Urethral Valves Research Articles

Síndrome de Goldenhar asociado a válvulas de la uretra posterior

To report a case with Goldenhar syndrome with posterior urethral valves. Goldenhar syndrome (oculoauriculovertebral dysplasia) is a condition featuring the following triad of anomalies: ocular abnormalities (epibulbar dermoids, coloboma), otic anomalies (low set ears, auricular appendage) and/or vertebral anomalies. Multiple malformations, including congenital heart, brain and renal disease. Posterior urethral valves should be treated with primary valve ablation. It is necessary to perform a careful evaluations of general malformations, especially renal malformations.

Does bladder augmentation stabilize serum creatinine in urethral valve disease? A series of 19 cases

Objective This study evaluates the results of bladder augmentation (BA) in 19 boys with posterior urethral valves, especially as regards its efficacy in stabilizing serum creatinine. Patients and methods In the period 1995–2005, 188 patients with urethral valves were surgically managed. Nineteen of these had undergone BA as a part of their surgical management after initial endoscopic valve ablation in 15 and diversion in four boys. The mean serum creatinine at the time of BA was 2.11 mg/dl. Results BA stabilized the serum creatinine in 14 but failed to do so in five boys. A serum creatinine level of more than 2 mg/dl at the time of BA was associated with a significantly worse rate of success. BA as part of an undiversion procedure in three boys was unsuccessful. Conclusion In an economic milieu where renal transplantation is not available for the majority of deserving children, careful selection is required before BA is considered as a surgical solution for the valve bladder. BA, when otherwise indicated, has been beneficial in children with pre-augmentation creatinine up to 2 mg/dl.

3414: Various prenatal sonographic findings of normal and abnormal fetal genitalia

Advances in prenatal US now enable one to diagnose first trimester gender. Also, gender assignment has significant clinical implications in fetus at risk for x-linked diseases as hemophilia, Duchenne’s muscular dystrophy. Ambiguous genitalia are seen as a component of chromosome anomaly as trisomy 13, triploidy, 13q syndrome. Structurally abnormal genitalia may occur as single malformation, or as components of congenital or inherited syndromes. We demonstrate normal sonographic fetal genital development as gestational age, abnormal male genital malformations as hypospadia, penile chordee, hydrocele, undescended testes, urethral valve, urethral atresia and abnormal female genital malformations as clitomegaly, hydrometrocolpos, rectovestibular fistula with imperforate anus, ovarian cyst with or without torsion. This sonographic acknowledge for fetal genitalia can be helpful in diagnosis and prenatal counseling of gender specific fetal anomalies.

Congenital pulmonary sling, aorto–pulmonary window and pulmonary vein obstruction as a diagnostic and therapeutic challenge in an infant with VACTERL association

In a newborn with anal atresia and urethral valves an incomplete VACTERL association, was diagnosed and a colon anus praeter was placed. Sweating and heart murmur led to cardiac diagnostics. By 2D colour Doppler echocardiography a huge atrial septal defect and pulmonary venous stenoses were diagnosed. Additionally, a pulmonary sling combined with an aortopulmonary window (AoPAw) was suspected and later confirmed by angiography. Heart failure developed and closure of the AoPAw, transplantation of the left pulmonary artery and closure of the atrial septal defect was performed at the age of 4 weeks. But the patient did not improve. Pulmonary hypertension with suprasystemic pressure in the right ventricle originating from a stenosis of the new orifice of the left pulmonary artery and obstruction of the right pulmonary veins was diagnosed by echocardiography and confirmed by angiography. At the age of 8 months the orifice of the left pulmonary artery was enlarged by a patch, the obstruction of left sided pulmonary veins was opened, and the rightsided pulmonary veins were newly implanted into the left atrium. Finally the inter-atrial communication was closed by a patch plastic. Again, postoperatively the patient improved only slightly. At the age of 10 months trans-septal catheterisation was performed. Angiography revealed a successful balloon dilatation of the long obstruction of the right pulmonary veins. Later on, an absorbable magnesium stent was implanted into the right upper pulmonary vein and medication with an endothelin antagonist was started. Temporarily the patient improved significantly. Within 6 weeks, right ventricular systolic pressure was again suprasystemic due to extreme inflow obstruction of the right pulmonary vein diagnosed by echocardiography. Severe heart failure developed and at the age of 1 year the patient died.

