Congenital anorectal malformation

의학과/석사[한글] 소아외과 환아중 많은 수는 선천성 기형을 갖고 있으며 근래 국내외 저자들의 보고를 비교하여 보면 한국의 선천성 직장항문 기형증은 외국에 비하여 높은 빈도를 보이고 있다. 선천성직장항문기형증은 외형적으로 진단이 가능하고 증상도 신생아기에 발현되므로 조 기 진단이 비교적 용이하다. 그러나 직장항문기형증의 치료목적이 생명을 구하는 것 뿐만 아니라 수술후 기능적, 사회적으로 얼마나 불편없는 생활을 영위해 가느냐에 있다 하겠다. 이에 저자는 1970년 1월부터 1980년 12월까지 만 11년동안 연세대학고 부속 세브란스 병원 소아외과에 입원하여 치료받은 102예의 선천성직장항문기형증 환아에 대하여 임상증상, 진단 및 해부학적분류, 치료와 수술후 사망율, 합병증 및 수술후 원격성적을 분석 관찰하어 그의 현황을 파악하고 문제점들을 찾아내어 향후 본 질환의 진단과 치료에 개선할 점들을 모색하고저 본 연구를 실시한 결과 다음과 같은 결론을 얻었다. 1) 남녀의 비는 1.5:1로 남아에 많았다. 2) 해부학적 형태별 분류는 TypeⅢ가 81%로 가장 많았으며 TypeⅢ A가 57%를 차지였다. 3) 누공은 남아에서 50%, 여아에서 69%에 있었으며 남아에서는 항문회음부누공과 직장요도누공이 가장 많았으나 여아에서는 항문외음부누공이 가장 많았다. 4) 동반기형은 23%에 있었으며 이중 근골격계기형이 가장 많았고 심맥관계기형과 비뇨생식계기형도 많았다. 고위기형이 저위기형에 비하여 동반기형이 많았다. 5) 진단법으로 시행한 방법중 Wangensteen Rice technique은 75%의 정확도를 보였고 누공조영술(fistuloram)과 원위부결장 조영술(distal cologram)은 90∼95%가 수술소견과 일치하였다. 6) 교정수술은 63%에서 시행하였고 TypeⅢ^^A 는 회음부항문형성술(perineal anoplasty)과 복회음부항문형성술(abdominoperineal pull-through operation)을 일차수술이나 이차수술로 시행하였으나 TypeⅢ^^B는 전예에서 예비적결장류형성술의 종류는 횡행결장루 (transv-erse loop colostomy)가 85%였고 S-형결장루(sigmoid colostomy)가 15%였다. 7) 합병증은 예비적결장루형성술후에는 24%에서 생겼고 교정 수술 후에는 30%에서 생겼다. 합병증중에는 항문협착증, 장폐쇄증, 탈홍(anal prolapse)이 많았다. 8) 전체 사망률은 17.6%였으며 수술사망율은 3.1%였다. 사망률은 고위기형일수록 높았고 동반된 선천성기형이 많을수록 높았다. 9) 수술후 원격성적은 저위기형이 고위기형에 비하여 훨씬 좋았다. Congenital Anorectal Malformation Seung Hoon Choi, M.D. Dept. of Medical School The Graduate School Yonsei University (Directed by Prof. Choon Kyu Kim, M.D.) Congenital abnormalities of the anus and rectum were said to occur about once in every 1500 newly born babies. The incidence in Korea seemed to be somewhat higher than that of foreigncountries. Establishment of diagnosis of anorectal malformation is relatively easy by clinical inspection and the symptoms were seen in the first few days of life. The goals of surgical treatment of congenital anorectal malformations are firstly, to have the children survive their surgical treatment, and, secondly, to obtain the maximum quality of the functional result. The author has tried to figure out the whole picture of the congenital anorectal malformation in Korea on the basis of experience in YUMC. The study is aimed to find out the problems in managing these patients and to establish guidelines for handling these patients through the clinicostastical analysis of the patients who were admitted to the Yonsei Medical Center for a period of 11 years from the January 1970 to December 1980, and the results were as follows: 1. The sex distribution was 60 males and 42 females with a significant high incidence in male with ratio of 1.5:1. 2. The commonest type of anorectal malformations was Type ⅢA. This type of anomaly occured in 57% of this study. 3. The overall incidence of fistulas in male was 50%; 69% of the females had fistulas. Common fistulas male were anopeineal fistula and rectourinary fistuls, the commonest fistula in female being anovulvar fistula. 4. The incidence of associated congenital lesions was 23%. In this series, musculoskeletal, cardiovascular and urinary deformities were common, in the order of frequency. The associated lesions were more frequent and of sinister significance in the high anomaly. 5. The accuracy of Wangensteen-Rice technique was 75%, whereas the accuracy of fistulogram and distal cologram disclosed 90 to 95%. 