대한초음파의학회지 2013; 32(2) 선천성수신증의초음파검사 이미정 Ultrasonography of Congenital Hydronephrosis 연세대학교의과대학세브란스어린이병원영상의학과방사선의과학연구소 J Korean Soc Ultrasound Med 2013;

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1 대한초음파의학회지 2013; 32(2) 선천성수신증의초음파검사 이미정 Ultrasonography of Congenital Hydronephrosis 연세대학교의과대학세브란스어린이병원영상의학과방사선의과학연구소 J Korean Soc Ultrasound Med 2013; 32: Received October 31, 2012; ccepted November 9, ddress for reprints : Mi-Jung Lee, MD, Department of Radiology and Research Institute of Radiological Science, Severance Children s Hospital, Yonsei University, 50 Yonsei-ro, Seodaemun-gu, Seoul , Korea. Tel Fax mjl1213@yuhs.ac Mi-Jung Lee, MD Department of Radiology and Research Institute of Radiological Science, Severance Children s Hospital, Yonsei University, College of Medicine, Seoul, Korea Hydronephrosis is the most common urogenital anomaly with the potential for obstructive process. nd ultrasonography, as a first-line imaging modality, is a useful tool for evaluation of prenatal and postnatal hydronephrosis, even though evaluation of renal function is still needed. In this article, we review the common causes of congenital hydronephrosis and the sonographic findings of those diseases. Key words : Ultrasonography; Hydronephrosis; Vesico-Ureteral Reflux; Ureterocele 서론선천성수신증은물리적또는기능적폐쇄에의해발생할수있다. 물리적폐쇄를일으키는원인으로는신우요관이행부폐쇄 (ureteropelvic junction obstruction), 중복요관 (duplex collecting system anomalies), 선천성거대요관 (primary megaureter), 그리고후부요도판막 (posterior urethral valve) 와같은병들이포함되며, 기능적폐쇄를일으키는원인으로는방광요관역류나 prune belly syndrome 등이포함된다 [1]. 대부분의선천성수신증은산전초음파에서발견되나, 간혹종괴처럼만져지거나옆구리통증, 혈뇨, 요로감염등의증상으로발견되기도한다. I. 검사방법산전초음파에서수신증이발견된경우, 생후다시초음파를하여이를확인하게된다 [2]. 그러나출생직후에는탈수나사구체여과율감소에의해수신증이없어보이거나정도가낮게 평가될수있기때문에, 생후 4-5일이후에검사하는것이원칙이다 [3]. 출생후초음파에서중등도이상의수신증이확인되면추가검사로방광요관역류확인을위한 voiding cystourethrography (VCUG) 와상대신기능평가를위한 diuretic renogram를시행하게된다 [4, 5]. 그러나신생아의신장은미숙하고이뇨제에대한반응이저하되어있을수있기때문에 diuretic renogram은생후 1-2개월후에하는것이좋다 [6]. 생후초음파에서수신증이보이지않거나경한확장이보이는경우추가검사나추적초음파검사에대해서는아직이견이많은상태이다. 그러나수신증이지속되는경우 VCUG를시행할수있고 [7], 수신증이보이지않는경우에도보통 1-2년후추적초음파검사를하게된다 [6]. 수신증진단은단순히 central echo complex의분리및확장과신배확장소견으로하며, 요관확장정도는폐쇄정도, 소변생성량, 요로계의자발적감압유무에따라결정된다. 그러나요관확장이모두요로폐쇄를의미하는것은아니며, 방광요관역류나요로감염, 요붕증 (diabetes insipidus) 에의해서도비

