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Pathologic Obstetrics > Congenital Abnormalities of the Reproductive Tract > Flashcards

Congenital Abnormalities of the Reproductive Tract Flashcards

1
Q

What is the common mesodermal ridge called?

A

Intermediate mesoderm

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2
Q

Anlage of abdominal cavity, urinary, and genital system

A

Intermediate mesoderm

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3
Q

What structure is in close association to the hindgut?

A

Urogenital ridge

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4
Q

When is the urogenital ridge formed?

A

3-5 weeks AOG

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5
Q

What do you call an elevation of the intermediate mesoderm?

A

Urogenital ridge

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6
Q

Urogenital ridge will become the?

A

Urogenital tract

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7
Q

Urogenital tract divides into?

A

Genital/gonadal ridge and Nephrogenic ridge/cord

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8
Q

Anlage of Ovaries

A

Genital/Gonadal Ridge

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9
Q

Anlage of Genitourinary tract

A

Nephrogenic ridge/cord

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10
Q

Nephrogenic ridge develop into?

A

Mesonephros with paired mesonephric duct

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11
Q

Other name for mesonephric duct

A

Wolffian duct

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12
Q

What structure is adjacent to Mesonephric duct?

A

Paramesonephric duct

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13
Q

Other name for paramesonephric duct

A

Mullerian duct

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14
Q

What structures drain into the cloaca?

A

Mesonephros, Mesonephric duct, Paramesonephric duct

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15
Q

Mesonephric duct or Wolffian duct will regress in the absence of what hormone?

A

Testosterone

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16
Q

Without testosterone, what structure will regress and what structure favors the growth?

A

REGRESS- Mesonephric duct/ Wolffian duct (Phenotypically male)
GROW- Paramesonephric duct/ Mullerian duct (Phenotypically female)

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17
Q

Anlage of uterus

A

Fusion of 2 Mullerian ducts

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18
Q

Primitive kidney

A

Mesonephros

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19
Q

Common opening for both urinary and alimentary tract

A

Cloaca

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20
Q

Emergence of mesonephric duct

A

3-5 weeks AOG

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21
Q

Emergence of 2 ureteric buds

A

4-5 weeks AOG

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22
Q

Emergence of Mullerian duct

A

4-5 weeks AOG

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23
Q

When is the cloaca divided by the urorectal septum?

A

7 weeks AOG

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24
Q

What structure will form when cloaca is divided by urorectal septum?

A

Rectum and Urogenital sinus

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25
Q

What are the 3 parts of urogenital sinus?

A

Cephalad/ Vesicle portion
Middle/ Pelvic portion
Caudal/ Phalic portion

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26
Q

Anlage of the urinary bladder

A

Cephalic/ Vesical portion of urogenital sinus

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27
Q

Anlage of the Female urethra

A

Middle/ Pelvic portion of urogenital sinus

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28
Q

Caudal/ Phalic portion of urogenital sinus will become what?

A

Distal vagina
Greater vestibular (Bartholin) glands
Paraurethral (Skene’s) glands

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29
Q

When is uterus starts to develop?

A

10 weeks AOG

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30
Q

What is being formed by cellular proliferation of the upper portion of fused Mullerian duct?

A

1st uterine cavity

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31
Q

What is the characteristic shape of 1st uterine gland?

A

Pyriform wedge

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32
Q

What happens as 20 weeks AOG

A

Complete uterine cavitation
Complete vaginal canalization
Formation of cervix and upper vagina

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33
Q

Anlage of Fallopian tube

A

Mullerian duct

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34
Q

Anlage of UPPER vagina

A

Mullerian duct

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35
Q

What will be the result if there is failure of 2 Mullerian ducts to fuse?

A

2 separate uterine horns

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36
Q

What will be the result if there is failure of resorption of the common tissue of 2 Mullerian ducts?

A

Persistent Uterine Septum

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37
Q

What structure connects to the fused Mullerian ducts?

A

Urogenital sinus

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38
Q

Fused Mullerian ducts + Urogenital sinus = ?

A

Sinovaginal bulbs

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39
Q

Sinovaginal bulbs will become?

A

Vaginal plate

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40
Q

Vaginal lumen is separated from urogenital sinus by?

A

Hymenal membrane

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41
Q

Hymenal membrane will degrade forming?

A

Hymenal ring

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42
Q

Results from failure of the inferior end of the vaginal plate which is the hymeneal membrane to canalize

A 
Imperforate hymen
Microperforate hymen
Cribriform Hymen
Navicular hymen
Septate hymen
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43
Q

History and PE findings of imperforate hymen

A

Bulging bluish membrane
Amenorrhea
Cyclic abdominal pain
Adolescent age

44
Q

4 major deformities that arise from defective Mullerian duct development

A
  1. Agenesis of both ducts, either focally or along the entire duct length
  2. Unilateral maturation of one mullerian duct with incomplete or absent development of the opposite duct results in defects associated with upper urinary tract abnormalities
  3. Absent or faulty midline fusion of the Mullerian ducts (most common)
  4. Defective canalization
45
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 1 is called?

