30th ESPU Congress - Lyon, France - 2019

S11: LOWER URINARY TRACT

Moderators: Alaa El Ghoneimi (France), Alex Turner (UK)

ESPU Meeting on Friday 26, April 2019, 08:10 - 09:08


08:10 - 08:15
S11-1 (LO)

EFFECT OF POP-OFF MECHANISMS ON BLADDER FUNCTION IN BOYS WITH POSTERIOR URETHRAL VALVES

Thomas DELEFORTRIE 1, Cyril FERDYNUS 2, Jean-Luc MICHEL 3, Eric DOBREMEZ 1, Alaa EL GHONEIMI 4, Annabel PAYE-JAOUEN 5 and Luke HARPER 1
1) CHU Pellegrin-Enfants, Pediatric Surgery, Bordeaux, FRANCE - 2) CHU F Guyon, USM, Saint-Denis, FRANCE - 3) CHU F Guyon, Pediatric Surgery, Saint-Denis, FRANCE - 4) Hopital robert debre, Pediatric surgery, Paris, FRANCE - 5) Hopital Robert Debré, Pediatric surgery, Paris, FRANCE

PURPOSE

We aimed to assess the effect of pop-off mechanisms on bladder function in boys with posterior urethral valves, as well as the relationship between renal and bladder function in these patients.

MATERIAL AND METHODS

In this retrospective study, we included all boys with PUV and a minimum 5 year follow-up, including renal and bladder function evaluation. Boys with PUV with and without pop-off mechanisms were divided into three severity groups for renal function according to nadir creatinine (NC), i.e. lowest creatinine during the first year following diagnosis, as well as three severity groups for bladder function as determined by clinical parameters and Uroflow. We compared bladder function between each group and also studied the relationship between bladder and renal function within the global PUV population.

RESULTS

We included 73 boys of which 22 had a pop-off mechanism. Average follow-up was 10 years. Fewer pop-off patients had severe bladder dysfunction as compared to the non-pop-off-group (13% vs 21%). As concerns the relationship between renal and bladder function, 41.4% of boys with NC < 35 had abnormal bladder function vs 47.4% of those with a NC between 35 and 75 μmol/L and 83.3% of boys with NC > 75 μmol/L (p<0.01).

CONCLUSIONS

In our series, an initial NC > 75 μmol/L was associated with a higher risk of long-term bladder dysfunction regardless of presence or absence of a pop-off mechanism.
Our results also suggest that pop-off mechanisms protect the bladder from severe dysfunction.


08:15 - 08:18
S11-2 (PP)

ASSOCIATED ANOMALIES IN POSTERIOR URETHRAL VALVES IN A NATIONAL COHORT

Ewan BROWNLEE 1, Ruth WRAGG 2, Andrew ROBB 2, Harish CHANDRAN 2, Marian KNIGHT 3 and Liam MCCARTHY 2
1) Birmingham Children's Hospital, Paediatric Urology, Birmingham, UNITED KINGDOM - 2) Birmingham Children's Hospital, Paediatric Urology, Birmingham, UNITED KINGDOM - 3) National Perinatal Epidemiology Unit, Oxford, UNITED KINGDOM

PURPOSE

What are the associated anomalies with posterior urethral valves (PUV)? is there any correlation with severity of renal disease? Are urinomas protective of renal function?

MATERIAL AND METHODS

A national audit (BAPS CASS) of referrals in the UK of boys diagnosed with PUV in a year was conducted. Details of abnormalities were correlated with plasma creatinine and renal dysplasia score (presence cortical thinning, reduced cortico-medullary differentiation, or cysts scored 1 each per renal unit (0-3)).
Data presented as number(%), median(interquartile range), analysed by Mann-Whitney U-test, p<0.05 taken as significant.
Ethics approval: NRES Committee South Central Oxford A (12/SC/0416)

