Anatomic Disorders of Female Reproductive System

7/27/2019 Anatomic Disorders of Female Reproductive System

1/42

Anatomic Disorders of Female

Reproductive System may result from:

Genetic mutation

Developmental arrest

Environmental insults that may exert theireffects at critical stages of embryonic

development


2/42

Normal Embryology

Overview


3/42


4/42


5/42

Congenital Ambiguity of the Genital

Tract


6/42

Female pseudohermaphroditism

Pathophysiology

-excessive androgen exposure of an embryo or

fetus

-excessive androgn exposure may stem from

adrenal abnormalities or nonadrenal sources

+ Treatment

-genitoplasty


7/42

Male Pseudohermaproditism

+ Pathophysiology

-insufficient androgen exposure of fetus

+ Presentation and Treatment

-external genetalia appear as normal female

-an incomplete form

-treatment consists of replacement with phy

siologic level of estrogen and creation of a fung

sional vagina either by dilatation or surgical vagi

noplasty


8/42

Disorders of Genetic or Gonatal

Development

*Gonatal Dysgenesis

-nondisjunction of parental chromosomes and

leads to abnormal gonadal development and

streak gonad

-The classic stigmata of turner syndrome

-short stature (final height less than 58 inches)

-widely spaced nipples

-webbed neck


9/42


10/42

*True Hermaphroditism

-a thrue hermaphroditism has both ovarian

an testicular gonadal tissue-the testes are usually small

-penis may be small

-azoospermia


11/42


12/42

*Embryonic testicular regression

-MIS may or may not be produced

-the uterus may be present or absent


13/42


14/42

Defects of the Bladder and Perineum

+Bladder exstrophy

-failure of the cloacal membrane to be

reinforced by an ingrowth of mesoderm

-the characteristic widening of the symphysis

pubis

-The urethra and vagina are typically short and

the vaginal orifice is frequently stenotic anddisplaced anteriorly.

-The clitoris is duplicated or bifid, and the labia,

mons pubis, and clitoris are divergent


15/42


16/42

Treatment

-Reconstruction of the female genitalia

D f t f th Clit i


17/42

Defects of the Clitoris

*Clitoral Anomalies

- bifid clitoris

- Female epispadias can be divided into threetypes vestibular, subsymphyseal, and

retrosymphysealwhich are differentiated by

the type of urethral involvement (Schey, 1980


18/42

Clitoromegaly

- fetal exposure to excessive androgens

-Adult women with exposure to androgenexcess may also present with some degree of

clitoromegaly


19/42

*Labial Fusion

-young neonates or prepubertal girls when the

labia and vagina are not adequately

estrogenized


20/42

Hymeneal Defects

*Description and Patient Presentation

-The hymen is the membranous vestige of the

junction between the sinovaginal bulbs and the

urogenital sinus

- A variety of hymeneal abnormalities

- microperforate, septate, cribriform, and

imperforate hymen


21/42


22/42

hydromucocolpos

In infants, the obstructed vagina may distend

from mucus accumulation


23/42

Hematocolpos

-adolescents present after menarche and

menstrual blood trapped in the vagina behind

the imperforate hymen


24/42

Treatment of Hymenal Defects

- Hymenectomy


25/42

Transverse Vaginal Septum

Description and Patient Presentation

-Vertical fusion refers to complete cavitation ofthe vaginal plate between the sinovaginal bu lbsand uterovaginal canal

-In neonates and infants, obstructive transversevaginal septum has been associated with fluid andmucus collection in the upper vagina, resulting in amass that that may be large enough to compress

abdominal or pelvic organs (Adaletli, 2007). It hasbeen reported to limit diaphragmatic movement,and neonatal deaths have been reported.


26/42

-Patients with obstructive transverse vaginal

septum usually present during adolescence with

cyclic lower abdominal pain, amenorrhea, and

gradual development of a central pelvic mass.


27/42

Diagnosis and Treatment

-The diagnosis is suspected when an abdominal

or pelvic mass is palpated or when a foreshortened

vagina and inability to identify the cervix isencountered. Diagnosis is confirmed by either

sonography or magnetic resonance (MR) imaging.

Magnetic resonance imaging is most helpful prior tosurgery to determine the thickness and depth of

the transverse septum

-surgical therapy


28/42

Longitudinal Vaginal Septum

-A longitudinal vaginal septum results from

defective lateral fusion and incomplete

reabsorption of the paired mllerian ducts


29/42

Mllerian Anomalies


30/42


31/42

Vaginal Agenesis

Females with vaginal atresia lack the lower

portion of the vagina, but otherwise have

normal external genitalia-


32/42

Mllerian Agenesis

the uterus, cervix, and upper part of the

vagina are absent -


33/42

Unicornuate Uterus

Arrested or defective development of only one

of the mllerian ducts results in a unicornuate

uterus -


34/42

Uterine Didelphys

-This anomaly is characterized by the

presence of two endometrial cavities, each with

a uterine cervix


35/42

Bicornuate Uterus

-A bicornuate uterus is caused by incomplete

lateral fusion of the mllerian ducts. It ischaracterized by two separate but

communicating endometrial cavities and a sin

gle uterine cervix-


36/42

Septate Uterus

-After lateral fusion of the mllerian ducts,

failure of their medial segments to regress can

create a permanent septum within the uterine

cavity

-septate uterus is associated with a marked

increase in spontaneous abortion (Heinonen,

2006


37/42

Arcuate Uterus

-An arcuate uterus is only a mild deviation

from the normally developed uterus

Cervical Defects


38/42

Cervical Defects

Cervical Defects

- These patients initially present similarly to

patients with other obstructive anomalies, that

is, with primary amenorrhea and cyclic

abdominal or pelvic pain.


39/42

Cervical Stenosis

Symptoms of stenosis in menstruating women

include dysmenorrhea, abnormal bleeding,

amenorrhea, and infertility -


40/42

Ovarian Anomalies

A supernumerary ovary is an ectopic ovary

that has no connection with the broad, utero-

ovarian, or infundibulopelvic ligaments


41/42

the term accessory ovary is used when excess

ovarian tissue is noted near a normally placed

ovary and is connected to it


42/42

Fallopian Tube Anomalies

Congenital Tubal Disease

- the appendix vesiculosa

Date post:	14-Apr-2018
Category:	Documents
Upload:	nelly-astika
View:	222 times
Download:	0 times

Anatomic Disorders of Female Reproductive System

Documents