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Objectives The aim of this retrospective study is to describe the importance of serum creatinine levels for the long-term renal outcome in cases of posterior urethral valve. Methods Records of 94 patients who underwent cystoscopic valve ablation were evaluated retrospectively. Demographic variables, radiologic findings, and serum creatinine levels were recorded. Patients were grouped according to the serum creatinine levels in the first month of life. Twenty-four patients' serum creatinine levels were found to be above 1 mg/dL in group 1 and ≤1 mg/dL in 70 patients in group 2. Results The initial findings of 74 patients were as follows: pyelonephritis in 24 (32.43%) patients, hydronephrosis in 20 (27.02%), and inability to urinate in 18 (24.32%). Prenatal hydronephrosis was present in 36 cases (38.29%). Of the 92 voiding cystograms, 53.26% had findings that included vesicoureteral reflux. The mean serum creatinine levels of the patients in group 1 increased above 1 mg/dL in the 2nd year, the significant difference between the two groups lasted up to 7 years of age, and no significant difference was observed at the age of 7. The mean serum creatinine levels of the patients in group 2 were observed to increase above 1 mg/dL by the 10th year. Conclusions In patients with posterior urethral valves, nadir creatinine, especially higher than 1 mg/dL, should be considered as high risk for end-stage renal disease. Therefore, clinicians should improve awareness about early detection and treatment of posterior urethral valves, and these patients should be monitored as early as possible for the risk of chronic kidney disease.

Posterior urethral valve (PUV) rarely causes bladder rupture. We experienced hydronephrosis due to ureteral obstruction after the natural repair of a ruptured bladder in a fetus with PUV. Fetal ascites and oligohydramnios were diagnosed at 26 weeks' gestational age. While we followed up with ultrasonography, we regularly removed the fetal ascites via abdominal puncture, injecting warm saline instead of amniotic fluid. At 35 weeks' gestational age, the infant was diagnosed with severe bilateral hydronephrosis, absent of ascites and oligohydramnios. Therefore, a Caesarean section was performed. After birth, the infant was diagnosed with hydronephrosis due to ureteral obstruction after the natural repair of a ruptured bladder associated with PUV. Thus, a ruptured bladder in a fetus with PUV that has naturally repaired should be closely monitored via ultrasonography for hydronephrosis due to ureteral obstruction.

Introduction and Aim: Antenatal treatment of obstructive uropathy, although widely performed, remains controversial. This study evaluated the long-term outcome of managing patients with posterior urethral valves (PUV), highlighting the effect of antenatal vesicoamniotic shunt placement for patients who underwent fetal surgery. Methods: The medical records of 58 patients with PUV were retrospectively reviewed from June 1998 to June 2004. On the basis of prenatal assessment of sonographic findings and serial urinary electrolytes and protein measurements, patients were divided into two groups: group 1 comprised patients who had antenatal vesicoamniotic shunt placement whereas group 2 comprised patients who underwent postnatal surgical correction of PUV. Their outcomes and long-term results were evaluated. Results: Patients were followed up from 6 months to 6½ years (mean 3.9 years). Group 1 included 12 patients who had vesicoamniotic shunt placement and were confirmed postnatally to have PUV. Four patients out of 12 died (33.3%); three out of the eight living patients had perinatal complications. Of the eight living patients, three (37.5%) underwent valve ablation and five (62.5%) underwent urinary diversion (three vesicostomies and two cutaneous ureterostomies). Renal function returned to normal in only four patients (50%). Radiological abnormalities (hydronephrosis and/or reflux) resolved in three (37.5%) patients, was downgraded in one (12.5%) patient and persisted in four patients (50%). Group 2 included 46 patients who were treated postnatally. Thirty-five patients (76%) underwent primary valve ablation, while 11 (24%) underwent urinary diversion (seven vesicostomies, four cutaneous ureterostomy and one pyelostomy). Renal function returned to normal in all patients who underwent valve ablation, except in three, while renal function returned to normal in only three of 11 patients who underwent urinary diversion. Radiological hydronephrosis and/or reflux resolved in 28 patients (60.9%), was downgraded in six patients (13%) and persisted in 12 patients (26.1%). Conclusions: Antenatal vesicoamniotic shunt placement makes no difference to the outcome and long-term results of patients with PUV and debate about its efficacy on renal outcome remains. Primary valve ablation is the keystone of treatment for patients with PUV that might achieve the primary goal of nephron preservation. The lowest creatinine concentration in the first year of life is the most appropriate predictor of future renal function.