RESULTS

Data collected October 2014 - September 2015, 25/26 centres submitted data (96%). 121 cases BOO; PUV 113(93%). Of PUV patients, 23(20%) had associated anomalies: 10 had urinary obstruction/oligohydramnios (group-1), 13 incidental (group-2), 90 isolated PUV (group-3). Group-1: Pulmonary hypoplasia(7), Talipes(3), hydrops(1). Three in group-1 also had Pyloric stenosis(2), cleft lip/palate, hand and foot contractures, craniosynostosis. In group-2 abnormalities included 4 CNS(IVH, Chiari Malformation, developmental delay), 3 GI(Hirschsprung's, Gastroschisis, GORD), 2 cardiac(PDA, pulmonary atresia), 3 syndromes(Downs, Alport's, Di George), 2 hypospadias, 1 each of Hypothyroidism, Hand/foot contractures, cleft lip/palate, polydactyly, hypothyroidism, idiopathic hypertriglyceridaemia. Urinomas were present in 9/113(8%) of PUV-boys.
The 3 abnormality groups had no difference in renal dysplasia, plasma creatinine.
Plasma creatinine for urinoma vs. non-urinoma PUV-boys was 24(21-33) micromol/l vs. 38(26-67) micromol/l, P<0.05. Dysplasia scores for urinoma vs non-urinoma boys were 2(0-3) vs. 1(0-1), P<0.01.

CONCLUSIONS

20% of PUV boys have another abnormality. Urinomas are protective of renal function, despite increased renal dysplasia.


08:18 - 08:21
S11-3 (PP)

POSTERIOR URETHRAL VALVES AND CONSANGUINITY

Gul NAWAZ and Ijaz HUSSAIN
SHIFA INTERNATIONAL HOSPITAL, UROLOGY, Islamabad, PAKISTAN

PURPOSE

Posterior urethral valve (PUV) is life-threatening congenital anomalies of the urinary tract which if not treated in time, results in vesicoureteric reflux, recurrent UTI, voiding dysfunction and renal insufficiency. Valves have occurred in siblings, twins, and in successive generations but its frequency and severity with consanguinity have never been studied. We aimed to determine the frequency and severity of PUV in the products of cousin marriage.

MATERIAL AND METHODS

We retrospectively reviewed the medical record of all 180 consecutive patients who underwent posterior urethral valve fulguration in last 9 years from Jan 2010 to April 2018. Diagnosis was made by voiding symptoms, ultrasonography and confirmed by voiding cystourethrogram (VCUG). History of first-degree cousin marriage and family history of PUV was specifically inquired in each case.

RESULTS

  Total 180 patients having mean age of 4.8 years (SD± 3.1) were treated for posterior urethral valve. Family history of first-degree cousin marriage was found in 39 (22%) patients. Furthermore, PUV was diagnosed in 15 (8.3%) male siblings of the child having family history of cousin marriage.

Parameter

Total number (%)

Hx of consanguinity

Antenatal diagnosed

48 (27%) (patients)

17 (35%)

Severe hydronephrosis

60 (26%) (units)

43(73%)

Bilateral hydronephrosis

50 (43%) (Patients)

17 (34%)

Bilateral Vesicoureteric reflux

60 (29.7%) units

19 (63%)

Chronic renal insufficiency

54 (30%) patients

19 (36%)

End stage renal failure

6 (3 %) patients

4 (66%)

CONCLUSIONS

Cousin Marriage is very common in this part of world. Significant number of patients with PUV has history of consanguinity. These patients present with more severe disease and early renal insufficiency. 


08:21 - 08:24
S11-4 (PP)

THE PREVALENCE OF VITAMIN D DEFICIENCY IN BOYS WITH POSTERIOR URETHRAL VALVES

Zeni HAVELIWALA 1, Giulia DEL RE 1, Massimo GARRIBOLI 2 and Joanna CLOTHIER 3
1) Evelina London, Paediatric Urology, London, UNITED KINGDOM - 2) Evelina London, London, UNITED KINGDOM - 3) Evelina London, Paediatric Nephrology, London, UNITED KINGDOM

PURPOSE

Vitamin D deficiency is widely prevalent in the western population and often severe in children with chronic kidney disease (CKD).  The high prevalence of CKD in posterior urethral valves (PUV) patients is concerning for the pleotropic and skeletal sequelae of vitamin D deficiency.  The aim of this study is to review the incidence of vitamin D deficiency in our cohort of patients.

MATERIAL AND METHODS

Single centre retrospective study of all patients followed up with PUV.  Collected all 25-hydroxy vitamin D levels measured over a 32 month period.  <30nmol/L defined as deficiency, 30-50nmol/L defined as insufficiency and > 50nmol/L considered sufficient.  Patients categorised as per their CKD stages. 

