Therapeutic endoscopic retrograde cholangiopancreatography in a patient with asplenia-type heterotaxy syndrome: A case report.

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.