Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines

Authors

  • Praveen Mathur
  • Rahul Gupta
  • Varsha Soni
  • Reyaz Ahmed
  • Ram Babu Goyal

DOI:

https://doi.org/10.47338/jns.v3.73

Keywords:

Polysplenia, Biliary atresia, Malrotation

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.

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Published

2014-01-05

How to Cite

1.
Mathur P, Gupta R, Soni V, Ahmed R, Goyal RB. Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines. J Neonatal Surg [Internet]. 2014Jan.5 [cited 2021May6];3(1):9. Available from: https://jneonatalsurg.com/ojs/index.php/jns/article/view/73

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