Prenatal sonographic characteristics and postnatal outcomes of congenital portosystemic shunt diagnosed during the fetal period: a systematic review.

BACKGROUND

Congenital portosystemic shunt (CPSS) is a rare vascular malformation which results in anomalous communication between the portal venous system and the systemic vein. The objective of this review is to describe the prenatal ultrasonic characteristics and postnatal outcomes of CPSS diagnosed prenatally, along with providing some suggestions for perinatal monitoring.

MATERIALS AND METHODS

A systematic literature search was conducted in PubMed and Ovid electronic databases in a period from January 2012 to May 2024, using the terms: "congenital portosystemic shunt", "ductus venosus", "Abernethy malformation" and "umbilical-portal-systemic venous shunt". All original studies reporting CPSS patients diagnosed prenatally were included. Studies published in languages other than English or studies that did not report the clinical information of patients were excluded. Two reviewers independently screened articles for inclusion and extracted data.

RESULTS

A total of 39 studies which included 525 cases were enrolled in the systematic review. Among the included patients, 49 (9%) had umbilical-systemic shunt (USS), 264 (50%) had ductus venosus-systemic shunt (DVSS), 159 (30%) had intrahepatic portal-systemic shunt (IHPSS), 32 (6%) had extrahepatic portal-systemic shunt (EHPSS) and 9 (2%) had mixed shunts which meant that they had more than one type of shunts. There were also 12(3%) patients were excluded because the specific type was not described in the study. CPSS patients could have severe comorbidities such as chromosomal aberrations and cardiovascular malformations. Compared with other groups, fetuses with IHPSS had the lowest incidence of severe comorbidities. Most patients received conservative treatment while interventional and surgical treatments were used for some patients.

CONCLUSION

We suggest that fetuses diagnosed with CPSS should be treated differently according to their types and clinical manifestations. IHPSS patients are more likely to have good outcomes so they may benefit from the "wait-and-see" approach while the other groups deserve closer monitoring. Personalized treatment is essential as CPSS patients can be asymptomatic or have severe complications.