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.
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.
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.
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Published online: August 18, 2020
Accepted: August 16, 2020
Received in revised form: August 13, 2020
Received: May 8, 2020
© 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.