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.

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.

X-AHC in infants may present as failure to thrive despite adequate nutrition. Extreme hyponatremia may be associated with little symptoms if developing slowly. Rehydration and slow correction of serum sodium with solutions containing less sodium than normal saline is essential.

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