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

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE

REPRODUCTIVE SYSTEM

Jing-Xin DingThe Obstetrics and Gynecology Hospital

of Fudan University

Normal Female Anatomy

Section 1The development of the female reproductive system

Testis-determing factor,TDF on the Y chromosome

The primordial germ cell to develop to ovary or testis

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 ）

Genital tract ——Mullerian duct (Paramesonephric duct)

• In the absence of anti-müllerian hormone (AMH), the mesonephric duct system degenerates, and the paramesonephric duct system subsequently develops.– Cephalic segment→Fallopian tubes– Middle segment→Uterus corpus and cervix

The development of the vagina

Inferior segment of the Mullerian tube→Upper segment of the vagina

The urogenital sinus → the lower 2/3 vagina

• External Genitalia——urogenital Fold

• Autonomic →Female• Androgen and its

enzyme (5α–Reductase) and its receptor→Male

Section 1 The development of the female reproductive system

SummaryTestis-determing factor,TDF on the Y

chromosome

The primordial germ cell to develop to ovary or testis

• Genital tract ——Mullerian duct (Paramesonephric duct)

Common Reason

1. Improper fusion of the paramesonephric ducts

2. Incomplete development of one paramesonephric duct

3. Failure of part of the paramesonephric duct on one or

both sides to develop

4. Absent or incomplete canalization of the vaginal plate

Section 2 Genital tract abnormalities

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

• a history of vague abdominal pain with approximately monthly exacerbations

• No menstruation

Clinical Manifestation

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

Diagnosis

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

II VAGINA CONGENITAL ABNORMALITIES

Congenital absence of vaginaMayer-Rokitansky-Küster-Hauser syndrome (MRKHS)

• The incidence is estimated to be 1 in 4000-10000 live-born girls.

• Primary amenorrhea

• Difficulty in sexual activity

• Most patients have primordial uterus without functional endometrium, no periodic abdominalgia.

Clinical manifestation

• 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

• Vaginal dilation treatment

• Surgical vaginoplasty

Treatment

Vaginoplasty in patients with MRKH syndrome

Davydov techniqueIntestinal vaginoplasty -

Segments of sigmoid colon is employed for vaginal replacement.

Mclndoe-Reed vaginoplasty (Abbe-Mclndoe-Reed) technique

Acellular porcine small intestinal submucosa(SIS) graft

Laparoscopic Davydov technique

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

Mclndoe-Reed vaginoplasty

• Vaginoplasty using acellular porcine small intestinal submucosa (SIS) graft

Acellular matrix graft promote the rapid ingrowth of surrounding

tissue and epithelialization of the neovagina is allowed to occur

Pictures under colposcopy (Schiller’s Iodine test)

1 week post surgery

2 month post surgery

The appearance of the neovagina 6 months post surgery

Atresia of Vagina

---lack the lower portion of the vagina ---the urogenital sinus does not participate in the formation of the vagina.

The same symptom with imperforate hymen.

– Cyclic lower abdominal pain

– Amenorrhea

Clinical Manifestation

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

Diagnosis

• Early surgery

• Vaginal dilation with vaginal mould post surgery

Treatment

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

Transverse Vaginal Septum

• 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

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

Clinical ManifestationObstructive 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.

• 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

• 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.

Surgical repair is dependent upon septal thickness.Smaller septa may be approached by excision with an end-to-end anastomosis of the upper to the lower vagina. Skin grafts may occasionally be necessary to cover a defect left by excision of very thick septa.

Treatment

Longitudinal Vaginal Septum

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.

• No syptoms

• Difficulty with intercourse

Clinical Manifestation

• --can be managed conservatively unless dyspareunia develops.

• Surgical treatment includes resection of the longitudinal septum.

Treatment

Oblique Vaginal Septum Syndrome

Herlyn-Werner-Wunderlich syndrome (HWWS)

Uterus didelphys with obstructed hemivagina. A. Complete obstruction. B. Partial vaginal communication.

C. Partial uterine communication.

Classification

• 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.

Clinical Manifestation

Wide excision of the obstructing septum.

Treatment

III UTERUS CONGENITAL ABNORMALITIES

Congenital atresia of the cervix

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

lack a cervix (cervical agenesis) An intact cervical body with obstruction of the cervical osa cervical body consisting of a fibrous bandcervical fragmentation

Classification

Rock JA, Carpenter SE, Wheeless CR, Jones HW. J Pelv Surg 1995;1: 129-133.

Diagnosis • Sonography

• MR imaging Treatment• Hysterectomy • Creation of an epithelialized endocervical tract and vagina• Conservative management with oral contraceptive pills

-- Arrested or defective development of only one of the müllerian ducts results in a unicornuate uterus

Unicornous uterus

[Clinical Manifestation]No symptomAn increased incidence of infertility, abortion, preterm labor and

rupture of the uterus during late pregnancy. [Diagnosis]• Sonography• MR imaging[Treatment] No treatment

Rudimentary horn of the uterus

Classification

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

labor.

-- failed fusion of the paired müllerian ducts

Uterus didelphys

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

--Incomplete dissolution of the midline fusion of the

paramesonephria

Septate uterus

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

Uterus bicornisSaddle form uterus

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

Summary

• a history of vague abdominal pain with approximately monthly exacerbations

• No menstruation

Clinical Manifestation

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

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

DiagnosisCHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

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

II VAGINA CONGENITAL ABNORMALITIES

• Congenital absence of vagina• Atresia of vagina

Transverse Vaginal Septum

Longitudinal Vaginal Septum

Oblique Vaginal Septum Syndrome

Herlyn-Werner-Wunderlich syndrome (HWWS)

III UTERUS CONGENITAL ABNORMALITIES

CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

RememberA young girl with cyclic abdominal pain

--- ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEM

--- Gynecologic examination and B ultrasonic examination

丁景新 Jingxin DingEmail: [email protected]

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