J Pediatr Surg. 2026 Feb;61(2):162759. doi: 10.1016/j.jpedsurg.2025.162759. Epub 2025 Oct 25.
ABSTRACT
BACKGROUND: Congenital portosystemic shunts (CPSS) are rare malformations of visceral vessels that lead to poor or absent intrahepatic portal flow. Therapeutic options have shifted away from liver transplantation towards either interventional or surgical closure with excellent results. The challenge to reliably predict portal venous pressure response to shunt occlusion, paired with significant interindividual variability of shunt anatomy has hindered the structured transition from open to minimally invasive surgical shunt closure. Here, we describe a modified classification of CPSS in a large cohort of children with CPSS that allowed us to systematically transition from laparotomy to minimally invasive shunt closure.
METHODS: Retrospective single center review of 51 children with CPSS who were evaluated at our center between 2014 and 2025.
RESULTS: By July 2025, a total of 51 children were evaluated at our center. Forty-four children had an extrahepatic portosystemic shunt (EPSS) (86.27 %), divided into central (A1) (n = 34, 66.67 %) and peripheral (A2) (n = 10, 19.6 %). Seven children had an intrahepatic portosystemic shunt (IPSS) (13.73 %), divided into persistent ductus venosus (B1) (n = 2, 3.92 %) and singular shunts (B2) (n = 5, 9,8 %). Of all 51 patients, one child underwent liver transplantation (1,96 %), five were referred for external follow-up without intervention (9.8 %) and four are currently being evaluated (7.84 %). Interventional treatment was performed in four cases (7.84 %). Traditional surgical shunt closure via laparotomy was carried out in 28 patients (54.9 %) with favorable outcomes. Since 2024, 9 additional cases of different variants of EPSS were managed laparoscopically (17.65 %) with excellent outcomes.
CONCLUSIONS: With increasing knowledge and detailed description of CPSS, if warranted, most if not all types of CPSS are amenable to minimally invasive shunt closure.
PMID:41591935 | DOI:10.1016/j.jpedsurg.2025.162759