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Transjugular intrahepatic portosystemic shunt in an infant
G Sergent1, F Gottrand, O Delemazure
1Service de Radiologic Est, Hôpital Huriez, C. H. R. U., Place de Verdun, F-59037 Lille Cedex, France.
Insights
A transjugular intrahepatic portosystemic shunt successfully managed ascites and bleeding in a cystic fibrosis patient. However, the patient later died from liver failure due to low-flow syndrome.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Interventional Radiology
Background:
- Cystic fibrosis can lead to biliary cirrhosis and complications like refractory ascites and intestinal bleeding.
- Management of portal hypertension in pediatric patients with cystic fibrosis presents unique challenges.
Observation:
- A 15-month-old girl with CF-related biliary cirrhosis presented with refractory ascites and recurrent intestinal bleeding.
- The patient underwent a successful percutaneous transjugular intrahepatic portosystemic shunting (TIPS) procedure.
- Post-procedure, ascites resolved, and bleeding ceased immediately.
Findings:
- The TIPS stent remained patent on Doppler ultrasound until day 22.
- The patient developed liver failure secondary to low-flow syndrome and severe hepatic ischemia.
- Mortality occurred on day 22, without recurrence of ascites or bleeding.
Implications:
- TIPS can provide temporary but significant symptom relief in pediatric patients with advanced liver disease and portal hypertension.
- Low-flow syndrome and hepatic ischemia are critical complications to monitor post-TIPS, even with initial procedural success.
- Further research is needed to optimize TIPS outcomes and manage complications in pediatric populations with cystic fibrosis.
Abstract:
A 15-month-old girl, who presented with biliary cirrhosis secondary to cystic fibrosis with refractory ascites and recurrent intestinal bleeding, underwent percutaneous transjugular intrahepatic portosystemic shunting. Immediately following the procedure the ascites disappeared and no further bleeding occurred. The stent shunt was patent on Doppler ultrasound until the 22nd day. The patient died on day 22 because of liver failure due to a low-flow syndrome with severe hepatic ischaemia, but with no recurrence of bleeding or ascites.