Objective To report our 12-year experience with endoscopic management of patients with concomitant anterior and posterior urethral valves. Methods We retrospectively reviewed the charts of patients referred to us for management of urethral valves from 2000 to 2012 to find cases with concomitant anterior and posterior valves. The diagnosis of valves was first suspected on voiding cystourethrography and confirmed by urethrocystoscopy. We collected available data on patients' age at diagnosis, clinical presentations, ultrasound and urodynamic findings, and surgical treatments. The final outcome at last follow up was also recorded. Results From 38 cases with anterior urethral valve, six (15.8%) presented concomitant anterior and posterior valves. The age at diagnosis in these patients ranged from antenatal diagnosis to 13 years. Initial presenting symptoms were recurrent urinary tract infection, incontinence, urosepsis and poor urinary stream. All valves were ablated by transurethral fulguration/resection using small-sized urethrocystoscopes. Among those with concomitant anterior and posterior valves, four patients had vesicoureteral reflux at presentation that resolved in two patients after valve ablation. One patient progressed to renal failure and required dialysis. Bladder hypercontractility and detrusor overactivity were the main urodynamic patterns in these patients. Conclusions Concomitant anterior and posterior valves seem to be more prevalent than previously assumed, and might be missed on initial assessment. Oblique view voiding cystourethrography with full-length delineation of the urethra is of paramount diagnostic importance when obstruction is suspected. A meticulous urethrocystoscopy should follow for confirming the diagnosis and endoscopic ablation/resection of the valves.

Objective Fetal serum β2-microglobulin has been shown to predict postnatal renal outcome in cases of fetal obstructive uropathy. We assessed the value of serial measurements of fetal serum β2-microglobulin in the prediction of postnatal renal outcome. Methods We retrospectively studied renal outcome in 42 fetuses with bilateral or low urinary tract obstruction that had fetal blood sampling on at least two occasions to assay serum levels of β2-microglobulin. Amniotic fluid volume at the time of each sampling was recorded. We classified renal outcome as either favorable (when postnatal renal function was normal) or adverse (when postnatal chronic renal failure occurred or when renal dysplasia at autopsy was noted). A β2-microglobulin cut-off of 5 mg/L and amniotic fluid index of 5 cm were used to predict postnatal renal outcome. Results Renal outcome was adverse in 28 cases and favorable in 14. In 12 (28.6%) cases, fetal serum β2-microglobulin concentration differed between the first and last measurement. Prediction of postnatal renal outcome was correct in 11 of these cases based on the last β2-microglobulin measurement. The sensitivity of β2-microglobulin in predicting renal outcome was significantly higher (P = 0.005) when using the last rather than the first measurement (96.4% vs 64.3%), with similar specificity for both measurements (85.7% vs 78.6%, non-significant). The sensitivity of amniotic fluid volume was also significantly higher (P = 0.005) when using the last rather than the first measurement (75.0% vs 35.7%), with similar specificity for both measurements (64.3% vs 71.4%, non-significant). Conclusion Sequential measurement of serum β2-microglobulin, performed for adverse ultrasound findings, such as renal parenchymal abnormality or decreasing amniotic fluid volume, predicts postnatal renal outcome more accurately than does a single assay. This may be due to possible worsening of renal injury with increasing duration of urinary tract obstruction. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Vascular complications are important causes of allograft loss in renal transplantation. A two and a half-month-old boy was diagnosed with posterior urethral valve and progressed to end-stage renal disease at eight yr of age. During the HD period, a central venous catheter was replaced three times for repeated thrombosis. The boy was found to be homozygous for FVL and heterozygous for both MTHFR (C677T) and PAI. At the age of 12, renal transplantation was performed from a deceased donor. Postoperative anticoagulation therapy was initiated with continuous intravenous administration of heparin at the dose of 10 IU/kg/h. HD was performed for the first three days. By the fourth day of transplantation, his urine output had increased gradually. Heparin infusion was continued for 18 days during hospitalization at the same dosage. Thereafter, he was discharged with LMWH. On the third month after transplantation, his serum creatinine level was 1.1 mg/dL and eGFR was 75.7 mL/min/1.73 m(2). He has still been using LMWH, and his eGFR was 78.7 mL/min/1.73 m(2) eight months after transplantation. Postoperative low-dose heparin treatment is a safe strategy for managing a patient with multiple thrombotic risk factors.

