Neonatal Cholestasis : Biliary Atresia, the Possible Solution

Abstract

Bachground : Neonatal cholestasis is defined clinically abs the accumulation in the blood conjugated bilirubin higher than 2 mg/dl or a fraction greater than 20 % of an elevated total bilirubin level. The clinical features of any form of cholestasis are similar, including jaundice, dark yellow urine and pale stools. The correct diagnosis of biliary atresia and neonatal hepatitis is important because the clinical course of these two diseases are different. In biliary atresia if Kasai operation would not be performed within 2 months of age, the child will end up with biliary cirrhosis and die at average age of one and a haff year.

Objective : To assess the problem of diagnosis and result of treatment in order to improve management guildline for neonatal cholestasis.

Study design : Retrospective descriptive study. The medical records of patients with neonatal cholestasis during Jury 1997 to July 2000 were used in this study.

Results : There 33 cases of patients, 20 males, 13 females with average age was 82.69 ± 34.39 days. A Conbination of blood test for congenital infection, imaging by ultrasound, DISIDA scan, exploratory laparotomy with intraoperative cholangiography (IOC) and histological study aided in establishing the diagnosis and differentiating biliary atresis from other causes of neonatal cholestasis. There were 13 cases of biliary atresia, 5 cases were neonatal hepatitis and 15 cases were cholestasis with non specific causes. The mean age was 78.07,57.0,97.73 days in group of libiary atresia, neonatal hepatitis, and cholestasis group respectively, The most reliable investigation was exploratory laparotomy and IOC. Kasai operation was done in only 8 cases. Only 2 had good result and liver transplantation was required in one of these. The others had cirrhosis. There were 3 cases in the neonatal hepatitis group and 7 cases in cholestasis group had results.

Conclusions : The early definite diagnosis of neonatal cholestasis is the most important especially biliary atresia which the early operative treatment is crucial. There for, the parents are given advise that jaundice with pale stools after age of 2 – 3 weeks should be regarded as abnormal. The first wellbaby examination should be happens at 4 weeks of age. The appropriate investigations should be performed for detection of biliary atresia which surgical procedure can safe their lives.

Key words : neonatal cholestasis, biliary atresia, neonatal hepatitis.