Mrs lulu Fasna k k2 nd yr msc nursingMIMS CON

• Congenital absence or closure of a normal body opening or tubular structure.

Definition

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.

Incidence• Biliary atresia affects approximately 1 in

10,000-15,000 births• Biliary atresia seems to affect girls more than

boys. • 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 BILE DUCT TypesThe 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,

midline liverpreduodenal portal vein,

polysplenia, 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 jaundice-free interval

Classification: Three main types of biliary atresia are surgicaly defined: 1 type I, the common bile duct is obliterated while the proximal bile ducts are

patent.

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

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 >90% of

cases.

• Infectious agentReovirus

rotavirus retrovirus

cytomegalovirus Human papilloma virus

, other agent

• AUTOIMMUNE MECHANISM• There is clearly a predominance of lymphocytes in

BA liver tissue. • 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 liver diseases

• It has been proposed that the embryonic form of BA is caused by defective development of the biliary tree.

• The association of anomalies of visceral organ symmetry with BA (the polysplenia or BA splenic malformation syndrome)

• genes that control normal situs development are also key regulators of normal extrahepatic bile duct development.

• Abnormal remodeling of the ductal plate leads to the ductal plate malformation that is present in congenital hepatic fibrosis and other bile duct dysplasias

• Vascular lesion/arteriopathy• Inherited mutations• polysplenia • asplenia syndromes • Other genes• Somatic mutations• Toxin exposure

• 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 liver.

• 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 to cirrhosis

• As the liver becomes scarred, it presses against the walls of the veins. This constricts the veins and blood cannot pass through them properly. The result is portal hypertension (high blood pressure in the portal vein).

• This congenital disorder begins to progress very soon after birth. In its most common form, extrahepatic biliary atresia, ducts outside the liver are affected first.

• Jaundiced infants with

• Acholic stool

• Dark urine

• Enlarged firm liver

• Late manifestations

• Liver Cirrhosis

• Varices from Portal Hypertension

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 bloodstream.

-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.

• Small proportion of children will present with vit k dependent coagulopathy and bleeding.

• Features of cirrhosisoAscites o SplenomegalyoHard 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 intestine.

• liver biopsy.

Investigations continue----Cholangiography.This is done by injecting contrast material

through the gallbladder. To find the communication between the

biliary tree and the gastrointestinal tract,

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,

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).

HOW IS BILIARY ATRESIA TREATED?

-The Kasai procedure is an operation to create an open duct so bile can drain from the liver.

-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.

• 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.

Guidelines for nutrition for a child with BA

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.

What are the complications of biliary atresia and what can be done for them?

• 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- to relive pruritus

• ursodeoxycholic acid (for itching). been shown to enhance bile flow

• .

• 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

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.

Monitor• stool color•ascites, peripheral edema,• hepato/splenomegaly• anorexia• urine color•lethargy•jaundice •bleeding •pruritus, vital signs, signs of dehydration, and weight