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
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
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.

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.

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.

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.