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.

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
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.

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.

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.

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.

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
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
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 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.

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.

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.

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.

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.

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.

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.

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
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
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.

Jejunoileal atresia (JIA) is a rare congenital disorder in newborns leading to intestinal obstruction, necessitating urgent surgery. A delay in diagnosis can result in severe complications like sepsis, bowel perforation, and even death. Over the years, improvements in neonatal care, surgical techniques, and nutrition have led to a marked decrease in mortality rates for JIA patients. While the exact cause remains uncertain, it is thought to result from an ischemic insult during later in utero. JIA is categorized into 4 types, detailing the structure and implications of each type. Clinically, mothers may exhibit polyhydramnios, and infants usually present with symptoms like bilious vomiting and abdominal distension. A significant percentage of affected infants do not pass meconium within the first day of life. Diagnosis is commonly through abdominal radiographs, with some cases requiring contrast enemas. Once diagnosed, immediate surgery is advised, with the primary goal to restore intestinal function and length. Nowadays, the survival rate for JIA patients exceeds 90%. This improvement is attributed to a better understanding of the condition, advancements in surgical techniques, and the introduction of total parenteral nutrition. The prognosis varies, with short bowel syndrome being a significant factor determining the outcome. Short bowel syndrome’s prognosis depends on the remaining small bowel length, the ileocecal valve’s presence, and dependence on long-term nutrition. In conclusion, the treatment and prognosis for JIA have seen substantial improvements due to advances in medical care. Early diagnosis and intervention are crucial. While many patients lead a normal life, those with complications like short bowel syndrome may require long-term care. Future efforts should emphasize refining surgical techniques, gaining a deeper understanding of JIA, and enhancing postoperative care. There's also a need for more research into JIA’s genetics and etiology.

Posterior reversible encephalopathy syndrome (PRES) is a distinctive neurological disorder characterized by specific clinical and radiological features. While its etiology is multifaceted, the vasogenic theory provides a comprehensive framework for understanding its pathogenesis. Notably, PRES has been observed in cancer patients undergoing cytotoxic chemotherapy, immunotherapy, and select neurosurgical interventions. In this context, we present an unprecedented case involving a 7-year-old girl who developed PRES subsequent to abdominal surgery for adrenal neuroblastoma, a scenario hitherto unreported in medical literature. The patient’s medical history encompasses a diagnosis of stage IV neuroblastoma - high risk. Her treatment course comprised six cycles of neoadjuvant chemotherapy, followed by an open right adrenalectomy coupled with retroperitoneal lymph node dissection. Following surgery, the patient’s clinical profile evolved to encompass the classic symptoms of PRES, including headache, blurred vision, and elevated blood pressure. Neuro-imaging confirmed the diagnosis, with subsequent robust clinical recovery achieved by day 12. Continuation of adjuvant therapy in accordance with established protocols was pursued. Successful management of PRES pivots on several crucial principles: timely recognition, meticulous hydration, vigilant blood pressure control, and attentive intensive care. By adhering to these tenets, complete clinical recuperation can be realized, sparing patients enduring neurological deficits or vision impairment. This case underscores the importance of remaining vigilant for PRES in postoperative settings, particularly in pediatric patients undergoing surgical interventions for malignancies. Further studies and vigilance are warranted to expand our understanding of this intriguing condition and optimize therapeutic strategies.

Fournier’s gangrene is a life-threatening necrotizing fasciitis of genitalia and perineum. It is an exceedingly rare disease in infants and presents a diagnostic and therapeutic challenge for pediatric surgeons. Risk factors for Fournier’s gangrene in children include low birth weight, premature birth, trauma, burns, immunocompromising conditions, and sepsis. We report a very rare case of Fournier’s gangrene in a female infant. A 1-month-old girl visited the emergency room with a fever (39.2°C) and skin discoloration in the suprapubic area. The skin color change spread rapidly from the genitalia and inguinal area to the abdominal wall and flank. Ultrasonography and computed tomography demonstrated air bubbles in the subcutaneous layer of the suprapubic and inguinal areas, which strongly indicated Fournier’s gangrene. An emergency operation was performed; a low transverse incision was made in the suprapubic area to open subcutaneous tissue from skin to fascia and curettage and irrigation were performed. Necrotizing fasciitis improved dramatically after surgical treatment. The baby is now in good condition and has grown normally after discharge from the hospital. Clinical suspicion of Fournier’s gangrene and quick decision to surgery contributed to good prognosis. This report would be helpful to clinicians in diagnosing and treating infant patients with Fournier’s gangrene.

