GYN: chapter 3

Uterus Unicornuate: AKA: Uterus Unicornis Unicollis:

* 1 of everything - Unilateral arrested development. - Frequently associated with a rudimentary horn on the opposite side.

Uterus Bicornuate ("heart shaped"): AKA: Uterus Bicornis Unicollis:

* 1 vag, 1 cx, 2 ut horns. *If the fundus dip is greater than >1cm. - Partial fusion failure Treatment: - Surg. not usually required

Uterus Duplex Bicornis: AKA: Uterus Bicornis Bicollis:

* 1 vag, 2 cx, 2 ut horns

Double Uterus / Didelphys and Double Vagina:

* 2 of everything - complete mid line failure of mullein duct fusion. - no communication btwn ends.

Subseptate Uterus: Complete Septate Uterus: - Degree of failure to reabsorb/ Apoptosis. - Fusion occurred Treatment: - Hysteroscopic resection of the septum. (decision based on poor reproductive outcomes)

*If the fundus dip is less than <1cm - Fertility with the septate ut is not typically compromised. -BUT poorest reproductive outcomes of all the MULLERIAN DUCT anomalies.

Complete Septate Uterus:

*If the fundus dip is less than <1cm - Fusion occurred - Degree of failure to reabsorb/ Apoptosis.

Arrested Development of the Mullerian Ducts:

- Can be bilateral or unilateral. - Rare, produces Agenesis or Hypoplasia of the Vagina and/or Uterus.

Klippel-Feil Syndrome:

- Characterized by congenital fusion of the cervical spine, a short neck, a low posterior hairline, and a limited range of cervical spine motion; associated with MRKH.

Paramesonephric / Mullerian Ducts:

- Paired ducts that become the oviducts,uterus, cervix, and upper vagina.

Arrested Development of the Mullerian Ducts - CLINICAL SIGNS:

- Present at puberty. - Primary amenorrhea with severe cyclic pelvic pain. Physical Exam: - Likely obstructed UT, resulting in hematometra. - If secondary sex characteristics are present, indicating normal ovarian function.

Hematometra: Hydrometra: Metra = Uterine Cavity

- Retention of blood in the uterine cavity. - Accumulation of watery fluid in the uterine cavity.

Hymen:

- Ringlike area of tissue that represents the opening to the vagina.

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome:

- The most common manifestation of complete agenesis of the vagina and uterus.

Wunderlich-Herlyn-Werner Syndrome:

- Uterus didelphys with obstructed unilateral vagina and associated ipsilateral renal and ureter agenesis.

Classification System of Mullerian Duct Anomalies:

1.) HYPOPLASIA or AGENESIS of: a.) Vagina b.) Cervix c.) Tubal d.) Combined 2.) UNICORNUATE: A1a.) Communicating A1b.) Noncommunicating A2.) No Cavity B.) No Horn 3.) UTERUS DIDELPHYS: 4.) BICORNUATE: a.) Complete b.) Partial c.) Arcuate 5.) SEPTATE UTERUS: a.) Complete b.) Partial 6.) DES (diethylstilbestrol) and drug related anomalies or Arcuate:

DES or Diethylstilbestrol:

A drug given to mothers to prevent early miscarriage from 1950-1970. Daughters have: - T shaped uterus - Infertility - Miscarriage - Vaginal/ Labia cancers (rare) Pregnancy: Increased risk of premature labour and perinantal mortality.

Ostium (pl.ostia)

A small opening, especially one of entrance into a hollow organ or canal.

Hematometrocolpos

Accumulation of menstrual blood in the uterus and vagina caused by either an imperforate hymen or other obstruction.

Hematocolpos -colpos = vagina

Accumulation of menstrual blood in the vagina resulting from a lower vaginal obstruction or imperforate hymen.

