Open AccessBiliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines
Author(s) -
Praveen N. Mathur,
Rahul Gupta,
Varsha Soni,
Reyaz Ahmed,
Ram Babu Goyal
Publication year - 2014
Publication title -
journal of neonatal surgery
Language(s) - English
Resource type - Journals
ISSN - 2226-0439
DOI - 10.47338/jns.v3.73
Subject(s) - dextrocardia , situs inversus , medicine , polysplenia , intestinal malrotation , biliary atresia , jaundice , atresia , biliary cirrhosis , gastroenterology , intestinal atresia , congenital hepatic fibrosis , cirrhosis , surgery , pediatrics , portal hypertension , disease , autoimmune disease , transplantation , liver transplantation
Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines. She developed jaundice in the first week of life. Kasai operation was performed but she developed cholangitis and septicemia 2.5 months after surgery and succumbed later.