Das gelbe Baby

Neonatal jaundice is common and usually harmless, mostly it is due to physiological jaundice. If the critical bilirubin plasma concentration is not exceeded, no further investigation or treatment is required; however, in neonates with persistent jaundice (> 2 weeks) a fractionated bilirubin screening is mandatory. The detection of conjunctival jaundice is particularly sensitive. If the proportion of direct bilirubin level exceeds 1.0 mg/dl (17 mmol/l), this is always an expression of hepatobiliary disease, which is generally referred to as neonatal cholestasis (NC) and needs a prompt investigation to determine the cause. Conjugated hyperbilirubinemia occurs in numerous diseases, which in principle can be based on four pathomechanisms: obstructive or nonobstructive cholestasis, hepatocellular damage, “isolated” disorders of the canalicular excretion of conjugated bilirubin. The incidence of NC is assumed to be approximately 1:2500 live births. Due to the reduced excretion of bilirubin in the stool, the major signs of obstructive cholestasis are also pale stools and/or yellow urine, which is normally as clear as water in neonates. In order to avoid severe, especially subsequent damage and for an early intervention NC should be considered an emergency. Depending on the etiology, treatment can be either purely supportive or (additionally) specific. Due to the frequency but also the crucial importance of an early intervention, biliary atresia (BA) should first and foremost be clarified. For this purpose, reliable direct visualization of the extrahepatic bile ducts (including the hepatic bifurcation) is absolutely essential. This is only possible using endoscopic retrograde cholangiopancreatography (ERCP) or open cholangiography. The open intraoperative visualization of the bile ducts is inherently technically unsuitable for reliably visualizing the patency of very fine bile ducts, which means that diseases such as Alagille syndrome are regularly misinterpreted as BA and patients unnecessarily undergo a hepatoportoenterostomy.