What is Persistent Cloaca?
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 happens in about 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 done to look at their anatomy as well as to see if there are any other malformations. Urologic malformations are common in persistent cloaca and often need immediate attention and close follow up.
How is Persistent Cloaca Treated?
Persistent cloaca is treated in phases so that immediate issues can be managed while further evaluation and surgery plans can be made.
Initial Management
- A physical exam and testing are done to diagnose any associated issues.
- Surgery is done to create a colostomy, or opening on the abdomen to divert stool to empty into a stoma bag.
- Some patients also need surgery to allow proper drainage of urine from the bladder.
- Some patients also need surgery to allow proper drainage of fluid from the vagina. This collection of fluid is known as hydrocolpos. Hydrocolpos can cause other serious medical problems if it is not treated. Some patients need a catheter inserted into the vagina to drain the hydrocolpos until their reconstructive surgery.
Formal Evaluation
The colorectal surgeon, a urologist, and a gynecologist will review your child’s records. They will develop a plan specifically for your child, which may include:
- Examination under anesthesia, vaginoscopy, cystoscopy and 3-D cloacagram
- This is done while your child is asleep, and allows the surgeon to understand your child’s anatomy so they can 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 how the bladder works.
You’ll have 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.
- About 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 make sure it is healing properly.
- 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.
What is Long-Term Management for Persistent Cloaca Like?
- Your child’s surgeon and urologist will talk with you about your child’s potential for successfully potty training or further treatment of the urinary tract.
- If your child cannot successfully potty train for stool (poop), they may attend a bowel management program. If your child cannot empty their bladder or stay dry from 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 a gynecologist is needed.
- Long-term follow-up with your child’s colorectal surgeon, urologist and gynecologist is key to your child’s physical and psychosocial health.
