Bilateral Vesicoureteral Reflux Research Articles

Cloacal malformations in the pediatric surgery department of Fez

Introduction: Cloacal malformations, which exclusively occur in females. These anomalies are rare. The immediate prognosis depends mainly on the severity of the associated malformations and relies on the quality of the surgical repair. Patients and methods: This is a retrospective descriptive study of 5 patients managed in the pediatric surgery department of the Hassan II University Hospital of Fez, during a 13-year period from January 2008 to January 2021. Results: The diagnosis is made postnatally following systematic clinical examination in the delivery room, with only one case presenting with intestinal obstruction. The malformative assessment revealed: One case with cardiopathy, abnormal renal ultrasound findings in 3 cases (predominantly uretero-pyelocaliceal dilation), and spinal anomaly found in 2 cases, and abnormal MRI findings in 3 cases, while not performed in 2 cases. Four contrast studies were performed after colostomy, revealing 2 rectovesical fistulas and 2 bilateral VUR (vesicoureteral reflux). Immediate postoperative course was uneventful, with no dehiscence, stenosis, or obstruction noted. One patient died due to cardiac decompensation at 3 months of age. Long-term evolution was characterized by the presence of anal incontinence. Conclusion: Cloacal malformation is a serious pathology, it can frequently lead to functional and vital prognosis without forgetting the psychological one. The prognosis depends mainly on the quality of management and the presence of associated malformations.

Urodynamic Abnormalities in Children with Primary Bilateral Vesicoureteral Reflux.

Bilateral vesicoureteral reflux (VUR) has been associated with poor long-term prognosis, and research focusing exclusively on its effect on urinary bladder function is lacking. This study aims to assess the urodynamic characteristics of children with bilateral VUR and identify factors influencing renal function. A retrospective analysis was performed on children diagnosed with bilateral VUR who underwent urodynamic examination in the outpatient department of our institution from January 2019 to January 2023. All patients underwent comprehensive assessments, including voiding cystourethrography, urinary system ultrasound and urodynamic examination, to analyse the urodynamic features associated with different clinical characteristics. The study included 148 children with bilateral VUR, amongst whom 92 (62.2%) exhibited urodynamic abnormalities, including idiopathic detrusor overactivity (DO) in 30 cases (20.3%) and dysfunctional voiding (DV) in 58 cases (39.2%). DV was prevalent in children under 5 years of age, whereas idiopathic DO was predominantly observed in children older than 2 years of age. Children older than 5 years of age exhibited a trend towards reduced bladder volume, whereas those younger than 2 years of age often had increased bladder volume and residual urine. Multivariate analysis identified bilateral severe VUR as an independent risk factor for abnormal estimated glomerular filtration rate. DV tends to be more common in younger children with larger bladder volumes and increased residual urine than in older children. By contrast, older children are more susceptible to idiopathic DO associated with smaller bladder volumes than younger children. Bilateral severe reflux increases the likelihood of renal functional abnormalities, which appear to be a consequence of congenital scarring rather than abnormalities in bladder function.

Revisiting the Blocksom Vesicostomy Technique in a very sick child with Bilateral high-grade VUR: A Case Report

Vesicoureteric reflux (VUR) is a common cause of repeated urinary tract infections (UTIs) in children. Vesicostomy is considered in selective cases of high grade as an initial temporary procedure to effectively empty the bladder, prevent urinary stasis and chances of reflux, and reduce the incidence of UTI, thereby protecting the upper tracts. The most commonly followed temporary urinary diversion procedure is the one described by Blocksom in 1957. The method is simple, effective, and easy to perform, reversible, protects the upper tracts, and minimizes the incidence of UTIs. Keywords: Vesicoureteric Reflux, Temporary Urinary Diversion, Vesicostomy, Blocksom Vesicostomy, Reversible.

