Liver Area (GB5 (

Under Supervision:

Assist Professor \ Aml

Abdelrazek
 ‫‪A.L \ Seham Nassar‬‬

‫‪Prepared by :‬‬

‫للا ‪1-‬‬‫هند عاطف محمد فتح‬

‫هند عبد العظيم ابراهيم شاهين ‪2-‬‬
‫هند عبده محمود درويش ‪3-‬‬
‫هنداويه احمد توفيق حجازى الدش ‪4-‬‬
‫هويدا محمد حامد سعد ‪5-‬‬
‫وسام سعد عبد الحميد عبد الل ‪6-‬‬

‫‪Outlines‬‬
‫❖‬ ‫‪Introduction‬‬

‫❖‬ ‫‪Definition‬‬

‫❖‬ ‫‪Pathophysiology‬‬

‫❖‬ ‫‪Causes‬‬

‫❖‬ ‫‪Risk Factors‬‬

‫❖‬ ‫‪Types‬‬
 ❖ Clinical Manifestations

❖ Complications

❖ Diagnosis

❖ Treatment

❖ Medical Management

❖ Pre and Post operative

Introduction
Biliary atresia is a gastrointestinal disorder in which
the biliary system is closed or absent. 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).

Definition
Bilary atresia:
▪ is a blockage in the tubes (ducts) that carry bile
from the liver to the gallbladder
▪ is a progressive fibrosing obstructive
cholangiopathy involving both the extrahepatic
and intrahepatic biliary system
▪ is a condition in infants in which the bile
ductstubes inside and outside the liver are scarred
and blocked so that bile can’t flow into the
 intestine, so bile builds up in the liver and damages
it. The damage leads to scarring, loss of liver tissue
and function, and cirrhosis.

The incidence rate :

The incidence of biliary atresia is approximately one in
10,000 children.

Bile formation and

excretion:
Bile is a physiological aqueous solution produced
and secreted by the liver. It consists mainly of bile salts,
phospholipids, cholesterol, conjugated bilirubin,
electrolytes, and water . Bile travels through the liver in a
series of ducts, eventually exiting through the common
hepatic duct. Bile flows through this duct into the
gallbladder, where it is concentrated and stored. When
stimulated by the hormone cholecystokinin (CCK), the
gallbladder contracts, pushing bile through the cystic duct
and into the common bile duct. Simultaneously, the
sphincter of Oddi relaxes, permitting bile to enter the
duodenal lumen. The hormone secretin also plays an
important role in the flow of bile into the small intestine.
By stimulating biliary and pancreatic ductular cells to
secrete bicarbonate and water in response to the presence
of acid in the duodenum, secretin effectively expands the
volume of bile entering the duodenum. In the small
intestine, bile acids facilitate lipid digestion and
absorption. Only approximately 5% of these bile acids are
eventually excreted. The majority of bile acids are
efficiently reabsorbed from the ileum, secreted into the
portal venous system, and returned to the liver in a process
known as enterohepatic recirculation

Pathophysiology
 Biliary atresia is a rare and fatal liver disease
.symptoms of the disease appear or develop about 2 to 8
weeks after birth. The normal cells in liver produce liquid
called bile. The color of bile is greenish yellow liquid. Bile
help to digest and break down fats through the muscle in
duodenum called spincter of oddi. Bile also carries waste
products from liver to the intestine for removal of the
body. The biliary system allow bile drain from liver to
intestine. When baby has biliary atresia, bile flow from
liver to the gall bladder is blockage. This cause bile
trapped inside the liver and don't allow storage of the bile
in gall bladder. This blockage also cause damage and
scarring liver cell (cirrhosis) and eventually liver failure.

