30th ESPU Congress - Lyon, France - 2019

S7: EXSTROPHY-EPISPADIAS COMPLEX

Moderators: Wolfgang Rösch (Germany), Marc David Leclair (France)

ESPU Meeting on Thursday 25, April 2019, 10:56 - 12:02


10:56 - 10:59
S7-1 (PP)

CLOACAL EXSTROPHY (CE) AFTER SINGLE STAGE INITIAL CLOSURE : WHERE DO WE STAND AND HOW DID THEY DO?

May BISHARAT, Sherif M. SOLIMAN, Alice MEARS, Wilson TO and Peter CUCKOW
Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM

PURPOSE

CE is the most challenging of abdominal wall defects affecting the hindgut, bladder and genitalia, with a high association of spinal dysraphism. Since 1999, our management includes a delayed, single-stage osteotomy-aided closure with stoma formation, incorporating distal hindgut if possible. A subsequent Kelly procedure is performed for phallic corporal advancement in males. At school age, an ileocystoplasty, Monti-Mitrofanoff and bladder neck reconstruction/closure are performed. Herein, we review the outcomes of this surgical strategy. 

MATERIAL AND METHODS

CE patients managed with initial single-stage closure between 1999-2018 were retrospectively reviewed. Outcomes include continence, renal function and educational attainment. A comparative review of literature for similar large CE series was performed. Data are presented as median (range).

RESULTS

Thirty-one CE children were identified (17 male, 14 female). Age at last review was 10.9 years (7 months - 25 years) with a follow up of 9.2 years (6 months - 19 years). 74% had spinal anomalies. 30/31(97%) are alive. One died of short gut in infancy. Age at initial surgery was 34 days (3- 455 days). 87.5% had a hindgut colostomy. Subsequently, 87% of boys underwent a Kelly procedure, and 57% underwent an augment. 93% maintain normal renal function. 20/30(67%) walk unaided, 4/30(13%) use a wheelchair and 5/30(17%) walking aids. All children/adolescents who have reached the endpoint of reconstruction are clean, with 85% in mainstream school/university education. Results were similar to series employing a staged approach.

CONCLUSIONS

Our data support the premise that a single stage CE closure, followed by a Kelly's procedure and further lower urinary tract reconstruction ultimately achieve protection of the upper urinary tract and enable reconstruction of a penis allowing an adherence to genetic and gonadal sex of rearing. Urinary and faecal cleanliness are attainable, facilitating integration into mainstream education. Families of this once hopeless anomaly should be counselled accordingly despite the high surgical cost.


10:59 - 11:02
S7-2 (PP)

SHORT URETHRA - LONG COMMON CHANNEL CLOACA: AN ALTERNATE SURGICAL STRATEGY WITH DUAL BENEFITS.

Anand UPASANI 1, Alexander CHO 1, Anu PAUL 1, Lyndsay ALLEN 1, D DESAI 1, Simon BLACKBURN 2, Joe CURRY 2 and Abraham CHERIAN 1
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital, Paediatric Surgery, London, UNITED KINGDOM

PURPOSE

Short-urethra(<1.5cm) with long-common-channel poses a management dilemma. Urogenital-separation utilising the common-channel for urethroplasty is an option(Wood et al. JPS2018;53:90-95). Potential risks include difficult CIC, fistula, stricture and fixed/adynamic bladder-outlet, leading to subsequent interventions. Primary-bladder-neck-closure(PBNC) is an alternate strategy and we use common-channel for vagina.

MATERIAL AND METHODS

Prospective cloaca-database with protocolised-care-pathway were analysed in 25-consecutive-patients(2012-2018). All patients with short urethral-length(<1.5cm) had PBNC. Patients were monitored - clinically(UTI), Ultrasound(KUB) for upper-tract-dilatation(UTD), creatinine/eGFR(renal function), nuclear-medicine-scans(scarring), non-invasive-urodynamics and check-vaginoscopy was performed at colostomy-closure).

