Inspissated bile syndrome (IBS) is a rare pediatric condition, with an estimated incidence of 1 in 175,000 live births. It results from the thickening or "inspissation" of bile, often leading to biliary obstruction, cholestasis, and pale or acholic stools. While IBS is typically associated with predisposing factors such as prematurity, sepsis, total parenteral nutrition, or certain medications, it can occur without identifiable risk factors, as seen in this case. Early recognition and treatment are critical in preventing more serious complications such as liver dysfunction or cirrhosis. We report the case of a 4-week-old full-term infant (born at 40 weeks’ gestation) presenting with acholic (white) stools and mild jaundice. Initial laboratory tests showed a total bilirubin level of 5.0 mg/dL (3.9 direct), gamma-glutamyl transferase (GGT) of 352 U/L, alkaline phosphatase (ALP) of 586 U/L, and mildly elevated liver function tests. An abdominal ultrasound demonstrated intra- and extrahepatic biliary dilatation, with echogenic material obstructing the distal common bile duct just proximal to the ampulla, findings highly suggestive of inspissated bile syndrome. The patient was started on ursodiol 40 mg twice daily, a bile acid that promotes bile flow and decreases the viscosity of bile. A follow-up MRCP performed 10 days later showed complete resolution of the biliary obstruction and no evidence of intra- or extrahepatic dilation. This case highlights not only the rarity of IBS but also its ability to present in the absence of typical risk factors such as prematurity, malabsorption disorders, or prolonged total parenteral nutrition. Additionally, it underscores the essential role of imaging modalities such as ultrasound and MRCP in diagnosing biliary obstruction and guiding appropriate management. IBS should be considered in the differential diagnosis for any neonate presenting with jaundice and acholic stools, as timely diagnosis and medical management can lead to full recovery and prevent more serious hepatobiliary complications. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Van Antwerp Emily, Makris Joseph

Keywords: Biliary, Abdominal Imaging, Abdominal Ultrasound