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Background: In literature studies exploring long-term psychosexual development and intimacy of adults living with the complex genitourinary conditions associated with classic bladder exstrophy (BE) are scarce, with small sample sizes and lacking in methodology., Objective: This study aims to examine areas of potential psychosexual distress in adults born with classic BE to develop targeted clinical interventions., Study Design: The validated Sexrelation Evaluation Schedule Assessment Monitoring (SESAMO) questionnaire was administered to all BE patients aged ≥18 years operated on in our tertiary referral center during infancy. Z-scores were calculated for each area of interest, considering i) gender (female vs male); ii) committed partnership status (singles vs couples); iii) the voiding technique adopted to empty the bladder., Results: A total of 33 (F:M 12:21; singles: couples 11:22) adults with BE were enrolled in the study at a median age of 39 (32-47) years. Overall, BE adults performed the worst regarding psychosexual identity (z-score:-1.282), pleasure (z-score:-0.915) and desire (z-score:-0.583); singles regarding relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples regarding extramarital sexuality (z-score:-1.175) and sexual communication (z-score:-0.255). When it came to gender, females significantly performed worse than males regarding psychosexual identity (-1.645 vs -1.282; p-value:<0.0001) and areas of pleasure (-1.126 vs -0.359; p-value:<0.001). Single females performed worse than males regarding actual masturbation (-0.763 vs 0.583; p-value:<0.05) and better regarding relational attitude (-1.226 vs -1.751; p-value:<0.05). Females in stable relationships performed worse than males regarding actual masturbation (-1.645 vs 0.306; p-value:<0.05) and better regarding sexual intercourse (1.866 vs -0.565; p-value:<0.01). The voiding techniques used to empty the bladder did not show any influence on these results., Conclusion: Adults with BE have a greater likelihood of experiencing a wide range of psychosexual difficulties. Identifying the specific areas of psychological distress can help them cope with their medical experience and actual clinical condition and clinicians plan adequate psychological interventions., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Bladder Exstrophy and Epispadias Complex (BEEC) is associated with psychosocial difficulties. Screening questionnaires, alongside consultation with a clinical psychologist, can help identify children/young people for further assessment and track trends over time to improve service delivery., Objective: To screen paediatric BEEC patients for a range of general psychosocial difficulties in a multi-disciplinary out-patient clinic setting and compare these results with our previous study and against published norms., Study Design: The current service evaluation collected data at outpatient BEEC clinic appointments between 2016 and 2022 (2016-2022 sample). Children aged 4-5, 10-11 and 14-15 years and their parents/proxy were asked to complete two standardised psychosocial questionnaires: Paediatric Quality of Life Inventory (PedsQL 4.0 Generic Core and Family Impact Module) and the Strengths and Difficulties Questionnaires (SDQ). 79 children (CYP) and 93 parent/proxy (P/P) responses were recorded. The sample included paired CYP and P/P responses for the PedsQL (n = 67) and SDQ (n = 35). The mean age for CYP was 9.9 years (SD 3.99, range 2-17), 69.8% (n = 120) of responses for male children., Results: The percentage of total CYP scores falling within the 'At Risk' category on the PedsQL increased in the 2016-2022 sample compared to our 2015 sample, indicating the prevalence of greater difficulties. Differences between P/P and CYP responses on both the PedsQL and SDQ favoured CYP. Age, gender, and diagnosis appeared to influence certain questionnaire responses, depending on respondent (CYP or P/P). A significant difference between P/P and CYP in the emotional domain of the PedsQL for those aged 13-18 was observed (p = 0.020), with P/P reporting greater difficulties, but this was not seen in the younger age ranges. Physical Health on the PedsQL were significantly lower for children with a cloacal exstrophy diagnosis, in comparison to bladder exstrophy and epispadias. P/P SDQ scores for boys were significantly higher in several domains., Conclusions: The results demonstrate the need for psychosocial screening, providing benchmarking for psychosocial difficulties within this patient group. Results indicate that patients accessing our clinic are reporting a higher level of challenge across psychosocial domains in recent years reflected by the percentage within the 'At Risk' category for psychosocial difficulty. Linked questionnaire data with condition specific information and surgical history would improve service evaluations. CYP reaching clinical thresholds are offered further psychological assessment within the service., Competing Interests: Conflict of interest None., (Crown Copyright © 2024. Published by Elsevier Ltd. All rights reserved.)

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

The bladder exstrophy-epispadia complex (BEEC) includes malformations with midline closing defects of the lower abdomen and external genitalia. Long-term consequences with urinary incontinence and sexual dysfunction, in spite of multiple surgical interventions, are common and expected to affect the patient's health-related quality of life (HRQOL). The extent and the predictive factors are, however, not known. New patient-reported outcome research is emerging, but valid and reliable condition-specific HRQOL are still missing. The aim of this review is to summarize and discuss the latest published reports (2015-2019) on HRQOL in patients with the BEEC and its relationship to incontinence and sexual factors., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of genitourinary malformations. Children risk long-term urinary and genital dysfunctions. To achieve a comprehensive understanding, this study aimed to review the literature on generic and disease-specific health-related quality of life (HRQOL) in BEEC patients, and methodologies used., Methods: A literature search 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), describing HRQOL of children and adolescents (n = 5) and adults only (n = 5), or integrated age populations (n = 11), were identified (median sample size 24, loss to follow-up 43%, response rate 84%). Overall HRQOL was reduced in BEEC patients compared to healthy references in 4/4 studies. Impaired physical or general health in BEEC patients has been described in 9 articles, diminished mental health in 11, restricted social health in 10, and sexual health/functioning or body perception impairments in 13 articles. Urinary incontinence was the most common factor related to worse HRQOL (12 studies). In six studies, HRQOL was better than healthy norms. In eligible studies (n = 5), the pooled estimate of the effect of BEEC indicated worse HRQOL for children and adults (0 > effect sizes < 0.5). Thirty-six HRQOL assessments were used, none developed and validated for BEEC., Conclusions: HRQOL in BEEC patients may be negatively impacted, particularly considering mental and social HRQOL. Sexual health/functioning or body perception impairments may be present in adolescents and adults. However, HRQOL is heterogeneously assessed and subsequent findings are differently reported. Additional research is warranted and can be improved.

Objective: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function., Design: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire., Setting: Maryland, United States., Participants: Review of a single-institution exstrophy-epispadias complex database resulted in 25 adult female patients with a history of POP treated at the authors' institution. Eleven patients participated and were included in the analysis., Main Outcome Measures: Urinary continence and sexual function., Results: All participants underwent surgical repair for prolapse, with 7 (63.6%) experiencing unsuccessful initial repair and subsequent recurrence. Median total number of POP repairs was 2.5 (1-4). After correction of POP, patients reported a median improvement in International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form scores of 11 (21 to -1) of 21 and in POP-Urinary Incontinence Sexual Questionnaire scores of 9.5 (6.5-33.0) of 48.0. With regard to urinary continence, 6 (54.5%) patients presently reported no incontinence, 3 (27.3%) reported mild incontinence, and 2 (18.2%) reported continuous incontinence., Conclusions: Pelvic organ prolapse poses significant reductions in quality of life for women born with exstrophy, with effects on urinary continence and sexual function. Identification and correction of prolapse seems to result in notable improvements in the lives of these patients.

Objectives: To identify the long-term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE)., Materials and Methods: A prospectively maintained institutional database comprising 1248 patients with exstrophy-epispadias was used. Men aged ≥18 years with CBE were included in the study. A 42-question survey was designed using a combination of demographic information and previously validated questionnaires., Results: A total of 215 men met the inclusion criteria, of whom 113 (53%) completed the questionnaire. The mean age of the respondents was 32 years. Ninety-six (85%) of the respondents had been sexually active in their lifetime, and 66 of these (58%) were moderately to very satisfied with their sex life. The average Sexual Health Inventory for Men score was 19.8. All aspects of assessment using the Penile Perception Score questionnaire were on average between 'very dissatisfied' and 'satisfied'. Thirty-two respondents (28%) had attempted to conceive with their partner. Twenty-three (20%) were successful in conceiving, while 31 (27%) reported a confirmed fertility problem. A total of 31 respondents (27%) reported undergoing a semen analysis or post-ejaculatory urine analysis. Of these, only four respondents reported azoospermia., Conclusion: Patients with CBE have many of the same sexual and relationship successes and concerns as the general population. This is invaluable information to give to both the parents of boys with CBE, and to the boys themselves as they transition to adulthood., (© 2017 The Authors BJU International © 2017 BJU International Published by John Wiley & Sons Ltd.)

Introduction: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase., Objective: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality., Study Design: 13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO)., Results: The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas (Summary Table)., Discussion: This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this research showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program., Conclusion: This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: The bladder exstrophy-epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life., Objective: The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC., Study Design: Fourteen mothers of children aged 6-10 years and affected by BEEC (9 boys and 5 girls) were interviewed. Data on the mothers' experiences were collected through semi-structured interviews (Table)., Results: The qualitative analysis of the interviews showed that participants described experiences that were characterised by emotions such as fear and anger. Each mother had implemented a different and, sometimes, dysfunctional strategy in order to cope with the complex situation of the son/daughter. The aspects that most clearly emerged from mothers' descriptions were (1) the traumatic situation at the birth of the baby, (2) the sense of embarrassment concerning the pathological condition as the child was growing and the consequent sense of isolation of the mother, and (3) the fluctuation of feelings towards the multidisciplinary staff, which was sometimes seen as an important source of help and some other times as too destabilising and not helpful at all., Discussion: The study provided some insight into the psychological and social conditions experienced by mothers of children with BEEC, which could serve as a basis for developing multidisciplinary teams with greater awareness about families living with this condition and better timing in addressing their needs., Conclusions: Mothers of children with BEEC show emotional and social difficulties. This is a crucial aspect to consider when planning a multidisciplinary approach to the treatment/therapy, especially considering that children examined in this study are approaching adolescence., (Copyright © 2016. Published by Elsevier Ltd.)

Purpose: We evaluated whether genital deformity has an impact on gender identity and sex role in patients operated on for bladder exstrophy-epispadias complex., Materials and Methods: A total of 62 adolescents and adults operated on for bladder exstrophy-epispadias complex were mailed questionnaires evaluating gender identity (Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults) and sex role (Bem Sex Role Inventory). Of the patients 33 responded and the results were compared with 99 gender matched controls., Results: On the gender identity questionnaire female patients had median scores similar to those of their gender matched controls (4.93 vs 4.89, p = 0.412) but in males the score was lower compared to controls (4.87 vs 4.96, p = 0.023), indicating somewhat more conflicted gender identity. However, no patient had gender dysphoria. Female sex role index was higher in female patients vs controls (5.9 vs 5.3, p = 0.003) but was comparable between male patients and controls (5.2 vs 5.0, p = 0.459). Masculine sex role indices were comparable between female patients and controls as well as between male patients and controls. Of 32 patients 17 were considered to have androgynous sex role, as were 24 of 97 controls (p = 0.004). The exact diagnosis (bladder exstrophy or epispadias) or dissatisfaction with appearance of the genitals had no impact on gender identity or on sex role indices., Conclusions: Male patients had lower gender identity scores compared to controls and female sex role was enhanced among female patients. Androgynous sex role was more common in patients vs controls. Gender dysphoria was not noted in any patient., (Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Background: The bladder exstrophy-epispadias complex (BEEC) comprises a spectrum of congenital anomalies that represents the severe end of urorectal malformations, and has a profound impact on continence as well as sexual and renal functions., Objective: The relation between severity of BEEC and its associated functional impairments, on one hand, and the resulting restrictions in quality of life and potential psychopathology determine the patients' outcome. It is important for improving further outcome to identify BEEC-related sources of distress in the long term. Genital function and sexuality becomes an important issue for adolescent and adult BEEC individuals. Hence, the present study focused on sexual function and psychological adaption in patients with BEEC., Study Design: In a multicenter study 52 patients (13 females, 39 males) with classic bladder exstrophy (BE) with their bladders in use were assessed by a self-developed questionnaire about sexual function, and psychosexual and psychosocial outcome. The patients were born between 1948 and 1994 (median age 31 years)., Results: Twelve of 13 (92%) females and 25 of 39 (64%) males with classic BE had answered the questions on sexual function. Of these, 50% females and 92% males answered that they masturbated. Females had sexual intercourse more frequently. Six (50%) females affirmed dyspareunia whereas only two (8%) males reported pain during erection. Eight (67%) females specified having orgasms. Eighteen (72%) males were able to ejaculate. Two males and none of the females lived in a committed partnership (Figure). Two (15%) females and 13 (33%) males answered all psychosocial questions. The majority of these patients had concerns about satisfactory sexuality and lasting, happy partnerships. A minority of patients of both sexes were willing to answer psychosocial questions. Sexual activity and relationships of many adult BE patients seems to be impaired. Not surprisingly, sexual activity and awareness were different in males and females even in a multi-organ anomaly., Discussion: To date, one of the main goals of the medical treatment of BEEC/BE patients is to enable normal sexual life and fertility. However, only a few outcome studies have focused on these issues with contradicting results, most of them not using standardized outcome measures. In accordance with other studies, our female BE patients have dyspareunia and most of our male BE patients were able to ejaculate. But the question of normal force of ejaculation, ejaculated volume, or semen analysis remains unanswered. Despite partial confirmation of previous findings, there is inconsistency referring to the outcome measured by the available studies. This might in part be explained by the fact that, other than this study, most previous studies are the result of single-institution experience. Thus, selection bias in the patient sampling due to different a clinical collective in different hospitals may be the consequence. Furthermore, patients' honesty and self-reflection in answering difficult questions regarding their sexual and cosmetic impairments is questionable. In addition, studies include a wide range of age groups and are connected with this life period. Fears and condition-specific anxieties might change over time. Hence, the strengths of this study are the nationwide and treating physician-independent data acquisition as well as the large sample size of adult patients with a very rare congenital malformation. Unfortunately, more detailed analyses on sexual function and current psychosocial situation, for example correlation of data with clinical symptoms such as continence status, was not possible as data were mainly not answered by patients., Conclusion: To improve the quality of life of patients with BEEC/BE, treatment and follow-up should emphasize physical but also psychological care in these patients. Physicians should further re-evaluate their preconceptions and should take care of the patients throughout their lives., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To evaluate health-related quality of life (HRQoL) and psychiatric symptom aspects in patients with bladder exstrophy and epispadias., Methods: Sixty-two adolescents or adults operated for BEE were mailed questionnaires evaluating HRQoL (RAND-36 questionnaire) and psychiatric symptoms (SCL-90 questionnaire). Thirty-two patients with a median age of 28 years responded. The results were compared with national reference values., Results: Overall, RAND-36 scores and SCL-90 scores were comparable in the patients and the referral population. However, 7 patients (21%) had high SCL-90 scores, suggesting mental health problems. The patients with unsatisfactory genital appearance, urinary incontinence, or bladder augmentation tended to have poorer HRQoL and psychiatric symptom scores., Conclusion: Most adults with BEE have satisfactory HRQoL and mental health. However, a possible mental health problem can be found in one-fifth of the patients. Abnormal genitalia and bladder function may have a negative effect on HRQoL and mental health. Bladder augmentation is not associated with better outcome than mild incontinence., (Copyright © 2015 Elsevier Inc. All rights reserved.)

An unusual case report to stress the complexity of development of identity and intimacy in people with bladder exstrophy. The psychosocial support seems to play a fundamental role to promote self-esteem of abdominal scarring and altered genital appearance., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Bladder Exstrophy and Epispadias Complex (BEEC) is associated with an increased risk of impaired mental health, quality of life, and psychosocial functioning. Therefore, screening patients to help identify and evaluate potential psychosocial difficulty is arguably an important consideration for BEEC Services., Objective: To screen paediatric BEEC patients for a range of general psychosocial difficulties in a multi-disciplinary out-patient clinic setting., Study Design: This cross-sectional evaluation was conducted between April 2012 and July 2013. Families attending BEEC multi-disciplinary out-patient clinics were asked to complete a range of standardised psychosocial questionnaires, including the Paediatric Quality of Life Inventory (PedsQL 4.0 Generic Core and Family Impact Module), the Strengths and Difficulties Questionnaire (SDQ), the Paediatric Index of Emotional Distress (PI-ED), and the Hospital Anxiety and Depression Scale (HADS). 108 children attended clinic of which 80 (74.1%) patients and their parents/carers completed some or all of the questionnaires. The mean patient age was 8.41 years (SD = 4.46, range = 1-18 years). There were more boys (N = 50, 62.5%) and the majority had a diagnosis of classic bladder exstrophy (N = 51, 63.8%), followed by primary epispadias (N = 22, 27.5%) and cloacal exstrophy (N = 7, 8.7%)., Results: Mean total scores fell within the average/normal range on all questionnaires used (See table below). However, variation around these means was high. Age, gender and diagnosis were found to significantly influence certain questionnaire responses with older-age groups, males, and those with classic bladder exstrophy particularly at risk across some domains. The children/adolescents self-reported better health related quality of life (HRQoL) scores than published results for a range of paediatric chronic health conditions. Differences between parent and child responses on both the PedsQL and SDQ favoured a more positive response on the child self-report questionnaire but were not statistically significant., Discussion: Mean scores on the measures used suggest a relatively optimistic picture of general psychosocial well-being, especially for HRQoL, in the BEEC population studied. Positive HRQoL outcomes have recently been reported for BEEC paediatric populations. Our results reflect this trend with better mean HRQoL scores than paediatric patients with a range of other chronic health conditions. However, this optimism is cautious given the limitations of this evaluation study and the high variation around the means. Limitations included the small sample size (especially for patients with cloacal exstrophy), the lack of a control group, the limited sensitivity of generic questionnaires in respect of BEEC-specific issues, and the low mean age of patients in the study. Future screening programmes may wish to consider measuring BEEC-specific variables (e.g. satisfaction with genital appearance/function); collecting information on medical aspects, such as continence, pubertal stage and frequency/timing of medical intervention; and asking both parents/carers (where possible) to complete the questionnaires., Conclusions: Screening questionnaire responses were used in conjunction with clinical psychology consultations to evaluate a range of psychosocial aspects in BEEC paediatric patients. Whilst mean scores on the measures used suggest a relatively optimistic picture, certain individual scores did fall within the clinical ranges, highlighting the potential need for further assessment. Developmentally tailored consultations with a clinical psychologist can provide detailed information around questionnaire responses and further assess BEEC specific aspects., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To investigate sexual function and quality of life in adolescent and adult women with classic bladder exstrophy (BE)., Materials and Methods: A two-part observational cross-sectional study with a questionnaire arm and a retrospective case review arm was performed. The study was undertaken in a centre providing a tertiary referral gynaecology and urology service. Outcomes were sexual function and quality-of-life scores., Results: A total of 44 patients with BE were identified from departmental databases and included in the study, of whom 28 (64%) completed postal questionnaires. Sexual function scores and quality-of-life visual analogue scales were significantly poorer compared with normative data., Conclusions: Bladder exstrophy has a detrimental psychological impact on women. In future, methodical multidisciplinary paediatric follow-up research will help to identify predictors of better and worse adolescent and adult outcomes. Development and evaluation of cost-effective psychological interventions to target specific problems is also warranted., (© 2014 The Authors. BJU International © 2014 BJU International.)

Background: Postoperative complications are related to the surgical procedures, of failures of initial bladder closure and influence the urological, aesthetical and orthopaedic outcomes., Materials and Methods: We reviewed four patients who underwent complex bladder exstrophy-epispadias repair over a period of 14 years. The outcomes of treatment were assessed using, aesthetic, urological and orthopaedic examination data. Orthopaedic complications were explored by a radiography of the pelvis., Results: Out of four patients who underwent bladder exstrophy surgical management, aesthetic, functional outcomes and complications in the short and long follow-up were achieved in three patients. The first patient is a male and had a good penis aspect. He has a normal erection during micturition with a good jet miction. He has a moderate urinary incontinence, which requires diaper. In the erection, his penis-measures 4 cm long and 3 cm as circumference. The second patient was a female. She had an unsightly appearance of the female external genitalia with bipartite clitoris. Urinary continence could not be assessed; she did not have the age of cleanness yet. The third patient had a significant urinary leakage due to the failure of the epispadias repair. He has a limp, a pelvic obliquity, varus and internal rotation of the femoral head. He has an inequality of limbs length. Pelvis radiograph shows the right osteotomy through the ilium bone, the left osteotomy through the hip joint at the acetabular roof., Conclusion: When, the epispadias repair is performed contemporary to initial bladder closure, its success is decisive for urinary continence. In the female, surgical revision is required after the initial bladder closure for an aesthetic appearance to the external genitalia. Innominate osteotomy must be performed with brilliancy amplifier to avoid osteotomy through to the hip joint to prevent inequality in leg length.

Purpose: Research on health related quality of life in patients with bladder exstrophy has demonstrated mixed results compared to population samples. Few studies have had a comparison group with a urological disorder and none correlated body image to health related quality of life. We compared health related quality of life in patients with bladder exstrophy to that in patients with kidney stones and correlated body image to health related quality of life in the bladder exstrophy population., Materials and Methods: Participants included 24 patients with kidney stones and 24 with bladder exstrophy. Patient age was 8 to 25 years. Participants completed the PedsQL (Pediatric Quality of Life Inventory) and the 25-item multiple choice UBIQ (Urological Body Image Questionnaire), which was created for the current study., Results: Compared to patients with kidney stones those with bladder exstrophy indicated significantly higher scores on physical functioning (p = 0.0005) and overall health related quality of life (p = 0.02) in the 8 to 17-year-old cohort. When health related quality of life was compared to body image in patients with bladder exstrophy, lower social scores were associated with those who worried about friends finding out about the condition (p = 0.01) and about having romantic relationships (p = 0.003). Lower social (p = 0.006) and emotional (p = 0.009) functioning scores were associated with patients who thought that they were less likely to have romantic relationships., Conclusions: The finding that patients with bladder exstrophy had a better physical functioning score than those with kidney stones is not surprising, given the morbidity of active stone disease. The negative correlations with body image in the bladder exstrophy population may be due to the possibility of others discovering the condition and the perceived likelihood of not having romantic relationships., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the emotional and behavioral functioning of children with bladder exstrophy-epispadias complex (BEEC), taking into account developmental and gender considerations. This study also sought to overcome methodological limitations of previous studies evaluating psychological well-being of children with BEEC., Methods: Eighty-six children were consecutively evaluated using the parent report version of the Behavior Assessment System for Children during visits to a multidisciplinary urology clinic., Results: Results indicated normative emotional and behavioral functioning across the sample. However, there was a significant effect of age, such that older children consistently had worse internalizing symptoms and adaptive functioning. Males tended to have more externalizing problems as they aged, and also tended to have lower levels of adaptive functioning but this was independent of age. The level of psychological impairment was unrelated to the specific type of BEEC, and was also unrelated to whether or not the patient had undergone continence surgery., Conclusion: Children with BEEC have a greater likelihood of experiencing a wide range of emotional and behavioral problems as they reach adolescence. These findings point to the need to prevent potential psychological distress by intervening with these children before they become clinically impaired., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To examine long-term quality-of-life, urinary continence and sexual function outcomes in patients diagnosed with bladder exstrophy (BE)., Patients and Methods: A total of 65 patients with BE and follow-up of at least 20 years were identified. After informed consent for inclusion in the study, the patients were asked to complete three validated questionnaires, the Short-Form 36 quality-of-life questionnaire (SF-36), the International Consultation on Incontinence Questionnaire (ICIQ) and the International Index of Erectile Function (IIEF), to assess quality of life, perceived urinary continence and sexual function., Results: In all, 21 patients responded to the questionnaires, yielding a 32% response rate. High scores in each of the eight dimensions of the SF-36 reflected a positive perception of quality of life by respondents; calculated scores were compared with those of a normal control group. The only significant difference found between the groups was that the study population perceived their general health to be poorer than those in the control group. High scores on the ICIQ indicate high levels of subjective incontinence in patients, with scores ranging from 0 to 21. Reporting scores of 0 (continent), 12/21 patients perceived their continence to be normal, 9/21 patients had scores >2 (mild), with one patient scoring a 16, and 11 (severe incontinence), patients reported no identifiable leakage during normal activities. There was a 29% response rate for the IIEF (15 patients completed this). IIEF scores were broken down into five dimensions and mean scores were calculated. The mean scores showed mild to moderate dysfunction in each category, including overall satisfaction with sexual experience., Conclusions: The patient-reported quality of life in patients with BE was normal in all dimensions, with the exception of perception of general health. Half of the patients reported normal continence and had no complaints of urinary leakage. Sexual function in males was significantly affected across all dimensions, with mild to moderate dysfunction., (© 2013 The Authors. BJU International © 2013 BJU International.)

Previous research investigating the psychosocial and psychosexual impact of living with the complex genitourinary condition bladder exstrophy has been limited in scope and methodological quality. However, the limited evidence suggests that people with bladder exstrophy commonly encounter difficulties that might negatively impact their experiences of intimacy. We conducted an interpretative phenomenological analysis to explore intimacy in 6 participants aged 16 to 56 years. Participants discussed how their parents and later they themselves concealed their health condition. This concealment was associated with feeling safe and protected, yet shameful. Participants also discussed developing intimate knowledge of their own emerging identity while developing intimacy with others, as well as the importance of sharing the experience of bladder exstrophy with others in the development of intimate relationships. We discuss the findings in relation to theoretical issues of concealment, shame, attachment, psychosocial development, intimacy, and chronic illness.

Purpose: Although research in patients with urological conditions indicates that functional limitations, pain, limited mobility and social restrictions can impact self-image, to our knowledge the influence of the visibility of the condition has not been examined. We assessed body image and self-image at school age through young adulthood in patients with bladder exstrophy compared to patients with kidney stones., Materials and Methods: Patients diagnosed with bladder exstrophy and kidney stones completed a 25-item multiple choice Urological Body Image Questionnaire for ages 13 to 25 years, which was created for the current study. It consists of questions on physical appearance, avoidance behaviors, urological condition disclosure, romantic relationships and future expectations. A modified Urological Body Image Questionnaire for patients 8 to 12 years old was created by removing questions on relationships and future expectations., Results: A total of 24 patients with bladder exstrophy and 24 with kidney stones 8 to 25 years old completed the questionnaire. Compared to patients with kidney stones, patients with bladder exstrophy were significantly more likely to avoid changing clothes in front of peers and to not tell friends about the condition (each p = 0.0001). Patients with bladder exstrophy were also significantly more likely to think that the condition would prevent having future romantic relationships (p = 0.002) and children (p = 0.003)., Conclusions: Differences in the visibility and privacy of the 2 conditions likely explain the discrepancy between the groups. Findings may call for an individual approach to assessing issues related to self-esteem and identity in patients with bladder exstrophy and may highlight the importance of receiving psychosocial support., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Today, young individuals with rare congenital anomalies as the Exstrophy-Epispadias-Complex (EEC) are mostly monitored interdisciplinary with a high standard of care and enthusiasm during childhood. However, when growing up through adolescence to adulthood adequate care-givers are not available at the moment in adult medicine in Germany and a concrete transition process has yet not been established. Over the past years, we put much effort in systematic evaluation of long-term outcome after reconstruction of the EEC in the newborn period to further improve outcome results. Beside predictive parameters for continence and long-term bladder function, genital function and fertility, as well as postoperative pelvic floor morphology and gynecological outcome, orthopedic results and psychosexual and psychosocial development in EEC were of major interest. As a consequence we currently develop a German-wide follow-up concept in EEC patients regarding age- and gender specific outcome issues. Long-term observations of the EEC outcome however, underline the unrestricted importance of careful long-term follow-up of all EEC patients, as well as the necessity of close cooperation of pediatric urologist, pediatric surgeons, urologists, orthopedic surgeons, gynecologists, andrologists, psychologists and urotherapists from early childhood and the need of knowledge transfer and hopefully a successful transition of the EEC individuals to general medicine., (© Georg Thieme Verlag KG Stuttgart · New York.)

Despite improvements in modern surgical reconstructive techniques, many patients with epispadias-exstrophy continue to experience urinary incontinence. Continent diversion is commonly performed to achieve urinary continence and improve quality of life. In this work we describe the population that can be considered for continent urinary diversion, consider the benefits and implications of concurrent augmentation and bladder neck closure, and review recent literature regarding continence outcomes and common complications. Even in this complex patient population, urinary continence can be reliably achieved by bladder augmentation and the use of intermittent catheterization via a catheterizable cutaneous stoma with or without closure of the bladder neck., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may persist or transform as the child grows. The pediatric urologist is ideally situated to identify signs or symptoms of early developmental psychopathology in these children. Presented in this article are techniques for identifying the child requiring full assessment and for establishing referral-consultants. Screening instruments are suggested, as well as how to use these to educate the parents and the child. Methods are provided to identify, as well as to educate, selected consultants in child psychology and psychiatry about the clinical realities of exstrophy., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of genitourinary malformations requiring multiple major reconstructive operations on each affected child. The need for surgical correction in this condition often continues through adolescence and into adulthood. Experience in caring for individuals with exstrophy-epispadias has taught us a great deal about the long-term functional, psychological, and social outcomes involved. Children undergoing repeated hospital admissions and extensive multiple operations have the potential for long-term adjustment problems with incontinence, ambulatory difficulties, psychological disturbance, sexual dysfunction, and issues surrounding self-esteem and social integration. By examining relevant published works from the world literature over the last 20 years, in this article we address with each of these areas and offers some insight into the ongoing issues., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: The aim of the study was to assess quality of life (QOL) of patients born with bladder exstrophy (BE) and reconstructed during early childhood in 7 French university hospitals (QUALEX study: QUAlity of Life of bladder EXstrophy)., Methods: Patients from 6 to 42 years old answered self-administered Short-Form 36 (SF-36), VSP-A (Vécu et Santé Perçue de l'Adolescent), VSP-AE (Vécu et Santé Perçue de l'Enfant), AUQUIE (AUto-QUestionnaire Imagé de l'Enfant), and general questionnaires about functional and socioeconomic data. Dimension scores were compared between adults and adolescents using SF-36 and adolescents and children using VSP-AE. Scores were also compared to the general French population., Results: Among the 134 eligible patients, 36 adults, 18 adolescents, and 17 children answered the questionnaire. There was no difference between responders and nonresponders in reconstruction criteria. Continence was achieved in 77% of adults, 65% of adolescents, and 12% of children. Adolescent QOL was globally superior to adults and children. Adult QOL was globally lower than the general population except on the physical dimension. Children's QOL was also globally lower than the general population except for relations with family and school work. Adolescents' scores on SF-36 were superior to the general population but lower on half of the dimensions with VSP-AE., Conclusion: Patients presenting with reconstructed BE have impaired QOL, and functional results seem to be the most likely predictive factor of health-related QOL score., (Copyright 2010 Elsevier Inc. All rights reserved.)

Bladder exstrophy (BE) is a rare but severe urogenital malformation that requires complex clinical management. It can lead to physical restriction as well as to psychosocial and psychosexual malfunction. For the first time, this qualitative study describes the experiences of persons living with BE from childhood into adulthood. The primary goal was to explore the experiences of persons with BE in order to optimise treatment, care and counselling at the children hospital in Zurich. Semi-structured interviews were conducted with 4 women and 16 men (38 to 68 years). Using content analysis techniques, three major categories with two respectively four and five subcategories emerged from the data. The first category "having a good life despite BE" describes the perception of the affected persons to lead a life worth living despite BE. It was categorised by episodes and experiences that were repeated throughout the persons' life, such as "being in hospital", "living with symptoms", "being different", "showing oneself" and "gathering information". The second category "the affected ones" demonstrated that "parents" and "partners" were affected too and provided considerable support to the person with BE. The third category "the influencing factors" revealed that the perception of living with BE was influenced by individual and contextual factors. The results of this study illustrated that persons with BE can live a good life, despite repeated health-related challenges, if the support provided by their caregivers permits a positive attitude towards their illness. For this, patients with BE and their family members require continuous and highly competent treatment, care and education by an interdisciplinary team.

Objective: There is a paucity of knowledge about long-term outcome issues in the bladder-exstrophy-epispadias complex (BEEC). Adult male BEEC patients were investigated in respect of bladder and renal function, fertility, genital function and psychosocial facts., Patients and Methods: In a cross-sectional study, 17 adult male BEEC patients (mean age 23.4 years) from a single centre were evaluated with a questionnaire, renal and bladder ultrasound, blood tests, hormonal profile and semen analysis., Results: Phenotypically one patient had complete epispadias and 16 had classical bladder exstrophy. Five patients underwent a one-stage functional reconstruction as a primary and 12 as a redo procedure. After a mean follow-up of 19.4 years, 15 bladders were preserved with 12 voiding per urethram and 3 performing intermittent catheterization; 2 were secondarily diverted. Significant residual urine was present in 10; kidneys were normal in 14 patients. Sixteen patients proved ejaculations, 3 had normospermia, 7 oligoasthenospermia and 6 azospermia. In patients with only one single bladder neck procedure normospermia was statistically significant., Conclusion: After functional BEEC reconstruction, long-term bladder function is preserved with mostly normal renal function. The number of bladder neck attempts has a significant influence on andrologic outcome. Detailed analysis may detect multifactorial pathogenesis from the impaired sperm quality in the BEEC.

Purpose: We describe the long-term psychosocial and sexual outcomes of patients born with bladder exstrophy treated with ureterosigmoidostomy at our institution., Materials and Methods: Of 42 patients born with bladder exstrophy between 1937 and 1968, 28 were located, of whom 25 agreed to participate in this study. Evaluation consisted of studying pediatric and adult medical records, medical examination, and reports of sexual and social history., Results: Four female and 21 male patients with a mean age of 50 years (range 39 to 67) were evaluated. Of the patients 21 (84%) were married or lived in a stable relationship, 6 (24%) had several children after normal conception and an additional 2 (8%) became parents with assisted reproductive technology. Nine patients (36%) reported no sexual activity or were unable to engage in sexual intercourse. All patients except 2 are professionally and socially successful., Conclusions: The long-term followup of this study of 37 to 68 years provides valuable information on patients who live with bladder exstrophy during a long period. Most followed patients who were born with bladder exstrophy and treated with ureterosigmoidostomy were able to lead satisfactory lives socially and professionally. However, at least 36% of the patients are severely restricted in their sexual lives.

Purpose: Currently chronic illness is not a recognized risk factor for suicide in children. Therefore, we evaluated vulnerability for suicidal behavior (suicide attempt and suicidal ideation) in our population of young patients with classic bladder exstrophy., Materials and Methods: We reviewed the charts of 121 patients 5 to 24 years old with classic bladder exstrophy only from a combined, 2-center database of 935. These patients were psychologically evaluated from 1996 to 2005 for preoperative readiness for urological surgery or for coping with medicosurgical health requirements., Results: Of 121 patients 18 (14.9%) experienced suicidal ideation. Of 38 patients (31.4%) older than 14 years 11 experienced suicidal ideation, 2 experienced a serious suicidal attempt and 1 completed suicide. In 36 patients (30%) there were no recorded data on suicidal behavior. All patients with suicidal behavior were male., Conclusions: Data from this study of classic exstrophy imply that there are clinically significant vulnerabilities for suicidal behavior in affected male children, adolescents and young adults. These findings argue for screening those with classic exstrophy for suicidal behavior and psychopathology.

Purpose: To examine the experiences of young people living with bladder exstrophy., Methods: Qualitative interviews with 16 adolescents aged 16-21 years, exploring the psychological and social impact of bladder exstrophy., Results: Participants described difficulties, solutions, and practical assistance needed to manage living with bladder exstrophy. Participants identified practical difficulties, their identities, and relationships as the three main areas of importance. They discussed challenges associated with disclosing the nature of their condition, being bullied, and sexual relationships. Despite the difficulties and challenges described, young people repeatedly expressed a determination to live their lives as normally as their peers did., Conclusion: Many examples of difficulties experienced in the past and the present are described. Young people report a range of creative coping strategies used to respond to the demands of living with a chronic health condition.

Purpose: Psychological functioning, as reflected in psychopathology, psychosocial functioning, gender identity, gender role and cognitive abilities, was examined in a sample of patients with cloacal exstrophy., Materials and Methods: Nine participants 11 to 37 years old completed the evaluation. Standardized measures were used and participant responses were compared to the norms of the various instruments. Gender reassigned participants with cloacal exstrophy were compared with nonassigned participants with cloacal exstrophy. Mean differences between the 2 groups were calculated using the t test., Results: Overall the participants with cloacal exstrophy reported good psychological functioning. Significant differences between reassigned and nonassigned participants were found in the area of depression. All participants had a stable gender identity. XY females showed more male-typical gender roles. There were no significant group differences on cognitive assessments. Reassigned and nonassigned participants did not differ in IQ., Conclusions: Although being born with cloacal exstrophy puts patients at risk for psychopathology and psychosocial problems, it does not necessarily mean that these problems will develop. With the appropriate support these patients can have remarkably well adjusted lives.

Purpose of Review: The patient with bladder exstrophy presents complex surgical and clinical management challenges to the pediatric urologist. This review aims to present recent developments in the care of these patients as well as to review contemporary medical outcomes., Recent Findings: Most of the early literature pertaining to bladder exstrophy has focused on surgical techniques and management. In the last 10-20 years, however, there has been an increase in the number of publications dealing with long-term outcomes in these patients. Psychosexual development, sexual function and social adaptation have received as much attention as urinary continence status, bladder, and renal function. This review will examine these topics., Summary: Despite the complex nature of bladder exstrophy, there are no well designed, prospective trials examining clinical outcomes in these patients. Until better trials are planned, executed and published, much of what we know about patients with bladder exstrophy will be based on retrospective observations with significant biases.