Development and multi-institutional validation of a deep learning model for grading of vesicoureteral reflux on voiding cystourethrogram: a retrospective multicenter study

Voiding cystourethrography (VCUG) is the gold standard for the diagnosis and grading of vesicoureteral reflux (VUR). However, VUR grading from voiding cystourethrograms is highly subjective with low reliability. This study aimed to develop a deep learning model to improve reliability for VUR grading on VCUG and compare its performance to that of clinicians. In this retrospective study in China, VCUG images were collected between January 2019 and September 2022 from our institution as an internal dataset for training and 4 external data sets as external testing set for validation. Samples were divided into training (N=1000) and validation sets (N=500), internal testing set (N=168), and external testing set (N=280). An ensemble learning-based model, Deep-VCUG, using Res-Net 101 and the voting methods was developed to predict VUR grade. The grading performance was assessed using heatmaps, area under the receiver operating characteristic curve (AUC), sensitivity, specificity, accuracy, and F1 score in the internal and external testing set. The performances of four clinicians (2 pediatric urologists and 2 radiologists) with and without the Deep-VCUG assisted to predict VUR grade were explored in external testing sets. A total of 1948 VCUG images were collected (Internal dataset=1668; multi-center external dataset=280). For assessing unilateral VUR grading, the Deep-VCUG achieved AUCs of 0.962 (95% confidence interval [CI]: 0.943-0.978) and 0.944 (95% [CI]: 0.921-0.964) in the internal and external testing sets, respectively, for bilateral VUR grading, the Deep-VCUG also achieved high AUCs of 0.960 (95% [CI]: 0.922-0.983) and 0.924 (95% [CI]: 0.887-0.957). The Deep-VCUG model using voting method outperformed single model and clinician in terms of classification based on VCUG image. Moreover, Under the Dee-VCUG assisted, the classification ability of junior and senior clinicians was significantly improved. The Deep-VCUG model is a generalizable, objective, and accurate tool for vesicoureteral reflux grading based on VCUG imaging and had good assistance with clinicians to VUR grading applicability. This study was supported by Natural Science Foundation of China, "Fuqing Scholar" Student Scientific Research Program of Shanghai Medical College, Fudan University, and the Program of Greater Bay Area Institute of Precision Medicine (Guangzhou).

Risk factors for new renal scarring in children with vesicoureteral reflux receiving continuous antibiotic prophylaxis

To investigate the risk factors for new renal scarring (NRS) in children with vesicoureteral reflux (VUR) receiving continuous antibiotic prophylaxis (CAP). This was a single-center cohort study. The clinical data of 140 children with grade I–V VUR receiving CAP were analyzed. In this study, exposure variables were sex, younger age at the initial diagnosis of UTI ≤ 12 months, the occurrence of breakthrough urinary tract infection (BT-UTI), high-grade VUR, bilateral VUR, etiology, presence of renal scarring at the initial diagnosis and ultrasound abnormalities. The outcome was NRS. A total of 140 children were included in the risk factor analysis of NRS, 73 of whom experienced NRS, an incidence rate of 52.14%. Multivariate Cox regression suggested that the presence of renal function impairment after the initial diagnosis of UTI (OR 3.411, 95% CI 1.5751–6.646) and the occurrence of BT-UTI while receiving CAP (OR 1.995, 95% CI 1.089–2.958) were independent risk factors for NRS. Multivariate Cox regression showed that high-grade VUR had no significant effects on NRS (OR 0.940, 95% CI 0.462–1.912, P = 0.864). No significant difference was identified in multivariate Cox regression analysis in the IV–V group (vs I–III group) (OR 0.960, 95% CI 0.565–1.633, P = 0.960). Renal function impairment after the initial diagnosis of UTI and the occurrence of BT-UTI while receiving CAP were independent risk factors for NRS. Neither univariate analysis nor multivariate analysis found a correlation between VUR grade and NRS.

