Congenital Portosystemic Shunts in Children: A Case Series From Single Centre

ABSTRACT

Background: Congenital portosystemic venous shunts (CPSS) are uncommon foetal vascular developmental anomalies where splanchnic venous flow bypasses liver. Four cases of CPSS are reported at our centre. Case Summary (1) Eight years old female child presented with Dengue with no features of chronic liver disease and normal liver function test (LFT). Ultrasonography (UGS) abdomen reported an incidental finding of abnormal vascular shunt in liver. Further imaging revealed an anastomosis between portal vein and intrahepatic part of inferior vena cava (IVC), hypoplastic portal vein and multiple nodules in bilateral liver lobes. Interventional Radiologist closed the anastomosis using vascular plug. Child sustained the procedure well. (2) Two months old female patient presented with high GGTP cholestasis, dysmorphism and deranged LFT. On USG abdomen there was intrahepatic portosystemic shunt. MDCT abdomen revealed 2 vascular shunts between left portal vein to middle hepatic vein and left portal vein. Cholestasis responded with symptomatic treatment, hence being followed-up for observation till 1year of age for complications and possibility of spontaneous closure. (3) Twenty-two days old, full term female child presented with convulsions and high GGTP cholestasis with multiple hematomas in brain. LFTs were deranged. 2D-ECHO showed small PFO. USG abdomen suggested a channel between left portal vein and hepatic vein. Patient tested COVID positive hence quarantined now and further evaluation is awaited. (4) One day old, late preterm male baby presented with respiratory distress and pulmonary hypertension with antenatal scan suggesting ductus venous agenesis with hepatic vascular malformation. Patient developed cholestasis with deranged LFT. 2D-ECHO showed PDA and ASD. MDCT abdomen revealed connection between main portal vein and intrahepatic IVC. Conclusions: CPSS has heterogeneous presentation. It can be diagnosed antenatal or postnatal, may be asymptomatic or may present as neonatal cholestasis and may be associated with anomalies. Management may vary from case to case and mainly depends on complications and age of presentation.