Journal
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
Volume 72, Issue 2, Pages 216-219Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPG.0000000000002932
Keywords
biliary atresia and splenic malformation syndrome; Kasai porto-enterostomy; Pediatric End-stage Liver Disease (PELD) score; portal cavernoma
Ask authors/readers for more resources
Biliary atresia can lead to rapid deterioration of liver function and may result in death within three years without treatment or liver replacement. However, there are rare cases where patients with biliary atresia splenic malformation syndrome can survive without the need for surgery, suggesting possible alternative mechanisms at play.
In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years. Remarkably, she remained in satisfactory condition in absence of ascites or severe hepatic dysfunction, when 4 other similar patients-managed during the same period of time-all had the usual clinical deterioration and ascites, with the need for liver replacement. To our knowledge, there is no similar report in literature. Possible pathogenetic mechanisms and the role of portal hypertension as important factors are discussed.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available