All cases of neonatal obstructive jaundice at St. Louis Children's Hospital were reviewed. The total of 57 included 32 cases of extrahepatic biliary atresia and 16 biopsy-proven cases of giant-cell hepatitis. As a result, the author has reached the following conclusions:
Giant-cell hepatitis can be distinguished from extrahepatic atresia in almost all cases by study of a liver biopsy. The important morphologic criteria include the presence in atresia of bile duct proliferation and of intraductal bile stasis, and the absence of significant hepatic architectural disorganization early in the course of the disease. Clinical data and most laboratory tests are of little use in distinguishing between atresia and neonatal hepatitis. Serial bilirubin determinations are useful, as falling levels over a period of weeks is strong evidence for hepatitis. At laparotomy cholangiography is the most valuable diagnostic tool, with frozen section of value when radiographic studies cannot be made.
Biliary atresia carries an extremely poor prognosis, for few patients have a correctable lesion (12% in this series). Furthermore, the mortality and morbidity of biliary tract surgery in infants is high, irrespective of the age of the patient at operation. This poor prognosis is not due to delay in operation, since cirrhosis and hepatic insufficiency develop relatively late in biliary atresia. Delay of operation until age three months will adversely affect only a very rare patient with atresia.
Neonatal hepatitis is a frequent cause of neonatal obstructive jaundice. Exploratory laparotomy in these patients has a high morbidity and mortality, especially when a prolonged ductal dissection is performed. Early operation (4-8 weeks) will result in more cases of hepatitis being subjected to a hazardous procedure. There is evidence to indicate that if operation is delayed until 3 months of age, many of these patients will show evidence of resolution of their disease, especially a serial fall in bilirubin, and need not be explored.
It is the final conclusion of the author that the management of obstructive jaundice in this age group should be conservative, and that operation should not be performed until a patient is three months of age, and even later if a falling trend is noted in serial bilirubins.
Neonatal giant cell hepatitis secondary to extrahepatic biliary atresia associated with choledochal cyst
