Exstrophy and Epispadias Workup

Updated: May 03, 2022
  • Author: Chad B Crigger, MD, MPH; Chief Editor: Marc Cendron, MD  more...
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Workup

Laboratory Studies

Before complex reconstruction of the urinary tract, it is important to obtain information about the patient's baseline renal function. In patients with cloacal exstrophy, losses from the terminal ileum short-gut physiology can result in significant electrolyte abnormalities. Any derangements in electrolyte balance or renal function should be addressed, and corrected if possible, prior to surgery.

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Imaging Studies

A complete imaging series that includes plain films, ultrasonography (US), and, if needed, magnetic resonance imaging (MRI) helps determine the severity of associated defects and facilitates early involvement of consulting services (eg, pediatric orthopedic surgery, pediatric neurosurgery, and pediatric general surgery). 

Baseline examination of the kidneys with US is recommended for all patients with exstrophy because increased bladder pressure after bladder closure can lead to hydronephrosis and upper urinary tract deterioration. Congenital upper urinary tract anomalies are uncommon with classic exstrophy and epispadias but are present in approximately one third of patients with cloacal exstrophy (eg, ectopic pelvic kidney, renal agenesis, or hydronephrosis).

Spinal US or radiography may be helpful. Myelodysplasia should be excluded in newborns with cloacal exstrophy. This can be accomplished by means of US early in life. In cloacal exstrophy, MRI is recommended to help identify occult abnormalities that may predispose to symptomatic spinal cord tethering.

Bilateral vesicoureteral reflux (VUR) is present in nearly all patients with classic bladder exstrophy. Voiding cystourethrography (VCUG) is performed in early childhood to assess bladder capacity in preparation for reconstructive continence surgery. Evaluation of the bladder neck and proximal urethra is recommended in patients with epispadias to facilitate the planning of surgical management.

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Histologic Findings

Histologic studies are not routinely performed. However, basic science and clinical investigations have revealed interesting histopathologic differences between samples from exstrophy bladders and samples from normal age-matched controls. [34]  Specifically, there is an increased ratio of extracellular matrix to muscle within the bladder wall; in particular, the amount of type III collagen is increased. This ratio seems to approach a more normal range (ie, relative increase in muscle) after successful bladder closure. Exstrophied bladders also have fewer myelinated nerve fibers than unaffected bladders do. [35]

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