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.

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)

Thirty three members of the Korean Association of Pediatric Surgeons were surveyed to determine the clinical manifestations and current management of infantile hypertrophic pyloric stenosis (IHPS). The members completed a registration form and a questionnaire that indicated their treatment preferences for patients treated during 1996. The response rate was 84.8 %, and 23 institutions participated. Three hundred and sixty-three cases of IHPS were analysed. The male to female ratio was 5.15: 1. The presenting symptoms were nonbilious vomiting in 363 cases(lOO %), palpable mass in the upper abdomen in 200 cases (55.1 %) and visible peristaltic waves in 67 cases(l8.5 %). Fluid therapy was done in 161 cases(44.4 %) for preoperative preparation. Twenty patients(5.5 %) were premature. There were 27 low birth weight babies(7.7 %). The onset of symptoms ranged from 1 to 14 weeks of age with a peak occurrence at 2 to 4 weeks of age. Two hundred and thirty-six cases(66.6 %) presented within 4 weeks of age. There was one set of male twins and a family history of twins. The modes of feedings were: breast feeding; 69 cases(24.5 %), formula feeding; 176 cases(62.4 %) and mixed feeding; 37 cases(l3.l %). The preferred diagnostic modalities were ultrasonography (US) 229 cases(63.l %), UGI series 27 cases(7.4 %) and US and UGI series combined 86 cases(23.7 %). Only 21 cases(5.8 %) were operated on without imaging study, ie. with positive physical finding. Perforation of the mucosa during pyloromytomy occurred in 5 cases. All were treated successfully with primary closure. Persistent vomiting over 2 weeks after operation were reported in 5 cases. Three wound infections, one aspiration pneumonia and one postoperative bleeding were reported. There was no mortality in 363 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.

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.