Analysis of Pediatric Surgery Using the National Healthcare Insurance Service Database in Korea: How Many Pediatric Surgeons Do We Need in Korea?

Background@#In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. @*Methods@#We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries.Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. @*Results@#The number of pediatric surgeries from 2002 to 2017 increased by 124%.Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. @*Conclusion@#The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.

Suspecting Intussusception and Recurrence Risk Stratification Using Clinical Data and Plain Abdominal Radiographs / 대한소아소화기영양학회지

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.

Analysis of Pediatric Surgery Using the National Healthcare Insurance Service Database in Korea: How Many Pediatric Surgeons Do We Need in Korea?

Background@#In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. @*Methods@#We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries.Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. @*Results@#The number of pediatric surgeries from 2002 to 2017 increased by 124%.Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. @*Conclusion@#The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.

Clinical comparison between the presence and absence of protein plugs in pediatric choledochal cysts: experience in 390 patients over 30 years in a single center

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.

Laparoscopic surgery: an effective and safe surgical method of pediatric inguinal hernia repair

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.

Suspecting Intussusception and Recurrence Risk Stratification Using Clinical Data and Plain Abdominal Radiographs / 대한소아소화기영양학회지

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.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies

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.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies

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.

Transverse Colon Volvulus around the Gastrostomy Tube in a Pediatric Situs Inversus Patient / 소아외과

An 18-year-old male patient with cerebral palsy and scoliokyphosis came to the emergency department with abdominal distension and vomiting. He was a situs inversus patient with a feeding gastrostomy tube. Sigmoid volvulus was initially suspected, so rectal tube insertion and endoscopic decompression were attempted, but failed. So he went through explorative laparotomy, and transverse colonic adhesion and twisting around the gastrostomy tube and gastric wall was identified. Adhesiolysis and resection with redundant transverse colon and end-to-end colocolic anastomosis was performed. He discharged with symptom free. Suspecting transverse colonic volvulus is important when the patient has anatomical anomalies and feeding gastrostomy tube. Timely diagnosis with proper radiologic imaging should be made. Surgical resection of the redundant colon is needed for successful management of transverse colonic volvulus.

Is Preoperative Ultrasonography Necessary in Pediatric Inguinal Hernia? / 소아외과

PURPOSE@#Preoperative ultrasonography (USG) in pediatric inguinal hernia has controversy. In this study, we analyzed the cases of pediatric inguinal hernia with/without preoperative USG and discussed whether USG is necessary.@*METHODS@#We reviewed medical records of 1,441 patients who underwent inguinal hernia repair in Seoul National University Children's Hospital between January 2011 and August 2016 retrospectively.@*RESULTS@#Male were 69.3% and age at operation was 37.8±36.5 months old. There were 150 patients (10.4%) performed USG preoperatively. The department ordered to perform USG included department of surgery (n=71), emergency medicine (n=42), pediatrics (n=26), urology (n=10) and outside hospital (n=1). The reasons of performing USG included evaluation for hernia laterality (n=82), incarceration (n=28), testis (n=15), request of parents (n=14), scrotal mass (n=6) and incidentally found during evaluation for another disease (n=5). Excepting 5 cases of incidental finding, of 145 cases with USG, 12 (8.3%) cases changed the surgical plan; change to bilateral repair from unilateral repair (n=5), emergency operation due to incarceration (n=4) which include 1 salpingo-oophorectomy, 1 open abdomen surgery and 2 hernia repair after reduction of ovary, change to co-operation of orchiopexy (n=2) and change to laparoscopic surgery from open surgery due to herniation of both ovaries into one inguinal canal (n=1). In group without USG (n=1,291), 5 patients (0.4%) had unexpected problems during operation; 2 co-operation of orchiopexy because of transverse testicular ectopia (n=1) and right undescended testis (n=1), 2 hypertrophy of major labia and 1 retroperitoneal lymphangioma at inguinal area misdiagnosed as inguinal hernia.@*CONCLUSION@#It was difficult to interpret the meaning of preoperative USG because not all patients had performed it. In this study, 10.4% of patients performed USG and 8.3% of them changed surgical plan. About 0.4% of patients without preoperative USG would have benefited from it for surgery if they had performed it. Since the percentage is too low, it is unreasonable to conclude that USG has diagnostic utility in inguinal hernia in this study.

Transverse Colon Volvulus around the Gastrostomy Tube in a Pediatric Situs Inversus Patient

An 18-year-old male patient with cerebral palsy and scoliokyphosis came to the emergency department with abdominal distension and vomiting. He was a situs inversus patient with a feeding gastrostomy tube. Sigmoid volvulus was initially suspected, so rectal tube insertion and endoscopic decompression were attempted, but failed. So he went through explorative laparotomy, and transverse colonic adhesion and twisting around the gastrostomy tube and gastric wall was identified. Adhesiolysis and resection with redundant transverse colon and end-to-end colocolic anastomosis was performed. He discharged with symptom free. Suspecting transverse colonic volvulus is important when the patient has anatomical anomalies and feeding gastrostomy tube. Timely diagnosis with proper radiologic imaging should be made. Surgical resection of the redundant colon is needed for successful management of transverse colonic volvulus.

Is Preoperative Ultrasonography Necessary in Pediatric Inguinal Hernia?

