PubMed Health⌕ Search

PubMed · 2313504

Inguinal pathology and its association with classical bladder exstrophy.

Abstract

One hundred thirty-four cases of classical bladder exstrophy, managed at our institution, were reviewed. Fifty-six percent of the boys and 15% of the girls developed inguinal hernias over an average follow-up time-span of 10 years. Thirty-one percent of the patients with hernias underwent repair at the time of initial bladder closure. Forty-six percent of the patients who developed a hernia were diagnosed during the first year following their initial procedure. More than 50% of the individuals in the latter category presented with an incarcerated hernia, and required emergent management. Boys managed by staged reconstruction had a statistically significant risk of developing an inguinal hernia (P less than .001) compared with boys undergoing primary cystectomy and diversion. We believe the increased incidence of herniation with this congenital anomaly is secondary to a lack of obliquity of the inguinal canal, due to pubic diastasis along with an increased elevation of intraabdominal pressure following initial closure of the abdominal wall and bladder plate. To decrease the attendant morbidity of incarcerated hernias in this population, we stress the need for careful physical examination of the inguinal region and spermatic cord prior to surgery, along with repair of the patent processus vaginalis at the time of initial repair.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

D A Husmann, G A McLorie, B M Churchill, S H Ein. 1990. Inguinal pathology and its association with classical bladder exstrophy.. https://doi.org/10.1016/0022-3468(90)90080-s

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

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.

Bladder Exstrophy↗