2 대한초음파의학회지 2013;32(2) 폐쇄성요관확장이생길수있다. 또한 renal hilum 부위에돌출된혈관에의해서도수신증과같은소견이나타날수있다. 신장내혈관에대한도플러검사도폐쇄성과비폐쇄성수신증을감별하는데도움이될수있다. 폐쇄성수신증에서는 renin과 angiotensin 등의호르몬이증가하여전체적인동맥혈관수축이나타나, 확장기동맥혈속도가낮아지고 resistive index(ri) 가높아지게된다 [1]. 신생아에서 RI가 0.85 이상, 소아및청소년에서는 RI가 0.70 이상인경우폐쇄성수신증을의심할수있다 [8, 9]. 그러나 RI 증가는폐쇄성수신증의특징적소견은아니다. 다른신질환에서도 RI가높아질수있고, 부분폐쇄성이나심한만성폐쇄성수신증에서는 RI가정상으로나타날수도있다. 또 ureteral jet 유무나빈도감소를폐쇄성수신증감별에이용하기도한다 [10, 11]. II. 선천성수신증을일으키는주요질환의초음파소견 1. 신우요관이행부폐쇄 (Ureteropelvic junction obstruction) 신우요관이행부폐쇄는가장흔한선천성요로폐쇄질환이다 [12]. 이는신우요관이행부위의요관평활근발달이상에의한것으로알려져있다. 이질환은방광요관역류나요관방광이행부폐쇄 (ureterovesical junction obstruction), 마제신, 중복요관등다른요로기형과동반될수있다. 특징적인초음파소견으로는 (1) 여러개의비슷한크기의신배확장과중등도이상의신우확장이있으며, 이들사이에연결이보이고, (2) 다양한두께의신실질이관찰되며, (3) 요관확장은동반되지않고, (4) 방광은정상으로보이는것이다 (Fig. 1). 신우확장은신배확장보다심하며, 아주드물게는방 Fig. 1. Ureteropelvic junction obstruction in a 2-month-old boy.. Ultrasonography of the left kidney shows diffusely dilated pelvis and calyces with severe parenchymal loss.. Transverse scan of the urinary bladder shows grossly normal bladder without visible retrovesical ureter dilatation. C. Intraoperative retrograde ureteropyelography demonstrates stenosis (arrow) at the ureteropelvic junction. C

3 이미정 : 선천성수신증의초음파검사 광요관역류나요관폐쇄에의한요관확장이동반될수있다 [1]. 심하게확장된신외신우 (extrarenal pelvis) 가신우요관이행부폐쇄로오인되는경우가있는데, 전자의경우에는신실질두께가정상이며신배확장이보이지않는다. 신우요관이행부폐쇄진단을놓치는경우는매우드무나, 출생직후탈수상태에서검사하는경우에는진단이어려울수있다. 치료는신장의기능에따라결정하는것이중요하다. 대부분경과관찰에서호전되나, 신기능이떨어지는경우 pyeloplasty 를시행한다 [12]. 2. 방광요관역류 (Vesicoureteral reflux) 방광요관역류는소변이방광에서요관으로거꾸로흐르는것을의미한다. 대부분의방광요관역류는선천적으로발생하며, 후부요관이방광으로들어가는부위의각도이상과길이 감소, 점막하주행등에의해역류방지를위한 flap-valve mechanism이저하되어발생한다. 또한이차적인원인으로는방광출구폐쇄, 배뇨장애, 신경성질환, 그리고 prune belly syndrome 등이있다. 초음파소견으로는주로요관과신우의확장을관찰하며, 확장정도가배뇨에따라변하는것을볼수있다 (Fig. 2). 그러나이런소견의민감도와특이도는매우다양하게보고되고있다 [13, 14]. 그러므로초음파에서요로계가정상소견을보인다고해서방광요관역류를배제할수없다는것을명심해야한다 [15]. 도플러검사를시행할경우소변의역류를컬러변화로알수있으며, 요관구의위치가정상보다좀더바깥쪽에위치한다는연구결과도있다 [16]. 또 ureteric jet 도플러파장형태가 monophasic인경우방광요관역류를의심할수있다 [17]. Fig. 2. Vesicoureteral reflux in a 1-month-old boy.. Ultrasonography of the kidney shows increased cortical echogenicity in the upper pole (arrow) suggestive of pyelonephritis with mild pelvic dilatation at first.. Pelvicalyceal dilatation has been aggravated during urination. C. Voiding cystourethrography demonstrates bilateral vesicoureteral reflux. C

