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1 99 소아외과 : 제 12 권제 1 호 2006 년 Vol. 12, No. 1, June 2006 항문직장기형 - 진단과신생아기처치 - 영남대학교의과대학외과학교실 이남혁 서 론 항문직장기형은출생후기본적으로시행되는신생아이학적검사에서정상위치에항문이없는것을봄으로쉽게알수있다. 그러나기형의종류가다양하고치료도그에따라달라서저위기형은신생아기에바로교정하지만고위기형은일단결장루를설치하고기형의본교정은수주혹은수개월뒤에한다 1. 그리고항문직장기형은동반기형의빈도가높다. 따라서직장항문기형의신생아기접근의요점은우선생명을위협하는동반기형이있는지확인하고적절한처치를하는것과기형을신생아기에바로교정할것인지아니면결장조루술을시행할것인지를판단하는데있다 1,2. Initial general management 항문직장기형은위장관폐쇄의일종이나장폐쇄로출생후조기에응급을요하지는 접수일 : 05 / 10 / 18 교신저자 : 이남혁, 대구광역시남구대명동 번지영남대학교의과대학외과학교실 Tel : 053) , Fax : 053) nhlee@med.yu.ac.kr 않는다 2. 그러므로항문이없는신생아는먼저금식과구위관삽관으로구토와기도흡인을방지하고수액과항생제를투여하면서기형의유형과동반기형에대한진단을실시한다 2,3. Decision making in newborns with anorectal malformations ( 그림 1,2) 기형의유형을진단하고결장루설치가필요한지판단하는데가장중요한방법은회음부에대한시진이다. 회음부의모양과태변이배출되는누공의존재와위치를확인함으로남아들의 % 와여아들의 90 % 정도에서시진만으로결장조루술의필요여부를판단할수있다 1. 외견상회음부의항문자리가우묵하게들어가그표시가완연하고회음부고랑이잘형성되어있거나 bucket handle deformity가있으면대계회음부누공이있는저위기형이고반대로회음부가편평하고회음부고랑이분명하지않으면고위기형이며항문거상근과괄약근의발육이미약한경우가많다 2,3. 시진으로회음부누공이확인되면남녀모두신생아

2 100 소아외과제 12 권제 1 호 2006 년 Newborn male with anorectal malformation Perineal Spine Sacrum observation Kidney US Spine US Urinalysis Cardiac Echo R/O Esophageal atresia hours Clinical evidence No clinical evidence Perineal fistula Bucket handle deformity Midline raphe fistula Anoplasty Mini PSAP No colostomy Meconium in urine Flat bottom colostomy PSARP 4-8wks Cross table lateral view > 1cm skin rectum distance <1cm skin rectum distance Mini PSAP No colostomy Fig. 1.Algorithm for neonatal management of the anorectal malformations in a male Newborn female with anorectal malformation Perineal observation Spine Kidney U/S Cardiac Echo Sacrum Spine U/S R/O esophageal atresia hours Single perineal orifice Cloaca COLOSTOMY Drain hydrocolpos Urinary diversion (If necessary) 6 mos Urol evaluation R/O hydrocolpos Vestibular fistula COLOSTOMY or Primary repair 4-8 wks Perineal fistula Anoplasty mini PSAP No colostomy No visible fistula Cross table lateral x-ray < 1cm skin rectum distance > 1cm skin rectum distance PSARVUP PSARP COLOSTOMY Fig. 2.Algorithm for neonatal management of the anorectal malformations in a female

