BILIARY ATRESIA

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BILIARY ATRESIA

• PROF. DR. SHAHENAZ M. HUSSIEN

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• Definition
• Biliary atresia is a condition in
  which the normal extrahepatic biliary system is
  disrupted. Progressive damage of extrahepatic and
  intrahepatic bile ducts secondary to
  inflammation, leading to fibrosis, biliary
  cirrhosis, and eventual liver failure.
• Incidence
• Biliary atresia affects approximately 1 in
  10,000-15,000 births and occurs in 2 distinct
  forms fetal-embryonic and postnatal.
• Types
• The fetal-embryonic form appears in the first 2
  weeks of life and 10-20 of affected neonates
  have associated congenital defects.
• The postnatal form of biliary atresia is
  typically found in neonates and infants aged 2-8
  weeks. Progressive inflammation and obliteration
  of the extrahepatic bile ducts occur after birth.
  This form is not associated with congenital
  anomalies, and infants may have a short
  jaundice-free interval.

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• Classification
• Three main types of biliary atresia are surgicaly
  defined
• 1 -In type I, the common bile duct is
  obliterated while the proximal bile ducts are
  patent.
• 2 -In type II, atresia of the hepatic duct is
  seen. In type IIa, the cystic and common bile
  ducts are patent, whereas in type IIb, the
  cystic, common bile duct and hepatic ducts are
  all obliterated.
• 3-Type III atresia refers to discontinuity of
  both right and left hepatic ducts to the level of
  the porta hepatis. Unfortunately, type III
  biliary atresia is common, accounting for gt90 of
  cases.
• Pathophysiology
• Infection with cytomegalovirus, group C
  rotavirus, and reovirus type 3 have been
  implicated in certain cases. Cholestasis almost
  certainly contributes to ongoing hepatocellular
  and biliary damage.
• Histologic findings on liver biopsy typically
  include acute or chronic inflammatory change with
  obstruction, fibrosis, and the proliferation of
  ductal and glandular element.

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• What are the symptoms of biliary atresia?
• Babies with biliary atresia usually
  appear healthy when they are born. Symptoms of
  the disease typically appear within the first two
  weeks to two months of life. Those symptoms
  include-A baby with biliary atresia usually
  develops jaundice at two or three weeks after
  birth.
• -Dark urine - The bilirubin is filtered by
  the kidney and removed in the urine.
• -Acholic stools (clay-colored stools) --
  because no bile or bilirubin coloring is being
  emptied into the intestine.
• -Also, the abdomen may become swollen from
  a firm, enlarged liver.
• -Weight loss and irritability -- develop
  when the level of jaundice increases.

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• How is biliary atresia diagnosed?
• Blood tests for liver function abnormalities.
• X-rays of the abdomen look for an enlarged liver
  and spleen.
• An abdominal ultrasound can tell if there is a
  small gall bladder or none at all.
• A nuclear test, called an HIDA scan, determines
  the flow of bile. In this scan, a radioactive dye
  is injected into the infant's vein. The dye acts
  like bilirubin.
• If the baby has biliary atresia, the
  liver will take up the dye but it will not be
  able to flow through the damaged biliary system
  into the small intestine.
• A liver biopsy tells if an infant is likely to
  have biliary atresia.
• If the biopsy shows that the infant
  probably has biliary atresia, further surgery
  will confirm the diagnosis.

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• Investigations continue----
• Cholangiography
• Exploratory laparotomy with surgical
  cholangiography is recommended. This is done by
  injecting contrast material through the
  gallbladder. To fined the communication between
  the biliary tree and the gastrointestinal tract,
• Sonography-guided percutaneous cholecystocholangio
  graphy radiographic contrast material is
  injected into the gallbladder under sonographic
  guidance and the extrahepatic biliary system is
  viewed by using fluoroscopy.
• Endoscopic retrograde cholangiopancreatography
  allows direct visualization of the extrahepatic
  biliary tree with the injection of radiologic
  contrast agent into the extrahepatic biliary
  system through the papilla of Vater.
• Duodenal intubation
• To perform this study, a nasogastric
  tube is placed in the distal duodenum. The
  absence of bilirubin in aspirated fluid suggests
  obstruction.
• Liver biopsy
• Percutaneous liver biopsy is useful in evaluating
  neonatal cholestasis.
• Histologic findings, including bile-duct
  proliferation and obstruction, may not be
  definitive in neonates younger than 2 weeks.
• Results of repeat biopsy at 2-week intervals
  confirm the diagnosis in as many as 95 of
  patients.

