List clinical features.
Jaundice
Prolonged neonatal jaundice (> 2 weeks)
Onset 2 weeks after birth is possible.
Acholic stools, dark urine
Hepatomegaly
List lab studies.
Conjugated hyperbilirubinemia
↑ Aminotransferases and alkaline phosphatase
↑ GGT
List ultrasound findings.
Absence of the gallbladder
No dilatation of the biliary tree
Describe liver biopsy.
Active inflammation with bile duct degeneration and fibrosis
Bile duct proliferation
Portal stromal edema
List other diagnostic options.
Hepatobiliary scintiscanning (HBSS): failed excretion of the tracer into the bowel
Intraoperative cholangiography to confirm diagnosis
What is the treatment?
Kasai procedure (hepatoportoenterostomy): a connection is created between the liver and the small intestine to allow for bile drainage.
In cases of liver cirrhosis: liver transplantation (primary reason for liver transplantation in children)
What are complications?
If undetected
Early biliary liver cirrhosis (at approx. 9 weeks of age)
Children may die within the first 2 years of life
Postoperative
Cholangitis in 50% of cases
Portal hypertension in > 60% of cases
Last changed2 years ago