Etiology and epidemiology of end-stage renal disease in Dutch children 1987–2001

In this retrospective study 351 children (<16.0 years) with end-stage renal disease (ESRD) accepted for renal replacement therapy (RRT) in the four Dutch pediatric centers were analyzed for the period 1987-2001. The data were compared with a previous study performed in 1979-1986. Eighty patients were of non-Dutch origin. An annual ESRD incidence of 5.8 patients per million of the child population (p.m.c.p.) was calculated, without significant changes with time. The final prevalence in Dutch children under 15 years of ESRD was 38.7 p.m.c.p. The most frequent primary renal disease leading to ESRD was urethral valves, with a significant increase vs. the previous observation period (14% vs. 6%). The distribution of primary renal diseases was similar in patients of non-Dutch origin and in Dutch patients. Peritoneal dialysis was the most frequent dialysis procedure initially applied (62% vs. 26% in the earlier observation period). Thirteen percent of all first transplantations (n=278) were pre-emptive and 19% from living donors. Five-year graft survival after a living-donor and a cadaver graft was 80% and 73%, respectively. Overall patient survival after 10 years on RRT was 94%.

Is Bladder Cycling Useful in the Urodynamic Evaluation previous to Renal Transplantation?

Objectives: To evaluate the usefulness of bladder cycling (BC) in differentiating between urodynamic abnormalities due to reversible bladder dysfunction and those due to preexisting lower urinary tract (LUT) abnormalities, and to determine if BC increases the diagnostic yield of urodynamic investigations (UIs) in these patients. Methods: Eleven patients with oligoanuria (<300 ml/day) were evaluated with complete UIs. All cases showed low compliance (mean 6 ml/cm H₂O) and low cystometric capacity (mean 146 ml) and were placed in a BC program. The indications for UIs were LUT symptoms in 2 patients, LUT abnormalities (myelodysplasia or urethral valves) in 3 patients, or the appearance of an extremely small bladder on cystogram (6 patients). BC was performed through a hypogastric catheter in an outpatient setting. UIs were repeated afterwards. Results: After BC, 5 patients (45%) continued to have low compliance (<8 ml/cm H₂O) and 4 patients (36%) had normal UIs. In 2 cases (18%), a previously unrecognized obstruction was diagnosed. No patient with LUT symptoms or abnormalities had a normal UI after BC. Conversely, 4 of 6 patients in whom BC was indicated due to a small bladder on cystogram had normal UIs after BC. Conclusions: BC is useful in explaining whether high bladder pressure is due to dysfunction or to preexisting disorders, and thus indicating the proper surgery for correction. Suprapubic BC significantly increases bladder capacity thus increasing the diagnostic yield of the UI.

