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Biliary Atresia
a guide
Medical Information Series
Welcome
This leaflet has been written for:
Parents/carers of a child with biliary atresia Young people diagnosed with biliary atresia (as a baby) Relatives and friends of families Healthcare and allied professionals, school, college, university and nursery teams Explain biliary atresia Explain the symptoms and diagnosis Discuss treatment and possible complications Conjugated jaundice in babies Guide to the liver Glossary of terms Routine investigations for liverdisease
Childrens Liver Disease Foundation (CLDF) also has leaflets in its support series which are available to download from our website childliverdisease.org. Leaflets can be mailed to UK patients free of charge, our contact details are on this leaflet. You may find it helpful to have a copy of CLDFs making the most of an appointment leaflet which will help you prepare for appointments and meetings following discharge. If you have been given this leaflet at a UK hospital you should have received a CLDF Introduction Pack, supplied, free of charge, to UK hospitals. We are happy to send you an Introduction Pack as well as a CLDF Pack if you live in the UK. Our contact details are on this leaflet.
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All are available on request to UK families and young people. Overseas families should contact CLDF to discuss their literature needs.
Introduction
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.
Showing the normal liver, gall bladder, bile ducts and upper guts.
Showing the same structures in a child with biliary atresia. The darkened areas represent the damaged bile ducts.
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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.
Yellow coloured urine Normally the urine of a newborn baby is colourless. If a babys urine is persistently yellow this can be an indication of liver disease. Pale stools Normally the stools of a baby are green or yellow. If the stools of a baby are grey, white, fawn or pale then this can be an indication of liver disease. Bleeding Prolonged bleeding from the umbilicus or bleeding elsewhere (e.g. a nosebleed) may be an indication of liver disease
A spleen which is in multiple pieces (polysplenia) or absent (asplenia). The spleen is an organ which sits under the left lower ribs next to the stomach and helps to maintain immunity against infection and removes blood cells, especially platelets at the end of their life. It is normally a solid single organ. An abnormal arrangement of intestine, known as intestinal malrotation. Some abnormalities of the veins in the abdomen. Heart defects.
The signs that have been described are non-specific and can indicate other disorders. Therefore, there will need to be a number of investigations in order to make a diagnosis. The tests need 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.
The presence of some or all of these features make up a condition known as biliary atresia splenic malformation syndrome. This subgroup of children with biliary atresia may be less likely to clear their jaundice after a Kasai operation. They also need additional tests such as a heart scan to check for problems. They are usually advised to have immunisations and take daily antibiotics to reduce their risk of infection from poor splenic function.
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If you wish, you will normally be allowed to go down to the operating theatre with your baby and stay until he/she is asleep.
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The surgeon will carry out the following operation, called the Kasai procedure (Kasai portoenterostomy).
Diagram 4
The surgeon will carry out the following operation called a Hepaticojejunostomy.
Diagram 6
All the abnormal bile ducts outside the liver are removed completely. At the highest point there are usually enough microscopic bile ducts which, if drained, will allow some bile flow to be re-established. The surgeon then joins a loop of intestine to the under surface of the liver.
The blocked bile duct is removed. This leaves a single open major bile duct that drains the liver. This is then joined to a loop of intestine. Usually there is a better restoration of bile flow from the operation and jaundice tends to resolve quicker.
Type B
The surgeon finds that there is a blockage in the bile duct outside the liver. See diagram 5.
Diagram 5
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Fluids These are given in order to maintain the correct sugar, salt and water levels in your babys body. Daily blood tests will be done to check these levels so they can be adjusted as needed. All fluid needed by your baby will be given through the drip until feeding is restarted. Pain relieving drugs These are often given continuously through the drip. A special pump may control the rate of this. Sometimes a baby will receive pain relief via an epidural catheter a fine tube inserted into the back at the time of operation and similar to that used in some women in labour. Pain relief will be discussed with you. Antibiotics These will be given immediately after the operation for at least 48 hours. If your baby does not develop a high temperature and once oral feeds are started, these will often be given orally rather than through the drip.
