Posterior urethral valves: prenatal diagnostic signs and outcome.

INTRODUCTION: Urethral valves can be of enormous clinical importance. Both the prognosis for an affected fetus and the indication for a prenatal therapeutic intervention depend to a high degree on the accuracy of the prenatal diagnosis. PATIENTS AND METHODS: The sonographic findings and the results of the postnatal diagnostic workup of 24 boys treated for urethral valves in our institute are analyzed in the present paper. RESULTS: Out of a group of 900 children, diagnosed prenatally as having urinary tract anomalies, the postnatal diagnostic workup revealed 24 boys suffering from posterior urethral valves. The combination of megacystis, oligohydramnios, and bilateral renal abnormalities was present in 3 boys, and it was only in these cases that urethral valves were correctly identified as the underlying pathology. The largest group (n = 16) of these fetuses presented with bilateral hydronephrosis with and without megaureter. Megacystis in any combination with other findings was detected only in 4 patients. Oligohydramnios as a sign of impaired renal function was observed in four pregnancies. Four children suffered postnatally from beginning renal insufficiency. Eight infants (33%) required some form of respiratory support. This group included the 4 newborns with oligohydramnios during the pregnancy. CONCLUSIONS: Posterior urethral valves represent a rare but severe congenital malformation. They can disrupt the development of the entire urinary tract and may be life-limiting to the affected boy. Only rarely, however, are they manifested as the complete pathological picture on prenatal ultrasound. In most cases, bilateral impairment of the fetal renal development suggests severe subvesical obstruction. Thus in cases with bilateral renal impairment on prenatal ultrasonography, posterior urethral valves are to be excluded postnatally. Oligohydramnios is a predictor of a poor outcome of the renal function.

Antenatal diagnostic aspects of unilateral multicystic kidney dysplasia--sensitivity, specificity, predictive values, differential diagnoses, associated malformations and consequences.

OBJECTIVES: Unilateral multicystic kidney dysplasia (MCKD) is the second most common urinary tract abnormality diagnosed antenatally. Whilst an isolated unilateral MCKD has a good prognosis, a poor outcome must be expected when MCKD is associated with other complex abnormalities. MATERIAL: Out of 11,176 cases, 693 fetuses were suspected of having urinary tract abnormalities. Urological findings were confirmed in 548 of them. Unilateral cystic kidney was diagnosed prenatally in 85 cases. RESULTS: The study results in a total of 107 cases with proven MCKD. Eighty-five pregnancies with a prenatal diagnosis of MCKD were analysed. The antenatal diagnosis of MCKD was confirmed in 56 cases. Fifty-one children were found to have unilateral MCKD where this had not been explicitly suspected from antenatal scanning. CONCLUSION: Unilateral MCKD is a malformation with an excellent prognosis for child survival and global renal function if encountered in isolation. However, our analysis of live infants and autopsy cases demonstrates a high proportion of severe associated malformations of the urinary tract and other organ systems.

Follow-up of unilateral multicystic kidney dysplasia after prenatal diagnosis.

OBJECTIVES: Since the introduction of antenatal diagnostic screening, multicystic kidney dysplasia (MCKD) has been diagnosed in 1 of 4300 live births. We analyze our own experience and demonstrate a management regime based on these results and existing studies. METHODS: Retrospective data analysis was carried out in 110 patients, prenatally diagnosed with MCDK. A total of 93 patients with confirmed diagnosis of unilateral MCDK were born alive and followed up in our institutions. RESULTS: A total of 110 unilateral cystic kidneys were prenatally diagnosed; 93 were confirmed postnatally. These children were retrospectively allocated to two treatment groups: 51 were operated upon; 42 were treated conservatively. A micturition cystourethrogram was performed 88 times, yielding 20 pathological findings. Vesicoureteral reflux was identified 11 times (12.5%). Eleven children (12%) had associated non-urological abnormalities. Cardiac and musculoskeletal malformations predominated. After 1995, nephrectomy was performed only when clinically indicated. Thirty-seven children were treated conservatively for between 4 months and 6 years (mean 33 months); 28 dysplastic kidneys exhibited size reduction. Complete involution was noted in 16 of 28 children between 7 and 29 months (mean 16.2 months). CONCLUSION: This study provides evidence that neonatal nephrectomy of unilateral MCDK is rarely required. It also shows that the discussed malformation is not an isolated developmental abnormality. In a high proportion, associated urogenital abnormalities were present and therapeutically relevant, and determined the overall prognosis.

