PATIENT INFORMATION ABOUT BILIARY ATRESIA
Dr.
Rajeev Redkar
MS, MCh (Peds), FRCS(Peds), FRCS(Ed), FRCS(G), FCPS, DNB, IAS
Consultant Pediatric Surgeon
Lilavati Hospital, Mumbai Shushrusha Hospital, Mumbai
This
information is designed to give a general overview of a rare condition
termed as "Biliary Atresia" which affects infants.
Before
reading this, it may be helpful to read about the liver and how it works
from any medical physiology book. One important function of the liver
is to make a substance called bile. Bile is a liquid,
which passes into the gut through small tubes called bile ducts. Bile
is necessary for the proper digestion of fat within the diet.
What
is Biliary Atresia?
Biliary
atresia is a condition in which inflammation develops within the bile
ducts around the time of birth. The inflammation can occur in any of the
bile ducts both inside and outside of the liver. This leads to bile duct
damage and reduces the flow of bile which subsequently causes scarring
(fibrosis or cirrhosis) of the liver. The bile ducts outside the liver
are always irreversibly damaged preventing any bile flow into gut. The
gall bladder is also affected.
What
causes biliary atresia?
Unfortunately
the cause of biliary atresia is not known. Research is being carried out
but it has not yet provided any definitive answers. There are various
theories attributing some viruses or autoimmune processes to cause the
pathology. Biliary atresia, however is NOT a hereditary condition.
Parents
frequently feel guilty that they may in some way have contributed to their
child having this problem. These feelings are groundless, as at present,
there is no known way of preventing or anticipating the occurrence of
biliary atresia.
What
are the signs of biliary atresia?
In the
first few weeks of life, babies with biliary atresia often seen well,
apart from being jaundiced (yellowing of the skin and whites of the eyes)
Jaundice
in newborn babies is very common and mostly it clears within the first
2 weeks of life. This is caused due to the change over of the newborn
baby's blood (hemoglobin) to the variety that is present in adults. However,
if the cause of the jaundice is due to a Liver Disease, then, frequently
the jaundice does not fade.
Other
important signs are:
Normally
the urine of a newborn baby is colourless. If the baby’s urine
is persistently yellow or brown this can be an indication of Liver
Disease.
Normally
the stools of a baby are green or yellow. If the stools are grey,
white, fawn or pale then this can be an indication of Liver Disease.
Prolonged
bleeding from the umbilicus or bleeding elsewhere (e.g. a nosebleed) may
be an indication of Liver Disease.
How
is biliary atresia diagnosed?
The signs
that have been described are non-specific and can indicate other disorders,
therefore there will be a need to number of investigations in order to
make a diagnosis. The tests are to be carried out in hospital and include
blood tests, scans and liver biopsy. Your baby will be admitted to hospital
for a period of about a week for the tests.
Once
other causes of Liver Disease have been excluded and investigations indicate
biliary atresia your baby will be scheduled for surgery. The doctors and
nurses will explain to you the type of surgery planned. The diagnosis
and type of biliary atresia will only be confirmed at the time of operation.
How
can biliary atresia be treated?
The initial
treatment for biliary atresia is a surgical operation called Kasai procedure
after the Japanese Surgeon who pioneered the operation. The aim of the
Kasai procedure is to allow bile to drain from the liver into the gut.
This is a major operation involving removal of the affected bile ducts
that are outside the liver, and making an alternative system of drainage
of bile from the liver into the intestines by making a "bile duct
like tube" by restructuring the small intestines.
Babies
with prolonged jaundice and suspected of having a Liver Disease such as
biliary atresia, will often need special feeds. The hospital Dietitian
will advise. There are a number of special milks available for babies
suffering from Liver Disease. These milks have fats, which are more easily
digested by babies with Liver Disease. Extra calories may also be added
to the feeds in the form of powders to help your baby maintain an adequate
weight gain. Most babies with biliary atresia are unable to gain weight
satisfactorily on breast milk alone. In these cases the Dietitian will
advise how to combine breast and bottle-feeding.
The
doctor may prescribe extra vitamins as babies with prolonged jaundice
also have problems absorbing certain vitamins.
