OB Gyn C4 Ch3 - AL: Congenital Anomalies of Female Genital System

How common are congenital reproductive anomalies?

0.1-0.5%

Most anomalies of the genital tract are a result of (3)

1. Total or partial atresia of the Müllerian ducts 2. Failure of Müllerian ducts to fuse 3. Failure of the uterovaginal septum to disappear

How often do urinary tract anomalies and genital tract anomalies occur simultaneously?

50%

Arcuate uterus is

A normal uterine variant characterized by a small dip in the fundus projecting into the endometrium. It is considered a nearly resolved septation of the uterus that causes no complications and is thereby a normal variant not an anomaly.

What is the most common Müllerian duct anomaly related to partial fusion of the Müllerian ducts?

Bicornuate uterus

Total atresia / complete agenesis of the Müllerian ducts results in

Complete agenesis of uterus (including cervix), vagina and Fallopian tubes.

Septate uterus is

Complete failure of median septum to disappear. Result is duplication of uterine cavity but no uterine horns (normal shaped fundus).

Hypoplastic uterus is

Congenital hypoplasia is the uterus. Small uterus.

Uterus dudelphys is aka

Duplication of uterus (including cervix) and vagina

Müllerian duct anomalies result from

Failure of normal development (at varying stages) of the Müllerian ducts.

What is the uterine septum composed of?

Fibromuscular tissue (extension of myometrium)

What are the shortcomings of 2D ultrasound for evaluating genital anomalies?

Genital Anomalies maybe mistaken for adnexal mass, fibroids, twin gestation, etc.

Uterine anomalies are associated with

Infertility or premature delivery due to smaller sizes and less ability to accommodate full term pregnancy.

What is the best modality for evaluating genital system anomalies?

MRI. If using ultrasound 3D is ideal.

What is the ultrasound appearance of uterus didelphys?

Most apparent on TRV plane, looks like duplicated cervix, 2 bodies, 2 endometrial cavities. No communication between sides.

Uterus (including cervix), Fallopian tubes, and vagina develop from the

Müllerian ducts

Uterus didelphys has

No communication between the two sides. Each side has one Fallopian tube, one uterine body, one cervix and one vagina.

Are ovaries usually involved in anomalies of the genital tract?

No, ovaries are not originated from the Müllerian ducts so they are typically unaffected.

Müllerian ducts are aka

Paramesonephric ducts

Partial atresia of Müllerian ducts results in

Partial agenesis of the uterus. The most common partial agenesis is the unicornate uterus.

Subseptate uterus is

Partial failure of median septum to disappear. Result is partial duplication of uterine cavity but no uterine horns (normal shaped fundus).

Failure of uterovaginal septum to resolve / disappear causes

Septate or subseptate uterus

What is the ultrasound appearance of a unicornate uterus?

Small uterine size, lateral uterine position. Fundus is small and looks more like the isthmus than a normal fundus (i.e. Narrow throughout)

What is a unicornate uterus?

The uterus is formed by only one Müllerian duct, while the other duct does not develop. It usually presents on the right lateral portion of the pelvis.

Bicollis bicornuate uterus has

Two uterine horns, a single vagina, 2 cervixes, variable fusion of uterine cavity. A heart shaped appearance.

Unicollis Bicornuate uterus has

Two uterine horns, a single vagina, one cervix, variable fusion of uterine cavity. A heart shaped appearance.

What are the 2 variations on uterus bicornis?

Unicollis (unicervical / one cervix) and bicollis (duplex bicornis / bicervical / two cervixes)

If you identify anomalies in the genital system what other system should be evaluated?

Urinary system

Bicornuate uterus is aka

Uterus bicornis, heart shaped uterus

Complete failure of Müllerian ducts to fuse leads to

Uterus didelphys

What is the ultrasound appearance of (sub)septate uterus?

Wide separation within the endometrial cavity created by septum which has the same echo texture as myometrium. May look like two completely separated endometrial cavities especially in TRV.