J Korean Soc Neonatol.  2011 May;18(1):117-123. 10.5385/jksn.2011.18.1.117.

A Clinical Study of Congenital Intrahepatic Portosystemic Shunt Diagnosed in Neonatal Period

• Kim JY
• Kim KA
• Lee YK
• Ko SY
• Shin SM
• Han BH

• KMID: 2188476
• DOI: http://doi.org/10.5385/jksn.2011.18.1.117

Abstract

PURPOSE
Congenital intrahepatic portosystemic shunts are rare disease and clinically asymptomatic shunts may be detected by chance on ultrasonogram before and after birth. We studied clinical course, treatment and prognosis of congenital intrahepatic portosystemic shunt at prenatal or neonatal period.
METHODS
Medical records of 8 patients which were diagnosed in intrahepatic portosystemic shunt in Cheil General Hospital from 2006 through 2010 were reviewed retrospectively.
RESULTS
Eight patients with congenital intrahepatic portosystemic shunts were identified. Six patients were diagnosed at prenatal radiological screening, including three cases of intrauterine growth restriction and two cases of preterm baby. One case with increased serum ammonia underwent coil embolization. In four cases including one case that presented elevated direct bilirubin, shunts were closed spontaneously within 11th month after birth. Two patients were diagnosed on abdominal sonogram after birth because of elevated direct hyperbilirubinemia, all of whom presented intrauterine growth restriction. Closure of shunts was confirmed during 4th month to 6th month.
CONCLUSION
Congenital intrahepatic portosystemic shunts are clinically asymptomatic mostly and spontaneous closure is expected within 2 years age. But occasionally they have severe complication, so clinical and radiological observation is needed. Specially in cases of intrauterine growth retardation without evident cause, the possible diagnosis of congenital intrahepatic portosystemic shunts should be considered and prenatal and postnatal examination should be performed. When prenatal diagnosis is made, fetal wellbeing should be monitored periodically until spontaneous closure of shunts.

Keyword

Congenital intrahepatic portosystemic shunts; Intrauterine growth restriction

MeSH Terms

Ammonia
Bilirubin
Fetal Growth Retardation
Hospitals, General
Humans
Hyperbilirubinemia
Mass Screening
Medical Records
Parturition
Portasystemic Shunt, Surgical
Prenatal Diagnosis
Prognosis
Rare Diseases
Ammonia
Bilirubin