A

Segmental Mullerian Hypoplasia or Agenesis

46
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 2 is called?

A

Unicornuate uterus

47
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 3 is called?

A

Uterine Didelphys

48
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 4 is called?

A

Bicornuate uterus

49
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 5 is called?

A

Septate uterus

50
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 6 is called?

A

Arcuate uterus

51
Q

American Fertility Society Classification of Mullerian Anomalies (1988) Class 7 is called?

A

Diethylstilbestrol related

52
Q

Segmental Mullerian Hypoplasia or Agenesis can affect what structures?

A

Vagina
Uterus
Fallopian tubes

53
Q

Upper vaginal agenesis is associated with uterine hypoplasia or agenesis and less often displays abnormalities of the
renal, skeletal and auditory systems.

A

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome

54
Q

MURCS triad

A

Mullerian duct aplasia, renal aplasia, Cervical Somite dysplasia

55
Q

True/False

Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is phenotypically female

A

True

56
Q

What is the obstetrical significance of vaginal agenesis?

A

Barrier to pregnancy, no vaginal intercourse possible unless corrected operatively

57
Q

What is the obstetrical significance of uterine agenesis?

A

Prevents pregnancy

58
Q

What is the obstetrical significance of complete mullerian agenesis?

A

Impossible pregnancy

59
Q

What is the obstetrical significance of vaginal atresia?

A

Associated with urologic abnormalities

Precludes pregnancy by vaginal intercourse

60
Q

What is the obstetrical significance of incomplete septum?

A

Interferes with the descent of fetal head

61
Q

Management for Agenesis

A

Invitro fertilization
Uterine transplantation
Surgery

62
Q

Management for vaginal septum

A

Cruciate incision
Cesarean delivery
Adequate analgesia, inferior attachment of the septum may be isolated, clamped, transected and ligated

63
Q

Types of vaginal septum

A

Longitudinal and transverse

64
Q

Etiology of congenital vaginal septum

A

Fusion or resorption defect

65
Q

2 types of Longitudinal septum

A

Complete or incomplete

66
Q

Divides the vagina into right and left portions

A

Complete Vaginal Septum

67
Q

Septa is extending thru the entire vaginal length

A

Complete Vaginal Septum

68
Q

True of False?

Complete vaginal septum prevent pregnancy or be a cause of labor dystocia because the vagina dilates unsatisfactorily

A

False

  • does not prevent pregnancy
  • dilates satisfactorily
69
Q

True or False?

Incomplete or Partially Obstructed Longitudinal Septum may interfere with fetal head descent

A

True

70
Q

May develop at any depth of the vagina but most commonly on the lower third

A

Transverse Vaginal Septum

71
Q

True or false?

Transverse vaginal septum may or may not be perforated therefore there may be obstruction and infertility

A

True

72
Q

How is uterine malformation being discovered?

A 
Routine pelvic exam
CS
Manual exploration
Tubal sterilization
Infertility evaluation
73
Q

What feature is most often indicative of malformed uterus by abdominal palpation?

A

Fundal notching

74
Q

Most common finding in uterine malformation

A

Arcuate uterus

followed by: (descending order)
Septate
Bicornuate
Didelphic
Unicornuate
75
Q

Diagnostic options for uterine malformation

A

Ultrasound
Hysterosalpingography- contraindicated in Pregnancy
MRI
Laparoscopy or Hysteroscopy

76
Q

Diagnostic modality that improves delineation of the endometrium and
internal uterine morphology, but only with a patent endometrial cavity.

Contraindicated in pregnancy.

A

Saline Infusion Sonography (SIS)

77
Q

What is the obstetrical significance of uterine malformation?

A 
Miscarriage
Ectopic pregnancy
Rudimentary horn pregnancy
Preterm delivery
Fetal growth restriction
Abnormal fetal lie
Uterine dysfunction
Uterine rupture
78
Q

Develops due to an underdeveloped or rudimentary uterine horn may be absent

A

Class II/ Unicornuate Uterus

79
Q 
True/ False
In class I Mullerian Abnormality, It may or may not communicate with the dominant horn and may or may not have an endometrium-lined cavity
A

False (Class 2 not class 1)

80
Q

How can you tell if it is a communicating or non-communicating horn in Class II Mullerian abnormality?

