Congenital Abnormalities of the Reproductive Tract Flashcards

(107 cards)

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
What are the 3 parts of urogenital sinus?
Cephalad/ Vesicle portion Middle/ Pelvic portion Caudal/ Phalic portion
26
Anlage of the urinary bladder
Cephalic/ Vesical portion of urogenital sinus
27
Anlage of the Female urethra
Middle/ Pelvic portion of urogenital sinus
28
Caudal/ Phalic portion of urogenital sinus will become what?
Distal vagina Greater vestibular (Bartholin) glands Paraurethral (Skene's) glands
29
When is uterus starts to develop?
10 weeks AOG
30
What is being formed by cellular proliferation of the upper portion of fused Mullerian duct?
1st uterine cavity
31
What is the characteristic shape of 1st uterine gland?
Pyriform wedge
32
What happens as 20 weeks AOG
Complete uterine cavitation Complete vaginal canalization Formation of cervix and upper vagina
33
Anlage of Fallopian tube
Mullerian duct
34
Anlage of UPPER vagina
Mullerian duct
35
What will be the result if there is failure of 2 Mullerian ducts to fuse?
2 separate uterine horns
36
What will be the result if there is failure of resorption of the common tissue of 2 Mullerian ducts?
Persistent Uterine Septum
37
What structure connects to the fused Mullerian ducts?
Urogenital sinus
38
Fused Mullerian ducts + Urogenital sinus = ?
Sinovaginal bulbs
39
Sinovaginal bulbs will become?
Vaginal plate
40
Vaginal lumen is separated from urogenital sinus by?
Hymenal membrane
41
Hymenal membrane will degrade forming?
Hymenal ring
42
Results from failure of the inferior end of the vaginal plate which is the hymeneal membrane to canalize
``` Imperforate hymen Microperforate hymen Cribriform Hymen Navicular hymen Septate hymen ```
43
History and PE findings of imperforate hymen
Bulging bluish membrane Amenorrhea Cyclic abdominal pain Adolescent age
44
4 major deformities that arise from defective Mullerian duct development
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
American Fertility Society Classification of Mullerian Anomalies (1988) Class 1 is called?
Segmental Mullerian Hypoplasia or Agenesis
46
American Fertility Society Classification of Mullerian Anomalies (1988) Class 2 is called?
Unicornuate uterus
47
American Fertility Society Classification of Mullerian Anomalies (1988) Class 3 is called?
Uterine Didelphys
48
American Fertility Society Classification of Mullerian Anomalies (1988) Class 4 is called?
Bicornuate uterus
49
American Fertility Society Classification of Mullerian Anomalies (1988) Class 5 is called?
Septate uterus
50
American Fertility Society Classification of Mullerian Anomalies (1988) Class 6 is called?
Arcuate uterus
51
American Fertility Society Classification of Mullerian Anomalies (1988) Class 7 is called?
Diethylstilbestrol related
52
Segmental Mullerian Hypoplasia or Agenesis can affect what structures?
Vagina Uterus Fallopian tubes
53
Upper vaginal agenesis is associated with uterine hypoplasia or agenesis and less often displays abnormalities of the renal, skeletal and auditory systems.
Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome
54
MURCS triad
Mullerian duct aplasia, renal aplasia, Cervical Somite dysplasia
55
True/False | Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is phenotypically female
True
56
What is the obstetrical significance of vaginal agenesis?
Barrier to pregnancy, no vaginal intercourse possible unless corrected operatively
57
What is the obstetrical significance of uterine agenesis?
Prevents pregnancy
58
What is the obstetrical significance of complete mullerian agenesis?
Impossible pregnancy
59
What is the obstetrical significance of vaginal atresia?
Associated with urologic abnormalities | Precludes pregnancy by vaginal intercourse
60
What is the obstetrical significance of incomplete septum?
Interferes with the descent of fetal head
61
Management for Agenesis
Invitro fertilization Uterine transplantation Surgery
62
Management for vaginal septum
Cruciate incision Cesarean delivery Adequate analgesia, inferior attachment of the septum may be isolated, clamped, transected and ligated
63
Types of vaginal septum
Longitudinal and transverse
64
Etiology of congenital vaginal septum
Fusion or resorption defect
65
2 types of Longitudinal septum
Complete or incomplete
66
Divides the vagina into right and left portions
Complete Vaginal Septum
67
Septa is extending thru the entire vaginal length
Complete Vaginal Septum
68
True of False? | Complete vaginal septum prevent pregnancy or be a cause of labor dystocia because the vagina dilates unsatisfactorily
False - does not prevent pregnancy - dilates satisfactorily
69
True or False? | Incomplete or Partially Obstructed Longitudinal Septum may interfere with fetal head descent
True
70
May develop at any depth of the vagina but most commonly on the lower third
Transverse Vaginal Septum
71
True or false? | Transverse vaginal septum may or may not be perforated therefore there may be obstruction and infertility
True
72
How is uterine malformation being discovered?