Causes
The exact cause of biliary atresia is not known. It is
believed that biliary atresia occurs in children due to
anatomical abnormalities during fetal development. The
bile ducts are not formed properly and the flow of bile gets
blocked. Some also believe that in some children, although
the bile ducts are properly formed, they get damaged
because of the hyperactive immune system due to
conditions such as viral infection soon after birth. Biliary
atresia is not contagious and genetic conditions have no
role to play in its development. Other possible causes for
biliary atresia are: infections with certain viruses
Exposure to toxins.
• Problem with the immune system.
• Inflammation (swelling) and scarring caused by
problems with the immune system.
• Exposure to harmful chemicals
• A permanent change in a gene’s structure, called a
genetic mutation.
• Biliary atresia is not an inherited disease, meaning it
does not pass from parent to child.

Risk factors
The exact cause of biliary atresia remains unknown,
however various factors increase the risk of this condition.
Some factors that increase the risk of developing biliary
atresia are:

• Preterm birth
• Poor maternal nutrition
• Maternal smoking
• Presence of congenital malformation
• Gender: females are more prone to biliary atresia as
compared to males.

• Gestational diabetes mellitus.

Type
Age-of-onset classification

▪ Perinatal biliary atresia. This is the most common

type. It appears after birth, most often when a baby
is about 2 to 4 weeks old.

▪ Fetal biliary atresia. This is less common. It

appears while a baby is still developing in the
mother’s womb.
Morphological Classification
▪ Type I: Obliteration of the common bile duct ▪ Type
II : divided to
✔ Type II a: Obliteration of the common hepatic
duct
✔ Type II b: Obliteration of the common bile
duct, hepatic duct, cystic duct with no
abnormality of the gallbladder, and cystic
dilatation at the porta hepatis
▪ Type III: Obliteration of the common bile duct,
hepatic duct, and cystic duct with no anastomosable
ducts at the porta hepatis; this is the most common
variety.
Clinical manifestations
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.

The symptoms of biliary atresia usually appear by the

age of two to six weeks include:

• Jaundice: a yellow coloring of the skin and eyes due

to a very high level of bilirubin (bile pigment) in the
bloodstream. Jaundice caused by an immature liver is
common in newborns. It usually goes away within the
first week to 10 days of life. A baby with biliary
atresia usually appears normal at birth, but develops
jaundice at 3 to 6 weeks after birth

• Abnormally pale stools and dark urine: caused by

the buildup of bilirubin (a breakdown product from
 hemoglobin) in the blood, the bilirubin is then filtered
by the kidney and removed in the urine

• Infants may also have swollen (distended)

stomach and/or abnormal enlargement of the liver
(hepatomegaly).

By the age of six to 10 weeks, additional symptoms

may also develop including:

• Poor weight gain

• Irritability and/or an increase in blood pressure within

the veins that carry blood from the intestine to the
liver (portal hypertension).

• If left untreated, biliary atresia may result in

permanent scarring of the liver (cirrhosis) and,
eventually, liver (hepatic) failure

Some children with biliary atresia may have additional

congenital abnormalities including:

• Malformations of the heart (e.g., levocardia, and

ventricular septal defects) and/or kidneys.

• Levocardia is a condition in which the heart is

malposition.

• Absence of the spleen (asplenia), the presence of

more than one spleen (polysplenia).
Complications
Biliary atresia causes liver damage and affect many
processes that allow the body to work normally. Without
treatment, infants with biliary atresia would develop
cirrhosis within 6 months and liver failure within 1 year.
By age 2 , untreated infants would need a liver transplant
to survive.

(1) Liver cirrhosis: In which the liver breaks down and

is unable to work normally. Scar tissue replaces healthy
liver tissue ,because the bile ducts in the liver are blocked
that cause the bile build up in the liver. In early stages of
cirrhosis ,the liver continues to work ,as the cirrhosis gets
worse the liver begins to fail.

(2) Portal hypertension: (is high blood pressure on the

portal vein).

Cirrhosis is partly blocking the flow of blood through the

liver.

Portal hypertension may lead to specific complications,

including:

• Enlarged blood vessels (Varices) which can

develop in the esophagus, stomach or both.
Varices can break open and cause life-threatening
bleeding in the digestive tract .

• Build up of fluid in the abdomen (ascites).

(3) Liver failure: (end-stage liver disease). The liver can
no longer perform important functions and children with
liver failure need a liver transplant to survive.

