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.

Delayed exstrophy repair (DER) does not compromise initial bladder development.

OBJECTIVE: Delayed exstrophy repair (DER) represents an alternative to early neonatal bladder closure. This study aims to define the consequence of DER on bladder growth in bladder exstrophy patients who underwent routine DER, compared with those who underwent immediate postnatal reconstruction. METHODS: Between 2000 and 2005, classic bladder exstrophy patients referred to the authors' institution underwent early neonatal bladder closure (group 1). Subsequently, classic bladder exstrophy patients referred to the authors' institution were treated with an elective DER (group 2). Bladder capacity was assessed between the age of 1 and 4 years with an unconscious cystogram. When dilating VUR was present, the volume of the contrast migrated into the ureter was calculated and subtracted. RESULTS: Sixty patients were treated between 2000 and 2012. Complete follow-up data were available for 45 patients and they were included in the study: 21 in group 1 (11 males) and 24 in group 2 (14 males). The mean (SD) bladder volumes were 72.85 (28.5) ml in group 1 and 72.87 (34.9) in group 2 (p = 0.99). CONCLUSION: In the authors' experience, DER does not reduce the subsequent bladder capacities compared with neonatal exstrophy closure.

Antenatal diagnosis of bladder/cloacal exstrophy: challenges and possible solutions.

OBJECTIVE: To identify the pitfalls in accurate antenatal diagnosis of bladder exstrophy (BE) and cloacal exstrophy (CE), and thus understand the challenges for antenatal counselling. METHODS: A prospectively maintained bladder exstrophy database of antenatal and live born referrals for BE/CE was used to identify patients. Data were collected about the antenatal scan findings and the outcome of pregnancy. RESULTS: Between 2003 and 2009, 40 new babies with BE/CE were referred and of them 10 had an antenatal diagnosis. Five patients did not have a diagnosis despite suspicious findings noted on antenatal scans and another three had a wrong diagnosis of BE/CE. Of the 16 referrals with antenatal suspicion of BE/CE, 5 opted for termination. At the 20-week scan, it was possible to identify the gender of the fetus in 3/16 cases only. CONCLUSIONS: Only a quarter of the babies born with BE/CE had received an antenatal diagnosis. Raising awareness about the condition amongst radiographers, and facilitating further scanning by a specialist fetal management unit if suspicious findings are noted, is crucial for improving the rate of detection. An antenatal diagnosis may not be reliable, and difficulty in identifying gender at the 20-week scan adds to the complexity of antenatal counselling. Magnetic resonance imaging and karyotyping may provide additional helpful information.

Salvage procedures to achieve continence after failed bladder exstrophy repair.

PURPOSE: We assessed the results from a single exstrophy center of salvage continence surgery after failed staged reconstruction for bladder exstrophy. MATERIALS AND METHODS: A total of 32 patients with bladder exstrophy had undergone salvage continence procedures. Indications for surgery included incontinence due to poor bladder capacity or failed bladder neck repair, and upper tract deterioration. Continence was defined according to the International Children's Continence Society as continent, intermittently incontinent and continuously incontinent. RESULTS: A total of 29 patients (91%) are continent, 3 (9%) are intermittently incontinent and none is continuously incontinent. One patient is continent after bladder augmentation and urethral clean intermittent catheterization. Two patients are continent and 1 is intermittently incontinent after bladder augmentation and modified Young-Dees bladder neck repair using urethral clean intermittent catheterization. One patient is continent using clean intermittent catheterization through a continent cutaneous diversion into a bladder substitution. A total of 19 patients are continent after bladder neck closure, bladder augmentation and continent cutaneous diversion using clean intermittent catheterization. Four patients are continent after cutaneous urinary diversion. Two are continent and 2 are intermittently incontinent after a Mainz II pouch. CONCLUSIONS: The majority of patients can still achieve continence following failed staged repair. Patients who have a low bladder leak pressure and who tolerate urethral catheterization can be consistently dry with bladder augmentation and bladder neck repair, which is a viable alternative to bladder neck closure, bladder augmentation and continent cutaneous diversion. Cutaneous urinary diversion has a role in selected patients. Mainz II pouch has not yielded consistent results. With better patient selection and increasing experience within specialist exstrophy centers fewer patients should require salvage continent surgery in the future.

Home dressing removal following hypospadias repair.

Hypospadias is a common congenital abnormality that is increasing in incidence. There are many articles describing different surgical techniques and the merits of different dressings following hypospadias surgery. This article describes postoperative nursing management in collaboration with each family following surgery. Listening to the wishes of parents identified ways in which improvements could be made to the patient journey. The involvement of parents in decision-making has been at the heart of developments in the care of boys who have hypospadias repair. A review of nursing care in combination with partnerships with paediatric community nursing teams has enabled the additional benefit of developing a home dressing removal service following hypospadias repair.

Intermittent catheterisation in school: a collaborative agreement.

Enabling children with medical needs to attend mainstream education can be a challenge for parents, teachers and health-care professionals. A lack of national standards on the management of continence in schools results in enormous variations in practice. This paper describes a collaborative approach to address the issues in schools in Manchester.