Mrs lulu Fasna k k
2 nd yr msc nursing
• Congenital absence orclosure of a normal
body opening ortubularstructure.
Biliary atresia is a condition in which the
normal hepatic biliary system is disrupted.
Progressive damage of extrahepatic and
intrahepatic bile ducts which occur secondary to
inflammation, leading to fibrosis, biliary cirrhosis,
and eventual liver failure.
• Biliary atresia affects approximately 1 in 10,000-
• Biliary atresia seems to affect girls more than
• There does not appear to be any link to
medications taken during pregnancy.
BASED ON THE PRESUMED TIMING OF THE
OBLITERATION OF THE LUMEN OF THE EXTRAHEPATIC
The fetal-embryonic form
appears in the first 2 weeks of life
10-20% of affected neonates have associated congenital defects. .
The fetal form, which occurs in up to 20% of cases of biliary atresia
. Associated congenital anomalies include
malrotation of abdominal viscera,
interrupted inferior vena cava,
preduodenal portal vein,
situs inversus, and congenital heart anomalies
• 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
Three main types of biliary atresia are surgicaly defined:
1 type I,
the common bile duct is obliterated while the proximal bile ducts are
2 -type II
atresia of the hepatic duct is seen.
In type IIa, the cystic and common bile ducts are patent,
In type IIb, the cystic, common bile duct and hepatic ducts are all
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 >90% of
• AUTOIMMUNE MECHANISM
• There is clearly a predominance of lymphocytes in BA
• 10 of 11 patients with BA were positive for serum IgG
and IgM antineutrophil cytoplasmic antibodies (ANCA in
BA patients compared with children and adults with other
• It has been proposed that the embryonic form of
BA is caused by defective development of the
• The association of anomalies of visceral organ
symmetry with BA (the polysplenia or BA splenic
• genes that control normal situs development are
also key regulators of normal extrahepatic bile
• Abnormal remodeling of the ductal plate leads to
the ductal plate malformation that is present in
congenital hepatic fibrosis and other bile duct
• The biliary system is the network of tiny tubular structures
and ducts that drain bile from the liver to the small
intestine, where it helps the digestive process
• Bile is a liquid secreted by liver cells, made
up of cholesterol, bile salts and waste
products (including bilirubin]
• Biliary atresia progressively destroys the
bile ducts that carry bile from the liver to
the intestine, beginning outside the liver
and later affecting bile ducts inside the
• The damaged ducts prevent the draining of bile
from the liver; as a result, bile trapped inside the
liver causes damage and scarring that can lead
• As the liverbecomes scarred, it presses against
the walls of the veins. This constricts the veins
and blood cannot pass through themproperly.
The result is portal hypertension (high blood
pressure in the portal vein).
• This congenital disorderbegins to progress very
soon afterbirth. In its most common form,
extrahepatic biliary atresia, ducts outside the
liverare affected first.
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.
Jaundice − a yellow coloring of the skin and eyes due to a
very high level of bilirubin (bile pigment) in the
-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,
-Weight loss and irritability -- develop when the level of
• Small proportion of children will present with vit k
dependent coagulopathy and bleeding.
• Features of cirrhosis
o Hard liver
How is biliary atresia diagnosed?
• Blood tests for liver function abnormalities.
• X-rays of the abdomen-
• look for an enlarged liver and spleen.
• abdominal ultrasound- to find out whether there is a small gall bladder
or none at all.
• A nuclear test-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
• liver biopsy.
.This is done by injecting contrast material through the
To find the communication between the biliary tree and
the gastrointestinal tract,
To performthis study, a nasogastric tube is placed in
the distal duodenum. The absence of bilirubin in
aspirated fluid suggests obstruction.
• Percutaneous liverbiopsy is useful in evaluating
• Histologic findings, including bile-duct proliferation and
Te n to 1 5 pe rce nt o f infants with biliary atre sia m ay be bo rn
with o the r pro ble m s:
• Heart defects.
• Spleen (polysplenia).
• Blood vessels (inferiorvena caval anomalies, pre-
duodenal portal vein).
• Intestine (situs-inversus ormalrotation).
HOWIS BILIARY ATRESIA TREATED?
-TheKasaiprocedureis anoperationto createanopenduct
-Thesurgeonremoves thedamagedducts outsideof theliver
(extrahepatic ducts) andreplaces themwithapieceof the
baby's ownintestine. This new duct allows biletopass
-TheKasaiprocedureis not acureforbiliaryatresia, but it
does allow babies to grow andhavefairlygoodhealthfor
-Whenthis proceduredoes not work, livertransplantation
cancorrect this problem.
• Age. Surgery is most successful in infants youngerthan
two to three months of age.
• Extent of liverdamage (cirrhosis) at the time of surgery.
• The numberand size of microscopic ducts in the scarred
tissue that can drain bile.
• The experience of the surgical and medical team.
Guidelines fornutrition fora child with
Childre n with live r dise ase have a faste r
m e tabo lism than he althy childre n. This m e ans
that childre n with biliary atre sia m ay re q uire m o re
calo rie s.
• Achild with biliary atre sia canno t pro pe rly dig e st
fats. This is be cause no t e no ug h bile g e ts to the
inte stine . Due to live r dam ag e , the re m ay also be
a lo ss o f vitam ins and pro te in.
• 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 orinfant formulas. MCT adds
extra calories that will help yourchild grow.
• High-calorie liquid feedings may be
recommended if yourchild is too ill to eat
normally. Feedings are given through naso-
• Altho ug h dig e stio n m ay re turn to no rm alafte r
surg e ry, e xtra vitam ins o r MCT o ilm ay be
ne e de d.
What are the complications of biliary atresia and
what can be done forthem?
• Infection in the bile ducts.
This is usually treated using intravenous
• Jaundice oritching may occur.
• These can often be treated successfully with
• cholestyramine- to relive pruritus
• ursodeoxycholic acid (foritching). been shown
to enhance bile flow
• Many patients with cirrhosis have changes in
blood flow through the liverand 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
• If thereis still not enoughbileflow withtheKasai
procedure, livertransplantationis afinal option. Aliver
transplant operationremoves thedamagedliverand
replaces it withanew liverfromadonor.
• Aftertransplant surgery, thechild's healthmayimprove
quitequickly. However, thechild's bodymight reject the
new organ. To prevent rejection, astrict scheduleof anti-
rejectionmedications must betaken.
• Afteratransplant, ongoinglifelongcareis required.
Frequent contact withphysicians andothermembers of the
transplant teamis also necessary.
• stool color
•ascites, peripheral edema,
• urine color
•pruritus, vital signs, signs of dehydration, and weight