(4) After the kasai procedure , it is common to get an

infection in the bile duct .

(5) Jaundice and itching may occur . These can often

be treated successfully with medication such as
cholestyramine and urso deoxycholic acid (for itching).
Diagnosis
To diagnose biliary atresia, the doctor will take the baby’s
medical history and perform a physical examination. A
pediatric hepatologist, a doctor who specializes in
working with children who have liver diseases, may also
order certain tests, including:

• Blood tests to check the levels of bilirubin and to

look for signs of liver damage

• Ultrasound, a procedure that transmits

highfrequency sound waves through body tissues.
The echoes are recorded and transformed into images
of the internal structures of the body.

• Liver biopsy (the removal of cells or tissue from the

liver for examination)

• Hepatobiliary scan, in which a radioactive

substance (tracer) is injected into the bloodstream. If
the tracer

is not excreted through the liver, the patient may have

biliary atresia.
Surgical treatment
There is no cure for biliary atresia. The main treatment is
a surgery called the Kasai Procedure ."Roux-en-Y" or a
"hepatoportoenterostomy".
- the Kasai procedure is done at an early age (younger
than three months).
- The surgeon may be able to operate through many
small incisions (laparoscopic surgery) instead of one large
(open surgery) incision. The surgeon will determine the
safest method of operating and will discuss this with you
before the procedure takes place. The operation will take
approximately 4 hours to complete.
- To perform the Kasai procedure, surgeons first
carefully remove the damaged ducts outside of the liver.
They use a small segment of the patient's own intestine to
replace the ducts at the spot where bile is expected to
drain. This segment not only connects to the liver, but also
connects to the rest of the intestine. The Y-shaped
passageway formed by the Kasai operation allows bile to
flow from the liver into the intestine.
- Normal restoration of bile flow and recovery of liver
function occurs in approximately one third of children
who undergo the Kasai procedure. These children may not
require liver transplantation. The remaining two thirds of
children who undergo the Kasai procedure will not have
adequate bile flow and liver function, and will eventually
require liver transplantation. Of this group, half will need
transplantation soon after the Kasai procedure and half
will need transplantation at a later time
Age at surgery:

Many studies have shown an improved outcome in

terms of clearance of jaundice

And native liver survival, the earlier the

portoenterostomy is performed. This has

Stimulated a public and professional campaign to

encourage early referral for biliary

Atresia and highlighted the necessity for

screening.6,7,10 Clearly the age at operation

Is an important factor and may be a for the extent of

liver damage.
*Histology:

It has been suggested that the extent of histological

abnormality at operation17 or

The morphology of the extra hepatic biliary

remnant influences both short

And long term outcome, but this has not been

found consistently.

*Surgical expertise:

By far the most important variable is the experience

of the surgeon and the surgical center.

*State of liver

➢ The degree of injury

➢ Children with cirrhosis at the time of diagnosis

should be evaluated for primary

Liver transplantation.

:-Note

If the Kasai procedure is unsuccessful, the infant

will usually require a liver

Transplant, sometimes between ages 1 and 2. Of all

infants who have had a
Kasai procedure, fully half still require liver
transplantation before age 5.

Liver transplantation is a highly successful

treatment for biliary atresia.

Survival after surgery has increased dramatically in

recent years. Children

With biliary atresia are now surviving well into

adulthood.

Medical Management
Medications and vitamins support liver function and boost
nutrition after surgery for biliary atresia.

1- Vitamins: Children with biliary atresia may have

difficulty absorbing vitamins A, D, E, and K, even after
surgery. Our doctors prescribe vitamin supplements to
replace these essential nutrients.
2- Steroids : are often given after surgery to prevent or
reduce inflammation in the liver and improve bile flow.
3- Bile Salts: bile salts are substances naturally found in
bile that help the body to digest fats, absorb fat-soluble
vitamins, and flush waste from the liver.

4-Brood-spectrum antibiotics: which are effective

against a wide range of bacteria, may be used to prevent
cholangitis and serious infection after surgery.
after surgery for biliary atresia. Some children may
eventually outgrow the need for these medications. Others
may need to take them throughout their lives.

