Liver complication linked to transplant need in biliary atresia: Study
Portal hypertension treatment improves long-term outcomes for patients
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Half of biliary atresia patients who have undergone standard Kasai surgery develop a serious liver complication called portal hypertension (PH), and this is linked to a higher chance of needing a liver transplant, a study in Japan shows.
Still, if portal hypertension is successfully treated, the chances of needing a liver transplant in adolescence and adulthood are expected to be similar to those seen in patients with milder or no portal hypertension, researchers noted.
The study, “Portal hypertension in biliary atresia: a Japanese biliary atresia registry study,” was published in Pediatric Surgery International.
Biliary atresia treatment typically involves the Kasai procedure
Biliary atresia is marked by blocked or absent bile ducts, the tubes that carry the digestive fluid bile from the liver to the small intestine. The resulting slowing of bile flow leads to symptoms such as jaundice, or a yellow discoloration of the skin and whites of the eyes, and pale stools that usually start in early infancy.
Treatment typically involves the Kasai procedure, a surgical procedure that creates a new tube to carry bile from the liver to the intestines. Still, over the long run, children with BA will often develop worsened liver disease, and a liver transplant may become the only therapeutic option.
“Although the main reason for LTx [liver transplant] is liver failure due to persistent or recurrent jaundice after KP [Kasai procedure], the development of severe portal hypertension (PH) can also be a reason for LTx,” the researchers wrote.
Portal hypertension is a condition marked by elevated blood pressure in the main vessel that delivers blood to the liver. And “although PH is a serious problem in patients with BA [biliary atresia] following KP, long-term outcomes have not been well documented,” the researchers wrote.
To learn more, they analyzed data from 3,777 biliary atresia patients included in the Japanese Biliary Atresia Registry and who were followed up for up to 40 years after Kasai procedure or liver transplant. None had signs of persistent or recurrent jaundice at an age of 10 years or older, and they had undergone the Kasai surgery at an age of no older than 3 months.
Nearly half of study population had PH
In the overall study population, 46.4% had PH. Regarding PH complications, 36.8% showed an enlarged spleen and 35.1% had gastroesophageal varices (GEV), or swollen veins in the esophagus and stomach.
A total of 759 participants survived for 20 years without needing a liver transplant (native liver survivors). Similar rates of PH, enlarged spleen, and GEV were observed in the 20-year native liver survivors.
The frequency of GEV peaked at five years of follow-up, while that of enlarged spleen peaked at 10 years of follow-up. Treatment for GEV, which involved procedures using a thin tube inserted in the digestive tract, began in early childhood. Management of an enlarged spleen, which included the surgical removal of the spleen or blocking blood flow to a part of the spleen, peaked at 15 years of follow-up.
“The onset and treatment rates showed downward trends after adolescence,” the team wrote.
A subgroup of 596 native liver survivors was divided into those without PH (290 patients), with untreated PH (178 patients), or with treated PH (128 patients). There were no significant group differences in sex, gestational age at birth, birth weight, or liver damage markers, among other factors.
This implies that even if patients with PH [require] treatment during childhood or adolescence, long-term outcomes comparable to those of patients with milder or no PH may be achievable following appropriate management.
Among participants with PH, those who required treatment had a higher incidence of GEV (97.7% vs. 71.9%) and overactive spleen (88.3% vs. 69.1%) than those who didn’t. The treated PH group also had undergone the Kasai procedure at an older age (63 years vs. 62 years) and had a significantly higher incidence of cholangitis, or inflammation of the bile ducts (54.8% vs. 50.9%).
The proportion of long-term native liver survivors was highest among patients without PH and lowest among those who required treatment for PH.
For instance, after 20 years, 89.2% of people without PH were still living with their original liver, compared with 49.6% of those who needed treatment for PH. Among those who were treated for portal hypertension, 80.8% were still living with their original liver. Similar differences were observed at 10 and 30 years.
Further analysis revealed that while the untreated PH group initially had a significantly higher rate of native liver survival at five and 10 years than the treated group, these differences diminished beyond the 15-year mark.
“This suggests that the impact of PH severity on prognosis diminished over time,” the researchers wrote. “This implies that even if patients with PH [require] treatment during childhood or adolescence, long-term outcomes comparable to those of patients with milder or no PH may be achievable following appropriate management.”
