This is a survey on congenital posterolateral diaphragmatic hernia, conducted by Korean Association of Pediatric Surgeons (KAPS). A registration form for each patient during the 5-year period between 2010 and 2014 and a questionnaire were sent to each member. Twenty-six members in 16 institutions returned completed forms. Total patients were 219. Prenatal diagnoses were done in 181 cases (82.6%). Preoperative mortality was 11.4%. Minimal invasive surgery was done in 61 cases (31.8%). Risk factors related with death were Apgar score, oxygenation index, preoperative pH and bicarbonate, O₂ saturation, the presence of hernia sac, and the size of defect. The neonatal survival and 1-year survival of total patients were 77.6% and 75.3%.

National survey for newborns surgery with congenital anomalies by Korean Association of Pediatric Surgeons (KAPS) was done. A questionnaire was sent to all members of the KAPS on March 2014. The current survey is to review three years status of the newborn surgery from 2012 to 2013. Thirty-four members (27.9%) took part in the survey that included data for the diagnosis, number and procedures of neonatal surgical cases. The result was discussed at the Topic Discussion section of the 30th Annual Congress of KAPS, 2014.

Minimal invasive surgery (MIS) has rapidly gained acceptance for the management of a wide variety of pediatric diseases. A questionnaire was sent to all members of the Korean Association of Pediatric Surgeons. Thirty one members (25.4%) took part in the survey that included data for the year 2012: demographic details, opinion regarding minimal invasive surgery and robotic surgery, spectrum of minimally invasive operations, and quantity of procedures. 48.4% of the respondents had more than 10 years experience, 35.5% less than 10 years experience, and 16.1 % had no experience. The respondents of the recommend MIS and perform MIS for surgical procedures are as follow; inguinal hernia (61.3%), simple appendicitis (87.1%), complicated appendicitis (80.6%), reduction of intussusceptions (83.9%), pyloromyotomy (90.3%), fundoplication (96.8%), biopsy and corrective surgery of Hirschsprung's disease (93.5%/90.3%), imperforate anus (77.4%), congenital diaphragmatic hernia (80.6%), and esophageal atresia (74.2%). The MIS procedures with more than 70% were lung resection (100%), cholecystectomy (100%), appendectomy (96.2%), ovarian torsion (86.7%), fundoplication (86.8%), hiatal hernia repair (82.6%), and splenectomy (71.4%). The MIS procedures with less than 30% were congenial diaphragmatic hernia reapir (29.6%), esophageal atresia (26.2%), correction of malroatation (24.4%), inguinal hernia repair (11.4%), anorectal malformation (6.8%), Kasai operation (3.6%).

The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was 66.2±28.7 days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was 1.1±1.3 years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was 8.6±2.9 months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.

A nationwide survey on Choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five-year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1: 3.4. The age of patients on diagnosis was 49.0±44.4 months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani classification, 238 patients (71.3%) were type I, 3 (0.9%) type II, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by cyst excision and Roux-Y hepaticojejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system: APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long-term follow-up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1 : 1.3. The age of patients on diagnosis with biliary atresia was on average 65.4 ±36.2 days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (91.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.

To understand the current status of neonatal surgery in Korea, a survey was made among the 36 members of the Korean Association of Pediatric Surgeons. The response rate was 75% (26 surgeons in 20 hospitals). Five hundred fifty three neonatal surgical patients treated in 1999 were analyzed. Regional numbers of patients were closely related to the regional population in most areas. Ano-rectal malformations (17%), pyloric stenosis (16%), Hirschsprung's disease (13%), atresia/stenosis of the gut (11%), esophageal atresia (8%) were the most common anomalies treated. The majority of operations were done within the first week of life. Seventy one per cent of cases were major life threatening or so-called neonatal index cases. Over-all mortality was 8 per cent. Higher mortality was observed in patients with diaphragmatic hernia (26%), gastro-intestinal perforation (18%), NEC (18%), and esophageal atresia(14%). Higher mortality was observed in patients with extremely low birth weight (33%) and low birth weight (18%). Associated anomalies were observed in 20%. Prenatal ultrasound was performed in 36 per cent with sensitivity of 20%. Result of this study was compared to the previous report (1994) and that of Japan (1998).