Wilcoxon Signed-Rank-Test(WSRT) was applied.

RESULTS

6/25 had PBNC with appendico-vesicostomy(Table-1). Median-age at reconstruction:14months(5-36). Median-follow-up:19months(1-48). Common-channel was used as vagina for all. No leak or fistula were noted. No mechanical problems with CIC-channel. 1/6 with poor-compliance has indwelling-catheter.1/6 had lower-UTI. No new upper tract dilation or renal-scarring was observed. There was no significant difference in early-versus-follow-up creatinine.

Table-1:

Case

Common channel
length-(cm)

Urethral
Length
(cm)

Spinal
anomaly
(Y/N)

Renal-function

Bladder-outcome

Renal
dysplasia (Y/N)

UTI

New
Scars
(Y/N)

New
UTD
(Y/N)

eGFR (ml/min/1.73m2)

Problemwith CIC

Reintervention

1

Atypical

0

Y

N

N

N

N

147

Y*

N

2

5

< 1.5

Y

Y

N

N

N

75#

N

N

3

Atypical

0

Y

N

N

N

N

106

N

N

4

3.5

 0.5

Y

Y

Y^

N

N

100

N

N

5

6.5

 1

Y

Y

N

N

N

101

N

N

6

3.5

< 1.5

N

N     

N

N

Awaited

144

N

N

                                 *Poor-compliance; # Pre-existing-significant-renal-dysplasia; ^Lower-UTI.

CONCLUSIONS

PBNC is a safe alternative in select-complex-cloaca and concurrently simplifies vaginal-management which may have required vaginal-substitution. Understanding longterm outcomes including subsequent interventions for the two approaches is paramount.


11:02 - 11:05
S7-3 (PP)

CLOACA: EARLY RENAL OUTCOMES USING A PROTOCOLISED MDT APPROACH

Anand UPASANI 1, Alexander CHO 1, Lyndsay ALLEN 2, Anu PAUL 1, Divyesh DESAI 3, Simon BLACKBURN 4, Joe CURRY 4 and Abraham CHERIAN 1
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital, Urodynamics, CNS, London, UNITED KINGDOM - 3) Great Ormond Street Hospital, Urodynamics Lead, London, UNITED KINGDOM - 4) Great Ormond Street Hospital, Paediatric Surgery, London, UNITED KINGDOM

PURPOSE

Renal impairment is a significant cause of morbidity in cloacal-malformation. Historically, CKD is reported in nearly 50% of the cases, 17% ESRD and 6% mortality at 5 years (Warne et al. Urology 2002;167:2548-2551). Our prospective-protocolised-multidisciplinary team(MDT) report early renal outcomes and analyse incidence of pre-existing and further renal damage.

MATERIAL AND METHODS

Prospective-cloaca-database of 25 consecutive patients, that followed a protocolised-care-pathway was analysed (2012 to 2018). Median age at reconstruction:12months(5-36). Median follow-up:32months(1-88). Renal outcome was monitored by regular clinical-reviews, serum creatinine, nuclear-medicine scans and eGFR.

Wilcoxon Signed-Rank Test(WSRT) and Fischer Exact Test(FET) were applied.

RESULTS

Of 25 patients, associated renal abnormalities 15(60%), hydronephrosis 7(28%), VUR 5(20%) and pre-existing renal-dysplasia 9(36%). 15(60%) had spinal dysraphism - 5 had untethering.

21(84%) have eGFR above 90ml/min/1.73m2. There was no progression of CKD-staging during this period. Median creatinine in early- life was 51 umol/L and improved with time to 31 umol/L at last follow-up. This was significant (WSRT, Z-value: -1.8969 @ p

Progressive renal-scarring was noted 4/25(16%). Correlation to presence of VUR(4/4) and pre-existing hydronephrosis(3/4) was significant (FET).

Concomitant-urology-interventions included 3 STING, 1 VUJO required balloon dilation followed-by uretero-cystostomy, 1 nephrectomy for non-functioning-dilated kidney.

