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The Abernethy malformation — myriad imaging manifestations of a single entity

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ABSTRACT

Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

No MeSH data available.


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An 8-year-old child, presenting with cyanosis, Axial images (A, B) showing multiple well-defined hypodense lesions showing arterial phase enhancement and becoming isodense on delayed images suggestive of adenomas. Reformatted sagittal MIP image (C) showing fistulous communication of main portal vein with IVC (white arrow). No appreciable intrahepatic portal branches are seen
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Figure 5: An 8-year-old child, presenting with cyanosis, Axial images (A, B) showing multiple well-defined hypodense lesions showing arterial phase enhancement and becoming isodense on delayed images suggestive of adenomas. Reformatted sagittal MIP image (C) showing fistulous communication of main portal vein with IVC (white arrow). No appreciable intrahepatic portal branches are seen

Mentions: Nodular hepatic lesions in patients with congenital portosystemic shunts may be single or multiple, and include regenerative nodular hyperplasia,[203740] focal nodular hyperplasia,[203334] Hepatic adenomas/hepatic adenomatosis[1620] [Figures 5 and 6] and hepatoblastoma.[4142]


The Abernethy malformation — myriad imaging manifestations of a single entity
An 8-year-old child, presenting with cyanosis, Axial images (A, B) showing multiple well-defined hypodense lesions showing arterial phase enhancement and becoming isodense on delayed images suggestive of adenomas. Reformatted sagittal MIP image (C) showing fistulous communication of main portal vein with IVC (white arrow). No appreciable intrahepatic portal branches are seen
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5036336&req=5

Figure 5: An 8-year-old child, presenting with cyanosis, Axial images (A, B) showing multiple well-defined hypodense lesions showing arterial phase enhancement and becoming isodense on delayed images suggestive of adenomas. Reformatted sagittal MIP image (C) showing fistulous communication of main portal vein with IVC (white arrow). No appreciable intrahepatic portal branches are seen
Mentions: Nodular hepatic lesions in patients with congenital portosystemic shunts may be single or multiple, and include regenerative nodular hyperplasia,[203740] focal nodular hyperplasia,[203334] Hepatic adenomas/hepatic adenomatosis[1620] [Figures 5 and 6] and hepatoblastoma.[4142]

View Article: PubMed Central - PubMed

ABSTRACT

Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

No MeSH data available.


Related in: MedlinePlus