Cholestasis, Extrahepatic: Choledochal Cyst

Key Features

• Extrahepatic neonatal cholestasis is characterized by

  • Complete and persistent cholestasis (acholic stools) in the first 3 months of life

  • Lack of patency of the extrahepatic biliary tree proved by intraoperative, percutaneous, or endoscopic cholangiography

  • Firm to hard hepatomegaly

  • Typical features on histologic examination of liver biopsy tissue

• Cause only 2–5% of cases of extrahepatic neonatal cholestasis; incidence is higher in girls and patients of Asian descent

• In most cases, the clinical manifestations, basic laboratory findings, and histopathologic features on liver biopsy are indistinguishable from those associated with biliary atresia

Clinical Features

• Cystic lesions located in all or part of the extrahepatic biliary system

• Recurrent episodes of right upper quadrant abdominal pain

• Fever

• Vomiting

• Obstructive jaundice

• Pancreatitis

• Right abdominal mass

• Infants and children with choledochal cysts are at increased risk for developing bacterial cholangitis

Diagnosis

• Ultrasonography or magnetic resonance imaging (MRI) reveals the presence of a cyst

Treatment

• Surgery is indicated for neonates once abnormalities in clotting factors have been corrected and bacterial cholangitis, if present, has been treated with intravenous antibiotics

• Excision of the cyst and choledocho–Roux-en-Y jejunal anastomosis are recommended