The purpose of the survey was to clarifY the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7: 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(> 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were higheR) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed

To understand the current status of pediatric surgical practice of the members of the Korean Association of Pediatric Sutgeons, a survey of the practice of the 31 members in both 1994 and 1995. Twenty five members(80%) representing 20 hospitals responded. An average of four hundred and seventy four cases pediatric surgical operations were performed at individual institution in 1995 with 40 newborn cases. Eighteen members(72%) ate currently working at university hospital. Fourteen institutions(70%) are currently classified as tertiary by the health insurance agency. The majority(l5 to 60%) of members are working in the metropolitan Seoul area, while five in Taegu area. Sixteen members reported having the title of department head/director. Four members reported occasional non-pediatric surgical practice. Nine members out of 20 reported having independent pediatric surgical out-patient clinic before the establishment of the association( 1985). Eight out of 15 members reported being appointed chief of pediatric surgery before 1985. In 20 institutions, 34 full time physicians(27 members, reportedly) are working in pediatric surgery. In regarding to pediatric surgical training, 16 members(64%) received an average of 16 months of training abroad, 5 members trained at home and abroad, and 4 from only at home. There are no differences in length of training periods in these groups. TWenty one members received their basic pediatric surgical training before 1985, the year of inauguration of the association. Twelve members received post-pediatric surgery refresher courses averaging 11 months' duration, after 2-11 years. Thirteen participants of this study belongs to the founding members of the association.

Eight pediatric patients with Peutz-Jeghers syndrome were treated from 1984 to 1994 at the Department of Surgery, Seoul National University Children's Hospital. We reviewed the clinical features of our own 8 cases as well as 56 cases reported in Korean literatures. The results were compared to those of western reports. Gastrointestinal complications in our series were more frequent than in the Korean series, but the distribution of polyps was similar. The Korean series showed the following characteristics compared to the western reports; demographic backgrounds were similar; gastrointestinal symptoms were more common; location of the polyp was more frequent in colon (2 times); and the most prevalent site of malignant change was the colon in Korean cases.

The survey on branchial anomalies was conducted by Korean Association of Pediatric Surgeons. A total of 173 cases were reported, which were managed by 36 members and cooperators during the three years from January 1, 1993 through December 31, 1995. The following results were obtained by retrospective analysis of the 173 cases of branchial anomalies. The presenting symptoms were cervical mass in 101 cases, pit with or without discharge in 71, cervical abscess in 47 and respiratory difficulty in 3. The average age of the patients with cervical abscess was 52 months. Seventy(79%) of 89 patients with branchial anomalies and a cystic mass had their first clinical manifestations by 1 year of age, while 40(51%) of 78 patients with only a branchial cyst had their first clinical manifestation in first year of life. Radiologic studies were carried out in 77 patients(43%). The preferred diagnostic modalities were ultrasonography(47 patients), simple neck radiogram(l9) and CT scan(17). Preoperative diagnosis was correctly made in 156(91%) of 173 patients. Seventeen patients were incorrectly diagnosed as thyroglossal duct cyst in 5 patients, cystic hygroma in 4, dermoid cyst in 3, and lymphadenopathy in 3. There were no remarkable difference in sex and laterality of presentation but bilateral lesions were found in 9(5%) patients and unusual locations of the anomalies were the manubrium, left subclavicular area, median cervial area, preauricular and parotid area. There were 78(45%) patients with cyst, 52(30%) patients with sinus, 35(20%) patients with fistula and 8(5%) patient with skin tag. Embryological classification was possible in only 64(37%) patients. The 2nd branchial anomaly was present in 50 (78%), the 1st branchial anomaly in 10(18%), and the 3rd or 4th branchial anomaly in 4(6%). Histopathological study of the lining epithelium(N=134) is recorded that 45% were lined with squamous epithelium, 17% with respiratory epithelium, 6% with. squamous and respiratory epithelium, 14% with inflammatory change. Lymphoid tissue was common(62%) in the wall of the lesions. Twelve(7%) of 158 patients had postoperative complications including wound complication, recurrence and facial nerve palsy.

