Purpose: Short bowel syndrome (SBS) is a critical condition in pediatric patients. It often results in dependence on parenteral nutrition (PN) and significant morbidity. This study aimed to review pediatric SBS patients from a single center and analyze clinical outcomes based on etiology and anatomical type. Methods: A total of 55 patients diagnosed with SBS at our institution from January 2004 to December 2018 were retrospectively analyzed. Clinical demographics and outcomes including growth and PN dependence were evaluated according to etiology and anatomical type. Results: The predominant cause of SBS was necrotizing enterocolitis (NEC). At the last follow-up, the mean weight-forage (z-score) was –2.00 ± 2.07 and 52.7% of patients were weaned off PN. Catheter-related bloodstream infection and PN-associated liver disease occurred in 36.4% and 20.0% of cases, respectively. Patients with motility disorders exhibited lower weight-for-age and longer durations of PN than those with NEC or intestinal atresia. Additionally, outcomes varied significantly by anatomical type, with type I patients showing higher PN dependence and lower rates of successful weaning off PN than type III patients. Conclusion The study suggests that clinical outcomes in pediatric SBS patients can vary depending on underlying etiology and anatomical type, indicating that tailored interventions might enhance patient outcomes. Further research is needed to identify independent prognostic factors for SBS and improve the quality of life of SBS children.

Purpose: The study aimed to evaluate the characteristics and operative-related factors in children who underwent fundoplication, analyze surgical outcomes categorized by disease entity and surgical indication, and identify prognostic factors for reoperation risk. Methods: A total of 109 pediatric patients who underwent fundoplication between 2008 and 2022 were retrospectively analyzed. Patients were grouped by disease entity and surgical indication. Underlying diseases, comorbidities, sex, gestational age, birth weight, preoperative symptoms, and operation-related factors were examined. Outcomes were classified as short-term and long-term adverse events. We investigated differences in clinical outcomes according to disease entity and surgical indication. Then we statistically identified preoperative predictors for the risk of reoperation. Results: The most common disease entity was neurological impairment (n = 92). Pulmonary comorbidity (42.2%) and aspiration/regurgitation (87.2%) were the most common. Most surgeries were performed laparoscopically (86.2%). There were 12 short-term and 25 long-term adverse events, with long-term events occurred more frequently in the neurological impairment (NIP) group compared to the non-NIP group (P = 0.04). None of the factors showed a significant relationship with the risk of reoperation. Conclusion Neurologically impaired children were more likely to experience long-term adverse events postfundoplication. However, no significant predictors for reoperation risk were identified.

Purpose: The study aimed to evaluate the characteristics and operative-related factors in children who underwent fundoplication, analyze surgical outcomes categorized by disease entity and surgical indication, and identify prognostic factors for reoperation risk. Methods: A total of 109 pediatric patients who underwent fundoplication between 2008 and 2022 were retrospectively analyzed. Patients were grouped by disease entity and surgical indication. Underlying diseases, comorbidities, sex, gestational age, birth weight, preoperative symptoms, and operation-related factors were examined. Outcomes were classified as short-term and long-term adverse events. We investigated differences in clinical outcomes according to disease entity and surgical indication. Then we statistically identified preoperative predictors for the risk of reoperation. Results: The most common disease entity was neurological impairment (n = 92). Pulmonary comorbidity (42.2%) and aspiration/regurgitation (87.2%) were the most common. Most surgeries were performed laparoscopically (86.2%). There were 12 short-term and 25 long-term adverse events, with long-term events occurred more frequently in the neurological impairment (NIP) group compared to the non-NIP group (P = 0.04). None of the factors showed a significant relationship with the risk of reoperation. Conclusion Neurologically impaired children were more likely to experience long-term adverse events postfundoplication. However, no significant predictors for reoperation risk were identified.

