When an infant first begins to develop in the uterus, the anorectal, urogenital and reproductive systems meet in the same opening, called a cloaca.
- Before birth, these 3 areas of the body should separate into 3 different tracts, each with its own passage, or channel, and opening to the outside of the body.
- When these 3 tracts do not separate, the infant girl will be born with a cloaca, which means the rectum/anus, the urethra (the tube through which urine flows) and the vagina share the same opening. This birth defect occurs in 1 per 20,000 live births.
Doctors who treat an infant who has this condition have three main concerns:
- Urinary control
- Bowel control
- Sexual function (future menstruation, intercourse and obstetric issues)
Patients can be seen by Texas Children's experts in Complex Urogenital Reconstruction.
Causes & Risk Factors
Persistent cloaca is caused by a birth defect. Its cause is not known.
Symptoms & Types
It can be difficult to diagnose this condition through ultrasound before birth. Most of the time the diagnosis is made by physical examination at birth.
Diagnosis & Tests
Diagnosis can be made by examining the perineum (the part of the body between the pubic bone and the anus). The presence of a single opening in this part of the body provides evidence of this condition.
Testing should be done to determine the extent of the problem. These tests may include:
- Cystoscopy-a procedure to see inside the bladder and the urethra
- Vaginoscopy-a procedure to see inside the vagina
- Endoscopy-a procedure to see inside the upper digestive tract
These procedures help the physician to find out important information for surgery. This includes:
- The length of the common channel, or passage, that the urethra, vagina and rectum/anus share
- The presence of a vagina
- The presence of a cervix
- The presence of an anal fistula (a small channel that can develop between the end of the bowel and the skin near the anus)
Treatment & Care
- Surgery to rebuild the anatomy (done in several stages)
- Goal: to achieve bowel and urinary control, as well as normal sexual function
Living & Managing
These infants will need long-term follow-up to see whether they have bowel and bladder problems. They may also need more surgery during their teenage years.
Possible long-term effects:
- Not having complete control over the bowel and the bladder- this can occur even with excellent surgical repair. These problems can be caused by poorly developed nerves, spinal cord defects or a poorly developed sacrum (bony structure that stabilizes the pelvis).
- For some patients, a bowel management program may be necessary for a better quality of life.