Polysplenia/Heterotaxy Syndrome Associated With Aortic Coarctation and Multiple Venous Anomalies: Multidetector Computed Tomography Findings

Abstract

Polysplenia/heterotaxy syndrome is a rare condition involving multiple gastrointestinal, vascular, and cardiac malformations. We present a previously unreported association of aortic coarctation, double superior vena cava, a left-hand side inferior vena cava with hemiazygos vein continuation and a right retroaortic renal vein with polysplenia/heterotaxy syndrome. Multidetector computed tomography with multiplanar reconstruction images were useful in the detection of these abnormalities.

Keywords

polysplenia syndrome heterotaxy syndrome aortic coarctation venous abnormality

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References

Peoples WM , Moller JH , Edward JE Polisplenia: a review of 146 cases. Pediatr Cardiol . 1983;4(2):129-137.

Plata-Muñoz JJ , Hernández-Ramírez D. , Anthón FJ , Podgaetz E. , Avila-Flores F. , Chan C. Polysplenia syndrome in the adult patient. Case report with review of the literature. Ann Hepatol. 2004;3(3):114-117.

Seo HI , Jeon TY , Sim MS , Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults . World J Gastroenterol. 2008;14(41):6418-6420.

Ahmetoğlu A. , Koşucu P. , Sarı A. , Gümele HR Polispleni sendromunda radyolojik bulgular . Tanısal ve Girişimsel Radyoloji Dergisi. 2002;8(4):510-512.

Kondrachuk O. , Yalynska T. , Yemets I. Heterotaxy syndrome (mirror-image dextrocardia, coarctation of the aorta, polysplenia): a previously unreported association . Pediatr Cardiol. 2009;30(6): 864-865.

Kapa S. , Gleeson FC , Vege SS Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome. J Pancreas. 2007;8(4): 433-437.

Winer-Muram HT , Tonkin Ild. The spectrum of heterotaxic syndromes . Radiol Clin North Am. 1989;27(6):1147-1170.

Van de Perre S. , Vanhoenacker FM , Petré C. , Van Doorn J. , De Schepper AM Heterotaxy syndrome. JBR-BTR . 2004;87(3):158-159.

Applegate KE , Goske MJ , Pierce G. , Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographcs. 1999;19(4): 837-852.

10.

Ooshima I. , Maruyama T. , Ootsuki K. , Ozaki M. Predodenal portal ven in the adult. J Hepatobiliary Pancreat Surg. 1998;5(4):455-458.

11.

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(6):921-923.