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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Congenital absence of portal vein, congenital extrahepatic portosystemic shunt
Definitions
Congenital malformation characterized by diversion of portal venous blood flow around liver, more commonly into inferior vena cava (IVC) than renal, iliac, or azygous veins
Type 1: Absence of portal vein with extrahepatic portosystemic shunt
Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
Type 2: Intact but hypoplastic portal vein with extrahepatic portosystemic shunt
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Hao Y et al: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett. 9(2):695-700, 2015
Nacif LS et al: Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging. 15:37, 2015
Alonso-Gamarra E et al: Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 31(3):707-22, 2011
Morgan G et al: Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29(9):1239-41, 1994
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Congenital absence of portal vein, congenital extrahepatic portosystemic shunt
Definitions
Congenital malformation characterized by diversion of portal venous blood flow around liver, more commonly into inferior vena cava (IVC) than renal, iliac, or azygous veins
Type 1: Absence of portal vein with extrahepatic portosystemic shunt
Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
Type 2: Intact but hypoplastic portal vein with extrahepatic portosystemic shunt
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Hao Y et al: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett. 9(2):695-700, 2015
Nacif LS et al: Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging. 15:37, 2015
Alonso-Gamarra E et al: Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics. 31(3):707-22, 2011
Morgan G et al: Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29(9):1239-41, 1994
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