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1 Korean Journal of Pediatric Urology Vol 1, No. 1: 46-50, 2009 ORIGINAL ARTICLE 배뇨중방광요도조영술에나타난남아의선천성요도이상 Congenital Urethral Abnormalities on the Voiding Cystourethrography in Boys Soo Dong Kim, Ju Hyung Seo, Chan Wook Shin, Won Yeol Cho From the Department of Urology, Dong-A University College of Medicine, Busan, Korea 김수동ㆍ서주형ㆍ신찬욱조원열 동아대학교의과대학비뇨기과학교실 교신저자조원열동아대학교병원비뇨기과부산시서구동대신동 3 가 1, Tel: Fax: urogate@dau.ac.kr Purpose: We aimed to investigate the relationship between vesicoureteral reflux (VUR) and the urethral abnormalities on the voiding cystourethrography (VCUG) in boys. Patients and Methods: Between October 2000 and September 2005, 91 boys, who presented with febrile urinary tract infection (UTI) or prenatal hydronephrosis, underwent a VCUG to evaluate the VUR. We also evaluated the urethral abnormalities and other urological anomalies. Results: The VUR was identified in 17 boys and the urethral abnormalities in 23. Four (23.5%) boys had VUR (3 high grade, 1 low grade) and urethral abnormalities as well. They underwent surgery for VUR and urethral abnormalities. Among 13 VUR patients without urethral abnormalities, 6 boys had high grade VUR. Patients with urethral abnormalities had more severe VUR. In the 19 boys who had urethral abnormalities without VUR, 9 (47.4%) had mild hydronephrosis or bladder wall thickening on ultrasonography. Conclusions: Our data suggest that the urethral abnormalities could contribute to aggravate the grade of VUR, and may account for secondary hydronephrosis or bladder wall thickening. So, for the appropriate diagnosis and treatment of VUR, careful observation of urethral abnormalities is very important. Key Words: Abnormalities, Congenital, Urethra (Received: March 6, 2009, Accepted: March 16, 2009) 서론소아에서방광요관역류는요로감염의위험인자로, 신반흔을남기며장기적으로신기능부전으로이어질수있는열성요로감염을유발하는경우가많다. 따라서방광요관역류의치료는신기능부전의발생을방지하기위한열성요로감염의예방과방광요관역류의소실에중점을두게된다. 소아방광요관역류의선천적원인으로방광요관이행부의근육발달미약또는요관의기형등으로나타나는구조적이상이가장흔하지만, 배뇨장애또한방 광요관역류를유발하는원인중하나로보고되고있다. 1.2 즉배뇨근불안정, 배뇨근-조임근협동장애와같은기능성배뇨장애를동반하거나후부요도판막과같은폐쇄성요도병변을가진경우방광요관역류를흔히동반하게된다. 이론의여지는있으나 1968년 Cobb 등 3 이처음보고한선천성구부요도협착 (Cobb's collar, anteriorurethral ring) 역시요도의폐쇄성병변으로배뇨장애를일으키게되어방광요관역류를초래할수있다고하였다. 선천성구부요도협착이있는환아에서배뇨장애를일으키는폐쇄성병변을제거해주면방광요관역류도자연소실되었다는보고들 4,5 을볼때요도이상역시방광요관역류의원인중하나이 46
