Elsevier

Journal of Pediatric Surgery

Volume 46, Issue 9, September 2011, Pages 1790-1793
Journal of Pediatric Surgery

Original article
Colorectal anomalies in patients with classic bladder exstrophy

https://doi.org/10.1016/j.jpedsurg.2011.03.019Get rights and content

Abstract

Aim

This study aims to determine the proportion and type of colorectal anomalies that occur in children born with classic bladder exstrophy (CBE).

Methods

All patients in a database of 1044 patients with the bladder exstrophy-epispadias-cloacal exstrophy complex were reviewed. Those with CBE had their complete medical records reviewed. Children noted to have gastrointestinal malformations were identified, and all aspects of their history were extracted.

Results

A total of 676 patients were identified with CBE, of whom 12 patients were identified who had a concomitant colorectal anomaly. In this population, the proportion of colorectal anomalies is 1.8%. The most common gastrointestinal anomaly was imperforate anus in 8 patients. Two patients had severe rectal stenosis requiring serial dilations, and 2 patients were born with congenital rectal prolapse.

Conclusions

Excluding all variants and cloacal patients with exstrophy, isolated colorectal anomalies occur at a rate of 1.8% in children born with CBE, a 72-fold increase compared with the general population. Although uncommon in this rare birth defect, the morbidity is significant and warrants prompt evaluation and treatment of both the genitourinary and colorectal anomalies.

Section snippets

Materials and methods

We reviewed 1044 patients in an institutionally approved database containing all patients with a diagnosis of exstrophy evaluated at our institution between 1974 and 2010. The inclusion criteria for this study were a diagnosis of CBE and complete and available medical records with a comprehensive, documented physical examination. Patients were excluded if they had an exstrophy variant, complete epispadias, cloacal exstrophy, or classic exstrophy with any associated spinal or neurologic anomaly.

Results

A total of 1044 patients with the exstrophy-epispadias-cloacal exstrophy condition have been evaluated or treated at our institution for a 36-year period. Of these, 676 have CBE, with the remaining 368 patients having cloacal exstrophy, epispadias, an exstrophy variant, or incomplete records for evaluation.

Of this cohort of 676 patients, boys were represented in a 2.5:1 ratio to girls, 482 patients to 193, respectively. Of those patients whose data on ethnicity/race were available for review,

Discussion

The existence of GI malformations in the exstrophy complex is well documented; however, most of these malformations present in patients with more severe forms of the disease, such as cloacal exstrophy [7]. Traditionally, the most common form of bladder exstrophy, CBE, has been classified primarily as a GU malformation. However, for the past 3 decades, patients presenting with CBE have also been found to have significantly higher proportions of associated congenital anomalies than the general

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  • Maternal and fetal risk factors for bladder exstrophy: A nationwide Swedish case-control study

    2016, Journal of Pediatric Urology
    Citation Excerpt :

    BE is the most common condition of the spectrum of genitourinary malformations ranging in severity from epispadias, affecting only the urethra and genitalia, to cloacal exstrophy, also involving the intestines. BE is commonly considered to be an isolated malformation, but associated major malformations, such as cleft lip-palate, spina bifida, and orthopedic and gastrointestinal anomalies, have also been described [1–4]. The prevalence of BE has been reported to range between 1.6 and 4.0 per 100,000 live births.

  • Congenital renal anomalies in patients with classic bladder exstrophy

    2012, Urology
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    In the gynecologic literature, the vaginal canal has been described as more vertical than horizontal, with a cervix inserting more distally on the superior vaginal wall close to the introitus.14 New reports have been published in 2011 on CBE reporting the incidence of isolated gastrointestinal anomalies, such as imperforate anus, to be between 1.8% and 2.5% in 2 large international series.15,16 Despite other anatomical variants being well described, the exstrophy literature does not contain any large series describing the variants of normal renal anatomy that exist in this population.

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