Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report

Abstract

BackgroundThe “heterotaxy syndrome” also called “situs ambiguus” is a rare condition in which the internal organs of the chest and abdomen are abnormally arranged. This syndrome is usually associated with a spectrum of health-related conditions involving birth defects in organs like the heart, spleen, liver, lungs and others.Case presentationWe present a case of a 29-year-old male who came with an episode of massive upper gastrointestinal bleeding. On arrival he was vitally unstable and after resuscitation underwent a contrast-enhanced CT scan examination. The CT scan identified the features of heterotaxy syndrome with left isomerism and an abnormal blush from the branch of splenic artery supplying the greater curvature of the stomach. Embolization of the splenic artery branches was performed with the help of gel foam. No abnormal blush was identified post-embolization. Later on, in his hospital stay the patient went into pulse less electrical activity and died.ConclusionUpper gastrointestinal bleed in the patients with heterotaxy syndrome is a rare presentation of a rare condition. Our case highlights the importance of diagnostic and interventional radiology in diagnosing such patients and identifying the site of bleed especially where endoscopic visualization is not possible and managing the bleeding efficiently.