.DOI: http://dx.doi.org/10.3126/njr.v3i2.9622 Nepalese Journal of Radiology Vol.3(2)July-Dec, 2013: 97-102

Highlights

Congenital extrahepatic porto systemic shunt (CEPS) is a rare condition in which the porto mesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt
Further evaluation with MR liver revealed T1-hypointense/T2-hyperintense heterogenous enhancing diffusion non restricting masses with similar vascular finding described in computed tomography (CT)
All the imaging findings lead to the diagnosis of Abernethy malformation with skeletal anomalies

Summary

Introduction

Congenital extrahepatic porto systemic shunt (CEPS) is a rare condition in which the porto mesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. Gangadhar et al Abernethy Malformation and the Spectrum of Imaging Findings: A Case Report splenomegaly and perisplenic collaterals, multiple liver masses and direct drainage of portal vein into the inferior vena cava with absence of intrahepatic portal branches. Further evaluation with MR liver revealed T1-hypointense/T2-hyperintense heterogenous enhancing diffusion non restricting masses with similar vascular finding described in CT.

Results

Conclusion