Chapter 29 - Fetal Genitourinary System Flashcards
A membrane that is present during early embryonic development that contributes to urinary bladder formation and development
Allantois
A way in which a disorder or trait can be inherited by a fetus; at least one of the parents has to be the carrier of the gene for the disease
Autosomal dominant
A birth defect in which the sex of the fetus cannot be determine
Ambigious Genitalia
An inherited disease that results in the development of renal, liver, and pancreatic cysts later on in life; also referred to as adult polycystic kidney disease
Autosomal Dominant Polycystic Kidney Disease
A way in which a disorder or trait can be inherited by a fetus; both parents must be carriers of the gene for the diseas
Autosomal Recessive
An inherited renal disease that results in bilateral enlargement of the fetal kidneys and microscopic renal cysts; also referred to as infantile polycystic kidney disease
Autosomal Recessive Polycystic Kidney Disease
A birth defect in which the bladder is located outside of the abdomen
Bladder Exstrophy
Dilation of the calices
Caliectasis
Enlargement of the clitoris
Clitromegaly
The embryonic structure that develops into the normal rectum and urogenital sinus
CloacA
Q
Birth defect consisting of omphalocele, bladder exstrophy, imperforate anus, and spina bifida; also referred to as OEIS complex
Cloacal Exstrophy
Enlargement of an organ secondary to an increased workload; often seen when part of the has been destroyed or when there is an absence or decreased function of paired organs
Compensatory Hypertrophy
The attachment of the lower poles of the kidneys by a band of renal tissue that crosses the midline of the abdomen
Horseshoe kidneys
Q
A fluid collection within the scrotum between the two layers of the tunica vaginalis
Hydrocele
The dilation of the renal collecting system resulting from the obstruction of the flow of urine from the kidneys to the bladder
Hydronephrosis
Q
Distention of the ureter with fluid because of an obstruction
Hydrouterter
Abnormal ventral curvature of the penis as a result of a shortened urethra that exits on the ventral penile shaft
Hypospadias
The sonographic appearance of a dilated fetal bladder and urethra in the presence of bladder outlet obstruction
“Keyhole sign”
The sonographic appearance of the adrenal gland in a parallel position within the abdomen as a result of renal agenesis
“Lying down Sign”
Fetal syndrome associated with microcephaly, occipital encephalocele, polydactyly, and polycystic kidneys
Meckel-Gruber Syndrome
An abnormally enlarged urinary bladder
Megacystitis
An enlarged ureter; can be congenital or acquired
Megaureter
The most common solid fetal renal mass
Mesoblastic Nephroma
An abnormally small penis
Micropenis
A fetal renal disease thought to be caused by an early renal obstruction; leads to the development of multiple noncommunicating cysts of varying sizes in the renal fossa
Multicystic Displastic Kidney Disease
Malignant tumor that can occur within the adrenal gland anywhere within the sympathetic nervous system
Nueroblastomac
A fetal disorder caused by an early renal obstruction; leads to small and echogenic kidneys that have cysts located along their margins
Obstructive Cystic Dysplasia
Q
Dilation of the renal pelvis; may also be referred to as pyelectasis
Pelviectasis
The region between the external genitalia and the anus
Perineum
Irregular thin membranes of tissue located within the male posterior urethra that does not allow urine to exit the urethra
Posterior ureteral valves
Facial features seen with severe oligohydroamnios, including low set ears, flattened nose wrinkled skin, and micrognathia
Potter Facies
Physical features of a fetus as a result of oligohydroamnios; characterized by bilateral renal agenesis, abnormal facies, pulmonary hypoplasia, and limb abnormalities; also referred to a Potter sequence
