1. THE FEMALE REPRODUCTIVE SYSTEM
CHAPTER 37
ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Jing-Xin Ding
The Obstetrics and Gynecology Hospital of Fudan University

2. Normal Female Anatomy CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Normal Female Anatomy

3. Section 1 The development of the female reproductive system
Ovary ——yolk sac endoderm
Primordial germ cell→urogenital ridge→（lacking testis-determing factor,TDF） →ovary（8W）
The primordial germ cells migrate from the yolk sac through the mesentery of the
hindgut to the posterior body wall mesenchyme at about the tenth thoracic level, which
is the initial site of the future ovary.
controlled by the presence or absence of
testis-determining factor, encoded on the Y chromosome.
In the absence of testis-determining factor, the
medulla regresses, and the cortical sex cords break up into isolated cell clusters (the
primordial follicles).
The paramesonephric or müllerian ducts form lateral to the mesonephric ducts; they
grow caudally and then medially to fuse in the midline.
---See Berek & Novak’s Gynecology Chapter 5.

4. Genital tract ——Mullerian duct
In the absence of AMH, the mesonephric duct system degenerates, and the paramesonephric duct system subsequently develops.
Cephalic segment→Fallopian tubes
Middle segment→Uterus
The Sertoli cells secrete a glycoprotein known as anti-müllerian hormone (AMH),
which causes regression of the paramesonephric duct system in the male embryo.
The absence of a Y chromosome and the resultant absence of müllerian inhibiting substance lead to the development of the paramesonephric system, with the regression of the mesonephric system. The upper vagina, cervix, uterine corpus, and fallopian tubes are formed from the paramesonephric (müllerian) ducts.
The paramesonephric ducts first arise at 6 weeks lateral to the cranial pole of the mesonephric duct and expand caudally. By 9 to 10 weeks, they fuse in the midline at the urogenital septum to form the uterovaginal primordium. Later, dissolution of the septum between the fused paramesonephric ducts leads to the development of a single uterus and cervix.
Figure 5.11 The embryonic development of the female genital tract. The formation of the
uterus and vagina. A: The uterus and superior end of the vagina begin to form as the
paramesonephric ducts fuse together near their attachment to the posterior wall of the primitive
urogenital sinus. B, C: The ducts then zipper together in a superior direction between the third and
fifth months. As the paramesonephric ducts are pulled away from the posterior body wall, they
drag a fold of peritoneal membrane with them, forming the broad ligaments of the uterus. A–C:
The inferior end of the vagina forms from the sinovaginal bulbs on the posterior wall of the
primitive urogenital sinus.
The lower 2/3 vagina is developed from the urogenital sinus.

5. The development of the vagina
Inferior segment of the Mullerian tube→Upper segment of the vagina
The urogenital sinus → the lower 2/3 vagina
Figure 5.11 The embryonic development of the female genital tract. The formation of the
uterus and vagina. A: The uterus and superior end of the vagina begin to form as the
paramesonephric ducts fuse together near their attachment to the posterior wall of the primitive
urogenital sinus. B, C: The ducts then zipper together in a superior direction between the third and
fifth months. As the paramesonephric ducts are pulled away from the posterior body wall, they
drag a fold of peritoneal membrane with them, forming the broad ligaments of the uterus. A–C:
The inferior end of the vagina forms from the sinovaginal bulbs on the posterior wall of the
primitive urogenital sinus.

6. External Genitalia——urogenital Fold
Autonomic →Female
Androgen and its enzyme (5α–Reductase) and its receptor→Male
Figure 5.12 The comparative development of the female and male external genitalia. A:
In both sexes, the development follows a uniform pattern through the seventh week and
thereafter begins to differentiate.

