ESPU Meeting on Friday 10, June 2022, 15:00 - 16:10
15:00 - 15:03
S15-1 (OP)
Waseem ABOULELA 1 and Ahmed ABDEL LATIF 2
1) Kasr al Ainy Cairo University, Pediatric urology department aboul Reech hospital, Cairo, EGYPT - 2) Beni suef university, Urology, Beni Suef, EGYPT
PURPOSE
To detect whether the use of alpha blockers in posterior urethral valve (PUV)pediatric patients after valve ablation will improve the persistent obstructive symptoms despite the absence of obstruction and if there is associated side effects of its use.
MATERIAL AND METHODS
A prospective ,single blinded randomized study was conducted at Kasr Al Ainy pediatric hospital (Aboulreesh), Urology Department, Cairo University on 50 male children between September 2019 and June 2021 with PUV. All children were treated by endoscopic ablation of PUV using cold knife and were followed clinically for voiding symptoms and with ultrasonography and laboratory tests. All patients underwent 2nd look cystoscopy one month after primary valve ablation to see residual valves as a routine procedure confirming no remnant of valve and still complaining of obstructive symptoms. They were divided in to 2 equal groups 25 patients each. Group A were given alpha blockers and group B placebo for 1 month. patients with remnant valve or abnormal ultrasound or laboratory tests were excluded from the study. urinary flow was assessed by uroflowmetry in patients who were old enough and continent to urine and by history from the parents in children who were not continent to urine . both drugs were given as single daily dose at night before sleep and recording of voiding dairy is required from the parents.
RESULTS
Marked improvement of obstructive symptoms in group A reaching about 90% (21 patients) while no mentioned improvement in group B was noticed . no side effects of both medication the alpha blocker and the placebo during its use.
CONCLUSIONS
The use of alpha blockers improve the obstructive symptoms in pediatric patients with PUV after valve ablation and in absence of any further urethral obstruction with no side effects noticed during the period of its use.
15:03 - 15:06
S15-2 (OP)
Jin Kyu (Justin) KIM 1, Adree KHONDKER 2, Dheidan ALSHAMMARI 2, Michael CHUA 3, Joana DOS SANTOS 3, Natasha BROWNRIGG 3, John WEAVER 4, Gregory TASIAN 4, Armando LORENZO 3 and Mandy RICKARD 3
1) University of Toronto, Urology, Toronto, CANADA - 2) Hospital for Sick Children, Urology, Toronto, CANADA - 3) The Hospital for Sick Children, Urology, Toronto, CANADA - 4) The Children's Hospital of Philadelphia, Urology, Philadelphia, USA
PURPOSE
We aim to assess whether tamsulosin facilitates efficient emptying of the lower urinary tract in posterior urethral valves (PUV) patients, thus mitigating the likelihood of progressing to clean intermittent catheterization or urinary diversion (CIC/UD).
MATERIAL AND METHODS
An institutional PUV database from 2000-2022 was reviewed. Outcomes of interest were progression to diversion in infants or CIC for older children. Following assessment of baseline characteristics, Kaplan-Meier survival curves (KMSC) and log-rank test were generated to assess differences in progression to CIC/UD between those who received tamsulosin and did not. Additional analyses were generated based on differences in baseline characteristics.
RESULTS
A total of 153 patients were included. Those who progressed to CIC/UD had lower baseline eGFR at 1-year (Table 1). Patients on tamsulosin were more likely to progress to CIC/UD vs. those who were not (40.9%, 18/44 vs. 21.9%, 23/105; p=0.005). KMSC confirmed this finding (log-rank p=0.011). KMSC stratified by chronic kidney disease (CKD) stage at 1-year showed a clear split of curves, demonstrating that worse CKD is associated with progression to CIC/UD (log-rank p=0.001). A Cox-regression controlling for CKD stage at 1-year showed that the effect of tamsulosin on CIC/UD did not reach statistical significance (p=0.058) while CKD stages remained strong predictors of progression to CIC/UD (p=0.006).
CONCLUSIONS
In our series, tamsulosin did not significantly impact progression to CIC/UD in children with PUV. Children with worse CKD at 1-year are more likely to require CIC/UD. Although tamsulosin may have other beneficial effects on the lower urinary tract, its value for preventing CIC/UD appears to be marginal.
