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Uro-Genital Sinus and Cloacal Anomaly

By Philip E. Gleason, MD

Urogenital sinus and cloacal anomalies are rare Pediatric Urology diagnoses in which the bladder, vagina and rectum may be partially or completely fused together. This section topic will provide introductory information on these complex conditions.

During development in-utero, the genito-urinary and intestinal tracts initially form together, technically known as a cloacal formation. As development progresses, the genito-urinary bladder, vagina and genitalia are separated from the bowel and intestinal tract by formation of the uro-rectal septum. In females, the bladder and urethra then further separate from the vagina.

Thus, normally, three organ systems open along the perineum. In girls there are three openings: the urethra from the bladder and urinary tract, the vagina from the genital tract, and the anus and rectum from the intestinal tract.

In boys, the urinary and genital tracts join together in the urethra with the opening from the penis, The intestinal tract again opens as a second opening from the anus and rectum. However, there can be a wide range, or spectrum, of urologic and intestinal abnormalities of the perineum.

The most common situations are more isolated abnormalities of the intestinal tract, including the anus and rectum, and are know as an imperforate anus. These can occur in both boys and girls and are primarily managed by Pediatric General Surgeons.

However, in females, if the intestinal and genito-urinary tracts are involved together, this is called a cloacal anomaly and will begin to involve the urinary tract and Pediatric Urologists.

If only the urinary and genital tract, involving the urethra and vagina, are involved, this is referred to as a uro-genital sinus abnormality. These occur most often in children with Congenital Adrenal Hyperplasia and are also discussed in the intersex and ambiguous genitalia section topics, but can occur independently as well.

The management of cloacal anomalies and uro-genital sinus abnormalities has progressed significantly over the last several years. At birth, the intestinal system is diverted away from the perineum to allow bowel movements with a diverting colostomy. The urinary systems and other organ systems are evaluated for any other abnormalities.

Then, over the next several months or year or so of age, a reconstruction is performed to separate the urinary and genital and intestinal tracts into their normal openings. Much of this work has been pioneered by Dr. Hardy Hendren at Boston Children's Hospital and Dr. Alberto Pena. Further contributions by Dr. Passerini in Italy and Dr. Rick Rink at Riley Children's Hospital in Indianapolis have advanced repairs by posterior sagittal ano-recto-vagino-plasty and by total urogenital sinus mobilization.