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


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


  • Types of Cervices



    1. Single
    2. Septate ; single muscular ring partitioned by a septum
    3. Double ; two distinct cervices

  • Types of Vaginas



    1. Single
    2. Longitudinally septated ;
    3. Double ; double introitus
    4. 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



    1. Edema


      • During labor --> Venous thromboses & hematomas can cause



    2. Inflammatory Lesions




      • Extensive perineal inflammation & scarring form hidradenitis supprative, lymphogranuloma venereum, Crohn disease. --> may create difficulty with vaginal delivery, episiotomy repair



    3. Bartholin Abscess




      • Drainage & suturing : bleeding (+)
      • Broad - spectrum antibiotics



    4. Bartholin Cysts




      • Asymptomatic cyst ⇒ Treatment after delivery
      • If dystocia (+) ⇒ needle aspiration



    5. 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



    6. 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




    1. Partially Atresia



      • By infection or trauma
      • Partial atresia --> can be overcomed during labor



    2. 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



    3. 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



    1. 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




    1. 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



    2. 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



    3. 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



    4. 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



    5. 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



    6. Enterocele




      • It symptomatic --> replacement & recumbent position
      • Surgical treatment of enterocele --> after delivery



    7. 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





    1. 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.



    2. 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.