Penny Book Chapter 29: The Fetal Genitourinary System
moiety
(renal) refers to a separate collecting system in the upper pole or the lower pole of the kidney in a duplex collecting system
bladder exstrophy
a birth defect in which the bladder is located outside of the abdomen
ambigous genitalia
a birth defect in which the sex of the fetus cannot be determined
pelvic kidney
a kidney located within the pelvis
hypospadias
abnormal ventral curvature of the penis as a result of a shortened urethra that exits on the ventral penile shaft
ureterovesicula junction obstruction
an obstruction located in the region where the ureter meets the bladder
The urachus is located
between the apex of the bladder and the umbilicus.
cloacal exstrophy
birth defect consisting of omphalocele, bladder exstrophy, imperforate anus, and spina bifida; also referred to as OEIS complex
In order for an autosomal recessive disease to be passed to the fetus,
both parents must be carriers of the disease
urachus
canal connecting the fetal bladder with the allantois; normally closes during fetal development and becomes a fibrous cord
pelviectasis
dilation of the renal pelvis; may also be referred to as pyelectasis
hydroureter
distension of the ureter with fluid because of obstruction
compensatory hypertrophy
enlargement of an organ secondary to an increased workload; often seen when part of an organ has been destroyed or when there is absence or decreased function of paired organs
clitoremegaly
enlargement of the clitoris
Meckle-Gruber syndrome
fetal syndrome associated with microcephaly, occipital encephalocele, polydactyly, and polycystic kidneys
polydactyly
having more than the normal number of fingers or toes
pelvocaliectasis
he dilation of the renal collecting system resulting from the obstruction of the flow of urine from the kidney(s) to the bladder; also referred to as pelvocaliectasis or pelvicaliectasi
neuroblastoma
malignant tumor that can occur within the adrenal gland and anywhere within the sympathetic nervous system
anhydramnios
no amniotic fluid
renal ectopia
refers to an abnormal location of the kidney or kidneys
vesicuureteral reflux
the retrograde flow of urine from the urinary bladder into the ureter
pulmonary hypoplasia
underdevelopment of the lungs
Urine comprises the greater part of amniotic fluid after
14 weeks.
The most worrisome consequence of oligohydramnios is
pulmonary hypoplasia, or underdevelopment of the lungs
sonographic findings of unilateral renal agenesis
- Absent kidney - Compensatory hypertrophy of the contralateral kidney - Visible urinary bladder - Normal amniotic fluid volume - Unilateral lying down adrenal sign - Undetectable renal artery branch with color Doppler (unilateral)
sonographic findings of bilateral renal agenesis
- Absent kidneys - Absent urinary bladder - Severe oligohydramnios or anhydramnios - Bilateral lying down adrenal signs - Undetectable renal artery branches with color Doppler (bilateral)
sonographic findings of autosomal recessive (infantile) polycystic kidneys disease
- Bilateral, enlarged echogenic kidneys - Absent urinary bladder - Oligohydramnios
sonographic findings of bilateral multicysti dysplastic renal disease
- Bilateral, smooth-walled, noncommunicating cysts of varying sizes located within the renal fossae - Absent urinary bladder - Oligohydramnios
sonographic findings of autosomal dominant (adult) polycysitc kidney disease
- Normal-appearing or bilateral, enlarged echogenic kidneys - Visible urinary bladder - Normal amniotic fluid volume - Cysts do not manifest until approximately the fifth decade of life
sonographic findings of obstructive cystic dysplasia
- Small, echogenic kidneys - Peripheral renal cysts - Bilateral hydronephrosis - Thick-walled urinary bladder - Oligohydramnios
sonographic findings of unilateral multicystic dysplastic renal disease
- Unilateral, smooth-walled, noncommunicating cysts of varying sizes located within the renal fossa - Compensatory hypertrophy of the contralateral kidney - Visible urinary bladder - Normal amniotic fluid volume
One condition associated with ARPKD is ________________________________, which is a fatal disorder that includes renal cystic disease, occipital encephalocele, and polydactyly
Meckel-Gruber syndrome,
siromelia
a fetal abnormality characterized by fusion of the lower extremities, renal agenesis, and oligohydramnios; may also be referred to as mermaid syndrome
obstructive cystic dysplasia
a fetal disorder caused by an early renal obstruction; leads to small and echogenic kidneys that have cysts located along their margins
multicystic dysplastic kidney disease
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
hydrocele
a fluid collection within the scrotum between the two layers of the tunica vaginalis
