Zhang YY, Ruan J, Fu Y. Therapeutic endoscopic retrograde cholangiopancreatography in a patient with asplenia-type heterotaxy syndrome: A case report. World J Gastrointest Surg 2024; 16(11): 3578-3583 [DOI: 10.4240/wjgs.v16.i11.3578]
Corresponding Author of This Article
Yan Fu, MMed, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, No. 374 Dianmian Road, Wuhua District, Kunming 650101, Yunnan Province, China. ky_fuyan@163.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastrointest Surg. Nov 27, 2024; 16(11): 3578-3583 Published online Nov 27, 2024. doi: 10.4240/wjgs.v16.i11.3578
Therapeutic endoscopic retrograde cholangiopancreatography in a patient with asplenia-type heterotaxy syndrome: A case report
Yu-Yan Zhang, Jiang Ruan, Yan Fu
Yu-Yan Zhang, Jiang Ruan, Yan Fu, Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China
Co-first authors: Yu-Yan Zhang and Jiang Ruan.
Author contributions: Zhang YY discovered and investigated the case, and contributed to manuscript drafting; Fu Y contributed to procedure execution; Ruan J contributed to manuscript drafting and revision; All authors approved the final submission version; Please kindly note that Zhang YY and Ruan J contributed equally as co-first authors.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yan Fu, MMed, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, No. 374 Dianmian Road, Wuhua District, Kunming 650101, Yunnan Province, China. ky_fuyan@163.com
Received: January 30, 2024 Revised: August 27, 2024 Accepted: September 9, 2024 Published online: November 27, 2024 Processing time: 274 Days and 1.4 Hours
Abstract
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.
CASE SUMMARY
Herein, we report successful endoscopic retrograde cholangiopancreatography (ERCP) in a 14-year-old male with asplenia-type HS that involved unique imaging findings and technical difficulties. His anatomic anomalies included complex congenital heart disease, midline liver placement, an absent spleen, a left-sided inferior vena cava, and dextroposition of the stomach and pancreas. He suffered from choledocholithiasis with obstructive jaundice, and the stone was successfully extracted with a basket following endoscopic papillary balloon dilation.
CONCLUSION
Although anatomic anomalies in HS increase the degree of technical difficulty when performing ERCP, they can be safely managed by experienced endoscopists, as illustrated by the present case. Identifying these variations with imaging modalities and being aware of them before initiating an invasive intervention are crucial to preventing potential complications.
Core Tip: Endoscopic retrograde cholangiopancreatography (ERCP) can be challenging in cases of anatomical variations. Owing to its rarity, ERCP in patients with asplenia-type heterotaxy syndrome is poorly understood. Although anatomic anomalies lead to increased technical difficulties and risks when standard endoscopic procedures are performed, a thorough preoperative evaluation and the performance of the procedure by a skilled endoscopist in this case enabled the successful completion of the intended procedure, demonstrating that ERCP can be safely performed in patients with asplenia-type heterotaxy syndrome.