Objective To evaluate prenatal sonographic signs that distinguish male fetuses with posterior urethral valves (PUV) from those with vesicoureteral reflux (VUR). Methods Antenatal data were retrospectively retrieved from all consecutive women delivering between 2003 and 2012 of a male newborn with a postnatal diagnosis of PUV or VUR. Prenatal parameters included fetal bladder characteristics, identification of a dilated posterior urethra, and change in shape and size in the fetal renal pelvis or ureter. Results 136 women gave birth to a male newborn with a postnatal diagnosis of PUV (n = 49) or VUR (n = 87). The presence of posterior urethral dilatation (21 [42.9%] fetuses versus 0 [0%], p = 0.000), a thickened fetal bladder wall (37 [75.5%] versus 4 [4.6%], p = 0.000) and anhydramnios (14 [28.6%] versus 0, p = 0.000) were strongly associated with the postnatal diagnosis of PUV. Change in shape and size in the fetal renal pelvis or ureter was observed in 15 (17.2%) of 87 children with VUR versus 1 (2.0%) out of 49 with PUV (p = 0.010). Conclusions Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR.

The cause of posterior urethral valves (PUV) is unknown, but genetic factors are suspected given their familial occurrence. We examined cases of isolated PUV to identify novel copy number variants (CNVs). We identified 56 cases of isolated PUV from all live-births in New York State (1998-2005). Samples were genotyped using Illumina HumanOmni2.5 microarrays. Autosomal and sex-linked CNVs were identified using PennCNV and cnvPartition software. CNVs were prioritized for follow-up if they were absent from in-house controls, contained ≥ 10 consecutive probes, were ≥ 20 Kb in size, had ≤ 20% overlap with variants detected in other birth defect phenotypes screened in our lab, and were rare in population reference controls. We identified 47 rare candidate PUV-associated CNVs in 32 cases; one case had a 3.9 Mb deletion encompassing BMP7. Mutations in BMP7 have been associated with severe anomalies in the mouse urethra. Other interesting CNVs, each detected in a single PUV case included: a deletion of PIK3R3 and TSPAN1, duplication/triplication in FGF12, duplication of FAT1--a gene essential for normal growth and development, a large deletion (>2 Mb) on chromosome 17q that involves TBX2 and TBX4, and large duplications (>1 Mb) on chromosomes 3q and 6q. Our finding of previously unreported novel CNVs in PUV suggests that genetic factors may play a larger role than previously understood. Our data show a potential role of CNVs in up to 57% of cases examined. Investigation of genes in these CNVs may provide further insights into genetic variants that contribute to PUV.

Objectives To compare outcomes of renal transplantation (RTx) in children with end-stage renal disease (ESRD) resulting from lower urinary tract dysfunction (LUTD) vs other causes. Patients and Methods A database of children (

Aim This study aims to evaluate posterior urethral valve (PUV) in a developing country with focus on the profile of the disease, determinants of outcome and the challenges of management. Methods Analysis of data collected prospectively from 31 patients managed for PUV at the University of Nigeria Teaching Hospital, Enugu from January 2008 to December 2009. Results The median age at presentation was 2.5 years (range 2 weeks–15 years). Only two (6.5%) had pre-natal diagnosis. Twenty-six (83.9%) had one or more complications of the disease at presentation. The mean estimated glomerular filtration rate was 39.5 mL/min/1.73 m2 (range 4.4–116.0). There was hydronephrosis involving 50 renal units, and vesicoureteral reflux involving 22 renal units. Eight cases required upper tract diversion and four required dialysis prior to definitive catheter ablation. After average follow-up of 12.6 months (range 3–22 months), 18 (58.1%) cases had clinical and radiological improvement, 9 (29%) had persisting poor renal function and 4 (12.9%) died from acute renal failure and end-stage renal disease. Associated pulmonary hypoplasia in neonates, presentation at adolescent age, bilateral vesicoureteral reflux and estimated glomerular filtration rate

Bladder rupture in a fetus is a rare occurrence. We report the first neonatal case of laparoscopic repair for prenatally diagnosed bladder rupture. A male neonate, who had presented with megacystis, bilateral hydronephrosis and hydroureters since 27 weeks-of-gestation, was delivered by emergency cesarean section because of sudden collapse of the bladder with massive ascites at 37 weeks-of-gestation. The diagnosis of bladder rupture was made by retrograde urethrocystography. Laparoscopic repair was carried out subsequent to urethral catheterization. Intraoperatively, laceration from the left side of the bladder dome to the posterior wall was observed. This tear was successfully repaired using a two-layer interrupted suture without any postoperative complications. Laparoscopic repair could be the treatment of choice for bladder rupture, because it has the advantage of closure of the perforated site more certainly than bladder decompression alone, and with better cosmesis than open repair.