Living-Unrelated (Paid) Renal Transplantation—Ten Years Later

Due to the increase of organ shortage and still inadequate development of cadaver transplantation, many end-stage patients from the Balkan region travel mostly to India to buy a kidney. Despite all the ethical dilemmas and discussions, organ sales is present nowdays in Third-World countries. Sixteen patients (13 from Macedonia and 3 from Kosovo, SCG) were observed clinically during a period of 10 years. Recipients of mean age 36.5 years (range 10 to 58) displayed the following underlying diseases: chronic glomerulonephritis ( n = 5), urethral valves with reflux ( n = 2), ADPKD ( n = 1), hypertensive nephropathy ( n = 4), lithiasis ( n = 1), and unknown cause of ESRD ( n = 3). The donor population was young (22 to 29 years). Most patient records did not include data on HLA, cross-match, MLC, kind of surgery, or usual pretransplant workup. The immunosuppressive protocol included CyA, PRED, and AZA or MMF. All transplanted patients were followed on an outpatient basis in our department; patients with complications were hospitalized. The 1, 3, 5, and 10 year Kaplan Meier graft survival rates were 78.6%, 50.2%, 33.3%, and 18.8%, respectively. Seven patients were lost (43.7%), two during the first month after transplantation, two at the end of the first year, and three at 5, 6, and 8 years thereafter. The main reasons for death were severe pulmonary infections with sepsis, hepatitis B with liver cirrhosis, Kala Azar, CMV, and cancer of the colon. Five grafts were lost due to repeated rejection episodes and chronic graft nephropathy. The last three cases remained with good renal function and actual serum creatinine values of 135 ± 9. In view of this experience, the authors cannot recommend this type of transplantation, not only from the ethical point of view, but also from frequent medical and surgical complications which are sometimes life threatening.

LIVING UNRELATED (PAID) RENAL TRANSPLANTATION - TEN YEARS AFTER

O5 Aims: to present data and overall clinical outcome of unrelated (paid) renal transplantations performed mostly illegally in India and Nepal in the follow up study of 10 years. Methods: We observed clinically 16 patients (14 males and 2 females) transplanted mostly illegally on the self initiative and arrangement in the third world countries (predominantly in India and Nepal) during the period of 10 years. The transplantations were performed in the period from 1992 to 1995. The mean age of the patients was 36.5 years (range 10-58) with followed underlying diseases: Chronic glomerulopathy (5 cases), Urethral valve with reflux (2 cases), ADPKD (one case) Hypertension with atherosclerosis (4 cases) Urinary Lithiasis (one case) and undefined ESRD (3 cases). The usual immunosuppression included mostly triple drug therapy (Neoral-CyA, Prednizolon and Azathioprin) and in the few unselective cases an induction with ATG was performed, to. The donor population is principally young (range 22-29 years). In the short report from the colleagues most important data are missing including the usual workup for the donor and recipient. It has been supposed that a minimum of 50% of HLA compatibility was accepted, but there were no precise data. Transplanted patients were dismissed from the hospital very early from 7th to 10th postoperative day, some of them with severe complications such as acute rejections, perirenal haematomas and urinary leakage. All transplanted patients were accepted and followed on the outpatient basis according to the well known principles. The patients with complications were hospitalised and treated urgently in the Department. The rejection episodes were biopsy confirmed and treated by MP pulls therapy and OKT3 in the case of steroid resistant rejection. The Kaplan-Meier graft and patient’s survival rate as well as the frequency and severity of clinical complications were analysed. Results: Kaplan-Meier 1, 3, 5 and 10 years graft survival rate was 78.58%, 50.2%, 33.3% and 18.5%, respectively. Seven patients are lost (43.7%), two of them during the first month after transplantation, two at the end of the first year and three 5, 6 and 8 years after. The main reasons for deaths were severe pulmonary infection with sepsis, hepatitis B with liver cirrhosis, Laishmaniasis, CMV disease, cancer of the colon and per renal abscesses with sepsis. A single case of partial thrombosis of the iliac artery with acute graft failure was detected in the first month after transplantation. Five grafts were lost due to to repeated rejection episodes during the first two years after transplantation. Graft chronic infection with pseudomonas was detected to, with or without wound infection. The last three cases remained still with good renal function and actual serum creatinin of 135+9 micromol/lit with Mycophenolate Mofetil, CyA and PRED as a maintenance therapy. Conclusions: After all, the authors can not recommend this type of transplantation not only from the ethical point of view, but also from frequent medical and surgical complications, sometimes life threatening. The authors conclude with the statement of his Holiness the Pap John Paul II during the Rome Congress of ITS: “Any procedure which tends to commercialise human organ or to consider them as items exchange or trade must be considered morally unacceptable, because the use of the body as an object is to violate the dignity of the human person”.

