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
The Korean Association of Pediatric Surgeons (KAPS) conducts annual nationwide surveys on various aspects of pediatric surgical diseases, with the results being discussed during KAPS’s annual spring meetings.
Methods
KAPS conducted two national surveys, in 1995 and 2016, to investigate esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). The authors analyzed data from these surveys to identify differences or changes in the annual occurrence, demographic characteristics, clinical presentation, preoperative diagnostic methods, anatomical type, associated anomalies, surgical treatment, and postoperative outcomes among patients with EA/TEF treated by KAPS members.
Results
The first and second national surveys included 148 and 211 patients with EA/TEF, respectively. Excessive salivation was the most prevalent clinical symptom in both surveys. Type C was the most common form of EA/TEF in both surveys. The first survey included 126 patients, all of whom underwent open surgery. In the second survey, 152 (78.4%) of 194 patients underwent open surgery, while 34 (17.5%) underwent thoracoscopic surgery. Primary esophageal repair was performed on 96 (76.2%) of 126 patients in the first survey and on 160 (82.5%) of 194 patients in the second survey. Anastomotic strictures developed in 21.4% and 32.5% of patients, anastomotic leakage in 22.2% and 10.3%, recurrent fistula in 2.4% and 4.2% during the first and second surveys, respectively. The respective survival rates for group A were 90.2% and 98.3% in the first and second surveys. For group B, the rates were 73.9% and 98.1%, and for group C, they were 34.5% and 68.1%, respectively, according to the Waterston classifications.
Conclusion
These nationwide surveys provide comprehensive information on the status, detailed treatment, and outcomes for Korean pediatric patients with EA/TEF. They are anticipated to be an invaluable resource and guide for pediatric surgeons seeking to expand their knowledge on EA/TEF and its treatment options.

Purpose
The establishment of enteral feeding is the end point of any intestinal anastomosis. This study examined the effects of early feeding (EF) as compared to delayed feeding (DF) on postoperative outcomes after intestinal anastomosis in children.
Methods
This was a randomized controlled pilot study to assess the effect of EF vs. DF in terms of time to reach full feed, along with wound infection and anastomotic leak.
Results
Twenty-eight patients were enrolled in both study groups. The median time to first feed in EF was 60 hours and 96 hours in DF. The median time to first bowel sound was 42 hours in EF and 48 hours in DF (p=0.208). The median time to first bowel movement was 72 hours in EF and 72 in DF (p=0.820). The median time of postoperative hospital stay was 5.5 days in EF and 6.0 days in DF (p=0.01). There was no significant difference in complications of wound infection, wound dehiscence, relook surgery, or anastomotic leak in both groups.
Conclusion
EF after intestinal anastomosis is safe and feasible in children after intestinal anastomosis.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Duodenal web (DW) is a rare congenital anomaly of the intestinal tract that can lead to severe dehydration and electrolyte imbalance. A 14-day-old boy presented with recurrent vomiting and weight loss and was diagnosed with DW. Duodenoscopy identified a pinhole structure in the second portion of the duodenum, prompting a subsequent endoscopic balloon dilatation procedure. Following the procedure, vomiting and abdominal distension resolved, and the patient was discharged on a regular diet. No symptoms recurred during follow-up.

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
The International Society for the Study of Vascular Anomalies (ISSVA) classification is crucial in diagnosing vascular anomalies (VAs), surpassing the International Classification of Diseases 10th Revision. This study aims to reevaluate diagnoses using ISSVA criteria and explore diagnostic patterns.
Methods
Analyzing 138 pediatric VA patients diagnosed via magnetic resonance imaging from 2018 to 2023 at Asan Medical Center, we reviewed clinical, imaging, pathology, and genetic data. Diagnoses were revised per 2018 ISSVA criteria, assessing discrepancies.
Results
Among 133 VA cases, 125 were malformations and eight were tumors, mostly in the head and neck. Clinical and imaging diagnoses disagreed in 51 cases. Some initially complex malformations were simplified. Lymphatic malformation cases shifted to VAs and vascular tumors were identified post-initial diagnosis.
Conclusion
Accurate diagnosis of VAs is essential for prognosis, treatment planning, and predicting outcomes. However, 14.2% of patients showed discordance between clinical diagnoses and imaging findings. Capillary malformations were often overlooked in imaging but became evident with relevant clinical findings. Adopting a multidisciplinary approach and a unified diagnosis based on ISSVA classification is crucial for clearly defining VAs.

The concurrent occurrence of colonic atresia, malrotation, and Hirschsprung’s disease in neonates is extremely rare. These anomalies often share embryologic origins and present overlapping clinical symptoms that complicate diagnosis and management. We report two neonatal cases with this rare triad. Case 1 involved a term neonate initially diagnosed with esophageal atresia and later found to have colonic atresia, malrotation, and Hirschsprung’s disease. Case 2 was a preterm neonate presenting with abdominal distension and perforation, ultimately diagnosed with the same triad. Both underwent staged surgical management, including Duhamel’s procedures after confirming aganglionosis. Awareness of the possible coexistence of these anomalies is essential in neonates with colonic atresia and non-fixed colon. Surgical planning should anticipate aganglionosis and include rectal biopsy. This report emphasizes the importance of early suspicion and multidisciplinary approach for optimal outcomes.

