Heterotaxy syndrome is a disease of embryo development resulting in abnormal distribution of thoracic and abdominal organs across the left-right axis. In this case, A 77-year-old gentleman was admitted with gastric outlet obstruction secondary to cholangiocarcinoma. This is on a background of heterotaxy syndrome, specifically . The patient's anatomical variations included a right-sided stomach, midline liver, and asplenia. Due to variant anatomy and risk of aspiration; endoscopy was abandoned in favor of surgical bypass via a gastrojejunostomy. Although technically challenging, complex upper abdominal surgery in heterotaxy syndrome has been described in the literature. Variations in anatomy observed in heterotaxy syndrome may render patients ineligible for resection of cholangiocarcinoma and increase the risk of complications. Careful preoperative planning with imaging and meticulous intraoperative dissection is required.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10686879PMC
http://dx.doi.org/10.1016/j.radcr.2023.10.050DOI Listing

Publication Analysis

Top Keywords

heterotaxy syndrome
20
gastric outlet
8
outlet obstruction
8
heterotaxy
5
syndrome
5
locally invasive
4
invasive cholangiocarcinoma
4
cholangiocarcinoma causing
4
causing gastric
4
obstruction heterotaxy
4

Similar Publications

Background: Surgical treatment of functional single ventricle combined with atrioventricular valve regurgitation remains a clinical challenge. The outcomes of atrioventricular valve repair in patients with single ventricle are limited.

Methods: A retrospective study was conducted of all 28 patients with functional single ventricle treated with single-ventricle palliation who underwent atrioventricular valve operation at the First Hospital of Tsinghua University between April 2007 and October 2022.

View Article and Find Full Text PDF

Heterotaxy syndrome, a condition in which the internal organs are abnormally arranged in the thorax or abdomen, is generally diagnosed early in life, often during the neonatal period. We present the case of a 42-year-old male who was incidentally diagnosed with polysplenia syndrome and subsequently diagnosed with heterotaxy syndrome. Upon further investigation, he was determined to have a sinus venosus type atrial septal defect.

View Article and Find Full Text PDF

Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A pilot study.

PLoS One

December 2024

Department of Medical Imaging, The Affiliated Suzhou Hospital of Nanjing Medical University, Gusu School of Nanjing Medical University, Suzhou, Jiangsu, China.

Objective: To analytically depict the associated malformations of polysplenia syndrome (PS) in adults via computed tomography (CT).

Materials And Methods: The incidence of malformations associated with PS in twelve adult patients was retrospectively analyzed via CT imaging.

Results: The number of splenic nodules ranged from three to twelve; the splenic nodules were located in the left upper quadrant in nine patients and in the right upper quadrant in three patients.

View Article and Find Full Text PDF

Polysplenia Syndrome and the Development of Heart Failure due to Associated Congenital Heart Defect.

J Belg Soc Radiol

December 2024

Department of Radiology, Sint-Franciscusziekenhuis, Heusden-Zolder, Belgium.

Patients with polysplenia syndrome can develop pulmonary hypertension and heart failure due to underlying congenital heart disease, underscoring the need for early recognition and intervention to prevent further progression of the condition.

View Article and Find Full Text PDF

Background: Asplenia-type heterotaxy syndrome (HS) is rare and refers to visceral malposition and dysmorphism. It is associated with a high infant mortality rate due to cardiac anomalies, and related digestive endoscopic interventions are poorly understood. With the improved long-term prognosis of these individuals after modern cardiac surgery, intra-abdominal anomalies have become increasingly significant.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!