Les cholestases néonatales

Abstract

Neonatal jaundice lasting more than two weeks needs urgent investigations, starting with examination of stool colour and blood tests with total and conjugated serum bilirubin. If neonatal cholestasis (NC) is confirmed, vitamin K should be immediately injected, and the child should be referred to a specialised centre for investigations and treatment. Biliary atresia (BA) is the first cause of NC. Its diagnosis is urgent, since the chance of success of the conservative surgical treatment (Kasai operation or variants) decreases rapidly as the age at surgery increases. Normal ultrasound scans cannot rule out BA. The diagnosis can often be suspected before surgery, and is confirmed by operative findings with or without cholangiogram. In case of failure to restore the biliary drainage, biliary cirrhosis progresses and leads to liver transplantation, generally in the first years of life. The other causes of neonatal obstructive jaundice are stones in the main bile duct, choledocal cyst and spontaneous perforation of bile ducts. Correction of biliary obstruction (by surgery or interventional radiology) is also urgent to prevent progression of liver fibrosis, which is very rapid in the first months of life. The most common medical aetiologies of NC are: Alagille syndrome, 1-antitrypsin deficiency, progressive familial intrahepatic cholestasis and cystic fibrosis. Other medical causes of NC are numerous, including infectious, hormonal, nutritional, metabolic or storage diseases. Some of the conditions may require a specific therapy.