Table-1 compares our results with published-literature.

 

Warne et al(2002)

Defoor et al(2015)

Current Study

Study-type

Retrospective

Retrospective

Prospective

Sample-size

64

44

25

Study-period

20 years(1980-2000)

7 years(2006-2013)

6 years(2012-2018)

GFR-method

51Cr-EDTA-clearance

Cystatin-C/DTPA

eGFR(Modified-Schwartz-formula)

GFR calculated for

38/64

44/44

25/25

CKD-1(> 90)

>80          6(16%)

38(86%)

21(84%)

CKD-2(60-89)

50-80       8(21%)

2(8%)

CKD-3(30-59)

25-49     13(34%)

5(11%)

-

CKD-4(15-29)

1(2%)

1(4%)

CKD-5(<15)

-

1(4%)

Progression of CKD

-

No

No

CONCLUSIONS

Pre-existing hydronephrosis and  VUR correlate with higher incidence of new renal scarring. Our outcomes are comparable to similar cohort in recent literature. A protocolised MDT approach offers the means for timely diagnosis and intervention to preserve renal function.


11:05 - 11:08
S7-4 (PP)

3D REAL TIME MRI-GUIDED INTRAOPERATIVE NAVIGATION OF THE PELVIC FLOOR DURING CLASSIC BLADDER EXSTROPHY AND CLOACAL EXSTROPHY CLOSURE - CUTTING EDGE TECHNOLOGY FOR SURGICAL SKILL EDUCATION

Heather DI CARLO 1, Eric MASSANYI 1, Bhavik SHAH 1, Mahir MARUF 2, Aylin TEKES 1 and John GEARHART 1
1) Johns Hopkins Medical Institutes, Baltimore, USA - 2) Johns Hopkins Medical Institutes, Urology, Baltimore, USA

PURPOSE

Intraoperative magnetic resonance imaging (MRI) guided navigation of the pelvic floor offers a novel technique for identification of the urogenital diaphragm fibers and the thickened muscular attachments between the posterior urethra, bladder plate and pubic rami during closure, allowing precise surgical skill education in this crucial step of reconstructive surgery of bladder exstrophy (BE).

MATERIAL AND METHODS

Institutional review board and Food and Drug Administration approval was obtained for use of Brainlab® (Munich, Germany) intraoperative MRI-guided navigation of the pelvic floor anatomy during closure of BE at the authors' institution. Pre-operative pelvic MRI was obtained one day prior to closure in patients necessitating pelvic osteotomies. Intraoperative registration was performed after pre-operative planning with a pediatric radiologist utilizing five anatomic landmarks immediately prior to initiation of surgery. Accuracy of identification of pelvic anatomy was assessed by two pediatric urologic surgeons and one pediatric radiologist.

RESULTS

Forty eight patients with BE at the authors' institution have successfully utilized Brainlab® technology to navigate and guide the dissection of the pelvic floor intraoperatively. All patients had 100% accuracy in correlation of gross anatomic landmarks with MRI identified landmarks intraoperatively, and all have had successful closure without any major complication.

CONCLUSIONS

Brainlab® intraoperative MRI-guided pelvic floor navigation and dissection is an effective way to accurately identify pelvic anatomy during BE closure. This technology offers a unique opportunity for surgical skill education in this complex reconstructive operation.


11:08 - 11:11
S7-5 (PP)

HEALTH-RELATED QUALITY OF LIFE AMONG INDIVIDUALS WITH BLADDER EXSTROPHY-EPISPADIAS COMPLEX: A SYSTEMATIC REVIEW OF THE LITERATURE

Michaela DELLENMARK-BLOM, Sofia SJÖSTRÖM, Kate ABRAHAMSSON and Gundela HOLMDAHL
The Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Department of Pediatric Surgery and Urology, Gothenburg, SWEDEN

PURPOSE

Children with bladder-exstrophy-epispadias-complex (BEEC) risk long-term urinary and genital dysfunctions. This study aims to review the literature on studies of health-related quality of life (HRQOL) in BEEC patients, and describe methodologies used.