Histological study of fetal kidney with urethral obstruction and vesicoureteral reflux: a consideration on the etiology of congenital reflux nephropathy.

A recent subject of interest regarding reflux nephropathy is the presence of renal abnormalities in neonates and infants who have no history of urinary tract infections. Debates have centered on the etiology of this renal abnormality - congenital reflux nephropathy; regarding whether it is the result of abnormal ureteral budding or of back pressure effect from sterile reflux. We examined the renal pathology of fetuses with urethral obstruction and vesicoureteral reflux, and we suggest herein a possible etiology of congenital reflux nephropathy. The renal pathology of seven autopsied fetuses with vesicoureteral reflux was studied. Reflux was demonstrated at autopsy by slow injection of contrast medium into the bladder. Severe urethral obstruction, either atresia or urethral valves, was evident in six of the subjects. In six subjects, abnormality of the urinary tracts was detected by prenatal ultrasonography. Of these six subjects, three revealed characteristics of prune belly syndrome. Reflux was graded as moderate in five subjects, and severe in two. In three subjects autopsied at 21 weeks gestation or earlier, the kidneys were well-developed with normal corticomedullary configuration, and nephrogenesis was retained. In three cases autopsied at over 25 weeks of gestation, the kidneys were grossly cystic, and the nephrogenic zone was completely absent. Contrast medium was observed not only in the dilated ducts and tubules, but also in the subcapsular cysts. Extravasation of the contrast medium was seen in the peritubular space. In the last subject with normal lower urinary tract, abnormal segments among normal cortical structures were observed. Our findings of renal pathology in fetuses with reflux are quite similar to those seen in fetal hydronephrosis. Back pressure from reflux probably damages the developing kidney leading to a degeneration of the ampullae and a reduction in the number of nephrons. Both dilatation of the collecting ducts and tubules, and extravasation of the urine may result in interstitial fibrosis. We postulate that one of the important etiologies of congenital reflux nephropathy may be the result of back pressure from sterile reflux.

Endoskopische Operationsverfahren in der Kinderurologie

At the beginning of the new millennium minimally invasive techniques have conquered a major role in surgery, also in Pediatric Surgery and in Pediatric Urology.The authors suggest that there are clear advantages in performing a nephrectomy or heminephrectomy laparoscopically,whereas the authors still favourize the open surgery in case of pyeloplasty.Vesicoureteral reflux is treated by endoscopic methods as well as open surgery according to the reflux grade. Side effects of injected agents are possible and have to be considered.The immediate treatment of an infantile ureterocele is the domain of primary endoscopic puncture or incision. Still definite care will be taken by open procedures along with reimplantation of the ureter(s). Endoscopic incision by laser or cold knife is the treatment of choice in newborns with urethral valves. Despite favorable outcomes and encouraging results in pediatric urologic patients, the indication, the feasibility and superiority of minimally invasive procedures have to be critically considered before challenging standards in open surgery.

Cell turnover in normal and abnormal kidney development.

As metanephric mesenchyme converts into nephrons, the first step is aggregation into a 'condensate'. Precursors inside this structure are proliferative and have a low rate of apoptosis, accompanied by expression of PAX-2 and BCL-2 survival molecules; conversely, cells at the borders of the structure have a high rate of apoptosis, probably a normal mechanism to regulate the number of cells in each nephron. Ureteric bud/collecting duct survival and mitosis may be determined partly by renal mesenchymal secreted molecules such as hepatocyte growth factor (HGF) and glial cell line-derived neurotrophic factor. Human kidney malformations often occur with lower urinary tract obstruction, e.g. cystic dysplastic kidneys caused by urethral valves. Deregulation of cell turnover occurs in these organs, with enhanced proliferation in cystic epithelium, accompanied by PAX-2, BCL-2 and HGF receptor expression, and apoptosis in surrounding mesenchyme, which transdifferentiates under the influence of transforming growth factor-beta1 into smooth muscle instead of forming nephrons. Similar abnormalities of cell turnover and gene expression can be generated by experimental fetal urinary flow impairment. Finally, renal mesenchymal apoptosis, associated with renal hypoplasia, can be induced experimentally by maternal low protein diet.

