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Posterior sagittal anorectal vaginal urethral plasty (PSARVUP) is a type of reconstructive surgery that is done to treat a condition that is present at birth called a cloacal malformation. Cloaca is a type of anorectal malformation that affects the rectum and urogenital tract in females. Females are normally born with three perineal openings: urethra, vagina and anus. Children born with a cloaca only have one opening because the urethra, vagina and rectum are joined together as a common channel instead of three separate ones.
During PSARVUP, the pediatric colorectal surgeon separates the urethra, vagina and rectum so there are three perineal openings at the end of the operation.
Usually on the second day of life, babies with cloaca will have surgery to create a colostomy, a surgical procedure to allow your baby's stools to pass. With a colostomy, the large intestine is divided into two sections, and the ends of the intestine are brought through small openings on the abdominal wall.
The upper section allows stool to pass through the stoma into a collection bag. The second, smaller stoma is called a mucus fistula. The purpose of the mucus fistula is to allow for future injection of contrast to define your child's anatomy prior to the reconstructive surgery. Contrast materials are substances given during imaging procedures, such as X-rays and CT scans, to allow pictures of the inside of the body to show up more clearly.
Your child will also have a hydrocolpos drainage if needed. Hydrocolpos is a vagina that is distended with fluid. The distended vagina can compress the trigone of the bladder (the area where the ureters drain) causing dilation of the kidneys.
Once we see that your baby is growing well, we can plan for PSARVUP surgery at about 2 to 6 months of age.
The type of surgery your child needs depends on the length of the common channel. The length of the common channel may vary between 1 to 10 cm.
A common channel length of 1 cm usually requires separation of the rectum from the vagina and an introitoplasty. An introitoplasty is the movement of the walls of the vagina to create an adequate opening to the entrance of the vagina. The perineal body (skin between rectum and vagina) is reconstructed and the rectum is placed within the center of the sphincter. The sphincter is a group of muscles located at the end of the rectum that surrounds the anus. It helps to control the release of stool.
A common channel length between 1 and 3 cm will require a PSARVUP with total urogenital mobilization. Your baby will be face down and the surgeon will make an incision through the middle of the buttocks. This is called the posterior sagittal approach. For the urogenital mobilization, the surgeon will separate the rectum from the vagina and place it within the center of the sphincter. The surgeon will then move the urethra and vagina together until they reach the perineum. The perineum is the area between the vagina and rectum.
During this surgery, the surgeon will make an incision through the middle of the buttocks. The surgeon will also do a total urogenital mobilization. If the urethra and vagina do not reach the perineum, the surgeon will make an incision through the abdomen to separate the urethra from the vagina.
For a common channel longer than 5 cm, the surgeon usually makes an incision in the abdomen to separate the structures. The type of vaginal reconstruction that will be done depends on your child's anatomy. Some patients may need a partial vaginal replacement using part of the bowel (intestines).
Some patients may have two large vaginas high in the abdomen that will allow for a vaginal switch maneuver. During this procedure, surgeons create a new vagina that is long enough to be switched down to the perineum.
Patients with cloaca have one chance to receive the right surgical procedure for the best potential functional health outlook they were born with for bowel, urinary and sexual/reproductive function. If the right surgical procedure is not done the first time, your child may have complications and need another surgery. Another surgery may restore the anatomy, but will not restore their functional prognosis.
Alberto Peña, MD from the International Center for Colorectal and Urogenital Care, created the operation for cloaca reconstruction. Dr. Peña and Andrea Bischoff, MD, have been working together for more than 10 years. They have the most experience in the world performing cloacal reconstruction and longest follow-up of these patients. Together they have operated on more than 600 patients born with cloaca.