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.

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

A survey on intestinal atresia was made among 34 members of Korean Association of Pediatric Surgeons about the patients who were treated from the January 1, 1994 to December 31, 1996. The response rate was 82.4%. Two hundred and fifteen patients were analyzed. The lesion occurred in 73 cases at duodenum, in 72 cases at jejunum, in 71 cases at ileum and 2 cases at cecum and sigmoid colon each. There were 2 cases of combined anomalies (DA + JA + IA and DA + JA). Male to female ratio was 1:1 in duodenal atresia. and 1.8:1 in jejunoileal atresia. Seventy-four cases (34.3%) were premature babies (DA 35.2%, JA:48.6%, IA:19.2%), and 62 cases (28.7%) had low birth weight (DA:39.4%, JA 33.0%, IA:13.7%). Antenatal diagnosis was made in 92 cases (43.6%). However 22 cases (23.9%) of them were transferred to pediatric surgeon after delivery. Maternal polyhydramnios was observed in 63 cases (28.9%). Seventy-five cases (34.4%) were taken only simple abdominal film for diagnostic studies. The associated malformations were observed in 54 cases (24.8%) of intestinal aresia and more frequently observed in duodenal atresia (35 cases, 47.9%). Meconium peritonitis due to intrauterine bowel perforation was more frequently associated with ileal atresia compared to duodenum and jejunum. The overall mortality rate was 30%. (Abbreviations: DA;duodenal atersia, JA;jejunal atresia, IA;ileal atrsia, PT;p-value in total, PDJ,DI,JI;p-value between two groups among duodenal, jejunal and ileal groups)

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.

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.