Preferred Label : Persistent urogenital sinus;
ICD-11 definition : A urogenital sinus anomaly is a rare birth defect in women where the urethra and vagina
both open into a common channel. The urogenital sinus is a part of the human body
only present in the development of the urinary and reproductive organs. It is the
ventral part of the cloaca, formed after the cloaca separates from the anal canal
during the fourth to seventh weeks of development. In females, the pelvic part of
the UG sinus gives rise to the sinovaginal bulbs, structures that will eventually
form the inferior two thirds of the vagina. This process begins when the lower tip
of the paramesonephric ducts, the structures that will eventually form the uterus
and vaginal fornices, come in contact with the UG sinus. Shortly afterwards, the sinovaginal
bulbs form as two solid evaginations of the UG sinus. Cells in these bulbs divide
to form a solid vaginal plate, which extends and then canalizes (hollows) to form
the inferior portion of the vagina. The female urogenital sinus also gives rise to
the urethra and vestibule of the vagina. Persistent urogenital sinus can be associated
with different syndromes such as Bardet-Biedel syndrome or Mc Kusick Kaufman syndrome.;
Origin ID : 1944331687;
Automatic exact mappings (from CISMeF team)
A urogenital sinus anomaly is a rare birth defect in women where the urethra and vagina
both open into a common channel. The urogenital sinus is a part of the human body
only present in the development of the urinary and reproductive organs. It is the
ventral part of the cloaca, formed after the cloaca separates from the anal canal
during the fourth to seventh weeks of development. In females, the pelvic part of
the UG sinus gives rise to the sinovaginal bulbs, structures that will eventually
form the inferior two thirds of the vagina. This process begins when the lower tip
of the paramesonephric ducts, the structures that will eventually form the uterus
and vaginal fornices, come in contact with the UG sinus. Shortly afterwards, the sinovaginal
bulbs form as two solid evaginations of the UG sinus. Cells in these bulbs divide
to form a solid vaginal plate, which extends and then canalizes (hollows) to form
the inferior portion of the vagina. The female urogenital sinus also gives rise to
the urethra and vestibule of the vagina. Persistent urogenital sinus can be associated
with different syndromes such as Bardet-Biedel syndrome or Mc Kusick Kaufman syndrome.
