Management and care after Kasai surgery in children with congenital biliary atresia

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Article written by Dr. Bui Thi Hang, High-tech Unit for Treatment of Cerebral Palsy and Autism - Vinmec Times City International Hospital

Congenital biliary atresia in children is a dangerous disease that should be treated early. Surgery is the best treatment available today. After surgery, the baby needs to be carefully cared for, to avoid biliary tract infections and portal hypertension.

Children after Kasai surgery need medical care including the following problems:
Diuretics; Provide nutrients; Provides fat-soluble vitamins; Prophylaxis of biliary tract infections; Management of portal hypertension and its consequences.

1. Diuretics

Doctors recommend that patients use the diuretic ursodeoxycholic acid (UDCA) after Kasai surgery.
The dose of UDCA diuretics ranges from 15 mg to 30 mg/kg/day and should not exceed 30 mg/kg/day to avoid potential toxicity. UDCA therapy should be discontinued when the total bilirubin level exceeds 15 mg/dl (257 micromol/l). Some studies suggest that the benefit of diuretics in the treatment of congenital biliary atresia is to reduce episodes of biliary tract infections and increase bile flow, but solid evidence from randomized trials is lacking.

2. Glucocorticoids

Drugs in this group have not proven to play a role in the treatment of biliary atresia after Kasai surgery, on the other hand, they have many side effects. However, in some cases, doctors may still choose to use glucocorticoids to treat some cases.

3. Nutrition

This is a common problem in children with congenital biliary atresia and difficult to overcome. Poor nutrition is one of the most important clinical problems and one of the most common indications for liver transplantation.
Calorie requirements: Several factors contribute to malnutrition in children with congenital biliary atresia including: cholestatic malabsorption, chronic hepatitis, and absence of a gallbladder. Because of malabsorption and metabolic changes, the total energy requirement in infants with congenital biliary atresia is approximately 130 to 150 percent of the total energy requirements of healthy infants. To compensate for the loss or catabolism, the daily protein requirement of infants is 3-4 g/kg/day and 2 to 3 g/kg/day in children.
To meet this nutritional requirement, a number of measures are taken as follows:
In infants: can be given condensed formula or expressed breast milk to increase energy for the baby
After surgery, Kasai can give the child 24 Kcal/Oz high-energy milk. If children still have growth retardation can use energy milk higher than 27 Kcal / Oz. As children get older, energy can be added to solid foods.

4. Provides fat-soluble vitamins

All children with congenital biliary atresia should be given fat-soluble vitamins according to the table below.
Once the jaundice is resolved and vitamins are adequate, the child can be switched to a standard dose multivitamin.
However, regular monitoring of vitamin levels should be continued to adjust the dose of vitamins provided to avoid deficiency or excess.

5. Complications

Children after successful bile drainage surgery must be monitored for complications such as biliary tract infection and portal hypertension. In addition, it is necessary to monitor the condition of the aforementioned fat-soluble vitamin deficiency.
Biliary tract infection This is a common complication in patients with biliary atresia who have had successful Kasai surgery. The incidence of cholangitis in these patients is between 40 and 90%, with the majority of patients experiencing at least one episode before the age of two years. Clinicians should suspect cholangitis in children when they present with fever for which the source of infection is unknown, particularly if fever is accompanied by decreased biliary secretion, malaise, and laboratory abnormalities. Because cholangitis can be life-threatening and can affect short- and long-term prognosis, clinicians prescribe prophylactic antibiotics for at least one year after Kasai surgery. Small, uncontrolled trials showed that the benefit of antibiotic prophylaxis outweighs the risk of antibiotic resistance
Prophylactic antibiotics Trimethoprim-sulfamethoxazole (4 mg/kg/day trimethoprim and 20 mg/kg/day) sulfamethoxazole) or neomycin (25 mg/kg/day divided four times daily) were equally effective in reducing the incidence of cholangitis.

Portal hypertension Chronic biliary hepatitis features of congenital biliary atrophy leading to advanced biliary cirrhosis. Biliary cirrhosis causes portal hypertension, which can lead to venous bleeding and ascites. The development of splenomegaly or a decreased platelet count after Kasai surgery suggests the possibility of progression to portal hypertension.
If portal hypertension leads to venous bleeding, this complication is usually controlled with sclerotherapy or elastic band ligation.
If ascites develops and is severe enough to affect respiratory function, it is usually treated endoscopically followed by chronic diuretics, beta-blockers, salt and/or water restriction, or combination of the above interventions.
To protect your child's health, you should have surgery at reputable medical facilities. Vinmec International General Hospital is a high-quality medical facility in Vietnam with a team of highly qualified medical professionals, well-trained, domestic and foreign, and experienced.
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Reference source: update.com