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pissn 2383-5036 eissn 2383-5508 J Korean Assoc Pediatr Surg Vol. 22, No. 1, June 2016 http://dx.doi.org/10.13029/jkaps.2016.22.1.1 Review Article 선천성기형에대한신생아수술현황 : 대한소아외과학회회원을대상으로한전국조사 김대연, 김성철, 김수홍, 김해영, 김현영, 남소현, 박귀원, 박준범, 박진영, 박태진, 서정민, 설지영, 신재호, 오정탁, 이명덕, 이석구, 이성철, 장은영, 장혜경, 정상영, 정성은, 정수민, 정연준, 정은영, 조민정, 최수진나, 최순옥, 최승훈, 최윤미, 한석주, 허태길 대한소아외과학회 Newborns Surgery with Congenital Anomalies: A National Survey of Its Members by Korean Association of Pediatric Surgeons DY Kim, SC Kim, SH Kim, HY Kim, HY Kim, SH Nam, KW Park, JB Park, JY Park, TJ Park, JM Seo, JY Seol, JH Shin, JT Oh, MD Lee, SK Lee, SC Lee, EY Jang, HK Jang, SY Jung, SE Jung, SM Jung, YJ Jung, EY Jung, MJ Cho, SJN Choi, SO Choi, SH Choi, YM Choi, SJ Han, TK Heo Korean Association of Pediatric Surgeons National survey for newborns surgery with congenital anomalies by Korean Association of Pediatric Surgeons (KAPS) was done. A questionnaire was sent to all members of the KAPS on March 2014. The current survey is to review three years status of the newborn surgery from 2012 to 2013. Thirty-four members (27.9%) took part in the survey that included data for the diagnosis, number and procedures of neonatal surgical cases. The result was discussed at the Topic Discussion section of the 30th Annual Congress of KAPS, 2014. Keywords: Neonate, National surveys, Surgery, Korea 서론한국에서의선천성기형에대한신생아수술의최근현황과변화추이를알아보기위해대한소아외과학회회원들을대상으로조사를하여 2014년제30회대한소아외과학회춘계학술대회에서주제토의시간을통해발표하고, 토의하였다. 이전의본학회의전회원대상의전국조사는 1996년부터 1999년까지 3년간의지수질환에대한조사가있었다 [1]. 대상및방법 2014 년 3월기준대한소아외과학회에정회원과준회원으로등록되어있는회원모두를대상으로이메일을통한설문 조사를하였다. 설문의내용은 1 회원의신상 ( 회원구분, 근무처, 소아외과근무연수, 소아외과세부전문의여부 ), 2 회원병원의최근 2년간소아외과전체와신생아평균수술수, 신생아중환자실병상수변화, 3 신생아질환의진단 ; International Classification of Diseases (ICD)-10 에따른진단 (2012년/2013년), 4 건강보험행위급여, 비급여목록표및급여상대가치점수관련 2013년도 12월말까지신설변경된보건복지부고지사항에따른수술코드에따른수술현황을조사하였다. 각군간의비교는 t test 에의해시행하였다. 수술중직장생검, 충수절제술, 흉관삽입술, 중심정맥카테터삽입술등은제외하였다. Received: May 17, 2016, Accepted: May 17, 2016 Correspondence: Dae Yeon Kim, Division of Pediatric Surgery, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. Tel: +82-2-3010-3961, Fax: +82-2-3010-6841, E-mail: kimdy@amc.seoul.kr Copyright 2016 Korean Association of Pediatric Surgeons. All right reserved. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Journal of Korean Association of Pediatric Surgeons 1

