33rd ESPU Congress in Lisbon, Portugal

S19: PUV

Moderators: Anthony Khoury (USA), Seppo Taskinen (Finland)

ESPU Meeting on Friday 21, April 2023, 15:10 - 15:50


15:10 - 15:13
S19-1 (OP)

PREDICTING POSTNATAL RENAL FUNCTION OF PRENATALY DETECTED POSTERIOR URETHRAL VALVES USING THE DIFFUSION-WEIGHTED MAGNETIC RESONANCE (DW-MRI) IMAGING WITH APPARENT DIFFUSION COEFFICIENT (ADC) DETERMINATION.

Jessica PINOL 1, Nicoleta PANAIT 1, Thierry MERROT 1, Mirna HADDAD 1, Katia CHAUMOÎTRE 2 and Alice FAURE 1
1) Children Timone Hospital, Pediatric Surgery, Marseille, FRANCE - 2) APHM, Radiology, Marseille, FRANCE

PURPOSE

To evaluate the applicability of diffusion-weighted magnetic resonance imaging and ADC determination for predicting postnatal renal function in fetuses with suspected PUV.

MATERIAL AND METHODS

Medical files of the fetuses with prenatal diagnosis of PUV who were evaluated antenatally by MRI from 2003 to 2022 were reviewed retrospectively. Data from prenatal follow-up included gestational age at diagnosis, ultrasound renal evaluation, fetal biochemistry, the DW-MRI with ADC determination after MRI, and postnatal outcome and follow up of blood and urinary parameters were noted. Prenatal parameters studied were correlated to postnatal renal function. 

RESULTS

Sixteen fetuses were included. Two pregnancies were terminated based on MRI evaluation. Of the remaining, 14 fetuses borned, 4 children had normal blood creatinine levels at follow-up and 10 altered renal function at birth. DW-MRI with ADC determination of the 10 cases with renal impairment after birth showed abnormal value (180 to 230mm2s-1). For the other 4 cases with normal renal function, the DW-MRI and ADC determination were in the normal ranges (110 to 165 mm2s-1).

CONCLUSIONS

The DW-MRI and ADC value is an interesting method to evaluate the postnatal renal function in male with prenatally diagnosis of posterior urethral valves. 


15:13 - 15:16
S19-2 (OP)

THE IMPLICATIONS OF DECREASED RENAL RESERVE: A MULTI-INSTITUTIONAL EVALUATION OF SOLITARY FUNCTIONING KIDNEYS IN POSTERIOR URETHRAL VALVES

Jin Kyu KIM 1, Michael CHUA 1, Armando LORENZO 1, Joana DOS SANTOS 1, Juliane RICHTER 1, Priyank YADAV 1, Adree KHONDKER 1, Irene PARABOSCHI 2, Adele GIANNETTONI 2, Pankaj MISHRA 2, Joanna CLOTHIER 2, Mandy RICKARD 1 and Massimo GARRIBOLI 2
1) The Hospital for Sick Children, Urology, Toronto, CANADA - 2) Guy's and St Thomas' NHS Trust, Urology, London, UNITED KINGDOM

PURPOSE

Posterior urethral valves (PUV) is a common etiology of chronic kidney disease (CKD) in childhood. The significance of solitary functioning renal units in patients with PUV has not been well described. Hence, we aim to compare the outcomes of PUV patients with and without solitary kidneys.

MATERIAL AND METHODS

Databases of PUV patients who were assessed at a Canadian institution (between January 2000 to June 2022) and a United Kingdom (UK) institution (between February 1995 to October 2021) were identified. Those with findings of solitary functioning renal-unit (SRU), confirmed on nuclear scan imaging (defined as <10% differential function of a renal unit), were compared to those with two functioning renal units. Outcomes assessed included progression to renal replacement therapy (RRT).

RESULTS

A total of 357 patients with PUV were identified (195 Canada, 162 UK). There were differences in baseline characteristics between the two institutions with regards to time to ablation (median 56 days, IQR 14-313 vs. 34 days, IQR 15-119, p=0.017), renal replacement therapy (30/195 vs. 11/162, p=0.012), and creatinine nadir value (median 40, IQR 29-52 vs. 30, IQR 23-52, p<0.001). A combined analysis comparing 287 two functioning renal-units to 69 single functioning renal-unit (SRU) showed higher likelihood of RRT (26/287 vs. 15/69, p=0.005) and creatinine nadir values (median 34, IQR 24-52 vs. 45, IQR 30-82, p=0.026) in SRU. Multivariate analysis confirmed the likelihood of RRT to be higher with patients with SRU (OR 3.133, 95% CI 1.515-6.477). The same was true for creatinine nadir (unstandardized ß 22.655, 95% CI 7.287-38.024).

CONCLUSIONS

PUV patients with solitary kidneys are more likely have a higher creatinine nadir and likelihood of RRT, thus representing a vulnerable subgroup of patients.  