2 김수동외 : 배뇨중방광요도조영술에나타난남아의선천성요도이상 47 며이에대한적절한진단과치료가중요할것으로생각한다. 이에저자들은열성혹은반복적인요로감염과산전수신증으로내원한환아중임상적으로방광요관역류가의심되어배뇨중방광요도조영술을시행한남아를대상으로방광요관역류와요도의구조적이상의존재유무를관찰하였고둘사이의상관관계에대하여알아보고자하였다. 1. 연구대상 Table 1. Characteristics of patients Characteristics Patient (%) No. of patients Mean age (mos) (range) <1 year 1 year Grade of reflux* Low grade High grade Laterality Bilateral Unilateral 대상및방법 2000년 10월부터 2005년 9월까지고열혹은반복적인요로감염이나산전수신증으로내원하여임상적으로방광요관역류가의심되어배뇨중방광요도조영술을시행한소아환아 140명중남아 91명을대상으로하였다. 전체 91명의나이는 1-110개월로평균연령은 27개월이었다. 연령별분포는 12개월미만이 71명, 12 개월이상은 20명이었다. 17명 (18.7%) 에서방광요관역류가관찰되었으며, 이중 8명 (47.1%) 은양측성이었고 9명 (52.9%) 은단측성이었다 (Table 1). 그중 12개월미만이 10명 (58.8%), 12개월이상이 7명 (41.2%) 이었다. 2. 연구방법 각환아의의무기록을후향적으로분석하여임상증상과치료및경과에대하여조사하였으며, 배뇨중방광요도조영술사진을재검토하여방광요관역류를확인하고동반된요도의기형유무를판단하였다 (1-110) 71 (78) 20 (22) 8 (47) 9 (53) 8 (47) 9 (53) *: low grade (grade I-III), high grade (grade IV-V) 배뇨중방광요도조영술에서방광요관역류의존재유무에따라방광요관역류가있는군과없는군으로분류하였다. 방광요관역류의정도는 International Reflux Study Committee에서정한등급체계를기준으로분류하였고 I, II, III 등급을저등급, IV, V 등급을고등급으로나누었다. 요도의기형에대한평가는 Cobb의고리 (Cobb's collar) 3 혹은선천성폐쇄성후부요도막 (congenital obstructive posterior urethral membrane; COPUM) 6 및 Young 등 7 이분류한후부요도판막 (posterior urethral valve) 이의심되는소견이있거나외요도괄약근부위의수축과함께후부요도의확장이있는경우요도이상이있음으로정의하였다. 방광요관역류의존재와요도이상의유무에따른역류등급의상관관계를분석하여요도이상이방광요관역류에미치는영향을분석하였으며, 역류및요도이상에대한수술적교정여부, 역류의소실정도및임상경과에대하여도알아보았다. 방광요관역류가없는경우는신장방광초음파소견을확인하여요도폐쇄가이차적으로신장이나방광의변화를초래하는지에대하여조사하였다. Table 2. Number of patients of urethral abnormality on voiding cystourethrography VUR (+) VUR ( ) 결 배뇨중방광요도조영술에서후부요도협착또는 Cobb s 고리로진단받은환아는 23명 (25.3%) 으로 12개월미만이 14명 (60.9%), 12개월이상이 9명 (39.1%) 이었다 (Table 2). 방광요관역류를보인 17명의환아중 4명 (23.5%) 에서요도이상을동반하였다. 반면방광요관역류가없는환아중에도 19명 (25.7%) 에서요도이상소견을보였다 (Table 2, Fig. 1). 방광요관역류와요도이상을함께가진 4명의환아에서고등급역류는 3명 (75%), 저등급역류는 1명 (25%) 이었다. 4명의환아모두예방적항생제를사용하였으나증상의호전없어고등급역류를보인 3명에서역류교정수술을시행 Urethral abnormality No. of patients (%) + + 과 4 (4.4) 13 (14.3) 19 (20.9) 55 (60.4) Total 91 (100)
3 48 Korean Journal of Pediatric Urology Vol. 1, 46-50, 2009 Fig. 1. Voiding cystourethrography. (A) Urethral abnormality with VUR. (B) Urethral abnormality without VUR. Table 3. Relationship between vesicoureteral reflux and the urethral abnormalities on the voiding cystourethrography Grade High Low VUR with uretrhral VUR without abnormality uretrethral abnormality Total 3 1 Total 하였고저등급역류 1명에서 Dextranomer/hyaluronic acid copolymer (Deflux R ) 내시경적주사법을시행하였다. 술후경과관찰중증상의재발및악화소견을보이는 1명의환아에서역류교정수술을재시행하였고, 3명의환아는술전요도경검사에서 Cobb의고리가있어내시경적요도절개술을시행하였다. 요도이상없이방광요관역류를보인 13명중고등급역류는 6명 (46.1%), 저등급역류는 7명 (53.9%) 이었다 (Table 3). 고등급역류환아중 2명은예방적항생제복용에도불구하고증상재발되어역류교정수술을시행하였다. 