Urethral Atresia Research Articles

Challenges and practical considerations in delivering botulinum neurotoxin to adults with congenital neurogenic lower urinary tract dysfunction with history of genitourinary reconstruction

Purpose:While intradetrusor botulinum neurotoxin (BoNT) injection is a well-established treatment of neurogenic detrusor overactivity, its administration in individuals with complex genitourinary (GU) reconstruction can be difficult. The objective of this report is to highlight our institution’s experience with challenges and practical solutions in delivering BoNT to this unique patient population. Materials and Methods:We performed a retrospective review of our institution’s database of adults with congenital neurogenic lower urinary tract dysfunction (NLUTD) with history of complex GU reconstruction who received intradetrusor BoNT injections performed by a single surgeon between 2016 and 2023. In patients with multiple injections, the most recent procedure was used as the index case for analysis. Results:A total of 17 patients were included, with a mean age of 27 years (range 21–46). The most common diagnosis was myelomeningocele (n=13), followed by sacral agenesis (n=2), Williams syndrome (n=1), and urethral atresia (n=1). The most common GU reconstruction was augmentation cystoplasty (n=11). 75% (12/16) of the patients had a continent catheterizable channel (CCC) and 31% (5/16) had undergone prior bladder neck reconstruction or closure. Conclusion:Additional considerations and instruments are required for safe and effective BoNT injections in individuals with congenital NLUTD and reconstruction. Adequate preparation by the staff and the provider may be needed. Our recommendation generally favors the utilization of a flexible cystoscope or a rigid scope with a smaller sheath size to minimize trauma to the CCC, bladder neck, or urethra. Additionally, it is advisable to consider reconstituting BoNT in higher concentrations to enable the administration of fewer injections. Furthermore, performing these procedures in the operating room under anesthesia allows for the development of a well-considered strategy prior to any attempts in the office setting We hope that this manuscript sparks discussion between pediatric urologists and adult urologist to expand our knowledge on strategies needed for delivering BoNT to individuals with complex and restricting GU surgeries.

Real-world safety of Levetiracetam: Mining and analysis of its adverse drug reactions based on FAERS database

Levetiracetam is a relatively new and widely utilized anti-seizure medication; however, limited information is available regarding its adverse effects. This study aims to thoroughly investigate, evaluate, and present evidence on the safety profile of Levetiracetam, relying on data from the FDA Adverse Event Reporting System (FAERS) database to facilitate informed clinical decision-making. We employed various statistical measures, including Reporting Odds Ratio (ROR), Proportionate Reporting Ratio (PRR), and analysis by the Medicines and Healthcare Products Regulatory Agency (MHRA), to identify signals of adverse reactions associated with Levetiracetam. Positive signals consistent with Designated Medical Event (DME) were singled out for focused comparison and discussion. The analysis of 26,182 adverse events linked to Levetiracetam as the primary suspected drug revealed 692 positive signals spanning 22 System Organ Classes (SOCs). Nervous system disorders were the most frequently reported, followed by psychiatric disorders, and general disorders and administration site conditions. 11 positive signals consistent with Preferred Terms (PTs) in DME were identified, predominantly concentrated in 6 SOCs. Among these, rhabdomyolysis, Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) exhibited relatively large values of A, ROR, and Chi-squared. Additionally, PTs related to spontaneous abortion, drug interaction, urethral atresia, ventricular septal defect, and atrial septal defect showed significant strength. The study indicates that Levetiracetam carries a potential risk of causing rhabdomyolysis, SJS, TEN, DRESS as well as spontaneous abortion. Signals related to drug interaction, urethral atresia, ventricular septal defect, and atrial septal defect warrant heightened attention in clinical use.

Troubleshooting Tips for Diagnosing Complex Fetal Genitourinary Malformations.

