Chapter 28: Pediatric Congenital Anomalies of Female Pelvis Flashcards

1
Q

What are the first parts of the genital system to develop?

A

the gonads

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2
Q

T/F: all embryos have identical pairs of genital ducts in the beginning?

A

TRUE!!

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3
Q

What determines the genetic sex of an embryo?

A

sperm

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4
Q

When do indications of maleness or femaleness show up?

A

9th gestational week

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5
Q

What ducts form most of the female genital tract?

A

paramesonephric (mullerian) ducts

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6
Q

The cranial parts of the paramesonephric ducts form:

A

uterine tubes

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7
Q

The caudal parts of the paramesonephric ducts fuse to form:

A

the uterus and part of the vagina

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8
Q

Early in development, both sexes appear similar until:

A

the 9th week

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9
Q

External sexual organs are fully developed by the ___ week

A

12th

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10
Q

The urogenital folds become the

A

labia minora

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11
Q

The labioscrotal swellings become the

A

labia majora

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12
Q

The phallus becomes the

A

clitoris

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13
Q

The endovaginal approach to pelvic scanning is reserved for

A

mature, sexually active teenage girls

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14
Q

Describe the newborn uterus?

A

prominent and thickened with a brightly echogenic endometrial lining caused by hormonal stimulation received in utero

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15
Q

What stimulate the initial size of the uterine cavity after birth?

A

maternal hormones

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16
Q

At 2-3 months the uterus

A

regresses to a prepubertal size and configuration, and the endometrial stripe echoes are not visualized

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17
Q

The uterus increases in size after age

A

7, with the greatest increase in size occurring after the onset of puberty, when the fundus becomes larger than the cervix

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18
Q

The blood supply to the uterus is

A

by bilateral uterine arteries, which are branches of the internal iliac arteries

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19
Q

T/F: the evaluation of the ovary in the young patient can be a challenge?

A

True, depending on location, size, and age of pt.

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20
Q

In the neonatal pt, where can the ovary be found?