7. Section 2 Genital tract abnormalities
Common Reason
Improper fusion of the paramesonephric ducts
Incomplete development of one paramesonephric duct
Failure of part of the paramesonephric duct on one or both sides to develop
Absent or incomplete canalization of the vaginal plate

8. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
I Imperforate hymen
-- the mildest form of the canalization abnormalities. It occurs at the site where the vaginal plate contacts the urogenital sinus.
--Blood accumulate in the vagina (hydrocolpos) or uterus (hydrometrocolpos) and result in a bulging hymen that is often bluish in color
See Berek & Novak’s Gynecology P1006

9. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
a history of vague abdominal pain with approximately monthly exacerbations
No menstruation
Clinical Manifestation

10. Diagnosis Physical Examination Auxiliary Examination
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Diagnosis
Physical Examination
A bulging hymen that is often bluish in color
A palpable cystic mass compressing rectum by anal examination.
Auxiliary Examination
B-mode ultrasonic examination： hydrocolpos or hydrometrocolpos

11. Treatment Paracentesis to confirm the diagnosis.
Making a cruciate incision to open the vaginal orifice, excising the redundant hymen, and suturing the hymen with absorbable materials.
Examining the cervix regularlly .
Treatment
--Placing a needle into the hematocolpos

12. II VAGINA CONGENITAL ABNORMALITIES
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
II VAGINA CONGENITAL ABNORMALITIES

13. Congenital absence of vagina
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Congenital absence of vagina
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS)

14. Clinical manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
The incidence is estimated to be 1 in live-born girls.
Primary amenorrhea
Difficulty in sexual activity
Most patients have primordial uterus, no periodic abdominalgia.
Clinical manifestation
The incidence is estimated to be 1 in live-born girls

15. Diagnosis Physical Examination
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Physical Examination
Normal secondary sexual characteristics
Generally, a complete form of vaginal agenesis is noted in 75% of patients with MRKHS, and approximately 25% have a short vaginal pouch.
B-mode ultrasound: the presence of rudimentary uterus without functional endometrium on the both side of the pelvis and intact bilateral adnexae.
Karyotype ：46，XX
Hormonal analyses: within the normal ranges
Diagnosis

16. Treatment Vaginal dilation treatment Surgical vaginoplasty
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Vaginal dilation treatment
Surgical vaginoplasty
Treatment
The therapeutic approach for the congenital absence of the vagina varied in different ways. Vaginal dilation treatment is a successful method of vaginal creation, and avoids the risks of surgery, however, the long-term agony and mental and emotional stress imposed on the patient is inevitable.

17. Vaginoplasty in patients with MRKH syndrome
Davydov technique
Intestinal vaginoplasty - Segments of sigmoid colon is employed for vaginal replacement.
Mclndoe-Reed vaginoplasty (Abbe-Mclndoe-Reed) technique
Acellular porcine small intestinal submucosa graft

18. Laparoscopic Davydov technique

19. Intestinal vaginoplasty - Segments of sigmoid colon is employed for vaginal replacement.
夹闭试验选择肠段血管
切取肠段18cm顺时针逆蠕动放置
自乙状结肠放置吻合器钉钻
自直肠放置管状吻合器

20. Vaginoplasty using acellular porcine small intestinal submucosa graft

21. Acellular matrix graft promote the rapid ingrowth of surrounding tissue and epithelialization of the neovagina is allowed to occur 21

22. Pictures under colposcopy (Schiller’s Iodine test)
1 week post surgery
Figure 2. (a) The appearance of neovagina under colposcopy 1 week post surgery. (b) Iodine test show there is no squamous epithelialization over SIS graft. (c) The appearance of neovagina under colposcopy 2 months post surgery. The neovagina had lots of new vasculars and granulation tissue and was easy to bleed during gynecologic examination. (d) Squamous epithelialization of the neovagina was noted by Schiller’s Iodine test.
2 month post surgery

23. The appearance of the neovagina 6 months post surgery

24. Atresia of Vagina ---lack the lower portion of the vagina
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Atresia of Vagina
---lack the lower portion of the vagina
---the urogenital sinus does not participate in the formation of the vagina.
It occurs because the urogenital sinus does not participate in the formation of the vagina.

25. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
The same symptom with imperforate hymen. cyclic lower abdominal pain, amenorrhea
Clinical Manifestation
a history of vague abdominal pain with approximately monthly exacerbations
No menstruation

26. Diagnosis Physical Examination Auxiliary Examination
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Diagnosis
Physical Examination
A palpable cystic mass compressing rectum by anal examination, but the location of the mass is higher than that in imperforate hymen.
No bluish bulging hymen
Auxiliary Examination
B-mode ultrasonic examination： hydrocolpos or hydrometrocolpos
MRI

27. Vaginal dilation with vaginal mould post surgery
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Early surgery
Vaginal dilation with vaginal mould post surgery
Treatment

