Persistent Urogenital Sinus
When an infant first begins to develop in the uterus, the anorectal, urinary/genital and reproductive systems meet in the same opening, called a cloaca.
- Before birth, these 3 systems should separate into 3 different areas of the body, each with its own passage, or channel, and opening to the outside of the body.
- When the urinary and reproductive tracts do not separate, an infant girl will be born with a persistent urogenital sinus, which means the vagina and the urethra (the tube through which urine flows) share the same opening.
2 general types of urogenital sinus conditions exist:
- Low confluence urogenital sinus
- High confluence urogenital sinus
Patients can be seen by Texas Children's experts in Complex Urogenital Reconstruction.
Causes & Risk Factors
Persistent urogenital sinus is caused by a birth defect. The cause is not known.
Symptoms & Types
Low confluence urogenital sinus characteristics:
- The common passage, or channel, that the vagina and the urethra share is short. The vagina is almost normal in length.
- The urethral opening is close to its normal location.
High confluence urogenital sinus characteristics:
- The common passage, or channel, that the urethra and the vagina share is long. The urethral opening is internal and the vagina is quite short.
- This type is sometimes associated with an anus (the opening through which solid waste passes through the body) that is located too far forward.
Diagnosis & Tests
- Before birth by an ultrasound
- After birth by a physical examination
Tests may include:
- Retrograde genitogram: Contrast dye is injected into the common channel and an x-ray is taken. This test gives more detailed information about the placement of the urethra and the vagina in relation to each other.
- Endoscopy: A special type of camera is inserted into the common channel so the doctor can see the anatomy.
- Ultrasound and MRI: These tests give detailed images of organs and structures inside the body.
Treatment & Care
- Surgery to separate the vagina and the urethra is the only treatment.
- The severity of the defect determines the type of surgery.
- The purpose of the surgery is to give your child as normal function and appearance of genitalia as possible.
- The results of the surgery depend in part on how severe a defect is present.
Living & Managing
- Infants, as they grow, should have excellent bladder control and normal sexual development, fertility and function.
- Infants, as they grow, may need life-long bladder treatment to help empty urine. Additionally, some may have decreased fertility or difficulty with sexual function.
At puberty, it may be necessary to reassess a girl's condition to determine whether more surgery is needed.