MATERIAL AND METHODS

A literature search on HRQOL in BEEC patients was conducted in Pubmed, CINAHL, Embase, PsycINFO, Cochrane, from inception to May 2018. A meta-analysis of HRQOL in BEEC patients compared to healthy references was performed.

RESULTS

Twenty-one articles (published 1994–2018) including 830 reports from patients or their parent-proxies, described HRQOL in children and adolescents (n=5), adults (n=5) or mixed age populations (n=11). Median sample size was 24, loss to follow-up 43% and response rate 84%. Four articles reported multi-center studies. Overall HRQOL was reduced in BEEC patients compared to healthy references in 4/4 studies. Impaired physical or general health in BEEC patients were described in nine articles, diminished mental health in eleven and social health in ten articles. This included descriptions of BEEC patients’ internalizing problems, bullying, loneliness and social restrictions. Thirteen studies demonstrated presence of their sexual health/functioning or body perception impairments. Urinary incontinence was the most common factor related to low HRQOL (12 studies). In six studies, HRQOL was better than healthy norms. In eligible studies (n=5) for the metaanalysis, the pooled estimate of the effect of BEEC indicated lower HRQOL in children or adults on several domains. Thirty-six HRQOL assessments were used; none developed and validated for BEEC.

CONCLUSIONS

BEEC patients may have impaired HRQOL, however, HRQOL is diversely investigated. Future research is warranted and should encompass strategies to increase study participation, multi-center collaborations, investigations stratified for psycho-developmental age and a standardized BEEC-specific HRQOL instrument including questions important for BEEC patients.  


11:11 - 11:26
Discussion
 

11:26 - 11:29
S7-6 (PP)

SUBSYMPHESEAL URETHROSCPIC GUIDED BLADDER NECK AND URETHRAL PLICATION IN FEMALE EPISPADIAS: LONG-TERM OUTCOME IN 25 PATIENTS

Nastaran SABETKISH, Shabnam SABETKISH and Abdol-Mohammad KAJBAFZADEH
Pediatric Urology and Regenerative Medicine Research Center, Section of Tissue Engineering and Stem Cell Therapy, Children's Center of Excellence, Tehran University of Medical Sciences, Tehran, ISLAMIC REPUBLIC OF IRAN

PURPOSE

Isolated female epispadias is an uncommon congenital anomaly. The aim of the present study was to describe long-term follow-up of female epispadias patients who underwent novel approach of subsympheseal urethroscpic guided bladder neck and urethral plication with external genitoplasty as well as monsplasty. 

MATERIAL AND METHODS

The records of 25 female patients referred for repair of epispadias were extracted from an institutionally approved database. All girls had regular yearly follow-up examination and urinary tract ultrasonography, post voiding urine residue measurement uroflowmetry and urine exam. Ten patients have had grade I-II vesicoureteral reflux without history of urinary tracts infection prior to reconstruction. All patients underwent subsympheseal urethroscpic guided bladder neck and urethral plication. For urethral elongation a strip of 2- 3 centimeter of shiny skin between the clitorial divergent was separated on the pedicled based flap. They were followed up for an average period of 8.3 years. 

RESULTS

In the last follow-up, 20 patients (80%) were totally continent without any postoperative complications; while injection of bulking agent into the bladder neck was needed in 3 children and were socially continent after further attempts. Incontinence rate was 4% in the last postoperative follow-up (n=1). One girl was on intermittent clean catheterization till puberty due to overcorrection and high post voiding residue.  

CONCLUSIONS

This technique provides satisfactory outcomes in selected female patients with epispadias without further bladder neck reconstruction. The majority of patients attained social dryness with minimum complication rate and best cosmetic results.