oligohydromnios
a lower-than-normal amount of amniotic fluid for the gestational age
allantois
a membrane that is present during early embryonic development that contributes to urinary bladder formation and development
autosomal dominant
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 recessive
a way in which a disorder or trait can be inherited by a fetus; both parents must be carriers of the gene for the disease
VACTERL
acronym for associated anomalies; stands for vertebral anomalies, anal atresia, cardiac anomalies, tracheoesophageal fistula or esophageal atresia, renal anomalies, and limb anomalies
OEIS complex
acronym that stands for omphalocele, bladder exstrophy, imperforate anus, and spina bifida; also referred to as cloacal exstrophy
ureterocele
an abnormality in which the distal ureter projects into the urinary bladder
megacystitis
an abnormally enlarged urinary bladder
micropenis
an abnormally small penis
megaureter
an enlarged ureter; can be congenital or acquired
autosomal dominant polycystic kidney disease
an inherited disease that results in the development of renal, liver, and pancreatic cysts late in life; also referred to as adult polycystic kidney disease
infantile polycystic kidney disease
an inherited renal disease that results in bilateral enlargement of the fetal kidneys and microscopic renal cysts; also referred to as autosomal recessive polycystic kidney disease
autosomal recessive polycystic kidney disease
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
ureteropelvic juction obstruction
an obstruction located in the region where the ureter meets the renal pelvis
caliectasis
dilation of the calices
The fetal urinary bladder normally fills and empties once in
every 30 to 45 minutes.
potter fascies
facial features seen with severe oligohydramnios, including low set ears, flattened nose, wrinkled skin, and micrognathia
renal agenesis
failure of the kidney to develop; may be unilateral or bilateral
MCDK disease is ________ if bilateral, but fortunately, most cases of MCDK are unilateral
fatal
posterior uretheral valves
irregular thin membranes of tissue located within the male posterior urethra that does not allow urine to exit the urethra
macroscopic
large enough to be discerned by the naked eye
renal pelvic diameter
measurement of the fetal renal pelvis; this dimension is obtained from the transverse kidney plane
ARPKD would be the most likely cause of e
nlarged, echogenic kidneys noted in utero.
Potter syndrome
physical features of a fetus as a result of oligohydramnios; characterized by bilateral renal agenesis, abnormal facies, pulmonary hypoplasia, and limb abnormalities; also referred to as Potter sequence
encephalocele
protrusion of the brain and meninges through a defect in the skull
Hydronephrosis is the most common fetal abnormality noted during an obstetric sonogram
pyelectasis
prune belly syndrome
syndrome that is a consequence of the abdominal wall musculature being stretched by the extremely enlarged urinary bladder
undescended testis
testicles that do not descend into the scrotum; also referred to as cryptorchidism
horseshoe kidney
the attachment of the lower poles of the kidneys by a band of renal tissue that crosses the midline of the abdomen
uretheral atresia
the congenital absence of the urethra
hydronephosis
the dilation of the renal collecting system resulting from the obstruction of the flow of urine from the kidney(s) to the bladder; also referred to as pelvocaliectasis or pelvicaliectasis
The most common renal anomaly is ________________________________, also referred to as a duplicated or double collecting system
the duplex collecting system
renal pelvis
the funnel-shaped collecting system in the central portion of the kidney that allows urine to flow from the kidney to the ureter
pelviureteral junction
the junction of the ureter and the renal pelvis
uteropelvic junction
the junction of the ureter and the renal pelvis
vesicoureteral junction
the junction of the ureter and the renal pelvis
ureterovesicular junction
the junction of the ureter and the urinary bladder
mesoblastic nephroma
the most common solid fetal renal mass
renal calices
the part of the collecting system that encompasses the apex of the renal pyramids
perineum
the region between the external genitalia and the anus
renal fossa
the region where the kidney is located in the abdomen
"keyhole" sign
the sonographic appearance of a dilated fetal bladder and urethra in the presence of a bladder outlet obstruction
"lying down" adrenal sign
the sonographic appearance of the adrenal gland in a parallel position within the abdomen as a result of renal agenesis
microscopic
too small to be seen by the naked eye and thus requiring the aid of a microscope