IPEG 2024 Round Table Discussion from East Asia - Dr. Sung Min Lee
With Dr. Sung Min Lee
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What the experts said
The patient was a female twin born at 36 weeks and 1 day gestational age via cesarean section and was underweight requiring NICU admission
From day 3, the patient developed acholic stools with elevated bilirubin and gamma-glutamyl transferase on blood tests
Abdominal ultrasound on day 6 revealed signs of biliary atresia
Initial Kasai operation was performed on day 9 of life at another hospital
After initial surgery, patient started feeding on post-operative day 4 and prednisone on day 7
Breast-colored stools were observed until post-operative day 14, but thereafter acholic stools were noticed
Follow-up ultrasound on post-operative day 26 showed persistent triangular cord thickness
SGOT, bilirubin, and gamma-glutamyl transferase levels continued to rise persistently after initial Kasai
Ultrasound and MRI at Severn Children's Hospital confirmed presence of triangular cord sign and findings consistent with biliary atresia despite prior Kasai operation
The imaging findings indicated that an incomplete or inappropriate surgery was performed at the initial operation
At redo surgery, when the previous portoenterostomy site was separated, bile was observed to be partially accumulated, indicating it was not completely obstructed
The remaining portal mass was removed and portoenterostomy was performed to cover the whole area
Post-operatively, AST and ALT levels rose sharply on the day of surgery and then steadily decreased
On post-operative day 23, when transaminases rose again, cholangitis was suspected and antibiotics were administered
Bilirubin levels increased in the first 2 days post-surgery and then steadily decreased, with improvement in stool color
Patient was discharged on post-operative day 29 after redo Kasai
The patient had portal vein branching variations that were not identified on pre-operative imaging at the first hospital
The remnant portal mass was hidden behind the right anterior portal vein branch and between the left portal vein
In cases with portal vein variations, surgeons need to exactly identify the branching anomaly and check for remnant portal mass
The first surgery may have been a hepaticojejunostomy rather than proper Kasai portoenterostomy based on operation records
Remnant portal mass can transform to fibrotic thickened mass, resulting in persistent jaundice
Almost all Korean surgeons do not adopt routine steroid use after Kasai operation based on nationwide survey
At Severn Children's Hospital, selective steroid therapy is used based on jaundice clearance status - if stools stay acholic after one week with no complications, steroids are started
Steroid therapy duration is 4 to 6 weeks after surgery, depending on presence of surgical complications and jaundice clearance status
If jaundice recurs within a few weeks after initial Kasai, it is likely a surgical/technical issue and redo should be considered
If jaundice occurs 4 or 6 months after initial Kasai, it is probably the underlying liver problem and transplantation should be considered rather than redo
After 4 or 6 months from initial surgery, a redo Kasai does not help very much
Age at Kasai operation is a very important factor for long-term native liver survival based on published articles and center data
Neonatal Kasai surgery cases have lower native liver survival than cases done between 30 to 60 days of age based on center experience
In very early Kasai surgery cases, bile duct maturation is less formed than in older patients, making exact dissection more difficult
Patients who underwent Kasai after 30 days and within 60 days had higher rates of jaundice clearance compared to neonatal cases
Very early Kasai operation may not always be beneficial as it may not allow for proper maturation of portal mass
Redo Kasai is much easier to perform than liver transplantation
Dr. Kasahara, a famous Japanese liver transplant surgeon, recommends doing everything possible to prevent liver transplantation
Success rate of redo Kasai depends on the quality of the first operation
Post-operative imaging with MRI can clearly visualize residual fibrous tissue and is important for decision-making about next steps