A choledochal cyst (CC) is an abnormal dilatation of the bile duct that is known to be a precursor of cholangiocarcinoma (CCA). Biliary intraepithelial neoplasia (BilIN) is a premalignant lesion that is considered to be found during the carcinogenesis of CCA. While BilINs are frequently identified in adult populations during pathology, there has been no report of them in the pediatric population. We first report a case of BilIN incidentally diagnosed from a CC in a 7-year-old child. This case suggests that BilIN should not be overlooked in children, especially those with risk factors such as anomalous pancreaticobiliary ductal union. Also, it supports the importance of early and complete resection of CC and its impact on preventing neoplastic changes in the biliary system.

Purpose
The
objective
of this study, conducted at a single center, was to determine the appropriate subgroup of pediatric appendicitis patients for nonoperative management (NOM) and evaluate the effectiveness of this approach. Furthermore, the study aimed to identify the factors that influence the likelihood of treatment failure.
Methods
Out of the 37 children diagnosed with acute appendicitis who underwent NOM, 31 patients successfully completed the treatment without the need for appendectomy. However, 6 patients experienced recurrence of symptoms and subsequently required appendectomy.
Results
The two groups of patients who experienced successful NOM and those who required appendectomy did not exhibit significant differences in terms of baseline characteristics, laboratory findings, or antibiotic treatment. Furthermore, factors such as the presence of appendicolith or perforation did not demonstrate a significant association with treatment failure. Additionally, the multivariable logistic regression analysis did not identify any predictors of recurrence.
Conclusion
The study emphasizes the viability of NOM even in cases of complicated appendicitis, especially in the era of coronavirus disease 2019 limiting the chance of surgical treatment. It has shown potential in reducing the risk of complications that may arise from early surgical intervention in high-risk patients.

Purpose
High jejunal atresia occurring near the ligament of Treitz is rare. It is a complicated type of small bowel atresia, and its treatment is challenging. Our study describes the clinical characteristics and proper management of high jejunal atresia.
Methods
We performed a retrospective review of seven neonates who underwent operations for high jejunal atresia between January 2000 and April 2021.
Results
The sex ratio showed a male predominance, and all patients were prenatally diagnosed with small bowel atresia. Six patients presented with a typical triple bubble appearance on the preoperative abdominal film. The location of the atresias were around the ligament of Treitz or less than 10 cm from the ligament of Treitz, and the types of atresia comprised three cases of type I, two of type IIIb, and two of type IV. Five of the seven patients underwent the operation without proximal enteroplasty. The median duration from operation to full feeding was 23 days, with three patients requiring more than a month to reach full feeding. There were no other operation-related complications. At the long-term follow-up, all patients tolerated oral feeding well.
Conclusion
This study indicates that high jejunal atresia close to the ligament of Treitz exhibits a high incidence of type I and complex types of atresia, and that primary anastomosis, without proximal enteroplasty, can be applied through surgical intervention.

Purpose
The Korean Association of Pediatric Surgeons (KAPS) has conducted annual national surveys, each year addressing a different topic regarding pediatric surgical diseases, and the data of these surveys are discussed at each respective annual spring meeting of KAPS.
Methods
In 2002 and 2017, KAPS conducted two national surveys for choledochal cysts. The authors reviewed the data from the national surveys and analyzed the differences or changes in demographic characteristics, clinical findings, diagnostic methods, treatment, and outcomes of pediatric patients with choledochal cysts who had been treated by KAPS members.
Results
A total of 348 and 241 patients with choledochal cysts were enrolled in the first and second national surveys, respectively. The male-to-female ratio was 1:3.4 and 1:2.7 in the first and second national surveys, respectively. In both national surveys, abdominal pain was the most common clinical symptom. The most common type of choledochal cyst was type I in 71.3% and 73.9% of patients in the first and second national surveys, respectively. In the first national survey, all 348 patients underwent open surgery. However, in the second national survey of 241 patients, open, laparoscopic, and robotic surgeries were performed in 102 (42.3%), 75 (31.1%), and 56 (23.2%) patients, respectively. Cyst excision with Roux-en-Y hepaticojejunostomy was performed in 339 (98.8%) of 343 patients in the first national survey and 240 (99.6%) of 241 patients in the second national survey.
Conclusion
These national surveys provide current status, general information, and comprehensive treatment and outcomes for pediatric patients with choledochal cysts in Korea. This study could provide significant knowledge and reference for pediatric surgeons seeking to better understand choledochal cysts and treatment options for this disease.

