Congenital intra-hepatic porto-systemic shunts diagnosed during intrauterine life: Systematic review

Objective: To describe the ultrasonographic characteristics of congenital porto-systemic venous shunts (CPSS) diagnosed during pregnancy, their outcomes, and their evolution. Methods: Two independent researchers selected 493 review articles and case reports through the analysis of titles, abstracts, and full text. The PubMed and LILACS databases were searched. Through the application of filters according to the PRISMA protocol, only six articles were used in the research. The following information was collected, when available: gestational age at diagnosis, gender, birth weight, type of shunt, associated anomalies/complications and treatment/progression. Results: The data were obtained from 27 cases, with 22 (82%) fetuses diagnosed with intra-hepatic CPSS and 5 (18%) with extra-hepatic CPSS. The median time of intrauterine diagnosis was 33 weeks. In 12 (57.1%) of the 21 pregnancies evaluated, delivery was preterm. The estimated fetal weight ranged from 1150 to 3760 g, with 4 (25%) cases at <3rd, 3 (18.75%) cases at <10th, 8 (50%) cases at <50th, and 1 (6.25%) case at >97th percentile for gestational age. The most frequent obstetric complication was fetal growth restriction, which occurred in nine (60%) cases. As for postnatal treatment, 19 (70.4%) cases were conservatively treated, and 8 (29.6%) cases required surgical intervention. Conclusion: The diagnosis of CPSS still represents a challenge during prenatal care. Its early identification aims to provide guidance to pregnant women and their families, as well as follow-up and anticipation of possible complications, in addition to the evaluation of the mode of delivery and postnatal follow-up, directing the short- and long-term prognosis.