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Urethral reconstruction in boys with classical bladder exstrophy.

H Lepor·E Shapiro·R D Jeffs
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Abstract

A total of 24 boys with classical bladder exstrophy underwent initial urethral reconstruction at our hospital between 1975 and 1982. Penile reconstruction in male patients with classical bladder exstrophy includes penile lengthening, release of the dorsal chordee and reconstruction of the urethra. The former 2 procedures are performed during the primary bladder closure and urethroplasty usually follows bladder neck reconstruction. A modified Young urethroplasty was done in 22 of the 24 patients. Preputial pedicle grafts of free full thickness skin grafts were used for urethroplasty in 2 boys with insufficient penile skin. Fistulas requiring surgical revision developed after urethroplasty in 21 per cent of the patients. A prior osteotomy was associated with a decreased fistula rate. The cosmetic and preliminary functional results of the penile reconstruction were assessed by parental interviews. The definitive assessment of the penile reconstruction will be determined when these boys reach sexual maturity.

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Bladder ExstrophyChildChild, PreschoolFistulaHumansInfantMaleOsteotomyPostoperative ComplicationsSurgery, PlasticSurgical FlapsUrethraJournal Article

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H Lepor, E Shapiro, R D Jeffs. 1984. Urethral reconstruction in boys with classical bladder exstrophy.. https://doi.org/10.1016/s0022-5347(17)50471-1

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PubMed2006

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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