15:06 - 15:09
S15-3 (OP)
Adree KHONDKER 1, Justin CHAN 1, Jin Kyu KIM 1, Michael CHUA 1, Brittney HENDERSON 2, Priyank YADAV 1, Joana DOS SANTOS 1, Gregory TASIAN 2, Mandy RICKARD 1 and Armando LORENZO 1
1) The Hospital for Sick Children, Division of Urology, Toronto, CANADA - 2) Children's Hospital of Philadelphia, Division of Urology, Philadelphia, USA
PURPOSE
The literature regarding the ideal initial surgical management of posterior urethral valves (PUV) patients is conflicted. Here, we aimed to determine long-term kidney and bladder outcomes in boys with PUV undergoing either primary valve ablation or urinary diversion.
MATERIAL AND METHODS
A systematic search was performed in March 2021. Comparative studies were evaluated according to Cochrane collaboration recommendations. Assessed measures included kidney outcomes (chronic kidney disease stage, kidney function) and bladder outcomes, among others. Odds ratios (OR) and mean difference with 95% confidence interval (CI) were extrapolated from available data. Random-effects meta-analyses were performed and confounders were assessed with subgroup analysis.
RESULTS
Thirty studies describing 1547 boys with PUV were included in this synthesis. Overall effect estimates demonstrate that patients undergoing primary diversion have significantly increased odds of developing kidney insufficiency [OR 0.60, 95%CI 0.44-0.80; p<0.001]. However, in patients with equal or unclear baseline kidney function between interventions, there was no significant difference in follow-up kidney outcomes [p=0.09-0.35]. There was no significant difference in the odds of developing bladder dysfunction on urodynamic study or requiring clean-intermittent catheterization with primary ablation rather than diversion [OR 0.89, 95%CI 0.49-1.59; p=0.68].
CONCLUSIONS
Current low-quality evidence suggests that long-term kidney and bladder outcomes are similar between primary ablation and primary diversion after adjusting for baseline kidney function. As patients managed with a diversion likely represent a more severe form of the disease, it remains possible that these patients derive benefit over primary ablation. Further research with adequate covariate control is warranted to investigate sources of heterogeneity and determine the best individualized approach. Our data also suggests that diversion does not lead to worse bladder function compared to ablation.
15:09 - 15:12
S15-4 (OP)
Priyank YADAV 1, Mandy RICKARD 1, John WEAVER 2, Antoine Fermin SELMAN 2, Michael CHUA 1, Jin Kyu KIM 1, Adree KHONDKER 1, Karen MILFORD 1, Daniel KEEFE 1, Marisol LOLAS 1, Joana DOS SANTOS 1, Lauren ERDMAN 3, Marta SKRETA 3, Eran ASHWAL 4, Bernarda VITERI 5, Greg RYAN 4, Gregory TASIAN 2 and Armando LORENZO 1
1) The Hospital for Sick Children, Urology, Toronto, CANADA - 2) Children's Hospital of Philadelphia, Urology, Philadelphia, USA - 3) The Hospital for Sick Children, Center for Computational Medicine, Toronto, CANADA - 4) Ontario Fetal Center, Obstetrics and Gynaecology, Toronto, CANADA - 5) Children's Hospital of Philadelphia, Nephrology, Philadelphia, USA
PURPOSE
The objective of this study was to compare the outcomes of prenatally versus postnatally diagnosed posterior urethral valves (PUV) by review of the literature and institutional databases of two large pediatric centres in North America.
MATERIAL AND METHODS
We conducted a systematic review and meta-analysis in April 2021 (PROSPERO ID - CRD42021243546) and included all studies that reported outcomes of prenatally and postnatally diagnosed PUV. In addition, all patients with PUV were identified at 2 large pediatric institutions in North America between 2000-2020. Baseline characteristics and outcome measures were compared between those who were diagnosed prenatally vs. postnatally. Outcomes of interest included progression of chronic kidney disease (CKD), the need for renal replacement therapy (RRT), and bladder dysfunction as determined by initiation of clean intermittent catheterization (CIC).