What
happens before the operation?
When
any operation is required some preparation is necessary. Blood tests will
be done so that your baby’s blood group can be identified in case
there is a need to give blood to your baby before, during or after surgery.
Your baby may require special medicines to prepare the gut for surgery.
Your
baby may also require some feeds to be replaced by special non-milk feeds
and/or some fluids to be given into the vein by means of drip. The nurses
on the ward will tell you what you should give and when
What
happens during the operation?
The aim
of the operation is to make a drainage channel to allow bile to drain
from the liver. The exact way in which this is done will depend on the
type of biliary atresia your baby has. During the operation the Surgeon
will examine the liver, look for abnormalities and may take a liver biopsy
(i.e. remove a tiny piece for further laboratory tests).
The
operation usually takes usually takes a whole morning or afternoon. The
surgeon takes as long as is needed to carry out what he / she needs to
do.
There
are 2 types of operations.
- The
surgeon finds no patent (open) ducts outside the liver.
This
is the most common finding noted in 85% of cases of biliary atresia.
The ducts are blocked (or missing altogether) right up to the liver
surface. Under this circumstance, the blocked ducts are removed completely
up to the liver surface. The surgeon cuts the bowel and also cuts into
the under surface of the liver to find patent (open) bile ducts, i.e.
microscopic bile ductules, which can drain bile effectively. The loop
of bowel is then joined to this area of the liver.
- The
surgeon finds patent bile ducts immediately below the liver containing
bile.
In
this type, the surgeon can join the loop of the bowel to the remaining
bile ducts.
The
surgeon will perform the operation, which is best for your baby. Experience
has shown no noticeable difference in long-term success rates between
the two operations.
What
happens immediately after the Kasai operation?
Your
baby will return to the ward, usually with Oxygen hood box over the upper
part of your baby’s body and head. This enables the nurse to control
the amount of oxygen and water vapour in the air your baby breathes. This
allows your baby to breathe more easily and to prevent chest infections
Sometimes
the babies have to be kept in Pediatric Intensive Care Unit for a few
days before shifting them to the ward.
There
will be a tube running up your baby’s nose and down into the stomach,
this is called a naso-gastric tube. This usually drains into a clear bag
or small pot and keeps the stomach empty which helps to prevent vomiting.
There
will also be an intravenous infusion or "drip" into a vein usually
in the back of the baby’s hand.
This
is used to give:
- Fluids,
which are given in order to maintain the correct sugar, salt and water
level in the body. Daily blood tests will be carried out in order to
check these levels so that they can be adjusted accordingly. All fluid
required by your baby will be given through the drip until feeding is
re-established.
- Pain relieving
drugs are often given through the drip or as suppositories.
- Antibiotics
will be given immediately after the operation and for at least 48 hours.
If your baby does not develop a high temperature and once oral feeds
are started these will be often be given orally rather than through
the drip.
A
dressing will cover the wound on your baby’s abdomen. A thin,
soft rubber drain may have been inserted below the stitch line during
the operation and this stays in for a few days to allow excess fluid
to drain away- a bag may be put over this to measure the amount of
fluid lost. The dressing will be changed as necessary and the stitch(es)
removed, if necessary, when the wound has healed – usually about
2 weeks after the operation.
By
the following day it may be possible for you to hold your baby, the
nurse will help you.
The
Doctors will listen to the tummy with a stethoscope for sound of the
bowel starting to work normally again after the operation. Once this
happens, usually about the third day the baby’s feeds will be
restarted with frequent small amounts of sugar water (dextrose) which
are slowly increased. When these are tolerated, diluted milk feeds
will be given and the strength of these will be slowly increased until
full strength feeds are resumed. If your baby was being breast fed
before diagnosis at least a partial return to this method may be possible.
It is important that the milk supply has been maintained by "
expressing" the milk while your baby is unable to feed. The ward
staff will help and advise you this.
When
will my baby be able to leave hospital and what happens next?
Providing
that there are no complications following surgery, your baby will be home
within two weeks of the operation. Before returning home the wound must
be healed and your baby must have a normal temperature and have started
to put on weight. You will be given an outpatient appointment.
