Exstrophy /
Epispadias Complex

Renal and urinary issues

  • The exstrophy/epispadias complex includes a broad spectrum of urologic conditions, ranging from mild distal epispadias (which may require no long-term management) to classic bladder exstrophy and even cloacal exstrophy.
  • Although some of these patients may have fewer long-term issues requiring multidisciplinary care than those with SB, their urologic conditions require lifelong follow-up.
  • UI becomes a significant issue for many women during pregnancy, but patients tend to return to baseline function after delivery.
  • These changes may cause worsening lower tract compliance, leading to transmission of elevated pressures to the upper tracts or worsening urinary incontinence.

Reasons for follow-up

Patients with a history of bladder exstrophy also require long-term monitoring for their potentially increased risk of pelvic malignancies.

Although the actual risk is unknown and depends on many factors, exstrophy patients may face a 65-fold increased risk of death from cancer when compared with the general population.

The prevalence of metastatic disease at or shortly after diagnosis of pelvic malignancy is markedly higher than that seen in the general population.

The historical cohort of patients who underwent ureterosigmoidostomy in childhood deserves special mention, because providers continue to encounter these patients in adult practice today.

Diversion of the urine into the fecal stream is associated with more than a 1700-fold increase in the risk of developing bladder or bowel adenocarcinoma.

The significant risk of malignancy in this group mandates close surveillance with annual colonoscopy, and since initiating routine surveillance there have been no reported deaths from colon cancer in the ureterosigmoidostomy population.

Transitional urology
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