RESULTS
The systematic review included 47 studies (3208 patients). The pooled effect estimates for progression to CKD3 or higher (OR 1.06 [95% CI 0.45, 2.47]) and the need for RRT (OR 1.39 [95% CI 0.64, 2.99]) between prenatal and postnatal groups was not significantly different. Similarly, no significant difference was noted with recurrent/breakthrough urinary tract infections (UTIs, p=0.68). Most studies had a moderate or serious risk of bias. In the institutional data, the first institution contributed 152 patients of which 39% were prenatally detected. There was no difference between prenatal and postnatal groups in the proportion of patients who required RRT or progressed to CKD stage ≥3. The time to RRT was significantly sooner for prenatally detected PUV. The second institution contributed 216 patients, of which 71% were prenatally diagnosed. Significantly more prenatally-diagnosed patients required RRT, however there was no difference in the age this outcome was reached.
CONCLUSIONS
The findings suggest that time of diagnosis is not a strong predictor of outcomes. Instead, management optimisation may delay long-term sequelae.
15:12 - 15:15
S15-5 (OP)
Michael CHUA 1, Jin Kyu (Justin) KIM 2, Tim VAN MIEGHEM 3, Joana DOS SANTOS 1, Eric MACKAY 1, Lauren ERDMAN 1, Marta SKRETA 1, Daniel KEEFE 1, Natasha BROWNRIGG 1, Armando LORENZO 1 and Mandy RICKARD 1
1) The Hospital for Sick Children, Urology, Toronto, CANADA - 2) University of Toronto, Urology, Toronto, CANADA - 3) Mount Sinai Hospital, Maternal Fetal Medicine, Toronto, CANADA
PURPOSE
We aim to configure a nomogram predicting post-natal diagnosis of posterior urethral valves (PUV) using antenatal ultrasound findings and to validate the diagnostic accuracy of the nomogram in comparison to the traditional keyhole sign (KHS).
MATERIAL AND METHODS
Bayesian meta-regression analysis identified that antenatal ultrasound findings that were highly predictive of PUV were: oligohydramnios, bilateral hydronephrosis, bilateral ureteral dilatation, megacystis, bladder thickening, and urinoma. A nomogram based on these findings was configured as an electronic calculator with the baseline 6% PUV incidence among male fetus with moderate-severe hydronephrosis. The validation of the nomogram was based on data from an institutional prospective database that includes cases referred to our antenatal clinic between January 2020-2022.
RESULTS
Based on 72 male infants with moderate-severe hydronephrosis, the KHS had a sensitivity and specificity of 44.44% (95% CI 13.7-78.8) and 96.83% (95% CI 89.0- 99.61%). The nomogram had sensitivity and specificity of of 100 (95% CI 66.37-100%) and 96.83 (5% CI 89.0- 99.61%). The KHS led to 5 false negatives, while nomogram had 2 false positives. The nomogram had a superior AUC on ROC curve compared to KHS (AUC: 0.976 vs 0.714). Youdin's index suggested a cut-off of 85% probability minimize false positives. The calculated number needed to predict was 2.3 for the KHS and 1.5 for the nomogram.
CONCLUSIONS
Given the long-term implication of missing a PUV diagnosis, our nomogram, which has a superior diagnostic accuracy compared to KHS, can be a valuable adjunctive diagnostic tool to trigger additional post-natal screening for male fetuses with hydronephrosis.
15:15 - 15:18
S15-6 (OP)
Irene PARABOSCHI 1, Adele GIANNETTONI 2, Pankaj MISHRA 3, Joanna CLOTHIER 4 and Massimo GARRIBOLI 5
1) Fundation IRCCS Cà Granda Policlinico di Milano, Paediatric Urology, Milano, ITALY - 2) Evelina London Children's Hospital, London, UK, Paediatric Urology, London, UNITED KINGDOM - 3) Evelina London Children's Hospital, London, United Kingdom, Paediatric Urology, London, UNITED KINGDOM - 4) Evelina London Children's Hospital, Department of Paediatric Nephro-urology, London, UNITED KINGDOM - 5) Evelina London Children's Hospital, Paediatric urology, London, UNITED KINGDOM
PURPOSE
Role of VURD syndrome in boys with PUV is controverse. Recent studies suggest a detrimental rather than protective role to renal function but none evaluated the children longitudinally. We aim to present a longitudinal case-control study.