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 has been maintained by expressing the milk while your baby is unable to feed. The ward staff will help and advise you with 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 or sent an outpatient appointment. Before your baby leaves hospital you will be taught all about the special medicines and feeds. You will be given an initial supply and you can arrange more through your GP. It is quite common for the hospital staff to advise your health visitor, GP and local hospital about your childs condition and any special aspects of care which are needed. They can often offer you useful support when you are at home. When you get home you need to 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 illnesses, just like any normal baby. Symptoms for which you should seek advice from your GP or hospital are:
A dressing will cover the wound on your babys tummy for the first few days. The dressing will be changed as necessary. The stitch(es) do not need to be removed as they dissolve after the wound has healed. By the following day it may be possible for you to hold your baby, the nurse will help you. If your baby is upset a dummy often helps. The doctor will listen to the tummy with a stethoscope for the sounds of the bowel starting to work normally again after the operation. Once this happens, usually about the third day, the babys feeds will be restarted gradually.
Your baby is obviously unwell Raised temperature for more than 24 hours More jaundice accompanied by paler stools and darker urine
Biliary Atresia a guide
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Unless there is some problem other than biliary atresia it is very important that your baby is immunised against Diphtheria, Whooping Cough, Tetanus, Polio, Hib and MMR in the normal way. It may be advised that your baby has Prevanar a new anti-pneumococcal vaccine, especially in the BASM patient. It does not matter if the immunisations are a little late starting because of the operation and hospital stay. You will be advised that your baby should receive vaccination against hepatitis A and B. If you have any queries regarding your child, please do not hesitate to contact your Specialist Nurse at the liver unit treating your child. If you would just like a chat and some reassurance the staff are always at the end of a phone, and like to hear how you are getting on. Childrens Liver Disease Foundation provides family support and will be pleased to hear from you. The contact details for CLDF are in this leaflet and the numbers for the hospital will be given to you before you leave.
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. Questran 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. Questran 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 Questran can be given all at once or split into two. For a toddler or older child needing Questran it is best given in one or two doses away from the main meal times and vitamins. Always leave at least two hours between giving Questran and vitamins.
What medicines may my baby need and why are they given?
The medicines needed will depend upon your childs 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 (cholangitis). Further courses will be prescribed as needed.
Spironolactone This may need to be given to help your baby get rid of excess fluid which otherwise collects in the abdomen and is called ascites. It is not needed in all cases. URSO (Ursodeoxycholic acid) This drug may be given at the onset of feeding after the Kasai. It is thought to help promote the flow of bile.
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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. Use paracetamol (panadol/calpol). Ibuprofen should not be given.
baby is less than eight weeks old at the time of the operation. 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 be seen for regular check-ups. More than 90% of patients with biliary atresia can be expected to survive these days. Of those that clear their jaundice some will achieve completely normal liver function and are unlikely to experience further problems. Children who clear their jaundice but who have persistently abnormal liver function tests may develop treatable problems such as portal hypertension or deteriorating liver function eventually requiring liver transplantation. Most children who undergo successful Kasai portoenterostomy will be well with their own liver ten years later. Results in the UK are equally encouraging although the operation has only been performed here since 1973.
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Liver transplantation became available for children in the 1980s, the results are now encouraging and are steadily improving. There is an overall 90% 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.
If cholangitis is left untreated it can cause further damage to the liver. That is why prompt investigation of any high temperature (over 37C) or a nonspecific illness lasting for more than 24hours is important. Cholangitis is most likely in the first year or two after the Kasai procedure.
Ascites This is pronounced Ay-sigh-tees and is an abnormal collection of fluid in the abdomen. CLDF produce a leaflet on ascites and it can be obtained on request. A small amount of ascites can be present for up to six 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 frequently needed 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 the childs blood leaks out of the blood vessels and collects in the abdomen. In severe cases it may also collect in other parts of the body. The first sign of this problem is often when clothes and nappies do not fit so well over the tummy. This usually occurs gradually but may be started or made worse by infection.
