A 28 y.o. woman comes in with LUQ pain

what do you notice on CT?

polysplenia.JPG

Our patient had heterotaxy syndrome with polysplenia. The word is derived from hetero meaning “different” and taxy meaning “arrangement”.Heterotaxy  syndrome is an embryologic abnormality where organs and vessels  do not follow the standard developmental pattern. It occurs in 1.4 per 10,000 infants. This is also known as situs ambiguous and is categorized based on the number of spleens.  Asplenic patients have a higher incidence of congenital heart disease which leads to earlier detection of the syndrome.

 This is an US of a 35 wk fetus showing the azygous to the R of the aorta in heterotaxy polysplenia syndrome; this baby had malrotation as well

This is an US of a 35 wk fetus showing the azygous to the R of the aorta in heterotaxy polysplenia syndrome; this baby had malrotation as well

Even though there are many spleens in some patients, the spleens may not be functional and these patients are at risk for sepsis with encapsulated organisms.

heterotaxy with liver on the L.JPG

Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited. Imaging of the heterotaxy syndrome. Radiographics. 1999, 19:837-852

Peoples WM, Moller JH, Edwars JE. Polysplenia: A review of 146 cases. Pediatr Cardiol. 1983;4:129-137.

Lin AE, Ticho BS, Houde K, et al. Heterotaxy. Associated conditions and hospital-based prevalence in newborns. Genet Med 2000;2:157-172.

Cupers S, Va  Linthout C, Desprechins B, et al. Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract 2018. Jan8;8(1), 1004.