MATERIAL AND METHODS
We retrospectively reviewed our cohort of PUV children who underwent valve resection under the age of 2 years. VURD syndrome was defined as unilateral loss of kidney function (<15%) associated with ipsilateral Grade IV-V VUR (on initial DMSA and MCUG). We compared children with (Group A) and without (Group B) VURD syndrome analysing the difference in i) nadir serum creatinine during the first year after valve resection ii) GFR between 10 and 5 years of age (ΔGFR=GFR10 years -GFR5 years).
RESULTS
115 patients met the inclusion criteria and had all data available; 15 (13%) presented with VURD syndrome. Median age at valve ablation was 41 (IQR: 27-228) days in Group A and 28.5 (IQR: 15-69.5) in Group B. Median serum nadir creatinine in Group A (25 μmol/L; IQR: 22-37) and B (26 μmol/L; IQR: 21.5-32.5) were not statistically different (p-value=0.990).
In 40 GFR at 5 and 10 years was available; six (15%) had VURD syndrome. The median ΔGFR was -13.1 (IQR:-21.7;-3.1) mL/min/1.73m2 in Group A and 1.8 (IQR: -15.4;10.9) mL/min/1.73m2 in Group B (p-value=0.1074).
CONCLUSIONS
Despite a trend of worsen values in long-term renal function, on a longitudinal evaluation, VURD syndrome does not seem to play a role neither on early renal function development nor in the renal deterioration in childhood.
15:38 - 15:41
S15-7 (OP)
Luke HARPER 1, Nathalie BOTTO 2, Matthieu PEYCELON 3, Jean-Luc MICHEL 4, Marc-David LECLAIR 5, Sarah GARNIER 6, Pauline CLERMIDI 7, Alexis ARNAUD 8, Anne DARIEL 9, Eric DOBREMEZ 10, Alice FAURE 9, Laurent FOURCADE 11, Nadia BOUDAOUD 12, Yann CHAUSSY 13, Fideline COLLIN 4, Laetitia HUIART 4, Cyril FERDYNUS 14, Valery BOCQUET 4 and Frederique SAUVAT 4
1) CHU Pellegrin-Enfants, Pediatric Surgery, Bordeaux, FRANCE - 2) Necker Enfants-Malades, Paris, FRANCE - 3) Hopital Robert Debre, Paris, FRANCE - 4) CHU F Guyon, Saint-Denis De La Réunion, FRANCE - 5) CHU Nantes, Nantes, FRANCE - 6) CHU Montpellier, Montpellier, FRANCE - 7) Hopital armand trousseau, Paris, FRANCE - 8) CHU Rennes, Rennes, FRANCE - 9) CHU Marseille, Marseille, FRANCE - 10) CHU Pellegrin-Enfants, Bordeaux, FRANCE - 11) CHU Limoges, Limoges, FRANCE - 12) CHU Reims, Reims, FRANCE - 13) CHU besançon, Besançon, FRANCE - 14) CHU La Réunion, Saint-Denis, FRANCE
PURPOSE
Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors for fUTI could allow for tailoring UTI prevention.
We performed a secondary analysis of the data from the CIRCUP RCT to specifically identify patient characteristics associated with a higher risk of fUTI.
MATERIAL AND METHODS
We analyzed the data of the control group of the CIRCUP randomized trial: children with PUV, not circumcised but put on antibiotic prophylaxis and followed prospectively for two years. We compared age, size and weight at birth, presence of VUR at diagnosis, presence of dysplasia on DMSA scan at 2 months of life, and nadir creatinine between children who presented a fUTI and those who did not. We also looked at age of presentation of the first episode of fUTI.
RESULTS
The study group consisted of 42 patients of which 17 presented at least one fUTI. There was a statistically significant effect of the presence of VUR on the presence of febrile urinary tract infections (p=0.03) with an odds ratio of 5.57 (CI95% = [1.13-27.52]). We also observed three distinct time periods for risk of presenting a fUTI with a first decrease in infection rate after the first 40 days of life, followed by a second decrease at 240 days of life.