Caecal duplication cysts (DCs) are rare anomalies and presentation varies according to size, site, the mucosal lining of the cyst, and age of the patient. The rarity of the condition and varied clinical presentation makes it difficult to diagnose preoperatively. A high index of suspicion and intraoperative examination of the whole bowel is essential to localize intraluminal DC. We herein describe a case of an intraluminal caecal DC in an infant along with its presentation, management, and review of pertinent literature.

Jejuno-ileal atresia is often associated with various other gut pathologies. A 5-day male presented with bilious vomiting, abdominal distension and non-passage of stools. Exploratory Laparotomy showed a type IIIA ileal atresia with midgut volvulus and atypical malrotation for which Ladd’s Procedure and anastomosis of atretic bowel was done. A fleshy tubular structure extracted from distal bowel showed ileal duplication. In JIA, the vascular theory of pathogenesis is widely accepted with multiple studies to suggest the same. The mechanism being mesenteric ischemia leading to intestinal necrosis, resorption, and atresia. It has been found that abdominal duplication cyst often occurs simultaneously with short bowel, intestinal atresia, stenosis and enteric duplication for which a common vascular pathogenetic mechanism has been suggested. Our case serves as an in vivo description of the proposed theory where the findings may be described elegantly. We describe and discuss the mesenteric ischemia theory in bowel atresia and hope to further our understanding about the same.

Purpose
During the coronavirus disease 2019 pandemic, with an aim to reduce the burden on the strained health care resources, operating on suspected perforated appendicitis while managing simple appendicitis conservatively was followed in our institution. The aim of this study was to determine the predictive value of clinical features and investigations for discriminating between perforated and non-perforated appendicitis in children and devise a scoring system.
Methods
A retrospective study of all consecutive children who underwent appendectomy for acute appendicitis at our institution, a tertiary care pediatric center in South India, between 1st June 2020 to 31st May 2021 (period, one year) was carried out. In line with our standard operative procedure suspected perforated appendicitis were operated while simple appendicitis were managed conservatively in our institution. Patient demographics details, clinical features and investigations data were collected, and univariate and multivariate analysis carried out.
Results
Total of 58 patients were included in the study. Longer duration of symptoms, leukocytosis, serum sodium <135 mEq/L, appendicolith and free fluid and abscess on ultrasonography are good predictors of perforated appendicitis. We have evolved a reliable scoring system to identify perforated appendicitis.
Conclusion
Accurate identification perforated appendicitis with our scoring system is possible and results in effective usage of health care resources.

Purpose
Increases in intestinal length (IL) in patients with short bowel syndrome (SBS) may differ by age and underlying diseases. This study analyzed the elongation rate of the remnant IL according to underlying disease to establish a therapeutic plan for intestinal rehabilitation.
Methods
The charts of SBS patients who underwent surgery at our center from 2000 to 2018 were retrospectively reviewed. The enrolled patients included those with a remaining IL less than 25% of the average and those with a remaining IL of approximately 50% who had been administered parenteral nutrition for more than one year. Patients were excluded if they underwent only one operation or two operations with the first being a serial transverse enteroplasty procedure, meaning that base length was not known.
Results
Ten patients were enrolled. Causes of SBS included total colonic aganglionosis (TCA) in seven patients (70.0%), necrotizing enteritis in two (20.0%), and malrotation/volvulus in one (10.0%). The IL growth rate in the three non-TCA patients was 9-fold higher than the estimated proportion, whereas the growth rate of the IL according to age was significantly lower in the TCA than in the non-TCA group (p=0.01).
Conclusion
The IL growth rate may differ between TCA and non-TCA patients in SBS. Rehabilitation protocols might consider the underlying disease. Additional larger trials are required to confirm these findings.

