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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

Preoperative axial CT (A) shows well-defined shunt between IVC and portal vein (red arrow). Postoperative axial CT (B) shows a surgical staple (black arrow) at site of portosystemic shunt with numerous collaterals in the periphery of liver (white arrow). Pre and post-op axial sections through lung bases (C, D) showing reduction in vascular congestion
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Figure 11: Preoperative axial CT (A) shows well-defined shunt between IVC and portal vein (red arrow). Postoperative axial CT (B) shows a surgical staple (black arrow) at site of portosystemic shunt with numerous collaterals in the periphery of liver (white arrow). Pre and post-op axial sections through lung bases (C, D) showing reduction in vascular congestion

Mentions: There are also other reports that suggest that hypoplastic PV branches are able to expand after the closure of shunt [Figure 11]. Liver transplantation is considered when medical and surgical methods fail especially in patients with complications.


The Abernethy malformation — myriad imaging manifestations of a single entity
Preoperative axial CT (A) shows well-defined shunt between IVC and portal vein (red arrow). Postoperative axial CT (B) shows a surgical staple (black arrow) at site of portosystemic shunt with numerous collaterals in the periphery of liver (white arrow). Pre and post-op axial sections through lung bases (C, D) showing reduction in vascular congestion
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 11: Preoperative axial CT (A) shows well-defined shunt between IVC and portal vein (red arrow). Postoperative axial CT (B) shows a surgical staple (black arrow) at site of portosystemic shunt with numerous collaterals in the periphery of liver (white arrow). Pre and post-op axial sections through lung bases (C, D) showing reduction in vascular congestion
Mentions: There are also other reports that suggest that hypoplastic PV branches are able to expand after the closure of shunt [Figure 11]. Liver transplantation is considered when medical and surgical methods fail especially in patients with complications.

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