Published online Nov 27, 2024. doi: 10.4240/wjgs.v16.i11.3578
Revised: August 27, 2024
Accepted: September 9, 2024
Published online: November 27, 2024
Processing time: 274 Days and 1.4 Hours
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.
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.
Although anatomic anomalies in HS increase the degree of technical difficulty when performing ERCP, they can be safely managed by experienced endosco
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.