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.

Purpose
Complicated vascular anomalies, characterized by encasing vital organ or diffusely locating unresectable lesion, pose therapeutic challenges with limited response to conventional treatment such as surgical resection or sclerotherapy. Sirolimus, an mammalian target of rapamycin inhibitor, has shown promising therapeutic effects in patients with vascular anomalies by inhibiting vascular endothelial growth factor, as reported in several studies. Here, we analyzed the treatment outcomes of patients who received sirolimus for complicated vascular anomalies at our institution.
Methods
Patients treated with sirolimus at the Department of Pediatric Surgery, Asan Medical Center from January 2018 to December 2021 were included. Sirolimus was administered twice daily at a dose of 0.8 mg per body surface area (BSA), with dose adjustments to achieve a target drug concentration of 8–12 ng/mL. Adverse drug effects and therapeutic responses were periodically assessed. Treatment efficacy was evaluated based on clinical findings pre- and post-sirolimus administration, absolute volume reduction of lesions through imaging tests (magnetic resonance imaging; MRI), and relative volume reduction adjusted to the patient's BSA.
Results
There were 16 females (50.0%) and 16 males (50.0%), with a median follow-up period of 41 months after sirolimus administration. Vascular anomaly types included lymphatic malformations (41%), venous malformations (28%), lymphovenous malformations (19%), and others (12.5%). The most common adverse effect was oral ulcer (6 patients). MRI volumetry revealed volume decreases in 17 patients (53.1%) with 22 patients (71%) exhibited lesion decreases relative to BSA. Notably, 9 patients (28.1%) had markedly decreased volume reduction based on absolute volume, and 12 (38.7%) based on volume compared to BSA.
Conclusion
Over a 2-year follow-up, sirolimus was effective in treating patients with complicated vascular anomalies, when administered with cautious consideration of side effects. A multidisciplinary approach is needed for evaluating treatment outcomes in these patients, necessitating further long-term research on adverse effects.

Purpose
Crohn’s disease (CD) is mainly presenting gastrointestinal symptoms but also may involve perianal diseases, with anal fistula being the most common. Anal fistula surgery performed without recognition that the patient has CD increases the complications such as delayed wound healing and anal sphincter injury. This study aimed to investigate clinical symptoms and surgical findings that could allow early diagnosis of CD in patients aged 10 to 19 years who underwent anal fistula surgery.
Methods
Among the 320 patients under the age of 19 who underwent anal fistula surgery, those who were diagnosed with tuberculosis fistula were excluded, medical records of 316 patients were examined. We investigated the characteristics of anal fistula and postoperative wounds as well as the colonoscopic and surgical findings.
Results
Compared to 272 patients not diagnosed with CD (non-CD group), 44 patients diagnosed with CD (CD group) showed significantly higher levels of C-reactive protein as well as anal discharge, family history of inflammatory bowel disease, history of anal fistula, diarrhea, abdominal pain, weight loss, complex anal fistula, recurred anal fistula, delayed wound healing and friable/edematous/granulation of surgical site.
Conclusion
For patients aged 10–19 years with anal fistulas, emphasizing early suspicion and an active diagnostic work-up is essential for early diagnosis of CD.

Hirschsprung disease (HSCR) is a genetic disorder with an incidence of 1:5000, seen in the pediatric age group. The association between HSCR and neuroblastoma (NBL), ends of the neurocristopathy spectrum is rare. Less than 10 cases of this association are reported in the literature and the association between the Phox gene and Sox10 gene in the pathophysiology of these is being studied. We report a one-year-old baby, who presented to us, with chronic constipation on regular enemas and laxative usage. There was a history of delayed passage of meconium. At the time of Duhamel’s pull through a well-defined, bilobed hard presacral mass, was encountered. Excision and coccygectomy were done and the pull was completed. The histopathology showed a well-differentiated NBL. Fludeoxyglucose positron emission tomography scan and the N-Myc amplification were negative and the patient was managed with expectant treatment. She is doing well over a 3-year follow-up with no recurrence and good resolution of bowel functions.

Morgagni hernia (MH) is a type of congenital diaphragmatic hernia that is rare and without any distinctive presentation. Chest radiographs can miss the diagnosis when solid organs instead of bowel loops are herniated. Echocardiography can perplex the diagnosis instead of aiding if MH is not suspected. We are here discussing the presentation and management of a neonate with MH, which was referred to our institute as a congenital heart disease.

Since the first introduction of robotic surgery systems in Korea in 2005, there has been a gradual increase in the number of robotic surgeries performed. However, robotic liver resection is one of the most complex procedures, and its application, especially to children, is still limited. Therefore, in this study, we aim to present our experiences with 2 pediatric patients who underwent robotic liver resection in Asan Medical Center and discuss the safety and feasibility of robot-assisted hepatectomy in pediatrics.