Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Incidence and outcome of congenital anomalies of the kidney and urinary tract detected by prenatal ultrasonography: a single center study

산전 초음파로 발견된 선천성 신 요로 기형의 발생률과 임상 결과: 단일 기관 연구

  • Lim, Gina (Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine) ;
  • Lee, Joo Hoon (Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine) ;
  • Park, Young Seo (Department of Pediatrics, Asan Medical Center University of Ulsan College of Medicine) ;
  • Kim, Kun Seok (Department of Urology, Asan Medical Center University of Ulsan College of Medicine) ;
  • Won, Hye-Sung (Department of Obstetric-Gynecology, Asan Medical Center University of Ulsan College of Medicine)
  • 임진아 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 이주훈 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 박영서 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 김건석 (울산대학교 의과대학 서울아산병원 비뇨기과학교실) ;
  • 원혜성 (울산대학교 의과대학 서울아산병원 산부인과학교실)
  • Received : 2008.09.12
  • Accepted : 2008.10.29
  • Published : 2009.04.15
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Abstract

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

목 적 : 산전 초음파로 발견된 선천성 신 요로 기형의 빈도와 임상 결과에 대해 알아보고자 하였다. 방 법 : 1989년 10월부터 2007년 10월까지 18년간 산전 초음파에서 발견되어 출생 후 서울아산병원 소아청소년과에서 선천성 신 요로 기형이 진단된 906예를 대상으로 의무 기록과 검사 결과를 조사하였다. 결 과 : 발생 빈도는 수신증, 다낭성 이형성 신, 중복 신장, 방광 요관 역류, 단일 신, 수뇨관 신증, 이소성 신장, 다낭성 신, 요관류, 후부 요도 판막의 순이었다. 수신증은 520예로 57.4%를 차지하였으며 이 중 20%가 수술이 필요하였다. 다낭성 이형성 신은 25.4%에서 다른 동반된 신 요로 기형이 있었다. 중복 신장은 57.9%에서 수술이 필요하였다. 방광 요관 역류는 남아에서 10배 많았다. 상염색체 열성형 다낭성 신의 7예 중 2예가 만성 신부전으로 진행하였다. 후부 요도 판막은 9예(1%)로 빈도가 낮았으며, 그 중 1예가 만성 신부전으로 진행하였다. 결 론 : 산전 초음파로 발견되는 선천성 신 요로 기형은 그 종류가 다양하며 임상 결과는 많은 예에서 양호하여 특별한 처치가 필요 없는 경우도 많으나, 일부에서는 신장 기능을 감소시키는 원인이 되거나, 반복적인 요로 감염의 원인이 되어 수술이 필요한 경우가 있으며 특히 양쪽 신장에 발생하는 기형은 적절한 처치를 하지 않으면 만성 신부전으로 진행하는 원인이 될 수 있다. 그러므로 각각의 기형에 대한 정확한 분포와 임상 결과를 알기 위해서는 장기적으로 다기관 뿐 아니라 전국적인 조사 연구가 필요하다.

Keywords

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