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Science
Medicine
Gynecology
Female Congenital Malformation
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Terms in this set (38)
Female congenital malformation
>rare conditions which affect the vagina and endometrium most often
>a congenital absence of the vagina is associated with anomalies of the uterus
>absence of a vagina may lead to an accumulation of menstrual blood within the uterus
>congenital absence of the uterus reflects failure of the mullerian ducts to develop
>associated with other anomalies of the urogenital tract
Congenital Vaginal Malformations
>septate vagina
>imperforate hymen
>vaginal adenosis
Septate Vagina
>failure of the embryonic mullerian duct to fuse properly and resulting median wall does not resorb
Imperforate Hymen
>An outflow tract abnormalities
>Female genital malformation occurs in 5% of the general population
Vaginal Adenosis
>due to diethylstilbestrol
Causes of outflow tract abnormalities
>embryologically, the lower 2/3 of the vagina develop from the urogenital sinus
>the upper vagina, cervix, uterus, fallopian tubes and ovaries form from the Mullerian Duct
>failure of vertical fusion or canalization of these 2 systems in utero results in these abnormalities
>the hymen originates from the embryonic vagina buds from the urogenital sinus
>it becomes perforated forming a central canal with communication between the upper vaginal tract and the vestibule of the vagina
Imperforate Hymen
>mot common
>Normally the central portion of the hymenal membrane is usually absent-creating the typical configuration of ring like structure at the level of the vaginal vestibule
>it may also remain undetected until puberty
Imperforate Hymen in the Neonate
>at birth, the presence of increased mucus secretion in the vagina secondary to maternal estrogen effect may result in a Mucocolpos appearing bulging hymenal membrane between labia
>the membrane may be white bc of the trapped mucoid material and may lead to UTI or bladder obstruction due to urethral compression
Imperforate Hymen in the adolescent
>adolescent usually present with primary amenorrhea (most common clinical presentation)
>other symptoms include lower abd or pelvic pain that may be cyclic, back pain, urinary retention, and constipation
>diagnosis made on examination when a distended bluish hymenal membrane is observed in the introitis - due to collected menstrual blood (hematocolpos)
Imperforate Hymen in Adolescent cont
>the trappe menstrual blood may back up into the uterus
>reflux of endometrial tissue and blood through the fallopian tubes may result in secondary endometriosis
>an accumulation of infected material within the vaginal cavity may cause ascending genital tract infection
Imperforate Hymen in the adolescent
...
Vaginal Adenosis due to DES
>occurs in daughter exposed in utero to DES
>refers to failure of the normal glandular epithelium that lines the embryonic vagina to be replaced by squamous epithelium during fetal development
>DES (morning after pill) a synthetic estrogen
Vagina Adenosis due to DES (cont)
> at 10 weeks of gestation, the upgrowth of squamous epithelium derived from the urogenital sinus replaces the glandular epithelium lining the vagina and exocervix
>DES exposure anywhere from the 10th to about the 18th week of gestation, arrests this transformation process and glandular tissue remains within the vagina
>manifest grossly as red granular patches on the vaginal mucosa
Vaginal Adenosis due to DES
>microspically-there is mucinous columnar cells, similar to the endocervix along with ciliated cells with eosinophilic cytoplasm- similar to the lining cells of the fallopian tube and endometrium
>the glandular cells ultimately undergo squamous metaplasia
>rare cases of clear cell adenocarcinoma of the vagina have also occurred in the daughters of women treated with DES
Clear Cell Adenocarcinoma due to DES
>rare tumor of the vagina encountered exclusively in women exposed to DES
>Develop most frequently on the anterior wall of the upper 3rd of the vagina
>common between 17 and 22
>abundant glycogen account for the clear nature of the cytoplasm and are essentially curable when small and asymptomatic
Clear Cell Adenocarcinoma due to DES
>although most all cases of clear cell adenocarcinoma are associated with vaginal adenosis very few women with adenosis develop this cancer
>in more advanced stages, they may spread by hematogenous or lymphatic routes
Vaginal Atresia
>congenital anomaly of the female genital tract that presents as a deformed obstructed and nonfunctional vagina that occurs in 1 in 4-5000 live female births
>often unnoticed until adolescence when pain and lack of normal menstrual flow indicated the condition- which is due to obstruction
Vaginal Atresia (cont)
>frequently associated with Rokatansky-Mayer-Kuster Hauser Syndrome
-congenital lack of a uterus, vaginal atresia, agenesis, despite normal ovaries and external genitalia
>surgery may repair atresia
Vaginal Septum
>congenital longitudinal or transverse partition within the vagina both corrected with surgery
Vaginal Septum
1. longitudinal
2. transverse
1. longitudinal- develops during embryogenesis due to incomplete fusion of the two mullerian ducts, resulting in a double vagina, with a chance of a double cervix and uterus
2.transverse-lso develops during embryogenesis when the two mullerian ducts fuse improperly to the urogenital sinus. A complete septum blocks menstrual flow and is a cause of primary amenorrhea
both- causes obstruction to flow and dyspareunia
Cervical Stenosis
>A congenital or acquired abnormality that occurs at the level of the internal os,
> The most common acquired cause is due to prior diathermy excision of cervical lesions
> Congenital stenosis is usually associated with dilation of the lower uterine segment, and may be an obstruction with menstrual flow.
