Suggest Treatment For Congenital Biliary Anomalies In Infants
Posted on Thu, 3 Apr 2014
100518
Question: Hello, my niece is only 2 weeks old and is having gallbladder problems it is working in a sluggish way, her liver enzymes are going up. The Doctors say that they have to go in and connect small intestine for it to work properly. Her liver is also centralized and not in the right place and she also has a heart murmur. My question is, with her being so young and with her conditions how XXXXXXX and what are the chances of this operation to be successful? Any answer would or thought/opinion I would greatly appreciate.
XXXX
Brief Answer:
Hi and welcome to HCM
Detailed Answer:
Thanks for the query.
Considering your description you are probably talking about congenital biliary anomalies. Most commonly we see biliary atresia and it is not actually related to gallbladder but to extrahepatic biliary system. It means that bile cant flow from liver to small intestines and this leads to serious liver damage and bilirubin elevation. This is very serious condition which always has fatal outcome if surgical procedure isnt performed. Operation you re talking about is so called Kasai procedure or hepatoportojejunostomy. It is complex procedure but in most hospitals it can be performed by experienced pediatric surgeons; there is some risk of intraoperative and postsurgical complications but this is not something that you should think of at this point. The procedure isnt problematic for itself, but further prognosis is hardly predictable because these children always need liver transplantation sooner or later. So Kasai procedure isnt permanent solution, it will just delay necesity for transplantation for several years. But also it prevents liver infections and allow bile drainage to intestines so baby can develop normaly for some time(from several months to several years). In every case you can expect that procedure will be done safely and then you can plan liver transplantation from living-donor which could be permanent cure for baby.
I am sure that everything will be fine. WIsh you good health. Regards
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Suggest Treatment For Congenital Biliary Anomalies In Infants
Brief Answer:
Hi and welcome to HCM
Detailed Answer:
Thanks for the query.
Considering your description you are probably talking about congenital biliary anomalies. Most commonly we see biliary atresia and it is not actually related to gallbladder but to extrahepatic biliary system. It means that bile cant flow from liver to small intestines and this leads to serious liver damage and bilirubin elevation. This is very serious condition which always has fatal outcome if surgical procedure isnt performed. Operation you re talking about is so called Kasai procedure or hepatoportojejunostomy. It is complex procedure but in most hospitals it can be performed by experienced pediatric surgeons; there is some risk of intraoperative and postsurgical complications but this is not something that you should think of at this point. The procedure isnt problematic for itself, but further prognosis is hardly predictable because these children always need liver transplantation sooner or later. So Kasai procedure isnt permanent solution, it will just delay necesity for transplantation for several years. But also it prevents liver infections and allow bile drainage to intestines so baby can develop normaly for some time(from several months to several years). In every case you can expect that procedure will be done safely and then you can plan liver transplantation from living-donor which could be permanent cure for baby.
I am sure that everything will be fine. WIsh you good health. Regards