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PubMed · 8162512

Inguinal hernias associated with bladder exstrophy.

Abstract

OBJECTIVE: To determine the incidence and complications of inguinal hernias in patients with bladder exstrophy. PATIENTS AND METHODS: A retrospective review of inguinal hernias and their management in 70 consecutive patients (50 boys and 20 girls) with bladder exstrophy managed by staged reconstruction. Follow-up data were available for 69 patients. RESULTS: During a mean follow-up period of 5.9 years (range 0.3-14), 42 (86%) boys and three (15%) girls developed inguinal hernias. These were bilateral in 35 (78%) cases. All but three of these patients presented during infancy. In girls, no instance of incarceration or recurrence was noted. In contrast, 14 (29%) boys developed an incarcerated hernia and seven (17%) developed recurrent inguinal hernias. All recurrences occurred in boys under 2 years of age, were associated with an indirect sac and were unrelated to the seniority of the surgeon performing the initial herniotomy. No recurrences occurred in eight boys undergoing inguinal herniotomy at the time of bladder closure. CONCLUSIONS: Boys with classical bladder exstrophy managed by staged reconstruction have a much higher incidence of inguinal hernias than previously recognized. Most are bilateral, present in early infancy and are prone to incarceration. Bilateral groin exploration and meticulous inguinal herniotomy at the time of bladder closure may reduce morbidity.

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BibTeXRIS

M D Stringer, P G Duffy, P G Ransley. 1994. Inguinal hernias associated with bladder exstrophy.. https://doi.org/10.1111/j.1464-410x.1994.tb07524.x

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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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