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: Post-transplantation lymphoproliferative disorders (PTLDs) after hematopoietic stem transplantation (HCT) or solid organ transplantation (SOT) result in poorer outcomes, including death. There are limited large cohort data on the incidence and natural course of PTLD in Asians. Materials and Methods: We investigated PTLD using Korean national health insurance claims data of 47,518 patients who underwent HCT or SOT in 2008-2020. Patient demographics, time and type of PTLD diagnosis, type of PTLD treatment, and death data were collected. We used Fine and Gray subdistribution hazard models to calculate the cumulative incidence and risk factors for PTLD. Results: During median follow-up of 5.32 years, PTLD occurred in 294 of 36,945 SOT patients (0.79%) and 235 of 10,573 HCT patients (2.22%). Cumulative incidence of PTLD were 0.49% at 1 year, 1.02% at 5 years, and 1.50% at 10 years post-transplantation. Age < 20 years (subdistribution hazard ratio [SHR] of 1.67 in age 10-19, SHR 1.51 in age 0-9), HCT (SHR 3.02), heart transplantation (SHR 2.27), and liver transplantation (SHR 1.47) were significant risk factors for PTLD. The presence of PTLD was associated with an increased risk of death (hazard ratio of 2.84). Overall, 5-year survival of PTLD patients was 68.9% (95% confidence interval, 64.9 to 73.2). Conclusion We observed a steady increase in PTLD over 10 years after HCT or SOT in this large cohort study. Pediatric age group, HCT, liver transplantation, and heart transplantation were suggested to be risk factors for PTLD, and PTLD was associated with a higher risk of death.

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.

Purpose: Most patients with perinatally detected subhepatic cysts receive information suggestive of a suspected diagnosis of choledochal cyst (CC). However, it is not uncommon to be finally diagnosed with cystic biliary atresia (CBA), a disease with a more unfavorable prognosis. This study aimed to investigate the distribution of the final diagnoses of perinatally detected subhepatic cysts and to compare patients diagnosed with CC and CBA.Method: We performed a retrospective review of patients with subhepatic cysts detected using ultrasonography during perinatal period, between January 2012 and December 2022.Result: This retrospective study included 52 patients with perinatal subhepatic cysts. Of these, 71.2% (37/52) were diagnosed with CC, 15.4% (8/52) with CBA, and 5.8% (3/52) with duplication of the alimentary tract. Only 1.9% (1/52) of the patients were diagnosed with biliary atresia, gallbladder duplication, mesenteric lymphatic malformation, or were normal. Of all patients, 86.5% (45/52) were diagnosed with CC or CBA, with CBA accounting for 17.8% (8/45). There were no statistically significant differences between the CC and CBA groups regarding the gestational age at which the cyst was first detected and the final size of the cyst measured on prenatal ultrasound. Conclusion Subhepatic cysts detected during the perinatal period are typically diagnosed as CC. However, this study revealed that 15.4% of all patients were diagnosed with CBA, despite no significant differences in prenatal ultrasound findings. Therefore, it is essential to consider the possibility of CBA in cases of perinatally detected subhepatic cysts.

Objective: To assess the feasibility of quantitatively assessing pancreatic steatosis using magnetic resonance imaging (MRI) and its correlation with obesity and metabolic risk factors in pediatric patients. Materials and Methods: Pediatric patients (≤ 18 years) who underwent liver fat quantification MRI between January 2016 and June 2019 were retrospectively included and divided into the obesity and control groups. Pancreatic proton density fat fraction (P-PDFF) was measured as the average value for three circular regions of interest (ROIs) drawn in the pancreatic head, body, and tail. Age, weight, laboratory results, and mean liver MRI values including liver PDFF (L-PDFF), stiffness on MR elastography, and T2* values were assessed for their correlation with P-PDFF using linear regression analysis. The associations between P-PDFF and metabolic risk factors, including obesity, hypertension, diabetes mellitus (DM), and dyslipidemia, were assessed using logistic regression analysis. Results: A total of 172 patients (male:female = 125:47; mean ± standard deviation [SD], 13.2 ± 3.1 years) were included. The mean P-PDFF was significantly higher in the obesity group than in the control group (mean ± SD, 4.2 ± 2.5% vs. 3.4 ± 2.4%; p = 0.037). L-PDFF and liver stiffness values showed no significant correlation with P-PDFF (p = 0.235 and p = 0.567, respectively). P-PDFF was significantly associated with obesity (odds ratio 1.146, 95% confidence interval 1.006–1.307, p = 0.041), but there was no significant association with hypertension, DM, and dyslipidemia. Conclusion MRI can be used to quantitatively measure pancreatic steatosis in children. P-PDFF is significantly associated with obesity in pediatric patients.