Fetal obstructive uropathy--diagnosis and possible treatments

Abnormalities of the urinary tract are found in up to 5% of newborns. They account for 25% of all prenatally diagnosed congenital defects and contribute 4% to perinatal mortality. When considering the prognosis of these anomalies, association with other pathological conditions has to be taken into account. We discuss the state of the art in detection and management of fetal urinary tract abnormalities as are standard at this tertiary center of fetal medicine. Posterior urethral valve, megaureter or ureteropelvic junction obstruction represent one end of a wide spectrum of obstructive uropathies, ranging from pathophysiologically minor deviations to severe impairment of renal function. Organ development, physiology of urine production and excretion as well as their potential disorders are presented. Sonographic and invasive diagnostic options in the evaluation of the urogenital tract are discussed. Careful assessment of each individual case and prognosis determine pre- and postnatal treatment.

Urodynamic Evaluation and Management prior to Renal Transplantation

Objectives: To evaluate the indications for urodynamics studies (UDS) previous to renal transplantation (RT) and the results of bladder cycling (BC), performed in selected cases.Methods: Among 475 RT, 33 patients (6.9%) required UDS according our protocol. Indications were: (1) low urinary tract symptoms (LUTS); (2) defunctionalized bladder (DB), and (3) complex urologic history (reflux, neurogenic bladder, urethral valves, etc.). DB was defined as a total urinary output of <300 ml/24 h. Patients with DB underwent BC through a suprapubic catheter, undergoing UDS after BC. The rate and type of urodynamic anomalies were compared among the 3 groups. Cystometric capacity (CysCap) and compliance were evaluated according to the etiology of renal failure, diuresis, and number of previous bladder surgeries.Results: Fifteen patients (45%) showed abnormal UDS, Increased pressure at cystometry was the most frequent finding (46%). The rate of abnormal UDS among the 3 groups was not statistically significant (66.6% in DB, 42.8% in LUTS, and 35% in those with complex urologic history). Compliance and CysCap were significantly lower in the patients with DB, showing a significant correlation with diuresis. These parameters did not correlate with the number of previous bladder surgeries. BC resulted in normal UDS in 3 patients (33%) and a change in diagnosis in 2 patients (22%).Conclusion: Our indications gave a good diagnostic yield. Patients with DB represent a group with different urodynamic findings. BC provides a more precise diagnosis or normalization of UDS in some cases.

Peritoneal Dialysis in Children under 5 Years of Age

We report our experience with maintenance peritoneal dialysis (PD) in small children. This is a retrospective analysis of the patient records of all children under the age of 5 years treated with continuous peritoneal dialysis (CPD) between 1986 and 1994 in Finland. Treatment was started and the patients were seen at the outpatient clinic at the Hospital for Children and Adolescents, University of Helsinki, every 3 months. Between these visits, they had controls at their local hospital every 2-4 weeks. The most common primary renal disease in these 34 patients was congenital nephrotic syndrome of the Finnish type (27 patients). Others were: congenital nephrotic syndrome (3 patients), polycystic kidney disease (1), urethral valve (1), neuroblastoma (1), and renal dysplasia (1). Mean age at onset was 1.6 years and median treatment time 9.3 months. Time spent in hospital decreased from 270 days/year in the 1980s to 150 days/year in the 1990s. Two children died (5.9%). The peritonitis rate on continuous cyclic peritoneal dialysis was 1:11.5 patient-months. Hernias were diagnosed in 29% of the patients. After 3 months half of the patients were on antihypertensive medication. Pulmonary edema was diagnosed once in 12 patients and twice in 2 patients. During the first 6 months on PD the mean height standard deviation score (hSDS) increased from -2.13 to -1.66 (p < 0.0001). The 6-month change in hSDS before initiation and 6 months after the start of CPD increased from -0.12 +/-0.68 to +0.59 +/- 0.64 (p = 0.0008). Our results indicate that peritoneal dialysis is feasible and safe in small children. Mortality was low and growth was good. The major challenges presented by CPD therapy were maintenance of optimal nutrition, avoidance of peritonitis, and control of volemia.

