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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 presented with cyanosis. Axial MIP images (A, B) of thorax reveals aneurysmal dilatation of pulmonary veins (white arrow) with numerous vascular channels likely pulmonary vascular malformations. Coronal and axial MIP images (C, D) show dilatation of splenic vein which unites with superior mesenteric vein and extends into retroperitoneum and then superiorly into the posterior mediastinum and drains into left brachiocephalic vein suggestive of porto systemic shunt (*). The VRT image (E) showing portal vein draining into left brachiocephalic vein (*)
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Figure 8: An 8-year-old child presented with cyanosis. Axial MIP images (A, B) of thorax reveals aneurysmal dilatation of pulmonary veins (white arrow) with numerous vascular channels likely pulmonary vascular malformations. Coronal and axial MIP images (C, D) show dilatation of splenic vein which unites with superior mesenteric vein and extends into retroperitoneum and then superiorly into the posterior mediastinum and drains into left brachiocephalic vein suggestive of porto systemic shunt (*). The VRT image (E) showing portal vein draining into left brachiocephalic vein (*)

Mentions: In type 1 shunts, the PV or any of its constituents may drain into any Systemic vein, including the left renal vein,[2247] the right renal vein,[22] and intrathoracic vein [Figure 8].[48] Communications have also been reported between the inferior mesenteric vein or its tributaries and left or right internal iliac vein.[5]


The Abernethy malformation — myriad imaging manifestations of a single entity
An 8-year-old child presented with cyanosis. Axial MIP images (A, B) of thorax reveals aneurysmal dilatation of pulmonary veins (white arrow) with numerous vascular channels likely pulmonary vascular malformations. Coronal and axial MIP images (C, D) show dilatation of splenic vein which unites with superior mesenteric vein and extends into retroperitoneum and then superiorly into the posterior mediastinum and drains into left brachiocephalic vein suggestive of porto systemic shunt (*). The VRT image (E) showing portal vein draining into left brachiocephalic vein (*)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 8: An 8-year-old child presented with cyanosis. Axial MIP images (A, B) of thorax reveals aneurysmal dilatation of pulmonary veins (white arrow) with numerous vascular channels likely pulmonary vascular malformations. Coronal and axial MIP images (C, D) show dilatation of splenic vein which unites with superior mesenteric vein and extends into retroperitoneum and then superiorly into the posterior mediastinum and drains into left brachiocephalic vein suggestive of porto systemic shunt (*). The VRT image (E) showing portal vein draining into left brachiocephalic vein (*)
Mentions: In type 1 shunts, the PV or any of its constituents may drain into any Systemic vein, including the left renal vein,[2247] the right renal vein,[22] and intrathoracic vein [Figure 8].[48] Communications have also been reported between the inferior mesenteric vein or its tributaries and left or right internal iliac vein.[5]

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