Good

1. CONGENITAL DEFECTS OF
FEMALE REPRODUCTIVE
ORGAN

2. Development
 Organogenesis begins in the 3rd embryonic
week & is essentially complete in the 10th
week(5-12th week in terms of clinical
pregnancy)

3. Development
 The early embryo is bipotential, with the
ability to develop male or female internal
and external genitalia.
 Male differentiation requires the active
secretion of testicular androgen,
testosterone, and a nonsteroid, mullerian
inhibitory factor (MIF)

4. Development
 In a normal female fetus, the absence of
testicular androgens and MIF results in
degeneration of the wolffian duct and in
development of mullerian structures.

5. Development
 There is a close relation between the development
of the genital and urinary systems
 Association between the mullerian and
mesonephric ducts has clinical relevance 
damage is often associated with anomalies that
involve the uterine horn, kidney and ureter
 In the male embryo, the mesonephric or wolffian
duct becomes the definitive internal duct system
(i.e., vas deferens, epididymis, seminal vesicles).

7. Development
 In the female embryo, the wolffian duct
degenerates, but some small nonfunctional
cystic remnants may persist.
 Paramesonephric or mullerian duct - forms
the internal female genital tract (i.e., upper
vagina, cervix, uterus, fallopian tubes)
 It develops later than the wolffian duct
(beginning in week 6)

9. DEVELOPMENTAL REPRODUCTIVE
TRACT ABNORMALITIES
• Embryogenesis of the reproductive tract
• Vulvar abnormalities
• Vaginal abnormalities
• Cervical abnormalities
• Uterine malformations
• DES-induced reproductive abnormalities

10. Uterine Malformations

11. Table I. Classification of Müllerian duct anomalies
Class I: Agenesis or segmental hypoplasia
•A
•B
•C
•D
•E
•Vaginal
•Cervical
•Fundal
•Tubal
•Combination of
anomalies
Class II: Unicornuate uterus
•A1
•A2
•B
•Rudimentary horn with
endometrium :
•Communicating
with the main
cavity
•Non-
communicating
with the main
cavity
•Rudimentary horn
without endometrium
•Absence of
rudimentary horn
Class III: Didelphys uterus (two horns, two cervices)
Class IV: Bicornuate uterus :
•IV A: bicollis
•IV B: unicollis
•IV C: arcuatus
Class V: Septate uterus :
•V A: complete
•V B: partial
Class VI: DES-related anomalies

14. MÜLLERIAN DUCT ANOMALIES
(AFS classification system)
Class I (hypoplasia/agenesis):
• Mullerian agenesis - The most common abnormality is
complete failure of mullerian duct development, which
results in complete absence of the vagina, cervix, uterus,
and fallopian tubes
• The most common form is the Mayer-Rokitansky-
Kuster-Hauser syndrome  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.

15. Class II (unicornuate uterus):
• the result of complete, or almost complete, arrest of
development of 1 müllerian duct
• it is a single-horned uterus with its corresponding
fallopian tube and round ligament

17. Class III (didelphys uterus):
• This anomaly results from complete nonfusion of
both müllerian ducts
• The individual horns are fully developed and almost
normal in size.
• A longitudinal or transverse vaginal septum may be
noted as well.
• Didelphys uteri have the highest association with
transverse vaginal septa but septa also may be observed
in other anomalies

18. Class IV (bicornuate uterus):
• results from partial nonfusion of the müllerian
ducts
• there is a single vagina and cervix but a variable
lack of fusion of the upper uterine cavity
• The central myometrium may extend to the level of
the internal cervical os (bicornuate unicollis) or
external cervical os (bicornuate bicollis).

19. Hysterosalpingo
gram radiograph
of a bicornuate
uterus

20. Class V (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

21. Hysterosalping
ogram
radiograph of a
septate uterus

22. • US may demonstrate a convex or flattened fundal
contour. The intercornual distance usually is normal or
decreased (<4 cm), and each uterine cavity usually is
small.
• The septum may be composed of muscle or fibrous
tissue and is not a reliable means of distinguishing
septate from bicornuate uteri. A more reliable means to
differentiate the two involves examining the fundal
contour (see Class IV).

23. Class VI (arcuate uterus):
• An arcuate uterus has a single uterine cavity with a
convex or flat uterine fundus, the endometrial cavity,
which demonstrates a small fundal cleft or
impression (>1.5 cm). The outer contour of the
uterus is convex or flat.
• This form is often considered a normal variant
since it is not significantly associated with the
increased risks of pregnancy loss and the other
complications found in other subtypes

24. Class VII (DES related):
• transverse septa
• cervicovaginal abnormalities are more likely to
have uterine abnormalities
• smaller uterine cavities, shorten upper uterine
segment, irregular cavities
• abnormalities of the oviduct

25. VULVAR ABNORMALITIES
• Complete atresia
• includes of atresia of the lower third of the
vagina
• Labial fusion
• most commonly due to congenital adrenal
hyperplasia

26. VAGINAL ABNORMALITIES
1. Atresia
- Complete: about 1/3 of women with vaginal
atresia have associated with urological
abnormalities
- Vaginal agenesis: usually due to Rokitansky-
Küster-Hauser syndrome
- Incomplete: faulty of development or the result
of scarring from injury or inflammation

27. VAGINAL ABNORMALITIES
2. Double vagina
• Difficult to distinguish the double from the
completely septate agina
3. Longitudinally septate vagina
• Commonly it forms when the distal ends of
the mullerian ducts fail to profuse properly

28. 4. Transversely septate vagina
•When it is located in the upper vagina, it is likely
to be patent, whereas those located in the lower
part of the vagina are more often complex
• A complete septum results in signs and
symptoms similar to those of an imperforate
hymen.
VAGINAL ABNORMALITIES

29. Treatment
- complete septum: incised to allow
drainage
- surgical correction of vaginal narrowing
should be performed only when the patient
is contemplating initiation of sexual
activity
VAGINAL ABNORMALITIES

30. CERVICAL ABNORMALITIES
1. Atresia
• The entire cervix may fail to develop
• This may be combined with incomplete
development of the upper vagina or lower
uterus
2. Double cervix
• Each distinct cervix results from separate
müllerian duct maturation