``` Routine pelvic exam CS Manual exploration Tubal sterilization Infertility evaluation ```
73
What feature is most often indicative of malformed uterus by abdominal palpation?
Fundal notching
74
Most common finding in uterine malformation
Arcuate uterus ``` followed by: (descending order) Septate Bicornuate Didelphic Unicornuate ```
75
Diagnostic options for uterine malformation
Ultrasound Hysterosalpingography- contraindicated in Pregnancy MRI Laparoscopy or Hysteroscopy
76
Diagnostic modality that improves delineation of the endometrium and internal uterine morphology, but only with a patent endometrial cavity. Contraindicated in pregnancy.
Saline Infusion Sonography (SIS)
77
What is the obstetrical significance of uterine malformation?
``` Miscarriage Ectopic pregnancy Rudimentary horn pregnancy Preterm delivery Fetal growth restriction Abnormal fetal lie Uterine dysfunction Uterine rupture ```
78
Develops due to an underdeveloped or rudimentary uterine horn may be absent
Class II/ Unicornuate Uterus
79
``` 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 ```
False (Class 2 not class 1)
80
How can you tell if it is a communicating or non-communicating horn in Class II Mullerian abnormality?
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
Superior diagnostic tool for unicornuate uterus
MRI | 3D ultrasound only increase accuracy
82
What is the obstetrical significance of unicornuate uterus?
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
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
Class III/ Uterus Didelphys
84
What is OHVIRA
Obstructed hemivagina and ipsilateral renal agenesis
85
True or false? | Class II may be isolated or with ipsilateral renal agenesis (OHVIRA or Herlyn Werner-Wunderlich Syndrome)
False (Class 3 not 2)
86
Complications of Class III
``` Miscarriage Preterm delivery Fetal growth restriction Breech presentation Increased Cesarean delivery rate (BUT LESS FREQUENT THAN CLASS 2) ```
87
PE finding in Class 3
Suspected with identification of a longitudinal septum and 2 cervices
88
Management for Class 3
Metroplasty (But some women deliver successfully → no surgical management is performed)
89
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)
Metroplasty
90
From a fusion anomaly | Forming 2 hemiuteri with a central myometrium running partially or completely to the cervix
Class 4/ Bicornuate Uterus
91
What is the obstetrical significance of bicornuate uterus?
Diminished fertility Abortion when the embryo implants on the septum Preterm deliveries Malpresentation
92
Management of Class 5
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
Why is there an increased risk of miscarriage in Class 4 and 5?
Due to the abundant muscle tissue in the septum or the intervening myometrium
94
Differential diagnosis for Class 4
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
Resorption defect which results into a uterine septum involving the cervix, vagina, and uterus
Class 5/ Septated uterus
96
What is the obstetrical significance of septated uterus?
Diminished fertility Abortion when the embryo implants on the septum Preterm deliveries Malpresentation
97
This malformation is only a mild deviation from the normally developed uterus
Class 6/ Arcuate uterus
98
Management for Class 6
Transabdominal cerclage Metroplasty No need for surgery for nonobstructive defects
99
Management of Class 6 for those with uterine anomalies, repetitive pregnancy loss, partial cervical atresia or hypoplasia may benefit
Transabdominal cerclage
100
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
Class 7/ Diethylstilbestrol-Induced Abnormalities
101
Structural abnormalities in Class 7
Transverse septa Circumferential ridges involving the vagina and cervix Cervical collars
102
Reproductive performance in Class 7
Impaired conception rates Higher rates of ectopic pregnancy Preterm delivery
103
Gynecologic effects in Class 7
Early menopause Risk for Cervical Intraepithelial Neoplasia (CIN) Breast cancer
104
Cervical abnormalities in Class 7
Partial or complete agenesis Duplication Longitudinal septa
105
Most important obstetrical relevance of Class 7
Complete cervical atresia is incompatible with conception
106
Vaginal abnormalities in Class 7
Double Vagina and Double introitus
107
Double introitus in Class 7 appears as?
"Double barreled Shotgun"