Vaginal Anomalies: Urogenital Sinus

The urethra and vagina are separate anatomical entities in normal females. But in rare instances, they are joined in what urologists call a urogenital sinus anomaly. The following information can help you talk to your child's urologist about correcting this rare birth defect.

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What are urogenital sinus anomalies?

A urogenital sinus anomaly is a defect present at birth in which the vagina and urethra open into a common channel, rather than separately. There are two general types of urogenital sinus anomalies. In a low confluence urogenital sinus anomaly, the common channel is short, the urethral opening is close to its normal location and the vagina is almost normal in length. In a high confluence urogenital sinus anomaly, the common channel is long, the urethral opening is internal and the vagina is quite short. This type is sometimes associated with an anus that is located too far forward.

Urogenital sinus malformations are usually diagnosed during infancy by physical examination. If a urogenital sinus defect is suspect in an infant, an examination called a genitogram will be performed. To do a retrograde genitogram, contrast dye will be injected into the common opening. An X-ray will then be taken which will permit the doctors to determine the length of the common channel and the spatial relationship between the urethra and the vagina. This information will allow the urologist to determine the type of urogenital sinus anomaly and implement the appropriate treatment.

If a retrograde genitogram is inadequate, endoscopy may be done. In endoscopy, a fiber-optic camera is inserted into the common channel, which will allow the anatomy to be seen and identified. Other tests used under special situations include an ultrasound and MRI.

Surgery to separate the vagina and urethra is the only treatment for urogenital sinus anomalies. Since there are many procedures for this operation, you and your child's doctor will decide the best approach, depending on the type of anomaly. If your child is diagnosed with a low confluence urogenital sinus anomaly, the surgeon will perform what is known as a flap vaginoplasty — a procedure to open the sinus so that the vagina and urethra have separate exterior openings. If she has a high confluence urogenital sinus anomaly, the surgeon will perform a pull-through vaginoplasty. In this procedure the vagina is brought to its normal location on the surface of the skin while the urethra continues to drain through what was once the common channel. Sometimes, a piece of skin or a section of bowel may be needed for this procedure.

If the problem is corrected, urination should be normal.

Yes, once the disorder has been corrected, she will be able to have a normal, enjoyable sex life.

Yes, once corrected, she should have no problem conceiving or bearing children.

Yes, she should be able to deliver children normally.

Reviewed October, 2009

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Glossary Terms

anatomy:
	The physical structure of an internal structure of an organism or any of its parts.
anus:
	Opening at the end of the digestive tract where feces (stool) leave the body. The final two inches of the rectum.
bowel:
	Another word for intestines or colon.
endoscopy:
	Procedure that utilizes an endoscope.
flap vaginoplasty:
	Surgical procedure to correct ambiguous genitalia by reconstructing the vagina.
gene:
	The basic unit capable of transmitting characteristics from one generation to the next.
genitogram:
	X-ray examination of the internal genital duct system.
ions:
	Electrically charged atoms.
MRI:
	Also referred to a magnetic resonance imaging. A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
pull-through vaginoplasty:
	Surgical procedure to create or repair an abnormal or absent vagina where the upper vagina is filled with blood so that the upper vagina can be brought down to the area where a normal vagina opening should occur.
retrograde:
	Backwards.
retrograde genitogram:
	Special contrast X-rays to diagnose ambiguous genitalia.
sinus:
	Abnormal opening. Elongated tract leading from pus-filled region of the body to the exterior or to the cavity of a hallow organ.
ultrasound:
	Also referred to as a sonogram. A technique that bounces painless sound waves off organs to create an image of their structure to detect abnormalities.
urethra:
	A tube that carries urine from the bladder to the outside of the body. In males, the urethra serves as the channel through which semen is ejaculated and it extends from the bladder to the tip of the penis. In females, the urethra is much shorter than in males.
urethral:
	Relating to the urethra, the tube tha carries urine from the bladder to outside the body.
urogenital:
	Refers to the urinary and reproductive systems.
urologist:
	A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)
vagina:
	The tube in a woman's body that runs beside the urethra and connects the uterus (womb)to the outside of the body. Sometimes called the birth canal. Sexual intercourse, the outflow of blood during menstruation and the birth of a baby all take place through the vagina.
vaginoplasty:
	An operation in which a concealed vagina is brought out onto the surface or its existing opening is widened.

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