Purpose: The study aimed to evaluate the characteristics and operative-related factors in children who underwent fundoplication, analyze surgical outcomes categorized by disease entity and surgical indication, and identify prognostic factors for reoperation risk. Methods: A total of 109 pediatric patients who underwent fundoplication between 2008 and 2022 were retrospectively analyzed. Patients were grouped by disease entity and surgical indication. Underlying diseases, comorbidities, sex, gestational age, birth weight, preoperative symptoms, and operation-related factors were examined. Outcomes were classified as short-term and long-term adverse events. We investigated differences in clinical outcomes according to disease entity and surgical indication. Then we statistically identified preoperative predictors for the risk of reoperation. Results: The most common disease entity was neurological impairment (n = 92). Pulmonary comorbidity (42.2%) and aspiration/regurgitation (87.2%) were the most common. Most surgeries were performed laparoscopically (86.2%). There were 12 short-term and 25 long-term adverse events, with long-term events occurred more frequently in the neurological impairment (NIP) group compared to the non-NIP group (P = 0.04). None of the factors showed a significant relationship with the risk of reoperation. Conclusion Neurologically impaired children were more likely to experience long-term adverse events postfundoplication. However, no significant predictors for reoperation risk were identified.

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.

Pediatric intestinal perforation is a surgical emergency that must be promptly addressed regardless of the specific cause. Here we report a case of colon perforation caused by indeterminate inflammatory bowel disease (IBD) in an autistic 13-year-old boy. Ulcerative colitis (UC) and lymphoma were first suspected but subsequently ruled out. The patient was previously hospitalized locally for 8 days due to diarrhea. He was diagnosed with UC and colon perforation in the emergency room. He then underwent subtotal colectomy with end ileostomy. Pathological examination of the colon showed multiple perforations with absence of chronic crypt change (a characteristic of UC), presence of undermining ulcers, and atypical lymphocyte infiltrations. Lymphoma was ruled out from immunohistochemistry and blood tests. Indeterminate colitis was finally suggested as the cause of perforation. Genetic analysis confirmed KBG syndrome, but no abnormalities otherwise known to be relevant to colitis. This case demonstrates that spontaneous colon perforation might occur in KBG syndrome patients suffering from severe enteritis without IBD, malignancy, or other conditions known to cause perforation, supporting the necessity of close monitoring when such patients present with severe symptoms including fever and abdominal distension without showing improvement.

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 aimed to compare clinical outcomes between thoracoscopic surgery (TR) and open surgery (OR) for esophageal atresia with distal tracheoesophageal fistula (EA with distal TEF) and to evaluate the feasibility of TR. Methods: We retrospectively analyzed the clinical data of 42 patients who underwent primary surgery for EA with distal TEF from January 2012 to December 2020. We compared the OR and TR groups based on patient characteristics, intraoperative outcomes, and early and late postoperative outcomes. Results: The TR group had longer operation times and a higher risk for intraoperative hypoxic events. Anastomosis leakage and stricture tended to occur more frequently in the TR group, although not statistically significant. The TR group required a significantly higher number of esophageal balloon dilatations (P = 0.006). Conclusion Successful TR for EA with distal TEF requires advanced anesthesia and efforts to overcome the learning curve of surgical skill with limited thoracoscopic instruments for neonates. Despite challenges, TR is considered a feasible method for EA with distal TEF patients when performed by experienced pediatric surgeons with appropriate anesthesia support.

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: Appendectomy is one of the most common surgeries in children. Although various radiological examinations are performed, they do not always reveal a definitive diagnosis of appendicitis. This study aimed to investigate the clinical course of equivocal appendicitis, identify the factors associated with appendectomy, and suggest appropriate management for these patients. Methods: Patients younger than 19 years who visited Seoul National University Bundang Hospital with a differential diagnosis of appendicitis from January 2013 to December 2017 were included. All participants conducted ‘appendiceal CT’ with a scoring scale of 1–5. The higher the score, the higher the likelihood of a radiologic diagnosis of appendicitis. We defined the appendicitis CT score of 2–4 as equivocal appendicitis (n = 143). Medical records were reviewed retrospectively for demographics, further examination as abdominal ultrasonography, and appendectomy status (yes or no). The mean follow-up period was 15.6 ± 71 days. Results: Equivocal appendicitis accounted for 16.7%. Additional ultrasonography test was performed in 24.5% (35 of 143). In total, 34 patients (23.8%) underwent appendectomy. Among the patients with appendiceal CT scores 2, 3, and 4, 4.9%, 50.0%, and 87.5% underwent appendectomy, respectively. Higher WBC count, higher appendicitis CT score, and readmission were significantly associated with appendectomy in patients with equivocal appendicitis. Conclusion Higher appendicitis CT score and WBC level were positively associated with appendectomy. Careful observation can be a treatment option in appendicitis CT score 2 or 3 groups. Appendectomy is the first-line treatment for patients with appendicitis score 4. Additional ultrasonography test is advisable to determine treatment modality for equivocal appendicitis.