Prenatally detected ureteropelvic junction obstruction: clinical features and associated urologic abnormalities

Abstract

Urologic congenital anomalies associated with ureteropelvic obstruction (UPJO) have been previously characterized; however, less data are available regarding these associations in a prenatally diagnosed population. A retrospective study was conducted to evaluate significant clinical features and urological anomalies associated with prenatally diagnosed UPJO. The records of 143 children with prenatally diagnosed hydronephrosis secondary to UPJO were retrospectively reviewed. The gender, side of obstruction, degree of hydronephrosis, associated clinical features, and urological anomalies were noted. Hundred and forty-three children (M/F = 2.7) with a total of 198 affected renal units (RU) presenting with unilateral (61%) or bilateral (39%) UPJO were enrolled. In cases of unilateral obstruction, the left side was affected in 60 children (68%). The grade of hydronephrosis was Grade 1 in 56 RU (28%), Grade 2 in 51 RU (26%), Grade 3 in 50 RU (25%) and Grade 4 in 41 RU (21%). Associated clinical features included prematurity (n = 7, 4.9%), twinning (n = 5, 3.5%) and presentation with renal failure (RF) (n = 2). Excluding contralateral UPJO, other urologic anomalies were encountered in 29 patients (20.3%). Associated vesicoureteral reflux (VUR) was encountered in 11 patients (7.7%, M/F = 2.7). Pyeloplasty was required more often in children with associated VUR (54.5 vs. 18.2%) (P = 0.01). Contralateral multicystic dysplastic kidney (MCDK) was encountered in six patients (M/F = 2), one of whom presented with RF. One child carried the diagnosis of Schinzel-Giedion syndrome (SGS), demonstrating severe developmental and neurological disorders and bilateral hydronephrosis. The more frequent occurrence of UPJO in males with predominantly left-sided location, association with VUR and MCDK, and increased frequency of bilaterality in our prenatally diagnosed patients were similar to historical reports. In addition, prematurity and twinning were independently associated with UPJO. The higher rate of pyeloplasty in patients with associated reflux warrants further investigation.

The Society for Fetal Urology consensus statement on the evaluation and management of antenatal hydronephrosis

“…However, randomized trials suggested that only 19e25% of children with prenatally diagnosed UPJ obstruction require surgical intervention [55,56]. There exists an increased incidence of other urological abnormalities, such as VUR and multicystic dysplastic kidney (MCDK), with UPJ obstruction [57].…”

Ultrasonographic Evaluation of Foetal Hydronephrosis

“…In order to identify the fetuses who will really benefit from intrauterine urinary diversions, the selection criteria was refined. This is based on amniotic liquid quantification (amniotic liquid index measured by ultrasonography), karyotype study and fetal urine electrolytes and protein dosage [15] . The serial evaluation of fetal urine has been postulated for allowing a better accurate selection of fetus who will benefit from urinary diversion [13] .…”

Pediatric congenital hydronephrosis (ureteropelvic junction obstruction): Medical management guide

“…A UPJO occurs more frequently on the left, and bilateral UPJO occurs in 20-39% of affected patients. 4,5 The rate of an additional urological anomaly in UPJO patients has been reported to be up to 50%. 5 The prevalence of VUR associated with a UPJO on the affected side as a malformation of the renal tract ranges from 7.3% to 11.3%.…”