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