나머지 4명의환아는 1년이상의예방적항생제사용후증상재발은없었고, 역류등급은저등급으로감소하였다. 저등급역류환아는예방적항생제사용만으로증상소실을보였다. 방광요관역류를동반하지않고요도이상소견만을보인 (Fig. 1B) 경우, 함께시행한신장방광초음파에서경도이상의수신증이 5명에서보였고, 4명에서는방광벽의비후를보여 19명중 9명 (47.4%) 에서명확한역류없이폐쇄성병변에의한이차적인변화로생각되는소견이관찰되었다. 방광요관역류를보이는경우까지포함하면 23명중 13명 (56.6%) 에서신장및방광의이 상소견을동반하였다. 고찰방광요관역류에배뇨장애의개념이처음도입된 8,9 이후방광요관역류의진행과치료에있어서요도의구조적이상과기능적배뇨장애에대한관심은점차증가하고있다. 10 Nasrallah와 Simon 11 은기능성배뇨장애를보이는방광요관역류환자에게요로감염의방지와배뇨습관의교정에중점을두어치료한결과방광요관역류의소실이쉽게이루어짐을보고하였다. 방광요관역류환자에서배뇨장애를무시하고교정수술을시행할경우, 술후요로감염의빈도가증가하고, 12 내시경적주입법의성공률을감소시킨다는보고도있다. 13 즉, 방광요관역류의일부는배뇨장애가원인인자로작용하며, 이에대한치료가방광요관역류치료의성공과실패를좌우하는중요한인자임을알수있다. 기능적배뇨장애와함께요도의폐쇄를일으키는해부학적이상도방광요관역류와밀접한관계를가지고있다. 소아에서요도의폐쇄성병변으로가장흔한것이후부요도판막증이며그빈도는남아 5,000-8,000명중 1명으로보고되고있고 14 처음발견시에후부요도판막증환자의 30-70% 에서방광요관역류를동반하고있다. Cobb s 고리또는 Moorman 환 (Moorman s ring) 이라고명명된선천성구부요도협착은 1968년미국의 Cobb 등 3 과 1972년독일의 Moorman 15 이질환단위로서보고하여임상적중요성을경고하였다. 선천성구부요도협착은발생학적으로외배엽조직으로부터발생하는요도의근위부 ( 전립선요도와막양부요
4 김수동외 : 배뇨중방광요도조영술에나타난남아의선천성요도이상 49 Fig. 2. Calssification of Cobb's collar. P: prostate, S: external sphincter, C: Cobb's collar 도 ) 는요생식동의음경부에서형성된요도와막양부요도의바로원위부에서만나게되는데이곳이구부요도와막모양요도의접합부라고주장하고, 이부분에배설강막 (cloacal membrane) 이불완전하게파열되어고리를생성한다고주장하였다. 16 선천성구부요도협착을 Cranston 등 17 은 3가지로분류하였는데, type I 은외요도괄약근직하에등성이형태 (ridge of tissue) 로, type II는명확한협착으로, type III는매우작은구멍의형태로나타난다고하였다 (Fig. 2). 하지만이러한선천성요도협착은질환단위로서는거의무시되고있었으나일본에서 Mori 등 18 이소아비뇨기과의중요한질환으로다루었고, 국내에서도 Cho 등 5 이배뇨중방광요도조영술에서 Cobb의고리를발견하여보고하는등그임상적중요성이새로이평가받고있다. 이를 Dewan 등 6 은다시외요도조임근과다른명확한사진을증거로하여선천성폐쇄성후부요도막으로정리하였으며, Stephen은 Young의후부요도판막분류에하나를더하여 type IV라고명명하였는데이는 Cobb 등과 Moorman이기술한고리혹은환과그위치와특성이일치한다. 19 Nonomura 등 4 은 Cobb의고리가발견된 39례에서경요도절개술만으로방광요관역류가소실혹은호전된다고하는등선천적구부요도협착은그존재와병적의미에대한이론이많지만그임상적인의미는무시할수없다. 저자들의연구에서도요도이상이있을때방광요관역류를동반한경우는 23례중 4례 (17.4%) 가있었으며, 방광요관역류없이요도이상소견만보인 19명의환아중 9명 (47.4%) 에서, 수신증과방광벽의비후를보여요도의 이상소견이발견된환아의 56.6% 에서요도의폐쇄성병변이이차적인신장과방광의이상을초래한것으로생각할수있었다. 비록이번연구에서요도이상유무와방광요관역류가동반되는빈도는차이가없었으나요도이상이있는경우역류의정도는요도이상을동반하지않은경우보다그정도가심한경향을보였고, 요도이상을동반하지않은방광요관역류환아에서는고등급역류라하더라도수술적교정이덜필요하였고, 항생제복용만으로도역류등급의감소를보였다. 결 영유아기의남아에서발견되는기능적배뇨장애나구조적요도이상은방광요관역류의악화및방광벽의비후, 경도의수신증을유발할수있으며, 또한그경과에도많은영향을미치므로방광요관역류를가진소아에서요도에대한연구와관찰은주의깊게시행되어야할것으로생각한다. 론 REFERENCES 1. Soygur T, Arikan N, Yesilli C, Gogus O. Relationship among pediatric voiding dysfunction and vesicoureteral reflux and renal scars. Urology 1999;54: Sillen U. Bladder dysfunction in children with vesicoureteric reflux. Acta Paediatr Suppl 1999;88: Cobb BG, Wolf JA Jr, Ansell JS. Congenital stricture of the proximal urethral bulb. J Urol 1968;99:629-31