3 이남혁 : 항문직장기형 - 진단과신생아기처치 기에바로기형을교정하지만남아의직장요로누공과여아의직장전정부누공, 직장질누공, 그리고총배설강기형은신생아기에결장루를설치한다. 이때고려할점은좁은누공을통해서직장의태변이외부로배출되기위해서는복압과직장의압력이상당히상승해야함으로누공이있어도생후수시간내에는태변이배출되지않는경우가많은것이다. 그러므로누공의유무에대한판단은출생후적어도 16-24시간정도지켜본후에하고기다리는동안에동반기형에대한검사를한다 2,3. 남아에서직장요로누공이있으면소변에태변과공기가섞여나오고소변검사에서편평상피세포를볼수있고사진상방광에공기음영이보일때가있다 3. 남아의성기에물에적신작은가제를올려두면소변에태변이섞여나오는지확인하는데도움이된다 2. 여아의외음부로태변이배출되면환자가움직이지않게잘고정한다음밝은조명아래에서외음부를자세히살펴보고외음부에있는개구부의수와위치로총배설강기형, 직장질루, 그리고직장전정부루를감별한다. 총배설강기형은여아의항문직장기형중가장고위기형으로외음부의크기가작고개구부가하나만있다. 총배설강기형은항문폐쇄이외에질폐쇄에의한수질증이나요로폐쇄로인한수신증을동반하고있을때가많고이를간과하면요로성폐혈증이나대사성산증에빠질위험이높다 5,6. 그러므로결장조루술을시행하기전에반드시복부및골반초음파검사로수질증과수신증의유무를확인하여필요하면결장루를설 치할때 vaginostomy나 cystostomy 를같이시행하도록한다 6. 대부분의수신증은질이확장되면서앞쪽의방광삼각부를압박하여요관에서방광으로소변의흐름에장애를초래하여생기고수질증을배액하면대부분해결된다. 수질증은먼저공통관으로도관을삽관하여배액을시도하고도관삽관만으로해결되지않으면치골상방에 tube vaginostomy를설치하고질이너무확장되어배꼽위까지올라올경우는 vaginostomy 로피부로바로배액하기도한다. 요도가너무좁거나방광의기능장애가있으면 cystostomy를시행한다 7,8. 총배설강기형으로결장루를설치할때는교정수술시긴장없이 pull through 할수있고또혹시질재건술이필요하면직장을이용할수있게장루원위부에충분한길이의결장을남기도록한다 5,6. 회음부에요도구와질구만있으며질구에서태변이배출되면직장질루로결장루술을시행한다. 그러나직장질루는빈도가아주드물고가끔질구아래에누공이있는직장전정부루와혼돈할수있으므로태변이질구의처녀막안쪽에서올라오는지분명히확인해야한다 3,9. 그리고간혹질구로보이는것이실제는전정부누공이고요생식동이분리되지않아질과요도가서로만나요도구에개구하거나아예무질증이어서외음부에개구부가둘만있을수있을수있으므로방사선검사나내시경검사로감별하여야한다 10,11. 약 24시간이후에도누공의증거가없는환자들은방사선검사로직장맹단이항문으로얼마나내려와있는지를보고결장루설치여부를결정한다 2,3. 직장말단부의위치를

4 102 소아외과제 12 권제 1 호 2006 년 방사선검사로정확하게알기위해서는직장상부의장내공기가직장을둘러싸고있는괄약근의압력을극복하고또찐득찐득한태변사이를지나직장말단부까지내려와야함으로방사선검사는출생후적어도 24시간정도지나고시행하도록한다. 고식적인방법은도립위에서시행하는 Wangensteen-Rice view 12 이나요즘은 cross table lateral view를많이시행하고있으며 13,14. 그외에초음파 15,16, CT 17, 혹은 MRI 18 검사를하거나직장을가는바늘로천자하여직장안으로조영제를주입하는방법을쓰기도한다 19. 방사선검사로직장맹단과항문위치의피부간의거리를측정하여 1cm 이내이면기형을바로교정하지만그이상이면결장조루술을시행한다 2,3. Associated anomalies 항문직장기형의 % 는하나이상의합병기형을동반하며일반적으로고위기형일수록동반기형의빈도가높다 1,20. 비뇨생식기의기형이 % 정도에서동반되며결장조루술을시행하기전에반드시복부와골반의초음파검사로비뇨생식기의기형을확인하고만약수술적처치가필요하면같이하도록한다. 척추및천골의기형의빈도는 % 정도이고 tethered cord도높게는 25 % 정도까지보고되고 21 있어척추와골반의 x-선검사와척추의초음파검사를하고필요하면 MRI를시행한다. 심장기형은약 10 % 정도에서동반되며이학적검사나흉부 x-선검사에서의심되면심장초음파검사를시행하여확인한다. 위장관기형중에서 는식도폐쇄증이 5 % 정도로가장흔히동반되며증상과구위관을삽관하는과정에서대부분알수있다 1,4. 그외에 VACTERL association, 다운증후군, Currarino triad(sacral vertebral anomaly, presacral tertoma, anorectal malformation), Townes-Brocks syndrome(anal, renal, limb, and ear anomalies) 과같은기형복합체를동반하기도한다 22. Colostomy 직장항문기형환자에서시행하는결장루는일반적으로하행결장과 S결장의경계부에양분형으로조성하고시술후원위부결장내의태변을깨끗이세척해서태변이굳어져직장에매복되는것을방지한다 3. 분리형장루는루우프형장루보다시술하는과정은조금복잡하지만대변이원위부결장으로넘어가는것을좀더완전히차단하여직장의분변매복과요로감염그리고본교정수술시수술창의대변오염과감염을좀더효과적으로방지할수있다 2. 결장루를조성하는위치는장루아래쪽으로배안에남는원위부결장의길이가교정수술시 pull-through를긴장없이시행하기에충분히여유가있는곳에한다. 그러나횡행결장루는장루하부에남는결장의길이가너무길어교정수술전에장세척과 distal colostogram 을시행하기가용이하지않고만약직장요로누공이있으면누공으로넘어온소변이장루로배출되지못하고결장점막에서흡수되어대사성산증을유발할수있다 3,23. 그리고횡행결장은하행결장보다유동성많

