Clinical Characteristics and Natural History of Antenatally Diagnosed Fetal Uropathy

Abstract

The clinical characteristics and natural history of 55 cases with antenatally diagnosed fetal uropathy were investigated. Percutaneous aspiration of the fetal pelvic or vesical urine was performed to decompress progressive unilateral hydronephrosis in 2 cases and to evaluate renal function in another 2 cases of bilateral hydronephrosis. As the postnatal diagnosis, upper urinary tract dilatation (hydronephrosis or hydronephroureter, 33 cases) and renal dysplasia (15 cases) made up 87% of all cases. A combination of hydronephrosis in one kidney and renal dysplasia in the other was also found in another 2 cases. Among 35 cases with upper urinary tract dilatation, 27 cases demonstrated pelviureteric junction stenosis and surgical intervention was necessary in 15 cases. In 17 cases with renal dysplasia, spontaneous regression was observed in only 3 cases and surgical intervention by means of percutaneous nephrostomy and nephrectomy was performed in 4 and 6 cases, respectively.

Fetuses and infants with congenital urinary system anomalies: correlation between prenatal ultrasound and postmortem findings

“…Other follow‐up studies of ultrasonography of urinary system anomalies include, for the most part, a combination of clinical and postmortem cases. Postnatal confirmation of the prenatal diagnoses is described in 50% to 78% of cases from the late 1980s 20,21 , 23 . In a recent study of 55 cases, there was an agreement of 81% between antenatal and postnatal diagnoses 4 . The major discrepancies consisted of difficulties in discriminating dysplastic kidneys from hydronephrosis. In an early second‐trimester sonographic screening, 21 anomalies consisting of unilateral renal agenesis, pelvic kidney and double collecting system were all confirmed postnatally or at postmortem examination 22 .…”

“…The overall prevalence of congenital urinary system anomalies has been reported as two per 1000 live births 2 . Urinary system abnormalities represent a large group of fetal anomalies detected by sonography 3 . Sonographic screening has disclosed an overall frequency of fetal uropathy of 0.28% and about two‐thirds of these demonstrated urinary tract dilatation 4,5 . In two different antenatal studies the most frequent cause of significant urinary tract dilatation was obstruction of the ureteropelvic junction 6,7 .…”

“…In two different antenatal studies the most frequent cause of significant urinary tract dilatation was obstruction of the ureteropelvic junction 6,7 . Upper urinary tract dilatation and renal dysplasia constitute the largest group of urinary system anomalies 2,4 , 8,9 . The incidence of bilateral renal agenesis is low, with an incidence of 0.1–0.3/1000 births 10–12 .…”

“…Autopsy studies comparing the prenatal ultrasound diagnosis with postmortem examination have shown that the prenatal detection of renal anomalies varies from 60% to over 90%. In these studies the urinary system anomaly was part of a general analysis of all types of anomalies and did not focus specifically on particular organ systems 13–19 . A few studies comparing prenatal and postnatal urinary system anomalies have focused on both clinical follow‐ups and postmortem examinations, where the detection rate has varied from 65% to 100% 4,20–22 . Except for one study 23 , they all comprised a limited number of cases, and minor renal anomalies were studied in only one study 22 . To our knowledge, a comprehensive focus on prenatal diagnoses of urinary system anomalies comparing them with autopsy results has not been done.…”

Recovery after relief of fetal urinary obstruction: morphological, functional and molecular aspects

“…Prenatal observations suggest that many of our assumptions regarding developmental renal abnormalities and obstruction previously described from a postnatal perspective may not be completely valid when seen from a perinatal viewpoint. On one hand, the condition appears more severe than expected, as, in a series of postnatally diagnosed patients, fetuses that were terminated, stillborn, or dead perinatally were excluded; on the other hand, a considerable proportion of the antenatally diagnosed cases demonstrates a benign postnatal clinical course that might have been ignored postnatally (17,19,33). An unexplained continuous spectrum of situations can be observed at any fetal stage, and its natural history remains unknown.…”