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- 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
- 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