A

A non-communicating horn will not be filled up with dye so it cannot be seen in hysterosalphingogram (HSG/HSSG) where
there is an infusion of a sterile saline into the uterine cavity thru the cervix

81
Q

Superior diagnostic tool for unicornuate uterus

A

MRI

3D ultrasound only increase accuracy

82
Q

What is the obstetrical significance of unicornuate uterus?

A

Increased incidence of infertility, endometriosis, and dysmenorrhea

Implantation in the normal-sized hemiuterus is associated with
incidence of:
Abortion
Preterm delivery
Fetal growth restriction
Breech presentation
Dysfunctional labor
Cesarean delivery
Ectopic pregnancy in rudimentary horns (includes noncommunicating cavitary rudiments, for which transperitoneal sperm migration permits ovum fertilization and
pregnancy)
83
Q

Arises from a complete lack of fusion of the 2 Mullerian ducts that results into 2 entirely separate hemiuteri (small uterus with smaller capacity), cervices, and 2 vaginas

Most women will have a double vagina or a longitudinal septum

A

Class III/ Uterus Didelphys

84
Q

What is OHVIRA

A

Obstructed hemivagina and ipsilateral renal agenesis

85
Q

True or false?

Class II may be isolated or with ipsilateral renal agenesis (OHVIRA or Herlyn Werner-Wunderlich Syndrome)

A

False (Class 3 not 2)

86
Q

Complications of Class III

A 
Miscarriage
Preterm delivery
Fetal growth restriction
Breech presentation
Increased Cesarean delivery rate
(BUT LESS FREQUENT THAN CLASS 2)
87
Q

PE finding in Class 3

A

Suspected with identification of a longitudinal septum and 2 cervices

88
Q

Management for Class 3

A

Metroplasty

(But some women deliver successfully → no surgical management is
performed)

89
Q

Resection of intervening myometrium with fundal recombination

(Rarely done) only performed in highly selected women such as those with unexplained miscarriage (ex. Several fetal losses at 26 weeks)

A

Metroplasty

90
Q

From a fusion anomaly

Forming 2 hemiuteri with a central myometrium running partially or completely to the cervix

A

Class 4/ Bicornuate Uterus

91
Q

What is the obstetrical significance of bicornuate uterus?

A

Diminished fertility
Abortion when the embryo implants on the septum
Preterm deliveries
Malpresentation

92
Q

Management of Class 5

A

Hysterescopic resection

(if the woman will present with poor obstetrical history like several abortions, history of preterm birth especially if it does not reach viability)

93
Q

Why is there an increased risk of miscarriage in Class 4 and 5?

A

Due to the abundant muscle tissue in the septum or the intervening myometrium

94
Q

Differential diagnosis for Class 4

A

Class 5/ Septated Uterus

(important since it is only managed with simple hysteroscopic resection)

3D ultrasound and MRI provide distinction between bicornuate uterus/Class 4 and septated uterus

95
Q

Resorption defect which results into a uterine septum involving the cervix, vagina, and uterus

A

Class 5/ Septated uterus

96
Q

What is the obstetrical significance of septated uterus?

A

Diminished fertility
Abortion when the embryo implants on the septum
Preterm deliveries
Malpresentation

97
Q

This malformation is only a mild deviation from the normally developed uterus

A

Class 6/ Arcuate uterus

98
Q

Management for Class 6

A

Transabdominal cerclage
Metroplasty
No need for surgery for nonobstructive defects

99
Q

Management of Class 6 for those with uterine anomalies, repetitive pregnancy loss, partial cervical atresia or hypoplasia may benefit

A

Transabdominal cerclage

100
Q

Rare now; in the 1960s or 1970s, DES was given as a medication to prevent miscarriage (threatened abortion, preterm labor, preeclampsia, and diabetes); several years after, several women who were exposed to DES in utero presented with several structural abnormalities

A

Class 7/ Diethylstilbestrol-Induced Abnormalities

101
Q

Structural abnormalities in Class 7

A

Transverse septa
Circumferential ridges involving the vagina and cervix
Cervical collars

102
Q

Reproductive performance in Class 7

A

Impaired conception rates
Higher rates of ectopic pregnancy
Preterm delivery

103
Q

Gynecologic effects in Class 7

A

Early menopause
Risk for Cervical Intraepithelial Neoplasia (CIN)
Breast cancer

104
Q

Cervical abnormalities in Class 7

A

Partial or complete agenesis
Duplication
Longitudinal septa

105
Q

Most important obstetrical relevance of Class 7

A

Complete cervical atresia is incompatible with conception

106
Q

Vaginal abnormalities in Class 7

A

Double Vagina and Double introitus

107
Q

Double introitus in Class 7 appears as?

A

“Double barreled Shotgun”

Pathologic Obstetrics (3 decks)

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