Transurethral laser lithotripsy using the Mitrofanoff urethral conduit for bladder stones: a case report

BackgroundThe Mitrofanoff (appendicovesicostomy) procedure is a contraindicated urinary modification that maintains urinary continence by forming a flap-valve mechanism at the site of anastomosis between the appendage and bladder wall, which is used as a guide for urinary drainage. This technique has been used by intermittent self-catheterization patients who have difficulty voiding from the native urethra or in cases where voiding from the abdominal wall would improve quality of life. However, the risk of stone formation is high due to intermittent urinary catheterization using the Mitrofanoff conduit urethrostomy as a conduit.Case presentationThe patient was a 22-year-old Asian-Japanese woman. At 6 years of age, she underwent bilateral vesicoureteral reflux surgery, Mitrofanoff urethrostomy using the appendix, abdominal wall plication, and vaginoplasty using the ileum. During follow-up, ultrasound performed due to persistent pain during urinary drainage revealed a 26 mm bladder stone. We performed ureteroscopic lithotripsy 6Fr using ureteral access sheath and made lithotripsy using Ho: YAG laser, then successfully removed the target stone.ConclusionsWe report a case of transurethral laser lithotripsy using the Mitrofanoff urethral conduit for bladder stones. Using with ureteral access sheath made lithotripsy and retrieved ureteral stone more effective.

Evaluation of the results of urodynamic studies in patients with vesicoureteral reflux.

To investigate urodynamic abnormalities associated with vesicoureteral reflux (VUR) in toilet-trained children. The data of 157 children who were diagnosed with vesicoureteral reflux and referred to our hospital between 2013 and 2022 were retrospectively examined. The urodynamic parameters were analyzed and correlated with age, gender, lower urinary tract symptoms (LUTS), reflux severity, and laterality. Overall, 131 (83.4%) patients had abnormal urodynamic findings with a male-to-female ratio of 1:1.4. The most common pathological finding was detrusor overactivity (DO), identified in 101 (64.3%) patients, followed by dysfunctional voiding (DV) in 74 (50.3%) patients. Children with VUR grades II and III exhibited a greater percentage of abnormal urodynamic findings than children with grades IV and V. The prevalence of DO was higher in children younger than 10 years old with unilateral and lower-grade VURs. DV was more frequent in children older than 10 years, with bilateral VUR, and higher grade VUR. The prevalence of LUTS, bowel and bladder dysfunction (BBD), and urinary tract infection (UTI) was higher among children with abnormal urodynamic findings. Children with VUR have a high incidence of urodynamic disorders. Urodynamic dysfunction may contribute to the pathogenesis of VUR, especially in mild cases.

Does previous endoscopic subureteric injection (STING) effect the outcomes of robot-assisted laparoscopic ureteral reimplantation surgery (RALUR) in children?

There is lack of evidence on the success of robot-assisted laparoscopic ureteral reimplantation (RALUR) for the treatment of vesicoureteral reflux (VUR) who had prior intervention. This study aimed to evaluate the effect of previous unsuccessful STING on the outcomes of RALUR in children. A total of 67 children treated with RALUR by a single surgeon for the treatment of VUR were scanned between February 2018 and April 2022. Two patients were excluded from the final data analysis due to the presence of a megaureter. Patients were divided into two groups: those with a history of STING (Group A - n:14) and those without STING (Group B - n:51). Patient characteristics, previous numbers of injections, console time, total operative time, perioperative and postoperative complications and clinical success data were collected. Clavien Dindo and Satava complication scales were used as the standard record of peri and postoperative complications. Radiographic success was defined as absence of reflux detected on postoperative voiding cystourethrography, whereas clinical success was defined as the absence of a febrile urinary tract infection during the follow-up. Mann-Whitney U and Chi-square tests and Fisher exact test were used where appropriate. A total of 36 (55.3%) female versus 29 (44.6%) male patients were operated for 96 refluxive ureters. Nearly half of the patients were with bilateral VUR (n=31). The mean follow-up was 20.2±15.4 months. The median age of patients was 59±31 (range: 28-132 months) versus 46±33.1 (range: 7-206 months) for groups A and B respectively (p=0.22). Gender, age, peri- and postoperative complication rates, and clinical success were comparable between the two groups. The median operative time and the console time was significantly higher in children with history of STING (op time: 142.5±27.4 versus 120±24.9min (p=0.008), console time: 117.5±28.2 versus 100±24.5min (p=0.011) for groups A and B, respectively. A total of six complications (9.2%) occurred with none of them were greater than Clavien grade 3b. The overall clinical success rate was 97%, with 2 cases of clinical failure. In both cases, VCUG demonstrated absence of VUR. The outcomes of our study provided that RALUR is effective with more than 95% success rates despite failed endoscopic injection procedures. The previous history of STING neither changes the success nor the complication rates of RALUR. However, this can lead to more challenging surgery by increasing the total operative times.

