Atresia biliar

Antecedentes

Vista intraoperatoria de atresia biliar extrahepática completa.

• Es rara, pero es la indicación principal para el trasplante de hígado en niñosMieli-Vergani G1, Vergani D. Atresia biliar. Semin Immunopathol. 2009 Sep;31(3):371-81. PMID: 19533128
• La etiología exacta es desconocida, pero la inflamación conduce a la destrucción de los conductos biliares intra y extrahepáticos
• El pronóstico mejora significativamente con el diagnóstico tempranoWadhwani SI1, Turmelle YP, Nagy R, Lowell J, Dillon P, Shepherd RW. Ictericia neonatal prolongada y el diagnóstico de atresia biliar: un análisis de tendencias en la edad de diagnóstico y resultados en un centro único. Pediatrics. 2008 May;121(5):e1438-40. PMID: 18443020
• Dos formas:
  • Perinatal/postnatal (90% de los casos), posiblemente relacionada con infección
  • Fetal/embrionaria (10%)
    • A menudo con otras malformaciones asociadas (p. ej enfermedad cardíaca congénita, polisplenia o asplenia, malrotación, situs inversus, malformaciones de la vena porta o la arteria hepática)

Gallbladder disease types

Gallbladder anatomy (overview).

Gallbladder anatomy

Bile duct and pancreas anatomy. 1. Bile ducts: 2. Intrahepatic bile ducts; 3. Left and right hepatic ducts; 4. Common hepatic duct; 5. Cystic duct; 6. Common bile duct; 7. Sphincter of Oddi; 8. Major duodenal papilla; 9. Gallbladder; 10-11. Right and left lobes of liver; 12. Spleen; 13. Esophagus; 14. Stomach; 15. Pancreas: 16. Accessory pancreatic duct; 17. Pancreatic duct; 18. Small intestine; 19. Duodenum; 20. Jejunum; 21-22: Right and left kidneys.

• Symptomatic cholelithiasis (biliary colic)
• Choledocholithiasis
• Acute calculous cholecystitis
• Ascending cholangitis
• Acalculous cholecystitis
• Biliary atresia
• Cholestasis of pregnancy

Características Clínicas

• Presentation often subtle
• Neonatal jaundice
  • High index of suspicion for jaundice that persists beyond 1st week of life or includes conjugated hyperbilirubinemia
• Hepatomegaly
• Acholic stools
• Dark urine
• Late findings: failure to thrive, ascites, splenomegaly

Differential Diagnosis

Indirect (Unconjugated) Hyperbilirubinemia

More common causes are listed first, followed by less common causes

• Breast milk jaundice
  • Due to substances in milk that inhibits glucuronyl transferase. It may start as early as 3rd day and reaches peak by 3rd week of life. It is unlikely to cause kernicterus
• Breast feeding jaundice
  • Patient does not receive adequate oral intake which then causes reduced bowel movement/bilirubin excretion. Best diagnosed by looking for signs of dehydration and comparing weight to birth weight.
• Blood group incompatibility: ABO, Rh factor, minor antigens
• Diabetic mother/gestational diabetes
• Internal hemorrhage
• Physiologic jaundice
• Polycythemia
• Sepsis
• Hemoglobinopathies: thalassemia
• Red blood cell enzyme defects: G6PD Deficiency, pyruvate kinase
• Red blood cell membrane disorders: spherocytosis, ovalocytosis
• Hypothyroidism
• Immune thrombocytopenic purpura
• Mutations of glucuronyl transferase (i.e., Crigler-Najjar syndrome, Gilbert syndrome)

Direct (Conjugated) Hyperbilirubinemia

Conjugated bilirubinemia implies a hepatic or post hepatic cause. More common causes are listed first.

• Hyperalimentation cholestasis
• Neonatal hepatitis
• Cytomegalovirus infection
• Sepsis
• TORCH infection (toxoplasma, other/syphilis, rubella, CMV, HSV)
• Biliary atresia
• Cystic fibrosis
• Hepatic infarction
• Inborn errors of metabolism (e.g., galactosemia, tyrosinosis)

Evaluation

Persistent neonatal cholestasis and biopsy-proven biliary atresia in a 1.5-month-old boy. US shows the triangular cord sign (arrow in a) and increased diameter of the hepatic artery (27 mm, arrow in b)

• Evaluate for other causes of neonatal jaundice, liver failure
• Assess for complications of liver disease (e.g. coagulopathy)
• RUQ US to screen for other abnormalities (though cannot definitively diagnose biliary atresia)
• Definitive diagnosis made by biopsy or surgical pathology

Management

• Surgery
  • Kasai procedure or liver transplant for those who fail kasai
• Coagulopathy (if present) responds to vitamin K supplementation

Disposition

• Admit

See Also

• Neonatal jaundice

External Links

References

https://pedemmorsels.com/biliary-atresia/