Purpose: To report a nationwide survey on neuroblastoma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2020. Methods: The clinical data of pediatric patients diagnosed with and treated for neuroblastoma from 2005 to 2019 in 19 hospitals of KAPS members were collected. Survival and prognostic factor analyses were performed using the log rank test and Cox proportional hazard analysis. A p-value <0.05 was considered significant. Results: A total of 669 patients with neuroblastoma were registered for the study. The results were presented and discussed at the 36th annual meeting of the KAPS, which was held in Seoul on August 21, 2020. Conclusion This study provides information on patient demographics, prognostic outcomes, and comprehensive treatment outcomes for neuroblastoma. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of neuroblastoma.

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.

Proliferative myositis (PM) is a rare benign soft tissue neoplasm with a distinctive pseudosarcomatous proliferative reaction of muscles in tumors. Its rapid growth and bizarre microscopic appearance often require a differential diagnosis from a sarcomatous lesion. It has been reported occasionally, mostly as case reports in adult patients. Herein, we present a neonatal case of PM. To the best of our knowledge, this is the first report in the neonatal period.

Nasal dermoids are congenital midline nasal lesions that occur along with encephaloceles and gliomas. They can cause both deformity of nasal structure and intracranial infection as they grow. Treatment for these lesions is be concerned with two aspects, the complete removal of the lesions and making the surgical scar cosmetically acceptable. To that goal, many surgical approaches such as vertical incision, transverse incision, lateral rhinotomy and open rhinoplasty have been introduced. A 12-month male child presented with palpable mass at nasal root. The mass was easily movable, non-compressible and did not present fistula. A well-defined cystic mass without intracranial extension was found on the computerized tomography scans. Open rhinoplasty approach was opted for according to the guardians’ preference to avoid visible facial scar, and the lesions were completely resected. The pathologic examination confirmed the lesion to be nasal dermoids. The columellar scar was negligible and there was no recurrence at 5 year-follow up after surgery.

Coronary artery calcification (CAC) is highly prevalent in patients with chronic kidney disease (CKD) and is associated with major adverse cardiovascular events and metabolic disturbances. The triglyceride-glucose index (TyGI), a novel surrogate marker of metabolic syndrome and insulin resistance, is associated with CAC in the general population and in patients with diabetes. This study investigated the association between the TyGI and CAC progression in patients with CKD, which is unknown. Methods: A total of 1,154 patients with CKD (grades 1–5; age, 52.8 ± 11.9 years; male, 688 [59.6%]) were enrolled from the KNOWCKD (KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease). The TyGI was calculated as follows: ln (fasting triglycerides × fasting glucose/2). Patients were classified into tertiles (low, intermediate, high) based on the TyGI. The primary outcome was annualized percentage change in CAC score [(percent change in CAC score + 1)12/follow-up months – 1] of ≥15%, defined as CAC progression. Results: During the 4-year follow-up, the percentage of patients with CAC progression increased across TyGI groups (28.6%, 37.5%, and 46.2% in low, intermediate, and high groups, respectively; p < 0.001). A high TyGI was associated with an increased risk of CAC progression (odds ratio [OR], 2.11; 95% confidence interval [CI], 1.14–3.88; p = 0.02) compared to the low group. Moreover, a 1-point increase in the TyGI was related to increased risk of CAC progression (OR, 1.55; 95% CI, 1.06–1.76; p = 0.02) after adjustment. Conclusion: A high TyGI may be a useful predictor of CAC progression in CKD.

Nasal dermoids are congenital midline nasal lesions that occur along with encephaloceles and gliomas. They can cause both deformity of nasal structure and intracranial infection as they grow. Treatment for these lesions is be concerned with two aspects, the complete removal of the lesions and making the surgical scar cosmetically acceptable. To that goal, many surgical approaches such as vertical incision, transverse incision, lateral rhinotomy and open rhinoplasty have been introduced. A 12-month male child presented with palpable mass at nasal root. The mass was easily movable, non-compressible and did not present fistula. A well-defined cystic mass without intracranial extension was found on the computerized tomography scans. Open rhinoplasty approach was opted for according to the guardians’ preference to avoid visible facial scar, and the lesions were completely resected. The pathologic examination confirmed the lesion to be nasal dermoids. The columellar scar was negligible and there was no recurrence at 5 year-follow up after surgery.

Nasal dermoids are congenital midline nasal lesions that occur along with encephaloceles and gliomas. They can cause both deformity of nasal structure and intracranial infection as they grow. Treatment for these lesions is be concerned with two aspects, the complete removal of the lesions and making the surgical scar cosmetically acceptable. To that goal, many surgical approaches such as vertical incision, transverse incision, lateral rhinotomy and open rhinoplasty have been introduced. A 12-month male child presented with palpable mass at nasal root. The mass was easily movable, non-compressible and did not present fistula. A well-defined cystic mass without intracranial extension was found on the computerized tomography scans. Open rhinoplasty approach was opted for according to the guardians’ preference to avoid visible facial scar, and the lesions were completely resected. The pathologic examination confirmed the lesion to be nasal dermoids. The columellar scar was negligible and there was no recurrence at 5 year-follow up after surgery.

Nasal dermoids are congenital midline nasal lesions that occur along with encephaloceles and gliomas. They can cause both deformity of nasal structure and intracranial infection as they grow. Treatment for these lesions is be concerned with two aspects, the complete removal of the lesions and making the surgical scar cosmetically acceptable. To that goal, many surgical approaches such as vertical incision, transverse incision, lateral rhinotomy and open rhinoplasty have been introduced. A 12-month male child presented with palpable mass at nasal root. The mass was easily movable, non-compressible and did not present fistula. A well-defined cystic mass without intracranial extension was found on the computerized tomography scans. Open rhinoplasty approach was opted for according to the guardians’ preference to avoid visible facial scar, and the lesions were completely resected. The pathologic examination confirmed the lesion to be nasal dermoids. The columellar scar was negligible and there was no recurrence at 5 year-follow up after surgery.

Infective endocarditis involving a prosthetic valve significantly increases the risk of mortality. Extrinsic coronary compression caused by perivalvular abscess in prosthetic valve endocarditis is extremely rare. We present a case of a 40-year-old man with chest pain and fever who had undergone tissue aortic valve replacement 15 years prior. He underwent coronary angiography due to refractory chest pain. Intracoronary nitrate exacerbated symptoms and caused instability in his vital signs despite vasospastic features on coronary angiography. Intravascular ultrasonography revealed a triangular deformation of the proximal left circumflex artery. He received percutaneous coronary intervention on the left circumflex artery with a drugeluting stent, followed by a redo aortic valve replacement. Upon surgical inspection, an abscess had formed due to the prosthetic valve endocarditis was found, and was compressing his coronary artery. In patients suspected of infective endocarditis with myocardial infarction, suspicion of coronary artery compression due to perivalvular abscess should be considered. Distinctive features detected via intravascular ultrasonography can aid in diagnosis. Furthermore, we propose that percutaneous coronary intervention may serve as a bridge to valve replacement surgery, allowing time for restoration of ischemic myocardium.