CONCLUSIONS
Antibiotic prophylaxis for fUTI prevention in boys with PUV could be tailored to circumcision status and presence of high-grade VUR. When given, antibiotic prophylaxis is probably most useful for the first 9 months of life.
15:41 - 15:44
S15-8 (OP)
Nora HANEY 1, Tamir SHOLKLAPPER 2, Chad CRIGGER 2, Angelica GRIGGS-DEMMIN 2, Shwetha MUDALEGUNDI 2 and Charlotte WU 2
1) Johns Hopkins Hospital, Urology, Baltimore, USA - 2) Johns Hopkins Hospital, Pediatric Urology, Baltimore, USA
PURPOSE
Urinary drainage for posterior urethral valves can be achieved with valve ablation (VA) or diversion in the form of vesicostomy (VES) or cutaneous ureterostomy (CU). The effect of these interventions on long-term bladder function remains debated, and voiding symptomatology after VES or CU reversal has been poorly characterized. Herein, we examine prevalence and scope of retention or incontinence symptomatology among PUV patients undergoing these interventions and determine rates of progression to augmentation.
MATERIAL AND METHODS
This is a single-institution retrospective cohort study. Retention Scores (R) were calculated 1 point for: retention behavior (double/timed void), alpha-blocker, intermittent catheterization, or overnight foley. Incontinence Scores (I) were calculated 1 point for: incontinence behavior (double/timed void), oral medication, or botox. Patients with R score above 3 or I score above 2 were deemed to have severe retention or incontinence symptomatology respectively. End stage bladder (ESB) was defined as need for bladder augmentation.
RESULTS
We identified 77 patients between 5-40 years old with median follow-up of 176 (54-485) months. Overall progression to ESB occurred in 11.8% of patients at a median age of 4.8y after diagnosis. There was no difference in the rates of severe retention or incontinence symptomatology between VA versus VES/CU (Table 1). There was also no significant difference in rate of ESB by intervention category VA (9.4%) versus VES/CU (17.4%; p=0.441).
| # Patients (n=76) | VA (n=53) | VES/CU (n=23) | p | |
| Severe Retention (R Score 3+) | 11 (14.4%) | 7 (13.2%) | 4 (17.4%) | 0.726 |
| Severe Incontinence (I Score 2+) | 6 (7.9%) | 5 (9.4%) | 1 (4.3%) | 0.661 |
| ESB | 9 (11.8%) | 5 (9.4%) | 4 (17.4%) | 0.441 |
CONCLUSIONS
Long term bladder outcomes for valve patients demonstrated similar voiding symptomatology and progression to end-stage bladder regardless of diversion status. Patients went on to ESB approximately 5 years after diagnosis at similar rates between groups.
15:44 - 15:47
S15-9 (OP)
Kamranbay GASIMOV 1, Muhammet Irfan DONMEZ 2, Zuhal BAYRAMOGLU 3, Vasileios TATANIS 4, Bilal CETIN 2, Muhammet Ali COLAK 5, Tayfun OKTAR 2 and Hasan Orhan ZIYLAN 2
1) Hacettepe University, Pediatric Urology, Ankara, TURKEY - 2) Istanbul University, Urology Department, Pediatric Urology Division, Istanbul, TURKEY - 3) Istanbul University, Radiology Department, Pediatric Urology Division, Istanbul, TURKEY - 4) Hacettepe University, Urology Department, Pediatric Urology Division, Ankara, TURKEY - 5) Hacettepe University, Urology Department, Ankara, TURKEY
PURPOSE
The aim of this study is to reveal bladder dysfunction non-invasively in patients operated for posterior urethral valve (PUV). For this purpose, findings obtained from shear wave elastography (SWE) were compared with urodynamic findings simultaneously.
MATERIAL AND METHODS
Bladder functions of the patients operated for PUV were examined with urodynamic study (UDS) and compared with SWE findings. Patients with vesicoureteral reflux were excluded from the study. The anticholinergic treatment that the patients had previously used was discontinued for 2 weeks.
