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.

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.

Background: Mac-2 binding protein glycosylation isomer (M2BPGi) has been established as a non-invasive biomarker for liver fibrosis. We evaluated the diagnostic efficacy of M2BPGi compared with those of other liver fibrosis markers in liver fibrosis in non-alcoholic fatty liver disease (NAFLD). Methods: We analyzed serum M2BPGi levels in 113 NAFLD patients. A pathologist graded liver fibrosis histopathologically. The diagnostic efficacies of serum M2BPGi and other liver fibrosis markers (aspartate aminotransferase to platelet ratio index, fibrosis index based on four factors, and NAFLD fibrosis score [NFS]) were evaluated using correlation, area under the ROC curve (AUC), logistic regression, and C-statistics. Results: Serum M2BPGi level and other liver fibrosis markers showed a moderate correlation with fibrosis grade. The AUC values of M2BPGi were 0.761, 0.819, 0.866, and 0.900 for diagnosing fibrosis (F) > 0, F > 1, F > 2, and F > 3, respectively. Logistic regression analysis showed M2BPGi as the only independent factor associated with F > 2 and F > 3. Although C-statistics showed that NFS was the best diagnostic factor for F > 2 and F > 3, M2BPGi with NFS had an increased C-statistics value, indicating that it is a better diagnostic model. Conclusions The serum M2BPGi level increased with liver fibrosis severity and could be a good biomarker for diagnosing advanced fibrosis and cirrhosis in NAFLD patients. A well-controlled, prospective study with a larger sample size is needed to validate the diagnostic power of M2BPGi and other fibrosis markers in NAFLD.

In one-stage transanal endorectal pull-through operation (TERPT) for Hirschsprung disease, preoperative evaluation by contrast enema (CE) is important tool in aspect of planning of surgical procedure as well as diagnosis. This study was to evaluate the significance of CE for identifying the extent of aganglionic bowel. A retrospective analysis was performed in 40 patients who underwent TERPT between 2003 and 2011. The authors reviewed the CE studies and their correlation with pathologic extent of aganglionosis. Total 66 contrast enemas were performed in 40 patients. Twenty patients underwent single CE, but 20 patients required multiple CEs. In single CE group, 17 had clear radiographic transition zone, but 3 had less definite transition zone. In multiple CE group, 17 patients who had equivocal finding in first or second CE had definite radiographic transition zone, but 3 patients of this group had less definite radiographic transition zones. Overall, 34 patients (85%) had clear radiographic transition zone by single or repeated CE. One (2.9%) out of 34 patients with clear radiographic transition zone had discordance between radiographic and pathologic transition zone. In contrast 4 (66.7%) out of 6 patients with equivocal radiographic transition zone had discordance between radiographic and pathologic transition zone. Observation of clear radiographic transition zone is important in preparation of TERPT, and repeated CE is helpful to reduce the discordance between radiographic and pathologic transition zone. Awareness of the possibility of discordance is also important if radiographic transitional zone is not clear.
Enema ; Hirschsprung Disease ; Humans ; Retrospective Studies

BACKGROUND: Patch angioplasty is an alternative surgical procedure to coronary artery bypass grafting (CABG) for left main coronary ostial stenosis. The purpose of this study is to evaluate the outcome of patch angioplasty by analyzing the short-term and long-term results. MATERIAL AND METHOD: Twenty nine patients who had undergone patch angioplasty due to left main coronary ostial stenosis between July 1991 and May 2003 were enrolled in the study. The mean age of the patients was 53.1+/-12.5 years. There were 8 males and 21 females, and there were 12 female patients who had no risk factor for atherosclerosis. Twenty six (89.7%) patients showed isolated coronary ostial stenosis without any distal coronary lesion. RESULT: Anterior approach was used in 28 patients and superior approach was used in one patient. Transsection of the main pulmonary artery was used in one patient. Concomitant CABG was performed in 4 patients because of left anterior descending artery lesions in 3 patients and unstable postoperative hemodynamic status in one patient. Hospital mortality had occurred in one patient (3.4%) and late mortality also in one patient, therefore the overall 5 year survival rate was 91.2+/-6.1%. Seventeen coronary angiographies were done in 13 patients (44.8%) postoperatively. Two distal patch stenoses, 1 proximal patch stenosis, and 1 new right coronary ostial lesion were identified and 3 percutaneous interventions and 1 CABG were performed during the follow-up period. The overall 5 year freedom from reintervention rate was 82.4+/-8.5%. Aortic regurgitation less than grade I had developed postoperatively in 4 patients and one patient showed progression of preexisting aortic regurgitation from grade II to III. CONCLUSION: Patch angioplasty in left main coronary ostial lesion showed acceptable short-term and long-term results in this study. However, restenosis at the patch anastomosis site and aortic regurgitation should be carefully investigated during the follow-up period.
Angioplasty* ; Aortic Valve Insufficiency ; Arteries ; Atherosclerosis ; Constriction, Pathologic* ; Coronary Angiography ; Coronary Artery Bypass ; Female ; Follow-Up Studies ; Freedom ; Hemodynamics ; Hospital Mortality ; Humans ; Male ; Mortality ; Pulmonary Artery ; Risk Factors ; Survival Rate

