fetal circulation

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CHAPTER 37 CHAPTER 37

ANATOMIC DISORDERS OF ANATOMIC DISORDERS OF

THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

Jing-Xin DingJing-Xin DingThe Obstetrics and Gynecology The Obstetrics and Gynecology

Hospital of Fudan University Hospital of Fudan University

Normal Female AnatomyCHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

Section 1 The development of the female reproductive systemSection 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• 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 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→

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

Section 2 Genital tract abnormalities

I Imperforate hymen

CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

-- 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 menstruationClinical Manifestation

Physical Examination A bulging hymen that is often bluish in color A palpable cystic mass compressing rectum by anal examination.

Auxil iary Examination

B-mode ultrasonic examination ： hydrocolpos or hydrometrocolpos

DiagnosisCHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE 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

I I 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, 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 techniqueDavydov technique

Intestinal vaginoplastyIntestinal vaginoplasty - - Segments of sigmoid Segments of sigmoid colon is employed for colon is employed for vaginal replacement.vaginal replacement.

Mclndoe-Reed Mclndoe-Reed vaginoplastyvaginoplasty (Abbe-(Abbe-

Mclndoe-Reed) Mclndoe-Reed) techniquetechnique

Acellular porcine Acellular porcine small intestinal small intestinal submucosa graftsubmucosa graft

Laparoscopic Davydov technique

Intestinal vaginoplasty - Segments of sigmoid colon is employed for vaginal replacement.

段血管夹闭试验选择肠切取段肠 18cm 逆蠕放置顺时针动

自乙状放置吻合器结肠钉钻自直放置管状吻合器肠

• Vaginoplasty using acellular porcine small intestinal submucosa 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 1 week post surgerysurgery

2 month post 2 month post surgerysurgery

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, amenorrheacyclic 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

Auxil iary Examination B-mode ultrasonic examination ： hydrocolpos or

hydrometrocolpos MRI

DiagnosisCHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

• Early surgery

• Vaginal dilation with vaginal mould post surgery

Treatment

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.

Obstructive transverse vaginal septumObstructive transverse vaginal septum

-- usually present during adolescence with -- usually present during adolescence with cyclic lower abdominal pain, amenorrhea, cyclic lower abdominal pain, amenorrhea, and gradual development of a central pelvic and gradual development of a central pelvic mass. mass.

Nonobstructive transverse vaginal septumNonobstructive transverse vaginal septum

-- complain of abnormal menstrual flow, pain -- complain of abnormal menstrual flow, pain with intercourse, difficulty in placing or with intercourse, difficulty in placing or removing tampons, or obstructed labor.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.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.

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.

• Complete longitudinal septum: No syptoms

• In complete longitudinal septum: 　　　 difficulty with intercourse

• The nonobstructed form 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.

ClassificationCHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

• 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.

Clinical ManifestationCHAPTER 37 ANATOMIC DISORDERS CHAPTER 37 ANATOMIC DISORDERS OF OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM

Wide excision of the obstructing septum.

Treatment

I I I UTERUS CONGENITAL ABNORMALITIES

Classif ication

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

Diagnosis • Sonography

• MR imaging

Treatment• Hysterectomy has been recommended

• 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 symptom

An increased incidence of infertility, endometriosis,

and dysmenorrhea [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

--Incomplete dissolution of the midline fusion of

the paramesonephria

Septate uterus

Uterus bicornisSaddle form uterus

THANKS FOR YOUR THANKS FOR YOUR TTENTIONTTENTION

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