Objectives: Extrahepatic Biliary atresia is a surgically correctable congenital anomaly in infants. As the surgical success of Kasai procedure depends upon early operation (i.e within 60 days of life), the dynamic changes of clinical profile of EHBA need to be studied. Early detection of EHBA is of paramount importance for ultimate survival of patients.

Method:  Twenty- Six infants of EHBA who were admitted in pediatric Gastroenterology ward of PGIMER from Jan 1996 to June 2001 were taken for study. The clinical profile, ultrasonogram, hepatobiliary scintigraphy (HIDA Scan), POC (Peroperative Cholangiogram), liver biopsy, LFT, routine blood test etc. were studied.

Results: Out of 26 cases, 18 were male and 8 were female. Age of infants varied from 1 month to 9 month. Onset of jaundice and clay coloured stool was variable, ranging from day 1 to day 21 after birth. Twenty out of 26 (76%) infants showed jaundice and clay coloured stool before 2 weeks of life. This is contrary to age old observation that jaundice of EHBA usually appears after 2 weeks of life. Out of 26 cases, 9 patients (34%) attended hospital for the first time before 2 months of age. Congenital anomaly was seen in one infant who had Dextrocardia with situs inversus. Only one infant was preterm and 25 were full term. Findings of ultrasonogram and HIDA scan were in concordance with POC findings. Kasai operation was done on 21 (9 infants before 2 months and 12 infants between 2-3 months). On 5 patients, Kasai was not done- their ages were 6 month, 7 months, 8 months, 8.5 months, 9 months. Three post operative patients who had cholangitis before one year of age were successfully treated. Those who were operated before 60 days of life were healthy at one year of age. Those who were not operated or operated after 60 days of life died before one year of age. Liver Biopsies of all infants were consistent with EHBA. Biopsy of infants who were more than 6 months of age had findings of EHBA with predominant portal fibrosis and early cirrhotic changes.

Conclusion:  Jaundice can appear before 2 weeks of age in EHBA. Kasai operation before 60 days have good survival at 1 year of age. Early detection and surgical intervention (less than 60 days) is mandatory.

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