what do you notice on CT?
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.
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.
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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.