> Examination reveals an elongated, rigid cervix with no elasticity and a small contracted os
Congenital Anomaly of the uterus
>Bicornis
>Uterus Didelphys
>Uterus Septae
Bicornis
is a uterus with a common fused wall between two distinct uterine cavities. Due to a failure of the common wall between the apposed mullerian ducts to degenerate, forming a single uterine cavity
Uterus Didelphys
refers to a double uterus with a double vagina. Again due to failure of the two mullerian ducts to fuse during embryonic life
Uterus septae
refers to a single uterus with a partial remaining septum, owing to failure of the wall of the fused mullerian ducts to fully resorb
Mayer Rokitansky Kuster-Hauser Syndrome
• A congenital malformation of the mullerian ducts to develop resulting in an absent uterus and a shortened malformed vagina
• Second most common cause of primary amenorrhea (after Turner Syndrome)
• Also known as Mullerian Agenesis
• Has development of female secondary sex characteristics, including breast development and pubic hair, has ovulation, and a normal 46XX chromosome complement.
Mayer Rokitanksky Kuster Hauser Syndrome
Prevalence is approx. 1 in 5000 live female births
• Comes to attention when, women at puberty, have no initial menstrual cycle
• Since there is no uterus, women cannot get pregnant. On U/S, there may be partial or total absence of the cervix and vagina
• There is also an association with renal anomalies (about 30%) hernias, and hearing defects.
• Vaginal dilators or surgery may be done to alleviate painful intercourse
Turner Syndrome
>complete or partial monosomy of x chromosome
>characterized by hypogonadism in phenotypic females; commonly affects female
>the majority of cases results from the complete monosomy-results in a 45 xo karyotype
Clinical feature of turner syndrome
>short, less than 150cm
>amenorrhea- single most important cause of primary amenorrhea accounting for 1/3 of the cases
>pt present during infancy with edema due to lymph stasis of the dorsum of the hand and feet
Turner syndrome cont
>webbing of neck - due to cystic hygroma
>as infant grow older the webbing become looseness of skin on the back of the neck
>broad shaped chest - widely spaced nipples
>pigmented nevi on the skin of the extremity and trunk
Turner Syndrome cont
>abnormal carrying angle of the arm called cubitus valgus
>congenital heart defects are common- coarctation of the aorta and bicuspid aortic valves
>cardio abnormalities are the single most important cause of death
Turner syndrome cont
>At puberty, there is failure to develop normal secondary sex characteristics-the genitalia remains infantile, breast development is inadequate, there is little pubic hair, and the ovaries are reduced to atrophic, fibrous strands (streak ovaries), resulting in infertility.
>The mental status is usually normal, but a few may exhibit some mental retardation.
Non disjunction involve the sex chromosome
...
Coarctation of the aorta
...
True Hermaphrodite
...
Pseudohermaphrodites
...
Male pseudohermaphrodite
...
46XY Disorder of sexual developmental male pseudohermaphroditsm
...
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