Urogenital Sinus

What is a urogential sinus?

A urogenital sinus (UG sinus) is a defect in females that occurs during fetal development. The defect involves the genitourinary tracts (organ system that includes the reproductive and urinary organs). There are many variations to this defect, but the most common involve a merging of the genital tract (vagina), and urinary tract (urethra) into one exit out of the body. Normally the genital tract and urinary tract will both have separate outlets from the body in a female. Children with a urogenital sinus will only have one outlet from the body for both tracts (the urethra, and vagina will merge together to make one tube). Typically the ovaries and fallopian tubes will be unaffected and normal. Many of these defects are diagnosed on prenatal ultrasound and the CHOC Urology team will be able to provide information to expecting families before and shortly after birth regarding their child’s further management.

What are the signs of a urogenital sinus?

There are many variations to an urogential sinus. Specific defects may involve:

• A female with a rectal opening and only one other opening from the body in the genital region.
• Vaginal duplication or vaginal atresia, an abnormal closure or absence of a vagina.
• A lack of development in other female reproductive organs, including the uterus, fallopian tubes or ovaries.
• A variation in the degree of development of the bladder, rectum, abdominal wall or reproductive organs.

How is a urogenital sinus diagnosed?

Some urogenital sinus defects will be diagnosed during a prenatal ultrasound. Others will be diagnosed during a physical examination after birth. Following birth, different studies may be completed to better understand the child’s unique anatomy. The child may have an ultrasound, MRI, VCUG or genito/sinogram, or cystoscopy and/or vaginoscopy, before a treatment plan will be developed. Learn more about the diagnostic procedures used by the specialists at the CHOC Urology Center.

How is a urogenital sinus treated?

A urogenital sinus requires surgical repair by one of our surgeons. The exact surgery and subsequent repair will be determined by the child’s family and our surgeon after considering the child’s unique anatomy. Often more than one surgery will be needed.

What is the long-term outlook for children with a urogenital sinus?

The long-term outlook for girls with urogenital sinus will depend greatly on the degree of the defect and any other associated anomalies or defects. Our specialists will discuss a child’s long-term outlook during her appointments.

In general, children with mild anomalies will have excellent bladder control and normal sexual development, fertility and function. Children with more severe anomalies may need life-long bladder intervention to help empty their bodies of urine. Additionally, some may have decreased fertility or difficultly with sexual function.