chapter 37 anatomic disorders of anatomic disorders of the female reproductive system jing-xin ding...
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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 menstruation
Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE 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 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
II VAGINA CONGENITAL ABNORMALITIES
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Congenital absence of vaginaMayer-Rokitansky-Küster-Hauser syndrome (MRKHS)
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
• 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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE 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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
• Vaginal dilation treatment
• Surgical vaginoplasty
Treatment
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Vaginoplasty in patients with MRKH syndrome
Davydov techniqueDavydov technique
Intestinal Intestinal vaginoplastyvaginoplasty - - Segments of Segments of
sigmoid colon is sigmoid colon is employed for employed for
vaginal vaginal replacement.replacement.
Mclndoe-Reed Mclndoe-Reed vaginoplastyvaginoplasty
(Abbe-Mclndoe-(Abbe-Mclndoe-Reed) techniqueReed) technique
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.
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
• 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.
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
The same symptom with imperforate hymen.
cyclic lower abdominal pain, amenorrheacyclic lower abdominal pain, amenorrhea
Clinical Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
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
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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Transverse Vaginal Septum
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
• 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 Manifestation
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Obstructive transverse vaginal septumObstructive transverse vaginal septum
-- usually present during adolescence -- usually present during adolescence with cyclic lower abdominal pain, with cyclic lower abdominal pain, amenorrhea, and gradual development amenorrhea, and gradual development of a central pelvic mass. of a central pelvic mass.
Nonobstructive transverse vaginal Nonobstructive transverse vaginal septumseptum
-- complain of abnormal menstrual -- complain of abnormal menstrual flow, pain with intercourse, difficulty in flow, pain with intercourse, difficulty in placing or removing tampons, or placing or removing tampons, or obstructed labor.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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
• 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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Longitudinal Vaginal Septum
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE 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.
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
• Complete longitudinal septum: No syptoms
• In complete longitudinal septum: difficulty with intercourse
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Clinical Manifestation
• The nonobstructed form can be managed
conservatively unless dyspareunia
develops. Surgical treatment includes
resection of the longitudinal septum.
Treatment
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Oblique Vaginal Septum Syndrome
Herlyn-Werner-Wunderlich syndrome (HWWS)
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
III UTERUS CONGENITAL ABNORMALITIES
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Classification
Congenital atresia of the cervix
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
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
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
[Clinical Manifestation]
No symptom
An increased incidence of infertility, endometriosis,
and dysmenorrhea [Diagnosis]• Sonography• MR imaging
[Treatment]
No treatment
Rudimentary horn of the uterus
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
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 CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
--Incomplete dissolution of the midline fusion of
the paramesonephria
Septate uterus
CHAPTER 37 ANATOMIC DISORDERS OF CHAPTER 37 ANATOMIC DISORDERS OF THE FEMALE REPRODUCTIVE SYSTEMTHE FEMALE REPRODUCTIVE SYSTEM
Uterus bicornisSaddle form uterus
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