6. Corrective operation was done in 63% of patients, Type ⅢA anomalies were managed by perineal anoplasty or abdominoperineal pull-through operation, these form of treatment done with of without preliminary colostomy. Type ⅢB anomalies were managed by some form of pull-through procedure such as abdominoperineal pull-through operation and sacroabdominoperineal pull-through operation, all of these procedures were done with preliminary colostomy. The site of colostomy was 85% in the transverse colon, 15% in the sigmoid colon. 7. The incidence of postoperative complication of colostomy was 25% and that of corrective operation was 30%. Common complications were anal stenosis, intestinal obstruction and anal prolapse. 8. The overall mortality rate was 17.6% and operative mortality rate was 3.1%. The mortality rate of high anomaly and associated anomaly were higher than the other patient. 9. The functional result was very much superior in the group of low anomaly. [영문] Congenital abnormalities of the anus and rectum were said to occur about once in every 1500 newly born babies. The incidence in Korea seemed to be somewhat higher than that of foreigncountries. Establishment of diagnosis of anorectal malformation is relatively easy by clinical inspection and the symptoms were seen in the first few days of life. The goals of surgical treatment of congenital anorectal malformations are firstly, to have the children survive their surgical treatment, and, secondly, to obtain the maximum quality of the functional result. The author has tried to figure out the whole picture of the congenital anorectal malformation in Korea on the basis of experience in YUMC. The study is aimed to find out the problems in managing these patients and to establish guidelines for handling these patients through the clinicostastical analysis of the patients who were admitted to the Yonsei Medical Center for a period of 11 years from the January 1970 to December 1980, and the results were as follows: 1. The sex distribution was 60 males and 42 females with a significant high incidence in male with ratio of 1.5:1. 2. The commonest type of anorectal malformations was Type ⅢA. This type of anomaly occured in 57% of this study. 3. The overall incidence of fistulas in male was 50%; 69% of the females had fistulas. Common fistulas male were anopeineal fistula and rectourinary fistuls, the commonest fistula in female being anovulvar fistula. 4. The incidence of associated congenital lesions was 23%. In this series, musculoskeletal, cardiovascular and urinary deformities were common, in the order of frequency. The associated lesions were more frequent and of sinister significance in the high anomaly. 5. The accuracy of Wangensteen-Rice technique was 75%, whereas the accuracy of fistulogram and distal cologram disclosed 90 to 95%. 6. Corrective operation was done in 63% of patients, Type ⅢA anomalies were managed by perineal anoplasty or abdominoperineal pull-through operation, these form of treatment done with of without preliminary colostomy. Type ⅢB anomalies were managed by some form of pull-through procedure such as abdominoperineal pull-through operation and sacroabdominoperineal pull-through operation, all of these procedures were done with preliminary colostomy. The site of colostomy was 85% in the transverse colon, 15% in the sigmoid colon. 7. The incidence of postoperative complication of colostomy was 25% and that of corrective operation was 30%. Common complications were anal stenosis, intestinal obstruction and anal prolapse. 8. The overall mortality rate was 17.6% and operative mortality rate was 3.1%. The mortality rate of high anomaly and associated anomaly were higher than the other patient. 9. The functional result was very much superior in the group of low anomaly.restrictio