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Congenital malformations of the female genital tract. (Unicornaute uterus.…
Congenital malformations of the female genital tract.
Embryology
Mullerian and Wolffian ducts are the primary organs for the internal reproductive systems of females and males respectively and they co-exist in the undifferentiated embryo until differentiation of either ovaries or testes (before 8 weeks of gestation)
Mullerian ducts differentiate into Fallopian tubes, uterus, cervix and upper part of the vagina
Wolffian ducts degenerate
in the male testicular production of AMH- anti-Mullerian hormone and androgens leads to the development of the Wolffian ducts into vas deferens and seminal vesicles
The time of the rise- 8-10 weeks of gestation
The pathogenesis of this anomaly cannot be clearly defined
they come into being in the result:
Agenesia Mullerian ducts
Hypoplasia Mullerian ducts
Failure of fusion of each set of the Mullerian tracts
Failure of canalisation of the Mullerian tracts
1% of all women
Clinical symptoms:
Asymptomatic, Primary amenorrhoea, Cyclic pelvic pain, Infertility and sterility, Abdominal mass
diagnosis
Frequently made clinically
Transvaginal sonographic- (two-dimensional ultrasound is the initial choice of diagnostic modality, but three-dimensional ultrasound is more sensitive)
Hysterosalpingography
Intravenous pyelography
Magnetic resonance imaging MRI
Hysteroscopy
Diagnostic laparoscopy
classification system of Mullerian anomalies -American Fertility Society.
I- agenesia Mullerian ducts ( complete absence of the uterus)
II- unicornaute uterus
III- duplication of the uterus
IV- bicornis uterus
V- septate uterus
VI- malformations of the uterus in the result of the working diethylstilbestrol
Defect of female genital tracts is often associated with other anomalies:
Renal defects ( unilateral agenesis, ectopia of one or both kidneys, horseshoe kidney)- 40-60% of patients
Skeletal abnormalities ( asymmetric, fused or wedged vertebrae, scoliosis, Klippel-Feil anomaly)- about 20% of patients
Heart defects
gynatresia
Imperforate hymen
Vaginal atresia and stenosis
Cervix atresia, uterus atresia (more often acquired)
Uterus atresia- Asherman’s syndrome
The result of the trauma to the endometrium from surgical procedures, primarily curettage, myomectomy, removal of septum and any other intrauterine surgery
Oligomenorrhea, amenorrhea, infertility
ACUTE/CHRONIC PAIN PELVIC/ABDOMINAL MASS
DIAGNOSIS: US MRI
Septate uterus et vagina - more often longitudinal septum
Complete septum, Incomplete
symptoms: Asymptomatic, Spontaneous abortions, Premature births
Treatment: hysteroscopic resection of the septum
duplications
Symmetrical:
complete utero-vaginal duplication
bicornis uterus
cervical duplication
asymmetrical
unicornous uterus
noncommunicating rudimentary uterine horn
Unicornaute uterus.
Subgroups
unicornous uterus with communicating rudimentary horn
noncommunicating horn with cavity
noncommunicating horn without a cavity
unicornous uterus without a rudimentary horn
symptoms
Dysmenorrhea, Dyspareunia, Chronic pelvic pain
Complications
Haematometra, Endometriosis, Infertility, Ectopic pregnancy in noncommunicating rudimentary horn
Rudimentary uterine horn
pregnancy
High risk of uterine rupture
80-90% of these pregnancies culminate in rupture, typically between the 10th and 20th weeks of gestation
Treatment
Laparoscopic resection of the rudimentary horn with pregnancy and the ipsilateral fallopian tube to prevent a further ectopic tubal gestation
Systemic methotrexate or feticide with intracardiac potassium chloride administration – alternatives or adjuncts to surgery in early gestation
Mayer Rokitansky Kuster Hauser syndrome
Congenital aplasia of the uterus and the upper part ( two-third) of the vagina
Type I- isolated
type II- associated with other malformations ( the upper urinary tract, the skeleton, the otologic sphere, the heart
clinical- primary amenorrhea in women with normal development of secondary sexual characteristics and normal external genitalia
vagina is reduced to a vaginal dimple with variable depth
The ovaries are normal and functional as well as the endocrine status
Karyotype is 46,XX with no visible chromosome modification
Etiology
Sporadic anomaly
Genetic cause- autosomal dominant trait with incomplete penetrance and variable expressivity
Treatment- surgical et non-surgical methods of creating a neovagina
Non-surgical treatment- Franks method
Vaginal dilatation should be considered the first-line therapy
This method uses dilators of gradually increasing size