Membrano-Bulbo-Urethral Junction Stenosis

Based on 4 cases of infravesical obstruction due to extreme caliber disproportion between the posterior urethra and the penile urethra, a pathophysiological mechanism for this dynamic obstruction is given and endoscopic treatment is described. Four cases of membrano-bulbo-urethral junction (MBUJ) stenosis, seen between September 1995 and April 1996, are described. Two boys had previous successful valve resection but still showed extreme ballooning of the posterior urethra. The other 2 boys showed bladder instability on urodynamics and the male variant of the spinning top urethra on voiding cystourethrography (VCUG). All cases were successfully treated by endoscopic incision at the 12 o'clock position of the kink between the posterior and the penile urethra which is seen when the full bladder is expressed. Disproportion in the posterior urethra, seen on VCUG, together with bad urinary flow measured on uroflowmetry raise the suspicion of MBUJ stenosis. Although rarely seen, extreme caliber disproportion in the male urethra can cause obstruction. Ballooning of the posterior urethra, caused by urethral valves, bladder instability resisted by voluntary sphincter contraction or congenital posterior urethral dilatation, creates an obstructive kink in the urethra comparable to some obstructions in ureteropelvic junction stenosis. If suspicion of such a form of obstruction arises, cystoscopy during pressure on the full bladder is mandatory in order to see the obstruction, descending as a membrane from the vault of the urethra.

Short- and Mid-Term Outcome of Different Types of One-Stage Hypospadias Corrections

This study is a retrospective analysis of the outcome of our one-stage hypospadias corrections. In the period from August 1979 to January 1991, 316 boys less than 14 years of age underwent a one-stage hypospadias correction in our hospital. For 283 patients, data on the familial incidence, associated urological pathology, age at the time of operation, operation technique and follow-up were analyzed. The familial incidence in our study group was 9.4%, associated cryptorchidism was seen in 8.6%, kidney abnormalities in 1.8% and urethral valves in 0.4%. The majority (65.3%) of the children were operated on before the age of 3 years. The operation was performed according to Magpi, Mathieu, Devine-Horton, an onlay, and tube formation according to Duckett and the double-faced island flap procedure. Including all postoperative disturbances, urethral complications were seen in 18.1% and skin-related complications in 4.6% of the patients. Even in experienced hands the overall complications rate is relatively high, and additional problems can be expected in the future. It is important to provide adequate information on the procedures and possible complication to the parents. The final long-term outcome after puberty has to be awaited.

Treatment of pediatric outpatients with complicated urinary tract infections

In an open and prospective study involving outpatient children with complicated urinary tract infections (UTI), we evaluated the therapeutic efficacy of ceftriaxone administered intramuscularly, once-daily--50 to 70 mg/kg, during 8 to 10 days. Initially, the selected patients exhibited at least two of the following clinical criteria: age below 6 months, any degree of toxicity, fever, strong suspicion or proved abnormalities of their urinary tracts and lumbar pain in children older than 4 years. Significant bacteriuria was demonstrated by urine culture in 40 patients (21 boys, 19 girls), whose ages ranged from 15 days to 6 years 9 months (median 3 months). The radiological studies revealed vesicoureteral reflux in 6 patients, urethral posterior valve in 1, and neurogenic bladder in 4. The main causative agents were Escherichia coli isolated in 30 patients, Klebsiella (4) and Proteus (4). The treatment was found to be effective in 38 patients (95%). There was failure of treatment in 1 patient and a symptomatic reinfection in another one. It was concluded that children with complicated UTI could be treated alternatively by once daily ceftriaxone.