Purpose
Enterostomies in premature infants are associated with a high incidence of complications. This study analyzed the factors associated with complications in very low birth weight (VLBW) infants who underwent enterostomy.
Methods
VLBW infants with preoperative weights below 1,500 g who underwent enterostomy between January 2003 and May 2018 were analyzed. The gestational age, corrected age, birth weight, weight at the time of surgery, surgery location, causative disease, laboratory findings, and complications were also analyzed.
Results
A total of 61 cases were included, consisting of 16 cases with stomal complications (26.2%); 12 prolapses (19.7%), 3 cases of parastomal hernias (4.9%), and 1 case of prestomal obstruction (1.6%). Premature infants born before 28 weeks gestation had approximately a three-fold increase in complications, albeit without statistical significance (p=0.11). These infants also had high incidences of bedside operations (p=0.003). Differences in surgical sites did not significantly impact the complication rate. Seven patients (11.5%) underwent reoperation for their complications. Twelve patients had prolapses, and six of these patients (50%) underwent reoperative surgeries. The mortality rate among the included patients was 24.6% (15 patients). These deaths were not associated with enterostomy, and seven deaths (46.7%) were attributed to neonatal sepsis and necrotizing enterocolitis.
Conclusion
VLBW infants, particularly those born before 28 weeks of gestation, typically have stomal complications. Therefore, surgical techniques should be aimed at minimizing stomal complications when operating on VLBW patients.

Purpose
Temporary stoma formation is a common procedure in pediatric surgery. Surgical site infection after stoma reversal surgery is a common complication, and the cosmetic effect after skin suturing is reduced. Purse-string closure has been suggested for stoma reversal, preventing surgical site infections and providing better cosmesis. Positive results have been reported abroad for the purse-string closure technique for stoma reversal in pediatric patients; however, no cases have been reported in Korea. Therefore, we aimed to investigate the outcomes of purse-string closure for stoma reversal in pediatric patients.
Methods
From October 2018 to September 2022, all pediatric patients who underwent stoma reversal at the Department of Pediatric Surgery were included. The electronic medical records of the patients were retrospectively reviewed. The following variables were analyzed: perinatal data including gestational age, sex, birth weight, Apgar score, and types of delivery; preoperative conditions including total parental nutrition, steroid use, and tracheal intubation; operational data such as diagnosis, types of stoma, age, body weight, skin closure methods and operation time; and postoperative data such as surgical site infection, transfusion, days until extubation, and start of enteral feeding. The serial wound healing process after purse-string closure was documented using photographs.
Results
Thirty-seven patients underwent stoma reversal surgery, 14 underwent purse-string closure, and 23 underwent conventional lineal closure. No surgical site infection occurred in the purse-string group and four cases of surgical site infection occurred in the conventional liner group. However, there were no statistically significant differences between both groups (p=0.276).
Conclusion
No surgical wound infection was found in pediatric patients who underwent purse-string closure for stoma reversal, and the cosmetic effect was promising. However, no statistically significant difference was found between the purse-string closure and conventional linear closure groups. Further multicenter studies involving a larger sample size are needed to determine statistical significance.

Purpose
The aim of this study was to compare the clinical features and examine the safety and feasibility of laparoscopic surgery versus open surgery in children with solid pseudopapillary tumors (SPTs).
Methods
Patients under age 18 diagnosed with SPT who underwent surgery for the condition at our single institution from January 2005 to December 2019 were retrospectively analyzed. The demographics and postoperative outcomes in the two groups were compared according to the surgical method and tumor locations.
Results
The 25 patients consisted of 23 females and two males with a mean age at surgery of 13.2 years (range, 6 to 18 years). The lesions were located in the pancreatic head (n=7, 28.0%), and body or tail (n=18, 72.0%). Fifteen patients (60.0%) were treated with laparoscopic surgery. In all patients, there was no difference in intraoperative and postoperative outcomes between the two groups. When analyzed according to tumor location, complications including postoperative pancreatic fistula did not show significant differences with the surgical method in both the head group and body or tail group.
Conclusion
Compared to open surgery, the clinical outcomes and complication rate of the laparoscopic approach in pediatric SPT patients was not significantly different. Therefore, laparoscopic surgery for SPTs in pediatric patients may be a feasible option.