11:29 - 11:32
S7-7 (PP)

COMPLICATIONS DURING THE LEARNING CURVE OF KELLY RADICAL SOFT-TISSUE MOBILISATION FOR BLADDER EXSTROPHY & EPISPADIAS

Solène JOSEPH 1, Sébastien FARAJ 2, Sajid SULTAN 3, Georges AUDRY 4, Julien ROD 5 and Marc-David LECLAIR 2
1) Hôpital Mère-Enfant, Pediatric Surgery, Nantes, FRANCE - 2) Hôpital Mère-Enfant, Pediatric Surgery, Nantes, FRANCE - 3) SIUT, Pediatric Urology, Karachi, PAKISTAN - 4) Hôpital Trousseau, Pediatric Surgery, Paris, FRANCE - 5) Caen University Hospital, Pediatric Surgery, Caen, FRANCE

PURPOSE

The radical soft-tissue mobilisation (RSTM) described by J.Kelly offers a unique opportunity of anatomical reconstruction aiming at improving genitalia outcomes and continence fonction in exstrophy-epispadias complex. However, it implies extensive perineal dissection and potential subsequent vascular risk. The aim of this study was to assess the morbidity of the RSTM during its learning curve.

MATERIAL AND METHODS

From April 2008 to Apr 2018, 71 children ( 45 bladder exstrophy - 21 epispadias - 5 cloacal exstrophy or other) underwent RSTM by a single surgical team at a median age of 12 months [0-107]. Among children with bladder exstrophy, 24 had previously undergone neonatal bladder closure (8/24 unsuccessful), whereas 21 underwent RSTM combined with delayed closure in a single-stage. Among 21 children with proximal epispadias, 18/21 underwent RSTM as primary reconstruction. No pelvic posterior or innominate osteotomy was performed whatever the age.The main criteria was occurence of surgical complication.

RESULTS

With a mean follow-up of 36 months [4-115], 29 complications occurred in 27 children (38%) : urethrocutaneous fistula (n=8), surgical site infection (n=2), parietal hernia (n=2), urethral stenosis (n=6), febrile UTI (n=11). 6/8 fistula closed spontaneously ; urethral stenoses were successfully treated with balloon dilatation in 4/6. No grade IV-V Clavien-Dindo complication was observed. Eleven children (15%) required revision surgery for grade III complications including urethral stenosis treatment (n=5),VU reimplantation (n=3), parietal hernia repair (n=1), urethrocele excision, and fistula closure.
Of note, no dehiscence occurred among the 29 exstrophy cases treated by RSTM combined with bladder closure as a single-stage. No ischemic complication nor late corpora cavernosa or hemi-glans atrophy was observed.

CONCLUSIONS

During its learning curve, the Kelly RSTM yielded acceptable complications and revision surgery rates, presumably similar to other major BEEC reconstructive procedures, without demonstrable specific morbidity related to extensive mobilisation of the corpora and anterior pelvic floor.


11:32 - 11:35
S7-8 (PP)

KELLY RADICAL SOFT-TISSUE MOBILISATION FOR BLADDER EXSTROPHY & EPISPADIAS : CONTINENCE RESULTS OF A PRELIMINARY COHORT

Solène JOSEPH 1, Sébastien FARAJ 1, Philippe RAVASSE 2, Yves HELOURY 1 and Marc-David LECLAIR 3
1) Hôpital Mère-Enfant, Pediatric Surgery, Nantes, FRANCE - 2) CHU de CAEN, Pediatric Surgery, Caen, FRANCE - 3) Hôpital Mère-Enfant, Chirurgie Infantile, Nantes, FRANCE

PURPOSE

From 2008 to 2018, a Kelly RSTM was performed as continence procedure in 71 consecutive BEEC patients, following a prospective protocol, by a single surgical team. Our aim was to report on the continence results of the initial group of this cohort.

MATERIAL AND METHODS

Inclusion criteria : children with bladder exstrophy (EXS) / incontinent proximal epispadias (EPI), successful bladder closure, having reached >3 y.o, and >12 months follow-up after undergoing RSTM.
Children underwent regular clinical examination/renal US, annual endoscopy and cystography under GA, and annual cystomanometry after the age of 3, if incontinent.
Continent score was : grade I (dry intervals, still wearing daytime protection), II (dry by day without protections, wet at nights), III (dry day and night).

