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Techniques to create continence in the failed bladder exstrophy closure patient.

Abstract

We reviewed retrospectively 315 patients with bladder exstrophy treated at our hospital between July 1976 and April 1992 to assess the outcome of those who failed primary closure of the bladder. Of the patients 47 required reclosure of the bladder, including 28 who have undergone a procedure to restore urinary continence. Methods used to achieve dryness included bladder neck reconstruction in 18 patients, bladder neck reconstruction along with augmentation in 4, augmentation alone in 4, repeat bladder neck reconstruction in 1, and reclosure with creation of a continent stoma and augmentation in 1. Nine of 18 patients who underwent primary bladder neck reconstruction are dry on intermittent catheterization, while 8 of the remaining 9 are dry and voiding without catheterization. Four patients who underwent primary bladder neck reconstruction and augmentation, and 4 who underwent augmentation after bladder neck reconstruction are dry on intermittent catheterization. The patient who underwent reclosure, bladder augmentation and creation of a continent abdominal stoma is dry on intermittent catheterization. Virtually all patients who failed the initial closure and later bladder neck reconstruction for continence require augmentation and intermittent catheterization to remain dry. Of 28 patients who underwent salvage procedures only 1 had upper tract changes. With attention to detail and the use of a variety of reconstructive techniques children who have failed exstrophy closure can achieve continence and have stable renal function.

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BibTeXRIS

J P Gearhart, D A Canning, D S Peppas, R D Jeffs. 1993. Techniques to create continence in the failed bladder exstrophy closure patient.. https://doi.org/10.1016/s0022-5347(17)35505-2

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MTHFR 677 TT genotype in a mother and her child with Down syndrome, atrioventricular canal and exstrophy of the bladder: implications of a mutual genetic risk factor?

Apart from Husmann and Vandersteen [in: Gearhart JP, Matthews R (eds) The Epispadias-Exstrophy Complex. Kluwer, New York, pp 199-206, 1999], we report only the second case of Down syndrome (DS) associated with exstrophy of the bladder (EB). Besides the appearance of DS, the newborn exhibited a complete atrioventricular canal (CAVC) and classical EB, including diastases of the symphysis, an epispadic penis and an open bladder plate. Despite current recommendations, the mother had not supplemented her intake of folic acid during the periconceptional period. In a comparable case, Al-Gazali et al. (Am J Med Genet 103:128-132, 2001) found the homozygous 677T allele of the methylenetetrahydrofolate (MTHFR) gene 677C-->T polymorphism in a mother and her child with DS and cervical meningomyelocele. They found that the mother, who also had not supplemented her folic acid intake, had a secondarily altered folate status with an increased homocysteine level, suggesting that the homozygous TT mutation in the MTHFR gene in both mother and her child had contributed to the presentation of DS and a neural tube defect. The combined clinical findings of the present case and the observations of Al-Gazali et al. led us to investigate the 677C-->T polymorphism in our mother-child pair. Likewise we found that mother and child were homozygous for the mutant 677T allele. Our findings support the suggestion of Al-Gazali et al. that the MTHFR 677TT could be a mutual genetic risk factor for the co-occurrence of trisomy 21 and midline defects, the risk of which may be reduced by periconceptional folic acid supplementation.

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