Fetal genitourinary anomalies can present a diagnostic challenge for the radiologist. The absence of a normally located kidney may represent agenesis or be secondary to a fusion or migration abnormality. A dilated renal pelvis should prompt evaluation for a specific cause, including ureteropelvic junction obstruction, reflux, or an obstructed duplicated system. Cystic parenchymal changes are characteristic of a multicystic dysplastic kidney but may also be seen in obstructive cystic dysplasia. There are numerous causes of megacystis including chromosomal (trisomy 18 syndrome), obstruction (posterior urethral valves, urethral atresia), or muscular dysfunction (prune belly syndrome, megacystis microcolon intestinal hypoperistalsis syndrome). Important mimics of a large bladder include hydrocolpos and urogenital sinus or cloacal malformation. Complications of genitourinary malformations are common and include oligohydramnios, urinary ascites, and urinoma. Making an accurate diagnosis often requires additional US views beyond those obtained in the standard fetal survey and occasionally performing fetal MRI. The appropriate use of orthogonal T2-weighted sequences, in conjunction with diffusion-weighted images for evaluation of the kidneys and gradient-recalled-echo sequences for evaluation of T1-hyperintense meconium in the colon, can play an integral role in diagnosis. Accurate diagnosis of fetal genitourinary malformations is vital to direct patient counseling and pregnancy management as outcomes are highly variable. Some conditions can be surgically corrected quite simply, some require multiple complex procedures, and some are lethal. The authors offer troubleshooting tips to narrow the differential diagnosis for four observations: unilateral absent kidney, dilated renal pelvis, cystic renal parenchyma, and megacystis and its mimics. ©RSNA, 2023 Test Your Knowledge questions are available in the Online Learning Center.

Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience

Intrauterine vesicoamniotic shunting (VAS) using a Somatex® shunt was shown to significantly affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO) [Figure1]. Data on postnatal surgical management and complications are largely lacking. To describe the postnatal management of patients with prenatal VAS for megacystitis in suspected severe LUTO. All male newborns with previous intrauterine VAS using a Somatex® shunt treated in our institution were retrospectively analyzed. We evaluated the spectrum of urethral pathologies and postnatal surgical management, especially focusing on shunt removal. Between 2016 and 2022, 17 patients (all male) were treated postnatally in our institution after VAS for suspected severe LUTO. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in eight patients (8/17). Seven shunts could be removed without further anesthesia as a bedside procedure. Ten patients required surgical shunt removal under general anesthesia due to migration (59%). Laparoscopic shunt extraction was performed in 8/10 cases. Most frequently, dislocated shunts were located incorporated in the detrusor in eight cases and the removal required a bladder suture in 2/8 patients. In one case, the shunt was removed from the abdominal wall and in one case from the intestine wall [Figure2]. Posterior urethral valves were found in 8/17 patients, 6/17 patients showed a urethral atresia and one patient had urethral duplication. In two patients, we identified a high grade bilateral vesicoureteral reflux without LUTO. In our observation, more than half of the newborns with megacystis in suspected LUTO require a shunt removal surgery after early VAS using a Somatex® shunt. Urethral atresia may be found more frequently in these patients. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Cloacal Malformation with Associated Urethral Atresia

Introduction Cloacal malformations comprise a heterogeneous group of anomalies that are considered the most complex anorectal malformations (ARMs) in females. Precise evaluation to identify the unique anatomy prior to reconstruction with collaboration between colorectal surgeons, urologists, and gynecologists is vital. Here, we present a rare anatomical variation in a patient with a cloacal malformation which affected operative and postoperative management. Case description A 6-year-old female with cloaca who underwent colostomy, vaginostomy, and vesicostomy as a newborn presented for reconstruction. Her VACTERL workup was negative except for an atretic right kidney. Her ARM index included the cloaca, a normal spine, and sacrum with a lateral sacral ratio of 0.7, predicting good potential for bowel continence. Cystoscopy through the vesicostomy showed a small bladder with normal ureteral orifices, and a closed bladder neck, with no identifiable urethra. A cloacagram showed an atretic common channel, a single small vagina, and a rectum below the pubococcygeal line. The patient underwent a posterior sagittal anorectovaginourethroplasty, vaginal patch using rectum, rectoplasty, and perineal body reconstruction. The urethra was not amenable to reconstruction, so the vesicostomy was preserved and a future Mitrofanoff was planned. Conclusion Urethral atresia is a rare and challenging finding in cloaca patients, and a vesicostomy is needed to drain urine in the newborn period. Preoperative examination under anesthesia, cystoscopy, vaginoscopy, and cloacagram are crucial to identify the precise anatomy and to plan accordingly.

