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Prune perineum surgical correction – Treatment of a rare syndrome

      Summary

      Introduction

      Prune Perineum Syndrome (PPS) is rare, with only three cases described in the literature. Treatment requires abdominal and pelvic surgeries. Our goal is to provide a video with steps of the surgical corrections of this syndrome.

      Materials and methods

      We present a case of an 8 months-old boy with PPS. At our first evaluation, he had already been submitted to a colostomy with mucous fistula and a vesicostomy.

      Results

      By the time of this publication, he had been submitted to six surgical procedures. First, osteotomy, followed by colostomy, cystoscopic evaluation, cystoplasty, perineal and abdominal correction with a dual mesh, bilateral orchidopexy, treatment of the vesicocolonic fistula, sigmoidectomy and appendicectomy.
      He is 6 years old, walks with no assistance, has satisfactory abdominal tonus which allows him to void with Valsalva Maneuver. Clean intermittent catheterization is also performed. He had no UTI since his first surgery.
      Although submitted to several procedures, treatment is not yet concluded. A future urethral dilation together with a genitoplasty might improve his quality of life.

      Conclusion

      PPS is a very rare condition, with scarce literature concerning its treatment. Multiple procedures might be necessary to correct malformations and improve patient's quality of life.

      Keywords

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