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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.


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A 2-month-old child presenting with severe pulmonary hypertension and cyanosis, Coronal reformatted MIP image (A) showing double SVC (curved arrow) with a small ASD. Coronal MINIP image (B) showing left bronchial isomerism. Axial section through liver (C) shows centrally placed liver with stomach on right side (*) and interrupted IVC (white arrow). Coronal reformatted MIP image (D) showing drainage of superior mesenteric vein through a well-defined shunt (black arrow) into interrupted IVC/azygous vein. No well-defined intrahepatic portal vein is seen. VR image (E) depicting the same
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Figure 4: A 2-month-old child presenting with severe pulmonary hypertension and cyanosis, Coronal reformatted MIP image (A) showing double SVC (curved arrow) with a small ASD. Coronal MINIP image (B) showing left bronchial isomerism. Axial section through liver (C) shows centrally placed liver with stomach on right side (*) and interrupted IVC (white arrow). Coronal reformatted MIP image (D) showing drainage of superior mesenteric vein through a well-defined shunt (black arrow) into interrupted IVC/azygous vein. No well-defined intrahepatic portal vein is seen. VR image (E) depicting the same

Mentions: The original report by Abernethy[1] included findings of right-sided heart and right aortic arch. A number of authors have reported cases of CEPS with associated cardiac anomalies including atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect, aortic stenosis, pulmonary stenosis, coarctation of aorta, and patent ductus arteriosus.[25202930] [Figure 4].


The Abernethy malformation — myriad imaging manifestations of a single entity
A 2-month-old child presenting with severe pulmonary hypertension and cyanosis, Coronal reformatted MIP image (A) showing double SVC (curved arrow) with a small ASD. Coronal MINIP image (B) showing left bronchial isomerism. Axial section through liver (C) shows centrally placed liver with stomach on right side (*) and interrupted IVC (white arrow). Coronal reformatted MIP image (D) showing drainage of superior mesenteric vein through a well-defined shunt (black arrow) into interrupted IVC/azygous vein. No well-defined intrahepatic portal vein is seen. VR image (E) depicting the same
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: A 2-month-old child presenting with severe pulmonary hypertension and cyanosis, Coronal reformatted MIP image (A) showing double SVC (curved arrow) with a small ASD. Coronal MINIP image (B) showing left bronchial isomerism. Axial section through liver (C) shows centrally placed liver with stomach on right side (*) and interrupted IVC (white arrow). Coronal reformatted MIP image (D) showing drainage of superior mesenteric vein through a well-defined shunt (black arrow) into interrupted IVC/azygous vein. No well-defined intrahepatic portal vein is seen. VR image (E) depicting the same
Mentions: The original report by Abernethy[1] included findings of right-sided heart and right aortic arch. A number of authors have reported cases of CEPS with associated cardiac anomalies including atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect, aortic stenosis, pulmonary stenosis, coarctation of aorta, and patent ductus arteriosus.[25202930] [Figure 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