4 대한초음파의학회지 2013;32(2) 식염수나초음파조영제를방광내에주입하여시행하는초음파검사도있다. 이검사를통하여방광에서요관으로역류하는 microbubbles를관찰하면역류를진단할수있으며, harmonic imaging을추가하면진단률을더높일수있다는보고도있다 [18-20]. 그러나아직일반적으로사용되지는않는방법이다. 3. 중복요관 (Duplex collecting system anomalies) 완전중복요관의경우두개의분리된신우신배시스템과두개의요관이각각의요관구로들어가게된다. 보통상극신요관은하극신요관보다내측아래쪽으로이소성삽입을하게되며 (Weigert-Meyer rule) 폐쇄를동반하는경우가많다. 또하극신요관은상극신요관보다외측위쪽으로수직으로들어가역류가동반되는경우가많다. 완전중복요관은여아에서더흔하며, 산전에발견되지않 는경우에는요로감염증상으로나타나는경우가많다. 요관이외괄약근보다아래쪽으로들어가는경우, 여아에서는요실금증상을보일수있고, 남아에서는잔립선염이나부고환염의소견을보일수있다. 상극신은이소성요관이나이소성요관낭종으로끝날수있다. 이소성요관은여아의경우질전정 (vestibule), 질, 요도, 그리고드물게는자궁, 자궁경부또는직장으로들어갈수있으며, 남아의경우방광경부, trigone의좀더내측아래쪽, 후부요도, 생식기구조, 또는직장으로들어갈수있다. 초음파상상극신은주로폐쇄에의한소견을보이게되나, 간혹형성이상이나형성부전에의해에코증가와작은낭종을동반한소견을보이기도한다 (Fig. 3). 하극신은정상소견을보이거나역류에의한요관확장을보이며, 신우요관이행부폐쇄가하극신에동반될수있다 [1]. 이소성요관낭종은방광내에서둥근형태의얇은벽을가진 Fig. 3. Duplex collecting system anomalies in a 1-month-old girl.. Ultrasonography of the kidney shows marked upper pole hydronephrosis with minimal dilatation of lower pole, indicative of a duplex collecting system.. Longitudinal scan of the urinary bladder shows dilated retrovesical ureter with lower ectopic insertion. Fig. 4. Primary megaureter in a 2-year-old boy.. Longitudinal image of the urinary bladder demonstrates a dilated ureter with narrowed juxtavesical ureteral segment corresponding to the aperistaltic segment (arrow).. Ipsilateral kidney is grossly normal without evidence of hydronephrosis or duplex collecting system

5 이미정 : 선천성수신증의초음파검사 에코가없는구조로보이게된다. 그러나간혹요관낭종이뒤집히거나요관으로함입되는경우방광게실처럼보일수있다. 대부분의요관낭종은방광기저에위치하나큰요관낭종의경우방광전체를채울수있고, 반대편요관이나방광경부의폐쇄를일으킬수있다. 이소성요관낭종의경우내시경적절개와 unroofing을시행하며, 상극신의수신증이심하고기능이저하될경우 heminephrectomy도시행할수있다. 4. 선천성거대요관 (Primary megaureter) 거대요관은요관의확장을의미하며, 선천성폐쇄에의해생길수도있고방광요관역류에의해발생할수도있다 [21]. 폐쇄에의한경우아래쪽요관이좁아져있으며연동운동이이루어지지않아기능적폐쇄를일으킨다. 이부위는병리학적으로 ganglion cell이없거나, 일부의경우에는근육형성부전과근위축, 벽의섬유화, 그리고콜라겐침착증가등의소견을보이게된다. 역류성거대요관은요관의벽내길이가짧고비스듬히주행할때발생하게된다. 이런구조적이상은요관방광이행부기능을저해하여역류를초래하게된다. 요관확장을보이는다른원인질환으로는신경성방광에의한요관폐쇄, 후부요도판막, 요관낭종, 요로결석, 요붕증에의한소변량증가등이있다. 선천성거대요관의초음파소견은요관의확장과함께다양한정도의신우신배확장을동반하게된다. 보통방광으로들어가는아래쪽 1/3 부위의요관이방추모양 (fusiform) 으로확장되어보이며, 좁아진끝부위에서는연동운동이보이지않고그위쪽은정상적이거나증가된연동운동이관찰된다 (Fig. 4)[1]. 중등도이상의거대요관에대해서는 ureteral tapering과 reimplanation 수술을시행하게되나, 경한정도의거대요관의경우에는보통추적관찰만하게된다. 5. 단순요관낭종 (Simple ureterocele) 단순요관낭종은단일요관에서방광벽내에위치한하부요도의점마하부분의물혹같은확장이다. 이는선천성협착에의한것일수도있고염증성협착에의해발생할수도있다. 중복요관에동반된요관낭종은여아에게많고, 단순요관낭종은남아에게많다. 성인의경우주로우연히발견되지만, 소아의경우요로감염증상과함께발견되는경우가많으며, 폐쇄나신장형성장애와동반되는경우도종종있다 [22]. 초음파상으로는둥근물혹같은형태가방광의 trigone 내에서관찰되며, 연동운동에따라부분적으로함몰되어보일수있다 (Fig. 5). 또뒤짚히거나방광밖으로탈출하여방광게실처럼보이기도한다. 수신증과요관확장이동반되어보일수있다. 6. 후부요도판막 (Posterior urethral valve) 후부요도판막은남아에서가장흔한요도폐쇄질환이다. 판막은 3개의타입으로분류되기도하며, 가장흔한것은 membranous urethra 부위에 verumontanum 아래위치한다 [23]. 후부요도판막에의해소변유출저항이커지고근위부요도과확장되며방광벽과방광경부에근육비대가생기게된다. 심한폐쇄의경우 prostatic utricle, prostatic duct, ejaculatory duct등도확장되게된다. 임상적으로신생아의경우신장이매우커지며, 산모양수과소증에의한폐형성저하증이나타나게된다. 소아의경우성장지연과성장장애, 요로감염, 그리고배뇨장애가나타나게된다. 초음파소견으로는양측성수신증과요관확장, 신실질감소, 늘어나고두꺼워진방광, 그리고늘어난 prostatic Fig. 5. Simple ureterocele.. Ultrasonography at the bladder level depicts thick-walled ureterocele in the urinary bladder.. Echogenic stone material (arrow) in the ureterocele with distal ureter dilatation can be seen on the follow-up ultrasonography image after 1 year