RESULTS

Two hundred and twenty six patients identified; 173 patients had levels recorded, of which 106 (61%) were found to have insufficiency or deficiency.  Vitamin D deficiency stratified according to CKD stage is show in table 1. 

 

CKD stage

Number of patients

Vitamin D insufficiency/deficiency (%)

1

48

26 (54%)

2

58

34 (58%)

3

26

15 (57%)

4

5

4 (80%)

5

36

23 (63%)

Table 1

CONCLUSIONS

There is a high incidence of vitamin D deficiency in this cohort, which does not seem to be correlated with renal function.  We would recommend routine vitamin D monitoring in all children with PUV.


08:24 - 08:27
S11-5 (PP)

THE LONGITUDINAL CHANGE IN RENAL AND BLADDER FUNCTION OF BOYS WITH POSTERIOR URETHRAL VALVES (PUV) BEFORE PUBERTY

Eskinder SOLOMON, Injoon HWANG, Joanna CLOTHIER and Massimo GARRIBOLI
Evelina London Children's Hospital - Guy's and St Thomas NHS Foundation Trust, Paediatric Nephro-Urology, London, UNITED KINGDOM

PURPOSE

Although PUV resection is effective in de-obstructing the urethra, the trajectory of bladder and corresponding upper tract function is not well documented. We aimed to assess the change in urodynamic parameters and eGFR of boys with PUV before puberty

MATERIAL AND METHODS

Single center retrospective longitudinal review of urodynamic (UD) findings and eGFR of boys with PUV at two time points: 5 and 10 years of age.

The urodynamic parameters documented include detrusor overactivity (DO), compliance (C= ∆bladder volume/∆detrusor pressure) and capacity.

RESULTS

Nineteen boys included, 1stUD at the median age of 5 (range 3.8-6 yrs), 2ndUD 4.7 yrs later.

Average (sd) change in eGFR is a decrease of 13.4 (+12.1) ml/min/1.73m2(p=0.07)

DO was documented in 12/19 patients at 5 years of age, in 6 of them DO persisted at 10 years of age. Only 2/7 patients developed new onset DO at 10 years of age. Compliance appeared compromised (C<20ml/cmH2O) in 9/19 patients at 5 years. However, by the age of 10, 16/19 patients had reduced compliance. There was statistically significant difference between the ∆eGFR for the group with poor compliance (-17.1ml/min/1.73m2) and normal C (0.06 ml/min/1.73m2) (p=0.02).

UDS parameter

5 yrs 

10 yrs

Total at 10 yrs

 

Detrusor overactivity

+ve  n= 12

+ve  n=6

+ve n=8

-ve   n=6

-ve   n=7

+ve  n=2

-ve n=11

-ve   n=5

 

Compliance

>20 ml/cmH2O?

yes  n= 10

yes  n=2

Yes n=3

no   n=8

no   n=9

yes  n=1

No n= 16

no  n=8

Mean capacity (+sd) 

262 + 126 ml

353+ 138 ml

 

CONCLUSIONS

The results of this study suggests that renal and bladder function worsens in PUV boys between the age of 5 to 10 years. The presence of poor compliance at the age of 5 significantly predict declining in renal function.


08:27 - 08:42
Discussion
 

08:42 - 08:45
S11-6 (PP)

INTRODUCTION OF A STANDARDISED REPORTING PROFORMA FOR PAEDIATRIC MICTURATING CYSTOURETHROGRAMS

Caoimhe WALSH 1, Kathryn WESSELY 2, Diane DE CALUWE 1, Nisha RAHMAN 1 and Marie-Klaire FARRUGIA 1
1) Chelsea and Westminster Hospital NHS Foundation Trust, Paediatric Surgery, London, UNITED KINGDOM - 2) Chelsea and Westminster Hospital NHS Foundation Trust, Paediatric Radiology, London, UNITED KINGDOM

PURPOSE

MCUGs are commonly requested to exclude VUR and bladder outlet obstruction. Useful additional information including timing and bladder volume at the start of reflux, ureteral anomalies and post-void drainage can be obtained from the cystograms, but are not routinely reported by radiologists.  A standardised MCUG reporting proforma was devised specifying key features to be reported. Our study compared reporting quality before and after introduction of the proforma.

