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.

Spinocerebellar ataxia type 3 (SCA3) is an autosomal-dominant neurodegenerative disorder caused by an expanded polyglutamine repeat in the ataxin-3 gene. The resulting mutant ataxin-3 protein forms intraneuronal inclusions that lead to neurodegeneration in the cerebellum and other brain regions. This study aimed to develop a novel nonhuman primate model of SCA3 to address the limitations of existing knock-in and transgenic models using an adeno-associated virus (AAV) to deliver the mutant gene. AAV viral vectors carrying mutant ataxin-3 were stereotaxically injected into the cerebellum of monkeys. The animals were monitored over an 8-week period, during which behavioral and neuroimaging assessments were conducted. This was followed by a detailed pathological examination. The AAV vector successfully spread throughout the cerebellum, and the expression of mutant ataxin-3 was confirmed. Neuroimaging revealed a reduction in N-acetylaspartate (NAA) levels, whereas histological analysis showed significant damage to the Purkinje cell layer. Notably, the monkeys exhibited sleep disturbances, a prodromal symptom commonly observed in human patients with SCA3. AAV-mediated delivery of mutant ataxin-3 can effectively replicate the key pathological and clinical features of SCA3 in primates. This approach offers a promising new model for studying disease mechanisms and evaluating potential therapies.

Purpose: Although numerous studies have identified potential risk factors for ipsilateral lymphedema development in patients with breast cancer following axillary node dissection, the risk factors for lymphedema in patients undergoing sentinel node biopsy without axillary dissection remain unclear. In this study, we aimed to determine the real-world incidence and risk factors for lymphedema in such patients. Methods: We conducted a single-center, retrospective review of medical records of patients with breast cancer who underwent sentinel node biopsy alone. The development cohort (5,051 patients, January 2017–December 2020) was analyzed to identify predictors of lymphedema, and a predictive model was subsequently created. A validation cohort (1,627 patients, January 2014–December 2016) was used to validate the model. Results: In the development cohort, 49 patients (0.9%) developed lymphedema over a median follow-up of 56 months, with most cases occurring within the first three years post-operation.Multivariate analysis revealed that a body mass index (BMI) of 30 kg/m2 or above, radiation therapy (RTx), chemotherapy, and more than three harvested lymph nodes significantly predicted lymphedema. The predictive model showed an area under the curve of 0.824 for systemic chemotherapy, with the number of harvested lymph nodes being the most significant factor. Patients were stratified into four risk groups, showing lymphedema incidences of 3.3% in the highest-risk group and 0.1% in the lowest-risk group. In the validation cohort, the incidences were 1.7% and 0.2% for the highest and lowest risk groups, respectively. Conclusion The lymphedema prediction model identifies RTx, chemotherapy, BMI ≥ 30 kg/m2 , and more than three harvested lymph nodes as significant risk factors. Although the overall incidence is low, the risk is notably influenced by the extent of lymph node removal and systemic therapies. The model’s high negative predictive value supports its application in designing tailored lymphedema surveillance programs for early intervention.

Purpose: Although numerous studies have identified potential risk factors for ipsilateral lymphedema development in patients with breast cancer following axillary node dissection, the risk factors for lymphedema in patients undergoing sentinel node biopsy without axillary dissection remain unclear. In this study, we aimed to determine the real-world incidence and risk factors for lymphedema in such patients. Methods: We conducted a single-center, retrospective review of medical records of patients with breast cancer who underwent sentinel node biopsy alone. The development cohort (5,051 patients, January 2017–December 2020) was analyzed to identify predictors of lymphedema, and a predictive model was subsequently created. A validation cohort (1,627 patients, January 2014–December 2016) was used to validate the model. Results: In the development cohort, 49 patients (0.9%) developed lymphedema over a median follow-up of 56 months, with most cases occurring within the first three years post-operation.Multivariate analysis revealed that a body mass index (BMI) of 30 kg/m2 or above, radiation therapy (RTx), chemotherapy, and more than three harvested lymph nodes significantly predicted lymphedema. The predictive model showed an area under the curve of 0.824 for systemic chemotherapy, with the number of harvested lymph nodes being the most significant factor. Patients were stratified into four risk groups, showing lymphedema incidences of 3.3% in the highest-risk group and 0.1% in the lowest-risk group. In the validation cohort, the incidences were 1.7% and 0.2% for the highest and lowest risk groups, respectively. Conclusion The lymphedema prediction model identifies RTx, chemotherapy, BMI ≥ 30 kg/m2 , and more than three harvested lymph nodes as significant risk factors. Although the overall incidence is low, the risk is notably influenced by the extent of lymph node removal and systemic therapies. The model’s high negative predictive value supports its application in designing tailored lymphedema surveillance programs for early intervention.

Purpose: Although numerous studies have identified potential risk factors for ipsilateral lymphedema development in patients with breast cancer following axillary node dissection, the risk factors for lymphedema in patients undergoing sentinel node biopsy without axillary dissection remain unclear. In this study, we aimed to determine the real-world incidence and risk factors for lymphedema in such patients. Methods: We conducted a single-center, retrospective review of medical records of patients with breast cancer who underwent sentinel node biopsy alone. The development cohort (5,051 patients, January 2017–December 2020) was analyzed to identify predictors of lymphedema, and a predictive model was subsequently created. A validation cohort (1,627 patients, January 2014–December 2016) was used to validate the model. Results: In the development cohort, 49 patients (0.9%) developed lymphedema over a median follow-up of 56 months, with most cases occurring within the first three years post-operation.Multivariate analysis revealed that a body mass index (BMI) of 30 kg/m2 or above, radiation therapy (RTx), chemotherapy, and more than three harvested lymph nodes significantly predicted lymphedema. The predictive model showed an area under the curve of 0.824 for systemic chemotherapy, with the number of harvested lymph nodes being the most significant factor. Patients were stratified into four risk groups, showing lymphedema incidences of 3.3% in the highest-risk group and 0.1% in the lowest-risk group. In the validation cohort, the incidences were 1.7% and 0.2% for the highest and lowest risk groups, respectively. Conclusion The lymphedema prediction model identifies RTx, chemotherapy, BMI ≥ 30 kg/m2 , and more than three harvested lymph nodes as significant risk factors. Although the overall incidence is low, the risk is notably influenced by the extent of lymph node removal and systemic therapies. The model’s high negative predictive value supports its application in designing tailored lymphedema surveillance programs for early intervention.

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.