6 대한초음파의학회지 2013;32(2) urethra( keyhole sign) 가보이게된다 [23]. 이는 transabdominal 또는 transperineal 영상에서모두관찰할수있으나, 소변이잘차지않은경우에는보기어렵다. 그외에신장형성장애, 요성복수, subcapsular or perirenal urinoma 등이보일수있다. 신장형성장애는산전고압역류에의한것으로, 신실질에코증가와낭종동반소견을보이게되며나쁜예후를의미한다 (Fig. 6). 요성복수나요낭종은신배의파열에의한소견이다. 치료는초기에 vesicostomy를시행하며, 이후 valve ablation을시행하게된다 [6]. 7. Prune elly syndrome (Eagle-arret syndrome) Prune elly syndrome은특징적으로복벽근의형성부전, 요로계기형, 그리고잠복고환의소견을보이는질환이다 [24]. 대부분신생아시기에진단되며, 거의모든환자가남아이다. 병리학적으로는기능을하는 nephron의수가감소하고, 신실 질의형성장애가보이며, 요관과방광의평활근이없는소견을보이나정확한원인은알지못한다. 환아들은반복적인요로감염과성장장애가나타나게되며, 여러가지다른기형을동반할수있다. 동반기형으로는장의이상회전이나폐쇄, 무공항문, 선천성거대결장, 선천성심질환, 내반족, 선천성고관절탈구등이있다 [24]. 초음파소견으로는다양한정도의수신증과요관확장, 커다란방광, 요막관게실, 요도확장, 그리고 prostatic utricle 확장등이있다 [23]. 보통수신증에비해심한요관확장을보이는데, 이는폐쇄에의한것보다는요관의연동운동감소와방광의소변배출기능감소에의한다. 신장형성장애에의한신실질에코증가와낭종을보일수있다. 후부요도판막과달리방광벽두께증가는보이지않는다. Fig. 6. Posterior urethral valve.. Transverse scan of the urinary bladder shows significant thickening of the bladder wall with marked bilateral ureters dilatation.. Ultrasonography of the kidney shows hydronephrosis with increased parenchymal echogenicity and multiple cysts suggestive of renal dysplasia. C. Voiding cystourethrography shows distention of the posterior urethra and abrupt caliber change (arrow) in the region of the external sphincter at the junction of the posterior and anterior urethra. C