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.

Posterior Urethral Valve: Factors Affecting the Renal Outcome

Background: Posterior urethral valve (PUV) is the most common cause of lower urinary tract obstruction in male children with associated sequlae. Different factors, such as age at presentation, initial and nadir serum creatinine, renal parenchymal echogenicity on initial USG, vesicoureteric reflux (VUR) , recurrent UTI, bladder dysfunction and the presence or absence of pop-off mechanism like VURD have significant impact on ultimate renal outcome. The main aim of the study was to observe the effects of different prognostic factors like age of presentation and preoperative VUR of the PUV respondents on postoperative long term renal outcome as well as changes of renal function status on the basis of initial and postoperative serum creatinine level in our settings. Material Methods: This was a quasi-experimental study and conducted in the Department of Pediatric urology of Bangladesh Shishu Hospital and Institute, Dhaka. We enrolled 58 male children of different ages having posterior urethral valve, who were admitted in the pediatric urology department from July,2018 to Dec,2021. Results: Total 58 patients included in our study. Among all patients 53.4% were aged between 1 month to 1 year, 39.7% of them were more than 1 year old and 6.9% of them were neonates. Among 31 respondents, 32.3 % had poor renal function 35.5 % of them also had moderately impaired renal function. Again, out of 23 respondents, 30.4% had poor renal function 6% had moderately impaired renal function. The relation between age category and postoperative renal function status (On the basis of postoperative eGFR) of the posterior urethral valve respondents were statistically not significant. Patients who had no VUR, 66.7% of them had normal renal function, 25% had moderately impaired renal function and 8.3% had poor renal function. Respondents who had bilateral VUR, 55% of them had poor renal function and 35% had moderately impaired renal function. Among respondents who had unilateral VUR, 28.6% of them had poor renal function and 28.6% had moderately impaired renal function but 42.9% had normal renal function. The relation between preoperative VCUG findings and postoperative long term renal function status of the PUV patients were statistically significant. 7 (100%) respondents who had preoperative abnormal renal function, more than 71.4% of them had returned to normal renal function after operative procedure. But abnormal renal function was present in 2 (28.6%) patients during follow up. In our study we found 29.3% of our patients had poor renal outcome and 29.3% patients had moderately impaired renal function within the mean follow up period of 19.33±12.38 months (ranges from 9months to 4 years). Conclusion: Our research highlights the significance of age at presentation, despite its statistically negligible effect on long-term renal outcomes. However, among the children with PUV following valve ablation and with a long-term follow-up, beginning serum creatinine and the presence of various types of VUR on initial VCUG had a significant effect (p.05) on postoperative renal function on the basis of serum creatinine.

