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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

Normal portovenous anatomy: (A) Intrahepatic shunt (B). Types of CEPS. Type 1a: Splenic vein and superior mesenteric vein drain directly into the inferior vena cava (IVC) (C). Type 1b: Portal vein drains directly into the IVC (D). Type 2: Shunt between portal vein and IVC (E)
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Figure 1: Normal portovenous anatomy: (A) Intrahepatic shunt (B). Types of CEPS. Type 1a: Splenic vein and superior mesenteric vein drain directly into the inferior vena cava (IVC) (C). Type 1b: Portal vein drains directly into the IVC (D). Type 2: Shunt between portal vein and IVC (E)

Mentions: Congenital portosystemic shunts are classified into intrahepatic and extrahepatic. In the intrahepatic shunts, the shunt is at the level of PV branches after its division whereas in the extrahepatic shunts the anastomoses are established between the tributaries of the portal or mesenteric system or main PV and a systemic vein [Figure 1].[4]


The Abernethy malformation — myriad imaging manifestations of a single entity
Normal portovenous anatomy: (A) Intrahepatic shunt (B). Types of CEPS. Type 1a: Splenic vein and superior mesenteric vein drain directly into the inferior vena cava (IVC) (C). Type 1b: Portal vein drains directly into the IVC (D). Type 2: Shunt between portal vein and IVC (E)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Normal portovenous anatomy: (A) Intrahepatic shunt (B). Types of CEPS. Type 1a: Splenic vein and superior mesenteric vein drain directly into the inferior vena cava (IVC) (C). Type 1b: Portal vein drains directly into the IVC (D). Type 2: Shunt between portal vein and IVC (E)
Mentions: Congenital portosystemic shunts are classified into intrahepatic and extrahepatic. In the intrahepatic shunts, the shunt is at the level of PV branches after its division whereas in the extrahepatic shunts the anastomoses are established between the tributaries of the portal or mesenteric system or main PV and a systemic vein [Figure 1].[4]

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