Eased symptoms may predict success of second biliary atresia surgery: Study
New operation for some children could help postpone liver transplant
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Infants with biliary atresia who show some signs of recovery after undergoing standard Kasai surgery — a procedure to restore the flow of the digestive fluid bile in the body — may benefit from a second operation if they later develop more symptoms, a new study suggests.
Having that second surgery, for young children whose symptoms eased with a first procedure, can help delay the need for a liver transplant, which typically is complicated by a lack of donors, according to the researchers.
Specifically, the study found that children whose jaundice — a symptom marked by yellowing of the skin and eyes — resolved temporarily after Kasai surgery had higher success rates for a second surgery. These youngsters were also less likely to need a liver transplant than those without jaundice clearance after initial surgery, the data showed.
“Reoperation effectively prolongs [jaundice-free] … liver survival in patients with prior clearance but recurrent jaundice, whereas it offers limited benefit to those without initial clearance, who should be prioritized for liver transplantation assessment,” the team wrote.
The study, “Clinical outcomes and influencing factors of reoperation in biliary atresia,” was published in the journal Pediatric Surgery International by a team of researchers in China.
Biliary atresia is a rare liver disease that can occur in infants. The condition causes blockage of the bile ducts, which are the pathways that transport bile from the liver to the small intestine. Bile buildup in the liver can lead to symptoms such as jaundice and cause liver damage, which, without treatment, can progress to liver failure.
Kasai surgery is first treatment for biliary atresia
The Kasai procedure, an intestinal surgery, is the first-line treatment for biliary atresia. It involves creating a new pathway for bile to drain from the liver to the small intestine, bypassing the bile ducts. This surgery is typically most successful early in life.
If liver problems don’t resolve after the first surgery, or temporarily resolve but then recur, patients may need a liver transplant.
However, “while liver transplantation offers definitive therapy, it is limited by donor scarcity, timing dilemmas, and long-term recipient management,” the researchers wrote. In this context, reoperation — either performing a second Kasai procedure or using another surgical strategy — may help postpone a liver transplant.
The question for clinicians is determining which patients are most likely to benefit from a second surgery and which should be assessed as early as possible for a transplant.
In this study, the team sought to “characterize subgroups most likely to benefit, evaluate outcomes of diversified surgical strategies, and provide updated evidence to inform individualized clinical decision-making.”
To that end, the researchers investigated factors affecting the success of reoperation among children with biliary atresia who initially underwent the Kasai procedure at a single center in Beijing. Medical records were examined for 19 patients: 15 girls and four boys.
The children’s median age was 59 days, or about 2 months old, at the time of Kasai surgery and 320 days, or about 10.5 months old, when the second operation took place. The most common types of surgery, each performed for about 40% of the children, were re-Kasai and intrahepatic cyst-jejunostomy, which is used to internally connect a swelled bile duct within the liver to the small intestine.
After a median of 494 days, or nearly 1.5 years, of follow-up, eight children (42%) were free of jaundice and hadn’t required a liver transplant, indicating the second surgery was a success. Four children (21%) saw a resolution in jaundice only after a liver transplant, and six (32%) continued to experience jaundice. One child died from a serious complication in the aftermath of the second surgery.
No jaundice after first operation predicts success for second
Here, the team looked for key differences between the eight children who had a successful second operation and the 11 who did not. The researchers found that jaundice clearance after the initial Kasai procedure was a significant predictor of a successful second surgery.
Of the 12 children whose jaundice cleared post-Kasai, eight (67%) had successful second surgeries. In contrast, none of the seven children without such clearance met these criteria.
In addition, children whose jaundice initially cleared were significantly more likely to undergo intrahepatic cyst-jejunostomy, while those without jaundice clearance were significantly more likely to undergo another Kasai procedure.
The team suggested that this reflected different underlying liver conditions, which influenced the surgical approach. The group with initial jaundice clearance likely had a higher rate of surgically accessible, localized bile duct obstruction, while children without jaundice clearance likely had more widespread problems affecting bile drainage.
Further statistical models showed that the rate of children with jaundice clearance and who avoided liver transplant was 84% at one year after the second surgery, and 71% at 1.5 years, showing a stabilization through 2.5 years. In contrast, this rate in children without jaundice clearance “declined continuously without plateau formation, with a median survival of only 1.342 years,” the team wrote.
The findings confirmed that “initial clearance is a critical prognostic marker for identifying likely reoperative beneficiaries,” the researchers wrote.
Together, these results indicate that children whose jaundice doesn’t clear after a Kasai procedure are unlikely to benefit from a second surgery, according to the researchers.
“Therefore, reoperation in this population should be considered with caution, and early evaluation for liver transplantation is advisable,” the team wrote.
Among the study’s limitations, the researchers noted its small sample size and retrospective nature. The follow-up period was also relatively short, so the researchers couldn’t draw conclusions about long-term outcomes of the second surgery.
“Future efforts should focus on continued follow-up of these postoperative patients to obtain long-term prognostic data for a more comprehensive assessment of the current findings,” the team wrote.
