Korean Journal of Nephrology 1988;7(2):395-412.

요로계의 선천성 기형에 관한 임상 통계학적 관찰

박경미 , 김병길 , 이재승 , 최승강

Abstract

A clinicostatisical study was done of children with congenital urinary tract anomalies who had been admit- ted to Youngdong Severance Hospital and Severance Hospital at Yonsei University College of Medicine during on 8 year period from January 1980 to March 1988. The rate of congenital anomalies among in-patient was 3.4%, without any remarkable annual difference. The highest rate of anomalies was found in cardiovas- cular system anomalies at 42.3%, followed by musculos- keletal system anomalies at 21.2%, and the anomalies of genitourinary ststems occurring third at a rate of 13.6%. The total number of congenital urinary tract anom- alies was 171 cases composed of 48 cases (28.1%) of ureteral anomalies, 43 cases (25.1%) of anomalies of renal position, size, and number, 43 cases (25.1%) of double collecting system, 20 cases (11.2%) of cystic kidney disease, 9 cases (5.3%) of urethral anomalies, 5 cases (2.9%) of bladder anomalies, and 3 cases (1.8%) of patent urachus. 91 cases (53%) had more than one another congenital anomalies and the most common congenital anomalies occurring with urinary tract anomalies were 40 cases of another kind of genitourinary tract anomalies followed by cardiovascular system and GI system anomalies. Of congenital anomalies of the urinary tract, anomalies of renal position, size, and number had the most frequently accompanying congenital anomalies in 40 cases. The ureteral anomalies included 16 cases of ureter- opelvic junction stenosis, 12 cases of ureterocele, 11 cases of ectopic ureter, and some cases of ureterovesical junction stenosis and multiple stenosis. Of ureteral anomalies, 48% were diagnosed between the ages of 2 mo. To 1 year, the male to female ratio was 1:1.7, 31 cases were on the left side, and 77.1% of the patient reveived operation. Most were detected by IVP with a diagnostic rate of 73%. Anomalies of renal position, size, and number include- ed 14 cases of hypoplasia, 11 cases of agenesis, 8 cases of double kidney, 7 cases of horseshoe kidney and some cases of ectopia and Ask-Upmark kidney. They were discovered during all pediatric ages with a male to female ratio of 1:1. IVP, ultrasonography, renal scan, RGP, angiography and CT all showed a high diagnostic rate. Double collecting systems included 24 cases of com- plete type and 19 cases of incomplete type, and two thirds were discovered before 5 years af age with a male to female ratio of 1;4. Six cases showed bilateral involvement and IVP showed a diagnostic rate of 80%. According to the Potter classification of cystic kidney disease, there were 11 cases of cystic dysplasia, 5 cases of adult polycystic kidney disease (APKD), and 4 cases of infantile polycystic kidney disease (IPKD). Of cystic dysplasia cases, 64% were discovered before 1 year of age, all APKD cases after 2 years of age, and 50% of IPKD cases before 1 month of age with a male to female ratio of 2.3:1. All cases of cystic dysplasia occurred unilaterally except one case while all cases of APKD and IPKD were bilateral. Two cases of APKD had a positive 'family history. Abdominal ultrasonography yielded a diagnostic rate of 81%.