Hydatid disease and tuberculosis are both endemic in India. The involvement of the kidney in any of these diseases is rare and co-occurrence of both pathologies at the same time is extremely rare. The common characteristics of both conditions i.e., remain asymptomatic for a long duration and present with non-specific symptoms make the diagnosis challenging. Moreover, the treatment of both pathologies at the same time requires multifaceted medical management and surgical intervention at the optimal time. Here, we are describing a case of renal hydatid with tuberculosis in a child, its presentation and management along with the review of pertinent literature.

Entomophthoromycosis is a rare fungal infection, infecting the immune competent children, in the tropical and sub-tropical areas. It is caused by Basidiobolus ranarum, which belongs to the order Entomophthoral. The diagnosis is delayed, as it presents with non-specific symptoms. The disease occurs following a trivial injury or insect bites and therefore presents as subcutaneous nodules over the trunk, buttock and thighs. Gastrointestinal (GI) infection and invasive spread are very rare. Very few cases of GI disease and liver entomophthoromycosis have been reported in the English literature. The diagnosis is confirmed on histopathology and fungal cultures. We discuss a case of liver entomophthoromycosis in a 3-year-old child, who presented with non-specific symptoms and palpable lump in the epigastric region. The child was worked up for malignancy and underwent left hepatectomy. Later, the histopathology report revealed entomophthoromycosis. Delay in diagnosis can be fatal. Entomophthoromycosis is an emerging fungal infection and should be an important differential in patients presenting with vague symptoms and lump abdomen.

Approximately 5% of Wilms tumors are bilateral. Multidisciplinary care including surgery, chemotherapy, and radiation has greatly improved the survival rates. With long-term survivorship, treatment should be planned to ensure quality survival. Nephron-sparing surgery (NSS) is now a recommended alternative to bilateral nephrectomy with renal replacement therapy in bilateral Wilms tumors. However, the feasibility of bilateral NSS is dependent on multiple factors like the size, location of the tumor, and response to chemotherapy. In advanced bilateral tumors, a) prolonging preoperative chemotherapy to achieve optimum response for performing a salvageable renal surgery and b) administering chemotherapy in between the staged nephrectomies, is an effective strategy. Response assessment with imaging will help in a) selection of appropriate surgery, b) timing and c) sequencing of surgery. We present a patient with bilateral Wilms tumor managed with neoadjuvant chemotherapy followed by staged bilateral NSS followed by adjuvant chemotherapy at our institute. The patient is alive with a follow-up of 5 years.

Purpose
Although total parenteral nutrition (TPN) is essential for patients with intestinal failure, intensive monitoring and management are required to provide sufficient nutrition and prevent complications. The adverse effects of prolonged TPN >5 years were evaluated in pediatric patients, and preventative methods and treatments were analyzed.
Methods
Pediatric surgery patients at a single center who had received parenteral nutrition (PN) for more than 5 years at a single center were analyzed retrospectively. Adverse effects and treatments were determined by review of their medical records. Complications were defined as catheter-related, metabolic, or nutritional.
Results
Seven patients receiving long-term PN had short bowel syndrome (SBS), including two each with necrotizing enterocolitis, congenital atresia, and pseudo-obstruction, and one with Hirschsprung’s disease. Six patients had a mean 6.4 ± 4.4 line infections (median: 6, range: 3–15). Central venous catheters were replaced a mean 7.3 ± 4.5 times (median: 8.5, range: 1–12). None of these patients had bilirubin concentrations above 2 mg/mL, and none experienced acute or chronic kidney injuries. Six children received vitamin D supplements, and one experienced rickets. Laboratory tests revealed a temporary lack of zinc or selenium, but no associated clinical symptoms. None had a vitamin B12 or folate deficiency. All seven patients were hospitalized for electrolyte imbalance a median 2.5 times (range: 1–17); all recovered with hydration and TPN. At the time of this writing, all seven patients remained alive, with below-average growth, and two were treated with growth hormones.
Conclusion
Appropriate catheter insertion, guardian education and management, as well as active responses to complications, are important-required to safely maintain long-term parenteral nutrition.

