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

Diagnosis:

  • 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

Treatment:

  • 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

Mild cases:

  • Infants, as they grow, should have excellent bladder control and normal sexual development, fertility and function.

Severe cases:

  • 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.