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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

A 13-year-old child presenting with altered sensorium, hematemesis, and seizures, Coronal reconstructions showing increased pulmonary vascularity seen as increased interstitial markings (A). Coronal MIP images (B) showing enlarged portal vein draining directly into the IVC through a side-to-side shunt (*). Sagittal MIP image (C) showing the portovenous communication. Axial sections (D) through liver do not reveal any well-defined portal branches. VR image (E) showing the same along with focal prominence of the portal vein
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Figure 3: A 13-year-old child presenting with altered sensorium, hematemesis, and seizures, Coronal reconstructions showing increased pulmonary vascularity seen as increased interstitial markings (A). Coronal MIP images (B) showing enlarged portal vein draining directly into the IVC through a side-to-side shunt (*). Sagittal MIP image (C) showing the portovenous communication. Axial sections (D) through liver do not reveal any well-defined portal branches. VR image (E) showing the same along with focal prominence of the portal vein

Mentions: This syndrome was first described in a patient with Abernethy malformation by Alvarez et al.;[18] it has subsequently been reported in a number of children with congenital portosystemic shunts.[21920] Patients frequently present with cyanosis and digital clubbing and are generally investigated for cardiac and pulmonary shunts. Pediatric patients presenting with structurally normal echocardiograms and unexplained cyanosis should be investigated to rule out the possibility of a CEPS with HPS, even if no previous history is present [Figure 3].


The Abernethy malformation — myriad imaging manifestations of a single entity
A 13-year-old child presenting with altered sensorium, hematemesis, and seizures, Coronal reconstructions showing increased pulmonary vascularity seen as increased interstitial markings (A). Coronal MIP images (B) showing enlarged portal vein draining directly into the IVC through a side-to-side shunt (*). Sagittal MIP image (C) showing the portovenous communication. Axial sections (D) through liver do not reveal any well-defined portal branches. VR image (E) showing the same along with focal prominence of the portal vein
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: A 13-year-old child presenting with altered sensorium, hematemesis, and seizures, Coronal reconstructions showing increased pulmonary vascularity seen as increased interstitial markings (A). Coronal MIP images (B) showing enlarged portal vein draining directly into the IVC through a side-to-side shunt (*). Sagittal MIP image (C) showing the portovenous communication. Axial sections (D) through liver do not reveal any well-defined portal branches. VR image (E) showing the same along with focal prominence of the portal vein
Mentions: This syndrome was first described in a patient with Abernethy malformation by Alvarez et al.;[18] it has subsequently been reported in a number of children with congenital portosystemic shunts.[21920] Patients frequently present with cyanosis and digital clubbing and are generally investigated for cardiac and pulmonary shunts. Pediatric patients presenting with structurally normal echocardiograms and unexplained cyanosis should be investigated to rule out the possibility of a CEPS with HPS, even if no previous history is present [Figure 3].

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