Urethral valves during the first year of life—A retrospective multicenter study: H.-J. Pompino, R.H. Bodecker, and U.A. Trammer. Eur J Pediatr Surg 5:3–8, (February), 1995

Urethral valves during the first year of life—A retrospective multicenter study: H.-J. Pompino, R.H. Bodecker, and U.A. Trammer. Eur J Pediatr Surg 5:3–8, (February), 1995

Long-term follow up to determine the prognostic value of imaging after urinary tract infections. Part 2: Scarring.

Long term follow up of children with urinary tract infections, in whom imaging investigations were performed at presentation, has been used to identify features that distinguish those at greatest risk of progressive renal damage. No single investigation at presentation was able to predict subsequent deterioration but, by employing a combination of imaging investigations, it was possible to separate groups with high or low probability of progressive damage. In the low risk group the incidence of progressive damage was 0.2% (95% confidence interval (CI) 0 to 1.3%). The combination of both scarring and reflux at presentation, or one only of these but accompanied by subsequent documented urinary tract infection, was associated with a 17-fold (95% CI 2.5 to 118) increase in the relative risk of progressive renal damage compared with children without these features. The recommended combination of investigations at presentation for girls of any age and boys over 1 year is ultrasound and dimercaptosuccinic acid (DMSA) scintigraphy in all, to detect both scarring and significant structural abnormalities, renography in children with dilatation of any part of the urinary tract on ultrasound, to distinguish dilatation from obstruction, and an isotope voiding study in all who have acquired bladder control. This gives the best separation between those at high and those at low risk of progressive damage with least radiation dose and lowest rate of instrumentation. Micturating cystourethrography (MCU) should be restricted to girls who have not acquired bladder control, unless there is reason to suspect a significant structural abnormality such as urethral valves. A single non-febrile urinary tract infection that responds promptly to treatment is not a justification for performing MCU in boys under 1 year or in children of any age with bladder control. No case can be made for any abbreviated schedule of investigation. These risk factors should be taken into account when designing follow up protocols.

Urethral Valves During the First Year of Life - A Retrospective, Multicenter Study

The diagnoses, treatment, and clinical courses of 178 children with urethral valves were analyzed in a retrospective, multicenter study. In group I the diagnosis had been made during the first 4 weeks of life, in group II between the 2nd and 12th months of life, after which treatment was begun. Statistical analysis of the patient data and results revealed clearly poorer results for patients in group I, who had the most severe congenital abnormalities: 10% of the infants in group I died within the 1st year of life as compared to 1% in group II. Among the survivors, 25% of children in group I had renal insufficiency as opposed to 8.2% in group II. The following parameters proved to be statistically significant risk factors with regard to prognosis: 1. Birth weight 2. Age at diagnosis 3. Renal function, particularly when initial creatinine levels exceed 200 mumol/l and fail to decrease below 100 mumol/l after successful therapy 4. Bilateral grade IV or V vesicoureteral reflux 5. Late incontinence (after conclusion of the 5th year of life). Statistical significance could be demonstrated for parameters 1-4, but due to the small number of patients not for parameter 5. The study showed that numerous neoimplantations associated with high complication rates were carried out in both groups, however, no statistically significant effect on long-term prognosis could be established. Our results did not confirm those of the Toronto group, who proposed that broadening the indications for supravesical urinary diversion would provide demonstrable parenchymal protection. No statistically significant relationship between therapeutic procedure and long-term clinical course could be shown.(ABSTRACT TRUNCATED AT 250 WORDS)