Purpose: Inguinal hernia (IH) repair is very commonly performed in children. While open repair (OR) is the standard approach, laparoscopic repair is increasingly used. This study was aimed to investigate safety and feasibility of laparoscopic repair of pediatric IH compared to OR. Methods: We retrospectively enrolled 105 pediatric patients with IH repair between January 2011 and October 2019. The laparoscopic procedures performed were laparoscopic percutaneous extraperitoneal closure (LPEC), and three-port mini-laparoscopic repair (TLR). The OR was performed as per usual technique. Results: Thirty-nine patients underwent OR, 16 LPEC, and 50 TLR. The preoperative laterality of IH was 45 patients (42.9%) on the right side, 50 (47.6%) on the left side, and 10 (9.5%) on both sides. It was, however, diagnosed postoperatively in 27 patients (25.7%) on the right side, 38 (36.2%) on the left side, and 40 (38.1%) on both sides. Of the 63 patients who presented with unilateral IH in the laparoscopic groups, 32 (50.8%) had synchronous contralateral patent process vaginalis (PPV) which were simultaneously repaired. This was significantly more common in children under 3 years of age. Operative time in unilateral or bilateral repair was significantly shorter in the laparoscopic repair groups (p < 0.001). Ipsilateral recurrence was not observed in any group. Metachronous contralateral IH occurrence was not significantly different between groups. Conclusion Laparoscopic IH repair may have benefit in terms of shorter operation time and diagnosis of unpredicted contralateral PPV compared to OR.

Farber disease (FD) is a rare lysosomal storage disorder that shows autosomal recessive inheritance. We report the case of a 58-month-old girl with FD, who was misdiagnosed with epithelioid hemangioendothelioma. The patient had undergone five surgeries for sacrococcygeal masses and three surgeries for scalp masses owing to misdiagnosis. Here, we describe this rare case of FD.
Diagnostic Errors ; Farber Lipogranulomatosis* ; Female ; Hemangioendothelioma* ; Hemangioendothelioma, Epithelioid ; Humans ; Scalp ; Wills

Purpose: The incidence of choledochal cyst (CC) with protein plugs is between 15.5%–40.4%. However, studies on CCs with protein plugs in children are limited. We aimed to analyze the clinical features, surgical findings, and complications of pediatric CCs with and without protein plugs. Methods: We retrospectively analyzed 390 patients who underwent surgery for CCs between January 1987 and September 2017. The patients were divided into 2 groups: groups A (CC with protein plugs) and B (CC without protein plugs). The presence of protein plugs was evaluated using preoperative images or identified during surgery. Results: One hundred forty-two (36.4%) patients had protein plugs in the pancreaticobiliary duct. The most common initial clinical presentation was abdominal pain, and its incidence was significantly higher in group A (66.2%) than in group B (54.8%) (P = 0.032). The incidence of accompanying pancreatitis was also significantly higher in group A (37.3% vs. 27.0%) (P = 0.040). Anomalous pancreaticobiliary ductal union (APBDU) was found in 261 patients (66.9%) and its incidence was significantly higher in group A (74.6% vs. 62.5%) (P = 0.014). Most protein plugs were found in the cyst (88.0%) and common channel (31.7%).The incidence of early complications was higher in group A; conversely, that of late complications did not differ. Conclusion Approximately 36.4% of the pediatric CC patients were accompanied by protein plugs. Abdominal pain, pancreatitis, and APBDU were more commonly observed among those with protein plugs than among those without; longterm complications did not differ between them.

Purpose: Although ultrasonography is the gold standard of diagnosing intussusception, plain abdomen radiograph (AXR) is often used to make differential diagnosis for pediatric patients with abdominal pain. In intussusception patients, we aimed to analyze the AXR and clinical data to determine the characteristics of early AXR findings associated with diagnosis of intussusception and recurrence after reduction. Methods: Between January 2011 and June 2018, 446 patients diagnosed with intussusception based on International Classification of Diseases-10 code of K56.1 were admitted. We retrospectively reviewed medical records of 398 patients who received air reduction; 51 of them have recurred after initial reduction. We evaluated six AXR features including absent ascending colon gas, absent transverse colon gas, target sign, meniscus sign, mass, and ileus. Clinical data and AXR features were compared between single episode and recurrence groups. Results: Two groups did not show significant differences regarding clinical data. Mean time to recurrence from air reduction was 3.4±3.2 days. Absent ascending colon gas (63.9%) was the most common feature in intussusception, followed by mass (29.1%). All of six AXR features were observed more frequently in the recurrence group. Absent transverse colon gas was the most closely associated AXR finding for recurrence (odds ratio, 2.964; 95% confidence interval, 1.327–6.618; p=0.008). Conclusion In our study, absence of ascending colon gas was the most frequently seen AXR factor in intussusception patients. Extended and careful observation after reduction may be beneficial if such finding on AXR is found in intussusception patients.