Technical details and long-term outcomes of P.A.D.U.A. for congenital urethral narrowing; a case series and review of the literature

The P.A.D.U.A. technique is a method of addressing congenital urethral narrowing. It involves passive dilation with a series of progressively larger indwelling catheters. Utilization is limited by scant literature, particularly regarding technical details and long-term durability. Tools for achieving safe and reliable urinary drainage are critical in these patients, who require careful stewardship of their kidney and bladder function. To describe long-term urethral patency and urinary function following P.A.D.U.A., and to provide sufficient technical detail to reproduce the technique. Patients with congenital urethral narrowing managed with P.A.D.U.A. were identified and chart review was performed. Details of catheter exchange sequences were compiled and described. The primary outcome was the attainment of adequate urethral caliber by successful completion of P.A.D.U.A., and the secondary outcome was voiding per urethra at most recent follow-up. P.A.D.U.A. achieved adequate urethral caliber in 9/11 (82%) of patients. This included seven patients with Prune Belly Syndrome, one with isolated urethral atresia, and one with a cloacal anomaly. P.A.D.U.A. failed to achieve urethral patency in one patient with urethral duplication, who was unable to progress through the catheter sequence, and one patient with Prune Belly Syndrome, who completed P.A.D.U.A. but developed recurrent narrowing one week later. There were no delayed failures of urethral patency. Patients who achieved patency underwent a median of seven catheter placements over 92 days. Median (range) initial and final catheter sizes were 3.5F (1.9-8F) and 14F (8-16F). While 82% achieved patency, only 3/11 (27%) were voiding spontaneously per native urethra at most recent follow-up. This series of patients undergoing P.A.D.U.A. for primary treatment of congenital urethral narrowing is the largest to date and provides granular technical details. It aligns with prior reports suggesting that P.A.D.U.A. achieves urethral patency in most patients with Prune Belly Syndrome or isolated urethral atresia, but has limited application in the hypoplastic duplicated urethra. Despite high rates of urethral patency in this select population, many patients will not achieve and maintain spontaneous voiding without catheterization. This is likely due to ongoing deterioration of bladder function caused by the prenatal developmental insult, paralleling the phenomenon seen in posterior urethral valves. P.A.D.U.A. is an effective and durable technique for achieving urethral patency. However, due to complicating factors such as the underlying bladder pathology present in many patients, urethral patency achieved with P.A.D.U.A. does not guarantee long-term safe and reliable spontaneous emptying per urethra.

Three new experimental models of anterior urethral stricture in rabbits.

