Related Experiment Videos
An unusual case of biliary atresia
M S Tullu1, V G Hanchate, P R Hira
1Department of Pediatrics and Radiology, Seth G.S. Medical College, KEM Hospital, Mumbai 400012.
Insights
This study reports a rare case of biliary atresia in a child with multiple intrahepatic biliary cysts, also known as bile lakes. The child also presented with pulmonary arteriovenous fistulae, another uncommon finding in biliary atresia.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Radiology
Background:
- Biliary atresia is a rare neonatal liver disease.
- Intrahepatic biliary cysts are an uncommon manifestation in biliary atresia.
Observation:
- A ten-month-old child with biliary atresia presented with multiple intrahepatic biliary cysts ('bile lakes').
- Abdominal imaging, including ultrasonography, CT, and MRCP, confirmed the presence of these cysts.
- Contrast-enhanced echocardiography revealed intrapulmonary shunting due to pulmonary arteriovenous fistulae.
Findings:
- The co-occurrence of 'bile lakes' and pulmonary arteriovenous fistulae in a patient with biliary atresia is exceptionally rare.
- This case highlights unusual imaging findings in a complex pediatric hepatobiliary disorder.
Implications:
- These rare findings expand the spectrum of potential complications in biliary atresia.
- Further research may elucidate the underlying mechanisms connecting these anomalies.
- Enhanced diagnostic imaging protocols may be considered for pediatric patients with biliary atresia presenting with atypical features.
Abstract:
Intrahepatic biliary cysts are rarely seen in the patients with biliary atresia. We describe a ten-month-old child with biliary atresia in whom the abdominal imaging studies (ultrasonography, computed tomographic scan and magnetic resonance cholangiopancreatography) revealed multiple intrahepatic biliary cysts ('bile lakes'). The child also had intrapulmonary shunting of blood due to pulmonary arteriovenous fistulae, which were demonstrated on contrast-enhanced echocardiography. Both these findings, 'bile lakes' and pulmonary arteriovenous fistulae occur rarely in biliary atresia.