Diagnostic Dilemma

Is it Caroli’s disease?

A 3-month-old boy born of non-consanguineous marriage presented with clay-colored stools since birth and jaundice along with high-colored urine for 8 days. He was born at full term by cesarean section delivery in view of cephalopelvic disproportion and his birth weight was 2.75kg. There was no significant antenatal or family history. Physical examination revealed pallor and weight of 3.3kg (<5th centile). He had icterus with hepatosplenomegaly. There was no dysmorphism. Laboratory investigations revealed hemoglobin of 8.1 gm%, white blood cells (WBC) of 16,000/cumm (33%polymorphs, 62% lymphocytes) with adequate platelet count. Liver function tests are depicted in Table 1. Ultrasound (USG) abdomen showed hepatomegaly with non-visualization of gallbladder and kidneys were normal. Hepatobiliary iminodiacetic acid (HIDA) scan showed the presence of tracer till common bile duct with sharp cut off and no excretion for 24 hours. Liver biopsy showed ballooning degeneration with normal bile ducts. An intraoperative cholangiogram showed a bile plug in the common bile duct. On flushing out the bile duct, there were multiple cystic dilated hepatic radicles suggestive of Caroli’s disease. Serum creatinine was normal (0.4 mg/dl). He was advised of regular follow-up.

Is it Caroli’s disease?
Expert Opinion :
Since there are intrahepatic biliary which are dilated and the kidneys are normal, it is suggestive of Caroli's disease.
Answer Discussion :
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