Heterotaxy Polysplenia Syndrome Causing Intermittent Vomiting Due to Malrotation of the Duodenum in an Adult

Abstract

Heterotaxy syndrome (situs ambiguus) is a rare condition in which the internal organs are abnormally arranged in the chest and abdomen. It occurs from an early embryological developmental disturbance with most cases being sporadic. In this report, we present a case of a 43-year-old female with heterotaxy syndrome who had anomalous right renal artery of high origin and preduodenal portal vein, anomalous orientation of the third portion of the duodenum causing intermittent symptoms and abnormal localization of intestinal and colonic segments. To the best of our knowledge, this the first report of association of these anomalies with heterotaxy syndrome.

Keywords

• heterotaxy
• polysplenia
• situs ambiguus