Fig. 1
Classification of biliary atresia (Source: Bryan Walters and Christopher Coppola)
(e)
No proved etiology, but may be due to prenatal infection initiating a process of inflammation and scarring in the biliary tree.
(f)
Associated conditions:
(i)
Polysplenia/asplenia.
(ii)
Situs inversus.
(iii)
Preduodenal portal vein.
(iv)
Cardiac anomaly.
(g)
Differential diagnosis:
(i)
Physiologic jaundice of newborn: should clear by age 2-weeks-old.
(ii)
ToRCH infections:
1.
Toxoplasmosis.
2.
Rubella.
3.
Cytomegalovirus.
4.
Herpes simplex virus.
(iii)
Alagille syndrome: paucity of interlobar bile ducts.
(iv)
Choledochal cyst and Caroli disease (intrahepatic ductal dilation).
(v)
Alpha 1 anti-trypsin deficiency.
(vi)
Cystic fibrosis.
2.
Clinical presentation:
(a)
History.
(i)
Jaundice.
(ii)
Dark urine.
(iii)
Pale white or grey stools.
(iv)
Poor weight gain.
(v)
Itching and irritability.
(b)
Physical examination:
(i)
Jaundice.
(ii)
Hepatomegaly/splenomegaly.
3.
Diagnosis:
(a)
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Laboratory workup:
(i)
CBC.