PURPOSE: Preoperative ultrasonography (USG) in pediatric inguinal hernia has controversy. In this study, we analyzed the cases of pediatric inguinal hernia with/without preoperative USG and discussed whether USG is necessary. METHODS: We reviewed medical records of 1,441 patients who underwent inguinal hernia repair in Seoul National University Children's Hospital between January 2011 and August 2016 retrospectively. RESULTS: Male were 69.3% and age at operation was 37.8±36.5 months old. There were 150 patients (10.4%) performed USG preoperatively. The department ordered to perform USG included department of surgery (n=71), emergency medicine (n=42), pediatrics (n=26), urology (n=10) and outside hospital (n=1). The reasons of performing USG included evaluation for hernia laterality (n=82), incarceration (n=28), testis (n=15), request of parents (n=14), scrotal mass (n=6) and incidentally found during evaluation for another disease (n=5). Excepting 5 cases of incidental finding, of 145 cases with USG, 12 (8.3%) cases changed the surgical plan; change to bilateral repair from unilateral repair (n=5), emergency operation due to incarceration (n=4) which include 1 salpingo-oophorectomy, 1 open abdomen surgery and 2 hernia repair after reduction of ovary, change to co-operation of orchiopexy (n=2) and change to laparoscopic surgery from open surgery due to herniation of both ovaries into one inguinal canal (n=1). In group without USG (n=1,291), 5 patients (0.4%) had unexpected problems during operation; 2 co-operation of orchiopexy because of transverse testicular ectopia (n=1) and right undescended testis (n=1), 2 hypertrophy of major labia and 1 retroperitoneal lymphangioma at inguinal area misdiagnosed as inguinal hernia. CONCLUSION: It was difficult to interpret the meaning of preoperative USG because not all patients had performed it. In this study, 10.4% of patients performed USG and 8.3% of them changed surgical plan. About 0.4% of patients without preoperative USG would have benefited from it for surgery if they had performed it. Since the percentage is too low, it is unreasonable to conclude that USG has diagnostic utility in inguinal hernia in this study.

Esophageal Atresia with Bronchogenic Cyst / 소아외과

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.

A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia / 소아외과

It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.

Early Experience of Pediatric Thoracoscopic Surgery Performed by a Pediatric Surgeon / 소아외과

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.

Thyroid Cancer in Pediatric Age: A Single Institution Experience / 소아외과

PURPOSE: Thyroid cancer is a rare disease in pediatric population, but its incidence rate is increasing. The aim of this report is to present a single institution experience of pediatric thyroid cancer and to identify clinical features, predisposing factors, and postoperative course of pediatric thyroid cancer. METHODS: We retrospectively reviewed 35 pediatric patients who underwent operation due to thyroid cancer at Seoul National University Children's Hospital between May 1997 and January 2017. The median follow-up period was 70 months (range, 5–238 months). RESULTS: The mean age at operation was 12.0±5.91 years and 27 patients were female. The underlying conditions in patients included history of chemoradiotherapy for previous other malignancies (n=4), hypothyroidism (n=3), history of chemotherapy (n=2), family history of thyroid cancer (n=1) and history of radiation therapy (n=1). The initial symptoms were palpable neck mass (n=21) and incidental findings (n=11). Total thyroidectomy (n=30) or unilateral lobectomy (n=5) were performed. There were 15 postoperative complications including transient hypocalcemia in 14 patients and Horner's syndrome in 1 patient. The most common pathologic cell type was papillary thyroid cancer (n=29). Extrathyroid extension and lymph node invasion were found in 25 patients and 27 patients, respectively. Thirteen patients showed multifocality. During follow-up period, 5 patients underwent additional operation because of tumor recurrence in lymph nodes. Lung metastasis was detected in 3 patients at the time of diagnosis and in 3 patients during follow-up period. The mortality rate was zero and mean disease-free survival was 83.7±47.9 months. CONCLUSION: Pediatric thyroid cancer has lower mortality rate and recurrence rate as seen in this study despite the advanced stage at diagnosis. A thorough follow-up of patients with an underlying condition such as history of chemoradiotherapy and understanding new pediatric guideline can be helpful to maximize patients' survival and prognosis.

A Case of Pediatric Nodal Marginal Zone Lymphoma / 소아외과

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.

Farber Disease Misdiagnosed as Hemangioendothelioma / 소아외과

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.

Trend of Pediatric Cholecystectomy: Clinical Characteristics and Indications for Cholecystectomy / 소아외과

PURPOSE: Number of pediatric cholecystectomy has been recently showing a gradually increasing trend. The purpose of this study was to investigate the clinical features of patients who underwent pediatric cholecystectomy, and the latest trend in cholecystectomy. METHODS: In the present study, we conducted a retrospective chart review on 47 patients who had undergone cholecystectomy at a single center. The entire patient population was divided into two groups, according to the time of cholecystectomy (early group, January 1999 to December 2006; late group, January 2007 to August 2014). RESULTS: The comparison between the early and late groups showed that the number of cholecystectomy increased from 13 to 34 cases representing a 2.6-fold increase. The mean patient age also increased from 5.94±4.08 years to 10.51±5.57 years (p=0.01). Meanwhile, laparoscopic surgery also increased from 15.4% to 79.4%, respectively (p<0.001). However, sex, mean body mass index, comorbidities, indications of cholecystectomy, and previous total parenteral nutrition were not statistically significant. CONCLUSION: The results of this study showed that pediatric cholecystectomy cases are increasing, particularly in the 10 to 19 years age group and laparoscopic cholecystectomies are also being performed at an increasing rate. When the patients were compared according to the time of cholecystectomy, there were no differences in other risk factors or indications for cholecystectomy.

Right iliac arterial aneurysm in a 4-year-old girl who does not have a right external iliac artery

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.