Purpose
To report the clinical progress and findings seen in pediatric airway surgeries performed by a single pediatric surgeon.
Methods
We retrospectively reviewed 32 pediatric patients with various airway diseases who underwent surgical correction performed by a single pediatric surgeon between January 2005 and March 2021. Surgical indications depended on the presence and severity of respiratory symptoms. Surgical results were considered good if symptoms improved and normal respiration was possible without dependence on a ventilator or tracheostomy, without postoperative complications, at the last postoperative follow-up.
Results
A total of 32 patients who underwent airway surgery were diagnosed with 3 types of surgical airway diseases: tracheomalacia (n=19), subglottic stenosis (n=10), and tracheal stenosis (n=3). In the treatment for tracheomalacia, surgical efficacy was 88.2% for aortopexy and 100.0% for tracheopexy. The postoperative mortality rate of slide thyrocricotracheoplasty for subglottic stenosis and slide tracheoplasty for tracheal stenosis was 20.0%. The age at the time of all airway surgeries ranged from 1.0 to 127.6 months (median, 4.8 months). The postoperative follow-up period ranged from 1.2 months to 16.6 years (median, 4.5 years).
Conclusion
Severe pediatric airway diseases with obstructive symptoms are life-threatening and require surgical treatment. Pediatric airway surgery performed in this study by a single pediatric surgeon had similar or better outcomes than in previous studies. However, more accurate diagnostic methods and improved surgical procedures for pediatric airway diseases require development.

Purpose
Anorectal malformations (ARMs) represent a wide spectrum of anomalies with various presentations and associated anomalies. The management of ARMs is still controversial and various managements for ARMs have been proposed. The aim of this study was to identify the status of ARMs in Korea and to analyze the data regarding classification, treatment and functional outcomes of ARMs among the Korean Association of Pediatric Surgeons (KAPS) members.
Methods
The KAPS has conducted a national survey for ARMs in 1999 and 2015 to analyze the differences and changes in the classification, treatment, and functional outcomes of ARMs.
Results
A total of 295 and 619 ARMs patients were enrolled in the second and third national survey, respectively. The most common type among male and female was the low type in the second national survey and low defects (cutaneous fistula, anal stenosis, membrane) in the third national survey. Most common associated anomalies were genitourinary system in the second national survey and cardiovascular system in the third national survey. Various surgical options including anoplasty, posterior sagittal anorectoplasty (PSARP) and laparoscopic surgery with or without colostomy were performed. Unfortunately, comparison regarding functional outcomes between 2 national surveys was not possible due to the use of different classification and functional assessment criteria.
Conclusion
We suggest making an ARMs registry at a national level so that multicenter functional outcome data can be collected for a better understanding and management of this rare anomaly.

Infantile hemangiomas are the most common vascular tumors of infants and are characterized by abnormal proliferation of endothelial cells and aberrant blood vessel architecture. The drug Sirolimus has been explored as a new therapeutic option in the management of vascular anomalies that do not respond to conventional treatments. This report presents a case of refractory multiple infantile hemangiomatosis with brain involvement that was successfully treated with sirolimus.

Catecholamine-producing tumors are rare, and the classical symptoms include maintained or paroxysmal hypertension, headaches, palpitations and sweating. However, its symptoms can vary greatly, which is why it has been called “the great masquerader.” We are reporting case of a patient with a left adrenal pheochromocytoma with multiple abdominal paragangliomas who presented with severe ischemic involvement of both the upper and lower limbs due to catecholamine induced vasospasm.

