선천성 식도 무공증 및 기관식도 누공 - 대한 소아외과학회 회원을 대상으로 한 전국 조사

Abstract

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 1, 1992 through December 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 returned 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(87.5%) was by far the most common and there were pure esophageal atresia(5.6%), H-type TEF(2.1%), 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 sugical 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 group A, 71.4-75% in $B_1$ 수식 이미지, $B_2$ 수식 이미지, and $C_1$ 수식 이미지, groups, and 28% in group $C_2$ 수식 이미지, and the overall survival rate was 71.4%. Thirty six(28.6%) of 126 cases died of pneumonia/sepsis(12 cases), respiratory failure(12 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 stricture.

국내에서 처음으로 기관식도기형에 관하여 대한소아외과 학회 회원을 대상으로 조사하였다. 본 연구는 두 부분으로 구성되어 있었는데 하나는 최근 3년간 출생한 기관식도기형의 등록된 자료를 분석하여 국내의 기관식도기형의 현황 즉 발병율 처치 및 치료 결과등에 관한 총체적인 경향을 외국과 비교 분석하여 우리 자료와 구미 보고와의 차별성 및 유사성을 분석하고저 했으며, 다른 하나는 질문서(questionnaires) 분석을 토대로 11차 대한소아외과학회 학술대회에서 토의된 내용을 기초로 회원들의 식도기형의 진단 치료, 술후 관리, 합병증 등에 대한 경험과 의견을 종합하여 고안을 작성하였다