Living Donor Liver Transplantation in Pediatric Situs Inversus: A Case Series and Systematic Review.

Liver transplantation (LT) in patients with situs inversus (S-I) is rare and technically challenging. The literature on such cases, particularly in pediatric populations, remains limited. Here, we report the surgical considerations and outcomes of two pediatric recipients with situs inversus abdominalis who underwent liver transplantation for biliary atresia (BA). We conducted a retrospective descriptive analysis of two pediatric patients with S-I who underwent LT for BA. Pretransplant imaging, intraoperative details, and posttransplant outcomes were evaluated. Additionally, we performed a systematic review of the literature on LT in patients with S-I. We describe the successful living donor liver transplantation (LDLT) of two pediatric patients with S-I abdominalis and BA, each presenting with complex anatomical variations, including polysplenia, intestinal malrotation, and vascular anomalies such as absence of the inferior vena cava (IVC). Both patients underwent technically demanding procedures requiring customized vascular and biliary reconstructions, along with definitive correction of intestinal malrotation to restore anatomical intestinal orientation. Both patients had an uncomplicated immediate postoperative course. During follow-up, one patient developed a diaphragmatic hernia that required surgical repair. Our systematic review confirms that, although rare, pediatric LT in patients with S-I is increasingly reported with favorable outcomes. These findings support the feasibility and safety of LT in children with S-I when guided by meticulous preoperative planning and intraoperative adaptability. Liver transplantation in pediatric patients with S-I is feasible and can be performed safely with careful preoperative planning and tailored surgical techniques. Our experience, together with the available literature, supports the inclusion of these patients as suitable candidates for transplantation despite their complex anatomy.