35th ESPU Joint Meeting in Vienna, Austria

S24: EXSTROPHY 2

ESPU Meeting on Friday 5, September 2025, 09:25 - 10:00


09:25 - 09:28
S24-1 (OP)

NATIONAL CENTRALIZATION OF BLADDER EXSTROPHY EPISPADIAS COMPLEX (BEEC) PATIENTS IN SWEDEN: A COMPARISON OF SHORT-TERM POSTOPERATIVE OUTCOMES

Lisa Karin Elisabet ÖRTQVIST 1, Malin AF PETERSEN 2, Gundela HOLMDAHL 2, Gillian BARKER 2, Gisela REINFELDT 2, Magnus ANDERBERG 2, Sofia SJÖSTRÖM 2 and Tomas WESTER 2
1) Karolinska University Hospital, Paediatric Surgery, Stockholm, SWEDEN - 2) Women and Children's health, Pediatric surgery, Stockholm, SWEDEN

PURPOSE

To assess short-term postoperative outcomes in bladder exstrophy epispadias complex (BEEC) patients following centralization of care. 

MATERIAL AND METHODS

This retrospective observational study included all patients who underwent primary surgery for BEEC in Sweden from 1st of July 2013 to 30th of June 2023. Surgical care of BEEC was centralized from four centres to one the 1st of July 2018. Patients treated in the 5 year-period prior to centralization were compared with those treated during the 5 year-period after centralization. Main outcomes were unplanned readmissions, surgical procedures, and complications graded according to Clavien Madadi >3 within 90 postoperative days.

RESULTS

Nineteen and 21 patients underwent primary surgery due to BEEC before and after centralization, respectively. Median age at first contact with a paediatric urologist and age at primary surgery was similar before and after centralization. Osteotomies were more common prior centralization (92% vs 36%, p=0.01). Median length of hospital stay after primary bladder closure was shorter after centralization (16 vs 26 days, p<0.001). Unplanned readmissions were required in 19 % (p=0.92) of patients both prior and after centralization and unplanned surgical procedures in 5% vs 10% (p=0.92) respectively. One complication grade IIIa occurred before centralization compared to two grade IIIa complications post centralization (p=0.92).

CONCLUSIONS

Centralization of care for BEEC did not delay time to first visit with paediatric urologist or time to primary surgery. Unplanned readmission, surgical procedures or postoperative complications did not increase after centralization. Performing osteotomies was less common post centralization, probably explaining shorter hospital stays.


09:28 - 09:31
S24-2 (OP)

MANAGEMENT OF FEMALE EPISPADIAS: A REVIEW OF 20 YEARS OF EXPERIENCE IN A SINGLE INSTITUTION

Tharanga GAMAGE 1, Emma SELLERS 2, Naima SMEULDERS 3, Navroop JOHAL 3 and Imran MUSHTAQ 3
1) Great Ormond Street Hospital Children, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital for Children London, Paediatric Urology, Lonodn, UNITED KINGDOM - 3) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM

PURPOSE

Female epispadias is a rare condition with limited long-term clinical outcomes reported. 

MATERIAL AND METHODS

We report a retrospective cohort study of 14 females presenting with epispadias in a single centre between 2000 and 2025. 

RESULTS

Thirteen patients had primary Kelly procedure. One patient initially underwent a urethrocervicoplasty but later had a Kelly repair for incontinence.Two patients were excluded from continence data assesment due to incomplete follow-up data.

 

Late presenters (>1yr)  

Early presenters (<1yr)  

Number of patients  

Presenting complaint  

Urinary incontinence  

Abnormal genitalia  

Average age of reconstruction  

5.5 yrs 

14 months  

EBC/FBC% at 5 yrs post-Kelly 

71.9% 

40.6% 

EBC/FBC% at 10 yrs-post Kelly 

77.3% 

55.3% 

EBC-expected bladder capacity, FBC-functional bladder capacity 

At 5-years post-Kelly procedure, 33.3%(4/12) achieved daytime dryness with spontaneous voiding, increasing to 50%(5/10) at 10-years. The patient who underwent urethrocervicoplasty without bladder neck reconstruction(BNR) experienced incontinence after five years and subsequently achieved daytime continence four years after a Kelly procedure.                                                                                               

Four patients required redo BNR, all of whom had small bladder capacities and three with notably short urethras. Only one patient showed significant improvement in continence following redo BNR.

