title: Biliary atresia associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines.
abstract: 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.
authors.affiliation: Department of Paediatric Surgery, SMS Medical College, Jaipur.
authors.forename: Praveen
authors.initials: P
authors.lastname: Mathur
authors.affiliation: Department of Paediatric Surgery, SMS Medical College, Jaipur.
authors.forename: Rahul
authors.initials: R
authors.lastname: Gupta
authors.affiliation: Department of Paediatric Surgery, SMS Medical College, Jaipur.
authors.forename: Varsha
authors.initials: V
authors.lastname: Soni
authors.affiliation: Department of Paediatric Surgery, SMS Medical College, Jaipur.
authors.forename: Reyaz
authors.initials: R
authors.lastname: Ahmed
authors.affiliation: Department of Paediatric Surgery, SMS Medical College, Jaipur.
authors.forename: Ram Babu
authors.initials: RB
authors.lastname: Goyal
keywords: Biliary atresia; Malrotation; Polysplenia