V04-05 ROBOT-ASSISTED LAPAROSCOPIC EXTRAVESICAL BILATERAL URETERAL REIMPLANTATION: LUAA TECHNIQUE

You have accessJournal of UrologyCME1 Apr 2023V04-05 ROBOT-ASSISTED LAPAROSCOPIC EXTRAVESICAL BILATERAL URETERAL REIMPLANTATION: LUAA TECHNIQUE Parviz Hajiyev, Jared Fialkoff, and Mohan Gundeti Parviz HajiyevParviz Hajiyev More articles by this author , Jared FialkoffJared Fialkoff More articles by this author , and Mohan GundetiMohan Gundeti More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003252.05AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: In today's era of minimally invasive surgery, robotic-assisted laparoscopic ureteral reimplantation (RALUR) is being popularly performed at many centers. We previously described the LUAA technique (Eur Urol 2016; 70(5):818–823). METHODS: Through this video, we share the step-by-step technical details of the “LUAA” (L-length of ureteric tunnel, U- the u stitch at UVJ, A- placement of the ureteral apical alignment suture and A- ensuring inclusion of adequate ureteral adventitia in detrusorrhaphy) for bilateral ureteric reimplantation. A 4 years old girl presented with recurrent urinary tract infections (UTI) . Her voiding cystourethrogram (VCUG) was suggestive of bilateral high grade of vesicoureteric reflux (VUR), and DMSA scan showed multiple scars. RESULTS: For this persistent bilateral VUR with breakthrough UTIs beyond the age of toilet training, she underwent a robotic-assisted laparoscopic ureteric reimplantation- bilateral (RALUR-b) by the “LUAA” technique. CONCLUSIONS: Postoperative she had USS one month and one year and was normal. The VCUG at 4 months showed resolved VUR bilateral. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e339 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Parviz Hajiyev More articles by this author Jared Fialkoff More articles by this author Mohan Gundeti More articles by this author Expand All Advertisement PDF downloadLoading ...

A novel endoscopic approach for management of hutch diverticulum concomitant vesicoureteral reflux with dextranomer/hyaluronic acid copolymer injection

There are various treatment options for symptomatic bladder diverticulum, including robotic-assisted laparoscopic bladder diverticulectomy, open and endoscopic techniques. But, to date, the optimal surgical technique remains unclear. To present the preliminary long-term follow-up results of a novel technique of dextranomer/hyaluronic acid copolymer (Deflux) plus autologous blood injection for correction of hutch diverticulum in patients with concomitant vesicoureteral reflux (VUR). We retrospectively reviewed four patients who had hutch diverticulum with concomitant VUR and had undergone submucosal Deflux following autologous blood injection. Patients with neurogenic bladder, posterior urethral valve, or voiding dysfunction were excluded from the study. Success was defined as the resolution of the diverticulum, hydronephrosis, and hydroureter on ultrasonography at a 3-month follow-up and long-term symptom-free period. Four patients with hutch diverticula were included. The median age at the time of surgery was 6.1 (range 3-8) years. Three of them had unilateral VUR, and one had bilateral VUR. During the procedure, a mean of 0.625ml Deflux plus a mean of 1.25ml autologous blood were injected submucosally for correction of VUR. Additionally, a mean of 1.62ml Deflux plus a mean of 1.75ml autologous blood were injected submucosally to occlude the diverticulum. The median follow-up was 4.6 (range 4-8) years. This method had excellent success in all patients in the current study with no postoperative complications such as febrile urinary tract infection, or diverticulum, hydroureter, or hydronephrosis in follow-up ultrasounds. Submucosal injection of Deflux plus autologous blood injection can be a successful endoscopic intervention for treatments of hutch diverticulum in patients with concomitant VUR. Deflux injection can be a simple and cost-effective technique.

Vesicoscopic ureteral reimplantation with a modified Glenn-Anderson technique for vesicoureteral reflux

