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Syndromic Biliary Atresia

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: 21 days ago
Updated: 15 days ago
Tags: PEDS
Modality/Study Types: US NM
Activities:
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History

Infant with jaundice and congenital heart disease.


Case Images


Diagnosis

Syndromic Biliary Atresia

Findings

US-Situs inversus with right sided spleen and stomach and midline liver. Color Doppler images of the porta hepatis show replacement of the common bile duct with an echogenic cord.

NM-Midline liver with non visualization of the bile ducts, gallbladder and bowel.

Discussion

Biliary atresia is a progressive fibroinflammatory disease that results in destruction of the intrahepatic and extrahepatic bile ducts. The etiology of biliary atresia is unknown, but it is postulated to be due to a combination of immunologic, viral, genetic, vascular and environmental factors.

The two main subtypes of biliary atresia are embryonic/fetal biliary atresia, also known as “syndromic” biliary atresia, which affects 10-20% of those with the disease and perinatal/postnatal biliary atresia, which affects 80-90%. Embryonic/fetal biliary atresia is associated with an increased incidence of additional congenital anomalies including asplenia, polysplenia, situs inversus, polycystic kidney, intestinal malrotation, small-intestinal atresia and positional abnormalities of the portal and hepatic veins.

The primary clinical manifestations of biliary atresia include jaundice and hyperbilirubinemia in the neonatal period, which can make diagnosis of the condition difficult to discern from other causes of neonatal jaundice. Labs will show a direct hyperbilirubinemia with elevated ALT, AST, alkaline phosphatase and gamma-glutamyltranspeptidase.

Abdominal ultrasound is the most common initial imaging modality used in diagnosis. The demonstration of a triangular cord in the vicinity of the portal vein may be specific for patients with biliary atresia but has low sensitivity. MR imaging of the biliary tree and direct imaging via cholangiography can also be used, and intra-operative cholangiogram is gold-standard for diagnosis.

Treatment of biliary atresia involves early recognition and early management with the Kasai procedure, in which bile can drain directly from the liver into the Roux-limb via microscopic ductules in the portal plate. After Kasai procedure, one-third to one-half of patients will require a liver transplant within the first year of life.

Reference

Moreira RK, et al. Biliary atresia: A multidisciplinary approach to diagnosis and management. Arch Pathol Lab med (2012):136_746-760.

Contributor

Ellen J. Junewick, PA-S



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