PATIENTS AND METHODS

A retrospective analysis of paediatric MCUG reports was undertaken from two patient cohorts. Cohorts A (41 reports) and B (51 reports) comprised reports written before and following distribution of the proforma, respectively. Reports from each cohort were assessed with respect to the parameters outlined on the standardised MCUG reporting proforma. Findings from both cohorts were compared to establish if the proforma influenced the content of reports. Statistical analysis was performed using R statistical computing software. Significance was defined as p value less than 0.05.

RESULTS

Statistically significant improvements were demonstrated (after proforma vs before proforma) in the reporting of the following features: Bladder outline normal/abnormal – 92% vs 56%, urethra normal/abnormal – 87% vs 68%, contrast volume instilled – 84% vs 61%, bladder emptying – 69% vs 17%. In patients with VUR, reporting of VUR timing – 96% vs 33% and VUR grade – 91% vs 40% was significantly improved.

CONCLUSIONS

Implementation of a standardised MCUG reporting proforma produced a substantial improvement in report quality and consistency, with statistically significant improvements noted in six of the seven key features.


08:45 - 08:48
S11-7 (PP)

BILATERAL SIMPLEX ECTOPIC URETERS: HOW TO SPARE RENAL FUNCTION AND ACHIEVE CONTINENCE

Jules KOHAUT 1, Henri LOTTMANN 1, Alaa CHEIKHELARD 1, Annabel PAYE-JAOUEN 2, Nathalie BOTTO 1, Olivia BOYER 3, Mathieu PEYCELON 4, Yves AIGRAIN 1, Stephen LORTAT-JACOB 1, Alaa EL GHONEIMI 4 and Thomas BLANC 1
1) Hôpital Necker - Enfants Malades, Department of Pediatric Surgery and Urology, Paris, FRANCE - 2) Robert Debré University Hospital, AP-HP, University Paris Diderot. Centre de référence de Malformations Rares des voies Urinaires (MARVU), Department of Pediatric Urology, Paris, FRANCE - 3) Hôpital Necker - Enfants Malades, Department of Pediatric Nephrology, Paris, FRANCE - 4) Robert Debré University Hospital, AP-HP, University Paris Diderot. Centre de référence de Malformations Rares des voies Urinaires (MARVU), Department of Pediatric Urology, Paris, FRANCE

PURPOSE

Bilateral simplex ectopic ureters (BSEUs) are defined as all bilateral implantation of the ureters below the bladder neck. The bladder is usually small and incontinent. The kidney may be dysplastic. Our aim was to report our results regarding renal function and continence in a cohort of 18 girls with BSEUs.

MATERIAL AND METHODS

We retrospectively reviewed medical charts of patients with BSEUs over a 30-year period, focusing on diagnosis, surgical management and outcome regarding renal function and continence. Diagnosis was based on physical examination, imaging and endoscopy.

RESULTS

Eighteen girls were diagnosed with BSEUs. Mean follow-up was 12 years [2-19 years]. Diagnosis was made during infancy (n=15), or after 5 years of age (n=3). Eight patients had unilateral (n=6) or bilateral (n=2) dysplastic kidney at diagnosis. Initial management was ureteroneocystostomy in native bladder (n=8), cutaneous urinary diversion (n=7), augmented bladder (n=2) and Mainz pouch (n=1). Ten girls have had multiple surgeries in order to keep the native bladder. Two girls have had a urinary diversion directly followed by a bladder augmentation. Final status was bladder augmentation (n=8), complete bladder replacement (n=3), cutaneous ureterostomy (n=2) and native bladder after ureteroneocystostomy with or without bladder neck procedure (n=5). Either bladder augmentation or complete bladder replacement with CIC allowed social continence in 9/11. Only two girls achieved social continence with good renal function without bladder augmentation. Three girls (including the 2 with bilateral dysplastic kidney) have had kidney transplantation, and 6 other girls have CKD (stage 2-4).

CONCLUSIONS

Renal function preservation must be the main goal in the management of BSEUs. It goes through early diagnosis, eventual temporary cutaneous urinary derivation and avoidance of "Utopic" surgical reconstruction. Social continence requires CIC.


