Updates

Persistent cloaca

Conditions

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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:

  1. Urinary control
  2. Bowel control
  3. 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

Treatment:

  • 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.