Neoplasia in adult exstrophy patients.
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During the last 3 years 20 patients (median age 8 years) have undergone construction of a continent urinary reservoir at our pediatric urology service. Of the patients 12 had failed exstrophy reconstruction, 7 had myelodysplasia with a neurogenic bladder and 1 had extensive bladder damage as a result of a previous operation. In 5 patients a continent reservoir was constructed after cystectomy performed in early childhood. Techniques of reconstruction included the Mitrofanoff procedure (4 patients), a modification of the Indiana pouch (12), a modification of the Benchekroun procedure (2), the use of the appendix in situ as a continence mechanism (2) and a modified Kropp procedure using a duplicate vagina for catheterization (1). The morbidity from these procedures was acceptable and consisted primarily of chronic diarrhea (4 patients), stone formation within the pouch (5) and the need to revise the continence mechanism (7). All but 1 patient maintained stable or improved upper tracts after diversion. Over-all, 85% of the patients experienced diurnal continence on an intermittent catheterization regimen, while 75% were dry at night. The plicated/intussuscepted ileal nipples required revision because of difficulty with catheterization (7 patients) and urinary leakage. We currently favor the use of the Benchekroun or Mitrofanoff techniques for the ease of construction and minimal requirements for revision.
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OBJECTIVE: To assess psychosexual function in adolescent males with genital anomalies. METHOD: Fourteen consecutive males with bladder exstrophy-epispadias, 14 to 19 years old, Tanner stages IV to VI, were assessed along with their parents, using a developmental questionnaire, Hollingshead socioeconomic status rating, Child Behavior Checklist, Youth Self-Report, semistructured psychiatric interview, detailed sexual history, and 5 written, open-ended questions. RESULTS: All subjects showed psychosexual dysfunction in terms of genital satisfaction and genital touching; only 2 had ever undressed in front of anyone; only 2 had ever masturbated and only after age 16; 8 had few friends and only 5 considered any girls as friends; all expressed heterosexuality but only 4 had dated, 1 at age 17 and 2 after age 18; only the two 19-year-olds had experienced sexual intercourse, at the age of 19. All had an anxiety disorder. Half had experienced a major depressive disorder. CONCLUSIONS: Psychosexual dysfunction and anxiety were universal and chronic in these males with genital anomalies, leading to social and sexual developmental impairment. Half had a mood disorder. Implications for adulthood as well as for children with other genital anomalies are unclear but deserve further study. Males with genital anomalies should be evaluated for psychosexual developmental impairment.
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A pair of monozygotic male twins discordant for ectopia vesicae urinariae is described. There is no reason to think that any exogenic factors contributed to the aetiology. There was no consanguinity and no history of congenital anomalies on either side of the family. Published reports concerning family incidence and twin reports are reviewed.
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PURPOSE: We explored the psychological adjustment of children with bladder or cloacal exstrophy. MATERIALS AND METHODS: We assessed 29 subjects with a mean age plus or minus standard deviation of 7.8 +/- 3.97 years using age appropriate standard psychological instruments. Psychological adjustment scores in the exstrophy group were compared to the norms of the various instruments. Subjects were divided into dichotomous groups according to several medical and demographic factors. For each factor the differences between the means of the 2 groups on the outcome variables were calculated using a t test. RESULTS: Children with exstrophy perceived their appearance more positively than the norm. Older children performed more poorly than younger children in adaptive behavior, specifically in skills related to functioning in school. Children who achieved continence after age 4 years were more likely to have problems with acting out behavior. There were no differences in adjustment in boys versus girls, bladder versus cloacal exstrophy, type of continence strategy or gender reassignment versus no reassignment. CONCLUSIONS: Children with exstrophy did not have clinical psychopathology. Differences existed in adaptive and acting out behavior rather than depression or anxiety, suggesting that improved outcomes may be achieved through a focus on normal adaptation rather than on potential psychological distress.