sodium-ethylxanthate and Epispadias

The major congenital anomalies of the genitourinary tract may result in disturbances of sexual and reproductive function. In general the children grow up with the same aspirations as their more normal peers, which are to marry, have intercourse and produce children. Some achieve this despite the deformities and in others specific reconstructive surgery may be needed. In exstrophy the vagina lies parallel to the floor when the girl is standing and the introitus is seen on the lower abdominal wall rather than in the perineum. Episiotomy is required in 34% and formal vaginoplasty in 23% of the cases. The exstrophy penis has a tight dorsal chordee that must be corrected to allow intercourse. The neurological and social consequences of myelomeningocele do not prevent patients from having an interest in sex. Those who are able to walk are likely to have normal sexual function compared to about 50% of those who are wheelchair bound. The recurrence risk for neural tube defects in their offspring is 1:50 for sons and 1:13 for daughters regardless of the sex of the affected parent. The physiological consequences of posterior urethral valves result in weak ejaculation in 50% and highly viscous and alkaline semen in 50% of the cases. Of male patients with ambiguous genitalia or micropenis 75% have normal intercourse.

Topics: Epispadias; Female; Humans; Male; Meningomyelocele; Penile Erection; Penis; Pregnancy; Pregnancy Outcome; Reproduction; Sex; Urogenital Abnormalities; Urogenital System; Vagina

To compare the histology of gonads excised from males with cloacal exstrophy (the most severe variant of the exstrophy-epispadias complex) undergoing early gonadectomy and gender reassignment (to obviate difficulties in reconstructing male external genitalia) with those published previously.. Testicular tissue was obtained from males with cloacal exstrophy undergoing gender reassignment shortly after birth. Slides were stained for histological evaluation and reviewed by one author. The histology was compared with known normal features for newborn testicular tissue. The mean age at the time of gender reassignment was 6 months.. There were no significant abnormalities in the architecture of the rete testis, epididymis or vas of the study specimens. There were more germ cells in some patients with cloacal exstrophy, but this finding was not specific and is of unknown clinical significance.. Despite the severity of the malformations in cloacal exstrophy, testicular histology is preserved. In highly selected genotypic males who have adequate phallic structures, congruent gender assignment is appropriate and early orchidopexy is recommended.

Topics: Bladder Exstrophy; Cloaca; Cryptorchidism; Epispadias; Humans; Infant; Male; Sex; Testis

We evaluated the long-term outcome of social integration and sexual function in 29 men with bladder exstrophy and epispadias.. Four men with epispadias and 25 with bladder exstrophy were available for long-term followup. Patients were interviewed by telephone regarding sexual history and social integration. Semen analyses were obtained in 8 cases.. Patient assessment of genital appearance was good or fair in 71%. Potency was present in all patients, and erections were straight in 66% and curved in 34% with no curvature so severe as to prevent sexual intercourse. Semen analysis showed a normal sperm count in 63% of the men and no azoospermia. Social integration was satisfactory: 100% of the men attended high school, 55% have a college education and all who are not attending school have full-time jobs.. Our long-term review demonstrates that despite what appears to be a significant sexual handicap, patients with exstrophy/epispadias can have adequate sexual function and overall successful social integration.

Topics: Bladder Exstrophy; Epispadias; Fertility; Follow-Up Studies; Humans; Male; Sex; Social Adjustment; Time Factors; Treatment Outcome

After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration or renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patients with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children.. Education, occupation and social development of patients with urinary diversion are uneventful. The cosmetic results achieved by genital reconstruction are satisfactory. In female patients, antefixation of the uterus should be performed before or together with an introitusplasty to prevent uterine prolapse. In male patients, however, surgery is performed at the expense of fertility. Detailed discussions with the patients and their patients should include not only the question of primary bladder closure versus urinary diversion, but also the pros and cons of correction of the external and-in females-the internal genitalia.

Topics: Adult; Bladder Exstrophy; Child; Child, Preschool; Epispadias; Female; Fertility; Follow-Up Studies; Humans; Male; Patient Satisfaction; Sex; Social Adjustment; Treatment Outcome