Abnormalities of the Reproductive Tract

Chapter 28. Abnormalities of the Reproductive Tract

1st year resident S.B. Cho M.D.

I. DEVELOPMENTAL REPRODUCTIVE TRACT ABNORMAITIES

Most of defects : sporadically

? ? Even minor defects : increased incidence in significant fetal & maternal hazards

A. Vulvar Abnormalities

1. Atresia

Complete atresia of vulva : precludes conception
Incomplete atresia of vulva due to adhesions & scars following injury or infection

B. Vaginal, cervical, uterine Abnormalities

Brief embryology of female genital tract

3 ~ 5wks ; metanephric duct + cloaca.
4 ~ 5wks ; two ureteric buds develops distally from the mesonephric ducts, begin to grow cephalad toward the mesonephros
Mullerian (paramesonephric) ducts : form bilaterally between the developing gonads 2 mesonephros.
Mullerian duct --> extends downward & laterally to the mesonephric ducts --> finally turn medially to meet & fuse together in the midline
Fused mullerian ducts --> descends to the urogenital sinus to join the mullerian tubercle.
Mullerian duct & mesonephric ducts --> damage to either duct system will most often be associated with damage to both - uterine horn, kidney & ureter
10th week : union of two mullerian ducts --> uterus formation, fusion begins in the midline --> caudally & cephalad.
Uterine cavity formed as the septum dissolved slowly
The vagina --> forms between the urogenital sinus & mullerian tubercle by a dissolution of the cell cord between the two structures

1. Genesis and Classification of Mullerian Abnormalities

Classification of embryological defects

Defective canalization of the vagina --> transverse vaginal septum
Unilateral maturation of mullerian duct, with incomplete or absent development of the opposite duct & upper urinary tract defects
Absent or faulty midline fusion of the mullerian ducts

Types of Cervices

Single
Septate ; single muscular ring partitioned by a septum
Double ; two distinct cervices

Types of Vaginas

Single
Longitudinally septated ;
Double ; double introitus
Transversely septate

2. Diagnosis of Vaginal Septa

By pelvic examination

3. Diagnosis of Cervical & Uterine Malformations

Simple inspection, bimanual examination,

During surgery,

Ultrasound,

Hysteroscopy & hysterography,

MRI

4. Urologic Evaluations

Up to one thirds of women with mullerian defects --> have auditory defects

C. Obstetrical Significance of Vaginal Abnormalities

1. Septa & Strictures

Complete septum ; no problem, incomplete septum ; cause dystocia
Congenital annular stricture or band --> during labor, softening & dilatation occurs
Transverse septum ; slight pressure or cruciate incision

2. Atresia

Complete atresia --> bar to pregnancy
Incomplete atresia --> due to tissue softening, during pregnancy, obstructions are gradually overcome

D. Obstetrical Significant of Cervical Abnormalities

1. Atresia & Stenosis

Complete stenosis --> pregnancy (-)
Incomplete stenosis --> tissue softening during pregnancy --> dilation occurs

E. Obstetrical Significance of Uterine Hypoplasia & Agenesis

1. Buttram & Gibbons Class 1.

Vaginal Hypoplasia or agenesis --> pregnancy (-)
Septate cervix ⇒ possible danger of rupture & hemorrhage

2. Buttram & Gibbons Classes Ⅱ through Ⅴ

Associated with abortion, ectopic pregnancy, preterm delivery, fetal growth restriction, abnormal fetal lie, uterine dysfunction, uterine rupture.

3. Reproductive Performance of Women with Unicornuate Uterus (Buttram & Gibbors Class Ⅱ)

Abortion --> partially explained by smaller uterine size & possible implantation of the zygote in a communicating rudimentary horn
Preterm labor, fetal growth retardation breech presentation, dysfunctional labor increased C-sec
delivery ; can be explained by small size
Tubal pregnancies & pregnancies in the rudimentary horn --> uterine rupture prior to 20wks.

4. Reproductive Performance in Women with Uterine Didelphys (class Ⅲ)

Complete reduplication of cervices & hemiuterine cavities
Overall successful pregnancy outcome --> 68%
Same complication as class Ⅱ.