Three patients underwent bladder neck bulking agent injections, but none achieved daytime dryness with this intervention alone.

CONCLUSIONS

50% of female epispadias achieved daytime continence with spontaneous urethral voiding at 10 years post-Kelly procedure.

Bulking agents to the bladder neck show minimal benefit for continence. 


09:31 - 09:39
Discussion
 

09:39 - 09:42
S24-3 (OP)

PSYCHIATRIC MORBIDITY IN BLADDER EXSTROPHY AND EPISPADIAS COMPLEX: A POPULATION-BASED CASE-CONTROL STUDY AND SYSTEMATIC REVIEW WITH META-ANALYSIS

Essi KYMÄLÄINEN 1, Niklas PAKKASJÄRVI 2, Max KARUKIVI 3, Päivi RAUTAVA 4, Bernd PAPE 5 and Liisi RIPATTI 6
1) University of Turku, The Department of Pediatric Surgery, Turku, FINLAND - 2) University of Helsinki, The Department of Pediatric Surgery, Helsinki, FINLAND - 3) University of Turku, The Department of Adolescent Psychiatry, Turku, FINLAND - 4) University of Turku, Department of Public Health, Turku, FINLAND - 5) University of Vaasa, The Department of Mathematics and Statistics, Vaasa, FINLAND - 6) Turku University Hospital, Department of Pediatric Surgery, Turku, FINLAND

PURPOSE

Bladder exstrophy and epispadias complex (BEEC) is a rare congenital anomaly with significant medical and psychosocial implications. We aimed to evaluate the prevalence and risk factors for psychiatric morbidity in BEEC patients employing a dual approach, combining a systematic review with a retrospective nationwide register study.

PATIENTS AND METHODS

We conducted a systematic literature review following PRISMA guidelines, including studies on psychiatric disorders or symptoms, or quality of life (QoL) in BEEC patients of all ages. Article quality was assessed with the Newcastle-Ottawa Scale. Prevalence data for psychiatric conditions along with scores from validated psychiatric and QoL questionnaires were extracted.
Additionally, we identified all BEEC patients born between 2001-2006 in Finland and a matched and randomised general population control group without congenital malformations. We evaluated the prevalence and risk factors for psychiatric diagnoses in this cohort up to year 2022.

RESULTS

Of 3850 retrieved results, 30 studies with 1179 participants were included in the review. Most studies showed mental health to be worse in BEEC patients compared to general population, while their QoL was not consistently worse. The overall prevalence of psychiatric morbidity was 31% [95% CI 17-47%].
In the Finnish cohort 80% (n=16/20) BEEC patients had a psychiatric diagnosis compared to 26% (n=21/80) of the controls (OR 11.2, 95% CI [3.37-37.4], p<0.001). Severity of the anomaly, number of surgeries, maternal unemployment or maternal psychiatric diagnosis were not significant risk factors.

CONCLUSION

BEEC is associated with an elevated risk of psychiatric morbidity. Our dual approach highlights the need for systematic mental health assessment and support in this population.


09:42 - 09:45
S24-4 (OP)

THE INFLUENCE OF SHAME ON FRIENDSHIP IN INDIVIDUALS WITH EXTROPHY-EPISPADIAS-COMPLEX

Johanna SEIBT 1, Anne-Karoline EBERT 2, Karolin HOLY 1 and Nicole Syringa HARTH 1
1) Ernst-Abbe-Hochschule Jena, University of Applied Sciences, Jena, GERMANY - 2) University of Ulm, Department of Urology and Pediatric Urology, Ulm, GERMANY

PURPOSE

The exstrophy-epispadias-complex (EEC) has not only physical but also complex psychological consequences. The aim was to test hypotheses about the association between shame, disclosure, quality of friendship and life satisfaction of individuals with EEC.

