Biliary Atresia

• Biliary atresia is a progressive fi brosing process of the extrahepatic

biliary tree in the neonate. It presents with jaundice and is life-
threatening if fl ow of bile is not restored.
Pathophysiology:
• (a) Incidence:
• (i) One in 10,000–18,000 births.
• (ii) Higher incidence in females.
• (iii) Higher incidence in Asian and African-American children.
• (b) Fibrosis and obstruction of biliary passages in the liver.
• (c) Progression to extrahepatic biliary tree.
• (d) Types of biliary atresia:
• classifi ed by how high into the liver the biliary tree is obstructed.
• Type I: (10 % of cases) Obliteration of the common bile duct.
• Type II: (2 % of cases) Obliteration of the common hepatic duct.
• Type III: (88 % of cases) Obliteration from the common bile duct up to
the right and left hepatic ducts into the porta hepatis (liver hilum).
• (e) No proved etiology, but may be due to prenatal infection initiating
a process of infl ammation and scarring in the biliary tree.
• (f) Associated conditions:
• (i) Polysplenia/asplenia.
• (ii) Situs inversus.
• (iii) Preduodenal portal vein.
• (iv) Cardiac anomaly.
• 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.
• Alagille syndrome: paucity of interlobar bile ducts. Normal Right
hepatic duct Left hepatic duct Liver Common hepatic duct Common
bile duct Pancreatic duct Pancreas Type I Type II Gallbladder
Duodenum Type III Fig. 1 Classifi cation of biliary atresia ( Source :
Bryan Walters and Christopher Coppola) C.P. Coppola 201
• (iv) Choledochal cyst and Caroli disease (intrahepatic ductal dilation).
• (v) Alpha 1 anti-trypsin defi ciency.
• (vi) Cystic fi brosis.
• Clinical presentation:
• (a) History.
• (i) Jaundice.
• (ii) Dark urine.
• (iii) Pale white or grey stools.
• (iv) Poor weight gain.
• (v) Itching and irritability.
Physical examination:
• (i) Jaundice.
• (ii) Hepatomegaly/splenomegaly.
Diagnosis:
• (a) Laboratory workup:
• (i) CBC.
• (ii) LFTs.
• (iii) ToRCH infection titers.
• (iv) Alpha 1 anti-trypsin level.
• (v) Sweat chloride.
Imaging:
• (i) Abdominal sonography: can show presence/absence of gallbladder,
dilation of intrahepatic and extrahepatic bile ducts.
• (ii) Hepatobiliary iminodiacetic acid (HIDA) scan: should occur after 5
days of phenobarbital treatment for best results. Will show if liver is
able to process bile and if there is an intrahepatic or extrahepatic
blockage. Normal HIDA scan rules out biliary atresia. Abnormal HIDA
scan needs follow-up with liver biopsy and/or cholangiogram.
• Liver biopsy: can diagnose the cholestasis of biliary atresia and
identify alternate causes such as hepatitis or paucity of interlobar bile
ducts (Alagille syndrome).
• (d) Intraoperative cholangiogram: when no other cause for the
jaundice is identifi ed and HIDA scan is suggestive of biliary atresia, an
open or laparoscopic evaluation of liver is performed. If not
performed already, a liver biopsy can be done at this time. The
gallbladder and extrahepatic bile ducts are examined for patency or
obliteration. A cholangiogram is then performed either through the
gallbladder or the cystic duct. Biliary atresia is identifi ed by blockage
of the extrahepatic bile ducts by obliteration and scarring.
• (e) In a few centers, neonatal endoscopic retrograde
cholangiopancreatography (ERCP) is available in neonates to
determine patency of the biliary tree.
Treatment:
• (a) Phenobarbital.
• (b) Exploratory laparotomy for liver biopsy and cholangiogram.
(c) Kasai procedure:
• (i) Kasai procedure is hepato-portoenterostomy.
• (ii) The extrahepatic biliary tree (gallbladder, common bile duct, and
common hepatic duct) is resected.
• (iii) An anastomosis is formed between the jejunum and the hilum of
the liver.
• (iv) A roux limb of jejunum is brought up to the liver hilum, and a
downstream roux-en-y anastomosis is created.
(c) Kasai procedure:
• (v) The level of anastomosis at the liver hilum is determined by how
extensive the bile ducts have been scarred. Usually the common hepatic
duct is resected, and if bile fl ows from the right and left hepatic ducts,
the anastomosis is made there.
• (vi) If there is no fl ow of bile, the right and left hepatic ducts are
resected up to the level of the hilar plate and the jejunum is
anastomosed to the surface of the liver without any formal connection
between intestinal wall and biliary ductal wall.
• (vii) Frozen section evaluation of a biopsy from the hilar plate can help
by revealing that patent biliary ductules have been reached, but this is
no guarantee of successfully postoperative fl ow of bile.
Postoperative care:
• 1. Patients are maintained NPO with IV fl uid until gut function returns.
• 2. LFT’s are measured to track drainage of bile.
• 3. Postoperative antibiotics may reduce the chance of cholangitis.
• 4. Steroids have been used in the past in an attempt to reduce the
ongoing process of scarring and atresia in remaining liver, but they are
not universally given after Kasai procedure.
• 5. Ursodeoxycholic acid aids bile flow.
• (d) Liver transplantation: when Kasai has not been performed by age 3-
monthsold, or if Kasai procedure is unsuccessful in producing bile
drainage from liver, the child is a candidate for liver transplantation.
Outcome:
• (a) Without operation to restore biliary drainage, 50 % of children die by age 5-years-old.
• (b) Complications of Kasai procedure:
• (i) Failure to drain bile/jaundice. Bile may drain initially and then cease when the process of atresia
extends further into the liver.
• (ii) Sepsis.
• (iii) Cholangitis.
• (iv) Bile leak.
• (v) Ascites.
• (vi) Leak from roux-en-y anastomosis.
• (vii) Bowel obstruction.
• (viii) Cirrhosis and liver failure.
• (ix) Portal hypertension.
• (x) Poor growth and malnutrition. (xi) Defi ciency of fat soluble vitamins A, D, E, and K.
• (c) Patients treated with Kasai portoenterostomy before age 2-
months-old have better prognosis than those treated later.
• (i) 1/3 patients have bile drainage with Kasai.
• (ii) 1/3 will survive with a liver transplant.
• (iii) 1/3 will die.