J Korean Soc Radiol.  2019 Nov;80(6):1271-1275. 10.3348/jksr.2019.80.6.1271.

Absence of the Intrahepatic Inferior Vena Cava with Polysplenia Syndrome on Multidetector Computed Tomography: A Case Report

• Hwang S
• Kim JA

• KMID: 2464926
• DOI: http://doi.org/10.3348/jksr.2019.80.6.1271

Abstract

Various anomalies of the inferior vena cava (IVC) can arise from a failure in the normal embryogenic processes. Agenesis of the intrahepatic segment of the IVC with azygos continuation, which is caused by failure of formation of the right subcardinal-hepatic anastomosis, is a rare IVC anomaly. In this paper, we report a case of interrupted IVC with azygos vein continuation, combined with polysplenia, intestinal malrotation, and truncated pancreas, which was incidentally found on abdominal CT and thoracic aorta CT angiography.

MeSH Terms

Angiography
Aorta, Thoracic
Azygos Vein
Heterotaxy Syndrome*
Multidetector Computed Tomography*
Pancreas
Tomography, X-Ray Computed
Vena Cava, Inferior*

Figure

• 

  Fig. 1 A 24-year-old man with incidentally-found polysplenia, intestinal malrotation, and truncated pancreas, shown on abdominal CT and thoracic aorta CT angiography. A. The axial and coronal images of abdominal CT in the delayed phase show that several spleens (arrowheads) are located on the left side of the upper abdomen along the greater curvature of the stomach; the pancreatic head is semiannular (arrow), and the tail shows a defect. B. The coronal image of abdominal CT in the delayed phase demonstrates that the small bowel loop is predominantly located on the right side (arrow), while the large bowel loop is predominantly on the left (arrowhead). C. The axial image of abdominal CT in the late arterial phase demonstrates that the superior mesenteric vein (arrow) is anterior to the superior mesenteric artery (arrowhead), contrary to their conventional positions. D. An axial image of thoracic aorta CT angiography in the delayed phase showing that both renal veins (arrows) drain into the enlarged azygos vein instead of the inferior vena cava. E. The axial and coronal image of thoracic aorta CT angiography in the delayed phase demonstrates an enlarged azygos vein (arrows) draining into the superior vena cava (arrowhead) at the level of the azygos vein arch. F. A coronal image of thoracic aorta CT angiography in the delayed phase reveals that the hepatic veins (arrows) drain directly into the right atrium.

Reference

1. Simson IW. Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology. 1982; 82:171–178.

2. Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000; 20:639–652.

3. Demos TC, Posniak HV, Pierce KL, Olson MC, Muscato M. Venous anomalies of the thorax. AJR Am J Roentgenol. 2004; 182:1139–1150.

4. Tsutsumi R, Nagata Y, Enjoji A, Ohno Y, Kamito H, Kanematsu T. Situs ambiguous with gastric cancer: report of a case. Surg Today. 2007; 37:676–679.

5. Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 1999; 24:178–184.

6. Shiraishi I, Ichikawa H. Human heterotaxy syndrome. -From molecular genetics to clinical features, management, and prognosis-. Circ J. 2012; 76:2066–2207.

7. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 1999; 19:837–852. discussion 853-854.

8. Rameshbabu CS, Gupta KK, Qasim M, Gupta OP. Heterotaxy polysplenia syndrome in an adult with unique vascular anomalies: case report with review of literature. J Radiol Case Rep. 2015; 9:22–37.

9. Soler R, Rodríguez E, Comesaña ML, Pombo F, Marini M. Agenesis of the dorsal pancreas with polysplenia syndrome: CT features. J Comput Assist Tomogr. 1992; 16:921–923.

10. Kobayashi H, Kawamoto S, Tamaki T, Konishi J, Togashi K. Polysplenia associated with semiannular pancreas. Eur Radiol. 2001; 11:1639–1641.