08:48 - 08:51
S11-8 (PP)

BLADDER INSTILLATIONS WITH SODIUM CHONDROITIN SULFATE SOLUTION AND HYALURONIC ACID CAN BE EFFECTIVE AND SAFE TO TREAT RECURRENT UTIS IN CHILDREN

Katerina PRODROMOU 1, Maria BOBADILLA 2, Claire FERGUSON 2, Helen Fiona MACANDREW 2 and Harriett CORBETT 2
1) Alder Hey Children's Hospital, Paediatric Urology, Lancaster, UNITED KINGDOM - 2) Alderhey Children's Hospital, Liverpool, UNITED KINGDOM

PURPOSE

Bladder instillations with Sodium Chondroitin Sulfate (SCS) and Hyaluronic Acid (IHA) are used to treat interstitial cystitis / painful bladder. Benefit is also reported in adults with recurrent urinary tract infections (UTIs) but data for this indication in children is lacking. We evaluate use of bladder instillations in children with recurrent UTIs.

MATERIAL AND METHODS

Patients  identified from specialist nurse records were studied retrospectively. Patients with neuropathic bladder or bladder pain alone were excluded.

The patients were divided into two groups: recurrent UTIs (group 1) and with UTIs with bladder pain (group 2). After 5 weekly bladder instillations of SCS or IHA, patients received monthly administrations. Complete response = UTI free, partial response = reduction in UTIs by more than 50%.

RESULTS

Nineteen girls (mean age 12 years) were treated with SCS or IHA between April 2015 and March 2018. Mean follow up from last instillation was 10 months (range 3 - 32). Fourteen received SCS, 4 IHA, one received both.

Group 1 (n=11), complete response in  6 (55%) and partial in 2 (18%),  no response in 3 patients. 

Group 2 (n=8), complete response  in 6 (75%) with no response in 2 patients. 

In total, 63% had a complete response and 11% a partial response, with no difference between groups nor in the response to SCS vs IHA (p>0.05, Fishers Exact test). One patient experienced vulvovaginitis, there were no other complications.

CONCLUSIONS

For selected children bladder instillations can safely resolve or reduce UTIs. 


08:51 - 08:56
S11-9 (VP)

CYSTOSCOPIC ASSISTED LAPAROSCOPIC EXCISION OF A LARGE BLADDER DIVERTICULUM IN A 1 YEAR OLD BOY

Zeni HAVELIWALA, Ravindar ANBARASAN and Massimo GARRIBOLI
Evelina London, Paediatric Urology, London, UNITED KINGDOM

PURPOSE

Bladder diverticulae are herniation of the bladder mucosa through an area of weakness in the bladder wall.  Ineffective emptying of the bladder can lead to infection, calculi or urinary incontinence.  The current management includes observation or surgical excision.  Symptomatic diverticulae may require resection.  Surgical approaches in children are described using an open extravesical technique.   We present a case of cystoscopic assisted laparoscopic bladder diverticulectomy in a 1 year old boy.

The patient had an antenatal diagnosis of bladder diverticulum, confirmed on postnatal ultrasound and micturating cystourethrography.  No upper tract anomalies were found.  The diverticulum measured 30x59x57mm.  Cystoscopy did not demonstrate urethral obstruction.  A non-invasive bladder emptying study was performed which demonstrated a large capacity bladder with incomplete emptying (PVR 52mls).  Laparoscopic bladder diverticulectomy was performed.

MATERIAL AND METHODS

The patient is placed in the lithotomy position.  Three 5mm ports are placed: umbilical, left and right flanks.  Cystoscopic illumination and hydrodistension is used at the neck of the diverticulum to help identify margins of diverticulum.  Dissection proceeded laparoscopically by incising peritoneal coverings with careful  identification of a displaced left vas deferens.  Cystoscopic illumination helped dissection of diverticulum and ensured preservation of the ureters.  The diverticulum has been excised and the mouth closed by double-layered continuous sutures.  A urinary catheter was left in situ for 10 days.

RESULTS

The post-operative course was unremarkable, with no urinary leak, infection or complication to date.

CONCLUSIONS

We describe a Laparoscopic bladder diverticulectomy with cystoscopic guidance as safe and effective technique in children.


08:56 - 09:08
Discussion