Cholangitis This is an infection if the bile ducts in the liver resulting in inflammation. This may cause a fever or worsening or recurrent jaundice or both. It needs urgent treatment with intravenous antibiotics which can only be given at your local hospital or the liver unit treating your child. If the treatment is given locally, please ask the doctors to contact the liver unit treating your child.
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The doctor may measure your childs girth at each hospital visit and/or may ask you to do this at home. If your childs tummy becomes noticeably bigger while at home you should see the 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 local Paediatrician in consultation with the liver unit and does not necessarily mean admission to hospital. Treatment is initially by medicine and/ or altering the diet to maintain calorie intake 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 childs diet and, in particular, their fluid intake without medical advice.
Never give your child aspirin or ibuprofen or any medicines that contain aspirin as this may trigger the onset of bleeding.
Itching (Pruritus) Some children with reduced bile flow experience itching of the skin. This can develop at any time and can make your child quite irritable and miserable. If you think your child may be affected consult your GP or liver unit. Additional medicine may be required. A leaflet on itching (pruritus) is available from the Childrens Liver Disease Foundation.
Portal Hypertension 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. It may occur due to scarring in the liver which causes back pressure in the portal vein. This may cause veins like varicose veins to develop in the lining of the gullet, stomach or gut. These veins become fragile and bleed. It is rare for this to occur before the child is 2 years old. Bleeding can cause black stools, marked pallor or vomiting of blood. If bleeding does occur, then contact your local hospital immediately. Treatment is available. Childrens Liver Disease Foundation has a separate leaflet on portal hypertension.
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Our parents say . . . . . . We dont know how we would have coped without CLDFs care and support. They have been just fantastic from the outset tremendous people, who are compassionate and so positive. They really care about families and children struggling with liver disease. When Emily was very ill we felt we were on the sidelines, knowing we couldnt influence the outcome and not in control. Getting involved in fundraising is something you can control and achieve a positive result. I really took comfort from that. Our young people say . . . Knowing CLDF is there is what I need. Ican call whenever I want. Whatever I think and feel is listened to. Even when I called to tell them it was my birthday! I feel really well. Its great that CLDF has given us the chance to meet other young people outside of the hospital and have a fun time. I want them to do more things likethis. Families Team fso@childliverdisease.org 0121 212 6008 Young Peoples Team ypsupport@childliverdisease.org 0121 212 6009
What are the roles of CLDFs Family and Young Peoples teams?
CLDFs Family and Young Peoples teams are here for you, whether you want to talk about issues affecting you, meet and share with others or just belong to a group which cares, knows what its like and is fighting to make a difference. You are not alone.
Childrens Liver Disease Foundation: 2002 Updated: November 2005; May 2006; February, September 2008; July 2010; April 2012
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Childrens Liver Disease Foundation is the UKs leading organisation dedicated to fighting all liver diseases of childhood.
It provides free of charge:
A huge selection of literature and online animations on the working of the liver available in print and online Information packs for a wide range of audiences, including young people, parents/carers, GP practices, schools and nurseries, friends and relatives Families and young peoples teams providing services in person, online, facebook, text and phone Developing services for adults diagnosed with a liver disease in childhood Website childliverdisease.org Young peoples website cldf-focus.org National event programme for families and young people to meet, share and have fun Secure online message board childliverdisease.org/forum
Around 75% of CLDFs annual income is derived from voluntary donations. Please help us to continue to support young people, families and adults diagnosed in childhood by making a donation. You can do this online or by completing the donation form in this leaflet. Even better, a regular direct debit gift will enable us to plan our work more fully. Thank you. Childrens Liver Disease Foundation 36 Great Charles Street Birmingham B3 3JY 0121 212 3839 info@childliverdisease.org
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