Kasai procedure (Hepatoportoenterostomy)

Kasai procedure or hepatoportoenterostomy is a surgical procedure for the treatment of biliary atresia. Biliary atresia generally occurs in a foetus (unborn baby) or immediately after the baby is born. In this condition, bile ducts are either missing, damaged, or misshaped. Bile ducts are tubular structures, carrying the bile from the liver to the gallbladder and then to the intestine. Bile is a yellow-green liquid made by the liver. It is essential for digestion, and helps in eliminating the waste from the liver to the outside of the body. Blockage in the bile duct can cause the bile to collect in the liver, resulting in liver damage.

Hence, in case of biliary atresia, the Kasai procedure should be done at the earliest. The Kasai procedure is not a cure, and will not be successful if the problem is in the bile duct inside the liver. However, without the surgery, the child will not be able to live for more than 18 to 24 months. He/she may need a liver transplant later even after the surgery. 

A doctor will recommend the Kasai procedure if your infant shows symptoms of biliary atresia and its presence is confirmed. 

Symptoms of biliary atresia are as follows:

• Jaundice
• Acholic stools
• Dark urine
• Large spleen or enlarged liver
• Poor growth
• Low oxygen levels
• Itchiness 
• Ascites
• Low body weight and poor nutrient absorption from food

A person with decompensated liver disease is not eligible for the Kasai procedure.

The following preparations are required before the surgery:

• The doctor may perform a physical examination and look for jaundice and symptoms such as dark urine, distended abdomen, poor weight gain, and light or pale-coloured stools in your child. 
• He/she may order blood tests to check the functioning of the child's liver.
• Your child may undergo the imaging tests such as hepatobiliary iminodiacetic acid (HIDA) scan that provides images of the bile flowing from the liver to the intestine, abdominal ultrasound and a cholangiogram
• A liver biopsy will also be performed where a sample of the liver tissue is checked for any malfunction.
• You may need to feed your baby special formula to make them healthy enough for the surgery and continue breastfeeding until the doctor advises you to stop.  
• Avoid giving your baby anything to drink or eat four to six hours prior to surgery.
• You will be asked to sign a consent form to allow the doctor to perform the surgery.

The procedure will be performed in the operating room and under general anaesthesia, a sleep-inducing medicine. The surgery can be open (one large incision) or laparoscopic (many small incisions), but the open surgery is generally performed.

The steps involved in the procedure are as follows:

• The surgeon will make one (or multiple small) incision in the abdomen of the child.
• They will then cut and remove the bile duct from outside of their liver.
• The surgeon will use a part of the child's intestine to make a connection between their liver and intestine. This creates a path for the bile to flow from the liver to the intestine.
• Finally, the surgeon will close the incision using dissolvable sutures.

The surgery generally takes four hours, and you can expect the following after the surgery:

• The medical staff will move your child to the recovery room, and you can accompany them. Once they wake up, they will be transferred to the paediatric section.
• While in the hospital, frequent blood tests will be done to check their liver function. For about two to three days after the surgery, the child will not be able to eat and will have an intravenous line for nutrition. 
• A drain will be attached at the incision to drain any accumulated fluid. This helps in healing of the wound. 
• Your child may need corticosteroids to lower irritation and swelling of the liver.
• A hospital stay of seven to 10 days will be required after the surgery.

Once you take your child back home, you need to take care of the following:

• Activities: There may be not any restrictions on activities. 
• Pain management: You will need to give your child pain medications like ibuprofen by mouth about three to four times a day only when needed. 
• Swelling: There may be some swelling at the operation site. 
• Bath: Your infant may have a bath as usual.
• Food: Feed the special baby formula and breast milk as these contain fats that the liver can absorb easily. 
• Vitamin supplements: You will need to give the baby fat-soluble vitamin A, vitamin D, vitamin E, and vitamin K supplements as per your doctor’s advice for an extended period to prevent the deficiency of vitamins. 
• Medicine: Medicine to replace bile acid will be needed for several years to improve the flow of bile. You will also need to give antibiotics to your child for some months after the surgery. This helps to prevent any infection that may occur when the bacteria present in the intestine travel to the liver and bile ducts (cholangitis).
• Vaccines: Your child will require vaccines for conditions that may affect them in their childhood to maintain their immunity against such diseases. You may have to give them regular flu shots. Your child will need vaccines for hepatitis A and hepatitis B as well, which are diseases that affect the liver.

The benefits of this procedure include:

• It postpones and, in some cases, eliminates the need for a liver transplant.
• It improves the health of the child for many years

When to see the doctor?

Visit or call the doctor immediately if you observe any of these symptoms:

• Jaundice that has worsened or re-occurred 
• Pain or swelling in the abdomen
• Blood in the vomit  
• Pale-coloured or watery stools
• Blood in stools
• Fever of 38^°C or more
• Absence of bowel movements for more than three days
• Swelling, redness, or fluid leakage from the surgery site
• Bleeding or bruising
• Confusion
• Fainting

The possible complications for the Kasai procedure are:

• Cholangitis (inflamed bile ducts)
• Intestinal obstruction
• Portal hypertension that can lead to liver failure
• Anastomotic leak
• Hepatopulmonary syndrome (characterised by shortness of breath, absence of oxygen in the body, and bluish colour of the skin of the fingers and nails)
• Ascites

A follow-up visit will be scheduled for your child two to three weeks after the Kasai procedure. 

Disclaimer: The above information is provided purely from an educational point of view and is in no way a substitute for medical advice by a qualified doctor.