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One stage bladder exstrophy and epispadias repair in newborn male.

We hereby describe the technique and our experience of a single-staged repair of bladder exstrophy and epispadias in two newborn males. The combined repair improves dissection and exposure of the bladder neck, posterior urethra and prostate, allowing better exposure for reconstruction and proper positioning in the pelvis. This approach should be avoided in premature babies and in patients with a very small penis. Although the complication rate of this procedure is low, large experience and expertise in exstrophy and epispadias is imperative prior to embarking in this major neonatal reconstruction.

Journal Article↗

Bladder exstrophy in a neonate at risk of transient myasthenia gravis: a role for remifentanil and epidural analgesia.

Infants born to mothers with myasthenia gravis may exhibit a transient form of the disease, with similar sensitivity to non-depolarizing neuromuscular blocking drugs. We report the case of an infant at risk who required major surgery when 48 h old for closure of bladder exstrophy. A combined epidural-general anaesthetic technique, with remifentanil supplementation, enabled us to avoid unnecessary neuromuscular blocking drugs and prolonged intensive care, which had been anticipated. The potential benefits of remifentanil and epidural analgesia in neonates are discussed.

Analgesia, Epidural↗

Bladder-neck repair in urinary bladder exstrophy.

A simple modification of an existing technique for bladder-neck reconstruction in exstrophy of the urinary bladder is reported. The technique involves tubularization of the posterior urethra up to just below the ureteric orifices. It differs from other techniques in that no part of the bladder tissue is used for buttressing the repair, but all is utilized for enhancing the bladder volume. Only 2 of 20 patients remained incontinent after bladder-neck reconstruction; the remaining 18 have achieved socially acceptable continence.

Bladder Exstrophy↗

Social integration, sexual behavior and fertility in patients with bladder exstrophy--a long-term follow up.

UNLABELLED: 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. CONCLUSION: 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.

Adult↗

Treatment of incontinence in children with bladder exstrophy after rectal urinary diversion: the anal sling procedure.

PURPOSE: An original technique for reinforcement of the anal sphincter using a sling prosthesis with a flexible fixing point that prevents incontinence and rectal prolapse is presented. MATERIALS AND METHODS: Of 43 patients with exstrophy who have undergone urinary rectal diversion between 1988 and 1997 incontinence was observed in 9 boys and 5 girls. From January 1993 to December 1998 these children were treated with an original method, the anal sling procedure. RESULTS: There were no postoperative complications. Investigations revealed no urinary or fecal incontinence after a mean followup of 3.2 years (range 6 months to 61/2 years). CONCLUSIONS: A short and simple operation eliminates incontinence after rectal urinary diversion in patients with bladder exstrophy. It can be used as an alternative to the formation of an isolated urinary reservoir.

Anal Canal↗

[The urodynamics of the lower urinary tract after reconstructive operations in bladder exstrophy].

The results of plastics of the bladder with local tissues in its exstrophy in 34 children were appraised according to the findings of clinical, functional, and morphological studies. The cosmetic effect was satisfactory in 22 patients, but control over urination remained unsuccessful as a rule. Enuresis after sphincteroplasty was linked with functional insufficiency of the trigonal muscle due to tissue dysembryogenesis. Another component of enuresis was dysfunction of the bladder attended by intravesical hypertension and uninhibited contractions when its size was small. The results of morphological studies allowed the authors to explain the character of the urodynamic disorders and the inefficacy of their nonoperative correction.

Bladder Exstrophy↗

[Bladder exstrophy].

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Bladder Exstrophy↗