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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

Ultrasound images of a 13-year-old child (same patient in Figure 3), (A) shows bulbous dilatation of portal vein (*) and direct shunt with IVC (arrow). Color Doppler image (B) showing the direct communication
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Figure 9: Ultrasound images of a 13-year-old child (same patient in Figure 3), (A) shows bulbous dilatation of portal vein (*) and direct shunt with IVC (arrow). Color Doppler image (B) showing the direct communication

Mentions: Doppler ultrasonography is a safe and noninvasive modality for the diagnosis of the intrahepatic vasculature and may demonstrate the shunt, including the hemodynamics involved such as the magnitude and direction of flow [Figure 9]. It may pick up congenital shunts preoperatively; however, it may not detect associated anomalies and may also be unable to evaluate the retroperitoneum well, particularly in adult patients.[184950] Thus, smaller shunts, particularly type 1a may not be well picked up. Ultrasound may not fully characterize liver lesions seen in these patients. Associated anomalies and findings particularly lung and cardiac anomalies will not be defined on ultrasound.


The Abernethy malformation — myriad imaging manifestations of a single entity
Ultrasound images of a 13-year-old child (same patient in Figure 3), (A) shows bulbous dilatation of portal vein (*) and direct shunt with IVC (arrow). Color Doppler image (B) showing the direct communication
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 9: Ultrasound images of a 13-year-old child (same patient in Figure 3), (A) shows bulbous dilatation of portal vein (*) and direct shunt with IVC (arrow). Color Doppler image (B) showing the direct communication
Mentions: Doppler ultrasonography is a safe and noninvasive modality for the diagnosis of the intrahepatic vasculature and may demonstrate the shunt, including the hemodynamics involved such as the magnitude and direction of flow [Figure 9]. It may pick up congenital shunts preoperatively; however, it may not detect associated anomalies and may also be unable to evaluate the retroperitoneum well, particularly in adult patients.[184950] Thus, smaller shunts, particularly type 1a may not be well picked up. Ultrasound may not fully characterize liver lesions seen in these patients. Associated anomalies and findings particularly lung and cardiac anomalies will not be defined on ultrasound.

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