Exstrophy-epispadias complex presenting in adulthood: a single-center review of presentation, management, and outcomes.

OBJECTIVE

To study the presentation, management, and outcome of patients with exstrophy-epispadias complex, who present in adulthood (aged >18 years).

MATERIALS AND

METHODS

A retrospective review of the electronic medical records of patients with exstrophy-epispadias complex managed from January 2001 to December 2010 was undertaken. Patients aged >18 years at presentation, with detailed medical records were selected. They were classified into 2 groups: group A (previously untreated) and group B (residual defects or complications after childhood surgery).

RESULTS

Thirty-nine patients with exstrophy-epispadias complex presented to our institution over this 10-year period. Of these 26 were adults (aged >18 years; range, 18-48 years). Detailed medical records could be obtained for 21 of them and they were included. Group A consisted of 4 patients--2 male and 2 female. All underwent cystectomy; 2 had an ileal conduit and 2 had ureterosigmoidostomy (Mainz II). All had improved quality of life and a stable renal function at follow-up. Group B consisted of 17 patients. Mean number of surgeries attempted previously was 4.4 (range, 2-13). Presentation was varied but primarily involved incontinence of urine (n = 12). Four patients were lost to follow-up, 2 were managed conservatively, and 11 underwent a surgical procedure. All patients returned to normal activity with an improvement in the quality of life postoperatively.

CONCLUSION

Exstrophy-epispadias complex is difficult to manage in resource-poor settings associated with illiteracy and poverty. However, successful rehabilitation and an improved quality of life are possible even in cases presenting in adults.