BackgroundThis study describes and compares three surgical procedures for the construction of urethral stricture (US) models in rabbits.MethodsForty adult male rabbits were allocated to four groups: 36 rabbits were randomly assigned to three experimental groups, while the remaining 4 were assigned to a sham group. The penis was separated from the rectum. Then along the ventral midline, a longitudinal penile skin incision was made while ensuring that the urethral mucosa was intact and the muscular layer was not completely incised. In group 1 (n=12), ventral semi-circumferential mucosa electrocoagulation of a 1-cm length of the anterior urethra was performed until ulceration occurred. In group 2 (n=12), the ventral urethral mucosa was incised, and electrocoagulation of the dorsal semi-circumferential mucosa was performed. In group 3 (n=12), whole-circumferential mucosa electrocoagulation was performed. In group 4 (n=4), no special treatment was performed. Four weeks later, urethrography, urethroscopy, and histological evaluation were carried out.ResultsThe weights of the rabbits in the four groups were comparable. There was no significant difference between groups 2 and 3 with regard to operative time, but the operative time in these groups was significantly longer than that in group 1 (group 2 vs. group 1: P<0.05, group 3 vs. group 1: P<0.001). After the surgery, urinary fistula with infection occurred in one rabbit in group 1, and one rabbit died due to urethral atresia in group 3. According to the urethrography and urethroscopy findings, 9 out of 12 rabbits in group 1, 5 out of 12 rabbits in group 2, and 11 out of 11 rabbits in group 3 developed US, while no rabbits in the sham group developed US. Histopathological examination revealed injury to the urothelium, inflammatory infiltration, a decrease in the amount of blood vessels and smooth muscle fibers, and a decrease in the amount of collagen fibers.ConclusionsCompared with the semi-circumferential procedures, the whole-circumferential procedure had a higher success rate. Therefore, this procedure seems to have potential for the construction of long-segment rabbit US models.

A rare case of aphallia associated with urethral atresia and multiple renal anomalies

Aphallia, or penile agenesis, is an extremely rare congenital anomaly, resulting from the failure of the genital tubercle to develop normally during the early period of embryogenesis. We present the case of a baby born at term by normal vaginal delivery to a primi mother with an uneventful antenatal history, except for oligohydramnios detected in the third trimester. Examination of the baby revealed an absence of phallus and external urethral meatus, normally descended testes, a normal scrotum, and a normally positioned patent anal orifice. Subsequent workup of the baby by radiological studies revealed a malrotated right kidney in the midline, multicystic renal dysplasia of the left kidney, and urethral atresia.

Z-scores of fetal bladder size for antenatal differential diagnosis between posterior urethral valves and urethral atresia.

ABSTRACTObjectiveTo construct reference values for fetal urinary bladder distension in pregnancy and use Z‐scores as a diagnostic tool to differentiate posterior urethral valves (PUV) from urethral atresia (UA).MethodsThis was a prospective cross‐sectional study in healthy singleton pregnancies aimed at constructing nomograms of fetal urinary bladder diameter and volume between 15 and 35 weeks' gestation. Z‐scores of longitudinal bladder diameter (LBD) were calculated and validated in a cohort of fetuses with megacystis with ascertained postnatal or postmortem diagnosis, collected from a retrospective, multicenter study. Correlations between anatomopathological findings, based on medical examination of the infant or postmortem examination, and fetal megacystis were established. The accuracy of the Z‐scores was evaluated by receiver‐operating‐characteristics (ROC)‐curve analysis.ResultsNomograms of fetal urinary bladder diameter and volume were produced from three‐dimensional ultrasound volumes in 225 pregnant women between 15 and 35 weeks of gestation. A total of 1238 urinary bladder measurements were obtained. Z‐scores, derived from the fetal nomograms, were calculated in 106 cases with suspected lower urinary tract obstruction (LUTO), including 76 (72%) cases with PUV, 22 (21%) cases with UA, four (4%) cases with urethral stenosis and four (4%) cases with megacystis‐microcolon‐intestinal hypoperistalsis syndrome. Fetuses with PUV showed a significantly lower LBD Z‐score compared to those with UA (3.95 vs 8.83, P < 0.01). On ROC‐curve analysis, we identified 5.2 as the optimal Z‐score cut‐off to differentiate fetuses with PUV from the rest of the study population (area under the curve, 0.84 (95% CI, 0.748–0.936); P < 0.01; sensitivity, 74%; specificity, 86%).Conclusions Z‐scores of LBD can distinguish reliably fetuses with LUTO caused by PUV from those with other subtypes of LUTO, with an optimal cut‐off of 5.2. This information should be useful for prenatal counseling and management of LUTO. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Bladder outlet obstruction