Potter syndrome
Syndrome that is a consequence of the abdominal wall musculature being stretched by the extremely enlarged urinary bladder
Prune Belly syndrome
Underdevelopment of the lungs
Pulmonary Hypoplasia
Failure of the kidney to develop; may be unilateral or bilateral
Renal agencies
The part of the collecting system that encompasses the apex of the renal pyramids
Renal Calices
Refers to an abnormal location of the kidneys
Renal Ectopia
The region where the kidney is located the abdomen
Renal Fossa
Measurement of the fetal renal pelvis; this dimension is obtained from the transverse kidney plane
Renal pelvic Diameter
Canal connecting the fetal bladder with the allantois; normally closes during fetal development and becomes a fibrous cord
Urachus
Q
An abnormailty in which the distal ureter projects into the urinary bladder
Ureterocele
An obstruction located in the region where the ureter meets the renal pelvis
Ureteropelvic Junction Obstruction
An obstruction located in the region where the ureter meets the bladder
URETEROVESICULAR JUNCTION OBSTRUCTION
Q
The congenital absence of the urethra
URETHRAL ATRESIA
the fetal kidneys develop within the pelvis and ascend into their normal position by ____ weeks
9
ectopic kidney found in the pelvis
PELVIC KIDNEY
kidneys that are attached at their lower poles
HORSESHOE KIDNEY
the ________ is located between the apex of the bladder and the umbilicus
URACHUS
the testicles move down into the scrotum during the ____ month of gestation
7TH MONTH
the fetal urinary bladder normally fills and empties once in every:
30-45 MINS
VACTERL stands for
vertebral anomalies, anal atresia, cardiac anomalies, tracheoesophageal fistula or esophageal atresia, renal anomalies, and limb anomalies
(association of 3 or more anomalies)
VACTERL may also be referred to as:
VATER sequence, VATER, or VACTEL syndrome
_________ abnormalities are the most frequent cause of oligohydramnios. if oligohydramnios is discovered, an analysis of the fetal _______ is warranted
RENAL
URINARY TRACT
during fetal development, around ___ weeks, the fetal kidneys begin to produce urine
9
Urine comprises the greater part of amniotic fluid after ___ weeks?
14
the most worrisome consequence of oligohydramnios is _________ or underdevelopment of the lungs
PULMONARY HYPOPLASIA
when the kidney is absent in the abdomen, the adrenal gland can be noted in a parallel, flattened position, a sonographic finding known as the:
“lying down” adrenal sign
bilateral renal agenesis, also known as ______ is a fatal condition.
POTTER SYNDROME
bilateral renal agenesis may be seen in conjunction with ________ and various cardiovascular malformations
SIRENOMELIA
unilateral renal agenesis is ______ common than bilateral renal agenesis. there is an average amount of amniotic fluid and the prognosis is good
MORE
**(always check fetal pelvis for a pelvic kidney)
features of potter syndrome: (5)
-bilateral renal agenesis
- abnormal facial features (potter facies)
- pulmonary hypoplasia
- limb abnormalities
- intrauterine growth restriction
in the presence of unilateral renal agenesis, the contralateral kidney will enlarge, a condition known as:
compensatory hypertrophy
sonographic findings of bilateral renal agenesis: (5)
- absent kidneys
- absent urinary bladder
- severe oligohydramnios or anhydramnios
- bilateral lying down adrenal signs
- undetectable renal artery branches w/ color
sonographic findings of unilateral renal agenesis: (6)
- absent kidney
- hypertrophy of the contralateral kidney
- visible urinary bladder
- normal amniotic fluid volume
- unilateral laying down adrenal sign
- undetectable renal artery branch with color doppler (unilateral)
in order for an autosomal recessive disease to be passed to the fetus,…
both parents must be carriers of the disease
in order to an autosomal dominant disease to be passed to the fetus,.
