Variations in the anatomic defects of the bladder exstrophy are well recognized, but their incidence is extremely low. Here the authors describe a rare case of superior vesical fissure in which a relatively large defect caused the whole bladder to prolapse outside. A review of literature found only 2 other cases that resembled our case. This particular variation of exstrophy is not only important owing to its extreme rarity but also raises a question for an embryologic explanation.
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