Before
your baby leaves hospital, you will be taught all about the special medicines
and feeds. It is quite common for the hospital staff to advise your General
Practitioner (GP) and local hospital about your child’s condition
and any special aspects of care, which are needed. They can offer you
useful support when you are at home.
When
you get home you must try and treat your baby as normally as possible.
Obviously you will be worried at times and this is quite normal. Babies
with biliary atresia get common illness just like any normal baby. However,
if your baby has a high temperature for more than 24 hours or becomes
jaundiced, or if there is an obvious change in colour of the urine and
stools, or your are worried, it is important for your to see your doctor
as soon as possible.
Unless
there is some problem other than biliary atresia it is very important
that your baby is immunized against Diphtheria, Whooping Cough, Tetanus,
polio, Hib and MMR in the normal way. It does not matter if the immumisations
are a little late starting because of the operation and hospital stay.
If
you or your local doctor have any queries regarding your child, please
contact the Consultant or Ward Sister at the hospital treating your child
What
medicines may my baby need and why are they given?
The medicines
needed will depend upon your child’s condition and progress but some
commonly used are:
- Antibiotics
– After the operation a course of antibiotics is initially given
by "drip" and completed by mouth. They are used to reduce
the risk of infection spreading from the gut into the liver. Further
courses will be prescribed as required.
- Vitamins
– Additional vitamins are necessary because poor bile flow can
reduce absorption of vitamins. Some may need to be given by injection.
- Phenobarbitone
– This may be given to increase the flow of bile. As it may make
your baby sleepy at first it is usually given in one dose a day, in
the evening.
- Cholestyramine
– This comes in the form of a powder in individual sachets. Each
sachet is mixed with water and given in liquid form. This may be given
to improve liver function and remove substances which may otherwise
cause the skin to itch. Cholestyramine can affect the absorption of
some drugs, especially vitamins. Therefore, with babies it is best
to give some at each feed time except when vitamins are given. As
your baby becomes older and manages larger volumes the daily dose
of Cholestyramine can be given all at once or split into two. For
a toddler or an older child needing Cholestyramine, it is best given
in one or two doses away from the main meal times and vitamins. Always
leave two hours between giving Cholestyramine and vitamins.
- Sprironolactone
– This may need to be given to help your baby get rid excess
fluid which otherwise collects in the abdomen and is called ascites.
It is not needed in all cases.
- URSO
– This drug may be given at the onset of feeding after Kasai.
It is thought to help promote the flow of bile.
- Ranitidine
– It is common to find stomach irritation after any type of abdominal
operation and this drug may be given to help alleviate this problem
If
your baby/child needs medicine to reduce a temperature, remember
aspirin, and drugs containing aspirin, must not be given; it is
safer to sponge your baby with lukewarm water but if this does not
work use paracetamol. Ibuprofen should not be given
How
is it possible to tell if the operation has been successful?
At the time
of operation the surgeon will not to be able to tell if bile drainage
will occur satisfactorily. The first stools after the operation are usually
very dark, they then will become paler. If bile drainage does occur the
stools gradually change to a yellow / green / brown colour and later the
urine becomes paler.
The
colour of the stools will be monitored whilst your baby in is hospital.
It is not unusual for there to be some variation in the colour of the
stools. It is impossible to say how long it may take to observe these
change as every baby is different.
If
bile drainage is established the jaundice gradually fades and blood tests
show that the chemical, bilirubin that causes jaundice, drops towards
a normal level. Since the operation may not drain bile from all section
of the liver it is not unusual for liver function tests to remain abnormal
even if the jaundice completely clears.
Satisfactory
bile drainage occur in approximately 80% of children who undergo the Kasai
operation before the age of eight weeks. The incidence of successful bile
drainage is lower if the baby is over 8 weeks old. However, even some
of the children who lose their jaundice may develop some of the complications
due to liver damage. So all children continue to seen for regular checkups.
Professor
Kasai who first performed this operation in Japan in the 1950’s has
found that 90% of patients who lose their jaundice are alive and well
at almost 15 years of age. Since the operation is a relatively new one,
the longer-term prognosis is, as yet, less certain.