Introduction: A urinoma is a fluid mass consisting of extravasated urine in the perirenal space. Its impact on renal function was analysed. Methods: All cases of prenatal and neonatal urinoma reported in the literature were analysed as well as two cases recently observed in our department. Results: A review of all prenatally diagnosed urinomas revealed 25 cases, for a total of 30 renal units. The cause of obstruction was: lower urinary tract (LUT) obstruction in 16 cases (62.5%), upper urinary tract (UUT) obstruction in seven cases (29.5%) and unknown in two cases (8%). A preserved renal function was found in only 30% of all cases. Furthermore, prognosis for renal function was better in posterior urethral valve (PUV) patients than in uretero-pelvic junction obstruction (UPJO) patients. In addition, we analysed 35 published cases of urinoma with a neonatal onset, without prenatal diagnosis, and secondary to LUT obstruction in 27 cases (77%), UUT obstruction in seven cases (20%), while the underlying cause was unknown in one cases (3%). A preserved renal function was observed in 84% of cases with UUT obstruction and in 80% of cases presenting a LUT obstruction. Conclusions: Prognosis concerning renal function seems to be mainly related to two factors: age at presentation and underlying diagnosis. Decompression of the urinary tract by urine extravasation produces the best results in terms of preservation of the renal function in LUT obstruction (75% in prenatal and 84% in postnatal cases); UUT obstruction however is associated with a good prognosis in the neonatal period (80% of preserved renal function) but with a severe impairment (only 20% of preserved renal function) in prenatally detected cases.

The fetal urinoma is a rare but important diagnosis, as it indicates substantial underlying obstruction with implications for the functionality of the affected kidney. This case series describes a single center's experience with the diagnosis and management of fetal urinomas. All 25 cases were diagnosed or referred to our medical center over an 11-year period. Most cases were secondary to either posterior urethral valves or ureteropelvic junction obstruction. Fetal interventions, including percutaneous drainage of the urinoma and cystoscopic alleviation of bladder outlet obstruction, were performed in 4 cases.

SUMMARY An anterior urethral valve causing bladder outlet obstruction in children is rare. The early identification and management of this condition is crucial as delay may lead to chronic renal damage. We present a case of a 10-month old male child diagnosed with an anterior urethral valve by micturating cystourethrogram. This case emphasizes the importance of visualizing the whole of the penile urethra in performing a paediatric MCU examination or this important diagnosis may be missed.

The aim of this study was to detect possible risk factors for UC and UTI following pediatric renal Tx and effect of these complications on outcome. One hundred and eight children who underwent living donor Tx between 2009 and 2015 were retrospectively included. Extraperitoneal approach was used with stented tunneled extravesical procedure. Mean recipient age was 9.89 ± 3.46 years while mean weight was 25.22 ± 10.43 kg. Seventy-three (67.6%) recipients were boys while 92 (85.2%) were related to donors. Urological causes of ESRD were present in 33 (30.6%) recipients (14 [13%] posterior urethral valve, 16 [14.8%] VUR, and 3 [2.8%] neurogenic bladder). Augmentation ileocystoplasty was performed in 9 (8.3%) patients. Mean follow-up was 39.3 ± 17.33 months. UC were detected in 10 (9.3%) children (leakage 4 [3.7%], obstruction 3 [2.8%], and VUR 3 [2.8%]) while UTIs were reported in 40 (37%) children. After logistic regression analysis, UC were significantly higher in children with cystoplasty (44.4% vs 6.1%; P = .001). UTIs were significantly higher in girls (51.4% vs 30.1%; P = .001) and in children with urological causes of ESRD (51.5% vs 30.7%; P = .049). UC and UTI were not significantly associated with increased graft loss or mortality. UC were significantly higher in children with cystoplasty while UTIs were significantly higher in girls and children with urological causes of ESRD. Presence of UC did not affect the rate of graft loss or mortality due to its early detection and proper management.

