ABSTRACT: Biliary atresia (BA) is a rare disease and the end result of a destructive inflammatory process in bile ducts, leading to fibrosis, liver cirrhosis., portal hypertension and liver failure

CONCLUSIONS : Although this disease is rare, a knowledge of the pathophysiology involved is essential to plan an appropriate anaesthetic. We have successfully managed such a rare case with the knowledge of pathophysiology of the disease.

Introduction
Biliary atresia (BA) is a rare disease and the end result of a destructive inflammatory process in bile ducts, leading to fibrosis, liver cirrhosis., portal hypertension and liver failure. Other abnormalities present are associated with embryological development like polysplenia, cardiac defects, situsinversus, anomalous hepatic artery, absent IVC and intestinal malrotation. We report the anaesthetic management of such a rare case with coagulopathy posted for kasaihepatoenterostomy.

Case Report
A 2-month-old 4.5 kg male child was diagnosed as extrahepatic biliary atresia and was scheduled for laparotomy. There was yellow discoloration of skin since 2 weeks, which was progressively increasing. He had icterus and hepatomegaly. His serum bilirubin was 13 mg/dL and INR was 2.15. Hepatobiliaryiminodiacetic acid (HIDA) scan was indicative of biliary atresia. There were no cardiac and respiratory system abnormalities. General anaesthesia was induced with fentanyl and propofol. After achieving neuromuscular blockade with atracurium trachea was intubated with endotracheal tube size 3.5 mm ID and lungs were ventilated with Jackson-Rees circuit. Anaesthesia was maintained with oxygen, air and isoflurane. Intraoperative vitals were stable. Bloodloss was replaced with packed red blood cells. Neuromuscular blockade was reversed with neostigmine and glycopyrrolate. Postoperatively, vitals were stable and the baby was shifted to the paediatric intensive care unit.

Conclusion
Although this disease is rare, a knowledge of the pathophysiology involved is essential to plan an appropriate anaesthetic.We have successfully managed such a rare case with the knowledge of pathophysiology of the disease.