Variants of the exstrophy complex: a single institution experience

Area of Science:

• Pediatric Surgery
• Urology
• Developmental Biology

Background:

• Bladder exstrophy and cloacal exstrophy are rare congenital anomalies.
• Variants of these conditions present diverse anatomical challenges and management strategies.
• Understanding these variations is crucial for optimizing patient outcomes.

Purpose of the Study:

• To review the presentation, management, and outcomes of rare bladder/cloacal exstrophy variants.
• To compare outcomes of different surgical approaches for continence.
• To identify factors influencing successful management in complex cases.

Main Methods:

• Retrospective review of a database of over 815 patients with exstrophy complex.
• Identification and analysis of patients with variants of epispadias, bladder exstrophy, or cloacal exstrophy.
• Evaluation of anatomical presentation, surgical management, continence procedures, and long-term outcomes.

Main Results:

• Twenty-five patients with exstrophy variants were analyzed, with varied primary closure times and follow-up durations.
• Continence was achieved in 6 patients without reconstructive surgery (4 with superior vesical fistulas).
• Bladder neck reconstruction (BNR) and continent diversion (CD) showed varied success rates; CD was effective in some cases post-BNR failure.

Conclusions:

• Early diagnosis and standardized closure techniques for variants like epispadias with bladder prolapse are vital for promoting bladder growth.
• Superior vesical fistulas can enable continence by preserving urinary sphincter function.
• Cloacal variants with intact innervation may be amenable to later reconstructive procedures such as the Pena procedure.