What Is Urogenital Sinus?
A urogenital sinus (UG sinus) is a female developmental abnormality during fetal development. The abnormality affects the genitourinary tracts (organ system encompassing the reproductive and urinary systems). This abnormality has various forms, but the most frequent include the vaginal tract (vagina) and urinary system (urethra) merging into one exit out of the body. In a female, the vaginal and urine tracts usually have distinct exits from the body. Children with urogenital sinuses will have just one outflow from the body for both systems (the urethra and vagina will join to form one tube). The ovaries and fallopian tubes are usually untouched and normal. Many of these problems can be detected on prenatal ultrasound, and the CHOC urology team will be able to offer information to expectant families about their child's future care before and immediately after delivery.
What Causes Urogenital Sinus?
It is not always possible to determine the cause of the urogenital sinus. Congenital adrenal hyperplasia (CAH) is a disorder in which one or both of a person's adrenal glands do not generate the enzyme required for hormone development. This issue affects both males and girls, but girls are more susceptible to developing abnormally shaped genitals as a result.
What Are the Symptoms of Urogenital Sinus?
An urogenital sinus can take several forms. Symptoms include:
- A female has only one additional entrance from the body in the vaginal area and a rectal orifice.
- Vaginal duplication, or atresia, refers to an abnormal closure or lack of a vagina.
- Other female reproductive organs, such as the uterus, fallopian tubes, or ovaries, do not develop.
- There is a difference in the development of the bladder, the rectum, the abdominal wall, and reproductive organs.
- The vaginal and urethra shared route, or channel, is somewhat short. The length of the vagina is about normal.
- The urethral opening is in its typical position. The urethral opening is internal, and the vaginal aperture is quite short. This kind is sometimes connected with a forward-located anus (the orifice via which solid waste flows through the body).
What Is the Diagnosis of Urogenital Sinus?
A prenatal ultrasound can sometimes detect the urogenital sinus. It is usually identified at the baby's initial inspection, shortly after birth. Typically, the doctor may prescribe blood tests to learn more about the patient's overall health and genetic concerns. The physician may also consult with a professional to assess them. Other tests, besides blood testing, can offer more information regarding the extent of the urogenital sinus.
Among these tests are:
- Retrograde Genitogram: A dye is injected into the shared urethra or vagina, and X-rays of the region are obtained. The dye aids in defining tissues and organs, allowing their size, shape, and position to be observed.
- Endoscopy: A camera is put into the shared urethra or vagina to examine the anatomy.
- Ultrasound: It is a method of transmitting high-frequency sound waves into bodily tissues. The echoes are captured and converted into video or photographic representations of the body's interior systems. This test determines whether there are any issues with the kidneys, the bladder, vagina, or rectum. It can also reveal any edema or urine accumulation caused by the birth abnormality.
- Magnetic Resonance Imaging (MRI): An MRI is a test that produces highly accurate pictures of the human body without X-rays using a huge magnet, radio waves, and a computer. In newborns with urogenital sinuses, the doctor may request an MRI to check the pelvis and spine for abnormalities.
What Is the Treatment of Urogenital Sinus?
The only way to cure the urogenital sinus is to separate the baby's vagina and urethra via surgery. In "low joined" situations, a surgeon will almost always perform a "flap vaginoplasty." This surgery entails making two distinct holes in the body, one for the vagina and one for the urethra. In "high joined" situations, the surgeon will often undertake a more involved procedure known as "pull-through vaginoplasty," in which the vagina is separated from the shared entrance and transformed into its entity.
The aperture is closed, and urine continues draining through the common entrance. A urogenital mobilization is another treatment that is utilized in conjunction with a flap vaginoplasty. Some females may also require clitoroplasty or labiaplasty. Clitoroplasty is the process of altering tissue to create a more normal clitoris. The nerves are kept to the greatest extent possible so that the patient can have a healthy sexual life as an adult. Labiaplasty is the remodeling of the tissues - the "lips" - that surround the vagina.
What Is the Prognosis of Urogenital Sinus?
Reconstructive surgery is required. Most patients remain fertile and continent, although around 50 percent require occasional catheterization.
What Are the Complications of Urogenital Sinus?
Persistent urogenital sinus is typically linked with various complicated anatomic abnormalities of the urinary, genital, and gastrointestinal systems. Failure to characterize these anomalies appropriately can lead to catastrophic problems. One such category of problems arises quite late in these children's clinical history. These are the complications of menarche. Anatomic anomalies that predispose to insufficient menstrual flow include the dual and septate vagina, uterus didelphys, vaginal atresia, and uterus bicornis unicollis. Early anatomical characterization and long-term monitoring of children with urogenital sinus abnormalities are critical to avoid potential future issues.
Conclusion
Urogenital sinus (UGS) is a sporadic congenital defect of the genitourinary system, and managing UGS remains a difficult challenge throughout childhood, adolescence, and adulthood, with little agreement, particularly on the timing of surgery and long-term sexual function follow-up. To overcome the rarity of this UGS, a multidisciplinary team of experts in psychology, endocrinology, pediatric urology, and urology must be organized to perform long-term collaborative management. More investigation into the etiology and genetic components will help us better comprehend this uncommon disease.
