Persistent Cloaca in Females
Persistent cloaca, a complex malformation, is defined as a defect in which the rectum, vagina and urinary tract are fused together into a single common channel that communicates exteriorly through a single perineal orifice located at the normal urethral site.
The diagnosis of persistent cloaca is easily established purely on clinical assessment. The patient's genitalia appears to be smaller than normal.
Meticulous inspection of the small labia discloses a single perineal orifice, which is the characteristic of this defect.
Urologic defects occur at a rate of 90 percent in children with a cloaca anomaly and may require immediate attention. These patients represent a potential urologic emergency.
An abdominal ultrasound examination must always be performed followed by a urologic workup when necessary before the surgical creation of a colostomy.
These infants require a diverting colostomy and concomitant urinary diversion (vesicostomy, ureterostomy) or vaginal diversion (vaginostomy) in cases of obstructed, distended vaginas.
When the infant is older than three months, the entire malformation is repaired with an operation called Posterior Sagittal Anorectovagino-urethroplasty / PSARPVUP.
Some surgeons wait until the infant is older than one year of age or weighs 20 pounds to more easily visualize the anatomy. However, some surgeons prefer to do the PSARVUP as early as possible as long as the infant is developing and gaining weight at a normal rate.
Approximately 40 percent of the time, it is necessary to open the abdomen simultaneously with the posterior approach to reach and mobilize a very highly located rectum and or vagina.
For more information or to request an appointment, please contact the Colorectal Center at Cincinnati Children's.