5 이남혁 : 항문직장기형 - 진단과신생아기처치 아점막탈출같은장루관련합병증의빈도가높다 따라서항문직장기형환자에서결장루는일반적으로 S-결장의근위부혹은하행결장과 S결장의경계부에완전분리형으로설치할것을권장한다 3. Neonatal Repair 회음부누공이있는환자에서정상항문의위치는회음부의모양을보거나 muscle stimulator 를이용하여괄약근의수축양상을보면쉽게확인할수있다. 누공이항문위치와가까울때는단순한 cutback procedure 나 Y-V 항문성형술같이항문의크기를뒤쪽으로넓혀주는술식만으로도충분하지만누공이항문으로부터앞쪽으로많이떨어져있을때는항문을괄약근내에위치시키고 perineal body를넓게재건하여대변으로인한질과요도의오염을방지하기위해항문전위술이나미니페냐수술을한다 4,26. 그리고누공의앞쪽을박리가필요한수술을할때에는반드시폴리관을삽관하여수술도중요도손상을방지하여야한다 1,27. 최근에는여러선천성기형의치료를좀더조기에그리고가능하면다단계수술보다는한번의수술로교정하려하고 28 술식도복강경을이용한수술이많이시도되고있다 29,30. 고위기형의직장항문기형도여러번에걸친마취와수술을한번으로줄이고장루관련합병증을피하기위해신생아기에바로교정수술을시행하기도한다 14,31,32. 그러나신생아들은해부학적으로미성숙하고기형의해부학적구조를가장명확히알수있는 distal colostogram없이수술하기에질, 방광, 요도, 그리고정관등의주위조직에손상을줄수있고 27 상부에장루가없으므로수술창이대변으로부터안전하지못해창상감염과창상파열의위험이높다 3. 그러므로고위항문직장기형에서교정수술시기의선택은술자의경험에달렸지만합병증과그에따른결과를고려해서신중히결정해야하고아직은신생아기에는결장루를설치하는 3기식접근이일반적인방법이다. 참고문헌 1. Paidas CN, Levitt MA, Pena A: Rectum and anus, in Oldham KT, Colombani P- M, Foglia RP, Skinner MA(eds): Principles and Practice of Pediatric Surgery, chap 89. Philadelphia PA, Lippincott Williams Wilkins, 2005, Pp Pena A: Management of anorectal malformations during the newborn period. World J Surg 17: , Shaul DB, Harrison EA: Classification of anorectal malformation-initial approach, diagnostic test, and colostomy. Semin Pediatr Surg 6: , Keily EM, Pena A: Anorectal malformations, in O'neill JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG(eds): Pediatric Surgery(ed 5), chap 95. St. Louis, Mosby, 1998, Pp Hendren WH: Cloaca, the most severe degree of imperforate anus. Ann Surg 228: , Pena A, Levitt MA, Hong AR, Midulla PS: Surgical management of cloacal malformations: a review of 339 patients. J P- ediatr Surg 39: , Levitt MA, Pena A: Pitfall in the management of the newborn cloaca. Pediatr S- urg Int 21: , 2005

