Heterotaxy syndrome with polysplenia: case report

Abstract

Heterotaxy syndrome (HS) or situs ambiguus is a rare condition, more common in women, in which internal organs are abnormally arranged within the thoracic and abdominal cavities. The condition may manifest as asplenia or polysplenia, characterized by the presence of multiple small and dysfunctional spleens. The severity of HS varies depending on the specific abnormalities present. With the increasing use of imaging techniques, incidental findings of polysplenia are likely to become more frequent. The objective of this report is to illustrate typical imaging findings of HS, aiming to familiarize radiologists and physicians with the spectrum of potentially associated anomalies, thereby improving the quality of care for affected patients. This report describes the case of a 53-year-old woman incidentally diagnosed with HS with polysplenia based on chest and abdominal computed tomography (CT).