Chapter 3 Congenital Genitourinary Abnormalities
Chapter 3 Congenital Genitourinary Abnormalities
spontaneous abortion, or midpregnancy and preterm delivery. Lumen remains separated from the urogenital sinus by the
hymeneal membrane.
GENITOURINARY TRACT DEVELOPMENT This membrane further degenerates to leave only the
Embryologically in females, the external genitalia, gonads, and mllerian ducts hymeneal ring.
each derive from different primordia and in close association with the urinary Close association of the Mesonephric (Wolffian) and Paramesonephric
tract and hindgut. (Mllerian) Ducts explains why there are commonly simultaneous abnormalities
Abnormal embryogenesis of these is thought to be multifactorial and can involving these structures
lead to sporadic anomalies. Half of females with Uterovaginal malformations have associated urinary
tract defects.
Anomalies most frequently associated with renal defects are Unicornuate
Uterus, Uterine Didelphys, and Agenesis Syndromes
Arcuate and bicornuate are less commonly linked
When these are identified, the urinary system can be evaluated with
magnetic resonance (MR) imaging, sonography, or intravenous
pyelography
Ovaries are functionally normal but have a higher incidence of anatomical
maldescent into the pelvis
MESONEPHRIC REMNANTS
Mesonephric ducts usually degenerate, however, persistent remnants may
become clinically apparent.
Mesonephric or Wolffian vestiges can persist as Gartner Duct Cysts.
Typically located in the proximal anterolateral vaginal wall but may be
found at other sites along the vaginal length.
They can be further characterized by magnetic resonance (MR) imaging.
Most are asymptomatic and benign
They May Measure Up To 7 Cm In Diameter
They usually do not require surgical excision.
An infected cyst occasionally requires marsupialization.
Intraabdominal Wolffian remnants in the female include a few blind tubules in
the mesovarium (Epophoron) as well as similar ones adjacent to the uterus
collectively (Parophoron)
Epophoron or parophoron may develop into clinically identifiable cysts
Included in the differential diagnosis of an adnexal mass
Uterine Abnormalities
Accurate population prevalence of these are difficult to assess because the best
diagnostic techniques are invasive
Population prevalence ranges from 0.4-5%, and rates in women with recurrent
miscarriage are significantly higher
Distribution of uterine anomalies as follows:
Bicornuate, 39%
Septate 34%
Didelphic 11%
Arcuate 7%
Unicornuate 5%
Hypo- Or Aplastic 4%
Mllerian anomalies may be discovered at routine pelvic examinations,
cesarean delivery, during laparoscopy for tubal sterilization, or during infertility
evaluation.
Depending on clinical presentation, diagnostic tools may include
Hysterosalpingography
Sonography
MR imaging
Laparoscopy
Hysteroscopy
Each has limitations, and these may be used in combination to completely
define anatomy.
Mllerian Agenesis
Class I segmental defects can be caused by mllerian hypoplasia or agenesis In women undergoing fertility evaluation Hysterosalpingography (HSG) is
These developmental defects can affect the vagina, cervix, uterus, or commonly selected for uterine cavity and tubal patency assessment.
Poorly defines the external uterine contour and can delineate only patent
fallopian tubes
cavities.
May be isolated or may coexist with other mllerian defects.
Contraindicated during pregnancy.
In most clinical settings, sonography is initially performed.
Vaginal Abnormalities
In addition to vaginal agenesis, there are two types of congenital septa. Transabdominal views may help to maximize the viewing field, but transvaginal
1) Longitudinal Septum: arises from a fusion or resorption defect. sonography (TVS) provides better image resolution.
2) Transverse Septum: results either from incomplete canalization or from For this indication, the pooled accuracy for TVS is 90-92%
vertical fusion failure between the down-growing mllerian duct system Saline infusion sonography (SIS) improves delineation of the endometrium
and the up-growing urogenital sinus. and internal uterine morphology, but only with a patent endometrial cavity.
Sacculation
Persistent entrapment of the pregnant uterus in the pelvis may lead to extensive
lower uterine segment dilatation to accommodate the fetus.
Sonography and MR imaging are typically required to define anatomy
Cesarean delivery is necessary when there is marked sacculation
An elongated vagina passing above the level of a fetal head that is deeply
placed into the pelvis suggests a Sacculation or an abdominal
pregnancy.
Foley catheter is frequently palpated above the level of the umbilicus
Recommended extending the abdominal incision above the umbilicus and
delivering the entire uterus from the abdomen before hysterotomy.
This will restore correct anatomical relationships and prevent
inadvertent incisions into and through the vagina and bladder.
Unfortunately, this may not always be possible
Uterine Torsion
It is common during pregnancy for the uterus to rotate to the right side.
Rarely, uterine rotation exceeds 180 degrees to cause torsion.
Most cases of torsion result from
Uterine leiomyomas
Mllerian anomalies
Fetal malpresentation
Pelvic adhesions
Laxity of the abdominal wall or uterine ligaments.