Advances in Pediatric Surgery

[English]

A Comparative Study of Three National Surveys on Biliary Atresia by the Korean Association of Pediatric Surgeons: Yeon Jun Jeong, Dayoung Ko, Hyunhee Kwon, Ki Hoon Kim, Dae Yeon Kim, Soo-Hong Kim, Wontae Kim, Hae-Young Kim, Hyun Young Kim, Seong Chul Kim, Younghyun Na, Jung-Man Namgoong, So Hyun Nam, Junbeom Park, Jinyoung Park, Tae-Jun Park, Jeong-Meen Seo, Ji-Young Sul, Joonhyuk Son, Hyun Beak Shin, Joohyun Sim, Soo Min Ahn, Hee Beom Yang, Jung-Tak Oh, Chaeyoun Oh, Joong Kee Youn, Sanghoon Lee, Ju Yeon Lee, Kyong Ihn, Hye Kyung Chang, Eunyoung Jung, Jae Hee Chung, Yu Jeong Cho, Yun Mee Choe, Soo Jin Na Choi, Seok Joo Han, In Geol Ho, Ji-Won Han; Adv Pediatr Surg 2025;31(2):47-58. Published online July 16, 2025; DOI: https://doi.org/10.13029/aps.2025.31.2.47

Abstract
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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.

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[English]

Comparative Analysis of National Surveys of Intestinal Atresia: A Retrospective Study by the Korean Association of Pediatric Surgeons: Jinyoung Park, Dayoung Ko, Eun-jung Koo, Hyunhee Kwon, Ki Hoon Kim, Dae Yeon Kim, Seong Chul Kim, Soo-Hong Kim, Wontae Kim, HaeYoung Kim, Hyun-Young Kim, So Hyun Nam, Jung-Man Namgoong, Junbeom Park, Taejin Park, Min-Jung Bang, Jeong-Meen Seo, Ji-Young Sul, Joonhyuk Son, Joohyun Sim, Soo Min Ahn, Hee-Beom Yang, Jung-Tak Oh, Chaeyoun Oh, Joong Kee Youn, Sanghoon Lee, Ju Yeon Lee, Kyong Ihn, Hye Kyung Chang, Yeon Jun Jeong, Eunyoung Jung, Jae Hee Chung, Min Jeong Cho, Yun-Mee Choe, Seok Joo Han, In Geol Ho, Jeong Hong; Adv Pediatr Surg 2025;31(1):8-15. Published online May 28, 2025; DOI: https://doi.org/10.13029/aps.2025.31.1.8

Abstract
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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.

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[English]

National Survey of Gastroschisis and Omphalocele by Korean Association of Pediatric Surgeons: Yeon Jun Jeong, Dayoung Ko, Eun-Jung Koo, Hyunhee Kwon, Dae Yeon Kim, Soo-Hong Kim, Wontae Kim, Hae-Young Kim, Hyun Young Kim, Seong Chul Kim, Younghyun Na, Jung-Man Namgoong, So Hyun Nam, Sungjoo Park, Junbeom Park, Jinyoung Park, Tae-Jun Park, Jeong-Meen Seo, Ji-Young Sul, Joonhyuk Son, Hyun Beak Shin, Joohyun Sim, Jung-Tak Oh, Chaeyoun Oh, Joong Kee Youn, Sanghoon Lee, Ju Yeon Lee, Cheolgu Lee, Kyong Ihn, Eunyoung Jung, Jae Hee Chung, Yong-Hoon Cho, Yun Mee Choe, Soo Jin Na Choi, Seok Joo Han, In Geol Ho; Adv Pediatr Surg 2024;30(2):39-51. Published online December 13, 2024; DOI: https://doi.org/10.13029/aps.2024.30.2.39

Abstract
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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.

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[English]

Clinical and Imaging Diagnosis of Vascular Anomaly: Experience of 137 Patients From a Single Center: Sujin Gang, Hyunhee Kwon, Jueun Park, Dae Yeon Kim; Adv Pediatr Surg 2024;30(2):52-63. Published online December 12, 2024; DOI: https://doi.org/10.13029/aps.2024.30.2.52

Abstract
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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.

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[English]

Robot-Assisted Hepatectomy in Pediatrics: Two Case Reports of Single Center: Jueun Park, Suhyeon Ha, Hyunhee Kwon, Jung-Man Namgoong, Dae Yeon Kim; Adv Pediatr Surg 2024;30(2):73-79. Published online June 10, 2024; DOI: https://doi.org/10.13029/aps.2024.30.2.73

Abstract
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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.

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[English]

Sirolimus Treatment of Complicated Vascular Anomalies: A Single Center’s Experience Over 2 Years: Jueun Park, Suhyun Ha, Hyunhee Kwon, Jung-Man Namgoong, Hee Mang Yoon, Pyeong Hwa Kim, Dae Yeon Kim; Adv Pediatr Surg 2024;30(1):14-22. Published online June 10, 2024; DOI: https://doi.org/10.13029/aps.2024.30.1.14

Abstract
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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.

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[English]

Perioperative Outcomes and Surgical Indications of Minimally Invasive Pancreatectomy for Solid Pseudopapillary Tumor in Pediatric Patients: Chong Won Lee, Jung-Man Namgoong, Dae Yeon Kim, Seong Chul Kim, Soo Young Lee, Yujeong Cho, Hyunhee Kwon; Adv Pediatr Surg 2018;24(2):76-85. Published online December 10, 2018; DOI: https://doi.org/10.13029/aps.2018.24.2.76

Abstract
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Purpose

We evaluated perioperative and long-term outcomes of minimally invasive surgery (MIS) and established indications of MIS in solid pseudopapillary tumor (SPT) in pediatric patients.

Methods

From October 1992 to April 2018, 66 patients (age, <18 years) diagnosed with SPT underwent either open pancreatectomy (OP) or MIS. Variables including postoperative complications and recurrence rates were retrospectively analyzed.

Results

Thirty-five patients underwent open surgery and 31 underwent laparoscopic/robotic surgery. Mean tumor size in MIS was significantly smaller than that in OP (4.3±1.8 cm vs. 7.6±3.5 cm, p=0.005). There were 4 cases of open conversion from laparoscopic surgery because of vessel encasements (n=2), bleeding (n=1), and pancreatic ductal injury (n=1). Solitary pseudopapillary carcinoma was diagnosed in 6 patients. Recurrence was observed in 3 and 1 patients who underwent OP and MIS, respectively (p=0.634). Tumor size, mass size/abdominal diameter (MS/AD) ratio, and degree of the portal or superior mesenteric vein involvement were the most important indications for MIS.

Conclusion

MIS is being widely used in pediatric surgeries with increased expertise and safety, especially in pancreatic diseases. Careful patient selection for MIS in regards with parameters such as MS/AD ratio and vessel abutment might be a feasible choice.

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[English]

Clinical Review of Spontaneous Neonatal Gastric Perforation: Hyunhee Kwon, Ju Yeon Lee, Jung-Man Namgung, Dae Yeon Kim, Seong Chul Kim; J Korean Assoc Pediatr Surg 2017;23(2):37-41. Published online December 20, 2017; DOI: https://doi.org/10.13029/jkaps.2017.23.2.37

Abstract
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Purpose

Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors.

Methods

There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed.

Results

The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study.

Conclusion

There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.