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.

Purpose: This study aims to investigate and compare the incidence, demographic characteristics, clinical manifestations, preoperative diagnostic methods, anatomical classifications, associated anomalies, operative treatments, and postoperative outcomes of patients with intestinal atresia treated by the members of the Korean Association of Pediatric Surgeons (KAPS) through three nationwide surveys. Methods: KAPS conducted 3 national surveys in 1998, 2010, and 2024 to examine the patients diagnosed with intestinal atresia. In preparation for the survey, we developed a customized case registration form to obtain data on patient sex, birth weight, gestational age, clinical manifestations, preoperative diagnostic methods, anatomical types, associated anomalies, operative treatments, and postoperative outcomes. Authorized KAPS members completed the case registration form. Results: The first, second, and third national surveys included 218, 222, and 236 individuals diagnosed with intestinal atresia, respectively. The male-to-female ratios were 1.5:1, 1.1:1, and 1.1:1, respectively. The first, second, and third national surveys revealed that 34.3%, 43.3%, and 53.4% of patients were born before 37 weeks of gestation, respectively. Additionally, 28.7%, 32.0%, and 40.7% of patients had a birth weight under 2,500 g. In the third national survey, duodenoduodenostomy was the most common procedure, performed in 70 out of 82 patients diagnosed with duodenal atresia. Resection and anastomosis were the main surgical procedures conducted in 47 out of 54 cases of jejunal atresia and 74 out of 92 cases of ileal atresia. The mortality rates in the first, second, and third national surveys were 13.8%, 3.6%, and 1.3% respectively, with the lowest rate observed in the third national survey. Conclusion These national surveys offer valuable insights into the current state of intestinal atresia, including specific surgical interventions and postoperative outcomes in South Korea. For pediatric surgeons aiming to enhance their understanding of intestinal atresia and its treatment options, these surveys could be an indispensable resource and guide.

Background: Extracorporeal membrane oxygenation (ECMO) is the only treatment option that can stabilize patients with congenital diaphragmatic hernia (CDH) with severe pulmonary hypertension. This study assessed the effects of a multidisciplinary ECMO team approach (META) as part of a quality improvement initiative aimed at enhancing the survival rates of neonates with CDH. Methods: The medical records of infants with CDH treated at a tertiary center were retrospectively reviewed. Patients were categorized into two groups based on META implementation. The META group (P2) were given key interventions, including on-site ECMO management within the neonatal intensive care unit (NICU), use of venoarterial modality, ECMO indication as a priority even before the use of inhaled nitric oxide, and preplanned surgery following ECMO discontinuation. These approaches were compared with standard protocols in the pre-META group (P1) to assess their effects on clinical outcomes, particularly in-hospital mortality. Results: Over a 16-year period, 322 patients were included. P2 had a significantly higher incidence of non-isolated CDH and higher rate of cesarean section compared with P1.Moreover, P2 had delayed time to surgical repair (9.4 ± 8.0 days) compared with P1 (6.7 ± 7.3 days) (P = 0.004). The overall survival rate at NICU discharge was 72.7%, with a significant improvement from P1 (66.3%, 132/199) to P2 (82.9%, 102/123) (P = 0.001). Among the 68 patients who received ECMO, P2 had significantly lower baseline oxygenation index and serum lactate levels before ECMO cannulation than P1. The survival rate of patients who received ECMO also remarkably improved from P1 (21.1%, 8/38) to P2 (56.7%, 17/30).Subgroups who could be weaned from ECMO before 2 weeks after cannulation showed the best survival rate. Conclusion META significantly improved the survival rate of newborn infants with CDH.Further interventions, including prenatal intervention and novel ECMO strategies, may help improve the clinical outcomes and quality of life.

Purpose: Sirolimus has emerged as a safe and effective treatment for complicated lymphatic malformations (LMs). We aim to prove the effectiveness and safety of sirolimus as a therapeutic option for patients with complicated LMs. Methods: Fifty-eight patients with complicated LMs treated with sirolimus for at least 6 months at multicenter between July 2018 and January 2023 were enrolled. All patients were administered oral sirolimus starting at 0.8 mg/m 2 every 12 hours, with target serum concentration levels of 8–15 ng/mL. Evaluation for clinical symptoms and LMs volume on MRI were reviewed to assess treatment response and toxicities. Evaluation of disease response was divided into 3 values:complete response, partial response (significant, moderate, and modest), and progressive disease. Results: The median age at the initiation of sirolimus treatment was 6.0 years (range, 1 month–26.7 years). The median duration of treatment was 2.0 years (range, 6 months–4.4 years). The most common lesions were head and neck (25 of 58, 43.1%). Forty-six patients (79.3%) demonstrated a reduction in LMs volume on MRI or improvement of clinical symptoms including 2 complete responses. The young age group and the patients who underwent few prior therapies showed better responses. None of the patients had toxicities attributable to sirolimus with a Common Terminology Criteria for Adverse Events grade of ≥3. Conclusion Oral sirolimus treatment brought a successful outcome without severe adverse effects. It could be the firstline therapy, especially for the young age group of complicated LMs, and an additional option for refractory lesions that did not respond to conventional treatment.

