Congenital ureteropelvic junction obstruction
- Laurence S Baskin, MD, FAAP
Laurence S Baskin, MD, FAAP
- Section Editor — Pediatric Urology
- Frank Hinman, Jr., MD, Distinguished Professorship in Pediatric Urology
- Chief Pediatric Urology
- Professor of Urology and Pediatrics
- UCSF Benioff Children's Hospital
Ureteropelvic junction (UPJ) obstruction is a partial or intermittent total blockage of the flow of urine that occurs where the ureter enters the kidney. The etiology of UPJ obstruction includes both congenital and acquired conditions. UPJ obstruction is the most common pathologic cause of antenatally detected hydronephrosis.
The epidemiology, pathophysiology, clinical features, and management of congenital UPJ obstruction will be reviewed here.
The reported incidence of UPJ obstruction is 1 in 500 live births screened by routine antenatal ultrasound; however, not all cases require surgical intervention [1,2]. UPJ obstruction is the most common anatomical cause of antenatal hydronephrosis.
Boys are affected with UPJ obstruction more commonly than are girls [3,4]. Lesions are found more frequently on the left than on the right side. The reported rate of bilateral involvement is approximately 10 percent [1,4].
Congenital UPJ obstruction is caused by anatomic lesions or functional disturbances that restrict urinary flow across the UPJ, resulting in hydronephrosis (figure 1 and picture 1) . Most cases are thought to be due to partial obstruction, because complete obstruction results in rapid destruction of the kidney. In some cases, partial obstruction may also lead to progressive deterioration of renal function. However, in many infants, an equilibrium state may develop in which renal function remains stable.
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- Intrinsic narrowing
- Extrinsic compression
- CLINICAL PRESENTATION
- Fetal and neonatal
- Older children
- Other anomalies
- Voiding cystourethrogram
- Diuretic renography
- Computed tomographic scan
- Magnetic resonance imaging
- DIFFERENTIAL DIAGNOSIS
- Older child
- Symptomatic patients
- - Pyelonephritis
- - Kidney stones
- Asymptomatic patients
- - Observation
- - Surgery
- Surgical options
- - Outcome
- Our approach
- - Surgery
- Postoperative follow-up
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS