BEEC psychosocial screening at Royal Manchester Children's Hospital.

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.

Health-Related Quality of Life in Patients with the Bladder Exstrophy-Epispadias Complex and Relationship to Incontinence and Sexual Factors: A Review of the Recent Literature.

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.

Patient-Reported Impact of Pelvic Organ Prolapse on Continence and Sexual Function in Women With Exstrophy-Epispadias Complex.

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.

Psychosocial screening at paediatric BEEC clinics: a pilot evaluation study.

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.

Aesthetic, urological, orthopaedic and functional outcomes in complex bladder exstrophy-epispadias's management.

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.

Emotional and behavioral functioning in children with bladder exstrophy-epispadias complex: a developmental perspective.

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.

Continent urinary diversion in the epispadias-exstrophy complex.

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.

Exstrophy in the adolescent and young adult population.

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.

A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex.

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.

Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study.

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.

Long-term followup (37 to 69 years) in patients with bladder exstrophy treated with ureterosigmoidostomy: psychosocial and psychosexual outcomes.

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.

Suicide and suicidal ideation in classic exstrophy.

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.

Psychopathology, psychosocial, gender and cognitive outcomes in patients with cloacal exstrophy.

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.

An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy.

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.

[Subjective developmental outcome in bladder exstrophy and epispadias. A pilot study]. / Entwicklungsergebnis bei Blasenekstrophie und Epispadie aus Patientensicht Eine Pilotstudie.

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.

Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery.

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.

Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex.

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.

Bladder exstrophy: psychological impact during childhood.

PURPOSE: We describe the impact of bladder exstrophy on the behavior, self-esteem and quality of life of children as well as on the parents, and analyze the need for psychological intervention. MATERIALS AND METHODS: All 7 boys and 8 girls 3 to 18 years old (median age 11) under treatment at a tertiary pediatric surgery clinic were included in our followup study. Medical and psychological evaluations were performed. Behavior was assessed using the semistructured Höök-Cederblad Child Behavior Interview and the Child Behavior Checklist questionnaire. Self-esteem was assessed by the self-rating I Think I Am questionnaire. Children and parents were interviewed separately. Quality of life was estimated using the Multiattribute Health Status Mark II classification system. RESULTS: After repeat operations and hospitalization 10 children were dry, although 9 required catheterization. Four children had some behavioral problems, which were manifest in 2. All but 1 male adolescent had good or very good self-esteem. Quality of life was decreased in most cases due to limited self-care, although emotional problems were few. All mothers had experienced the birth as a traumatic event and 5 parents had had psychiatric symptoms. CONCLUSIONS: Self-esteem may be maintained despite multiple operations, urinary leakage and deviant genitalia but the abnormality had a great impact on children and on the lives of the families. Parents and children required individual intervention from a multidisciplinary team during different stages of childhood.

Bladder exstrophy: staged reconstruction.

The modern staged approach to bladder exstrophy reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron in the 1970s. Although varied surgical approaches have been tried in the bladder exstrophy condition, the staged approach to bladder exstrophy repair has withstood the test of time. Progress continues to be made in evaluating the outcome of older types of staged reconstruction, with continuing modification and improvements in the modern approach to staged reconstruction. This treatise will update the reader on recent advances in the treatment of bladder and cloacal exstrophy.

The outcome of patients with classic bladder exstrophy in adult life.

PURPOSE: We determined the outcome of 20 older adult bladder exstrophy patients regarding urinary continence, sexual function, fertility and psychosocial integration. MATERIALS AND METHODS: A total of 16 men and 4 women completed an anonymous questionnaire and the charts were reviewed for medical history. RESULTS: Of 9 patients who void spontaneously 6 are dry for 4 hours and 3 for 2 hours. Among the remaining 11 patients 5 are dry on clean intermittent catheterization, 3 who had undergone uterosigmoidostomy are dry for more than 4 hours and 3 had an incontinent stoma. Four men and 2 women are married, including 2 men and 1 women who had a total of 7 children. Ten of 16 men (63%) reported that they ejaculate a few cubic centimeters in volume, 3 ejaculate only a few drops and 3 have no ejaculation (2 of whom underwent cystectomy). Semen analyzed in 4 patients (3 with azoospermia and 1 with oligospermia) and average volume of ejaculate was 0.4 cc (range 0.2 to 1). All women reported regular and normal menstrual periods. A total of 15 patients experienced normal erections that were described as satisfactory by 8 and not satisfactory by 6 due to a small penis, with dorsal chordee in 1. Of the 16 men 12 (75%) experienced satisfactory orgasms while 10 had participated in sexual intercourse with complete partner satisfaction in 9. Half of the men and all women describe intimate relationships as serious and longterm. Of the 20 patients 15 (75%) achieved a high level of education. CONCLUSIONS: Bladder exstrophy patients generally achieve good results but the fertility of most men is in doubt.