1. Ovaries from the germ cells that migrate from the yolk sac into the mesenchyme
of the peritoneal cavity and developed into ova and supporting cells.
2. Lower third of vagina developed from the ascending Sino vaginal bulb.
3. Uterus, fallopian tubes &upper two thirds of vagina developed from the fusion
of two Müllerian ducts &this is our subject for discussion
. Complete formation of the genital tract depend on the completion
of 3 stages
• Organogenesis
If one of the tubes fail to develop it results in unicornuate uterus if both
this cause uterine agenesis or hypoplasia.
• Fusion
Two kinds of fusion
1. Lateral fusion process by which the
two Müllerian ducts fuse together to form the uterus, tubes &cervix failure
of this process cause bicornuate or didelphys uterus.
2. Vertical fusion formation of vagina
completed by fusion of the lower part of Müllerian duct that form the upper
two thirds &the ascending Sino vaginal bulb that forms the lower one third incomplete
vertical fusion lead to imperforated hymen.
• Septal resorption
Normally the median part of the fused Müllerian ducts forms septum that resorped
to form single uterine cavity failure of this resorption lead to septate uterus
complete or partial.
Frequency
It occur in0.1_0.5%in healthy fertile population
Clinical presentation
It differ in each patient according to the type of abnormality but nearly the
most common presentations are
• Infertility
• Recurrent abortion
• Preterm labor
• Intra uterine growth retardation
• Primary amenorrhea in imperforated hymen & haematocolpus
Diagnosis
Clinical history &physical examination can not give much about the diagnosis
so the best way is by using imaging techniques:
1. Hysterosalpingography
Have false positive results of 38% & falls negative results of28%it also can
not assess the outer fundal contour making it impossible to differentiate between
septate &bicornuate uterus.
Special criteria putted in mind to confirm the diagnosis by H S G
• Intercornual distance
The distance between the distal ends of the horns
If less than 2 cm means septate uterus
If more than 4 cm means bicornuate uterus
2-4 cm means normal uterus
• Intercornual angle
Formed by the most medial aspects of the two uterine hemi cavities
If less than 60 means septate uterus
If more than 60 means bicornuate uterus
• T shaped uterus means DES exposure
2. MRI
It allow imaging of the uterus in the coronal plane it can assess the uterine
cavity as well as the uterine contour but it is coasty& require referral to
an imaging facility so the diagnosis will be delayed.
3. Three dimensional ultrasound
It provide information that is the same or better than that obtained with MRI
less expensive and readily available in most centers which enables expedited
diagnosis.
When combined with saline infusion 3 D ultrasound yield information similar
to that obtained by HSG.
When scanning a gynecology patient especially if a uterine abnormality is imaged
the kidneys need to be assessed.
Classification of Müllerian duct abnormalities. And its surgical
treatment
Anatomy: Müllerian duct anomalies are categorized most commonly lasses according
to the American Fertility Society (AFS) Classification Scheme (1988 into 7)
as follows:
Class I (hypoplasia/agenesis): This class includes
entities such as uterine/cervical agenesis or hypoplasia. The most common form
is the Mayer-Rokitansky-Kuster-Hauser syndrome, which is combined agenesis of
the uterus, cervix, and upper portion of the vagina. Patients have no reproductive
potential aside from medical intervention in the form of in vitro fertilization
of harvested ova and implantation in a host uterus occur in15-40%& also some
times skeletal abnormalities.
Diagnosis of vaginal agenesis
- presented as primary amenorrhea
- Normal secondary sex characteristics
- Pelvic examination reveals patulous urethra
- Vagina can be completely absent or shunt vaginal pouch can be present.
- Uterus cannot be palpated on rectal examination
- Ultra sound show absent uterus and sometimes renal abnormalities
- Laparoscopy is not indicated unless the diagnosis cannot be determined by
the above findings
- Treatment
1. Non surgical treatment by using graduated dilators may take several months
to few years before a functional vagina is formed so surgery remains the most
effective method of treatment
2. Surgical treatment by vaginoplasty the aim is to create new vagina surgical
treatment should be considered only when the patient wishes to become sexually
active and is highly motivated to use vaginal prosthesis for several months
after surgery there are various methods of vaginal reconstruction we will discuss
some of these methods
- Full thickness skin graft
- Transposition flaps.
Class II (unicornuate uterus): A unicornuate uterus
is the result of complete, or almost complete, arrest of development of 1 Müllerian
duct If the arrest is incomplete, as in 90% of patients, a rudimentary horn
with or without functioning endometrium is present. If the rudimentary horn
is obstructed, it may come to surgical attention when presenting as an enlarging
pelvic mass. If the contra lateral healthy horn is almost fully developed, a
full-term pregnancy is believed to be possible (see didelphys uterus).
