Abstract

Background Portosystemic shunts occur frequently in the cirrhosis setting. Congenital portosystemic shunts are uncommon. Congenital shunts are usually incident detected by ultrasound in infants but rare in adult. The majorities are intrahepatic portosystemic shunts and involve communication between the portal vein and hepatic veins or inferior vena cava. In the case of an extrahepatic portosystemic shunt, which is even less common than an intrahepatic shunt, portomesenteric blood drains into a systemic vein. Materials and Methods We collected 8 cases congenital portosystemic shunt which was diagnosed in recent 10 years. There are 4 men and 4 women with age range from 55-80 years olds (mean age: 66.1 years old). The patients had experienced various symptoms, including disturbance of consciousness (n = 4), abdominal pain (n = 4) that was thought not to be related portosystemic shunt. They were diagnosis by ultrasound (5 cases) and CT scan (8 cases). Laboratory test results were as follows: ammonium elevation in 4 cases, B carrier in 2 cases, and normal laboratory test in 2 cases. Results There were 5 intrahepatic and 3 extrahepatic portosystemic shunts. All 5 intrahepatic portosystemic shunts were diagnosed by ultrasound with color Doppler, but 3 extrahepatic portosystemic shunts were diagnosed by contrast enhancement CT scan. Percutaneous embolization with vascular plug was treatment in 4 cases with hepatic encephalopathy. The hepatic encephalopathy was improvement in treatment of patients. The other 4 cases were observation because of no any symptoms and signs. Conclusions • Imaging plays a crucial role in diagnosis and follow-up of patients with congenital portosystemic shunt, including ultrasound for intrahepatic shunt and CT scan for extrahepatic shunt. • Ultrasound (US) with color Doppler is the initial imaging modality for congenital intrahepatic portosystemic shunt. • Diagnosis of congenital extrahepatic portosystemic shunt is confirmed by contrast enhanced CT scan. • Definite curative surgical or endovascular therapy can be employed for symptomatic congenital portosystemic shunt.

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