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The Abernethy malformation — myriad imaging manifestations of a single entity

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

An 8-year-old child presenting with cyanosis, CT angiographic MIP images (A) showing a well-defined large tortuous intrahepatic shunt (*) between the main portal vein and the middle hepatic vein. The middle hepatic vein is enlarged (B). Reconstructed coronal MIP (C) and VR images (D) showing another shunt between the left portal vein and left hepatic vein (arrow) and the large intrahepatic shunt
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Figure 2: An 8-year-old child presenting with cyanosis, CT angiographic MIP images (A) showing a well-defined large tortuous intrahepatic shunt (*) between the main portal vein and the middle hepatic vein. The middle hepatic vein is enlarged (B). Reconstructed coronal MIP (C) and VR images (D) showing another shunt between the left portal vein and left hepatic vein (arrow) and the large intrahepatic shunt

Mentions: Type 1—Single tube-like vessel connecting the right branch of PV to IVC [Figure 2]


The Abernethy malformation — myriad imaging manifestations of a single entity
An 8-year-old child presenting with cyanosis, CT angiographic MIP images (A) showing a well-defined large tortuous intrahepatic shunt (*) between the main portal vein and the middle hepatic vein. The middle hepatic vein is enlarged (B). Reconstructed coronal MIP (C) and VR images (D) showing another shunt between the left portal vein and left hepatic vein (arrow) and the large intrahepatic shunt
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: An 8-year-old child presenting with cyanosis, CT angiographic MIP images (A) showing a well-defined large tortuous intrahepatic shunt (*) between the main portal vein and the middle hepatic vein. The middle hepatic vein is enlarged (B). Reconstructed coronal MIP (C) and VR images (D) showing another shunt between the left portal vein and left hepatic vein (arrow) and the large intrahepatic shunt
Mentions: Type 1—Single tube-like vessel connecting the right branch of PV to IVC [Figure 2]

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