6 104 소아외과제 12 권제 1 호 2006 년 8. Shimada K, Hosokawa S, Matsumoto F, Johnin K, Naitoh Y, Harada Y: Urological management of cloacal anomalies. Int J Urol 8: , Pena A: Anorectal malformations. Semin Pediatr Surg 4:35-47, Rosen NG, Hong AR, Soffer SZ, Rodriguez G, Pena A: Rectovaginal fistula: a common diagnostic error with significant consequences in girls with anorectal malformations. J Pediatr Surg 37: , Levitt MA, Stein DM, Pena A: Rectovaginal fistula with absent vagina: a unique anorectal malformation. J Pediatr Surg 33: , Wangensteen GH, Rico CO: Imperforate anus. a method of determining the surgical approach. Ann Surg 92:77-81, Narasimharao KL, Prasad GR, Katariya S: Prone cross-table lateral view, an alternative to the invertogram in imperforate anus. Am J Radiol 140: , Goon HK: Repair of anorectal anomalies in the neonatal period. Pediatr Surg Int 5 : , Donaldson JS, Black CT, Reynolds M, Sherman JD, Shkolnik A: Ultrasound of the distal pouch in the infants with imperforate anus. J Pediatr Surg 24: , Schuster SR, Teele RL: An analysis of ultrasound scannig as a guide in determining "high" or "low" imperforate anus. J Pediatr Surg 14: , Ikawa H, Yokoyama J, Sanbonmatsu T, Hagane K, Endo M, Katsnmata K, Kohda E: The use of computerized tomography to evaluate anorectal anomalies. J Pediatr Surg 20: , Sachs TM, Applebaum H, Touran T, Taber P, Darakjian A, Colleti P: Use of MRI in evaluation of anorectal anomalies. J Pediatr Surg 25: , Wagner ML, Harberg FJ, Kumar AP, Singleton EB: The evaluation of imperforate anus utilizing percutaneous injection of water soluable iodide contrast material. Pediatr Radiol 1:34-40, Endo M, Hayashi A, Ishihara M, Marie M, Nagasaki T, Saeki M: Analysis of 1992 patients with anorectal malformations over the past two decades in Japan. J Pediatr Surg 34: , Levitt MA, Patel M, Rodriguez G, Gaylin DS, Pena A: The tethered spinal cord in patients with anorectal malformations. J Pediatr Surg 32: , Sydorak RM, Albanese CT: Laparoscopic repair of high imperforate anus. Semin Pediatr Surg 11: , Wilkins S, Pena A: The role of colostomy in the management of anorectal malformations. Pediatr Surg Int 3: , Gardikis S, Antypas S, Mamoulakis C, Demetriades D, Dolatzas T, Tsalkidis A, Chatzimicael A, Polychronidis A, Simopoulos C: Colostomy type in anorectal malformations: 10-year experience. Minerva Pediatr 56: , Patwardhan N, Kiely EM, Drake DP, Spitz L, Pierro A: Colostomy for anorectal anomalies: High incidence of complications. J Pediatr Surg 36: , Javid PJ, Barnhart DC, Hirschl RB, Coran AG, Harmon CM: Immediate and long-term results of surgical management of low imperforate anus in girls. J Pediatr Surg 33: , Hong AR, Acuna MF, Pena A, Chaves L, Rodriguez G: Urologic injuries associated with repair of anorectal malformations in male patients. J Pediatr Surg 37: , Pierro A: Recent advances in neonatal

7 이남혁 : 항문직장기형 - 진단과신생아기처치 surgery. Semin Neonatol 8: , Georgeson KE, Robertson DJ: Minimally invasive surgery in the neonate: review of current evidence. Semin Perinatol 28: , Georgeson KE, Inge TH, Albanese CT: Laparoscopically assisted anorectal pull-through for high imperforated anus-a new technique? J Pediatr Surg 35: , Moore TC: Advantages of performing the sagittal anoplasty operation for imperforate anus at birth. J Pediatr Surg 25: , Albanese CT, Jennings RW, Lopoo JB, Bratton BJ, Harrison MR: One-stage correction of high imperforate anus in the male neonate. J Pediatr Surg 34: , Liu G, Yuan J, Geng J, Wang C, Li T: The treatment of high and intermediate anorectal malformations: one stage or three procedures? J Pediatr Surg 39: , 2004

8 106 소아외과제 12 권제 1 호 2006 년 Anorectal Malformations:Diagnosis and Management in Neonatal Period Nam-Hyuk Lee, M.D. Department of Surgery, College of Medicine, Yeungnam University Daegu, Korea Anorectal malformations comprise a spectrum of disease and the majority of patients have one or more abnormalities that affect other systems. In evaluating a newborn with anorectal malformation, the decision regarding the need for a colostomy and detection and management of any life threatening associated anomalies are thetwo most important considerations. Perineal inspection provides the clue to the surgical approach in about % of male and 90 % of female newborn baby. The remaining patients who do not show any clinical evidence need radiologic evaluation to decide whether a colostomy should be performed. In most cases the decision to make a colostomy should not be made until the baby is 20 to 24 hours old and evaluation to rule out the presence of associated anomalies completed. A divided colostomy at the junction of the descending and sigmoid colon is recommended for anorectal malformations. (J Kor Assoc Pediatr Surg 12(1):99~106), Index Words:Anorectal malformation, Diagnosis, Management, Newborn Correspondence:Nam-Hyuk Lee, M.D., Department of Surgery, College of Medicine, Yeungnam University Daemyung-Dong, Nam-Gu, Daegu , Korea Tel : 053) , Fax : 053) nhlee@med.yu.ac.kr

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<4D F736F F F696E74202D20C1F7C0E5C7D7B9AEB1E2C7FC20C8AFBEC6BFA1BCAD20B5BFB9DDC7CFB4C220C0CCBAD0C3B4C3DFC1F52E707074> 직장항문기형환아에서동반하는이분척추증 -12 예환아에대한전향적연구 김성민 1, 장혜경 2, 심규원 3, 김동석 3, 오정탁 2, 최승훈 4, 한석주 2 연세대학교의과대학외과학교실, 용인세브란스병원외과 1 연세대학교의과대학외과학교실, 세브란스어린이병원소아외과 2 연세대학교의과대학신경외과학교실, 세브란스어린이병원소아신경외과 3 연세대학교의과대학외과학교실, 강남세브란스병원소아외과