28. Transverse Vaginal Septum
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Transverse Vaginal Septum

29. Failure of vertical fusion ( complete cavitation of the vaginal plate between the sinovaginal bulbs and uterovaginal canal).
More common in the upper portion, that is, at the junction between the sinovaginal plate and the caudal end of the fused müllerian ducts
Vertical fusion refers to complete cavitation of the vaginal plate between the sinovaginal bulbs and uterovaginal canal. Transverse vaginal septum may be caused by a failure of this process.
Transverse vaginal septum can develop at any level within the vagina but is more common in the upper portion, that is, at the junction between the sinovaginal plate and the caudal end of the fused müllerian ducts.
--See Williams Gynecology

30. The septum may be obstructive, with accumulation of mucus or menstrual blood, or may be non-obstructive, allowing for egress of mucus and blood.

31. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Clinical Manifestation
Obstructive transverse vaginal septum
-- usually present during adolescence with cyclic lower abdominal pain, amenorrhea, and gradual development of a central pelvic mass.
Nonobstructive transverse vaginal septum
-- complain of abnormal menstrual flow, pain with intercourse, difficulty in placing or removing tampons, or obstructed labor.

32. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
The diagnosis is suspected when an abdominal or pelvic mass is palpated or when a foreshortened vagina and inability to identify the cervix is encountered.
Diagnosis
The diagnosis is suspected when an abdominal or pelvic mass is palpated or when a foreshortened vagina and inability to identify the cervix is encountered.

33. Diagnosis is confirmed by either sonography or magnetic resonance (MR) imaging. Magnetic resonance imaging is most helpful prior to surgery to determine the thickness and depth of the transverse septum.
Diagnosis is confirmed by either sonography or magnetic resonance (MR) imaging. Magnetic resonance imaging is most helpful prior to surgery to determine the thickness and depth of the transverse septum.
In addition, MR imaging may identify if a cervix is present, thereby differentiating a high vaginal septum from cervical agenesis.
Figure: Magnetic resonance image of complete low transverse septum with obstruction. Marked hematocolpos is identified (arrows) in this 13-year-old female. The relatively low signal intensity on T2-weighted imaging is consistent with subacute blood. The uterus is seen above the hematocolpos.

34. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Treatment
Surgical repair is dependent upon septal thickness. Skin grafts may occasionally be necessary to cover a defect left by excision of very thick septa. Smaller septa may be approached by excision with an end-to-end anastomosis of the upper to the lower vagina.
Surgical repair is dependent upon septal thickness and skin grafts may occasionally be necessary to cover a defect left by excision of very thick septa. Smaller septa may be approached by excision with an end-to-end anastomosis of the upper to the lower vagina.

35. Longitudinal Vaginal Septum
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Longitudinal Vaginal Septum

36. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Results from defective lateral fusion and incomplete reabsorption of the paired müllerian ducts. These septa are generally seen with partial or complete duplication of the cervix and uterus.

37. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Complete longitudinal septum: No syptoms
In complete longitudinal septum:　　　difficulty with intercourse
Clinical Manifestation
黏膜壁上端近宫颈，完全纵隔下端达阴道口
　　　　　　　　　　不全纵隔未达阴道口

38. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
The nonobstructed form can be managed conservatively unless dyspareunia develops. Surgical treatment includes resection of the longitudinal septum.
Treatment

39. Oblique Vaginal Septum Syndrome
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Oblique Vaginal Septum Syndrome
Herlyn-Werner-Wunderlich syndrome (HWWS)
In addition, an obstructive variety of longitudinal vaginal septum can develop . Ipsilateral renal agenesis is extremely common with this malformation.

40. Classification Uterus didelphys with obstructed hemivagina.
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Classification
Uterus didelphys with obstructed hemivagina.
Complete obstruction.
Partial vaginal communication.
Partial uterine communication.

41. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
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Clinical Manifestation
Typically the patient presents in adolescence with normal menarche, but reports worsening monthly unilateral vaginal and pelvic pain.
In Type II and Type III, the patients have prolonged period (bloody discharge post menstruation).
On examination, a patent vagina and cervix is noted, but a unilateral vaginal and pelvic mass can be seen. The mass represents obstruction of one of the hemivaginas associated with uterine duplication.

42. Treatment Wide excision of the obstructing septum.
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Treatment
Wide excision of the obstructing septum.