15:16 - 15:19
S19-3 (OP)

DOES THE PRESENCE OF PRESSURE "POP-OFFS" IMPACT VOIDING EFFICIENCY AND BLADDER OUTCOMES IN POSTERIOR URETHRAL VALVES?

Adree KHONDKER, Priyank YADAV, Jin Kyu KIM, Michael CHUA, Natasha BROWNRIGG, Juliane RICHTER, Joana DOS SANTOS, Armando LORENZO and Mandy RICKARD
The Hospital for Sick Children, Urology, Toronto, CANADA

PURPOSE

The protective role of pressure pop-offs, consisting of vesicoureteral reflux (VUR) and ipsilateral kidney dysplasia, in posterior urethral valve (PUV) is controversial. Here, we aimed to determine the role of pressure pop-offs on long-term bladder outcomes in PUV patients.

MATERIAL AND METHODS

A retrospective chart review was conducted for toilet-trained children with PUV managed at our institution between 2000 and 2022, only excluding cases without recorded uroflowmetry studies. Patients were stratified by VUR status and by the presence of VURD syndrome (high-grade VUR + ipsilateral kidney dysplasia). Outcomes included uroflowmetry parameters and initiation of clean-intermittent catheterization (CIC).

RESULTS

We identified a total of 101 patients who met study inclusion criteria, with a median follow-up of 114 months (IQR 67, 169). The median age of first and last uroflowmetry was 57 months (IQR 48, 82) and 120 months (IQR 89, 160), respectively. Patients with pressure pop-offs had similar flow velocity, post-void residuals, and bladder voiding efficiency to other PUV patients at last follow-up uroflowmetry. On survival analysis, patients with pressure pop-offs had no significant difference in risk of requiring CIC compared to patients without pop-offs (HR 1.92, p=0.06). Kidney dysplasia was independently associated with increased risk of CIC (HR 3.16, p=0.02) while VUR was not (HR 1.85, p=0.07) 

CONCLUSIONS

We show that boys with pressure pop-offs are not at higher risk of poorer voiding and intermittent catheterization than others with PUV. By extension, VURD syndrome does not confer protection against poorer bladder function.


15:19 - 15:30
Discussion
 

15:30 - 15:33
S19-4 (OP)

TOILET TRAINING ACHIEVEMENTS IN CHILDREN WITH PUV

Adele GIANNETTONI 1, Michela MARINARO 1, Irene PARABOSCHI 1, Pankaj MISHRA 1, Joanna CLOTHIER 1 and Massimo GARRIBOLI 2
1) Evelina London Children's Hospital, Paediatric Nephro-Urology, London, UNITED KINGDOM - 2) Evelina London Children's Hospital - Guy's and St Thomas NHS Foundation Trust, Paediatric Urology, London, UNITED KINGDOM

PURPOSE

Posterior Urethral Valves (PUV) are often associated with bladder and bowel disfunction. We aimed to explore characteristics, timing and influencing factors for continence achievement in a cohort of boys born with PUV

MATERIAL AND METHODS

Retrospective analysis of toilet training in PUV children looked after in our institution. Age at day- and night-time continence was recorded as well as presence of urinary accidents, need for bladder medications, clean intermittent catheterization(CIC) and urinary diversion. Faecal continence and need for bowel medication were also recorded.

Day- and night-time urinary continence was compared in children with(Group A) and without(Group B) associated cognitive comorbidities.

RESULTS

280 children were evaluated(valve ablation performed at median age of 87(IQR: 18-512) days).

Toilet training was completed by 78% of children at median age:36 (IQR: 30-48) months, during the day and by 70% at median age:40.5 (IQR: 30-59) months at night.

Episodes of urinary incontinence were reported by 21.5% of children toilet trained during the day(n=39 small leaks; 4 large leaks) and by 17.7% of children continent at night(n=31 small leaks; n=2 large leaks).

Seventy(25%) children were on bladder medication (n=63 antimuscarinic, n=8 alpha-blockers) and 53(18.9%) on CIC. Urinary diversion was performed in 17:11 ureterostomies and 6 vesicostomies.

Seventy-three(26.1%) children suffered from constipation and 64 required bowel medications.

Fifty-three(18.9%) presented associated comorbidities: n=9(3.2%) Autism Spectrum Disorder (ASD); n=8(2.9%) Attention Deficit Hyperactivity Disorder (ADHD); n=15(5.4%) Trisomy 21; n=21(7.5%) specified cognitive difficulties.

Children with associated comorbidities reached day- and night-time continence at older age: Group A: 46(38-72) months vs Group B: 36(30-45) months; p-value<0.0001 and Group A: 53(IQR: 36-72) months vs Group B: 38.5(IQR: 30-52) months; p-value<0.01, respectively.

CONCLUSIONS

Despite a quarter of the cohort require bladder and/or bowel medication, children born with PUV achieve continence at similar age compared to their peers.

