Definition and Overview

Also known as Kasai procedure, hepatoportenterostomy is a reconstructive procedure performed to treat biliary atresia, an end-stage liver condition that is common among very young children. It involves the use of a small part of the intestine to replace an obstructed biliary duct that has caused the buildup of bile, a fluid produced by the liver intended for better absorption of fat.

From the liver surface, the bile flows to the intrahepatic duct, which then delivers it to the extrahepatic duct, which carries the bile to the gallbladder that stores the bile for future use and the small intestine. When these ducts are blocked, bile accumulates over time, which can lead to a more life-threatening condition like cirrhosis or scarring of the liver. Kasai procedure is performed to avoid a more permanent damage to the liver, reduce or stop scarring, and allow the proper drainage of bile.

Who Should Undergo and Expected Results

The Kasai procedure is performed on patients diagnosed with biliary atresia, or the blockage of the biliary ducts. This is a rare condition with unknown origin and normally affects children between 2 and 4 weeks. However, the disease can also be diagnosed while the baby is still in the womb (fetal biliary atresia) or when he’s already past two months old.

The most common symptom of biliary atresia is jaundice or the yellowing of the whites of the eyes and skin, which happens when the liver loses its ability to remove bilirubin from the broken-down red blood cells. Other signs include poor weight gain, stunted growth, excessive amounts of bilirubin detected in the urine (also indicated by the change of urine colour to dark yellow or brown), and change in colour of stool.

The disease may be confirmed with exams like a liver function panel and imaging tests such as scans and X-rays that can reveal the size of the liver and if the ducts are obstructed. A biopsy, or the removal of an organ tissue sample, will also be performed if the results of other tests are inconclusive.

The surgery can be done on children at any age, but it is best performed before the child reaches 2 years old so the likelihood of a permanent organ damage due to cirrhosis can be minimised. When performed on infants under 60 days old, the success rate is up to 80%. However, a liver transplant may still be needed in the future and can be carried out when the child has already reached adulthood.

How Does the Procedure Work?

Hepatoportenterostomy requires an intensive planning in collaboration with a surgical team composed of an anaesthesiologist, paediatrician, and pediatric surgeon, as well as the parents or guardians of the child. Before the child undergoes the procedure, certain tests are performed to confirm that he is fit to undergo surgery and determine the severity of the condition and scarring of the liver. In certain cases, Kasai surgery is set aside in favour of a liver transplant.

To start the procedure, the anaesthesiologist administers general anaesthesia while monitoring the child’s vitals. The surgeon then decides to perform either an open or laparoscopic surgery. In a traditional open surgery, a large incision is made in the area of the abdomen that is close to where the liver is found to access the organ. In a minimally invasive or laparoscopic procedure, the surgeon creates three to four small incisions in the same abdominal area, inserts a probe, uses the probe’s camera to check the actual condition of the liver and the surrounding organs and tissues, and operates through the remaining incisions.

Once the organ is accessed, the surgeon proceeds to locate the source of the blockage and removes it along with the gallbladder, then connects a part of the small intestine to the problematic area to allow the bile to flow from the liver to the small intestine.

The procedure, which can take up to 4 hours, ends with suturing the incisions before the child is wheeled into the recovery room. As the procedure is linked to various risks and complications, the patient is advised to stay in the hospital for a few days for close observation.

Possible Risks and Complications

Kasai procedure may cause malabsorption of food that’s high in fat or fat-soluble vitamins like A and K, so he may be encouraged to maintain a diet that’s low in fat or supplement himself with the said vitamins. Infants 6 months old and below can benefit greatly from exclusive breastfeeding since breast milk is very low in fat content.

Reference:

  • A-Kader HH, Balistreri WF. Neonatal cholestasis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 348.1.
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