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Exstrophy-epispadias complex: are the kidneys and kidney function spared?

Roxana Cleper1,2, Danith Blumenthal3,4, Yossi Beniamini5

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Pediatric patients with exstrophy-epispadias complex (EEC) frequently show signs of kidney injury, including elevated blood pressure and decreased kidney function. Early, long-term monitoring by pediatric urology and nephrology teams is crucial for managing kidney health in these complex cases.

Keywords:
BladderBlood pressureDMSA kidney scanExstrophy-epispadias complexKidney functionUrinary tract dilatation

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Area of Science:

  • Pediatric Nephrology
  • Urology
  • Congenital Malformations

Background:

  • Exstrophy-epispadias complex (EEC) is a severe congenital malformation requiring multiple surgical reconstructions.
  • Patients with EEC are at high risk for surgical and infectious complications.
  • Kidney function assessment is critical in managing pediatric EEC patients.

Purpose of the Study:

  • To evaluate kidney function in pediatric patients diagnosed with exstrophy-epispadias complex (EEC).
  • To identify the prevalence of kidney injury markers in EEC patients.
  • To explore associations between urinary tract dilatation, kidney defects, and blood pressure.

Main Methods:

  • A cross-sectional study involving pediatric patients with EEC followed since birth.
  • Nephrological evaluation included blood pressure, blood/urine chemistries, and imaging (ultrasound, DMSA scan).
  • Data collected between 2017-2020 from a dedicated pediatric urology clinic.

Main Results:

  • 74% of 43 EEC patients exhibited at least one sign of kidney injury.
  • Elevated blood pressure (29%), decreased eGFR (12%), and proteinuria/albuminuria (36%) were common.
  • Urinary tract dilatation correlated with kidney defects and elevated blood pressure; clean intermittent catheterization (CIC) showed lower rates of decreased eGFR and elevated BP.

Conclusions:

  • The high incidence of kidney injury in pediatric EEC necessitates early and continuous monitoring.
  • Joint pediatric urology and nephrology management is essential for long-term kidney health.
  • Findings underscore the importance of comprehensive nephrological surveillance in EEC patients.