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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
- Type 1: Absence of portal vein with extrahepatic portosystemic shunt
- 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
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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References
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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
- Type 1: Absence of portal vein with extrahepatic portosystemic shunt
- 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
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