A

anywhere between the lower pole of the kidneys and the true pelvis

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21
Q

The mean ovarian volume is stable up to ___ years of age

A

5

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22
Q

Ovarian volume gradually _____ until _____ is reached

A

increases, puberty

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23
Q

Usually ovarian texture is

A

homogenous, though small follicles may be seen

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24
Q

The appearance of the ovary in the neonatal period is ______

A

heterogeneous secondary to tiny cysts

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25
The blood supply to the ovary is from the
ovarian artery that originates directly from the aorta and from the uterine artery which supplies an adnexal branch to each ovary
26
The diagnosis and classification of a congenital anomaly of the uterine cavity requires
the visualization of the uterine cavity or cavities and the serosal margin(s)
27
Congenital uterine abnormalities occur in appoximately ___-___% of females
0.5-1%
28
Congenital uterine abnormalities are associated with an increased incidence of
abortion and other obstetric complications later in life
29
The uterus and upper third of the vagina are derived from the
embryonic mullerian ducts
30
The mullerian ducts that form the uterus and upper third of the vagina must elongate, fuse, and form lumens between what weeks?
7th and 12th
31
When the proper sequence fails to occur, what three types of mullerian abnormalities occur:
- improper fusion - incomplete development of one side - incomplete vaginal canalization
32
Describe Class 1 of mullerian anomalies:
segmental mullerian agenesis or incomplete vaginal canalization is suspected with a young girl reaches puberty without menstruation. produces a transverse vaginal septum or vaginal atresia
33
Class 1 of mullerian anomalies produces what?
- transverse vaginal septum OR | - vaginal atresia
34
Vaginal atresia is diagnosed by the development of
- hydrocolpos - hydrometrocolpos - hematometrocolpos
35
What is Class 2 of mullerian anomalies?
unicornuate uterus
36
What is unicornuate uterus related to?
infertility and pregnancy loss
37
How does unicornuate uterus appear sonographically?
long and slender (cigar shaped) and deviated to one side
38
What is typically apparent with unicornuate uterus on the contralateral side?
renal agenesis
39
What is Class 3 of mullerian anomalies?
Uterus Didelphys (complete duplication of uterus, cervix, and vagina.)
40
T/F: Class 3 (uterus didelphys) is usually associated with fertility problems and requires treatment?
False
41
How does Class 3 (uterus didelphys) appear sonographically?
two endometrial echo complexes
42
What is Class 4 of mullerian anomalies?
bicornuate uterus (duplication of the uterus with a common cervix, this bilobed uterine cavity has wide-spaced cavities)
43
T/F: the bicornuate uterus is related to low fertitility
false
44
When is a bicornuate uterus often detected?
in early pregnancy, when a gestational sac is present in one horn and the decidual reaction in the other
45
The bicornuate uterus is best ID'd on the
transverse sonogram through the superior portion of the two horns of the uterus
46
What is Class V. of the mullerian anomalies?
Septate Uterus (two uterine cavities are seen closely spaced, with one fundus and sometimes two cervical canals or a vaginal septum
47
What condition has the highest incidence of fertility problems?
Class V, Septate Uterus
48
What part of the uterus is useful in distinguishing between the septate and bicornuate uterus?
external contour
49
What is Class Vl of mullerian anomalies?
Exposure to DES in utero (diethyistilbestrol is a synthetic preparation drug that possesses estrogenic properties)
50
How many Americans received the drug DES?
5-10 million
51
What was DES found to cause?
vaginal malignancies in the daughters of mothers who were given the drug
52
How does the uterus of Class Vl look?
normal in size and shape externally, the cavity is T-shaped with an irregular contour (condition difficult to diagnose with US)
53
What is ambiguos genitalia?
embryo has the potential to develop as a male or female, errors in sexual dev. result in ambiguous genitatlia or hermaphroditism
54
True hermaphrodites have both
ovarian and testicular tissue
55
Most hermaphrodites have what kind of karyotypes?
46XX (true), 46XX/46XY (mosaics)
56
Female pseudohermaphrodites have what karyotype?
46XX
57
Most common cause of pseudohermaphroditism
congential virilizing adrenal hyperplasia (increased production of androgens leads to masculinization of the external genitalia (enlarged clitoris, abnormalities of the urogenital sinus, and partial fusion of the labia majoa)
58
How might the external genitalia be masculinized with pseudohermaphroditism?
androgenic hormones, which reach it via the placenta from the maternal circulation
59
What is true precocious puberty?
always isosexual and involves the development of secondary sexual characteristics and an increase in the size and activity of the gonads
60
Describe precocious pseudopuberty
involves the maturation of secondary sexual characteristics but NOT the gonad
61
The neonatal ovaries are similar in function and anatomy to
the pubertal and adult ovaries
62
Factors that contribute to the follicular growth in utero include
FSH, maternal estrogens, and hCG
63
T/F: Most fetal ovarian cysts resolve spontaneously but CAN persist into the neonatal period
True
64
Small follicular cysts of what size are a common and normal finding in neonatal ovaries?
3-7mm
65
There is a higher incidence of larger ovarian cysts in infants of mothers with
toxemia, diabetes, and Rh isoimmunization
66
Most common primary complications of ovarian cysts
hemorrhage and salpingotorsion
67
How might a patient with ovarian torsion present?
with pain, vomiting, fever, abdominal distention, leukocytosis and peritonitis
68
Ovarian hemorrhage in the pediatric ovary may result from
torsion or spontaneously in a nontwisted cyst
69
If an ovarian cyst is large, complications may include
bowel obstruction, thorax compression with pulmonary hypoplasia, urinary tract obstruction, or incarceration with an inguinal hernia
70
Differential considerations of ovarian cysts pediatrically include
hydrometrocolpos, cystic meconium peritonitis, urachal cysts, and anterior meningocele
71
What are very rare in the neonate?
neoplastic lesions such as cystadenoma, cystic teratomas, and granulosa cell tumors
72
Most ovarian torsions occur when?
within the first two decades of life
73
Torsion of the ovary occurs more commonly when what are present?
ovarian cysts or tumors
74
Torsion results in compromise of
arterial and venous flow
75
Clinical symptoms of ovarian torsion include
sever onset of abdominal pain
76
T/F: Ovarian teratomas (tumors) are uncommon in the neonate and adolescent?
True
77
When found, germ cell tumors account for ___% of ovarian neoplasms in patients under 20 years old
60%
78
What is the most common pediatric germ cell tumor?
benign mature teratoma or dermoid cyst
79
Clinically, most patients with a dermoid tumor are
asymptomatic, but when the tumor become large, abdominal pain or distention may occur
80
Most frequent complication of a teratoma is
torsion (pain can mimic appendicitis)