Background: Extracorporeal membrane oxygenation (ECMO) is the only treatment option that can stabilize patients with congenital diaphragmatic hernia (CDH) with severe pulmonary hypertension. This study assessed the effects of a multidisciplinary ECMO team approach (META) as part of a quality improvement initiative aimed at enhancing the survival rates of neonates with CDH. Methods: The medical records of infants with CDH treated at a tertiary center were retrospectively reviewed. Patients were categorized into two groups based on META implementation. The META group (P2) were given key interventions, including on-site ECMO management within the neonatal intensive care unit (NICU), use of venoarterial modality, ECMO indication as a priority even before the use of inhaled nitric oxide, and preplanned surgery following ECMO discontinuation. These approaches were compared with standard protocols in the pre-META group (P1) to assess their effects on clinical outcomes, particularly in-hospital mortality. Results: Over a 16-year period, 322 patients were included. P2 had a significantly higher incidence of non-isolated CDH and higher rate of cesarean section compared with P1.Moreover, P2 had delayed time to surgical repair (9.4 ± 8.0 days) compared with P1 (6.7 ± 7.3 days) (P = 0.004). The overall survival rate at NICU discharge was 72.7%, with a significant improvement from P1 (66.3%, 132/199) to P2 (82.9%, 102/123) (P = 0.001). Among the 68 patients who received ECMO, P2 had significantly lower baseline oxygenation index and serum lactate levels before ECMO cannulation than P1. The survival rate of patients who received ECMO also remarkably improved from P1 (21.1%, 8/38) to P2 (56.7%, 17/30).Subgroups who could be weaned from ECMO before 2 weeks after cannulation showed the best survival rate. Conclusion META significantly improved the survival rate of newborn infants with CDH.Further interventions, including prenatal intervention and novel ECMO strategies, may help improve the clinical outcomes and quality of life.

Background: Extracorporeal membrane oxygenation (ECMO) is the only treatment option that can stabilize patients with congenital diaphragmatic hernia (CDH) with severe pulmonary hypertension. This study assessed the effects of a multidisciplinary ECMO team approach (META) as part of a quality improvement initiative aimed at enhancing the survival rates of neonates with CDH. Methods: The medical records of infants with CDH treated at a tertiary center were retrospectively reviewed. Patients were categorized into two groups based on META implementation. The META group (P2) were given key interventions, including on-site ECMO management within the neonatal intensive care unit (NICU), use of venoarterial modality, ECMO indication as a priority even before the use of inhaled nitric oxide, and preplanned surgery following ECMO discontinuation. These approaches were compared with standard protocols in the pre-META group (P1) to assess their effects on clinical outcomes, particularly in-hospital mortality. Results: Over a 16-year period, 322 patients were included. P2 had a significantly higher incidence of non-isolated CDH and higher rate of cesarean section compared with P1.Moreover, P2 had delayed time to surgical repair (9.4 ± 8.0 days) compared with P1 (6.7 ± 7.3 days) (P = 0.004). The overall survival rate at NICU discharge was 72.7%, with a significant improvement from P1 (66.3%, 132/199) to P2 (82.9%, 102/123) (P = 0.001). Among the 68 patients who received ECMO, P2 had significantly lower baseline oxygenation index and serum lactate levels before ECMO cannulation than P1. The survival rate of patients who received ECMO also remarkably improved from P1 (21.1%, 8/38) to P2 (56.7%, 17/30).Subgroups who could be weaned from ECMO before 2 weeks after cannulation showed the best survival rate. Conclusion META significantly improved the survival rate of newborn infants with CDH.Further interventions, including prenatal intervention and novel ECMO strategies, may help improve the clinical outcomes and quality of life.

