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PubMed · 11547108

Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights.

Abstract

PURPOSE: We present the pelvic floor anatomy of the major pelvic floor musculature in classic bladder exstrophy, including the levator ani, obturator internus and obturator externus. By improving our knowledge of pelvic floor anatomy we hope to understand better the relationship of the pelvic floor to the bony anatomy as well as the role of osteotomy in changing pelvic floor anatomy to enhance urinary control after surgery. MATERIALS AND METHODS: 3-Dimensional computerized tomography was done in 6 boys and 1 girl, including 5 patients 2 days to 5 months old (mean age 7 months) undergoing primary closure and 2 who were 4 and 8 years old undergoing repeat closure. The pelvic floor musculature, including the levator ani, obturator internus and obturator externus, in these cases was compared to that in 26 age and sex matched controls. RESULTS: The levator ani musculature encompasses a significantly wider area of 9.5 cm.2 in patients with classic bladder exstrophy than in controls. The anterior segment of the levator ani was shorter (1.2 cm.) and the posterior segment of the levator ani was longer (2.5 cm.) than in controls. The degree of divergence of the levator ani in classic exstrophy was significantly more outwardly rotated (38.8 degrees) than controls. In addition, the transverse diameter of the levator hiatus was 2-fold that in our control group and in that of published controls, while the length of the hiatus was 1.3-fold that in normal controls. There was also significant flattening, involving a 31.7 degree decrease in steepness between the right and left halves of the levator ani, of the puborectal sling in classic bladder exstrophy versus controls. Because of these findings, there is more anterior superior rotation in the pelvic floor in exstrophy cases. The obturator internus was more outwardly rotated (15.1 degrees) in exstrophy and the obturator externus also showed more outward rotation (16.9 degrees) than in controls. CONCLUSIONS: This study provides better understanding of the pelvic floor anatomy in classic bladder exstrophy. Significant differences have been documented in the pelvic floor in classic bladder exstrophy cases and controls. Hopefully these differences may have a pivotal role in providing new insight into long-term issues, such as urinary and fecal incontinence, and pelvic organ prolapse, in classic bladder exstrophy.

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BibTeXRIS

A A Stec, H K Pannu, Y E Tadros, P D Sponseller, E K Fishman, J P Gearhart. 2001. Pelvic floor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights.. https://pubmed.ncbi.nlm.nih.gov/11547108/

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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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