What
happens if the biliary atresia is not treated or treatment is unsuccessful?
In
these cases bile produced in the liver builds up causing damage to the
cells of the liver eventually resulting in severe scarring (cirrhosis).
If this continuous liver function deteriorates, complications occur and
general health suffers. In the past sadly these children often died in
infancy, but liver transplantation has altered this outlook and is now
an accepted form of treatment. It is only considered when other treatments
are ineffective.
Liver
transplantation became available for children in the 1980’s, the
results are now encouraging and are steadily improving. There is an overall
80% one-year survival rate for liver transplantation. Liver transplantation
is now possible in children of any age.
If
liver transplantation is indicated for your child this will be discussed
with you by the team caring for you and your child. Diet and medication
will be intensified in order to ensure that your baby or child is as well
as possible leading up to transplant, and progress will be monitored.
The aim of liver transplantation is to restore your child to good health.
What
other problems might occur?
A
number of problems may occur of which you should be aware:
This
is an infection of the bile ducts in the liver resulting in inflammation.
This
may cause a fever or jaundice or both. It requires urgent treatment
with intravenous antibiotics which can only be given at your local
hospital or the Unit treating your child. If the treatment is given
locally, please ask the doctors to contact the Liver Unit treating
your child, if you attend one.
If
cholangitis is left untreated it can cause further damage to liver.
That is why prompt investigation of any high temperature (over 370
C) or a non-specific illness lasting for than 24 hours is important.
This
is pronounced Ay – sigh – tees and is an abnormal collection
of fluid in the abdomen. This can be present for up to 6 weeks after
the operation but should then disappear. If it recurs at any time
this is usually a sign that the disease has affected the ability of
the liver to make a protein called albumin.
One
of the blood tests performed frequently after the operation and each
time blood is taken in outpatients is to measure the albumin level.
When the albumin level is too low, water from child’s blood "leaks"
out of the blood vessels and collects in the abdomen (in severe cases
it may also collect in other parts of body).
The
first sign of this problem is often when clothes do not fit so well
over the tummy. This usually occurs gradually but may be started or
worse by infection.
The
doctor may measure your child’s abdominal girth at each hospital
visit and/or may ask you to do this at home. If your child’s
tummy becomes noticeably bigger while at home you should see your
GP to exclude other causes such as wind or constipation for which
he/she can advise you. Treatment for ascites may also be started by
your GP or your local pediatrician in consultation with the specialist
unit and does not necessarily mean admission to hospital.
Treatment
is initially by medicine and/or altering the diet to maintain calorie
in take but reduce fluid and salt intake. In more serious cases it
may be necessary to admit your child to hospital and give albumin
intravenously through a "drip". You should never attempt
to alter your child’s diet and, in particular, their fluid intake
without medical advice.
Portal
hypertension means high blood pressure in the portal vein, the main
vein carrying blood from the gut to the liver and is different from
high blood pressure. This may occur due to scarring in the liver,
which causes backpressure to the portal vein. This may cause veins
like varicose veins to develop in the lining of the gut, stomach or
gullet.
These
veins become fragile and bleed. It is rare for this to occur before
the child is two years old. Bleeding can cause black stools, pallor
or vomiting of blood. If bleeding does occur, then contact your local
hospital immediately. Treatment is available.
NEVER
GIVE YOUR CHILD ASPIRIN OR IBUPROFEN OR ANY MEDICINES THAT CONTAINS ASPIRIN
AS THIS MAY START THE ONSET OF BLEEDING.
Some
children with reduced bile flow experience itching of the skin.
This can develop at any time and can make a child quite irritable
and miserable. If you think your child may be affected consult your
GP or specialist unit additional medicines may be required.
What
will happen in the future?
Your
baby / child’s growth and development will be followed up by periodic
visits to the hospital. The frequency will vary with each child. Blood
tests will be performed to measure the bilirubin level and the liver function.
Periodically an ultrasound may be performed to assess the size of the
liver and other structures in the abdomen (e.g. the spleen).
It
may be that at some point in the future a short admission to hospital
may have to be arranged, no matter how well your child is doing to generally
assess progress.
Last created on 21-02-2001
Last updated on 01-07-2006
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