Female genital malformations include various
forms of developmental and morphological malformations
of the vagina, cervix, uterus, adnexa
and associated malformations (VCUAM) (
Congenital anomalies of the cervix are rarely
isolated, and are more commonly associated with
other uterine and vaginal anomalies. Cervical
anomalies may manifest as agenesis, dysgenesis,
obstruction, abnormal length, inadequate width,
and hypertrophy (
Several imaging tools have been used in the assessment
of Müllerian duct anomalies (MDAs). Although
magnetic resonance imaging (MRI) is considered
the gold standard for definitive diagnosis and
classification of genito-urinary anomalies, especially
for complex cases, hysterosalpingography is still an
important tool in the early evaluation of infertility.
Contrast medium which is slowly injected into the
uterus through the cervical canal, provides good outlines
of the uterine cavity and fallopian tubes, as well
as the cervical canal and isthmus (
We retrospectively reviewed 38574 hysterosalpingograms (HSGs) performed over a 29-year period (January 1985-December 2013) by one author (G.Sh.). The indications for HSG included infertility, abnormal uterine bleeding, and symptoms related to uterine fibroids. This review illustrates the various radiographic appearances of congenital malformations of the utero-cervix with a brief overview of the embryologic features.
The female genital tract develops from a pair of
Müllerian ducts between 6 and 12 weeks of gestation.
The process involves three main stages: i.
Development of both Müllerian ducts that form
the fallopian tubes, uterus, and cervix and upper
two thirds of the vagina, whereas failure of this
stage results in agenesis/hypoplasia or a unicornuate
uterus, ii. Fusion of the lower Müllerian ducts
leads to formation of the uterus and cervix while
defects in this phase result in a bicornuate uterus,
and iii. Canalization and septal resorption of the
central septum which results in a single uterine
cavity and cervix, whereas failure of this stage
leads to a septate or arcuate uterus. Mesonephric
or Wolffian ducts play a role as inductors for adequate
development, fusion, and resorption of the
walls of Müllerian ducts (
By week 20, the process of development is completed. Development of both Müllerian ducts and the urinary tract occurs from a common ridge of the mesoderm; hence, anomalies of the urinary tract are commonly observed in females with genital malformation.
Accurate classification of a female genital tract
malformation is necessary to prevent inadequate
surgery and achieve optimal treatment. Until now,
several classification systems for female genital
tract anomalies have been proposed: the American
Fertility Society Classification System (currently
American Society for Reproductive Medicine,
The AFS classification is based mainly on the description
of uterine changes and most widely used
as the main classification system for its simplicity,
friendliness and clinical usefulness. However, this
system is associated with limitations in efficient
categorization of female genital anomalies. The accompanying
malformation (such as duplex vagina),
unusual or/and complex malformations especially
those in group I (hypoplasia/agenesis) not fit and
are described completely in the AFS classification
and so often fails to correctly identified and treated
The use of embryological-clinical classification
of genitor-urinary malformations seems to unify
the current embryological and pathogenic concepts
and appear to be the most clinically useful
The accurate diagnosis of such cases is considered essential for optimal treatment and in support of the embryologic concept.
Agenesis/dysgenesis of the cervix is rare and
usually occurs in association with complete or partial
vaginal agenesis (
Uterus didelphys results from complete failure of Müllerian duct fusion and accounts for approximately 5% of MDAs (
Didelphys uterus in a 31 year-old-woman with 3 years of primary infertility. Hysterosalpingogram demonstrates two symmetrical separate cavities, two cervical canals and presence of double vagina.
The bicornuate uterus represents approximately 25% of MDAs and results from incomplete fusion of the Müllerian ducts at the level of the uterine fundus (
Bicornuate uterus with various degrees of duplication of the cervix in different patients (AFS class IV). A. Bicornuate uterus, consisting of two symmetric uterine cavities with communication at the uterine isthmus, and also intercorneal angle greater than 105º. Single cervix and vagina are present, B. Intervening cleft extends to the endocervical canal, and C. Extension to the level of external os.
A septate uterus results from partial or complete failure of resorption of the uterovaginal septum after fusion of the paramesonephric ducts, which occurs in approximately 35% of MDAs (
Hysterosalpingography of septate uterus demonstrates a variable degrees of cervical septation in different patients (AFS class V) (
Exposure to DES in utero results in multiple, benign abnormalities of the genital tract and clear cell adenocarcinoma of the vagina. DES has been associated with T-shaped and irregular configurations of the endometrial cavity, constrictive bands, structural cervical changes, and cervical anomalies that include hypoplasia, cervical ridges, and cervical collars (
Hysterosalpingography is an excellent screening tool to diagnose DES-related uterine anomalies. The radiographic appearance includes an irregular, narrowed endocervical canal with a shortened upper uterine segment and small, typically irregular cavity that yields a T-shaped uterine configuration (
In 1984, Toaff et al. (
DES-exposure uterus in a 28-year-old infertile patient. Note T- shaped appearance of the endometrial cavity with a long narrowed irregular endocervical canal.
During >29 years of one author’s experience (G.SH) in performing HSGs, some rare cases of communicating uteri have been observed and reported (
Some cases of communicating uteri with duplication of carvical canal (
Hysterosalpingography in a 23 year-old-woman with 5 years of primary infertility. The uterus is septate with a midcorporeal communication (straight arrow). The cervix is partially septate and the cervical septum ends a few millimeters above the single external os (open arrow) (
Bicornuate uterus in a 26 year-old-woman with 3 years of primary infertility. Hysterosalpingogram shows the bicornuate uterus with two sites of communication between two uterocervical canal, midcervical (curved arrow) and isthmic (asterisk); septum of the cervix ends several millimeters above the single external os (straight arrow) (
Hysterosalpingography in a 27-year-old woman with primary infertility of 5 years. The uterus is bicornuate with a low cervical communication and left atretic hemicervix. The cannula is located in the right hemicervix. The right external os is visible. Injection of contrast into the right hemicervix was noted to opacify the left hemiuterus and cervix through the low cervical communication (arrow) (
Some unclassified cases of normal or septate uteri that communicate with double cervices with or without vaginal septa have been previously described (
Hysterosalpingogram of a 36 year-old woman shows an normal uterus with septate cervix and vagina.
In the second case, the proximal part of the cervical canal was double, but the distal portion was single and one cervical opening was present (
Infertility of a 27 year-old woman investigated. HSG demonstrates invagination of distal part of cervical canal within proximal part of cervix. In this patient proximal part of cervical canal is double but the distal portion is single and the patient has one cervical opening.
The third case was a bicornuate uterus with communication at the isthmic level, a septate cervix, and normal external cervical os and vagina (
A 29- year-old woman with a history of 5 years of primary infertility. HSG represents a bicornuate uterus with communication at the level of isthmus, a septate cervix, and normal cervical os and vagina. The patient has no history of previous vaginal and cervical septum resection.
In the last case, HSG demonstrated a left blind hemivagina (atretica) with left renal agenesis (Herlyn-Werner- Wunderlich syndrome,
A 24-year-old woman with a history of 3 years infertility. HSG shows a septate uterus with a left blind hemivagina(atretica) and left renal agenesis (Herlyn-Werner- Wunderlich syndrome).
Although congenital anomalies of the utero-cervix in the setting of MDAs are rare, the impact on a woman’s reproductive potential can be significant. Anomalies of the cervico-uterus are widely diagnosed by HSG. The diagnostic value of HSG in the detection of anomalies varies, depending on the type of malformation. Accurate diagnosis of these cases, especially the cases with any classification system, is important for optimal treatment and categorization of each anomaly.