Absence of hepatic segment of IVC with azygos continuation
Definitions
IVC is interrupted above renal veins
Hepatic veins drain directly into right atrium
Large azygos vein provides lower extremity venous return
Occasionally, large hemiazygos vein carries venous return
Due to persistent embryonic right supracardinal vein and failed development of suprarenal part of subcardinal vein
Associated with congenital heart disease and situs abnormalities, especially polysplenia (heterotaxy syndrome)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Yildirim A et al: An unusual case of heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein and large venous ectasia. Congenit Heart Dis. 6(3):262-5, 2011
Mamidipally S et al: Azygous continuation of inferior vena cava. J Am Coll Cardiol. 56(21):e41, 2010
Morita S et al: Pelvic venous variations in patients with congenital inferior vena cava anomalies: classification with computed tomography. Acta Radiol. 48(9):974-9, 2007
Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
Demos TC et al: Venous anomalies of the thorax. AJR Am J Roentgenol. 182(5):1139-50, 2004
Plata-Muñoz JJ et al: Polysplenia syndrome in the adult patient. Case report with review of the literature. Ann Hepatol. 3(3):114-7, 2004
Yilmaz E et al: Interruption of the inferior vena cava with azygos/hemiazygos continuation accompanied by distinct renal vein anomalies: MRA and CT assessment. Abdom Imaging. 28(3):392-4, 2003
Bass JE et al: Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 20(3):639-52, 2000
Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
Gayer G et al: Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 24(2):178-84, 1999
Jelinek JS et al: MRI of polysplenia syndrome. Magn Reson Imaging. 7(6):681-6, 1989
Munechika H et al: Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr. 12(2):328-30, 1988
Cohen MI et al: Accessory hemiazygos continuation of left inferior vena cava: CT demonstration. J Comput Assist Tomogr. 8(4):777-9, 1984
Schultz CL et al: Azygos continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr. 8(4):774-6, 1984
Siegfried MS et al: Diagnosis of inferior vena cava anomalies by computerized tomography. Comput Radiol. 7(2):119-23, 1983
Allen HA et al: Case report. Left-sided inferior vena cava with hemiazygos continuation. J Comput Assist Tomogr. 5(6):917-20, 1981
Ginaldi S et al: Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 4(1):112-4, 1980
O'Reilly RJ et al: The lateral chest film as an unreliable indicator of azygos continuation of the inferior vena cava. Circulation. 53(5):891-5, 1976
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Inferior vena cava (IVC)
Synonyms
Interruption of IVC
Absence of hepatic segment of IVC with azygos continuation
Definitions
IVC is interrupted above renal veins
Hepatic veins drain directly into right atrium
Large azygos vein provides lower extremity venous return
Occasionally, large hemiazygos vein carries venous return
Due to persistent embryonic right supracardinal vein and failed development of suprarenal part of subcardinal vein
Associated with congenital heart disease and situs abnormalities, especially polysplenia (heterotaxy syndrome)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Yildirim A et al: An unusual case of heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein and large venous ectasia. Congenit Heart Dis. 6(3):262-5, 2011
Mamidipally S et al: Azygous continuation of inferior vena cava. J Am Coll Cardiol. 56(21):e41, 2010
Morita S et al: Pelvic venous variations in patients with congenital inferior vena cava anomalies: classification with computed tomography. Acta Radiol. 48(9):974-9, 2007
Bartram U et al: Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 88(4):278-90, 2005
Demos TC et al: Venous anomalies of the thorax. AJR Am J Roentgenol. 182(5):1139-50, 2004
Plata-Muñoz JJ et al: Polysplenia syndrome in the adult patient. Case report with review of the literature. Ann Hepatol. 3(3):114-7, 2004
Yilmaz E et al: Interruption of the inferior vena cava with azygos/hemiazygos continuation accompanied by distinct renal vein anomalies: MRA and CT assessment. Abdom Imaging. 28(3):392-4, 2003
Bass JE et al: Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 20(3):639-52, 2000
Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
Gayer G et al: Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 24(2):178-84, 1999
Jelinek JS et al: MRI of polysplenia syndrome. Magn Reson Imaging. 7(6):681-6, 1989
Munechika H et al: Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr. 12(2):328-30, 1988
Cohen MI et al: Accessory hemiazygos continuation of left inferior vena cava: CT demonstration. J Comput Assist Tomogr. 8(4):777-9, 1984
Schultz CL et al: Azygos continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr. 8(4):774-6, 1984
Siegfried MS et al: Diagnosis of inferior vena cava anomalies by computerized tomography. Comput Radiol. 7(2):119-23, 1983
Allen HA et al: Case report. Left-sided inferior vena cava with hemiazygos continuation. J Comput Assist Tomogr. 5(6):917-20, 1981
Ginaldi S et al: Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 4(1):112-4, 1980
O'Reilly RJ et al: The lateral chest film as an unreliable indicator of azygos continuation of the inferior vena cava. Circulation. 53(5):891-5, 1976
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