Can an Infant Have Biliary Atresia Without Jaundice and Dark Urine?
No, an infant cannot have biliary atresia without jaundice and dark urine—these are defining clinical features of the disease. Biliary atresia invariably presents with conjugated hyperbilirubinemia (jaundice) and dark urine due to renal excretion of water-soluble conjugated bilirubin 1, 2, 3.
Why These Features Are Mandatory
Jaundice from conjugated hyperbilirubinemia is the hallmark presentation of biliary atresia, typically appearing within the first 2-8 weeks of life and persisting beyond 2-3 weeks 1, 2, 3, 4.
Dark urine occurs universally because conjugated bilirubin is water-soluble and excreted by the kidneys, creating the characteristic dark coloration 1.
The pathophysiology makes these symptoms inevitable: biliary atresia causes complete obstruction of bile flow from obliteration of the extrahepatic biliary system, leading to progressive accumulation of conjugated bilirubin in the bloodstream 2, 3.
The Complete Clinical Triad
Beyond jaundice and dark urine, biliary atresia presents with:
Acholic (pale/clay-colored) stools are the third cardinal feature, distinguishing biliary atresia from other causes of neonatal cholestasis 5, 1, 2, 3.
Hepatomegaly develops as progressive cholestasis damages the liver 6, 3.
Failure to thrive and poor weight gain reflect malabsorption of fat-soluble vitamins and progressive liver dysfunction 1, 3.
Critical Diagnostic Thresholds
Any conjugated bilirubin >1.0 mg/dL (>25 μmol/L) when total bilirubin ≤5 mg/dL requires urgent evaluation for biliary atresia or other cholestatic disease 1.
Jaundice persisting beyond 2-3 weeks with conjugated hyperbilirubinemia defines pathologic neonatal cholestasis requiring immediate intervention 5, 1.
All infants with conjugated hyperbilirubinemia must be urgently referred to specialized centers, as success of the Kasai portoenterostomy depends critically on performing the procedure before 60 days of age, ideally before 8 weeks 1, 2, 3, 4.
Common Pitfall to Avoid
The absence of jaundice and dark urine effectively rules out biliary atresia. If an infant lacks these features, alternative diagnoses should be pursued. However, visual estimation of jaundice severity is unreliable, particularly in darkly pigmented infants—always obtain objective bilirubin measurements 5.