Persistent cloaca is a defect in which the rectum, vagina and urinary tract are connected into a single common channel. This channel opens into a single opening typically located at the normal site of the urethra (where urine exits the body). It occurs in approximately 1 in 20,000 births.

It is important that your child’s doctor determines the specific type of cloaca before recommending surgery for your child. Your child will have some testing to look at her anatomy and also to see if there are any other malformations. Urologic malformations are common in persistent cloaca and often need immediate attention and close follow-up.

Initial Management

• Physical exam and testing to diagnose any associated issues
• Surgery to create a colostomy, or opening on the abdomen for stool to empty into a stoma bag
• Some patients also require surgery to allow proper drainage of urine from the bladder
• Check for a condition called hydrocolpos, which is a collection of fluid in the vagina. Hydrocolpos can cause other serious medical complications if it is not treated. Some patients require a catheter inserted into the vagina to maintain drainage of the hydrocolpos until their reconstructive surgery.

Formal Evaluation

The surgeon, urologist and gynecologist will review your child’s records. They will develop a plan unique to your child, which may include:

• Examination under anesthesia, vaginoscopy, cystoscopy and 3-D cloacagram: A procedure performed while your child is asleep, which allows the surgeon to understand your child’s anatomy in order to plan the reconstructive surgery.
• Kidney ultrasound: To check how well the kidneys and bladder are emptying.
• Blood tests: To check kidney function.
• Spinal ultrasound or MRI: To rule out tethered spinal cord, a condition in which the spinal cord is abnormally attached to the spine.
• Spine X-ray: An X-ray of the lower spine, which helps the surgeon predict your child’s potential for bowel control. The development of the lower spine is associated with the development of the nerves and muscles responsible for bowel control. This part of the spine also affects the function of the bladder.

Office visits with your child’s surgeon, urologist and gynecologist to discuss test results and future treatment options.

Reconstructive Surgery

• Your child will have PSARVUP surgery to separate the bowel, urinary tract and vagina.
• Approximately eight weeks after this surgery, the colostomy can be closed.
  • At the time of the colostomy closure your child’s gynecologist will examine the vagina to ensure proper healing.
• Your child’s urologist will decide if more urology testing or surgery is needed.

Long-Term Management

• Your child’s surgeon and urologist will counsel you regarding your child’s potential for successfully potty training or further treatment of the urinary tract.
• If your child is unable to successfully potty train for stool, she may attend our bowel management program. We will find the enema that keeps her clean of stool for 24 hours.
• If your child is unable to empty her bladder adequately or remain dry for urine, the urologist can help you manage this with intermittent catheterization and/or other medical therapies.
• When your child begins to show signs of puberty (breast bud development, pubic hair) follow-up with the gynecologist is needed.
• Long-term follow-up with your child’s colorectal surgeon, urologist, and gynecologist is key to her physical and psychosocial health.