Gallstone ileus occurs when there is a fistula between the gallbladder and the small intestine, by which the gallstone reaches the small bowel and thereby inducing bowel obstruction. This condition is very rare in the overall population, accounting for only 0.1–5%. It is also very rare in the pediatric population and in the patients who undergo Kasai operation as well. Worldwide, only two cases have been reported. Gallstone production may be facilitated under certain circumstances, and migrated stone induces ileus. Diagnosis is usually done by imaging studies such as abdominal ultrasonography and computed tomogram (CT). Surgical removal of stone is decisive treatment. The 22-year-old female patient with a history of Kasai operation at age of 1 month visited emergency department for abdominal pain. The most reasonable impression was the acute exacerbation of the chronic cholangitis, but it was improved soon after intravenous antibiotics treatment. At the same time, mechanical ileus was diagnosed and exacerbated during admission. CT was done to find out the cause. Small bowel obstruction by a round material was observed. By considering its clinical course and the imaging, the material was suspected to be gallstone. The patient underwent enterolithotomy under general anesthesia. She was fully recovered from gallstone ileus after stone removal. There has been no recurrence of ileus nor cholangitis. When a patient with a history of Kasai portoenterostomy complains mechanical ileus, the clinician need to allow for gallstone ileus referring patient’s history and medical condition which promotes to induce stone formation.

Biliary atresia is a progressive sclerosing cholangiopathy of bile ducts. Most of the time it is an isolated anomaly but can present with syndromic forms. The biliary atresia splenic malformation (BASM) syndrome is associated with splenic anomalies, vascular anomalies, and visceral asymmetry along with biliary atresia. The surgical anatomy of BASM is distinctive and creates a challenge for the surgeons. We are describing a case of BASM with situs inversus and highlighting the approach, intraoperative anatomy, and surgical intricacies of Kasai portoenterostomy in such a situation with the review of pertinent literature.

Purpose
Gastroesophageal reflux disease (GERD) frequently affects patients with esophageal atresia (EA). GERD is often resistant to medical therapy, and anti-reflux surgery in the form of fundoplication is required. However, there are no generally accepted indications for fundoplication in EA patients with significant gastroesophageal reflux (GER). This study aimed to report the outcomes and review the clinical characteristics and treatment of GERD with Nissen fundoplication among EA patients.
Methods
The medical records of patients with EA repair who underwent Nissen fundoplication for GERD treatment from January 2005 to December 2018 were retrospectively reviewed. Twenty patients were included in this study.
Results
In total, 166 patients with EA underwent treatment during the study period. Among these, 20 (12%) patients underwent Nissen fundoplication. The median age at the time of fundoplication was 5.3 (range, 1.6–49.4) months, and the mean body weight was 6.3±2.4 kg. The indications for Nissen fundoplication were hiatal hernia in 18 (90%) patients, feeding intolerance in 17 (85%), recurrent aspiration pneumonia in 13 (65%), GER-related refractory anastomosis stenosis in seven (35%), and reflux-related acute life-threatening events in five (25%). The median follow-up duration after Nissen fundoplication was 5.7 (range, 2.4–10.7) years; two (10%) patients experienced surgery-related complications, 11 (55%) had recurrent hiatal hernia, and three (15%) required re-operation. Feeding intolerance improved (p<0.001), and the number of esophageal dilatations decreased following Nissen fundoplication (p=0.038). There was no mortality during the follow-up period.
Conclusion
Nissen fundoplication was required in patients with more complicated EA and was performed at a young age. Nissen fundoplication was relatively effective in controlling EA-associated GERD. However, Nissen fundoplication in patients with EA may be associated with a high rate of recurrent hiatal hernia. A careful follow-up is necessary because poor long-term outcomes are common for such patients.

Purpose
Report of a nationwide survey on lymphangioma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2019.
Methods
The authors reviewed and analyzed the clinical data of pediatric patients who started treatment for lymphangioma in hospitals of KAPS members from 2011 to 2013. Their follow-up data is also included in the study.
Results
A total of 532 patients with lymphangioma from 18 institutes were registered for the study. The results were discussed at the 35th annual meeting of KAPS, which was held in Gyeongju on June 13–14, 2019.
Conclusion
This study provides general information on lymphangioma and comprehensive treatment outcomes for this disease. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of lymphangioma.

Intussusception recurs in 10% to 20% of childhood intussusception after successful nonoperative reduction. Multiple recurrences of intussusception are associated with the presence of a pathologic lead point. We describe a case of a 9-month-old boy with 4 times recurrent intussusception after nonoperative reduction caused by diffuse large B cell lymphoma lesion of the ileum. Clinical suspicion and surgical approach is needed to find rare cause of recurrent intussusception.