RESULTS

A total of 30 children met inclusion criteria : 16 EXS (14M-2F) and 14 EPI (5M-9F), aged 7.25 y.o [3-15] at last follow-up. Overall, 21/30 (70%) were dry at least by day after a median follow-up of 5.4 years [1.5-10].
Among EXS patients, 10/16 (63%) had continence grade II-III, of whom 6/10 voided per urethra (in addition to 2/10 dry under CIC, and 2/10 dry after bladder augment+CIC). Six EXS patients with persistent grade I continence had bladder capacity of 25% [8-32] of expected capacity for age, and may ultimately require bladder augmentation.
In EPI group, 11/14 (80%) achieved at least daytime continence.
Overall, 4/30 (13%) children had insufficient resistances requiring additional bulking agents cervical injections. In the opposite, 3/30 (10%) had symptomatic bladder outlet obstruction requiring CIC only.

CONCLUSIONS

The Kelly RSTM allows to achieve acceptable social continence and voiding per urethra in half of BEEC patients (EXS 38%-EPI 70%). The rate of EXS patients requiring bladder augmentation after RSTM could be as high as 50%, traducing a limited impact of the Kelly procedure on bladder growth potential.


11:35 - 11:38
S7-9 (PP)

IMPACT OF PELVIC IMMOBILIZATION TECHNIQUES ON THE OUTCOMES OF PRIMARY AND SECONDARY CLOSURES OF CLASSIC BLADDER EXSTROPHY

Mohammad ZAMAN 1, Matthew KASPRENSKI 1, Mahir MARUF 1, Karl BENZ 1, Rachel DAVIS 1, John JAYMAN 1, Heather DICARLO 1, Paul SPONSELLER 2 and John GEARHART 1
1) Johns Hopkins Hospital, Pediatric Urology, Baltimore, USA - 2) Johns Hopkins Hospital, Pediatric Orthopedic Surgery, Baltimore, USA

PURPOSE

A potential determinant of successful bladder closures in patients with classic bladder exstrophy (CBE) is the postoperative pelvic immobilization technique. This study investigates the success rates of primary and secondary bladder closures based on various immobilization techniques from a high-volume exstrophy center.

MATERIAL AND METHODS

A prospectively maintained institutional exstrophy-epispadias complex database of 1336 patients was reviewed for patients with CBE who have underwent primary or secondary closures between 1975 and 2018 and subsequently had a known method of pelvic immobilization. Patients were divided into two groups: primary and secondary closures. Associations between closure outcomes and immobilization techniques were determined. 

RESULTS

A total of 486 patients with primary closures and 107 patients with secondary closures met the inclusion criteria. In total, 348 (71.6%) primary closures were successful. As shown in the table, the success rates of primary closures were highest in patients immobilized with modified Buck’s and Bryant’s Traction (94.9% and 79.3%, respectively) and lowest in those with spica cast (49.6%). A propensity score adjusted logistic regression (adjusting for osteotomy status, period of closure, location of closure, and closure type) revealed that modified Buck’s traction had a 5.58 (95% CI 1.74-23.01, p = 0.008) compared to spica casting during the primary closure. For the secondary closure group, there were 95 (88.8%) successful secondary closures. Success rates were highest in modified Buck’s traction (97.2%) and lowest with spica cast (66.7%). 

CONCLUSIONS

Success rates for primary closures were highest using modified Buck’s traction with external fixation and lowest for spica casts. Similarly, for secondary closures, the best outcomes were achieved using modified Buck’s traction with external fixation and the lowest success rates were associated with spica casts.


11:38 - 11:41
S7-10 (PP)

URINARY CONTINENCE AND RENAL SCARRING IN PATIENTS WITH CLASSIC BLADDER EXSTROPHY

Raimondo Maximilian CERVELLIONE, David KEENE, Jennifer POWELL, Alan DICKSON and Tamas CSERNI
Royal Manchester Children's Hospital, Paediatric Urology, Manchester, UNITED KINGDOM

PURPOSE

To assess the urinary continence and the presence of renal scarring in patients treated because of classic bladder exstrophy (BE) in a high volume exstrophy centre. 

