Urogenital sinus is a rare fetal abnormality of the urogenital system. This abnormality is specific to female fetuses. A single tissue is supposed to split in three to form urethra, vaginal opening and anus during development. In some babies, this development gets disturbed and the tissue does not split. These babies are then born with a combined opening for both urethra and the vagina known as urogenital sinus.
Urogenital sinus is a rare but complicated condition. It seriously affects the quality of life of the child and can only be repaired through surgery.
There are two types of urogenital sinus
In this type the opening compensating for urethra and vagina is short. Urethra and vaginal have normal size and their location is also correct.
This is the more serious type. In this type, the anus is farther and the combined opening is long.
Causes:There is no single widely agreed upon cause of urogenital sinus. Some of the speculated causes are as follows
Congenital Adrenal Hyperplasia (CAH):
CAH is a condition in which one or both of the adrenal glands do not produce required enzymes. When these enzymes are not produced, certain hormones are unable to develop. Hormones play a big role in development of the baby and thus CAH can lead to a developmental abnormality such as urogenital sinus.
Virilization is another condition related to adrenal hormones. Over-production of male sex hormones in female fetuses leads to virilization which can also cause urogenital sinus.
Urogenital is a birth defect and is usually detected at the time of birth. The pediatrician who checks the baby initially usually can tell if there is a chance of an abnormality. Once the abnormality is detected the doctor might order blood tests and perform a physical exam. Two methods that confirm the diagnosis are as follows.
This procedure is basically an X-ray. A dye is first injected into the combined opening. This dye when viewed under X-ray points out the length of the opening. This test also gives information about the nature of fusion of urethra and vaginal opening.
In some cases, the Genitogram might not give the whole picture. In such situations an endoscopy is performed. A fiber optic camera is put inside the opening and the anatomy is observed on a big screen.
The only treatment for urogenital sinus is surgery. The type of surgery will depend upon the severity of the case.
Clitoroplasty: The tissue of the combined opening is reshaped surgically to form a female clitoris. This procedure involves the risks of nerve damage. The complications might include low or no sex drive after the procedure.
Flat-vaginoplasty: This procedure is required for low type urogenital sinus. The surgeon opens up the area of the combined opening so that urethra and vaginal opening can be separated.
Pull-through vaginoplasty: This procedure is required for high type of urogenital sinus. The vagina is relocated and a piece of the bowel is used to create a separate opening. The urethra still uses the combined opening for drainage.
Labioplasty: This surgery involves reconstruction of labia using tissues from the nearby skin.
If your baby has been diagnosed with urogenital sinus, you should visit a top urologist in Pakistan as soon as possible. You can also visit our website at oladoc.com and look for top urologists in Lahore, Karachi and Islamabad. You can also call our helpline at 042-3890-0939 to get directed to the specialists that are suitable for your specific concerns.