EP07.07: Congenital portosystemic fistulas: a case series

Congenital portosystemic fistulas (CPF) are rare congenital malformations characterised by abnormal vascular communications between the portal system and the caval system. They can be isolated or may be associated with other congenital anomalies, including intrauterine growth retardation (IUGR). 4 patients were referred to our unit after prenatal diagnosis of CPF, identified by ultrasound during the early third trimester. Among these cases, 3 were associated with IUGR, and one presented with bilateral clubfoot where parents refused the amniocentesis. After the diagnosis, fetuses underwent a closed follow-up and fetal cardiac ultrasound. Fetal hemodynamics were normal, except for one case with moderate biventricular hypertrophy. Induction of labour was performed in two cases at 37 weeks due to pre-eclampsia, and in one case due to severe IUGR. The fourth case underwent a repeat Caesarean section at 37+6 weeks due to preterm premature rupture of membranes. After birth, abdominal and cardiac ultrasound was provided for each newborn. All newborns demonstrated good adaptation to extrauterine life, remained asymptomatic, and were clinically and hemodynamically stable. Spontaneous closure of the fistulas occurred without surgical intervention. Early detection of CPF through prenatal screening can improve postnatal outcomes and prognosis. Most CPF cases have a favourable outcome and remain asymptomatic in childhood. Spontaneous closure typically occurs within the first two years of life without sequelae in the majority of cases. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.