5. Reproductive Performance in Women with Bicornuate & Septate Uteri (class Ⅳ & Ⅴ)

Marked increased in abortions ; due to abundant muscular tissue in the septum
70% of bicornuate, 88% of septate uterus --> pregnancy loss occurs prior to 20wks
It pregnancy established --> preterm delivery, abnormal fetal lie, cesarean delivery rate increased

# Management of Uterine abnormalities

1. Cerclage

Uterine didelphys & unicornuate & bicornuate Uterus --> therapeutic & prophylactic cervical cerclage
Partial cervical atresia & cervical hypoplasia ; transabdominal cerclage
DES exposed Women with cervical hypoplasia --> transvaginal cerclage

2. Metroplasty

Bicornuate Uterus - septal resection & recombination of fundi
Septate uterus
- Hysteroscopic resection of the septum
- Postoperative intrauterine device insertion & hormonal therapy --> not necessary to prevent septal fusion
- Uterine didelphys - transabdominal metroplasty

F. Diethylstilbesteral - Induced Reproductive Tract Abnormalities

1. Structural Abnormalities

One forth to one half of women exposed to DES in utero --> identifiable structural variations in the cervix & vagina ; transverse septa, circumferential ridges
Two thirds of exposed women --> uterine cavity abnormalities (evident on hysterography)

; smaller uterine cavities, shortened upper segments, T-Shaped cavities
Half of women with uterine defects --> cervical detects cf) esp. hypoplastic cervix
Oviduct abnormalities - shortening, narrowing, absence of fimbriae

2. Reproductive Performance

Lower conception rates
Spontaneous abortions, ectopic pregnancies, preterm birth; increased

Ectopic Pregnancies
- Increased ; 7%, may be due to tubal abnormalities, decreased uterine size

Abortions & Preterm Labor : increased incidence due to cervical incompetence --> prophylactic cerclage
Infertility
- Poorly understood ; associated with cervical hypoplasia & atresia
- Successful pregnancies ⇒ by using zygote intrafallopian transfer technigues

3. Treatment

II. ACQUIRED REPRODUCTIVE TRACT ABNORMAITIES

A. Vulvar Abnormalities

Edema
- During labor --> Venous thromboses & hematomas can cause
Inflammatory Lesions
- Extensive perineal inflammation & scarring form hidradenitis supprative, lymphogranuloma venereum, Crohn disease. --> may create difficulty with vaginal delivery, episiotomy repair
Bartholin Abscess
- Drainage & suturing : bleeding (+)
- Broad - spectrum antibiotics
Bartholin Cysts
- Asymptomatic cyst ⇒ Treatment after delivery
- If dystocia (+) ⇒ needle aspiration
Urethral Diverticulae, Cysts, & Abscesses
- Due to trauma & infection of periurethral gland
- Abscess --> usually resolve spontaneously, asymptomatic cyst formation
- Diverticulae --> can cause proteinuria of obscure cause
- Treatment --> after delivery
Condyloma Accuminata
- Extensive lesion --> Vaginal delivery (-)
- Predelivery eradication to prevent dystocia, secondary infection, hemorrhage which may result in amnionitis, preterm labor, episiotomy dehiscence
- Vertical transmission & it's treatment; controversy

B. Vaginal Abnormalities

Partially Atresia
- By infection or trauma
- Partial atresia --> can be overcomed during labor
Gartner Duct Cyst
- May protrude into the vagina --> can be confused with a cystocele
- May or may not slip above presenting part --> if may not, aseptic aspiration is needed
Genital Tract Fistulas form Parturition
- Due to compression of fetal head & bony pelvis
- Vesicovaginal fistula, vesicouterine fistula, vesicocervical fistula
- With no infection ⇒ heal spontaneously

C. Cervical Abnormalities

Stenosis
- By extensive cauterization, difficult labor associated with infection & considerable tissue destruction
- Cryotherapy & laser therapy --> less likely to produce stenosis
- LLETZ --> not associated with pregnancy outcome

D. Uterine Displacement

Anteflexion
- Frequently observed in early pregnancy
- In late pregnancy, particularly when the abdominal wall is very lax --> anteflexion can occur
- Marked anteflexion --> associated with diastasis recti & a pendulous abdomen
- Cervical dilatation & engagement can be inhibited
- Abdominal binder
Retroflexion
- No pathologic state
- Incarcerated uterus : growing of retroflexed uterus remains incarcerated in the hollow of the sacrum
- Symptom ; abdominal discomfort, inability to void
- Bladder catheterization & kneechest position
Sacculation of the Uterus
- Persistent entrapment of the pregnant uterus in the pelvis by old inflammatory diseases or endometriosis --> anterior uterine sacculation
- Aggressive treatment of Asherman syndrome --> posterior uterine sacculation
- Elongated vaginal passing above the level of a fetal head deeply placed into the pelvis
- Extension of abdominal incision
Prolapse of the Pregnant Uterus
- In early pregnancy --> cervix may protract through vagina, bat resolves as pregnancy progresses
- No change of uterus position --> incarceration develop during the third or fourth months
- Treatment ; pessary, recumbent position
Cystocele & Rectocele
- Large cystocele --> UTI, large rectocele ⇒ constipation
- Both of lesions --> associated with blocking the normal descent of fetus.
- Cystocele --> often associated with stress incontinence ; worsened by pregnancy
Enterocele
- It symptomatic --> replacement & recumbent position
- Surgical treatment of enterocele --> after delivery
Torsion of Pregnant Uterus
- Rotation of pregnant uterus --> most often to right
- If torsion (+) --> sufficient to arrest circulation ; rare condition