Bladder outlet obstruction

Hydroureter

Hydroureter

Renal pelvic dilation

Renal pelvic dilation

First Case of a Term Male Born with Aphallia and Complete Urethral Atresia

Aphallia is an exceedingly rare condition often associated with an array of genitourinary anomalies. Classically, aphallia was thought to have to co-exist with a urethral fistula for adequate urine outflow to avoid the sequelae of oligohydramnios, while the absence of a fistula has historically been incompatible with life. We report the first case of a prenatally unrecognized aphallia with complete urethral atresia in a full-term baby, without an associated fistula or ectopic urethra. We postulate a urachal cyst noted on prenatal ultrasound resolved into a patent urachus providing sufficient outflow to avoid sequelae of oligohydramnios and allowed for term birth.

Patent urachus and bladder outflow obstruction\u2014chance or consequence? A study of a cohort of patients with complete patent urachus presenting to a tertiary urological center and a review of literature

BackgroundTo explore the association between patent urachus and bladder outflow obstruction (BOO). A retrospective review of patient records over a 35-year period (1983–2018) with complete patent urachus was performed. Antenatal ultrasound findings were noted, and postnatal investigations included ultrasound (US), micturating cystourethrogram (MCUG), functional nuclear medicine scans (MAG3, DTPA, and DMSA), and serum creatinine. Associated anomalies and management in all patients were analyzed.ResultsSixty-six patients with all types of urachal remnants were identified of whom only 16 had a patent urachus. All presented clinically with a discharging umbilicus, 10/16 confirmed on MCUG and 4 had umbilical cord cysts on antenatal US. Twenty-five percent had associated bladder outlet obstruction (BOO): etiologies included atresia of posterior urethra, congenital urethral hypoplasia, urethral atresia with prune belly syndrome, and sacrococcygeal teratoma. Vesicoureteral reflux (VUR) was confirmed in 37%, and four of them had bladder outlet obstruction (BOO).ConclusionWith patent urachus, bladder outflow obstruction occurs in the minority. Based on our findings, we commend US and cystogram to document VUR. The isolated PU should be treated nonoperatively up to a year of age. Renal function should be checked with the finding of VUR. The etiopathogenesis of the condition remains uncertain.

Fetal bladder outflow obstruction: Interventions, outcomes and management uncertainties

Fetal lower urinary tract obstruction (LUTO) is classically based on prenatal ultrasound identification of a dilated/ thick-walled bladder, bilateral hydronephrosis, dilated ureters and a dilated posterior urethra (also known as the “keyhole sign”) in a male fetus. Although the most common underlying diagnosis is posterior urethral valves, the prenatal appearance may be similar with urethral atresia or stenosis, the Prune-Belly Syndrome, or even a cloacal anomaly in a female. These conditions form part of the Congenital Anomalies of Kidney and Urinary Tract (CAKUT) spectrum, which is the commonest cause of end-stage renal disease in children. Although it is difficult to predict postnatal renal function from the prenatal appearance, studies have recently identified predictive features (based on ultrasound findings and fetal biochemistry), and established staging systems to assist with counselling, and, where indicated, patient selection for in-utero intervention. Current in-utero therapy includes amnio-infusion, vesico-amniotic shunting, and fetal cystoscopy with valve ablation or urethral stenting. Postnatal survival and renal functional outcomes, complications and management uncertainties are described, highlighting areas of future development.

Obstructive Nephropathy in Children – A Review

Obstructive nephropathy is any affection of the urinary tract characterized by impairment of urine flow through the tract and which, if left untreated, will cause progressive renal damage. Causes in children are congenital or acquired. Congenital causes are pelvi-ureteric junction obstructions, posterior urethral valves (PUV), urethral atresia, phimosis and meatal stenosis. The acquired causes are calculi, post-traumatic and post-inflammatory strictures and meatal stenosis. There are some manifestations like prune-belly syndrome, hydronephrosis and renal failure. Diagnostic investigations include ultrasonography, intravenous urography, cystography and renography. Advanced technologies have impacted on the treatment of the different lesions such as utero vesico-amniotic shunt and endoscopic valve ablation for PUV and minimally invasive techniques for urolithiasis. Nephrectomy may be indicated in a unilateral damaged kidney. Sometimes treatment may fail because of pretreatment irreversible renal damage. Such as end-stage renal failure is an indication for renal transplantation. So, proper treatment is essential to prevent end-stage renal failure.&#x0D; Bangladesh Med J. 2017 Sep; 46 (3): 107-113

