Document Type : Case Report
1 Gynaecology and Obstetrics Specialist Registrar, Hospital Garcia de Orta, Almada, Portugal
2 Gynaecology and Obstetrics Consultant, Hospital Garcia de Orta, Almada, Portugal
3 Gynaecology and Obstetrics Senior Consultant, Hospital Garcia de Orta, Almada, Portugal
Female genitourinary tract malformations are extremely
common, being found in around 5.5% of the general
population and 8% of infertile women, specifically affecting
as many as 25% of women with infertility due to
A 39 year-old woman was referred to our institution due
to secondary infertility. Menarche was at 14 years of age,
with regular cycles and slight dysmenorrhoea. She had
experienced a term caesarean section 8 years prior due
to failure to progress, and had been trying to get pregnant
for 3 years. Her past medical history was unremarkable.
On gynaecological examination external genitalia and vagina
were normal; two cervical orifices in an anteroposterior
disposition were clearly visualized (
Speculum examination showing anterior (black arrow) and posterior (white arrow) cervical OS.
Hysterosalpingogram showing a normal uterine cavity with contrast extravasation through a posterior cervical canal (black arrow).
Transvaginal ultrasound displaying one internal cervical os (black arrow) and two cervical canals (white arrows).
An extremely rare müllerian malformation is described,
which questions classical knowledge of developmental
embryology. An extensive literature search was conducted
revealing only a few similar cases (
The aetiology for most of the congenital anomalies of
the female genital tract is unknown. The importance of
normal embryological development lays in its reproductive
consequences, and also in concomitant urological abnormalities
(which are more common with more proximal
defects) and in quality of life (for possible dysmenorrhoea
or dyspareunia in obstructive defects). In 1988 the ASRM
attempted to classify these anomalies (
Two main theories, both described in 1960s, are the
foundations for the classification system of the ASRM.
The first theory, described by Crosby and Hill (
Acién et al. (
More recently, the European Society of Human Reproduction
and Embryology (ESHRE) developed an
anatomy-based consensus on congenital anomalies of the
female genital tract and its related clinical significance,
in a comprehensive and accessible system. Here, cervical
and vaginal anomalies are categorized into independent
Embryology of the female genital tract is not completely understood, as the mechanism of müllerian development is more complex than previously described. Acién’s segmentary theory is currently the one that best explains segment malformations as the one presented here. This theory puts into question our decade-long understanding of embryological development of the female reproductive system, and supports the classification system of müllerian anomalies of ESHRE. The fact that in our case the two cervices were displayed in an anteroposterior fashion also calls into question the fact that development may not always occur in a side-by-side manner.