This report present the result of the national survey of pediatric surgeons' preferences on diagnosis and treatment of Hirschsprung's disease(HD) carried out in 1993. The questionnaires were sent to twenty-seven members of the Korean Association of Pediatric Surgeons (KAPS) working in twenty-four institutions. The questionnaires were designed to determine the individual surgeon's preference for the methods of diagnosis and treatment of the disease. Twenty-three pediatric surgeons from twenty institutions returned completed forms. The total number of patients diagnosed with HD in 1992 was 190 in this group. The estimated incidence of HD was 1/3,900. The most important symptom was delayed meconium passing and the most preferred diagnostic procedure was barium study. Anorectal manometric examination was carried out by 13 pediatric surgeons and 19 confirmed the diagnosis before operation by rectal biopsy, 12 with full-thickness biopsy and 7 with suction. Frozen section biopsy during operation was done by 22 surgeons. Eight surgeons did one stage operation if the age of the patient is suitable. Definitive operation was usually done at the age of 6 to 11 months. The most preferred operation was Duhamel procedure done by 19. Enterocolitis was the most serious complication of HD. Most of patients had normal continence within 6 to 12 months after operation. The follow-up period was less than 6 years in 16 surgeons. The results were presented at the 9th annual meeting of KAPS in June of 1993. This is the first national survey of HD and it can provide guidelines of diagnosis and treatment of Hirschprung's disease even though it is not a detailed study of patient data.

To understand the current status of neonatal surgery in Korea, a suvey was made among the 27 members of Korean Association of Pediatric Surgeons. Response rate among surgeons was 78 perecnt, eighteen hospitals participated in this study. Five hundred and three cases of neonatal surgical patients were analyzed. In Korea, about 50% of cases were treated at the hospital in the capital city area. Regional number of patients were closely related to the regional population. Imperforate anus(19%), atresia/stenosis of gut(12%), and Hirschsprung's diseases(12%) were sitting at the top in the list. Majority of operation was done within the first week of life, especially during the first 24 hour period. Eighty per cent was major or so called index cases. Mortality in so-called index cases was 17%. High mortality was observed in patients with diaphragmatic hernia(47%), gastrointestinal perforation(65%) and esophageal atresia(28%). Low birth weight babies showed higher mortality in gastro -intestinal perforation, esophageal atresia and abdominal wall defect. These were compared to 1993 survey of Japanese Society of Pediatric Surgeons.

The first national survey on esophageal atresia and tracheoesophageal fistula was conducted to access the current status of its incidence. clinical manifestation, preoperative diagnosis and management, type of its anomaly, associated anomalies, and surgical results and course. The 43 members of the Korea Association of Pediatric Surgeons received questionnaires and registration forms to be filled out on each patient who were born during the three years from January I, 1992 through Decestricurember 31, 1994. Questionnaires composed of six broad areas which include 1) preoperative diagnosis and management, 2) surgical technic, 3) long gap, 4) postoperative management, and 5) complications and courses. A total of 148 cases was returned by 28 members working at 23 institutions and 27 members returped questionnaires. We obtained the following results by analysis of the 148 cases of tracheoesophageal anomalies. The incidence of tracheoesophageal anomaly was about 1/10,000-11,000 in 1994, which is one third of that of anorectal malformations in Korea and the distribution of the patients was almost proportionate to the size of each province. Both sexes are about equally affected. Majority of the members make diagnosis of tracheoesophageal anomaly by taking a simple infantogram with a radiopaque tube in upper pouch and a little under half(46%) prefers to perform echocardiography as a part of preoperative management to identify congenital heart disease and lateralize the aorta.

Esophageal atresia with distal TEF(87.50/0) was by far the most common and threre were pure esopahgeal atresia(5.6%), H-type TEF(2.l%), and so on. About half(49%) of the patients had one or more associated anomalies in addition to tracheoesophageal anomalies. Congenital heart disease was associated in 46 cases(31 %), anorectral malfomations in 19 cases(13%), musculoskeletal anomalies in 15 cases(10%), genitourinary anomalies in 10 cases(7%) and gastrointestinal anomalies in 7 cases(5%). Postoperatively, parenteral nutrition and assisted ventilation were given in 66% and 52% of patients respectively. Ninety three(74%) of 126 cases who underwent surgical procedure, experienced one or more complications such as respiratory complication (65%), leak(22%), stricture(21%) and so on. The survival rates related to the Waterston risk categories were 90.2% in grpup A, 71.4-75% in B₁, B₂, and C groups, and 28% in group C₂ and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(l2 cases), and congenital heart disease(4 cases). With short term follow-up, 69% of patients have been excellent whereas remainders of the cases have suffered from some sort of morbidity related to gastroesophageal reflux, recurrent respiratory infection, and esophageal stricure.