BACKGROUND: The aims of this study are to verify the result of the surgical treatment of ALCAPA and to identify the postoperative changes of left ventricular dimensions and mitral regurgitation (MR). MATERIAL AND METHOD: Fifteen patients operated on since 1985 were included in the study. The patients operated on before 1998 (n=9) showed heterogeneous properties with various surgical strategies and cardiopulmonary bypass techniques. However, six patients were operated on with the established surgical strategy since 1998; 1) Dual perfusion and dual cardioplegic solution delivery through ascending aorta and main pulmonary artery, 2) Coronary transfer by rolled-conduit made of pulmonary artery wall flap, and 3) Additional mitral valvular procedure was not performed. RESULT: Median age of the study group was 6 months (1 month to 34 years). The operative methods were left subclavian artery to left coronary artery anastomosis in 1, simple ligation in 2, Takeuchi operation in 2, and coronary reimplantation in 10 pateints. The mean follow up period was 5.5<5.8 years (2 months 14 years). There were one early death (6.7%) and one late death. Overall 5-year survival rate was 85.6+/-9.6%. The Z-value of left ventricular end-diastolic and end-systolic dimensions were 6.4+/-3.0 and 5.1+/-3.6 preoperatively, and decreased to 1.7+/-1.9 and 0.8+/-1.6 in 3 months (p<0.05). Significant preoperative MR was identified in 6 patients (40%) and all the patients showed immediate improvement of MR within 1 month postoperatively. There were 3 cases of reoperation due to coronary anastomosis site stenosis and recurrence of MR. However, there was no mortality nor late reoperation in the patients operated on after 1998. CONCLUSION: The surgical treatment of ALCAPA showed favorable survival and early recovery of ventricular dimensions and mitral valvular function. Although long-term reintervention was required in some cases of earlier period, all the cases after 1998 showed excellent surgical outcome without long-term problem.
Aorta ; Bland White Garland Syndrome ; Cardioplegic Solutions ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Coronary Vessels* ; Follow-Up Studies ; Humans ; Ligation ; Mitral Valve Insufficiency* ; Mortality ; Perfusion ; Pulmonary Artery* ; Recurrence ; Reoperation ; Replantation ; Subclavian Artery ; Survival Rate

BACKGROUND: The long-term results of combined mitral valve repair and aortic valve replacement (AVR) have not been well evaluated. This study was performed to investigate the early and long-term results of mitral valve repair with AVR. MATERIAL AND METHOD: We retrospectively reviewed 45 patients who underwent mitral valve repair and AVR between September 1990 and April 2002. The average age was 47 years; 28 were men and 17 women. Twelve patients had atrial fibrillation and three had a previous cardiac operation. The mitral valve disease consisted of pure insufficiency (MR) in 34 patients, mitral stenosis (MS) in 3, and mixed lesion in 8. Mitral valve disease was due to rheumatic origin in 24 patients, degenerative in 11, annular dilatation in 8, and ischemia or endocarditis in 2. The functional anatomy of mitral valve was annular dilatation in 31 patients, chordal elongation in 19, leaflet thickening in 19, commissural fusion in 13, chordal fusion in 10, chordal rupture in 6, and so on. Aortic prostheses used included mechanical valve in 32 patients, tissue valve in 12, and pulmonary autograft in one. The techniques of mitral valve repair included annuloplasty in 32 patients and various valvuloplasty of 54 techniques in 29 patients. Total cardiopulmonary bypass and aortic cross clamp time were 204+/-62 minute and 153+/-57 minutes, respectively. RESULT: Early death was in one patient due to low output syndrome (2.2%). After follow up of 57+/-37 months, late death was in one patient and the actuarial survival at 10 years was 96+/-4%. Recurrent MR developed grade II or III in 11 patients and moderate MS in 3. Three patients required reoperation for valve-related complications. The actuarial freedom from recurrent MR, MS, and reoperation were 64+/-11%, 86+/-8%, and 89+/-7% respectively. CONCLUSION: Combined mitral valve repair with AVR offers good early and long-term survival, and adequate techniques and selection of indication of mitral valve repair, especially in rheumatic disease, are prerequisites for better long-term results.
Aortic Valve* ; Atrial Fibrillation ; Autografts ; Cardiopulmonary Bypass ; Dilatation ; Endocarditis ; Female ; Follow-Up Studies ; Freedom ; Humans ; Ischemia ; Male ; Mitral Valve Stenosis ; Mitral Valve* ; Prostheses and Implants ; Reoperation ; Retrospective Studies ; Rheumatic Diseases ; Rupture

BACKGROUND: Mitral valve repair rather than replacement for mitral regurgitation (MR) offers a number of well-accepted benefits. However, the surgical results of repair for mitral valve endocarditis remain largely unknown. MATERIAL AND METHOD: Fourteen patients who underwent mitral valve repair for MR caused by mitral valve endocarditis from April 1995 through October 2001 were reviewed retrospectively. There were 9 male patients and mean age was 32+/-10 years. Four patients had previous embolism and 2 had active infections. The grade of MR were III in 6 patients and IV in 8. Operatively, mitral annuloplasty was performed in 12 patients and various valvuloplasty techniques were applied in all patients. One patient had immediate valve replacement due to residual MR after weaning of cardiopulmonary bypass. RESULT: There was no early operative death. Early postoperative transthoracic echocardiography revealed no or grade I of MR and no or mild mitral stenosis in 13 patients. After the mean follow-up of 36 months, there was no late death, and no or grade I of MR in 11 patients (84.6%) and no or mild mitral stenosis in 12 patients (92.3%). Reoperation required in one patient (7.1%). The cumulative freedom from recurrent MR and valve-related reoperation at 5 years were 91+/-9% and 75+/-22%, respectively. CONCLUSION: This study suggests that mitral valve repair for mitral regurgitation caused by endocarditis offers good early and intermediate survival and functional improvement without reinfection, and it is an attractive alternative to valve replacement in selective patients with bacterial endocariditis.
Cardiopulmonary Bypass ; Echocardiography ; Embolism ; Endocarditis* ; Follow-Up Studies ; Freedom ; Humans ; Male ; Mitral Valve Annuloplasty ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Mitral Valve* ; Reoperation ; Retrospective Studies ; Weaning