The primary target parameters were detrusor pressure, bladder pressure, and to determine the correlation between bladder compliance and shear wave speed (SWS). Apart from that, upper system imaging, bladder capacity measurement and postvoid residual urine amount were recorded.
RESULTS
Twenty-eight patients who underwent endoscopic posterior urethral valve ablation between 2006 and 2018 were included into this study. A statistically significant correlation was found between vesical pressure (Pves) and SWS at 25%, 50%, 75%, 100% occupancy, while detrusor pressure (Pdet) was correlated with SWS at 75%, 100% occupancy. A statistically significant difference was found between the compliance <20 ml/cmH2O and ≥20 ml/cmH2O groups in the SWS measurement values of the anterior wall of the empty bladder. SWS values were higher in the group with compliance lower than 20 ml/cmH2O.
A linear regression model was created to estimate Pdet and Pves based on SWS values.
100% Pdet = (SWS x 1.104) - 12.798
50% Pves = (SWS x 0.775) + 4.027
CONCLUSIONS
In the results of the study, the parameters required in the bladder dysfunction follow-up of PUV patients were mostly predicted by shear wave elastography method. In order to replace the traditional method of urodynamics, studies with larger patient population and patients with different pathologies are needed.
15:47 - 15:50
S15-10 (OP)
Luke HARPER 1, Nathalie BOTTO 2, Matthieu PEYCELON 3, Jean-Luc MICHEL 4, Marc-David LECLAIR 5, Sarah GARNIER 6, Pauline CLERMIDI 7, Alexis ARNAUD 8, Anne DARIEL 9, Eric DOBREMEZ 10, Alice FAURE 9, Laurent FOURCADE 11, Nadia BOUDAOUD 12, Yann CHAUSSY 13, Laetitia HUIART 4, Cyril FERDYNUS 4, Valery BOCQUET 4 and Frederique SAUVAT 4
1) CHU Pellegrin-Enfants, Pediatric Surgery, Bordeaux, FRANCE - 2) Necker Enfants-Malades, Paris, FRANCE - 3) Hopital Robert Debre, Paris, FRANCE - 4) CHU F Guyon, Saint-Denis De La Réunion, FRANCE - 5) CHU Nantes, Nantes, FRANCE - 6) CHU Montpellier, Montpellier, FRANCE - 7) Hopital armand trousseau, Paris, FRANCE - 8) CHU Rennes, Rennes, FRANCE - 9) CHU Marseille, Marseille, FRANCE - 10) CHU Pellegrin-Enfants, Bordeaux, FRANCE - 11) CHU Limoges, Limoges, FRANCE - 12) CHU Reims, Reims, FRANCE - 13) CHU besançon, Besançon, FRANCE
PURPOSE
Posterior urethral valves (PUV) represent the most common cause of Lower Urinary Tract Obstruction (LUTO) in boys and a common cause of chronic kidney disease. Thus, having a child with PUV can significantly impact family health-related quality of life (HRQL). We decided to assess the impact of having a boy with PUV on the family unit by using the Impact on Family Scale (IFS).
MATERIAL AND METHODS
The IFUP study was added to the CIRCUP trial in 2015. We used the validated French version of the IFS developed by Stein et al. which was given to 37 families of boys with PUV. We received the answers of both parents in 30 cases, just the mother in 6 and just the father in 1.
RESULTS
67 parents answered the questionnaire (29 men and 38 women). The median score was 53.0 (45.0-58.0).
There were differences in response between parents: 40% of men and 30% of women stated that their activities were limited by the child's disease. 10% of women and 30% of men stated that the child's disease had influenced their income. 25% of couples split up during the study period.
Presence of fUTIs did not influence global score. Parents' state of health and loss of revenue impact their HRQL more than the severity of the child's disease.
CONCLUSIONS
Having a child with PUV has a significant impact on families, on their health, income, and relationship. This information should be shared during counselling and addressed by healthcare systems.
