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.

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.