Meconium peritonitis and pseudo-cyst formation: prenatal diagnosis and post-natal course.

OBJECTIVES: Intra-uterine bowel perforation can occur secondary to a variety of abnormalities and cause sterile peritonitis in the fetus (generalised = type I). If sealing of the perforation does not take place, a thick-walled pseudo-cyst can form (type II). METHODS: Over a 12-year period, 21 616 pregnancies were screened for gastro-intestinal malformations using prenatal ultrasound. We identified 1077 cases suspicious of surgically correctable malformations. Post-natal diagnoses and outcome were worked up retrospectively. RESULT: We found 96 fetuses with suspected gastro-intestinal malformations. Prenatal bowel perforation with meconium peritonitis was confirmed in 11 cases. In 5 of these 11, the correct diagnosis had been predicted prenatally. One child presented as a fetal and neonatal emergency (case report). Ten of the eleven infants were operated on during their first day of life. Intra-operative findings were atresia (n = 4), meconium ileus (n = 6) and no obvious cause (n = 1). Two children suffered fatal complications. CONCLUSION: Meconium peritonitis and meconium pseudo-cysts as its special manifestation are assessable by prenatal diagnosis but present in different ways. They can present as fetal ascites or echogenic bowel and cause fetal or neonatal distress, requiring close observation and highly specialised care.

[Retrospective analysis of differential therapeutic measures in children with double kidney and ureter and hydronephrosis]. / Retrospektive Analyse des differentialtherapeutischen Vorgehens bei Kindern mit Doppelnieren mit Ureter duplex und Harntransportstörung.

In this study, we analyzed the findings of 79 duplex kidneys in 64 children treated at our institution between 1987 and 1999. Several reasons for impaired urine transport in a duplex kidney were clarified. A total of 55% of all patients manifested a variety of abnormal findings during prenatal screening. In the course of postnatal diagnostic work-up, renal units were classified according to their leading pathomorphological features: 44% showed a ureterocele, 29% nonfunctioning units at first diagnosis, and 27% vesicoureteric reflux. Nonfunctioning moieties had been heminephrectomized in most cases. In such nonfunctioning cases in the presence of associated ureteroceles, relief drainage was unsuccessful in our study. Reflux rarely matures. Ureterocystoneostomy is successful in 80% of our cases. In functioning units with a ureterocele, endoscopic therapy may be the one and only intervention needed. However, they commonly require secondary reconstructive measures.

Survival in second trimester oligohydramnios secondary to bilateral pelviureteral junction obstruction.

The extent and onset of obstruction in hydronephrosis determine the varying degrees of renal impairment. Bilateral hydronephrosis, especially in combination with oligohydramnios, is considered a negative predictor for pregnancy outcome. We describe a case of bilateral pelviureteral junction obstruction causing severe oligohydramnios between 25 and 29 weeks of gestation. The prenatal and postnatal findings and treatment are demonstrated. In the presence of bilateral renal impairment and oligohydramnios, our patient had an unfavorable prognosis. The respiratory and renal function, however, were better than expected. We show how urinary tract reconstruction and neonatal intensive therapy can result in an acceptable outcome.

Radical prostatectomy for high grade prostatic intraepithelial neoplasia.

High Grade Prostatic Intraepithelial Neoplasia (HGPIN) has been recognized as the most likely precursor of invasive carcinoma of the prostate. Close surveillance and follow-up are indicated if subsequent procedures fail to identify carcinoma. There is still considerable controversy about the natural history of high grade PIN and most authors agree that its identification should not influence or dictate therapeutic decisions. We performed a prophylactic radical prostatectomy in such a case which has not been reported in the world literature.