5 50 Korean Journal of Pediatric Urology Vol. 1, 46-50, Nonomura K, Kanno T, Kakizaki H, Koyama T, Yamashita T, Koyanagi T. Impact of congenital narrowing of the bulbar urethra (Cobb's collar) and its transurethral incision in children. Eur Urol 1999;36: Cho WY, Yoon JB. Two cases of congenital urethral stenosis with recurrent urinary tract infection. Korean J Urol 1994;35: Dewan PA, Keenan RJ, Morris LL, Le Quesne GW. Congenital urethral obstruction: Cobb's collar or prolapsed congenital obstructive posterior urethral membrane (COPUM). Br J Urol 1994;73: Young HH, Frontz RH, Baldwin TC. Congenital obstruction of the posterior urethra. J Urol 1919;3: International Reflux Study Commitee. Medical versus surgical treatment of primary vesicoureteral reflux. Pediatrics 1981;67: Hinman F Jr, Baumann FW. Complications of vesicoureteral operations from incoordination of micturition. J Urol 1976;116: Han SW. The overlooked cause of vesicoureteral reflux in children-urethral abnormalities. Korean J Urol 2004;45: Nasrallah PF, Simon JW. Reflux and voiding abnormalities in children. Urology 1984;24: Barroso U Jr, Jednak R, Barthold JP, Gonzales R. Outcome of ureteral reimplantation in children with the urge syndrome. J Urol 2001;166: Capozza N, Lais A, Matarazzo E, Nappo S, Patricolo M, Caione P. Influence of voiding dysfunction on the outcome of endoscopic treatment for vesicoureteral reflux. J Urol 2002;168: King LR. Vesicoureteral reflux. In: Kelalis PP, King LR, Belman AB, Editors. Clinical pedicatric urology. 2nd ed. Philadelphia: W.B. Saunders Co.; 1985; Moormann JG. Congenital bulbarurethral stenosis as a cause of diseases of the urogenital junction. Urologe A 1972;11: Arey LB. Developmental anatomy. A textbook and laboratory manual of embryology. 7th ed. Philadelphia: Saunders; 1965; Cranston D, Davies AH, Smith JC. Cobb's collar-a forgotten entity. Br J Urol 1990;66: Mori Y, Matsui M, Ogino T, Hosokawa S, Tsujimoto S, Ihara H, et al. Treatment of congenital urethral stenosis (urehtral ring) in children: optic internal urethrotomy in the congenital bulbar urethral stenosis in boys. Jpn J Urol 1989;80: Stephen FD. Congenital intrinsic lesions of the posterior urethra. In: Stephens FD, Smith ED, Hutson JM, editors. Congenital anomalies of the urinary and genital tracts. Oxford: Isis Medical Media; 1996;91-116
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