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In the last decades the surgery for total continent reconstruction has been changed by the introduction of intermittent catheterized stoma with the purpose to preserve urinary tract function, urinary continence, elimination of fecal soiling, preservation of quality of life with complete self-sufficiency. We report a rare case of complete incontinence in caudal regression syndrome in whom the appendix was long enough to be divided for creation of both channels for the treatment of urinary and fecal incontinence. A 9-year-old male patient with complete incontinence in caudal regression syndrome was submitted to a Mitrofanoff appendicovesicostomy procedure (AV) in conjunction with appendicocecostomy procedure (ACE). The appendix was divided into two different parts preserving adequate perfusion. Fecal continence was achieved for a period of 18-20 hours after a colonic irrigation with 500 ml of saline solution one time daily, while urinary continence was obtained after suburethral endoscopic injection of dextranomer/hyaluronic acid (Deflux) and intermittent catheterization every 3 hours with an evident reduction of upper urinary tract dilatation. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.
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