Agenesis

Arrested devoplement of the paramesonephric ducts produces: Complete arrestment of the vagina and uterus. Most common manifestation is MRKH

Hypoplasia

Arrested devoplement of the paramesonephric ducts produces: partial arrestment; is more rarely encountered and is characterized by a normal uterus and a small vaginal pouch.

Uterus Bicornuate ("heart shaped") IMAGE:

Bicornuate uterus: Coronal reconstructed view of a bicornuate uterus, showing that the endometrial cavity is divided into two horns and the surface of the uterus is also divided or indented in its superior portion, making this different from a septate uterus.

Klippel-Feil Syndrome*:

Characterized by congenital fusion of the cervical spine, a short neck, a low posterior hairline, and a limited range of cervical spine motion; associated with Mayer-Rokaitansky-Kuster-Hauser syndrome.

VAGINAL SEPTUM:

In addition to obstruction from vaginal agenesis, defects of vertical vagina fusion can result in the formation of a transverse vaginal septum, which can also cause obstruction and produce hematocolpos in patients having a uterus with functional endometrial tissue.

Arrested Development of the Mullerian Ducts - PREGNANCY:

Incompatible with uterine a genesis. - Little Possibility, depending on the degree of hypoplasia.

Double Uterus / Didelphys and Double Vagina IMAGE:

Left and right uterus, hematocolpos and right ovary transverse

Apoptosis

Mechanism by which the uterine septum regresses.

Arrested Development of the Mullerian Ducts - TREATMENT:

Nonsurgical: - Graduated Dilators to progressively widen an opening. Surgical: - Vaginoplasty

Mullerian / Paramesonephric Ducts

Paired ducts that become: - Oviducts - Uterus - Cervix - Upper Vagina

Sinovaginal Bulb:

Part of the vaginal plate of the urogenital sinus, which forms the lower 20% of the vagina.

Hysterosalpingography:

Radiographic imaging of the uterus and fallopian after injection of radiopaque material.

Metroplasty:

Reconstructive surgery on the uterus used primarily to correct a septate uterus.

Vaginal Septum

Results from defects of vertical vagina fusion; which can also cause obstruction and produce hematocolpos in patients having a uterus with functional endometrial tissue. - most freq. location is at the junction of the upper and middle third of the vaginal canal. - treatment: twofold surgical therapy - successful pregnancy

Rudimentary horn

can have a uterine cavity with functional endometrium, and in some cases, there may be communication with the endometrium of the main horn.

Vaginoplasty

is used to create a vagina

uterus bicornis unicollis

one vagina, one cervix, and two uterine horns.

uterus bicornis bicollis

one vagina, two cervices, and twp uterine horns

absent vagina

sonographic appearance: absence of normal vaginal echoes

gartner duct cyst

sonographic appearance: anechoic small mass in anterolateral vagina

Hydrocolpos or hematocolpos

sonographic appearance: anechoic to hypoechoic, pear shaped mass with no normal vaginal echoes located adjacent and posterior to the bladder

double vagina

sonographic appearance: either no significant change in appearance of normal vagina or enlarged vagina; endovaginal scanning may demonstrate second vagina

intact hymen

sonographic appearance: in postmenarchal symptomatic patient, possible finding of hematocolpos or hematometrocolpos

vaginal septa

sonographic appearance: normal vaginal echoes

duplicated uterus with unilateral imperforate vagina

sonographic appearance: vagina appears normal; lateral to normal uterus, ananechoic or echo-filled mass might be seen, kidney ipsilateral to mass is usually absent

Uterus unicornis

this anomaly occurs when there is complete or incomplete failure of one mullerian duct to elongate while the other duct develops normally -associated with rudimentary horn on the opposite side -treatment: resection; poor preg. outcomes

Agenesis / Hypoplasia of the Uterus and Vagina:

~ 5-10% of mullerian duct anomalies are attributed to arrested development of the paramesonephric ducts; this produces either hypoplasia (partial arrestment) or agenesis of the vagina and uterus (complete arrestment)