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)
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.
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.