7 이미정 : 선천성수신증의초음파검사 결 선천성수신증은물리적또는기능적폐쇄에의해발생할수있으며, 흔한원인으로는신우요관이행부폐쇄, 방광요관역류, 중복요관, 선천성거대요관, 단순요관낭종, 후부요도판막, 그리고 prune belly syndrome 등이포함된다. 산전초음파에서수신증이발견된경우생후다시초음파를하여이를확인하게되나, 생후초음파에서수신증이보이지않거나경한확장이보이는경우추가검사나추적초음파검사에대해서는아직이견이많은상태이다. 신우요관이행부폐쇄는가장흔한선천성요로폐쇄질환으로, 치료결정에는신장기능이중요하다. 방광요관역류는다양한원인으로생길수있으며, 초음파에서정상으로보인다고해서배제할수는없다. 중복요관에서는 Weigert-Meyer rule을알고이소성요관이나요관낭종을확인하는것이필요하다. 그외남아에서흔한후부요도판막과드물지만특징적인 prune belly syndrome을감별하는것이필요하다. 요 수신증은가장흔한요로계기형으로폐쇄성병변의가능성이있다. 초음파는산전후수신증환아의 1차적검사방법으로, 신기능에대한추가검사가필요하기는하나매우유용한검사방법이다. 이논문에서는선천성수신증을일으키는주요질환들과각질환에서의초음파소견에대해알아보고자한다. 론 약 References 1. Siegel MJ. Pediatric sonography. 4th ed. Philadelphia: Lippincott Williams & Wilkins, 2011; Yavascan O, ksu N, nil M, et al. Postnatal assessment of growth, nutrition, and urinary tract infections of infants with antenatally detected hydronephrosis. Int Urol Nephrol 2010;42: Clautice-Engle T, nderson NG, llan R, bbott GD. Diagnosis of obstructive hydronephrosis in infants: comparison sonograms performed 6 days and 6 weeks after birth. JR m J Roentgenol 1995;164: ksu N, Yavascan O, Kangin M, et al. Postnatal management of infants with antenatally detected hydronephrosis. Pediatr Nephrol 2005;20: Skoog SJ, Peters C, rant S, Jr., et al. Pediatric vesicoureteral reflux guidelines panel summary report: clinical practice guidelines for screening siblings of children with vesicoureteral reflux and neonates/infants with prenatal h- dronephrosis. J Urol 2010;184: Yamacake KG, Nguyen HT. Current management of antenatal hydronephrosis. Pediatr Nephrol Estrada CR, Peters C, Retik, Nguyen HT. Vesicoureteral reflux and urinary tract infection in children with a history of prenatal hydronephrosis--should voiding cystourethrography be performed in cases of postnatally persistent grade II hydronephrosis? J Urol 2009;181: ; discussion Platt JF. Duplex Doppler evaluation of native kidney dysfunction: obstructive and nonobstructive disease. JR m J Roentgenol 1992;158: Okada T, Yoshida H, Iwai J, et al. Pulsed Doppler sonography of the hilar renal artery: differentiation of obstructive from nonobstructive hydronephrosis in children. J Pediatr Surg 2001;36: de essa J, Jr., Denes FT, Chammas MC, et al. Diagnostic accuracy of color Doppler sonographic study of the ureteric jets in evaluation of hydronephrosis. J Pediatr Urol 2008;4: Cvitkovic Kuzmic, rkljacic, Rados M, Galesic K. Doppler visualization of ureteric jets in unilateral hydronephrosis in children and adolescents. Eur J Radiol 2001;39: Karnak I, Woo LL, Shah SN, Sirajuddin, Ross JH. Results of a practical protocol for management of prenatally detected hydronephrosis due to ureteropelvic junction obstruction. Pediatr Surg Int 2009;25: Grazioli S, Parvex P, Merlini L, Combescure C, Girardin E. ntenatal and postnatal ultrasound in the evaluation of the risk of vesicoureteral reflux. Pediatr Nephrol 2010;25: Tsai JD, Huang FY, Tsai TC. symptomatic vesicoureteral reflux detected by neonatal ultrasonographic screening. Pediatr Nephrol 1998;12: rophy MM, ustin PF, Yan Y, Coplen DE. Vesicoureteral reflux and clinical outcomes in infants with prenatally detected hydronephrosis. J Urol 2002;168: ; discussion Leung VY, Chu WC, Yeung CK, Metreweli C. Doppler waveforms of the ureteric jet: an overview and implications for the presence of a functional sphincter at the vesicoureteric junction. Pediatr Radiol 2007;37: Kljucevsek D, Kljucevsek T, Kersnik Levart T, Kenda R. Ureteric jet Doppler waveform: is it a reliable predictor of vesicoureteric reflux in children? Pediatr Nephrol 2009;24: Darge K. Voiding urosonography with ultrasound contrast agents for the diagnosis of vesicoureteric reflux in children. I. Procedure. Pediatr Radiol 2008;38: Darge K. Voiding urosonography with US contrast agents for the diagnosis of vesicoureteric reflux in children. II. Comparison with radiological examinations. Pediatr Radiol 2008;38: Papadopoulou F, nthopoulou, Siomou E, Efremidis S, Tsamboulas C, Darge K. Harmonic voiding urosonography

8 대한초음파의학회지 2013;32(2) with a second-generation contrast agent for the diagnosis of vesicoureteral reflux. Pediatr Radiol 2009;39: Hodges SJ, Werle D, McLorie G, tala. Megaureter. Scientific World Journal 2010;10: Zerin JM, aker DR, Casale J. Single-system ureteroceles in infants and children: imaging features. Pediatr Radiol 2000;30: Levin TL, Han, Little P. Congenital anomalies of the male urethra. Pediatr Radiol 2007;37: Goulding FJ, Garrett R. Twenty-five-year experience with prune belly syndrome. Urology 1978;12:

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