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