15:50 - 15:53
S15-11 (OP)
Alice XIANG, John WEAVER, Iqra NADEEM, Neeta D'SOUZA, Many RICKARD, Gregory TASIAN, Dana WEISS, Karen MILFORD, Armando LORENZO and Christopher LONG
Children's Hospital of Philadelphia, Pediatric Urology, Philadelphia, USA
PURPOSE
Posterior urethral valves (PUV) occur in patients with Trisomy 21 at a rate of 3-4%; far higher than the general population. Our genetic understanding of PUVs and Down Syndrome (DS) is in its infancy, with the majority of literature consisting of case reports. In this study, we present the largest known series of DS patients with PUVs. We hypothesized patients with DS and PUVs would have worse renal outcomes.
MATERIAL AND METHODS
We queried our prospectively managed multi-institutional database of PUV patients from 1990 to 2021. Out of 566 patients with PUV, we identified 18 patients with a concomitant diagnosis of DS. In addition, we performed a systematic review of case reports describing the presentation of children with PUV and DS. The demographics, renal function outcomes, voiding habits, surgical interventions, and radiology images were aggregated and analyzed.
RESULTS
We found that DS patients underwent initial valve ablation or diversion at a similar age to the overall cohort (Table 1). DS patients also had a similar nadir creatinine at 1 year of life and a similar rate of renal failure when compared to the overall cohort. However, we found that only 11 (36.1%) patients with DS were able to void volitionally, compared to 458 (83%) of PUV patients in the general cohort, P-value 0.014. This difference was strengthened when we included patients with DS and PUV from the literature in our analysis, P-value 2.6x10-10.
CONCLUSIONS
While patients with Trisomy 21 and PUV have similar renal outcomes to other PUV patients in terms of renal function and progression to renal failure, they have a significantly lower chance of voiding volitionally with continence. Medical comorbidities and behavioral factors likely contribute to this phenomena. Physicians should be aware of this when treating this population and potentially have a lower threshold to proceed with a catheterizable channel in these patients.
15:50 - 15:53
S15-12 (OP)
Alice XIANG, John WEAVER, Iqra NADEEM, Neeta D'SOUZA, Mandy RICKARD, Gregory TASIAN, Dana WEISS, Karen MILFORD, Armando LORENZO and Christopher LONG
Children's Hospital of Philadelphia, Pediatric Urology, Philadelphia, USA
PURPOSE
Posterior urethral valves (PUV) occur in patients with Trisomy 21 at a rate of 3-4%; far higher than the general population. Our genetic understanding of PUVs and Down Syndrome (DS) is in its infancy, with the majority of literature consisting of case reports. In this study, we present the largest known series of DS patients with PUVs. We hypothesized patients with DS and PUVs would have worse renal outcomes.
MATERIAL AND METHODS
We queried our multi-institutional database of PUV patients from 1990 to 2021. Out of 566 patients with PUV, we identified 18 patients with a concomitant diagnosis of DS. In addition, we performed a systematic review of case reports describing the presentation of children with PUV and DS. The demographics, renal function outcomes, voiding habits, surgical interventions, and radiology images were aggregated and analyzed.
RESULTS
DS patients had a similar age of diagnosis, nadir creatinine, and renal failure rate compared to the overall cohort. Only 36.1% DS patients could void volitionally,compared to 83% in the general cohort, P-value 0.014.
|
Table 1. PUV Presentation in Trisomy 21 |
PUV and DS n = 18 |
Total PUV cohort n = 548 |
P-value |
|
Age at initial ablation or diversion (median, IQR) |
1142.5 (774) |
419.7 (869) |
0.146 |
|
Cr nadir at 1 yr of life (median, IQR) |
0.45 (0.3) |
0.4 (0.4) |
0.442 |
|
ESRD on Dialysis or require renal transplant |
2 (11.1%) |
50 (9.1%) |
0.823 |
|
Voiding without assistance |
11 (36.1%) |
458 (83%) |
0.014 |
|
Average Follow up (IQR) |
6.97 yrs (1-13) |
6.77 (2-12) |
0.921 |
CONCLUSIONS
While patients with Trisomy 21 and PUV have similar renal outcomes to other PUV patients in terms of renal function and progression to renal failure, they have a significantly lower chance of voiding volitionally with continence. Medical comorbidities and behavioral factors likely contribute to this phenomena. Physicians can consider a lower threshold to proceed with a catheterizable channel in these patients.