Vesicoureteral reflux (VUR), commonly referred to as urinary reflux, is one of the most common congenital urological anomaly to occur during childhood. Historically, open vesicoureteral reimplantation has been the gold standard in the surgical treatment of VUR. Currently, vesicoscopic ureteral reimplantation is used as a minimally invasive alternative to open ureteral reimplantation. Most vesicoscopic procedures are performed using the transtrigonal Cohen technique. As a non-transtrigonal technique, the vesicoscopic Politano-Leadbetter technique is also performed, but requires dissection outside the bladder under a narrow surgical field, and in boy carries a risk of vas deferens injury. This study evaluated surgical outcomes and perioperative findings for vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique in children with VUR. Eighteen consecutive children who underwent vesicoureteral ureteral reimplantation using a modified Glenn-Anderson technique were included in this study. The surgical procedure was explained and surgical outcomes and perioperative findings were evaluated. Patients comprised 9 boys and 9 girls with 29 cases of ureteral reflux (7 unilateral cases, 11 bilateral cases). All procedures were performed laparoscopically, with no cases requiring conversion to open surgery. Median operative time was 143min for unilateral VUR and 194min for bilateral VUR. Only one case showed a complication (Clavien-Dindo grade 1). The remaining 17 cases showed no complications, with removal of the urethral catheter and discharge 2 days postoperatively. Seventeen of the 18 cases underwent postoperative voiding cystourethrography, showing no VUR in all cases. Vesicoscopic ureteral reimplantation is a minimally invasive alternative to open surgery, with most procedures performed using a transtrigonal Cohen technique. Regardless of whether the surgery is open or laparoscopic, a disadvantage of the Cohen technique is that postoperative transureteral treatment may not be possible. The Politano-Leadbetter technique has been reported as a non-transtrigonal technique. However, this requires dissection outside the bladder under a narrow surgical field, and carries a risk of vas deferens injury in boys. In this study, vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique provided good surgical outcomes with minimal perioperative complications and easy manipulation under a wide field of view. Although many minimally invasive treatments are available, vesicoscopic ureteral reimplantation using a modified Glenn-Anderson technique is safe and effective for patients with VUR 4 years old. To demonstrate the further utility of this procedure, long-term outcomes and safety evaluations are needed in a larger number of cases.

A case report of urethral stricture of a 3 months old female child

Background: Urethral stricture is a narrowing of the urethra which can either be congenital or acquired. In most cases, it is an acquired disorder. Congenital stricture is less common and even less commonly found in paediatric population. Case presentation: A 4-Month-old baby attended outpatient department with complaint of high fever and excessive crying that was more pronounced before and during urination along with whitish discharge mixed with urine. Urine R/M/E detected plenty pus cell. Left ureter appears grossly dilated, tortuous in USG. Constriction is noted in the distal part of urethra with proximal dilatation with bilateral vesico-ureteric reflux. Conclusion: As, no other cause i.e., iatrogenic, traumatic, inflammatory, post-infectious, was found out, there remain only one possibility for this baby having urethral stricture which is idiopathic or congenital cause. Bangladesh Journal of Medical Science Vol. 22 No. 01 January’23 Page : 239-241

Associação entre refluxo vesicoureteral e anomalia do cromossomo 22 em anel - relato de caso

Vesicoureteral reflux (VUR) is characterized by retrograde urine flow, caused by impaired occlusion of the intramural portion of the ureter by contraction of the detrusor muscle during urination or secondary to altered bladder dynamics, ureteral obstruction and neuromuscular diseases, such as Phelans syndrome -McDermid. This syndrome is a rare and underreported disease, resulting from the formation of chromosome 22 in a ring and has VUR as its main urological manifestation. The objective of this study is to alert the medical community about one of the differential diagnoses in the VUR etiology, based on the case of a female patient, 4 years and 2 months old, referred to the Pediatric Surgery service of the Mário Covas State Hospital with recurrent infection of urinary tract associated with impaired renal function and bilateral VUR evidenced on cystourethrography. During investigation, the phenotypic findings encouraged the performance of a karyotype that confirmed the alterations found in Phelan-McDermid syndrome. VUR early diagnosis was essential to establish the appropriate therapy, which involved clinical and surgical approaches aimed at improving quality of life, preserving the renal parenchyma and preventing further future complications.