MATERIAL AND METHODS

During the pre-registered study, data were collected anonymously via four self-help groups in Germany and Switzerland and a matching Instagram account. This cross-sectional survey included the ‘Chronic Illness-related Shame Scale', subscales of the ‘McGill Friendship Questionnaire', and the ‘Short Scale to Measure General Life Satisfaction', slightly modified. The disclosure scale was self-created and tested using exploratory factor analysis. reliabilities for all scales were acceptable to very good. For hypothesis testing, Pearson-correlations and linear regressions were used (significance level of 5%). For gender differences, t-tests were conducted.

RESULTS

106 adults (47 men, 58 women) completed the questionnaire. Results show a negative correlation between shame and social support (r=-0.209, p=0.032), intimacy (r=-0.249, p=0.010) and life satisfaction (r=-0.408, p=0.000). The less shame, the greater the tendency to talk about EEC in the best friendship with approach goals (r=-0.329, p=0.001). Additionally, in affected friendships a positive correlation between disclosure with approach goals and positive feelings (r=0.375, p=0.000), social support (r=0.358, p=0.000) and reliable alliance (r=0.310, p=0.001) was found. Contrary to expectations, men reported tendencially more shame than women (t(1,103)=-1.699, p=0.092).

CONCLUSIONS

In EEC the level of shame and disclosure play a significant role in the context of friendship. Preventively established emotion trainings and specified social skills trainings could help patients to cope better with life challenges.


09:45 - 09:48
S24-5 (OP)

HOW TO DEVELOP A CONDITION-SPECIFIC HEALTH-RELATED QUALITY OF LIFE QUESTIONNAIRE IN CHILDREN WITH BLADDER EXSTROPHY-EPISPADIAS COMPLEX?

Ulrika SVENNINGHED 1, Elin ÖST 2, Gundela HOLMDHAL 2, Lisa ÖRTQVIST 2, Magdalena BOIJE 2, Sofia SJÖSTRÖM 1, Cecilia LINDSTRÖM GRUBER 2 and Michaela DELLENMARK BLOM 1
1) THE QUEEN SILVIA CHILDREN'S HOSPITAL, SAHLGRENSKA UNIVERSITY HOSPITAL, DEPARTMENT OF PEDIATRIC SURGERY, Gothenburg, SWEDEN - 2) ASTRID LINDGRENS CHILDREN'S HOSPITAL, KAROLINSKA UNIVERSITY HOSPITAL, DEPARTMENT OF PEDIATRIC SURGERY, Stockholm, SWEDEN

PURPOSE

Items of a condition-specific health-related quality of life (HRQoL) questionnaire should be generated from affected children’s/parents’ experiences to ensure important content for them is measured. Such a questionnaire has not existed for children with bladder exstrophy-epispadias-complex (BEEC). We hypothesized that children with BEEC experience specific impact due to their condition. The study aim was to describe those experiences to develop of a condition-specific questionnaire.

MATERIAL AND METHODS

Prospectively, ten focus groups with 37 participants (14 children aged 8-18 matched for different severity of BEEC; 23 parents of children with BEEC aged 0-18) were held at two tertiary pediatric urology departments in Sweden, led by a moderator, audio-recorded and transcribed. Reports of children’s BEEC-related HRQoL were extracted from transcripts, content analyzed, categorized into HRQoL domains to aid item generation. According to well-established principles of qualitative research, no controls or hypothesis testing were used.

RESULTS

1730 experiences were identified and allocated into seven HRQoL domains, several sub-domains each, aiding item generation for a condition-specific HRQoL questionnaire:

CONCLUSIONS

This is the first reported focus group study in BEEC children and reveals their possible physical, psychological and social impact in life. This information enables the development of a condition-specific HRQoL questionnaire for children with BEEC. This is needed to improve patient-centered care and research.


09:48 - 10:00
Discussion