J Korean Assoc Pediatr Surg 2016;22(1):1-5 결과전체회원 122명중 34명이응답하였다 (27.9%). 정회원 58명중 24명 (41.4%), 준회원 64명중 10명 (15.6%) 이응답하였다. 세부전문의는 29명이었다. 이번조사에응답한회원기관은가톨릭대학교의과대학서울성모병원, 건국대학교병원, 경북대학교병원, 경상대학교병원, 계명대학교동산의료원, 부산대학교병원, 인제대학 교의과대학부산백병원, 일산백병원, 해운대백병원, 서울대학교어린이병원, 성균관대학교의과대학삼성서울병원, 연세대학교의과대학세브란스병원, 강남세브란스병원, 서울아산병원, 인하대학교병원, 전남대학교병원, 전북대학교병원, 충남대학교병원, CHA 의과학대학교분당차병원등이었다. 조사한회원병원의신생아중환자실병상수는 2012 년초 550 병상에서 2013년말 632병상으로 14.9% 증가하였다. 전 Table 1. Newborn Surgery by ICD-10 Classification No. of patients Q39 Congenital malformations of oesophagus Q39.0 Atresia of oesophagus without fistula 11 Q39.1 Atresia of oesophagus with tracheo-oesophageal fistula 113 Q39.X Congenital malformations of oesophagus 3 Q40 Other congenital malformations of upper alimentary tracts Q40.0 Congenital hypertrophic pyloric stenosis 52 Q40.1 Congenital hiatus hernia 3 Q40.2 Other specified congenital malformations of stomach 2 Q40.8 Other specified congenital malformations of upper alimentary tract 4 Q41 Congenital absence, atresia and stenosis of small intestine Q41.0 Congenital absence, atresia and stenosis of duodenum 45 Q41.1 Congenital absence, atresia and stenosis of jejunum 44 Q41.2 Congenital absence, atresia and stenosis of ileum 37 Q41.8 Congenital absence, atresia and stenosis of other specified parts of small intestine 7 Q41.9 Congenital absence, atresia and stenosis of small intestine, part unspecified 11 Q41.X Congenital absence, atresia and stenosis of small intestine 11 Q42 Congenital absence, atresia and stenosis of large intestine 3 Q42.0 Congenital absence, atresia and stenosis of rectum with fistula 3 Q42.1 Congenital absence, atresia and stenosis of rectum without fistula 3 Q42.2 Congenital absence, atresia and stenosis of anus with fistula 111 Q42.3 Congenital absence, atresia and stenosis of anus without fistula 110 Q42.X Congenital absence, atresia and stenosis of large intestine 4 Q43 Other congenital malformations of intestine Q43.0 Meckel diverticulum 25 Q43.1 Hirschsprung disease 100 Q43.3 Congenital malformations of intestinal fixation 57 Q43.4 Duplication of intestine 7 Q43.6 Congenital fistula of rectum and anus 4 Q43.7 Persistent cloaca 12 Q43.8 Other specified congenital malformations of intestine 17 Q43.X Congenital malformation of intestine, unspecified 12 Q44 Congenital malformations of gallbladder, bile ducts and liver Q44.2 Atresia of bile ducts 6 Q44.3 Congenital stenosis and stricture of bile ducts 1 Q44.4 Choledochal cyst 24 Q44.5 Other congenital malformations of bile ducts 1 Q44.6 Cystic disease of liver 3 Q45 Other congenital malformations of digestive system 11 Q79 Congenital malformations of the musculoskeletal system, not elsewhere classified Q79.0 Congenital diaphragmatic hernia 70 Q79.2 Exomphalos 23 Q79.3 Gastroschisis 13 ICD, International Classification of Diseases. 2 Journal of Korean Association of Pediatric Surgeons

