emberiology

Abnormalities of female genital tractby Dr. Dalya M. Abdulrahman

An absent or incomplete migration of the paramesonephric duct in the direction of the UGS is responsible for an atresia and/or complete or incomplete aplasia of the uterus, which is usually associated with renal abnormalities. This syndrome is called the Mayer Rokitansky Kuster Hauser syndrome.

Unilateral atresia, leading to a uterus unicornis unicollis

Uterus didelphys bicollis

Uterus bicornis bicollis

Most frequent
Uterus bicornis unicollis

Uterus septus

Uterus subseptus unicollis

Uterus subseptus bicollis

When no vaginal plate develops, this leads to a vaginal aplasia that, though, only very rarely occurs in isolation. Due to their partly common origin uterine abnormalities are mostly associated with those of the vagina.

Absent perforation of the hymenimperforated hymen

Missing hymen perforations are rare. During organogenesis a layer of endodermal tissue divides the vagina from the urogenital sinus (future vaginal vestibule). This tissue layer degenerates during the 5th month and leaves only the hymen behind. If this degeneration fails to occur, the hymen perforation does not occur and mucus from the cervical glands, stimulated by the (maternal) estrogen, collects above the hymen. This can lead to a hydrometrocolpos. Sometimes this abnormality manifests itself only in adolescence by a painful amenorrhoea with a hematometrocolpos and retention of urine. It occur in 0.1_0.5%in healthy fertile population

Clinical presentation of congenital anomalies 

It differ in each patient according to the type of abnormality but nearly the most common presentations are: - A symptomatic  -Infertility - Pelvic pain -Primary dysmenorrhea - Dyspareunia - Recurrent abortion -Preterm labor -Intra uterine growth retardation -Abnormal lie and presentation of the fetus - Rarely ectopic pregnancy -Primary amenorrhea or cryptomenorrhea in imperforated hymen & haematocolpus, retention of urine.

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% & false negative results of 28% it also can not assess the outer fundal contour making it impossible to differentiate between septate &bicornuate uterus.

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.


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. some times associated with 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 short 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 of vaginal agenesis

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 treatment2. 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 .

Vaginal dilator

Class II (unicornuate uterus)

Class II (unicornuate uterus)

Surgical treatmentWomen 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 an endometrium.

Class III (didelphys uterus)

Class IV (bicornuate uterus)

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, the septum may be composed of myometrium or fibrous tissue.

Class V (septate uterus)

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.

Intrauterine septum as seen by hysteroscopy

Vaginal septum

Thank you