Cloacal Malformation

The cloacal malformation or anomaly is a complex congenital problem defined by a confluence or coming together of the urinary tract, vagina, and rectum to form a single common channel that opens on the perineum. It is this common channel that is referred to as a cloaca.

This problem occurs exclusively in females and carries an incidence of approximately 1 in 20,000. Occasionally, this diagnosis may be considered when a combination of abnormalities of the bladder and vagina are detected antenatally, but most often it is diagnosed during the immediate newborn period due to the discovery of a single perineal opening.

What Is the Cause of Cloacal Abnormalities?

The cause of this defect is unknown.

How Are Cloacal Malformations Treated?

The management of cloacal malformations requires a collaborative effort by experienced pediatric general surgeons and pediatric urologists.  Initially, the goals are to identify associated abnormalities, to provide drainage of the urinary tract and to divert the bowel using a colostomy. Antibiotic prophylaxis (low dose once a day antibiotic) is given to prevent urinary tract infections.  Imaging studies important in the newborn period include: ultrasonography of the kidneys, bladder, and other pelvic organs (renal bladder, pelvic ultrasound), plain films of the spine and sacrum, and spine ultrasound.  A commonly associated genitourinary abnormality occurring in 50% of cases is hydrometrocolpos, which is a fluid collection within the bladder and uterus that may press on the base of the bladder neck, leading to obstruction of the bladder.  Often, there is dilation of the kidney drainage system (hydronephrosis), and vesicoureteral reflux (regurgitation of urine from the bladder to the kidneys) may be present.  Spine and sacral are abnormalities are common and include hemivertebra (incomplete development of one side of a vertebra) and incomplete development of the sacrum.  The purpose of spinal ultrasound or spine MRI is to detect a “tethered cord.”   This problem is present in 40% of children with cloacal malformations refers to an abnormal position of the spinal cord within the bony spinal column.  When such problems are detected, pediatric neurosurgeons and orthopedists are consulted.

While the initial goal is to stabilize the child and relieve blockages in the urinary and intestinal tract, the long-range goals aim to restore anatomy and function.  Great variation exists in anatomy and corrective efforts must be individualized.  Further imaging and endoscopy (using a lighted telescope to view internal structures) are necessary before repairs are performed in order to define the type of repair that is appropriate. Anorectovaginourethoplasty is the name given to the simultaneous and comprehensive repair of the urethra, vagina, and rectum, bringing each to a separate opening on the perineum.  This operation may be done through a midline incision in the perineum if the confluence is relatively close to the surface or may require both abdominal and perineum approaches for a high confluence.

Ultimately, the desire is for children to gain urinary and bowel control and to be able to function sexually.  Achieving these goals may be limited by the severity of the cloacal anomaly.  Even with successful repair, some children may require bladder catheterization to empty their bladders and may remain vulnerable to urinary tract infections. For this reason, children are followed at regular intervals by a urologist throughout life.  Our pediatric urologists are committed to providing long-term care for children with cloacal anomalies.  Generally, patients can live happy, energetic, and productive lives with bowel and bladder social continence.