Acquired Urethrovaginal Fistula and Urethral Atresia in a Patient with a Sacrococcygeal Teratoma

Acquired urethrovaginal fistulae and urethral atresia are rare findings in pediatric patients, but have been described in adult patients related to trauma or iatrogenic injury. Little exists in the published literature to guide management of such conditions in children, but lessons learned from congenital causes can help. Herein we discuss the preoperative evaluation and management of a child with an acquired urethrovaginal fistula and urethral atresia likely related to in utero compression from an intrapelvic sacrococcygeal teratoma and provide several images detailing the complex anatomy.

Surgical selection and outcome for treating the post-traumatic posterior urethral strictures via transperineal approach

Objective To evaluate the outcome of various operative selection for treating posterior urethral stricture via transperineal approach. Methods The clinical data of 5 000 patients with posterior urethral stricture or obstruction from January 1990 to January 2017 were analyzed retrospectively. All patients were male. The age of those patients ranged from 18 months to 77 years old, mean 36.6 years old. Pelvic crush injury caused by car accident occurred in 2010 cases. Falling injury occurred in 1680 cases. Pelvic compressed injury occurred in 1 310 cases. Accompanied visceral damage occurred in 2 590 cases, including the liver and spleen rupture in 920 cases, lower limb fracture in 1 200 cases. 2 200 cases accepted urethral realignment under emergency. 2 800 cases were treated with pubic cystostomy. All patients were undergone a retrograde and voiding urethrogram. 562 patients accepted urethral ultrasongraphy, and 2 448 patients accepted urethroscopy. 204 patients accepted MRI examination. The mean stricture length was 4.3 cm, ranged from 1.8 to 8.6 cm. Posterior urethral stricture was found in 810(16.2%)cases. The complete obstruction of posterior urethra was found in 4 190(83.8%)cases, of which the length of the distraction defects≤3 cm was found in 2 650(53.0%)cases and the length of the distraction defects>3 cm was found in 1 540(30.8%)cases. Bladder calculi was found in 2 300 cases. The perineal fistula or abscess was noticed in 290 cases. False passage was found in 460 cases. Urethra rectum fistula was found in 160 cases. Bladder neck open was noticed in 89 cases. Repairing was performed via a simple anastomosis after urethral mobilization in 1 700 patients, via separation of the corporeal bodies in 1 302 patients, via separation of the corporeal bodies and inferior pubectomy in 1 910 patients and via scrotal skin flap urethroplasty in 68 patients. Pull-through operation was performed in 20 patients. Results Postoperative follow-up were conducted from 6 to 72 months with average duration of 23 months. The overall successive results after operation was 92.2%(4 608/5 000), which the Qmax was more than 15 ml/s .The successive rate of urethroplasty were 97%(1 649/1 700 )in simple anastomosis; 93%(1 211/1 302 )in separation of the corporeal bodies; 88% (1 680/1 910) in separation of the corporeal bodies and inferior puberctomy; 78%(53/68) in scrotal skin flap urethroplasty and 83%(15/18) in pull-through operation. The successive rate were 96%(778/810)in posterior urethral stricture; 95%(2 517/2 650)in distraction with the length of obstruction less than 3 cm and 86%(1 324/1 540)in distraction with the length of obstruction more than 3 cm. Conclusions The transperineal end to end anastomotic urethroplasty has become the first-line therapy for posterior urethral atresia. The length of the strictures or distraction defect which is lower than 3 cm is much more successfully corrected. Key words: Urethral stricture; Urological surgical procedure; Transperineal; Wound and injuiry

Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction

Vesicoamniotic shunting (VAS) and other bladder drainage techniques for fetal lower urinary tract obstruction (LUTO) have been proven to ameliorate pulmonary hypoplasia and increase survival in patients with an initial poor prognosis. Currently there are limited prognostic tools available during gestation to evaluate and predict postnatal renal function. The aim was to describe the prenatal growth of the renal parenchymal area (RPA) in patients with LUTO and determine its application as a predictor of renal function at one year of life. The study population comprised a retrospective cohort of all infants who survived the fetal VAS to birth. Renal growth and size were measured using imageJ software to calculate the RPA in sequential prenatal ultrasounds. The parenchymal area was measured from the image of each kidney with the greatest longitudinal length. These measurements were further correlated and analyzed as a predictor of end-stage renal disease (ESRD) within the first year of life. Etiologies of LUTO in the 15 male fetuses included eight posterior urethral valves, four Eagle-Barrett/prune belly syndrome, two urethral atresia, and one megacystis microcolon intestinal hypoperistalsis syndrome. All patients had patent shunts, in place, at birth. Furthermore, ultrasonographic parameters such as oligohydramnios, keyhole sign, and bladder wall thickness showed no statistical difference between groups. Renal parenchymal growth correlated with postnatal renal function in both the ESRD (r=0.409, p=0.018) and the non-ESRD (r=0.657, p<0.001) groups. Most notably, RPA during the 3rd trimester predicted ESRD with the best cut-off point determined to be 8cm2 (sensitivity, 0.714; specificity, 0.882; and positive likelihood ratio, 6.071) (Table). Despite definitive VAS for LUTO, postnatal morbidity and mortality remain high, emphasizing the role of renal dysplasia in postnatal renal failure, in spite of urinary diversion. Renal growth statistically differs between groups in the 3rd trimester of gestation; RPA development appears stagnant in patients that developed ESRD within the first year of life. In contrast, patients that did not develop ESRD continued to have renal parenchymal growth in a linear fashion. This suggests that prenatal RPA may be predictive of postnatal ESRD. RPA measurement during the prenatal period could play an important role as a non-invasive tool to predict postnatal renal function and to anticipate postnatal clinical interventions.

Long-term renal outcome in infants with congenital lower urinary tract obstruction

Congenital Lower Urinary Tract Obstructions (LUTO) is a heterogeneous group of diseases in which urine elimination is obstructed at the level of bladder neck or urethra. The aim of the study is to evaluate the long-term renal outcome of patients suffering of congenital LUTO. We retrospectively reviewed patients with congenital LUTO. All patients had at least 1 year follow-up. Data on surgery, renal imaging and Schwartz estimate creatinine clearance were collected. Incidence of Chronic Renal Disease (CRD) is presented with Kaplan-Meier method. 40 patients were included, 23 patients with Posterior Urethral Valve (PUVs) and 17 patients with other aetiologies: anterior urethral valve (2), urethral atresia (2), urethral stenosis (2), cloacal malformations (2), obstructive ureterocele (1), bladder trigone malformation (1) and neonatal bladder-sphincter dysfunction without neurological abnormalities (7). Incidence of CRD at age 10 years was 37% in congenital LUTO, 42% in PUVs and 30% in other aetiologies, and was significantly higher in PUVs (P=0.032). Renal prognosis was significantly worsened by discover of retentional bladder wall changes in initial cystoscopy, and by loss of parenchymal differentiation or cortical microcysts in first ultrasonography. The use of urinary diversion was significantly higher in LUTO of other aetiologies. A high incidence of CRD is observed in patients with congenital LUTO, significantly higher in patients with PUV. LUTO of other aetiologies require step by step surgical management and higher use of urinary diversion. Precise initial evaluation in cystoscopy and ultrasonography is required and participate to evaluate future renal outcome. 4.