Objectives: To evaluate the hypothesis that anxiety disorders are common comorbid conditions in children with the epispadias-exstrophy complex., Methods: Twenty consecutive outpatient exstrophy subjects ranging in age from 5 to 22 years were assessed using a formalized semistructured psychiatric evaluation and were categorized according to the Diagnostic and Statistical Manual of Mental Disorders, version IV, criteria. Disorders were identified if subjects endured moderate to severe impairment in their home, academic, and social environments., Results: All 20 subjects met the criteria for at least one anxiety disorder; 19 met criteria for more than one anxiety disorder. The adolescent subjects described a gradual waning of some specific symptoms some time after surgical correction of the physical conditions (eg, incontinence) but intensifying sexual anxiety with age., Conclusions: The epispadias-exstrophy complex appears to be associated with clinically significant vulnerabilities for anxiety disorders in children.

Classic bladder exstrophy is characterized by displaced pelvic floor musculature and significant skeletal and genitourinary defects. A paucity of data exist evaluating long-term pelvic floor function in exstrophy patients after ureterosigmoidostomy. This study is an initial attempt to evaluate the prevalence of urofecal incontinence, pelvic organ prolapse, and overall quality of life in patients who have had ureterosigmoidostomies. Fifty-two individuals who underwent ureterosigmoidostomy between 1937 and 1990 were identified through the Ureterosigmoidostomy Association and the Johns Hopkins bladder exstrophy database and mailed questionnaires approved by the Institutional Review Board (Johns Hopkins). Data were analyzed with SigmaStat 3.0 (SPSS, Inc., Chicago, IL). Eighty-three percent of the subjects responded, with a mean age of 44.4 years (range, 14-73 years) and mean of 40.9 years (range, 14-65 years) after ureterosigmoidostomy. Prevalence of daily urinary and fecal incontinence was 48% (n = 20) and 26% (n = 11), respectively, whereas the prevalence of weekly combined urofecal incontinence was 63% (n = 27). The incidence of pelvic organ prolapse in this cohort was 48% (n = 20). In these patients, a significant risk of urofecal incontinence and pelvic organ prolapse exists. Long-term follow-up studies are needed to understand the role of pelvic floor musculature in this complex birth defect.

Cloacal exstrophy is an extremely rare and devastating complex of congenital abnormalities resulting in multiple debilitating morbidities. Infants with this disorder require numerous surgical interventions to repair gastrointestinal, genitourinary, spinal, and orthopedic malformations. Treatment involves the combined efforts of many medical and nursing specialists. This article discusses the embryology, clinical presentation, and management (both early and long term) of cloacal exstrophy, with emphasis on gender reassignment issues and quality of life.

Objective: To identify problems in the long-term psychosocial and developmental outcome specific to patients with the bladder exstrophy-epispadias complex (BEEC), using a self-developed semi-structured questionnaire, as there are various techniques of reconstruction to repair BEEC but to date neither patients nor surgeons have a clear answer about which type gives the most acceptable long-term results., Patients and Methods: Increasingly many patients with BEEC reach adulthood and wish to have sexual relationships and families. To date, no studies have used disease-specific psychological instruments to measure the psychosocial status of patients with BEEC. Thus we contacted 208 patients with BEEC, and 122 were enrolled, covering the complete spectrum of the BEEC. The data assessed included the surgical reconstruction, subjective assessment of continence, developmental milestones, school performance and career, overall satisfaction in life, disease-specific fears and partnership experiences in patients aged >18 years. We compared affected females and males to assess gender-associated differences in quality of life., Results: Affected females had more close friendships, fewer disadvantages in relation to healthy female peers and more partnerships than the males. Family planning seemed to be less of a problem in affected females. There were no gender differences in the adjustments within school and professional career, which was very good in general., Conclusion: Future studies are needed to assess the disease-specific anxieties, considering gender-specific differences.

Purpose: We report the psychosocial and psychosexual development of children and adolescents with the exstrophy-epispadias complex (EEC) after complete functional repair using the Erlangen single stage technique., Materials and Methods: In a long-term retrospective followup of an average of 11.1 years 100 patients with EEC (76 boys and 24 girls, mean age 14.5 years) were evaluated with respect to medical history, and received a general questionnaire concerning their social and psychosocial situation. A total of 54 patients who were 15 years or older (mean age 18.5) received an additional questionnaire to assess detailed sexual history., Results: Of the patients 81% returned the general questionnaire within 3 weeks. School education level and social integration were high. In about 25% of the patients impairment of daily life was significant, and in 58.7% peer relations were altered. Of the adolescent group 76% answered the special questionnaire. Genital satisfaction and genital touching were rated low, and avoidance of nudity in public areas was common. All patients expressed heterosexuality and 43.9% had engaged in sexual intercourse but 58.5% displayed anxiety about sexual activity. It is noteworthy that 93.9% expressed an interest in psychological assistance., Conclusions: Despite a high degree of social integration and adult adaptation, children and adolescents with EEC suffer from psychosocial and psychosexual dysfunction requiring special questionnaires for adequate assessment. Anxiety about genital appearance and sexual activity is a common phenomenon among adolescents with EEC, even when they present with nearly "normal" genitalia and participate with satisfaction in sexual activity. Further studies are needed to understand the exstrophy problem and supply all patients with EEC with the individual care they need.

The bladder exstrophy-epispadias complex (BEEC) belongs to one of the most devastating urological malformations affecting the complete urinary tract including the genitalia. A semi-standardized questionnaire was used to evaluate the functional and psychosocial developmental outcome in 122 affected patients recruited from German-speaking self-referral support groups. The questionnaire covered mode of reconstruction, subjective assessment of continence, milestones of child development, education, school performance, level of life satisfaction, anxieties, and in patients older than 16 years partnership experience. The results showed a mean of one surgery in the age group 0-4, of four surgeries in both age groups 5-13 and 14-20, and seven surgeries in patients aged 20 and over. The continence rates for the latter three age groups were 36, 64, and 80%, respectively. We found that the neurocognitive development was unremarkable. Most patients showed above average performance in school and in their profession. On the other hand, they exhibited psychological anxieties and worries in their experience with sexuality and partnerships. Future studies will be necessary to clarify the association of urological status, functional impairments, and psychosocial adaptation as a basis for improved approaches to comprehensive care and support.

Objective: To determine the factors that control quality of life as perceived by adolescent patients with bladder exstrophy, and to compare their views using standard instruments., Patients and Methods: Sixteen patients (mean age 19 years, range 16-21, 11 male and five female) were recruited from the departmental database; they represented 46% of those available for the study. After giving informed consent, each had a semi-structured interview, augmented by completing a self-reported scale, with the principal investigator. They then completed the Culture-Free Self-Esteem Inventory-2 (CFSEI-2) and the Brief Symptom Inventory (BSI). The interviews were recorded on audiocassette, transcribed verbatim and evaluated using interpretative phenomenological analysis., Results: In the interviews there was a remarkable consistency in the domains identified as important to the patients. There was a wish to be normal and to be treated as such. This was defined (amongst other items) as being able to void with an appropriate noise, being treated as peers at school and at home, and having an umbilicus. All patients reported some bullying (all but one in the past), but only severely in three. Concerns about self-image centred on scars and genital appearance. Very similar and effective coping strategies had been created, including practical (e.g. suitable clothes) and emotional (e.g. joking, control of revealed information) aspects. Special arrangements made to help (care by a special assistant or use of a disabled lavatory at school) served only to emphasize their abnormality and were resented. No overt psychiatric or psychological morbidity was detected. There was no difference in scores with the CFSEI-2 or BSI from established age-related norms., Conclusion: This study confirms the anecdotally reported strong resilience and personality of adolescents with exstrophy. The domains that patients considered important were not those that their carers might have expected or that are used in standard quality-of-life instruments. No morbidity was identified by the two instruments used. In exstrophy, and perhaps in other uncommon conditions, the patients' views of relevant domains should be considered in assessing quality of life.

Objectives: To report the long-term surgical outcome in a group of patients with bladder exstrophy treated from 1960 to 1982, and to assess physical health, social integration and sexual function, as attempts at functional closure during the development of this surgery resulted in patients with differing surgical status of the genitourinary organs., Patients and Methods: A review of medical record archives revealed 36 patients; of these, seven had died, six were untraceable and six declined to participate, leaving 15 evaluable subjects (seven men and eight women, mean age at follow-up, 35 years). Data were collected from medical records and direct interviews were conducted using a semi-structured questionnaire, after obtaining informed consent. The Short Form-36 (SF-36) v2 Health Survey instrument was used to assess health and well-being., Results: Three patients have retained the use of their bladder, and 11 had initial bladder closure but required (at a mean age of 3.1 years) various urinary diversions. Eight patients had a pelvic osteotomy. Of 27 functioning renal units, 25 are in reasonable to good condition. Six men and six women had genital reconstruction. Four men are capable of penetrative intercourse and ejaculate; six women manage penetrative intercourse and five have orgasms. All patients attended mainstream school and 13 achieved examination success. Nine patients took vocational training and 11 work full-time. Five patients are married and five are in long-term relationships. Two men have achieved three pregnancies and one women has had a child. The mean total SF-36 score (maximum 3600) was 2763 in men and 2235 in women., Conclusions: Surgery for bladder exstrophy has been developing for more than 40 years and the legacy of early attempts at functional closure is a population of adults who have a diversity of lower urinary tracts, good preservation of renal function overall and acceptable sexual function. They are usually robust, healthy and well-adjusted individuals functioning well in society, often in full-time employment and long-term relationships. Adolescent follow-up must be clearly focused, incorporating a multidisciplinary team approach to facilitate a seamless transition into adulthood.

Background: Cloacal exstrophy is a rare, complex defect of the entire pelvis and its contents that occurs during embryogenesis and is associated with severe phallic inadequacy or phallic absence in genetic males. For about 25 years, neonatal assignment to female sex has been advocated for affected males to overcome the issue of phallic inadequacy, but data on outcome remain sparse., Methods: We assessed all 16 genetic males in our cloacal-exstrophy clinic at the ages of 5 to 16 years. Fourteen underwent neonatal assignment to female sex socially, legally, and surgically; the parents of the remaining two refused to do so. Detailed questionnaires extensively evaluated the development of sexual role and identity, as defined by the subjects' persistent declarations of their sex., Results: Eight of the 14 subjects assigned to female sex declared themselves male during the course of this study, whereas the 2 raised as males remained male. Subjects could be grouped according to their stated sexual identity. Five subjects were living as females; three were living with unclear sexual identity, although two of the three had declared themselves male; and eight were living as males, six of whom had reassigned themselves to male sex. All 16 subjects had moderate-to-marked interests and attitudes that were considered typical of males. Follow-up ranged from 34 to 98 months., Conclusions: Routine neonatal assignment of genetic males to female sex because of severe phallic inadequacy can result in unpredictable sexual identification. Clinical interventions in such children should be reexamined in the light of these findings., (Copyright 2004 Massachusetts Medical Society)

Objective: To review of the sexual and urogynaecological issues faced by a large cohort of women with the exstrophy-epispadias complex (EEC)., Patients and Methods: The study comprised 83 women and girls with EEC; a confidential survey was mailed to identify their social and sexual concerns. Fifty-six women had classical bladder exstrophy (CBE), 13 had female epispadias (FE) and 14 had cloacal exstrophy (CE). Data on the initial method of reconstruction and urogynaecological problems were obtained from a review of the hospital records. Information on continence, infection and sexual function was obtained from 34 completed surveys., Results: The bladder was closed in 51 patients with CBE and 13 with CE. Urinary calculi developed in 10 patients with CBE, two with FE and three with CE. Vaginal and uterine prolapse occurred an earlier age in patients with EEC. Eight women had 13 pregnancies, eight of which resulted in normal healthy children. Overall continence was achieved in 85% of the women surveyed. Urinary tract infections remained a frequent problem for women with EEC; only 27% of respondents indicated that they were infection-free. Women aged > 18 years (24) who responded indicated that they had appropriate sexual desire; 16 were sexually active and the mean age for commencing sexual activity was 19.9 years. Six patients had dyspareunia and 10 indicated that they had orgasms. However, five additional patients indicated that they had restricted intercourse, as they were dissatisfied with the cosmesis of their external genitalia., Conclusions: Sexual and gynaecological issues become increasingly important in patients with EEC as they become adults. Understanding these issues faced by patients with EEC as they mature will permit better counselling of future patients.