Nursing Management

Pre operative:
Investigations like clotting profile and chest ray as
clinically indicated

Nutritional management

Children with liver disease have more metabolic needs

than healthy children. This means that children with
biliary atresia may need more calories.

A child with biliary atresia and jaundice 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.

• For formula-fed infants, consideration will be given

to switching to a feed with medium chain triglyceride
as the predominant lipid source eg PeptiJunior

• For breast-fed infants, consideration will be given to

introducing some supplemental formula feeds using
 a medium chain triglyceride based feed eg
PeptiJunior while allowing the baby to continue some
breast feeding. Another option would be to add MCT
to expressed breast milk.

Vitamin supplementation

• Children with BA are almost always deficient in

fatsoluble vitamins A, D, E and K

• Vitamin doses should be titrated according to growth

and vitamin levels

Parental education and support

• All families whose babies are suspected of having

BA will have access to a Clinical Nurse Specialist
(CNS) who can provide education about BA and liver
disease

• Families should be offered a referral to a social

worker and the Consult Liaison Team for
psychological supply

Intra operative:

• ➢ Anesthetic considerations
• Percutaneous central line will usually be placed. As
far as possible, the right sided .
• Neck veins should be avoided as these may be
required during subsequent liver
Transplantation.
• Urinary catheterization will be required
Consideration will be given

To epidural analgesia

• ➢ Intravenous fluids
• Intravenous fluid management should be prescribed
following the Starship

Intravenous Fluids.

Post operative
The aim of treatment after surgery is to encourage normal
growth and development. If bile flow is good, the child is
given a regular diet. If tests show that bile flow is reduced,
a low-fat diet and vitamin supplements will be required,
since the absorption of fats and vitamins is impaired.

Guidelines from the doctor for child's nutrition may

include:
 • Talking medicines to prevent the body’s immune
system from attacking, or rejecting, the new
liver(antibiotics).

• A well-balanced diet, consisting of three meals a day

plus small snacks in between meals
• Vitamin supplements (Specifically Vitamins A, D,
E, and K as these are absorbed in fat, and children
with biliary atresia cannot absorb these well)
• 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 a special tube (nasogastric tube) that is
placed in the nose and guided down the esophagus
and into the stomach.

Nursing diagnosis:

1- Hyperthermia related To Inflammatory Damage due to

progressive Extrahepatic biliary duct .

2-Ineffective breathing pattern related to an increase in

abdominal distension.

3-Imbalanced Nutrition Less Than Body

Requirements related to anorexia and Impaired
absorption of fat .

4-Impaired bowel elimination (diarrhea) related to

intestinal malabsorption

4- Skin integrity related To accumulation of bile salts in

the network.

5- Deficient fluid volume related to nausea and vomiting

Reference
Haber BA, Erlichman J, Loomes KM. Recent
advances in biliary atresia: prospects for novel
therapies. Expert Opin Investig Drugs. 2008 Dec.
17(12):1911-24. [Medline].
Fischler B, Ehrnst A, Forsgren M, et al. The viral
association of neonatal cholestasis in Sweden: a
possible link between cytomegalovirus infection
and extrahepatic biliary atresia. J Pediatr
Gastroenterol Nutr. 1998 Jul. 27(1):57-
64. [Medline].
 Chang MH, Huang HH, Huang ES, et al.
Polymerase chain reaction to detect human
cytomegalovirus in livers of infants with neonatal
hepatitis. Gastroenterology. 1992 Sep.
103(3):1022-5. [Medline].
Wilson GA, Morrison LA, Fields BN. Association
of the reovirus S1 gene with serotype 3-induced
biliary atresia in mice. J Virol. 1994 Oct.
68(10):6458-65. [Medline]. [Full Text].
Steele MI, Marshall CM, Lloyd RE, Randolph VE.
Reovirus 3 not detected by reverse
transcriptasemediated polymerase chain reaction
analysis of preserved tissue from infants with
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21(3):697702. [Medline].