Yeon Jun Jeong, Dayoung Ko, Hyunhee Kwon, Ki Hoon Kim, Dae Yeon Kim, Soo-Hong Kim, Wontae Kim, Hae-Young Kim, Hyun Young Kim, Seong Chul Kim, Younghyun Na, Jung-Man Namgoong, So Hyun Nam, Junbeom Park, Jinyoung Park, Tae-Jun Park, Jeong-Meen Seo, Ji-Young Sul, Joonhyuk Son, Hyun Beak Shin, Joohyun Sim, Soo Min Ahn, Hee Beom Yang, Jung-Tak Oh, Chaeyoun Oh, Joong Kee Youn, Sanghoon Lee, Ju Yeon Lee, Kyong Ihn, Hye Kyung Chang, Eunyoung Jung, Jae Hee Chung, Yu Jeong Cho, Yun Mee Choe, Soo Jin Na Choi, Seok Joo Han, In Geol Ho, Ji-Won Han
Adv Pediatr Surg 2025;31(2):47-58. Published online July 16, 2025
Purpose Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey.
Methods This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival.
Results The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey.
Conclusion While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.
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