- Surgical treatment
Women with unicornuate uterus are not generally considered for reconstruction
metroplasty the only surgical indication is the presence of endometrium In the
accessory horn and this can be removed by laparoscopic hemi hysterectomy while
the operation not indicated for rudimentary horn lacks and endometrium.
Class III (didelphys uterus): This anomaly results
from complete nonunion of both Müllerian ducts the individual horns are fully
developed and almost normal in size. Two cervices are inevitably present. A
longitudinal or transverse vaginal septum may be noted as well. Didelphys uteri
have the highest association with transverse vaginal septa but septa also may
be observed in other anomalies. Consider metroplasty; however, since each horn
is almost a fully developed uterus, patients have been known to carry pregnancies
to full term treatment is different according to the presentation.
1. Uterine didelphys with obstructed unilateral vagina full excision on marsuplization
of the vaginal septum. Also laparoscopy preferred to be done intra operatively
to treat associated endometriosis or adhesions if present
2. uterus didelphys non obstructed these patients are not candidates for surgical
unification. Fortunately they have minimum associated problems &the fertility
is good.
Class IV (bicornuate uterus): A bicornuate uterus
results from partial nonunion of the Müllerian ducts the central myometrium
may extend to the level of the internal cervical os (bicornuate unicollis) or
external cervical os (bicornuate bicollis). The latter is distinguished from
didelphys uterus because it demonstrates some degree of fusion between the two
horns, while in classic didelphys uterus, the two horns and cervices are separated
completely. In addition, the horns of the bicornuate uteri are not fully developed;
typically, they are smaller than those of didelphys uteri. Some patients are
surgical candidates for metroplasty surgical treatment
Although numbers of metroplasty procedures are available the strassmann procedure
is the surgical treatment of choice for unifying the bicornuate and didelphys
uteri this is simply explained by fusing the fundi of the uteri if 2 cervices
are present their unification is not recommended
Hysteroscopy resection is contraindicated in this setting because it can result
in uterine perforation. Class V (septate uterus): A septate uterus results
from failure of resorption of the septum between the two uterine horns. The
septum can be partial or complete, in which case it extends to the internal
cervical os histologically, and the septum may be composed of myometrium or
fibrous tissue. The uterine fundus is typically convex but may be flat or slightly
concave (<1-cm fundal cleft). Women with septate uterus have the highest incidence
of reproductive complications. Differentiation between a septate and a bicornuate
uterus is important because septate uteri are treated using transvaginal hysteroscopic
resection of the septum, while if surgery is possible and/or indicated for the
bicornuate uterus, an abdominal approach is required to perform metroplasty.
Surgical treatment
The treatment of choice is transcervical lyses of uterine septum combined with
concurrent laparoscopy to reduce the risk of uterine perforation. During septal
incision
The operation done by hysteroscopy which is introduced through the cervix and
cutting of the septum done & preferably to put IUCD for at least 2 months to
prevent intrauterine adhesions formation others found this is unnecessary and
may provoke local inflammation with subsequent synechiae
Class VI (arcuate uterus): An arcuate uterus
has a single uterine cavity with a convex or flat uterine fundus, the endometrial
cavity, which demonstrates a small fundal cleft or impression (>1.5 cm). The
outer contour of the uterus is convex or flat This form is often considered
a normal variant since it is not significantly associated with the increased
risks of pregnancy loss and the other complications found in other subtypes.
This abnormality doesn't need any interference because researches found there
is no difference in reproductive outcome compared with normal
Class VII (diethylstilbestrol-related anomaly):
Several million women were treated with diethylstilbestrol (DES; an estrogen
analog prescribed to prevent miscarriage) from 1945-1971. The drug was withdrawn
once its teratogenic effects on the reproductive tracts of male and female fetuses
were understood. The uterine anomaly is seen in the female offspring of as many
as 15% of women exposed to DES during pregnancy. Female fetuses who are affected
have a variety of abnormal findings that include uterine hypoplasia and a T-shaped
uterine cavity. Patients also may have abnormal transverse ridges, hoods, stenoses
of the cervix, and adenosis of the vagina with increased risk of vaginal clear
cell carcinoma. Imaging findings are path gnomonic for this anomaly).
Conclusion
Not all the types of uterine abnormalities can affect fertility & also not all
the abnormalities correctable surgically Uterine Abnormalities (Müllerian duct
anomalies) are an uncommon but often treatable cause of infertility. Patients
with Müllerian duct anomalies are known to have a higher incidence of infertility,
repeated first trimester spontaneous abortions, fetal intrauterine growth retardation,
fetal malposition, preterm labor, and retained placenta. The role of imaging
is to help detect, diagnose, and distinguish surgically correctable forms of
Müllerian duct anomalies from inoperable forms. In some correctable lesions,
the surgical approach is altered based on imaging findings