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• Who is at risk for biliary atresia?
• Biliary atresia is a rare disorder. About one in
  15,000 to 20,000 babies do not have complete bile
  ducts.
• Biliary atresia seems to affect girls more than
  boys.
• There does not appear to be any link to
  medications taken during pregnancy.
• Do children with biliary atresia have other
  associated abnormalities?
• Ten to 15 percent of infants with biliary atresia
  may be born with other problems
• Heart defects.
• Spleen (polysplenia).
• Blood vessels (inferior vena caval anomalies,
  pre-duodenal portal vein).
• Intestine (situs-inversus or malrotation).

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• How is biliary atresia treated?
• -The Kasai procedure is an operation to create
  an open duct so bile can drain from the liver. It
  is named after the surgeon who developed it.
• -The surgeon removes the damaged ducts outside
  of the liver (extrahepatic ducts) and replaces
  them with a piece of the baby's own intestine.
  This new duct allows bile to pass from the liver
  into the intestine.
• -The Kasai procedure is not a cure for biliary
  atresia, but it does allow babies to grow and
  have fairly good health for several years.
• -When this procedure does not work, liver
  transplantation can correct this problem.
• Success with this procedure is related to
• Age. Surgery is most successful in infants
  younger than two to three months of age.
• Extent of liver damage (cirrhosis) at the time of
  surgery.
• The number and size of microscopic ducts in the
  scarred tissue that can drain bile.
• The experience of the surgical and medical team.

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• Guidelines from your doctor for your child's
  nutrition may include
• Children with liver disease have a faster
  metabolism than healthy children. This means that
  children with biliary atresia may require more
  calories.
• A child with biliary atresia cannot properly
  digest fats. This is because not enough bile gets
  to the intestine. Due to liver damage, there may
  also be a loss of vitamins and protein.
• A well-balanced diet, consisting of three meals a
  day plus small snacks in between meals.
• Vitamin supplements.
• Adding medium-chain triglyceride (MCT) oil to
  foods and liquids or infant formulas. MCT adds
  extra calories that will help your child grow.
• High-calorie liquid feedings may be recommended
  if your child is too ill to eat normally.
  Feedings are given through naso-gastric tube.
• Although digestion may return to normal after
  surgery, extra vitamins or MCT oil may be needed.

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• What are the complications of biliary atresia and
  what can be done for them?
• After the Kasai procedure, it is common to get an
  infection in the bile ducts. This is usually
  treated using intravenous antibiotics.
• Jaundice or itching may occur. These can often be
  treated successfully with phenobarbital (for
  jaundice), cholestyramine and ursodeoxycholic
  acid (for itching).
• Many patients with cirrhosis have changes in
  blood flow through the liver and intestines.
  These changes may produce problems such as easy
  bruising of the skin, nasal bleeding, retention
  of body fluid and varices in the stomach and
  esophagus.
• If retention of body fluid occurs, it can be
  treated with diuretics and potassium replacement.
  

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• Liver Transplant
• If there is still not enough bile flow with the
  Kasai procedure, liver transplantation is a final
  option. A liver transplant operation removes the
  damaged liver and replaces it with a new liver
  from a donor.
• After transplant surgery, the child's health may
  improve quite quickly. However, the child's body
  might reject the new organ. To prevent rejection,
  a strict schedule of anti-rejection medications
  must be taken.
• After a transplant, ongoing lifelong care is
  required. Frequent contact with physicians and
  other members of the transplant team is also
  necessary.

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