ASSOCIAÇÃO ENTRE REFLUXO VESICOURETERAL E ANOMALIA DO CROMOSSOMO 22 EM ANEL - RELATO DE CASO

Vesicoureteral reflux (VUR) is characterized by retrograde urine flow, caused by impaired occlusion of the intramural portion of the ureter by contraction of the detrusor muscle during urination or secondary to altered bladder dynamics, ureteral obstruction and neuromuscular diseases, such as Phelans syndrome - McDermid. This syndrome is a rare and underreported disease, resulting from the formation of chromosome 22 in a ring and has VUR as its main urological manifestation. The objective of this study is to alert the medical community about one of the differential diagnoses in the VUR etiology, based on the case of a female patient, 4 years and 2 months old, referred to the Pediatric Surgery service of the Mário Covas State Hospital with recurrent infection of urinary tract associated with impaired renal function and bilateral VUR evidenced on cystourethrography. During investigation, the phenotypic findings encouraged the performance of a karyotype that confirmed the alterations found in PhelanMcDermid syndrome. VUR early diagnosis was essential to establish the appropriate therapy, which involved clinical and surgical approaches aimed at improving quality of life, preserving the renal parenchyma and preventing further future complications.

Vertebral anomalies associated with anorectal malformations incidentally found on contrast studies: a case series

Background: Spinal congenital malformations are closely associated with other congenital anomalies, are usually asymptomatic and be found incidentally. Moreover, spine abnormalities such as hemivertebra may cause further complications if left untreated. Among patients with VACTERL anomalies, spine anomalies account for 60-95% of them. This report aimed to describe rare and interesting cases of spinal malformation in children to provide further information on the importance of a proper approach in such cases. Case Presentation: We present three pediatric cases of vertebral anomalies associated with anorectal malformations incidentally found in contrast studies. All patients have a history of anorectal abnormalities at birth and are referred to the hospital for contrast studies. The first case found semi-segmented hemivertebra at T8-9 levels, cleft at T10 level and high-type anorectal malformations (ARMs). The second case showed unsegmented hemivertebra at the level of L1 and butterfly vertebra at T10, approximately at the same level as the skin lesion, with the possibility of occult spina bifida and high-type ARMs. The third case revealed bilateral vesicoureteral reflux (VUR), grade 1 right hydroureteronephrosis and left hydroureter, with semi-segmented hemivertebra at the level of L3, unsegmented hemivertebra at L4-5 and an extra lumbar vertebra, possibly a lumbosacral transitional vertebra (LSTV). Conclusion: Thorough screening and other examinations such as contrast studies could reduce their morbidity as more sophisticated imaging may be done to better visualize the anatomical malformation.

P13.06: Valve Bladder Diversion, Making Possible a Kidney Transplant

Introduction: The late diagnosis in a patient with posterior urethral valve (PUV) and end-stage kidney disease is a challenging scenario which may need renal replacement therapy and reconstruction of the urinary tract. Reducing recurrent urinary tract infections (UTI) is the main urologic objective in these patients in order to offer them a kidney transplantation (KT). Many factors must be considered to define the best moment to perform the reconstruction, that could be done prior, during or after kidney transplantation. Patient and Method: This is a 5-year-old boy who was admitted for febrile urinary tract infection. During the admission end-stage kidney disease and PUV were diagnosed. The first step was to perform a cystoscopy and valvular ablation. Videourodynamyc studies after surgery showed a bladder’s capacity of 50 ml, low compliance, incomplete emptying associated with high grade bilateral vesicoureteral reflux. Hemodialysis began as renal replacement therapy, antibiotic prophylaxis and anticholinergics were indicated. Four months later, an open vesicostomy was performed due to persistent febrile urinary tract infections. Eleven months after diagnosis, the patient was transplanted using a graft from a deceased donor. At the same time of transplant surgery, the right kidney and ureter were resected through a right hockey stick incision. Two months after transplantation another endoscopic residual valve ablation was done. After seventeen months from kidney transplant, the left kidney and ureter were resected by laparoscopy. Urodynamic studies continued showing high voiding detrusor pressure. While patient kept his bladder diverted, incontinence was managed with five diapers a day and maintained scholar and physical activities. At two years of follow-up, a third valvular endoscopic revision and ablation was performed associated with circumcision and replacing the open vesicostomy with a suprapubic tube. Intermittent opening was indicated and after three months, the tube was removed. Six months after the last surgery, bladder’s capacity improved from 100 ml to 250 ml, bladder emptying has no residual volume and no incontinence. Kidney ultrasonography did not present hydronephrosis and cystography showed no VUR. Twenty nine months after KT the patient remained urinary tract infections free, creatinine level was 0.68 mg/dL and clearance 102 ml/min/1.73 m2. Conclusion: Open vesicostomy is a simple and safe procedure to manage UTI and allows kidney transplant in PUV valve bladders. Urological surveillance after transplant involves clinical assessment, urodynamic studies, endoscopic and reconstructive surgeries. Bladder cycling after transplant improves storage and augmentation procedures may not be needed.

