Management of Pruritus in Biliary Atresia Post-Kasai Procedure
Oral cholestyramine is the first-line treatment for pruritus in a child with biliary atresia after Kasai procedure, not IV corticosteroids. 1, 2, 3
First-Line Treatment: Cholestyramine
Cholestyramine should be initiated at 4 g/day, titrating up to a maximum of 16 g/day as tolerated for management of cholestatic pruritus in post-Kasai patients. 1
The mechanism involves binding bile salts in the intestinal lumen, preventing their reabsorption and reducing dermal deposition that causes itching. 3
Critical timing consideration: Cholestyramine must be administered 2-4 hours before or after ursodeoxycholic acid (UDCA) to avoid binding interactions, as UDCA is routinely used post-Kasai to promote bile flow. 1, 4
Practical administration tip: Give cholestyramine at breakfast time (one hour before or after eating) if the gallbladder is in situ, and consider mixing with orange squash and refrigerating overnight to improve palatability. 1
Most patients do not benefit from doses exceeding 8-12 g/day, and constipation is the primary side effect. 1
Second-Line Treatment: Rifampicin
If cholestyramine fails or is not tolerated, rifampicin is the recommended second-line agent, starting at 150 mg once to twice daily and titrating to a maximum of 600 mg/day based on symptoms and liver function monitoring. 1, 2
Monitor liver function tests every 2-4 weeks when using rifampicin due to hepatotoxicity risk, particularly important in children with already compromised liver function post-Kasai. 1, 2
Consider vitamin K supplementation if the patient is icteric while on rifampicin. 1
Third-Line Options for Refractory Pruritus
For pruritus unresponsive to cholestyramine and rifampicin, consider naltrexone (starting at 12.5 mg/day, titrating slowly to 50 mg/day to avoid opiate withdrawal-like symptoms). 1, 2, 5
Naltrexone has been specifically reported as effective in a 17-month-old child with biliary atresia whose pruritus was refractory to antihistamines, topical steroids, cholestyramine, and rifampicin. 5
Alternative third-line agents include selective serotonin reuptake inhibitors (sertraline 100 mg/day, titrated to symptoms) or gabapentin, though gabapentin lacks strong efficacy evidence. 1, 2
Why NOT IV Corticosteroids
Corticosteroids are used post-Kasai to reduce inflammatory bile duct changes and improve bile flow, NOT to treat pruritus. 4, 6
When steroids are used post-Kasai, they are given as oral prednisolone (4 mg/kg) starting 1 week after surgery, with the goal of achieving lower postoperative bilirubin levels, not managing pruritus. 6
There is no evidence supporting IV corticosteroids for cholestatic pruritus management in biliary atresia patients. 1
Important Caveats
Antihistamines have limited efficacy for cholestatic pruritus and work primarily through sedative properties rather than addressing the underlying mechanism. 1, 2
Liver transplantation is highly effective for intractable pruritus (often resolving within 24 hours) and should be considered when pruritus is persistent and refractory to all medical therapies, as it significantly improves quality of life. 1, 2
Monitor for worsening cholestasis or development of complications (cholangitis, portal hypertension) that may indicate Kasai failure and need for transplantation evaluation. 1, 7, 8