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: To report the findings of a perception survey on intestinal malrotation conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2021. Methods: The perceptions on intestinal malrotation regarding clinical decision making of the KAPS members were collected through web-based survey. Results: A total of 22 surgeons were answered for this study. The results were presented and discussed at the 37th annual meeting of KAPS, which was held in Seoul on June 18, 2021. Conclusion This study provides the clinical decisions of the KAPS members on the intestinal malrotation. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of intestinal malrotation.

Hepatobiliary ascariasis (HA) is a rare condition associated with significant morbidity. Laparoscopic extraction of Ascaris lumbricoides from the biliary tract is a safe approach in patients who do not improve with antihelminthic treatment and when retrograde endoscopic cholangiography is not feasible or when not all nematodes can be removed using this method. Here I present the technique used in two pediatric patients with HA.

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.

Intrapulmonary bronchogenic cysts are rare congenital anomalies that often present diagnostic and management challenges due to nonspecific symptoms. We report a one-year-old female with progressive respiratory distress who was initially misdiagnosed with pneumothorax. Imaging revealed a large intrapulmonary cyst, and surgical excision confirmed the diagnosis. Early intervention ensured a favorable outcome, with no recurrence in one year. This case highlights the importance of considering bronchogenic cysts in pediatric respiratory distress and emphasizes the value of timely surgical management.

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 aimed to evaluate postoperative outpatient follow-up practices among pediatric surgeons in Korea for five common congenital diseases: esophageal atresia with tracheoesophageal fistula (EA/TEF), anorectal malformation (ARM), Hirschsprung’s disease (HSCR), choledochal cyst (CC), and inguinal hernia (IH). Methods: A web-based survey consisting of 43 questions was distributed to members of the Korean Association of Pediatric Surgeons. The survey assessed the timing, frequency, and duration of outpatient follow-up, as well as disease-specific practices. Results: Of 154 invited surgeons, 45 (29.2%) responded. Most scheduled the first follow-up visit within one week after discharge. During the first postoperative year, follow-up visits were commonly held every three months, followed by six months or annual intervals. Most surgeons concluded follow-up before age 18; however, 15.6%–37.8% reported continuing follow-up into adulthood depending on the disease. Variation was observed in disease-specific practices: 44.4% routinely performed contrast studies for EA/TEF follow-up; sizes #14–15 Hegar dilators were most used in ARM; only 6.7% performed routine rectal irrigation in HSCR. For CC, 88.9% checked both blood tests and ultrasonography. Most IH patients received only one follow-up visit. Conclusion While early postoperative follow-up practices among pediatric surgeons in Korea appear relatively consistent, wide variation exists in long-term strategies and disease-specific protocols. This reflects the tendency to rely on individual clinical judgment and highlights the need for standardized, national consensus.

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.

Intestinal failure (IF) is a term used to define the state where intestine’s function is significantly reduced, to the point where adequate growth and hydration cannot be maintained. In such cases, intravenous nutritional support is essential for sustaining the patient’s life. In pediatric patients, the most common cause of IF is short bowel syndrome (SBS). Due to the prolonged treatment and high complication rates, management of SBS remains a continuous challenge to many physicians. Herein, we report the case of a 2,260 g premature female infant born at 35-week gestational age with type 4 jejunoileal atresia. She presented with ultrashort bowel syndrome, having a bowel length of less than 15 cm, but ultimately achieved gut autonomy and restored bowel function through successful intestinal rehabilitation within the first two years of life.

A ciliated foregut cyst is a rare developmental anomaly. It develops from the primitive foregut. It is usually located supra-diaphragmatically. Its localization in the gallbladder is very infrequent and has been sparsely reported. We report a rare case of a ciliated cyst of the gallbladder in an 11-year-old female, who presented with complaints of upper abdominal pain for 2 months. She was suspected to have gallbladder duplication or gallbladder diverticulum on imaging. The histopathology reported this anomaly as a ciliated foregut cyst. The ciliated cyst of the gallbladder is a benign congenital lesion. Abdominal ultrasonogram and computed tomography/magnetic resonance imaging are suggestive of a cystic lesion of the gallbladder. The definitive diagnosis is by histopathological examination. This is a rare clinicopathological condition in the pediatric age group. The recommended treatment is laparoscopic cholecystectomy. The role of conservative management has not been established due to the rarity of the condition.