only one of the parents has to be the carrier
autosomal dominant disorders tend to be _____ severe than recessive disorders
LESS
__ would be the most likely cause of enlarged, echogenic kidneys noted in utero
ARPKD
(autosomal recessive polycystic kidney disease)
one condition associated with ARPKD is ________, which is a fatal disorder that includes renal cystic disease, occipital encephalocele, and polydactyly
MECKEL GRUBER SYNDROME
the cysts with ARPKD are:
MICROSCOPIC
sonographic findings of autosomal recessive (infantile) polycystic kidney disease): (3)
- bilateral, enlarged echogenic kidneys
- absent urinary bladder
- oligohydramnios
_________ does not typically manifest until approximately the fourth or fifth decade of life
ADPKD
sonographic findings of autosomal dominant (adult) polycystic kidney disease: ADPKD (4)
- normal appearing or bilateral, enlarged echogenic kidneys
- visible urinary bladder
- normal amniotic fluid volume
- cysts do not manifest until 4th/5th decade of life
thought to be caused by an early, first trimester obstruction of the ureter:
MCDK
MCDK disease is fatal if ________
BILATERAL
sonographic findings of bilateral multicystic dysplastic renal disease MCDK: (3)
- bilateral, smooth walled, noncommunicating cysts of varying sizes located within the renal fossae
- absent urinary bladder
- oligohydramnios
sonographic findings of unilateral multicystic dysplastic renal disease: (4)
- unilateral, smooth-walled, noncommunicating cysts of the varying sizes located within the renal fossa
- hypertrophy of the contralateral kidney
- visible urinary bladder
- normal amniotic fluiD VOULME
___________, like MCDK disease is caused by an early renal obstruction
obstructive cystic dysplasia
a ________, or a severe bladder outlet obstruction, early in gestation, can lead to bilateral obstructive cystic dysplasia, in which case oligohydramnios will be present
URETROCELE
unilateral obstructive cystic dysplasia is most often caused by:
pelviureteral junction or vesicoureteral junction obstruction
bilateral cystic dysplasia may be associated with:
bilateral cystic dysplasia may be associated with:
sonographic findings of bilateral obstructive cystic dysplasia: (5)
small, echogenic kidneys
2. peripheral renal cysts
3. bilateral hydronephrosis
4. thick-walled urinary bladder
5. oligohydramnios
________ is the most common fetal abnormality noted during an obstetric sonogram
HYDRONEPHROSIS
enlargement of the bladder is called _________ whereas dilation of the ureter may be referred tp as ________ or _________
megacystis
megaureter or hydroureter
the measurement of the renal pelvis is made in the anteroposterior plane and should not exceed ___ before 20 weeks or ___ after 20 weeks gestation
7mm
10mm
the most common cause of hydronephrosis in the neonate and the most common form of fetal renal obstruction
UPJ OBSTRUCTION
UPJ is located at:
LOCATED AT THE RENAL PELVIS AND THE URETER
that cause of UPJ obstruction may be due to:
irregular development of the smooth muscle in the area of the UPJ
fetal pyelectasis (dilation of renal pelvis) can be s sonographic marker for:
DOWN SYNDROME
sonographic findings of ureteropelvic junction obstruction: (3)
- hydronephrosis
- normal uterus (nonvisual)
- normal bladder
Describes the condition in which there is a blockage of the flow of urine out of the urinary bladder
bladder outlet obstruction
_____ are a common cause of bladder outlet obstructions in male fetuses
posterior urethral valves
the “____” sing is seen when there is dilation of the urinary bladder & the posterior urethra
Keyhole
sonographic findings of posterior urethral valves (5)
- keyhole sign
bilateral hydrouter - bilateral hydronephrosis
- oligohydramnios
- thickened bladder wall
Caused by megacystis, a massively dilated urinary, seen mostly in make fetuses which leads to bladder outlet obstruction
prune belly syndrome
prune belly syndrome describes the result of
the abdominal wall musculature being stretched by the extremely enlarged urinary bladder
sonographic findings of prune belly syndrome (4)
- dilated bladder and possibly urethra (keyhole)
- absent abdominal musculature
- undescended testis
- megacystis & hydronephrosis
the obstruction that has the least common cause of hydronephrosis in the fetus:
UVJ obstruction
unilateral UVJ obstructions lead to:
bilateral UVJ obtructions lead to:
normal amounts of amniotic fluid
oligohydramnios
sonographic findings of ureterovesicular junction obstruction: (4)
- hydronephrosis
- dilated ureter
- normal bladder
- normal amniotic fluid (if unilateral)
the ______ is the embryonic structure that develops into the rectum and urogenital sinus. can result in the combination of the urinary, genitals, and intestinal tract, emptying on the perineum
cloaca
with _______, there is an omphalocele, bladder exstrophy, imperforate anus, and spina bifida
cloacal exstrophy aka OEIS complex
sonography findings of bladder exstrophy: (3)
.1. lower abdominal wall mass inferior to the umbilicus
2. absent urinary bladder
3. normal kidneys
the most common solid fetal renal mass is the:
mesoblastic nephroma (hamartoma of the kidney)
sonographic findings of the mesoblastic nephroma: (1)
- solid, homogenous mass within the renal fossa and may completely replace the kidney
Sperm carries what chromosome?
Ovary always contributes what chromsome?
X or a Y
X
an X chromosome from the sperm will yield ____, which is female offspring
XX
an X chromosome from the sperm will yield ____, which is female offspring
XX
the combination of a Y chromosome will yield XY, which is:
MALE OFFSPRING
abnormal ventral curvature of the penis as a result of a shortened urethra that exits on the ventral penile shaft:
HYPOSPADIAS
the most common female finding is ________
CLITORMEGALY
the most common malignant abdominal mass in neonates is the:
NEUROBLASTOMA
uring an 18 week sonogram, multiple cysts of varying sizes are noted within the renal fossa of a male fetus. the other kidney appears to be normal. which of the following would be an associated finding?
a. megacystis
b. ovarian dysgenesis
c. hypospadias
d. normal amniotic fluid level
d
the most common malignant adrenal pediatric tumor is the:
a. nephromblastoma
b. pheochromocytoma
c. hepatoblastoma
d. neuroblastoma
d
the “keyhole” sign would be seen in all of the following situations except:
a. urethral atresia
b. prune belly syndrome
c. autosomal dominant polycystic renal disease
d. posterior urethral valves
c
what is the most common fetal abnormality noted during an obstetric sonogram?
a. anencephaly
b. spina bifida
c. cleft lip
d. hydronephrosis
d
the “lying down” adrenal sign would be seen in all of the following situations except:
a. unilateral renal agenesis
b. bilateral renal agenesis
c. potter syndrome
d. pyelectesis
d
the birth defect in which the sex of the fetus cannot be determined defines:
a. renal agenesis
b. ovarian dysgenesis
c. clitorimegaly
d. ambiguous genitalia
d
what measurement should the renal pelvis not exceed prior to 20 weeks gestation?
a. 2 mm
b. 10 mm
c. 7 mm
d. 1.2 cm
c
cloacal exstrophy is associated with all of the following except:
a. omphalocele
b. spina bifida
c. encephalocele
d. imperforate anus
c
the renal cystic disease that results in the development of cysts late in adulthood is:
a. multicystic dysplastic renal disease
b. autosomal dominant renal disease
c. autosomal recessive polycystic disease
d. obstructive cystic dysplasia
b
that is the most common cause of hydronephrosis in the neonate and the most common form of fetal renal obstruction?
a. UVJ obstruction
b. UPJ obstruction
c. vesicoureteral reflux
d. urethral atresia
b
bladder exstrophy describes:
a. absence of the cloaca
b. protrusion of the bladder
c. external position of the bladder
d. enlargement of the bladder
c
which of the following would result in compensatory hypertrophy?
a. unilateral renal agenesis
b. bilateral renal agenesis
c. pelvic kidney
d. horseshoe kidneys
a
which of the following would cause a bladder outlet obstruction?
a. posterior urethral valves
b. fetal ovarian cyst
c. pelviectasis
d. pelvocaliectasos
a
which of the following is associated with enlarged echogenic kidneys and microscopic renal cysts?
a. MCDK disease
b. obstructive cystic dysplasia
c. hydronephrotic syndrome
d. ARPKD
d
the “lying down” adrenal sign describes the sonographic findings of:
a. enlarged bladder and urethra
b. renal agenesis
c. MCDK disease
d. posterior urethral valves
b
the “I” in OEIS complex stands for:
a. imperforate anus
b. ilial dysfunction
c. irregular bladder enlargement
d. iniencephaly
a
another name for pelvocaliectasis is:
a. caliectasis
b. hydrocele
c. hydronephrosis
d. pyonephrosis
c
which of the following best describes hypospadias?
a. OEIS complex in the presence of a hydrocele
b. the chronic obstruction of the renal pelvis and urethra
c. the underdevelopment of the scrotum in the presence of a hydrocele
d. an abnormal ventral curvature of the penis
d
the “keyhole” sign describes the sonographic findings of a(n):
a. enlarged bladder and dilated urethra
b. bilateral renal agenesis
c. unilateral renal agenesis
d. dilation of the renal pelvis and proximal ureter
a
what is the term for enlargement of the urinary bladder?
a. posterior urethral valves
b. urethral atresia
c. prune belly syndrome
d. megacystis
d
Numerous noncommunicating anechoic masses are noted within the left renal fossa of a fetus at 20 weeks gestation. What is the most likely etiology of these masses?
a. ARPKD
b. ADPKD
c. MCDK disease
d. hydronephrosis
c
fluid surrounding the fetal testicle is referred to as:
a. hydroureter
b. hydronephrosis
c. hydrocele
d. hydroscrotum
c
fusion of the lower poles of the kidneys describes:
a. renal agenesis
b. horseshoe kidneys
c. moiety
d. meckel-gruber syndrome
b
the syndrome associated with an occipital cephalocele, cystic renal disease, and polydactly is:
a. meckel-gruber syndrome
b. potter syndrome
c. VACTERL association
d. sirenomelia syndrome
a
which of the following is not a component of prune belly syndrome?
a. megacystis
b. undescended testis
c. dilated urinary bladder and urethra
d. abdominal muscle hypertrophy
d
OEIS complex is also referred to as:
a. bladder exstrophy
b. omphalocele
c. potter syndrome
d. cloacal exstrophy
d
obstruction at the level of the UPJ would lead to dilation of the:
a. renal pelvis and bladder
b. bladder and ureter
c. ureter and renal pelvis
d. renal pelvis and calices
d
the most common location of an ectopic kidney is within the:
a. lower abdomen
b. pelvis
c. chest
d. contralateral quadrant
b
pyelectasis refers to:
a. enlargement of the urinary bladder, ureter, and renal calices
b. dilation of the ureter
c. dilation of the renal pelvis
d. enlargement of the ureter only
c
prune belly syndrome is caused by:
a. an enlarged bladder
b. unilateral renal agenesis
c. bilateral renal agenesis
d. hypospadias
a
all of the following would be associated with oligohydramnios except:
a. bilateral MCDK disease
b. unilateral renal agenesis
c. bilateral renal agenesis
d. ARPKD
b
what is the most common fetal renal tumor?
a. neuroblastoma
b. nephroblastoma
c. mesoblastic nephroma
d. wilms tumor
c
The type of renal cystic disease associated with adult liver and pancreatic cysts is:
a. MCDK
b. ARPKD
c. ADPKD
d. VATER
c
having more than the normal number of digits:
a. polydactyly
b. clinodactyly
c. multidigitopia
d. sirenomelia
a
crytorchidism describes:
a. bilateral pelvic kidneys
b. urethral atresia
c. undescended testicles
d. ovarian dysgenesis
c
an obstruction at the ureterovesicular junction would lead to dilation of the:
a. bladder and urethra
b. bladder, urethra, and ureters
c. bladder, urethra, ureters, and renal collecting system
d. ureter and renal collecting system
d
Before 9 weeks, the fetal kidneys are located within the:
a. renal fossae
b. pelvis
c. chest
d. umbilical cord
b
which of the following is the most common renal anomaly?
a. horseshoe kidneys
b. pelvic kidneys
c. renal agenesis
d. duplex collecting system
d
failure of the kidneys to form is called:
a. hydronephrosis
b. renal dysplasia
c. renal agenesis
d. renal ectopia
c
which of the following would be the most likely cause of bilateral, enlarged echogenic fetal kidneys and oligohydramnios?
a. ARPKD
b. MCKD
c. renal cystic dysplasia
d. ADPKD
a