E. Uterine Leiomyomas

Rice & colleagues (1989) : 6700 pregnancies ⇒ 1.4% complicated
Pedunculated subserosal myoma --> torsion with necrosis ⇒ detachment
Change of Myoma during pregnancy ; red, carneous degeneration ⇒ hemorrhagic infarction
Focal pain, with tenderness on palpitation & low-grade fever moderate leukocytosis
DDx : with appendicitis, placental abruption, ureteral stone, pyelonephritis

Effects of Pregnancy
- By estrogen, progesterone & other growth factors ⇒changes of myoma size during pregnancy
- Estrogen receptor of myoma ⇒its estrogen level increases too much, down regulation can occur
- Lev-Tott & co-Workevs (1987)
  - lst trimester ; myomas of all sizes ⇒ unchanged or increased (early response due to increased
    estrogen)
  - 2nd trimester ; small myomas (2~6cm) remained unchanged or increased larger myomas ⇒ become smaller
  - 3rd trimester ⇒ regardless of initial myoma size, usually remained uncharged or decreased in size.
Effects of Myoma Size, Location & Number on Pregnancy
- Rice & associates (1989)
  - Myomas greater than 5cm ; significantly, increased rates of preterm labor, placental abruption, pelvic pain, cesarean delivery
- Hasan & co-workers (1990)
  - No association with respect to myoma size except for on increased incidence of obstructed labor.
- Postpartum hemorrhage
  - Not increased incidence, but if occur ⇒ massive hemorrhage (+) & corrected by hysterectomy (Hasan & associates, 1990)
- Lower uterine segment myomas
  - Increased incidence of retained placental (Lev-Toaff & colleagues, 1987)
- Conclusions
  - Growth of myomas during pregnancy ⇒ unpredictable
  - Placental implantation over or in contact with a myoma ⇒ increases the likelihood of placental abruption, abortion, preterm labor, postpartum hemorrhage
  - Multiple myoma ⇒ associated with fetal malposition & preterm labor
  - Degeneration of myoma ⇒ associated with a characteristic sonographic patterns
  - The incidence of cesarean delivery ⇒ increased

1) Cervical Myomas

Myomas in the Cervix or in the lower uterine segment ⇒ obstructed labor, confusion with fetal head

2) Imaging of Myomas

By ultrasonography : can be confused with ovarian masses, molar pregnancies, ectopic pregnancies, missed abortions, bowel abnormalities
MRI : superior to US, especially in correctly identifying uterine myomas

3) Myomectomy during Pregnancy

Limited to pedunculated type
Dissection of Myoma during pregnancy or at the term of Delivery ⇒ contraindicated due to
bleeding
Typically, myomas will undergo remarkable involution after delivery ⇒ myomectomy

4) Myomectomy Before Pregnancy

After myomectomy ⇒ significant risk of uterine rupture during subsequent pregnancy
Rupture may occur early in gestation (Golan & associates 1990a)

F. Endometriosis

Most women ⇒ No complication
Rupture of endometrial cyst ⇒ DDx with pyelonephritis, acute appendicitis, tubal pregnancy,
Enlarging pelvic endometriosis ⇒ dystocia

G. Adenomyosis

Rarely associated with complication
If, complication (+) ⇒ uterine rupture, ectopic pregnancy, uterine atony, placenta previa

H. Ovarian Tumors

1. Benign Ovarian Tumors

Serious complication during pregnancy ⇒ torsion, infarction, obstruction to vaginal delivery
Most common type ; cystic tumor
Beischer & associates (1971) - 164 ovarian tumors diagnosed during pregnancy
- one forth ⇒ cystic teratoma
- one forth ⇒ mucinous cystadenoma
- 2.4% ⇒ malignant
The most frequent & serious complication of benign ovarian cyst during pregnancy ⇒ torsion
- Most common in 1st trimester may result in cystic rupture
Diagnosis of Ovarian tumor during pregnancy ⇒ difficult due to abdominal enlargement
Early in pregnancy ⇒ less than 6cm enlargement ; d/t corpus luteum formation
Thornton & Wells (1987)
- 5cm or less ; left alone
- 5~10cm ; it cystic ⇒ observation , it contains septae or nodules ⇒resection
- Over 10cm ; resection
Hess & colleagues (1988)
- Elective resection of any ovarian mass 6cm or large that persists after 16 weeks
Serial ultrasonography & color doppler sonography & MRI
Ovarian tumor markers ⇒ rarely helpful in doubtful cases
Laparotomy
- Malignancy suspected ⇒ surgery must be done
- Tumor is impacted in the pelvis ⇒ C-sec delivery & tumor resection

2. Carcinoma of the Ovary.