43. III UTERUS CONGENITAL ABNORMALITIES
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
III UTERUS CONGENITAL ABNORMALITIES
The most common anomalies of the uterus result from either incomplete fusion of the paramesonephric ducts, incomplete dissolution of the midline fusion of those ducts, or formation failures. Failure of fusion is most evident in uterus didelphys, which presents with two separate uterine bodies, each with its own cervix and attached fallopian tube and vagina. A bicornuate uterus with a rudimentary horn also represents a fusion failure. Less complete fusion failure is seen in the bicornuate uterus with or without double cervices. Incomplete dissolution of the midline fusion of the paramesonephria explains the septate uterus. Failure of formation can be seen in the unicornuate uterus. All of these conditions occur in normal karyotypic and phenotypic females but can be associated with important anomalies of the urinary system.
Although all of these anomalies can occur spontaneously, they may also be caused by early maternal exposure to certain drugs. The most notable of these drugs is diethylstilbestrol (DES). A DES-exposed female infant has an increased risk for a small, T shaped endometrial cavity or cervical collar deformity. DES exposure in utero can also produce fallopian tube abnormalities ,although it does not appear to cause abnormalities of the urinary tract.

44. Classification CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Classification

45. Congenital atresia of the cervix
CHAPTER 37 ANATOMIC DISORDERS OF
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Congenital atresia of the cervix

46. Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF
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Clinical Manifestation
lack the upper vagina. The uterus, however, usually develops normally.
similarly to patients with other obstructive anomalies-- primary amenorrhea and cyclic abdominal or pelvic pain.
endometriosis may have developed secondary to retrograde menstrual flow
Because of the common müllerian source, women with congenital absence of the cervix typically also lack the upper vagina. The uterus, however, usually develops normally.
These patients initially present similarly to patients with other obstructive anomalies, that is, with primary amenorrhea and cyclic abdominal or pelvic pain. If a functional endometrium is present, a patient may have a distended uterus, and endometriosis may have developed secondary to retrograde menstrual flow. A single midline uterine fundus is the norm, although bilateral hemi-uteri have also been described

47. Diagnosis Treatment Sonography MR imaging
Hysterectomy has been recommended
Creation of an epithelialized endocervical tract and vagina
Conservative management with oral contraceptive pills
Radiographic studies, sonography, and MR imaging are helpful in evaluating the anatomy. If imaging demonstrates an obstructed uterus, hysterectomy has been recommended by Rock (1984). In contrast, Niver (1980) reported creation of an epithelialized endocervical tract and vagina in three patients. Significant morbidity, including infection, recurrent obstruction, and death due to sepsis, however, has been reported with establishment of such a vaginal-uterine connection by Casey (1997). Alternatively, conservative management with oral contraceptive pills may be used to suppress retrograde menses and possible endometriosis until a patient is ready to evaluate reproduction options. Thus, the uterus may be retained for possible reproductive potential. For example, Thijssen and associates (1990) reported a successful pregnancy using zygote intrafallopian tube transfer in a patient with cervical agenesis.

48. CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Unicornous uterus
-- Arrested or defective development of only one of the müllerian ducts results in a unicornuate uterus
Arrested or defective development of only one of the müllerian ducts results in a unicornuate uterus

49. [Clinical Manifestation] No symptom
An increased incidence of infertility, endometriosis, and dysmenorrhea
[Diagnosis]
Sonography
MR imaging
[Treatment]
No treatment
Women with a unicornuate uterus have an increased incidence of infertility, endometriosis, and dysmenorrhea .

50. Rudimentary horn of the uterus
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Rudimentary horn of the uterus

51. Classification

52. [Clinical Manifestation]
Dysmenorrhea in type B.
Pregnancy loss and premature labor.

53. -- failed fusion of the paired müllerian ducts
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
-- failed fusion of the paired müllerian ducts
Uterus didelphys
A didelphic uterus results when there is failed fusion of the paired müllerian ducts

54. --Incomplete dissolution of the midline fusion of the paramesonephria
CHAPTER 37 ANATOMIC DISORDERS OF
THE FEMALE REPRODUCTIVE SYSTEM
Septate uterus
--Incomplete dissolution of the midline fusion of the paramesonephria
Incomplete dissolution of the midline fusion of the paramesonephria explains the septate uterus.

55. Uterus bicornis Saddle form uterus

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