The presence of cognitive comorbidities influences late acquisition of continence


15:33 - 15:36
S19-5 (OP)

POST-PUBERTAL DETRUSOR MYOGENIC FAILURE IS RARE AMONG A LARGE COHORT OF PATIENTS WITH PERINATALLY-DIAGNOSED POSTERIOR URETHRAL VALVES

Andrew GABRIELSON 1, Logan GALANSKY 1, Edwin SMITH 2 and Charlotte WU 1
1) JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE, JAMES BUCHANAN BRADY UROLOGICAL INSTITUTE, Baltimore, USA - 2) EMORY UNIVERSITY SCHOOL OF MEDICINE, UROLOGY, Atlanta, USA

PURPOSE

Reports suggest that patients with posterior urethral valves (PUV) often develop myogenic failure (MF) after puberty. However, the prevalence of MF has not been established. If MF is a common outcome, we hypothesize differential utilization of antispasmodic medications and rates of CIC between pre-pubertal and post-pubertal males.

PATIENTS AND METHODS

A retrospective cohort study was conducted using TriNetX from 2006-2022. Patients with PUV who underwent valve ablation within 1 year of life were stratified into pre-pubertal (<9y) and postpubertal (>13y) arms. Antispasmodic and alpha-blocker use, rates and time-to-first CIC or appendicovesicostomy, and detrusor underactivity/atony on urodynamics (UDS) were queried using ICD10 codes.

RESULTS

A total of 237 patients (180 pre-pubertal, 57 post-pubertal; median age 5 and 17 years) were included. Rates of detrusor underactivity/atony on UDS were low and comparable between groups (1.7% vs 0%, p=0.44). There was no difference in the utilization of CIC or appendicovesicostomy (15.0% vs 7.0%, p=0.18). Pre-pubertal patients were significantly more likely to be prescribed antispasmodics (44.4% vs 15.8%, p<0.001) but not alpha-blockers (12.2% vs 10.5%, p=0.31) compared to post-pubertal patients.

Pre-Pubertal (<9y)

n=180

Post-Pubertal (>13y)

n=57

P-value

Oxybutynin/Mirabegron Use

80(44.4%)

9(15.8%)

<0.001

Alpha-Blocker Use

12(12.2%)

6(10.5%)

0.31

CIC or Appendicovesicostomy

27(15%)

4(7.0%)

0.18

Detrusor Underactivity/Atony on UDS

3(1.7%)

0(0%)

0.44

CONCLUSIONS

In this contemporary cohort of PUV patients, there was no increased use of CIC or appendicovesicostomy once patients reached adolescence. More pre-pubertal patients were managed with antispasmodics which may reflect a greater incidence of detrusor overactivity in young children or a shift in practice favoring earlier aggressive medical treatment. These data suggest that progression to MF is rare, contrary to historical reports.


15:36 - 15:40
S19-6 (LOP)

★ ANTI-FIBROTIC EFFECT OF TOCOTRIENOLS FOR BLADDER DYSFUNCTION DUE TO PARTIAL BLADDER OUTLET OBSTRUCTION

Nao IGUCHI 1, M. İrfan DÖNMEZ 2, Anna MALYKHINA 1 and Duncan WILCOX 3
1) University of Colorado Denver, Surgery, Aurora, USA - 2) İstanbul University, Urology, İstanbul, TURKEY - 3) Children's Hospital Colorado, Surgery, Aurora, USA

PURPOSE

The aim of this study was to investigate potential beneficial effects of tocotrienols which have been suggested to inhibit Hypoxia inducible factor (HIF) pathway, on partial bladder outlet obstruction (PBOO)-induced bladder pathology.

MATERIAL AND METHODS

PBOO was surgically created in juvenile male mice. Sham-operated mice served as the control. Each group of animals received daily oral administration of either tocotrienols (T3) or soybean oil (SBO, vehicle) from day 0 to 13 post-surgery. Bladder function was examinedin vivo by void spot assay. At 2 weeks post-surgery, the bladders were subjected to 1) physiological evaluation of detrusor contractilityin vitro using bladder strips, 2) histology by H&E staining and collagen imaging, and 3) gene expression analyses by qPCR.

RESULTS

A significant increase in the number of small voids was observed after 1 week of PBOO compared to the control groups. At 2 weeks post-surgery, PBOO+SBO mice showed a further increase in the number of small voids, which was not observed in PBOO+T3 group. PBOO-induced decrease in detrusor contractility was similar between two treatments. PBOO induced bladder hypertrophy to the same degree in both SBO and T3 treatment groups, however, fibrosis in the bladder was significantly less prominent in the T3 group than the SBO group (1.8- vs. 3.0-fold increase in collagen content compared to the control). Enhanced levels of HIF target genes in the bladders were observed in PBOO+SBO group, but not in PBOO+T3 group compared to the control.

CONCLUSIONS

Oral tocotrienol treatment reduced the progression of urinary frequency and bladder fibrosis by suppressing HIF pathways triggered by PBOO.


15:40 - 15:50
Discussion