Purpose: This study compared clinical and radiologic differences between cystic biliary atresia (cBA) and choledochal cyst (CC) type Ia/b. Methods: Infants (≤12 months old) who were diagnosed with cBA or CC type Ia/b from 2005 to 2019 were retrospectively reviewed. Imaging features on preoperative ultrasonography (US) and magnetic resonance imaging (MRI) were compared between the cBA and CC groups. Logistic regression and area under the receiver operating characteristic curve (AUC) analyses were performed for the diagnosis of cBA. Changes in cyst size were also evaluated when prenatal US exams were available. Results: Ten patients (5.5% of biliary atresia cases) with cBA (median age, 48 days) and 11 infants with CC type Ia/b (Ia:Ib=10:1; median age, 20 days) were included. Triangular cord thickness on US (cutoff, 4 mm) showed 100% sensitivity and 90.9% specificity (AUC, 0.964; 95% confidence interval [CI], 0.779 to 1.000) and cyst size on MRI (cutoff, 2.2 cm) had 70% sensitivity and 100% specificity (AUC, 0.900; 95% CI, 0.690 to 0.987) for diagnosing cBA. Gallbladder mucosal irregularity on US and an invisible distal common bile duct on MRI were only seen in the cBA group (10 of 10). Only the CC group showed prenatal cysts exceeding 1 cm with postnatal enlargement. Conclusion Small cyst size (<1 cm) on prenatal US, triangular cord thickening (≥4 mm) and gallbladder mucosal irregularity on postnatal US, and small cyst size (≤2.2 cm) and an invisible distal common bile duct on MRI can discriminate cBA from CC type Ia/b in infancy.

A 6-year-old male who lived with a mother in a single-parent family was referred to the emergency room with multiple traumas. There was no specific finding on CT scan of the other hospital performed 55 days before admission. However, CT scan at the time of admission showed common bile duct (CBD) stenosis, proximal biliary dilatation and bile lake formation at the segment II and III. Endoscopic retrograde biliary drainage was performed, but the tube had slipped off spontaneously 36 days later, and follow-up CT scan showed aggravated proximal biliary dilatation above the stricture site. He underwent excision of the CBD including the stricture site, and the bile duct was reconstructed with Roux-en-Y hepaticojejunostomy. Pathologic report of the resected specimen revealed that the evidence of trauma as a cause of bile duct stricture. While non-iatrogenic extrahepatic biliary trauma is uncommon, a level of suspicion is necessary to identify injuries to the extrahepatic bile duct. The role of the physicians who treat the abused children should encompass being suspicious for potential abdominal injury as well as identifying visible injuries.
Abdominal Injuries ; Bile Ducts ; Bile Ducts, Extrahepatic ; Bile ; Child ; Child Abuse ; Child ; Common Bile Duct ; Constriction, Pathologic ; Dilatation ; Drainage ; Emergency Service, Hospital ; Follow-Up Studies ; Humans ; Lakes ; Male ; Mothers ; Multiple Trauma ; Single-Parent Family ; Tomography, X-Ray Computed ; Wounds, Nonpenetrating

PURPOSE: Santulli enterostomy has been used for various surgical abdominal conditions that require temporary diversion of bowel during a neonatal period. The aim of this study was to report clinical outcomes of Santulli enterostomy and to evaluate its usefulness. METHODS: Between January 2000 and December 2016, 40 neonates who underwent Santulli enterostomy were enrolled; Santulli enterostomies were performed for 25 patients without previous laparotomy (primary Santulli group) and 15 patients with previous laparotomy (secondary Santulli group). RESULTS: Small bowel atresia is the first common indication of Santulli enterostomy (22/40, 55.0%), and luminal discrepancy between proximal and distal bowel was the most common determinant factor of Santulli enterostomy (17/40, 42.5%). The median age at surgery and mean birth weight were 2 days and 2,480 g respectively in the primary group, and 71 days, 2,340 g respectively in the secondary group. Operation time was significantly longer in the secondary group than the primary group (156±48 minutes vs. 224±95 minutes, p=0.019), and there was no difference in the time taken to initiation of oral feeding between the two groups. Santulli enterostomy closure was performed at median 65 days after Santulli enterostomy for primary group and 70 days for secondary group. Six complications (15.0%) were found after Santulli enterostomy, and nine complications (24.3%) after Santulli enterostomy closure (p=0.302). The incidence of complications was significantly higher in secondary group than in primary group (4.5% vs. 53.3%, p=0.001), and the reoperation rate was also significantly higher in the secondary group (4.5% vs. 46.7%, p=0.004). CONCLUSION: Santulli enterostomy could be applied as a temporary enterostomy in neonatal patients with various surgical abdominal diseases. Considering the high complication rate after secondary Santulli enterostomy closure, decision making on the timing of enterostomy closure should be done with caution.
Birth Weight ; Decision Making ; Enterocolitis, Necrotizing ; Enterostomy ; Humans ; Incidence ; Infant, Newborn ; Intestinal Atresia ; Laparotomy ; Methods ; Peritonitis ; Phenobarbital ; Reoperation