OBJECTIVES To evaluate sexual function and fertility in adult patients treated for posterior urethral valves (PUV) in childhood and to compare these patients with the normal population. To examine if patient characteristics such as chronic renal failure (CRF), history of cryptorchidism and bladder neck incision in childhood have an impact on sexual function. PATIENTS AND METHODS Information on sexual function was assessed using questions from the International Index of Erectile Function (IIEF). In addition, the patients were asked if they had had children or been treated for CRF. Out of 108 adult patients with PUV, 67 (62%) returned the questionnaires. Sexual function in these patients was compared with 201 controls and paternity rates were compared with a national database. RESULTS The mean (sd) age of the patients and controls was 38 (9) and 38 (7) years, respectively. Six percent of the patients and 9% of the controls reported problems in achieving erection during sexual stimulation and 9% of the patients and 10% of the controls reported problems with the hardness of erection (P = nonsignificant). Ejaculation was absent in 1/61 sexually active patients (2%). The only significant risk factor in patients for erectile dysfunction (ED) was increasing age. Thirty-three (49%) of all 67 patients and four (57%) of the seven patients with kidney transplantation had had children. The paternity rates were similar to those in corresponding age groups of the general Finnish population. Eight patients (12%) had attempted to have children without success. CONCLUSIONS Men treated for PUV have a similar prevalence of ED and similar paternity rates to men without PUV. Erectile function and paternity rates can be satisfactory in spite of CRF.

Kamal MM, El-Hefnawy AS, Soliman S, Shokeir AA, Ghoneim MA. Impact of posterior urethral valves on pediatric renal transplantation: A single-center comparative study of 297 cases. Pediatr Transplantation 2011: 15: 482–487. © 2011 John Wiley & Sons A/S. Abstract: Objective: This is a retrospective long-term evaluation of the renal allografts and bladder functions in pediatric recipients who had posterior urethral valves (PUV). Patients and methods: Between March 1976 and February 2009, 2033 live-donor renal transplantations were carried out in our center. Of these, 297 (14.2%) were in the pediatric age (≤18 yr). The pediatric recipients included 20 (6.7%) boys who developed end-stage renal disease as a late complication of PUV and recognized as group I while the remaining 277 pediatric recipients were defined as group II. Demographic characteristics, post-transplant complications and graft function were compared among both groups. Patient and graft survivals of both groups were also estimated. Moreover, the bladder function of the study group was evaluated by urodynamic studies. Results: Patients with PUV (group I) were significantly younger than group II. Although the overall rate of urological complications in both groups was essentially similar, the incidence of urinary fistulae and urinary tract infection were higher in group I. The mean (SD) follow up periods for group I and II were 4.7 (4.1) and 6.4 (4.8) yr, respectively. At last follow up the serum creatinine values were similar among patients of both groups. Moreover, there were no differences in graft or patient survival at five and 10 yr. Detrusor over-activity could be elicited in only one of group I patients. Schafer nomogram showed non-obstructed pattern in all cases. Conclusion: Good functional outcome could be achieved for patients with PUV if renal transplantation is necessary. Pre-transplant surgical procedures may be required such as nephroureterectomy, cytoplasty or injection of refluxing ureters. A robust anti-refluxing uretero-vesical anastomosis is important, and can be achieved by a Lich-Gregoir procedure.

Fetal interventions have been proposed for treatment of severe lower urinary tract obstruction (LUTO), as this condition is associated with high rates of perinatal mortality and postnatal renal impairment. The rationale for in utero treatment for those cases is based on the possibility of relieving the obstruction, improving the amniotic fluid volume, and preventing renal and bladder damage. Candidates for fetal intervention should be rigorously selected based on the confirmation of severe LUTO (dilated bladder and bilateral hydronephrosis), oligohydramnios or anyhydramnios and 'favorable' fetal urinalysis (dependent on gestational age). Nowadays there are two different therapeutic options with specific technical approaches. Vesico-amniotic shunting is an easier procedure, but with a higher frequency of related complications. Fetal cystoscopy can be used for diagnostic purpose and for treatment of posterior urethral valves, with suggestive advantage of allowing a more physiological release of the obstruction. According to the literature, estimated survival rates and postnatal normal renal function frequencies are approximately 40 and 50% after vesico-amniotic shunting and 75 and 65% after fetal cystoscopy, respectively.

We report the case of a fetus with severe megabladder, displaying the ‘keyhole’ sign on ultrasound imaging, that underwent cystoscopy at 22 weeks' gestation. There was a familial history of mild urethral atresia. Fetal cystoscopy revealed congenital urethral atresia. A guide wire was advanced through the fetal urethra and a transurethral vesicoamniotic stent was placed successfully. The fetus was delivered at 36 weeks' gestation and postnatal cystoscopy confirmed the absence of posterior urethral valves or urethral atresia. The infant was 5 years old with normal renal function at the time of writing. We conclude that fetal cystoscopic placement of a transurethral stent for congenital urethral stenosis is feasible. Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd