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2nd Year GYN > Chapter 28: Pediatric Congenital Anomalies of Female Pelvis > Flashcards

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
Q

The blood supply to the ovary is from the

A

ovarian artery that originates directly from the aorta and from the uterine artery which supplies an adnexal branch to each ovary

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26
Q

The diagnosis and classification of a congenital anomaly of the uterine cavity requires

A

the visualization of the uterine cavity or cavities and the serosal margin(s)

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27
Q

Congenital uterine abnormalities occur in appoximately ___-___% of females

A

0.5-1%

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28
Q

Congenital uterine abnormalities are associated with an increased incidence of

A

abortion and other obstetric complications later in life

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29
Q

The uterus and upper third of the vagina are derived from the

A

embryonic mullerian ducts

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30
Q

The mullerian ducts that form the uterus and upper third of the vagina must elongate, fuse, and form lumens between what weeks?

A

7th and 12th

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31
Q

When the proper sequence fails to occur, what three types of mullerian abnormalities occur:

A
  • improper fusion
  • incomplete development of one side
  • incomplete vaginal canalization
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32
Q

Describe Class 1 of mullerian anomalies:

A

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

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33
Q

Class 1 of mullerian anomalies produces what?

A
  • transverse vaginal septum OR

- vaginal atresia

34
Q

Vaginal atresia is diagnosed by the development of

A
  • hydrocolpos
  • hydrometrocolpos
  • hematometrocolpos
35
Q

What is Class 2 of mullerian anomalies?

A

unicornuate uterus

36
Q

What is unicornuate uterus related to?

A

infertility and pregnancy loss

37
Q

How does unicornuate uterus appear sonographically?

A

long and slender (cigar shaped) and deviated to one side

38
Q

What is typically apparent with unicornuate uterus on the contralateral side?

A

renal agenesis

39
Q

What is Class 3 of mullerian anomalies?

A

Uterus Didelphys (complete duplication of uterus, cervix, and vagina.)

40
Q

T/F: Class 3 (uterus didelphys) is usually associated with fertility problems and requires treatment?

A

False

41
Q

How does Class 3 (uterus didelphys) appear sonographically?

A

two endometrial echo complexes

42
Q

What is Class 4 of mullerian anomalies?

A

bicornuate uterus (duplication of the uterus with a common cervix, this bilobed uterine cavity has wide-spaced cavities)

43
Q

T/F: the bicornuate uterus is related to low fertitility

A

false

44
Q

When is a bicornuate uterus often detected?

A

in early pregnancy, when a gestational sac is present in one horn and the decidual reaction in the other

45
Q

The bicornuate uterus is best ID’d on the

A

transverse sonogram through the superior portion of the two horns of the uterus

46
Q

What is Class V. of the mullerian anomalies?

A

Septate Uterus (two uterine cavities are seen closely spaced, with one fundus and sometimes two cervical canals or a vaginal septum

47
Q

What condition has the highest incidence of fertility problems?

A

Class V, Septate Uterus

48
Q

What part of the uterus is useful in distinguishing between the septate and bicornuate uterus?

A

external contour

49
Q

What is Class Vl of mullerian anomalies?

A

Exposure to DES in utero (diethyistilbestrol is a synthetic preparation drug that possesses estrogenic properties)

50
Q

How many Americans received the drug DES?

A

5-10 million

51
Q

What was DES found to cause?

A

vaginal malignancies in the daughters of mothers who were given the drug

52
Q

How does the uterus of Class Vl look?

A

normal in size and shape externally, the cavity is T-shaped with an irregular contour (condition difficult to diagnose with US)

53
Q

What is ambiguos genitalia?

A

embryo has the potential to develop as a male or female, errors in sexual dev. result in ambiguous genitatlia or hermaphroditism

54
Q

True hermaphrodites have both

A

ovarian and testicular tissue

55
Q

Most hermaphrodites have what kind of karyotypes?

A

46XX (true), 46XX/46XY (mosaics)

56
Q

Female pseudohermaphrodites have what karyotype?

A

46XX

57
Q

Most common cause of pseudohermaphroditism

A

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
Q

How might the external genitalia be masculinized with pseudohermaphroditism?

A

androgenic hormones, which reach it via the placenta from the maternal circulation

59
Q

What is true precocious puberty?

A

always isosexual and involves the development of secondary sexual characteristics and an increase in the size and activity of the gonads

60
Q

Describe precocious pseudopuberty

A

involves the maturation of secondary sexual characteristics but NOT the gonad

61
Q

The neonatal ovaries are similar in function and anatomy to

A

the pubertal and adult ovaries

62
Q

Factors that contribute to the follicular growth in utero include

A

FSH, maternal estrogens, and hCG

63
Q

T/F: Most fetal ovarian cysts resolve spontaneously but CAN persist into the neonatal period

A

True

64
Q

Small follicular cysts of what size are a common and normal finding in neonatal ovaries?

A

3-7mm

65
Q

There is a higher incidence of larger ovarian cysts in infants of mothers with

A

toxemia, diabetes, and Rh isoimmunization

66
Q

Most common primary complications of ovarian cysts

A

hemorrhage and salpingotorsion

67
Q

How might a patient with ovarian torsion present?

A

with pain, vomiting, fever, abdominal distention, leukocytosis and peritonitis

68
Q

Ovarian hemorrhage in the pediatric ovary may result from

A

torsion or spontaneously in a nontwisted cyst

69
Q

If an ovarian cyst is large, complications may include

A

bowel obstruction, thorax compression with pulmonary hypoplasia, urinary tract obstruction, or incarceration with an inguinal hernia

70
Q

Differential considerations of ovarian cysts pediatrically include

A

hydrometrocolpos, cystic meconium peritonitis, urachal cysts, and anterior meningocele

71
Q

What are very rare in the neonate?

A

neoplastic lesions such as cystadenoma, cystic teratomas, and granulosa cell tumors

72
Q

Most ovarian torsions occur when?

A

within the first two decades of life

73
Q

Torsion of the ovary occurs more commonly when what are present?

A

ovarian cysts or tumors

74
Q

Torsion results in compromise of

A

arterial and venous flow

75
Q

Clinical symptoms of ovarian torsion include

A

sever onset of abdominal pain

76
Q

T/F: Ovarian teratomas (tumors) are uncommon in the neonate and adolescent?

A

True

77
Q

When found, germ cell tumors account for ___% of ovarian neoplasms in patients under 20 years old

A

60%

78
Q

What is the most common pediatric germ cell tumor?

A

benign mature teratoma or dermoid cyst

79
Q

Clinically, most patients with a dermoid tumor are

A

asymptomatic, but when the tumor become large, abdominal pain or distention may occur

80
Q

Most frequent complication of a teratoma is

A

torsion (pain can mimic appendicitis)

2nd Year GYN (9 decks)

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