WOFAPS 2025 8th World Congress of Pediatric Surgery

Alejandro Madurga, Maria Victoria Lopez-Canelada, Maria Alos, Maria Dolores Ponce, César Oterino, Javier Serradilla, Maria Velayos, Juan Carlos Lopez Gutierrez, Esteban Frauca, Ane Andres, Francisco Fernandez-Oliveros

Hospital Universitario La Paz

INTRODUCTION
Congenital portosystemic shunts (CPSS) represent a heterogeneous group of malformations with diverse manifestations and outcomes, due to their embryological complexity and potential influence of genetic factors.

MATERIALS/METHODS
We conducted a retrospective review of patients with CPSS evaluated at our center since 2010. Patients were divided into those with extrahepatic CPSS (EH-CPSS) and intrahepatic CPSS (IH-CPSS), and the following variables were compared: age, symptoms, type of CPSS, neuropsychiatric, cardiac, endocrine, and pulmonary abnormalities, liver disease, treatment, and outcomes.

RESULTS
We included 89 patients (49 male/40 female), with a mean age at diagnosis of <3 months (range: prenatal to 10 years). Significant differences were found in the presence of liver disease (27%; 57% EH vs. 4% IH, p<0.05), structural heart disease (68%; 87% EH vs. 38% IH, p<0.05), and vascular anomalies (37%; 75% EH vs. 21% IH, p<0.05). However, no differences were observed in the association with pulmonary hypertension (16.5%), fish odor syndrome (12%), or neuropsychiatric abnormalities (38%).

IH-CPSS (88%) showed a favorable outcome, particularly those associated with the ductus venosus. Spontaneous closure occurred in 50%, 24% required closure with an intravascular device, and 26% remained open. Among EH-CPSS (12%), six were closed through interventional radiology, three required liver transplantation, two remained open, and one died without treatment. Only two cases closed spontaneously.

CONCLUSION
CPSS is a heterogeneous condition with unpredictable outcomes. The association of EH-CPSS with heart disease, liver disease, and vascular anomalies opens a new avenue for research in this pathology.