체환자수는 893 명이었다. 질환으로는선천성식도폐색 128 예, 선천성소장폐색 155예, 쇄항 231예, 선천성장고정이상 100 예, 선천성담도폐색 6예, 총담관종 24예, 선천성횡격막탈장 70 예, 선천성복벽결손 36 예가있었다. 중복을고려하면전체질환은 961 예였다. ICD-10 에따른환자분포는 Table 1과같다. 수술은총 893 명에서시행되었다. 한번에두가지수술을시행한경우가 75 예, 세가지수술을시행한경우가 7예있어서, 총 975개의수술을시행하였다. 그중최소침습수술로시행한경우가 144 예로 16.1% 였다. 저출생체중아는전체 893 명중 213 명으로 24.0%, 미숙아는 127 명으로 13.2% 였고, 수술당시체중 2.5 kg 미만은 121 명으로 13.5% 였다. 수술시간은평균 108.7±75.9분, 중간값 95분 (5-820분) 이었다. 그중수술장체류시간은평균 157.3±89.3분, 중간값 142분 (3-946 분 ) 이었다. 재태연령, 출생시와수술시체중에따른비교는 Tables 2-4 와같다. 재태연령에따라나누었을때, 36주미만의미숙아는 127 명 (14.2%) 이었다. 미숙아와만삭아의평균수술시간은 114.9±68.3분과 107.8±77.1분이었고, 평균수술장시간은 162.6±73.2분과 156.7±91.5분으로미숙아에서시간이더걸렸으나통계적의미는없었다. 출생체중 2,500 g 미만의저출생체중아는 214명 (23.9%) 이었다. 저출생체중아의평균수술시간은 122.6±90.5 분으로정상체중아 104.5±70.2 분에비해수술시간이길었다 (p=0.002). 수술장체류시간도저출생체중아가 175.0±115.5분으로정상체중아 151.6± 78.6 분보다더길었다 (p=0.001). 수술시체중에따라비교해보았을때, 수술시체중 2,500 g 미만인경우평균수술 Table 2. The Comparisons according to Gestational Age (n=893) <36 wk 36 wk p-value No. of patients 127 766 Operation time (min) (A) 114.9±68.3 107.8±77.1 0.291 Operation room time (min) (B) 162.6±73.2 156.7±91.5 0.485 B A 45.1±23.2 48.6±31.9 0.113 Cost (10,000 Won) 3,467±4,287 1,332±1,617 0.000 시간과수술장체류시간 123.8±89.3 분과 177.0±11.7 분으로그이상인경우 103.9±70.2분과 151.2±78.6분에비해모두길었다 (p<0.05). 합병증은수혈을필요로하는출혈은수술중 35명 (3.9%), 수술후 51명 (5.7%) 에서있었다. 염증성합병증은 bacteremia 가 28명 (3.1%), 창상감염이 23명 (2.6%), 입원기간중재수술이 61건 (6.8%), 인공호흡기를일주일필요로하는경우가 111명 (12.4%) 에서있었다 (Table 5). 재원기간은평균 25.4±31.4 일, 중간값 17 일 (1-200일) 이었다. 사망은 48 명 (5.4%) 이었다. 그중신생아기사망은 31 명 (3.5%) 이었다. 진료비는평균 16,355,911±23,217,612원이었고, 중간값 10,033,871원 (276,274-266,652,505원) 이었다. 선천성소장폐색 ( 십이지장, 공장, 회장 ) 은 134예있었다 (Table 6). 최소침습수술은 22예 (16.4%) 에서시행되었다. 평균수술시간은 131.1±59.5분이었고, 전체수술장체류시간은 180.1± 65.4 분이었다. 재원기간은 33.5±37.8 일이었고, 신생아기사망은 3예 (2.2%) 에서있었다. 쇄항의저위기형에대한수술은 124예있었다 (Table 7). 전체환자중쇄항은 231 명이었고, 그중저위기형은 134명이었다. 평균수술시간은 55.1±53.0 분 (5-310 분 ) 이었고, 전체수술장체류시간은 95.9±60.2 분 (18-365분) 이었다. 재원기간은 14.4±13.4 일 (3-77 일 ) 이었고, 신생아기사망은 1 예 (0.8%) 에서있었다. 선천성식도폐색에대한수술은 103예 (11.5%) 였다 Table 4. The Comparisons according to Body Weight on Operation (n=893) <2,500 g 2,500 g p-value No. of patients 221 672 Operation time (min) (A) 123.8±89.3 103.9±70.2 0.003 Operation room time (min) (B) 177.0±11.7 151.2±78.6 0.000 B A 51.6±43.8 47.0±25.1 0.054 Cost (10,000 Won) 2,731±3,499 1,271±161.3 0.000 Table 5. Complication (n=893) Table 3. The Comparisons according to Birth Weight (n=893) <2,500 g 2,500 g p-value No. of patients 214 679 Operation time (min) (A) 122.6±90.5 104.5±70.2 0.002 Operation room time (min) (B) 175.0±115.5 151.6±78.6 0.001 B A 50.7±44.5 47.2±25.0 0.153 Cost (10,000 Won) 2,768±3,548 1,274±1,606 0.000 No. (%) of patients Intraoperative bleeding 35 (3.9) Postoperative bleeding 51 (5.7) Bacteremia 28 (3.1) Wound infection 23 (2.6) Other complication 37 (4.1) Reoperation during hospitalization 61 (6.8) Ventilator more than 1 wk 111 (12.4) Non-surgical complication 44 (4.9) Journal of Korean Association of Pediatric Surgeons 3

