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.

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.

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.

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.

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.

Purpose: Autologous adipose tissue-derived stem cells (ASCs) have been proposed for patients with refractory Crohn disease, but research is lacking in pediatric patients. This Phase IV study evaluated the efficacy and safety of ASCs in children with refractory Crohn’s fistulae. Methods: Patients with a refractory Crohn’s fistula who did not have conventional therapy for more than 3 months or with a recurrent complex Crohn’s fistula were included. All patients were at least 14 years old. Patients with infection, poor condition, or active Crohn disease with a disease activity index of 450 and above were excluded. Five patients were treated with ASCs from 2014 to 2015 in Asan Medical Center. ASC administration was adjusted according to fistula size (1 mL per cm 2 ). We evaluated the efficacy and safety 8 weeks after injection and followed patients for 6 months. Results: Fistulae were healed in 4 patients by 8 weeks after ASC injection. Of these 4 patients, 1 had complete fistula closure and sustainability after 6 months. The other 3 with healing effects had less than 50% fistula closure by 6 months.None of these 4 patients have persistent fistulae. One patient had no healing effect, and seton ligation was performed 8 months after ASC injection. There were no adverse effects related to ASC administration. Conclusion ASC therapy is a simple and well-tolerated therapeutic option for children with refractory Crohn’s complex fistulae. Complete closure was well-sustained. However, more data from a larger number of patients are needed.