Congenital Intrahepatic Portosystemic Venous Shunt Between Portal Vein and Hepatic Vein: Perinatal Ultrasonographic Detection and Follow-up

  • Byoung Hee Han, Department of Radiology, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Mi Jin Song, Department of Radiology, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Min Hwan Moon, Department of Radiology, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Young-Ho Lee, Department of Radiology, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Jee Yeon Min, Department of Pediatrics, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Kyung Ah Kim, Department of Pediatrics, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea
  • Yeon Kyung Lee, Department of Pediatrics, Kwandong University College of Medicine, Cheil General Hospital & Women’s Healthcare Center, Korea

    • Objective: To show variable fetal and neonatal ultrasonographic findings of IPVS and the results of follow-up.
    Methods :We detected and followed up 6 congenital IPVS by fetal and neonatal US.
    Results: On fetal US, IPVS were detected in 2. There were abnormal intrahepatic tubular structure in 1, prominent hepatic vein in 1 and congestive heart failure(CHF) in 1. Other findings were intrauterine growth retardation(IUGR) in 4, echogenic bowel in 2, placentomegally in 2, small amniotic fluid in 1, single umbilical artery in 1 and persistent right umbilical vein in 1. Neonatal US showed abnormal communication of a branch of Lt. portal vein with a hepatic vein in 5 and communication of two branches of Lt. portal vein with two hepatic veins in 1 There was coincidental shunt between hepatic artery and portal vein in 1. Four of the six congenital IPVS were spontaneously closed on follow-up US (2-11months), one of them was treated with coil embolization during neonatal period, and the last one is not closed until now.
    Conclusions: US demonstrated congenital IPVS in just two fetuses. However, we suggest that if the secondary findings such as abnormal tubular structure, prominent hepatic vein, and CHF are detected, IPVS should be suspected. IUGR is nonspecific but the most common associated finding. Neonatal US is useful for the confirmation, detection of prenatally undetected congenital IPVS, and classification. Clinical observation and follow-up with US until closure are the first choice for management of congenital IPVS.