1. Mullerian duct Abnormalities
N.Cem FICICIOGLU, MD, Ph.D,.AA, MBA
Prof in OB&GYN

2. the development of paramesonephric (müllerian) ducts
The differentiation of the gonad- Y chromosome, SRY gene
Gonad is not testis- the absence of antimüllerian hormone-the development of paramesonephric (müllerian) ducts
The formation, fusion and resorption of müllerian ducts
In the normal absence of the Y chromosome or the sexdetermining region of the Y chromosome (SRY gene) the somatic sex cords of the indifferent gonad do not produce TDF. In the absence of TDF, differentiation of the gonad into a testis and its subsequent production
of antimüllerian hormone and testosterone do not occur.The indifferent gonad becomes an ovary. Complete ovarian differentiation seems to require two X chromosomes (XO females exhibit ovarian dysgenesis, in which ovaries have precociously degenerated germ cells and no follicles and are present as gonadal “streaks”). The first recognition of a developing ovary around weeks 9–10 is based on the temporal absence of testicular-associated features (most prominently, the tunica albuginea) and on the presence of early meiotic activity in the germ cells

3. the development of paramesonephric (müllerian) ducts
Sertoli cell
Leydig cell
AMH
Testosteron
5 α reductase
DHT
The regression of
mullerian duct
The development of
mesonephric Ducts
The differantion of
external genitalia

4. the development of paramesonephric (müllerian) ducts
Both ducts, paramesonephric (müllerian) ducts and mesonephric ducts in fetus, up to eight weeks; bipotent period
One of them disappears in 12th week
The differantion occurs by AMH secreted by sertoli cell and testosterone secreted by leydig cell
In the absence of AMH, uterus, fallopian tubes and 1/3 upper vagina develop from müllerian ducts
The uterine tubes are derivatives of the cranial segments of the paramesonephric (müllerian) ducts, which differentiate
in the urogenital ridge between the sixth and ninth weeks

5. the development of paramesonephric (müllerian) ducts
Fetal yaşamın 6. haftasına kadar mezonefrik ve paramezonefrik olmak üzere iki kanal bulunur. Y kromozomu ve testis varlığında ve dolayısıyla mıf varlığında mezonefrik kanal ve erkek genitaller gelişir. Mıf yoksa paramezonefrik kanal gelişir ve yaklaşık 12. haftadan itibaremn artık tek kanal vardır
Sequential development of the reproductive tract explains the variation in observed anomalies.The paramesonephric ducts arise laterally to the mesonephric or Wolffian duct system. Without suppression from the SRY gene, development of the paramesonephric or Mu¨ llerian duct system is stimulated. These ducts migrate caudally and medially. Canalization of the Mu¨ llerian ducts, with a cranial lumen opening into the peritoneal cavity, results in the formation of the lateral fallopian tubes. Fusion of the caudal ducts in the midline, followed by regression of medial aspects, yields a single uterine cavity and cervix Normal development of the female reproductive tract involves a series of complex processes characterized by the differentiation, migration, fusion, and subsequent canalization of theMu¨ llerian system13. Uterine anomalies result when these processes are interrupted. Mu¨ llerian agenesis, characterized by a failure of the Mu¨ llerian ducts to develop, results in inability to conceive.
The m€ullerian ducts arise from the urogenital ridge of the embryo as an invagination of the celomic epithelium at about 5 to 6 weeks of embryonic life. The ducts grow downward immediately to fuse in the midline at about 9 weeks and then are united with the endodermal bulbs of the urogenital sinus to form the remainder of the vagina. This process is completed by 12 to 14 weeks of gestation, and reabsorption of the septum is completed by week 19 to 20 embryonic life. In the absence of m€ullerian inhibiting factor produced by the testes, the paramesonephric m€ullerian ducts progress to normal female development.

