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

Abstract Background The “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 he...

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Main Authors: Mahrukh Ali, Noman Khan, Jehanzeb Shahid, Samana Zainab Shah, Aimun Raees
Format: Article
Language:English
Published: SpringerOpen 2022-06-01
Series:The Egyptian Journal of Radiology and Nuclear Medicine
Subjects:
Online Access:https://doi.org/10.1186/s43055-022-00813-0
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author Mahrukh Ali
Noman Khan
Jehanzeb Shahid
Samana Zainab Shah
Aimun Raees
author_facet Mahrukh Ali
Noman Khan
Jehanzeb Shahid
Samana Zainab Shah
Aimun Raees
author_sort Mahrukh Ali
collection DOAJ
description Abstract Background The “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 presentation We 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. Conclusion Upper 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.
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spelling doaj.art-7b2ac547c1014bb99493313645cb3fe72022-12-22T00:23:46ZengSpringerOpenThe Egyptian Journal of Radiology and Nuclear Medicine2090-47622022-06-015311510.1186/s43055-022-00813-0Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case reportMahrukh Ali0Noman Khan1Jehanzeb Shahid2Samana Zainab Shah3Aimun Raees4Section of Gastroenterology, Department of Medicine, Aga Khan University HospitalSection of Gastroenterology, Department of Medicine, Aga Khan University HospitalDepartment of Radiology, Aga Khan University HospitalSection of Gastroenterology, Department of Medicine, Aga Khan University HospitalSection of Gastroenterology, Department of Medicine, Aga Khan University HospitalAbstract Background The “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 presentation We 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. Conclusion Upper 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.https://doi.org/10.1186/s43055-022-00813-0Heterotaxy syndromeUpper gastrointestinal bleedGreater curvatureStomachCase report
spellingShingle Mahrukh Ali
Noman Khan
Jehanzeb Shahid
Samana Zainab Shah
Aimun Raees
Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
The Egyptian Journal of Radiology and Nuclear Medicine
Heterotaxy syndrome
Upper gastrointestinal bleed
Greater curvature
Stomach
Case report
title Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
title_full Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
title_fullStr Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
title_full_unstemmed Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
title_short Heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach: a case report
title_sort heterotaxy polysplenia syndrome presenting with bleeding from the greater curvature of stomach a case report
topic Heterotaxy syndrome
Upper gastrointestinal bleed
Greater curvature
Stomach
Case report
url https://doi.org/10.1186/s43055-022-00813-0
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