The impact of classic bladder exstrophy-epispadias repair in adult patients without continence

Background: Bladder exstrophy-epispadias complex is a rare congenital anomaly. Very few patients grow with the condition intact in their adulthood which lead to compromise in the results of repair. We herein report our experience of correction of the defects in the adult patients in single stage without doing osteotomy andurinary diversion. Methods: The sample size was 13 patients over ten years. All were treated in single stage. Bladder neck repair was done using paraurethral tissue, primary abdominal closure was done without flap, epispadias repair was done using modified Cantwell-Ransley procedure and hernial repair was done without mesh. No osteotomy was done. All the patients were followed regularly. Minimum period of follow-up was one year. Urinary continence, change in habits and subjective assessment of well-being were also noted.Results: 86.4% patients were totally incontinent. Bladder and anterior abdominal wall were closed in all the patients. Complete epispadias was possible in15.3% patients and rest were converted into coronal hypospadias. Hernia was repaired in 23% patients. All the patients were satisfied with their cosmetic results and they were able to interact socially. Conclusions: Surgical correction of the exstrophy-epispadias complex is possible in adulthood in the hands of experts in single stage without osteotomy. Regular cystoscopic examination and urine cytology yearly are recommended to diagnose the malignant changes in the native bladder as early as possible. Psychological, social and self-level of satisfaction is high among these patients after the surgical correction.

A wretched condition

Although rare, cleft bladder was known to the ancients: the infants did not die after birth, but grew up incontinent. Combined with abnormal genitalia, the malformation was confused with hermaphroditism, the most loathed congenital anomaly. Three grades were discerned in the 17th century: epispadias, where the urethra opens at the anterior side of the penis; bladder exstrophy with open anterior bladder wall; and cloacal exstrophy with common outlet for stool and urine. Explanations of its origin included obstructed urethra, short umbilical cord, and bursting allantois, bladder, abdominal wall, or cloacal membrane. As the incontinent children suffered from painful inguinal eczema, urine collecting devices were constructed from copper and tin, and from rubber once vulcanization was invented. Correcting operations were attempted from 1852 with limited success. Today, stepwise corrective surgery begins by closing the bony pelvis, bladder, and the abdominal wall, followed by epispadias repair and penis remodelling. No controlled trials or international protocols have come forth, and the long-term prognosis is all but satisfying.

A case report on continent isolated male epispadias.

Epispadias is a rare congenital anomaly in which the urethral opening is on the dorsal surface of penis. Its incidence is 1 in 117000 newborn males and 1 in 484000 in newborn females. Its etiology is considered as because of failure of medial migration of mesenchyme between the ectodermal and endodermal layers of cloacal membrane due to premature rupture of cloacal membrane. Epispadias often presents as exstrophy epispadias complex, a wide spectrum of abnormalities that consist of classic bladder exstrophy, Epispadias and cloacal exstrophy. This case was not a part of exstrophy epispadias complex and it was an isolated continent epispadias which is extremely rare and it was managed with Cantwell Ransley epispadias repair technique.