Approaches to Biliary Atresia Care at Cincinnati Children's - Amy Taylor, MD - Successes in collaborative management of the post-kasai patient
With Dr. Amy Taylor
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What the experts said
Cincinnati Children's implemented clinical protocols for post-Kasai care in 2017
Infants 30-40 days old routinely go to PICU for first 24 hours post-Kasai for closer monitoring and pain management support
Post-Kasai patients receive IV cefoxitin for approximately 3 days
All stool diapers are inspected, saved, marked with time and date using Sharpie, and reviewed by the entire team during morning rounds
Cincinnati's steroid protocol targets infants <45 days with acolic stools (after ruling out infection) or patients >45 days with liver inflammation on biopsy
The steroid protocol consists of a 5-day burst followed by gradual 25% reduction over 4 weeks as outpatient
Escalated antibiotics include piperacillin-tazobactam or cefepime-flagyl (if renal concerns), with outpatient Augmentin
After implementing the 2017 protocol in 20 sequential patients, successful bile drainage (bilirubin <2 at 3 months) increased from 40% to 84% versus historical controls
Two-year transplant-free survival improved from 50% to 69% (11/16 patients) after protocol implementation, showing a trend but not achieving statistical significance
In the past 2 years, 8 out of 9 patients (89%) offered Kasai had successful bile drainage, with median age at Kasai of 53 days (range 29-109)
The one patient without drainage presented at 83-86 days of life and has undergone liver transplant
Post-discharge hepatology follow-up occurs every 1-2 weeks early post-Kasai
Weekly pre-visit planning sessions involve hepatologists, liver nurses, and administrators reviewing patients scheduled for the next 2 weeks
Fat-soluble vitamins are often not covered by insurance, requiring philanthropic vitamin fund support
Every patient is discussed before starting steroids; the protocol is tailored and customized rather than applied universally
Biliary atresia is heterogeneous, with patients presenting at different times with different stages of fibrosis
The START trial was properly conducted, properly powered, and showed no benefit to steroids in a well-designed trial
The most recent Kasai patient at 83 days with slightly pigmented stools and no inflammation findings did not receive steroids and did not achieve drainage
A 70-day-old patient with sporadic biliary atresia would not receive steroids under Cincinnati's protocol
Total bilirubin less than 2 at 3 months post-Kasai is the criterion used to consider a patient for Kasai revision
There is early translational data on how the microbiome shapes in humans with biliary atresia, but reliable intervention strategies are not yet understood
For recurrent cholangitis prophylaxis, the antibiotic escalation sequence is Augmentin, then Bactrim, then potentially broader coverage
Bactrim prophylaxis is continued as long as tolerated, with monitoring for leukopenia and agranulocytosis
Japanese group data shows Kasai outcomes can be improved, but 2-3 year transplant rates are closer to US data, requiring careful long-term follow-up
Kasai revision involves curettaging the hilar plate to remove debris, not re-cutting the entire anastomosis—only taking down the anti-mesenteric side
Some recurrent cholangitis cases are due to a blind loop distal to the drainage site on the stapled side of the roux limb, requiring resection of the redundant tip
Fellows are taught to build the portoenterostomy only several millimeters from the stapled bowel edge to avoid leaving a spur that promotes bacterial overgrowth
Total bilirubin less than 2 mg/dL at 3 months after Kasai predicts 2-year outcomes in biliary atresia
86% of patients with total bilirubin <2 at 3 months had transplant-free survival at 2 years versus 20% with bilirubin >2
Patients with lower bilirubin at 3 months post-Kasai had better growth and weight gain
In 240 children with BA who had native liver at 2 years (median follow-up 5 years), prognostic factors for better transplant-free survival included lower total bilirubin, less ascites, less cholangitis, higher albumin, and better platelet counts
Physical and motor impairment on Bailey examination at 1 year was associated with ascites and low Peli disease score
Mental, cognitive, and language impairment at 1 year was associated with ascites and low weight Z score
Unsuccessful Kasai hepatoportoenterostomy was a risk factor for impairment in both physical/motor and cognitive domains at 2 years
The START trial showed a trend (72% vs 57%, not statistically significant) toward better outcomes with steroids in infants less than 70 days old
The King's College trial showed that steroid administration in the younger cohort (less than 70 days) had better outcomes
The START trial showed similar rates of postoperative complications including cholangitis and infectious risk, occurring earlier in patients on high-dose steroid protocol
The immunological response to biliary obstruction is driven by the microbiome, with experimental data showing maternal diet/feeds affect immune cell response
A recent JPGN meta-analysis on post-Kasai antibiotics concluded there is no role for antibiotics post-Kasai, though the data was incredibly heterogeneous
The ChildrenNetwork showed that the rate of cholangitis even in patients older than 1 year of life is still 15%