A case of minimal change disease associated to Rubinstein Taybi syndrome

Introduction: Focal segmental glomerulosclerosis (FSGS) is the main glomerular nephropathy secondary to malformative uropathies. The latter can be included in the poly malformative syndromes in the pediatric population. However, other glomerular impairments may rarely be associated with some polymalformative syndromes as reported in this rare case of a minimal change disease (MCD) associated with Rubinstein Taybi syndrome (RSTS). Materials and methods: We report a case of pure nephrotic syndrome in a young male patient with RSTS. Case presentation: It is about a 27 year old young patient diagnosed since birth with a Rubinstein Taybi. In 1994, he was hospitalized in pediatrics for acute pyelonephritis. Retrograde urethrocystoscopy (RUC) showed bilateral vesicoureteral reflux (VUR) grade 3 with functional renal asymmetry on scintigraphy. The patient was admitted in nephrology department in 2019 for a pure and intense nephrotic syndrome with abrupt onset. The initial diagnosis retained was glomerular nephropathy particularly a FSGS secondary to his malformative uropathy. Subsequently, in view of the persistence of an intense nephrotic syndrome, the absence of VUR at the RUC, as well as the functional renal symmetry at the scintigraphy, it was decided to perform a renal biopsy. The biopsy showed an optically normal kidney with the presence of some meningial C3 deposits. MCD was then retained. The patient received corticosteroid therapy with a favorable clinical and biological response. Conclusion: Even if reflux nephropathy remains the most frequent cause of glomerulopathy secondary to malformative uropathy, we should not hesitate to perform a renal biopsy in front of an intense nephrotic syndrome with no functional renal impact of the VUR.

Vesicoscopic Cross-Trigonal Ureteral Reimplantation for Vesicoureteral Reflux: Intermediate Results.

For the treatment of vesicoureteral reflux, the introduction of vesicoscopic procedures offers new perspectives for improving patient comfort and quality. Our aim was to examine whether minimally invasive vesicoscopic cross-trigonal ureteral reimplantation (VCUR) would meet expectations. Between 2012 and 2021, 99 girls and 35 boys with high-grade vesicoureteral reflux (VUR) underwent VCUR. For two boys, we failed to establish the pneumovesicum, leading to conversion to open surgery. The mean age was 4.5 years, ranging from 10 months to 18 years. VCUR was successfully performed in 132 patients, including 75 patients with bilateral VUR and 12 children with double ureters with unilateral or bilateral VUR, corresponding to a total of 229 operated ureters. The mean time of operation was 151 min for all patients. There were no perioperative complications, with the exception of three cases of pneumoperitoneum without consequences. Postoperatively, we recognized three cases of acute hydronephrosis, two of them required transient drainage. Three patients developed extravasation of urine after the postoperative removal of the transurethral catheter, rapidly resolved by new drainage. In two patients, we combined VCUR with laparoscopic heminephrectomy and opposite laparoscopic nephrectomy, respectively. Overall, mean postoperative hospital stay was 4.2 days. We observed recurrent VUR in seven ureters, resulting in a success rate for VCUR of 96.9%. These results demonstrate the feasibility of VCUR and its potential to displace open surgery with high safety and wide applicability.