Background: Extracorporeal membrane oxygenation (ECMO) is the only treatment option that can stabilize patients with congenital diaphragmatic hernia (CDH) with severe pulmonary hypertension. This study assessed the effects of a multidisciplinary ECMO team approach (META) as part of a quality improvement initiative aimed at enhancing the survival rates of neonates with CDH. Methods: The medical records of infants with CDH treated at a tertiary center were retrospectively reviewed. Patients were categorized into two groups based on META implementation. The META group (P2) were given key interventions, including on-site ECMO management within the neonatal intensive care unit (NICU), use of venoarterial modality, ECMO indication as a priority even before the use of inhaled nitric oxide, and preplanned surgery following ECMO discontinuation. These approaches were compared with standard protocols in the pre-META group (P1) to assess their effects on clinical outcomes, particularly in-hospital mortality. Results: Over a 16-year period, 322 patients were included. P2 had a significantly higher incidence of non-isolated CDH and higher rate of cesarean section compared with P1.Moreover, P2 had delayed time to surgical repair (9.4 ± 8.0 days) compared with P1 (6.7 ± 7.3 days) (P = 0.004). The overall survival rate at NICU discharge was 72.7%, with a significant improvement from P1 (66.3%, 132/199) to P2 (82.9%, 102/123) (P = 0.001). Among the 68 patients who received ECMO, P2 had significantly lower baseline oxygenation index and serum lactate levels before ECMO cannulation than P1. The survival rate of patients who received ECMO also remarkably improved from P1 (21.1%, 8/38) to P2 (56.7%, 17/30).Subgroups who could be weaned from ECMO before 2 weeks after cannulation showed the best survival rate. Conclusion META significantly improved the survival rate of newborn infants with CDH.Further interventions, including prenatal intervention and novel ECMO strategies, may help improve the clinical outcomes and quality of life.

Purpose: This study provides insights into the prevalence at birth, clinical characteristics, and outcomes of gastroschisis and omphalocele in Korea over the past decade, addressing the lack of localized data despite advanced healthcare capabilities. Methods: The study retrospectively analyzed data from 20 pediatric surgical centers in Korea from January 2012 to December 2021, including 269 patients diagnosed with gastroschisis or omphalocele. Data variables included gender, gestational age, birth weight, associated anomalies, type of defect, surgical interventions, and outcomes. Results: The study covered 269 patients, with 80 gastroschisis and 189 omphalocele cases. Gastroschisis prevalence at birth remained stable at 2.15 per 100,000 live births, while omphalocele increased to 5.08 per 100,000. Both conditions had similar gender ratios (0.95). Gastroschisis patients had lower birth weights (2,463.90±505.50 g) and smaller head circumferences (31.97±1.86 cm) compared to omphalocele patients (2,757.65±761.24 g, 32.78±2.64 cm). Omphalocele cases had more associated anomalies, especially cardiovascular issues. Prenatal diagnosis rates were high: 93.7% for gastroschisis and 86.4% for omphalocele. About 96.3% of gastroschisis and 84.1% of omphalocele patients were born in their treatment hospitals. Gastroschisis patients underwent surgery sooner (average 3.5 days) and started feeding later (16.5 days) than omphalocele patients (average 56.5 days to surgery, 6.6 days to start feeding). Hospital stays and follow-up durations were similar, averaging around 782.6 days for gastroschisis and 800.3 days for omphalocele patients. Survival rates were 89.7% for gastroschisis and 87.1% for omphalocele. Conclusion The study highlights the need for early diagnosis, centralized care, and specialized surgical approaches to optimize outcomes for gastroschisis and omphalocele patients in Korea. Enhanced prenatal screening and surgical protocols are recommended to improve these patients' prognosis.

Purpose: We analyzed the timing of inguinal hernia repair in premature infants in the neonatal intensive care unit (NICU) considering recurrence, incarceration, and other complications. Methods: In this multicenter retrospective review, premature infants (<37 weeks) in the NICU diagnosed with inguinal hernia between 2017 and 2021 were segregated into 2 groups based on the timing of inguinal hernia repair. Results: Of 149 patients, 109 (73.2%) underwent inguinal hernia repair in the NICU and 40 (26.8%) after discharge. Preoperative incarceration did not differ, but complications with recurrence and postoperative respiratory insufficiency were higher in the NICU group (11.0% vs. 0%, P = 0.029; 22.0% vs. 5.0%, P = 0.01). Multivariate analysis showed that the significant factors affecting recurrence were preoperative ventilator dependence and body weight of <3,000 g at the time of surgery (odds ratio [OR], 16.89; 95% confidence interval [CI], 3.45–82.69; P < 0.01 and OR, 9.97; 95% CI, 1.03–95.92; P = 0.04). Conclusion Our results suggest that when premature infants are diagnosed with inguinal hernia in the NICU, inguinal hernia repair after discharge may decrease the odds of recurrence and postoperative respiratory insufficiency. In patients who have difficulty delaying surgery, it is thought that surgery should be performed carefully in a ventilator preoperatively or weighed <3,000 g at the time of surgery.