6. the development of paramesonephric (müllerian) ducts

7. Mullerian duct Abnormalities
Sporadic abnormalities
Multifactoriel,
Poligenic
Familial
Uriner system abnormalities accompany with vagina, cervix, fallopian tubes
The different stages of arrest in mullerian ducts differentiation are manifested in various clinical entities. Unilateral absence of a mullerian duct results in a unicornuate uterus, and medial absence of the ducts results in absence of the uterus. Lack of fusion at various stages results in a didelphic uterus with a double cervix and septate vagina. Failure of reabsorption of the medial septum in the uterus results in a septate uterus

8. Prevalance %5,5, in selected population %8, in infertile population
%13,3, pregnancy loss
%24,5, reccurent pregnancy loss

9. Mullerian duct Abnormalities
Congenital
Acquired
Myomas
Polyps
Intrauterine adhesions

10. Classification of Mullerian duct Abnormalities
The different stages of arrest in mullerian ducts differentiation are manifested in various clinical entities. Unilateral absence of a mullerian duct results in a unicornuate uterus, and medial absence of the ducts results in absence of the uterus. Lack of fusion at various stages results in a didelphic uterus with a double cervix and septate vagina. Failure of reabsorption of the medial septum in the uterus results in a septate uterus

11. AFS Classification of Mullerian duct Abnormalities

12. AFS Classification of Mullerian duct Abnormalities

13. uterus didelphys
Complete failure of fusion of the paired Mullerian ducts results in duplication of the uterine corpus and cervix called uterus didelphys. A longitudinal vaginal septum is present in most women with a didelphys uterus, and may facilitate the early diagnosis when identified on routine speculum examination

14. bicornuate uterus
A results from failure of the Mullerian ducts to completely fuse. The central myometrium may extend to the level of the internal cervical os (bicornuate unicollis) or external cervical os (bicornuate bicollis). The latter is distinguished from uterus didelphys because it demonstrate some degree of fusion between the two horns, whereas in classic uterus didelphys the two horns and cervices are separated completely.

15. Septate uterus
The partition between the ducts is thought to resorb in a caudal to cranial direction
Failure of complete resorption results in a fibromuscular septum that can be partial or complete—dividing the uterine cavity and cervical canal into two parts
The partition between the ducts is thought to resorb in a caudal to cranial direction. Failure of complete resorption results in a fibromuscular septum that can be partial or complete—dividing the uterine cavity and cervical canal into two parts. Recent reports have described anomalies that do not fit this model
such as women with a septate uterus with cervical duplication and a longitudinal vaginal septum and a woman with a double cervix and vagina with a normal uterus and blind cervical pouch. In 1967, Musset suggested that fusion occurs at the level of the uterine isthmus and simultaneously proceeds in both directions. According to this hypothesis, the dual cervix and vagina could be explained by the failed fusion of the Mullerian ducts in the caudal direction,
beginning at the uterine isthmus.

16. Septate uterus

17. Differantial diagnosis of bicornuat and septate uterus
Fundal eksternal konturun tubal ostiumlardan geçen düz çizginin altında ise veya bu mesafe <5 mm ise
BİKORNUAT UTERUS
Apeks bu çizginin 5 mm ve üzerinde ise
UTERİN SEPTUM

18. Unicornuat uterus
A unicornuate uterus represents a uterine malformation where the uterus is formed from one only of the paired Mullerian ducts while the other Mullerian duct does not develop or only in a rudimentary fashion. The sometimes called hemi-uterus has a single horn linked to the ipsilateral fallopian tubes that faces its ovary

21. diagnosis Physical examination Ultrasonography, 2D and 3D HSG MRI
Laparoscopy

22. Diagnosis of septate uterus

23. Clinical outcomes
Symptoms depend on the severity and type of the defect
Dysmenorrhea, chronic pelvic pain, dyspareunia, hematocolpos, hematometra in adolescent
Treccurent pregnancy loss
Preterm labor
Abnormal fetal presentation
Infertility