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Submitted by Amer Hussien, M.D & Hesham Al-Inany M.D

Congenital anomalies of the uterus

Approximately 10% of infants are born with some abnormality of the genitourinary system.


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Embryology of female genital system
Gametogenesis: a basic review


Obstetric and gynecological significance

Segmental Mullerian agenesis or hypoplasia (Class I)

-  Vaginal hypoplasia or agenesis renders pregnancy virtually impossible , and even in those rare cases in which a uterus is reattached surgically to a neovagina , successful pregnancy is extremely rare .

- The various types of vaginal septa can be dilated , displaced or surgically divided

- The septate uterus functions remarkably well , but during labor there is danger of rupture and hemorrhage.

Disorders of lateral fusion (Buttram and Gibbons classes II through V)

A) Obstetrical : Obstetrical difficulties may arise in this group due to failure of the uterus to dilate and hypertrophy appropriately to accommodate the growing fetus

1. Abortion

2. Ectopic pregnancy and rudimentary horn pregnancy

3. Preterm labor

4. Uterine dysfunction , obstructed labor or even uterine rupture

5 Placenta accreta

6. Fetal :

- Intrauterine growth retardation                   

- Abnormal fetal lie

B) Gynecological :

1. Menstrual Difficulties : Dysmenorrhea and menorrhagia

2. Infertility                              

3. Endometriosis

Reproductive performance of women with unicornuate uterus

- The incidence of unicornuate uterus in a series of uterine anomalies was about 14%. This was likely an underestimate because the major diagnostic method used was HSG , which can not identify noncommunicating uterine horn . 90% of of unicornuate uteri with rudimentary horn there were no communication between the horns .

- Gynecological significance: increased incidence of infertility, endometriosis (due to retrograde menstruation)  and dysmenorrhea .

- Obstetrical significance : Pregnancy outcome is poor .

1. The increased incidence of abortion is due to smaller uterine size and possible implantation of the zygote 9in a communicating uterine horn .

2. Preterm labor, IUGR, breech presentation, uterine dysfunction and increased incidence of CS are due to smaller hemiuterine size . It has the poorest fetal survival (40%)

3. Tubal pregnancies and pregnancies in a rudimentary horn . Pregnancy may occur in a communicating rudimentary horn or a noncommunicating horn when trans peritoneal migration of gametes . In 70% of pregnancies with a rudimentary horn , uterine rupture prior to 20 weeks gestation was reported . Intraperitoneal hemorrhage in such cases may be life-threatening , but in rare cases fetal survival have been reported .

Reproductive performance of women with uterine didelphys

- Uterine didelphys is distinguished from bicornuate and septate uterus by presence of complete reduplication of cervices and hemiuterine cavities . In one series all had longitudinal vaginal septa as well

- Obstetrical significance :  similar to to those seen with unicornuate uterus except for ectopic and rudimentary horn pregnancies . Multiple gestation is unusual but not rare and also there may be a delayed interval between fetal deliveries from each hemiuterus .

Reproductive performance of women with bicornuate and septate uteri

- There is marked increase in the abortion rate . The exception is for arcuate uterus , which is merely a slight deviation from normal . Pregnancy losses in the first 20 weeks of pregnancy were observed by Buttram and Gibbons (1979) to be 70% for for bicornuate and 88% for septate uterus . It has been reported that the chance for a liveborn child increases with each pregnancy loss

- The high pregnancy wastage is duo to partial or complete implantation on the largely avascular septum , poor blood supply, diminished intrauterine space ,associated cervical incompetence , ? luteal phase defect (TeLinde) due to local vascular distortion and alteration of estrogen and progesterone receptors in malformed uteri.

- Increased incidence of preterm labor , abnormal fetal lie , uterine dysfunction and CS .

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