MATERIAL AND METHODS

Data was prospectively collected on consecutive classic BE patients treated primarily in a single exstrophy centre between 1999 and 2013 (patients currently at least 5 years old). The following exclusion criteria were adopted: patient lost to follow-up, severe comorbidities and continent procedure performed within 12 weeks. Outcomes measured included: age, gender, surgical history, urinary continence (defined as continent, intermittently incontinent and continuously incontinent) and presence of renal scarring on DMSA scan. 

RESULTS

Sixty-four patients were identified. Thirteen were excluded: 5 lost to follow-up, 6 had co-morbidities (2 autism, 2 ADHD, 1 developmental delay, 1 epilepsy), 2 had a recent continent procedure. Fifty-one were included in the study (24 males) with a mean age of 11 years. 34/51 (66%) had a functional reconstruction: 23 had primary closure only, 8 bladder neck reconstruction, 3 bladder augmentation; 17/51 (33%) had a non-functional reconstruction: 15 bladder neck closure + bladder augmentation + Mitrofanoff formation and 2 urostomy. Overall 40 (78%) patients are continent, 8 (16%) are intermittently incontinent and 3 (6%) are incontinent. 42/51 had a DMSA: 25 (60%) showed no renal scarring and 17 (40%) showed renal scarring, 7 of which was bilateral.

CONCLUSIONS

In a high volume exstrophy centre, the treatment of classic BE has allowed a functional reconstruction in 2/3 of the patients with good overall urinary continence. However, a significant number of them has developed renal scarring. 


11:41 - 11:44
S7-11 (PP)

BLADDER HERNIATION AS AN AUTO-AUGMENTATION TECHNIQUE IN BLADDER EXSTROPHY: INITIAL EXPERIENCE IN PATIENTS WITH SMALL BLADDER TEMPLATE

Shabnam SABETKISH, Nastaran SABETKISH and Abdol-Mohammad KAJBAFZADEH
Pediatric Urology and Regenerative Medicine Research Center, Section of Tissue Engineering and Stem Cell Therapy, Children's Center of Excellence, Tehran University of Medical Sciences, Tehran, ISLAMIC REPUBLIC OF IRAN

PURPOSE

Surgical techniques for management of newborns with bladder exstrophy epispadias complex (BEEC) with small bladder template have remained undetermined. Herein, we aim to present our long-term experience of bladder plate herniation technique in patients with inadequate bladder template.

MATERIAL AND METHODS

Our institutional database of exstrophic patients treated and followed between 2006 and 2015, showed that 10 had an inadequate bladder template that was not suitable to be closed in newborn period. The bladder underlying fascia was opened and the exstrophic bladder was fixed above the peritoneal cavity so that the abdominal pressure would be directly transferred to the posterior bladder wall and protrudes the bladder template. By this phenomenon bladder bulging causes gradual bladder expansion and auto-augmentation. Inguinal hernia was also fixed during this process to increase the pressure transferred to the exstrophic bladder (n=5). Bladder surface was measured while the patient was crying and when the bladder was enlarged. These children were followed during the next 6 to 8 months and underwent primary closure if the template was adequate enough.

RESULTS

All patients experienced an uneventful postoperative period without any complications. The bladder was enlarged with maneuvers increasing the abdominal pressure as well as during laughing/crying.The average bladder surface was increased about 2.5 to 3 times at the last follow-up. The bladder was enlarged enough so that the patients were all prepared for undergoing primary closure.

CONCLUSIONS

This technique seems to be feasible in patients with small-sized bladder and may be performed before the primary closure to increase the success rate. In addition, this technique may be performed in patients who do not attain adequate capacity for future augmentation and ureteral reimplantation.


11:44 - 12:02
Discussion