Purpose: Although ultrasonography is the gold standard of diagnosing intussusception, plain abdomen radiograph (AXR) is often used to make differential diagnosis for pediatric patients with abdominal pain. In intussusception patients, we aimed to analyze the AXR and clinical data to determine the characteristics of early AXR findings associated with diagnosis of intussusception and recurrence after reduction. Methods: Between January 2011 and June 2018, 446 patients diagnosed with intussusception based on International Classification of Diseases-10 code of K56.1 were admitted. We retrospectively reviewed medical records of 398 patients who received air reduction; 51 of them have recurred after initial reduction. We evaluated six AXR features including absent ascending colon gas, absent transverse colon gas, target sign, meniscus sign, mass, and ileus. Clinical data and AXR features were compared between single episode and recurrence groups. Results: Two groups did not show significant differences regarding clinical data. Mean time to recurrence from air reduction was 3.4±3.2 days. Absent ascending colon gas (63.9%) was the most common feature in intussusception, followed by mass (29.1%). All of six AXR features were observed more frequently in the recurrence group. Absent transverse colon gas was the most closely associated AXR finding for recurrence (odds ratio, 2.964; 95% confidence interval, 1.327–6.618; p=0.008). Conclusion In our study, absence of ascending colon gas was the most frequently seen AXR factor in intussusception patients. Extended and careful observation after reduction may be beneficial if such finding on AXR is found in intussusception patients.

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.
Bronchogenic Cyst* ; Esophageal Atresia* ; Esophagus ; Ligation ; Parturition ; Tracheoesophageal Fistula

Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.
Adolescent ; Adult ; Biopsy ; Child ; Humans ; Immunohistochemistry ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma* ; Male ; Prognosis ; Young Adult

Background: Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. Methods: From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. Results: Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). Conclusion A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Pediatric arterial aneurysm is rare disease. Among them, idiopathic-congenital arterial aneurysm is extremely rare. This is a case report of right common iliac artery idiopathic aneurysm with absence of right external iliac artery. A 4-year-old girl who had been complaining of intermittent abdominal pain since 2 years prior presented with a right lower abdominal mass that had been palpable since 6 months prior. Abdominal CT revealed a 5.2 cm × 4.5 cm × 5.1 cm, right-sided, partially thrombosed, saccular, iliac artery aneurysm. She underwent to operation, aneurismal resection. A pathological examination confirmed that it was a true aneurysm, considering that all layers of the vascular wall were stretched with no deficit. The patient was discharged 3 days after the surgery without any complication. Five months passed since the surgery, and the patient is doing well without any abdominal or leg pain.
Abdominal Pain ; Aneurysm* ; Child ; Child, Preschool* ; Female* ; Humans ; Iliac Aneurysm ; Iliac Artery* ; Leg ; Rare Diseases ; Tomography, X-Ray Computed ; Vascular Surgical Procedures

PURPOSE: Minimally invasive surgery (MIS) in abdomen and thorax has been widely accepted for pediatric diseases. Thoracoscopic surgery has the advantage of less pain, better cosmetic outcomes and less musculoskeletal sequelae in comparison to open surgery. We would like to share our initial experience with thoracoscopic surgery performed by one pediatric surgeon. METHODS: We performed a retrospective review of patients who underwent thoracoscopic surgery by one pediatric surgeon between April 2010 and August 2017 in Department of Pediatric Surgery, Seoul National University Children's Hospital. RESULTS: There were totally 18 cases; 8 cases for esophageal atresia, 3 cases for congenital diaphragm hernia, 2 cases for diaphragm eventration, 2 cases for esophageal duplication cyst, 2 cases for pleural mass and 1 case for esophageal bronchus. At the operation, median age was 9.5 months (range, 0-259 months) and median body weight was 9.4 kg (range, 1.9-49.4 kg). Median operative time was 157.5 minutes (range, 45-335 minutes). There was no case of open conversion and 2 cases of minor leakage at anastomosis site in case of esophageal atresia. Median follow-up month was 5 months (range, 0-87 months). During follow-up, 4 cases of esophageal atresia showed anastomosis site narrowing and average 2.5 times (range, 1-5 times) of esophageal balloon dilatation was done. CONCLUSION: We performed thoracoscopic surgery in case of esophageal, diaphragm disease and pleural mass. Thoracoscopic surgery can be an effective and feasible option of treatment for well-selected pediatric patients of intra-thoracic disease including esophagus, diaphragm and mediastinum disease.
Abdomen ; Body Weight ; Bronchi ; Diaphragm ; Diaphragmatic Eventration ; Dilatation ; Esophageal Atresia ; Esophagus ; Follow-Up Studies ; Hernia ; Humans ; Mediastinum ; Minimally Invasive Surgical Procedures ; Operative Time ; Pediatrics ; Retrospective Studies ; Seoul ; Thoracoscopy* ; Thorax