J Korean Assoc Pediatr Surg 2016;22(1):1-5 Table 6. The Status of Congenital Small Bowel Obstructions (n=134) Table 8. The Status of Esophageal Atresia (n=103) Combined operation 23 (17.2) Stomy operation 2 Gastrostomy 1 Operation for imperforate anus 5 Operation for rotation anomaly 6 Bowel resection and anastomosis 2 Operation for esophageal atresia 6 Operation for abdominal wall defect 1 Minimal invasive surgery 22 (16.4) Operation time (min) 131.1±59.5 (40-360) Operation room time (min) 180.1±65.4 (87-410) Complication 36 (26.9) Bleeding 17 Bacteremia 5 Wound infection 3 Others 11 Reoperation 9 (6.7) Ventilator care ( 7 day) 5 (3.7) Hospitalization (day) 33.5±37.8 (1-353) Perinatal mortality 3 (2.2) s are presented as n (%), n only, or mean±sd (range). Table 7. The Status of Imperforate Anus (n=124) Combined operation 7 (5.6) Operation for small bowel obstruction 3 Operation for esophageal atresia 4 Minimal invasive surgery 10 (8.1) Operation time (min) 55.1±53.0 (5-310) Operation room time (min) 95.9±60.2 (18-365) Complication 9 (7.3) Bleeding 1 Bacteremia 0 Wound infection 5 Others 3 Reoperation 2 (1.6) Ventilator care ( 7 day) 3 (2.4) Hospitalization (day) 14.4±13.4 (3-77) Perinatal mortality 1 (0.8) s are presented as n (%), n only, or mean±sd (range). (Table 8). 동반수술은 23 예 (22.3%) 에서시행되었다. 재수술은 8예 (7.8%) 에서시행되었고, 신생아기사망은 5명 (4.9%) 에서있었다. 선천성복벽결손에대한수술은 38명 (4.3%) 이었다 (Table 9). 재수술은 7명 (18.4%) 에서있었고, 신생아기사망은 2예 (5.3%) 있었다. 선천성횡격막탈장은 75 예있었다 (Table 10). 최소침습수술은 27예 (36.0%) 에서시행되었다. 신생아기사망은 15 예 (20.0%) 있었다. Combined operation 23 (22.3) Stomy 9 Gastrostomy 5 Operation for imperforate anus 4 Operation for small bowel atresia 3 Pyloromyotomy 1 Anal stricturoplasty 1 Minimal invasive surgery 18 (17.5) Operation time (min) 176.2±63.6 (64-344) Operation room time (min) 237.5±83.0 (96-661) Complication 28 (27.2) Bleeding 12 Bacteremia 6 Wound infection 2 Others 8 Reoperation 8 (7.8) Ventilator care ( 7 day) 43 (41.7) Hospitalization (day) 38.3±36.9 (12-271) Perinatal mortality 5 (4.9) s are presented as n (%), n only, or mean±sd (range). Table 9. The Status of Congenital Abdominal Wall Defects (n=38) Combined operation 9 (23.7) Excision of urachal cyst remnant 1 Small bowel resection and anastomosis 3 Primary closure for perforated bowel 1 Operation for small bowel atresia 1 Operation for rotation anomaly 3 Operation time (min) 78.1±50.4 (28-245) Operation room time (min) 116.8±52.7 (41-265) Complication 7 (18.4) Bleeding 3 Bacteremia 1 Wound infection 1 Others 2 Re-operation 7 (18.4) Ventilator care ( 7 day) 12 (31.6) Hospitalization (day) 30.8±33.9 (1-170) Perinatal mortality 2 (5.3) s are presented as n (%), n only, or mean±sd (range). 선천성장회전이상에대한수술은 62 예있었고, 신생아기사망은없었다 (Table 11). 장루수술은모든 153예 (17.1%) 시행되었다 (Table 12). 평균수술시간은 118.9±59.3 분, 수술장체류시간은 161.4 분이었다. 재수술은 18 예 (11.8%) 였고, 신생아기사망은 4명 (2.6%) 에서있었다. 신생아시기에시행된선천성유문근비후에대한유문근절개술은 49예에서시행되었다. 그중 18예 (36.7%) 가최소 4 Journal of Korean Association of Pediatric Surgeons

