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Patient Story

A 4-week-old full-term female is seen for routine follow-up with her pediatrician. Her mother reports that she has been taking breast milk well with good weight gain. She has no specific concerns; however does report she noticed lighter colored stools recently. On physical exam, jaundice and scleral icterus are noted, along with dark urine in her diaper. The pediatrician orders labs drawn urgently and calls the pediatric gastroenterologist for immediate assistance. The work-up is expedited and she is found to have biliary atresia (Figures 61-1 and 61-2). The Kasai portoenterostomy procedure is performed to treat the cholestatic jaundice. This surgery involves exposing the porta hepatis and attaching part of the small intestine to the exposed liver surface to allow bile to drain out of the liver into the intestines.


Biliary atresia on a hepatobiliary nuclear scintigram. Selected static images of the abdomen and pelvis seen here show good uptake of Tc-99m mebrofenin radiotracer in the liver (large arrow); however, there is no evidence of excretion into the biliary tree, gallbladder, or intestines. Images are taken at 1 min (A), 15 min (B), 30 min (C), 60 min (D), 4 hrs (E), and 24 hrs (F) after tracer injection. There is excretion into the bladder (small arrow) via the kidneys from backup of tracer in the blood pool. Lack of bowel visualization on 24-hour images is suggestive of biliary atresia. (Used with permission from Shyam Srinivas, MD.)


Biliary atresia on a liver biopsy. The typical changes seen on liver biopsy (hematoxylin and eosin stain) in a patient with biliary atresia. Note the characteristic portal edema, marked bile ductular proliferation, and chronic cholestatic changes to the periportal hepatocytes. In the inset, ductular cholestasis can be appreciated with bile duct plugging (arrow). (Used with permission from Thomas Plesec, MD.)


  • Neonatal jaundice can be a common finding in the first 2 weeks of life.

  • Majority of cases are caused by physiologic jaundice and breast milk jaundice, characterized by unconjugated hyperbilirubinemia, which can spontaneously resolve.

  • However, neonatal jaundice persisting after the 2nd week of life is concerning and requires urgent investigation to differentiate unconjugated hyperbilirubinemia from the less frequent, but pathological and more serious condition of cholestatic jaundice.

  • Cholestatic jaundice is characterized by the elevation of conjugated bilirubin, indicating impaired hepatobiliary function.

  • Conjugated hyperbilirubinemia has been defined as (1) direct bilirubin >1 mg/dL if total bilirubin is <5 mg/dL or (2) direct bilirubin >20 percent of the total bilirubin if the total bilirubin >5 mg/dL.1

  • Neonatal cholestatic jaundice can be serious and requires early detection by the ...

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