Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Objective: This study aimed to determine the impact of gestational age (GA) cut-off on the clinical outcome of congenital diaphragmatic hernia (CDH) and assess whether prematurity influences the predictive value of fetal lung volume measurement. Methods: We investigated the medical records of infants with CDH between January 2014 and August 2021. We classified the patients into term CDH (TCDH) and preterm CDH (PCDH) groups and compared their clinical characteristics, including fetal lung volume measured by the observed-toexpected lung-to-head ratio (O/E LHR). Results: Among 145 infants with CDH, 23 (15.9%) were preterm. Mean O/E LHR was significantly higher in survivors than in non-survivors with no difference between TCDH and PCDH groups.Mortality rate was significantly higher in infants with GA <34 weeks (80%) than in late preterm infants (16.7%). O/E LHR, rather than GA <34 weeks, was a risk factor predicting mortality in multivariate analyses. Predictive power of O/E LHR was high in the TCDH and PCDH groups, respectively. The incidence of chronic lung disease did not differ between PCDH and TCDH. Conclusion O/E LHR significantly predicted mortality in preterm infants, suggesting preterm birth alone should not be the determinant of early CDH treatment strategy.

Purpose: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.

Objective: This study aimed to determine the impact of gestational age (GA) cut-off on the clinical outcome of congenital diaphragmatic hernia (CDH) and assess whether prematurity influences the predictive value of fetal lung volume measurement. Methods: We investigated the medical records of infants with CDH between January 2014 and August 2021. We classified the patients into term CDH (TCDH) and preterm CDH (PCDH) groups and compared their clinical characteristics, including fetal lung volume measured by the observed-toexpected lung-to-head ratio (O/E LHR). Results: Among 145 infants with CDH, 23 (15.9%) were preterm. Mean O/E LHR was significantly higher in survivors than in non-survivors with no difference between TCDH and PCDH groups.Mortality rate was significantly higher in infants with GA <34 weeks (80%) than in late preterm infants (16.7%). O/E LHR, rather than GA <34 weeks, was a risk factor predicting mortality in multivariate analyses. Predictive power of O/E LHR was high in the TCDH and PCDH groups, respectively. The incidence of chronic lung disease did not differ between PCDH and TCDH. Conclusion O/E LHR significantly predicted mortality in preterm infants, suggesting preterm birth alone should not be the determinant of early CDH treatment strategy.

Objective: This study aimed to determine the impact of gestational age (GA) cut-off on the clinical outcome of congenital diaphragmatic hernia (CDH) and assess whether prematurity influences the predictive value of fetal lung volume measurement. Methods: We investigated the medical records of infants with CDH between January 2014 and August 2021. We classified the patients into term CDH (TCDH) and preterm CDH (PCDH) groups and compared their clinical characteristics, including fetal lung volume measured by the observed-toexpected lung-to-head ratio (O/E LHR). Results: Among 145 infants with CDH, 23 (15.9%) were preterm. Mean O/E LHR was significantly higher in survivors than in non-survivors with no difference between TCDH and PCDH groups.Mortality rate was significantly higher in infants with GA <34 weeks (80%) than in late preterm infants (16.7%). O/E LHR, rather than GA <34 weeks, was a risk factor predicting mortality in multivariate analyses. Predictive power of O/E LHR was high in the TCDH and PCDH groups, respectively. The incidence of chronic lung disease did not differ between PCDH and TCDH. Conclusion O/E LHR significantly predicted mortality in preterm infants, suggesting preterm birth alone should not be the determinant of early CDH treatment strategy.

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: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.