Table 10. The Status of Congenital Diaphragmatic Hernia (n=75) Table 12. The Status of Stomy Operation (n=153) Combined operation 3 (4.0) Operation for rotation anomaly 2 Stomy 1 Minimal invasive surgery 27 (36.0) Operation time (min) 118.9±59.3 (25-310) Operation room time (min) 161.4±64.3 (30-338) Complication 15 (20.0) Bleeding 10 Bacteremia 2 Wound infection 1 Others 2 Reoperation 5 (6.7) Ventilator care ( 7 day) 32 (42.7) Hospitalization (day) 20.7±13.7 (1-70) Perinatal mortality 15 (20.0) s are presented as n (%), n only, or mean±sd (range). Table 11. The Status of Congenital Rotational Anomaly (n=62) Combined operation 20 (32.3) Stomy 4 Operation for intestinal obstruction 2 Operation for small bowel atresia 7 Gastrostomy 1 Operation for abdominal wall defects 3 Kasai operation 1 Repair of congenital diaphragmatic hernia 2 Minimal invasive surgery 2 (9.2) Operation time (min) 107.2±60.4 (15-360) Operation room time (min) 162.0±74.5 (25-416) Complication 17 (27.2) Bleeding 5 Bacteremia 2 Wound infection 5 Others 5 Reoperation 3 (4.8) Ventilator care ( 7 day) 8 (12.9) Hospitalization (day) 27.1±30.9 (5-210) Perinatal mortality 0 s are presented as n (%), n only, or mean±sd (range). 침습수술로시행되었고, 수술시간은 53.9±39.5 분 (19-270 분 ), 수술장체류시간은 95.5±45.3 분 (40-321분) 이었다. 재수술은 3예 (6.1%) 있었다. Hirschsprung 병에대한일차교정수술은 58예에서시행되었고, 그중 14예 (24.1%) 에서최소침습수술로시행되었다. 담관낭종에대한수술은 25 예에서 Combined operation 30 (19.6) Operation for small bowel atresia 2 Operation for esophageal atresia 11 Bowel resection and anastomosis 4 Diverticulectomy 1 Gastrostomy 2 Operation for imperforate anus 7 Operation for rotation anomaly 2 Repair of congenital diaphragmatic hernia 1 Minimal invasive surgery 14 (9.2) Operation time (min) 118.9±59.3 (25-310) Operation room time (min) 161.4±64.3 (30-338) Complication 36 (23.5) Bleeding 16 Bacteremia 4 Wound infection 4 Others 12 Reoperation 18 (11.8) Ventilator care ( 7 day) 17 (11.1) Hospitalization (day) 20.7±13.7 (1-70) Perinatal mortality 4 (2.6) s are presented as n (%), n only, or mean±sd (range). 시행되었고, 그중 7예 (28.0%) 는최소침습수술로시행되었으며, 선천성담도폐색에대한수술은 8예에서시행되었다. 고찰 선천성기형에대한전국적인등록사업은매우중요한과제로일시적인설문조사가아닌지속적인대한소아외과학회의사업이되어야할것이다. CONFLICTS OF INTEREST No potential conflict of interest relevant to this article was reported. REFERENCE 1. Lee MD, Kim SY, Kim WK, Kim IK, Kim SC, Kim SK, et al. Index cases in pediatric surgery: a national survey by the Korean Association of Pediatric Surgeons, 2000. J Korean Assoc Pediatr Surg 2001;7:147-56. Journal of Korean Association of Pediatric Surgeons 5