Class 17: GI & GU Anomalies Flashcards
what is bilateral renal agenesis?
no renals – not compatible with life
another name for bilateral renal agenesis?
potter syndrome
which part of the population does potter syndrome more commonly occur in?
males
what is the most severe GU anomaly?
bilateral renal agenesis
what weeks will severe oligohydramnios be seen with potter syndrome?
16-28 weeks
potter syndrome will lead to oligohydramnios, which will lead to __ ___
pulmonary hypoplasia
which part of pregnancy is directly related to the fetal production of urine?
2nd half of pregnancy
the following are secondary signs of which GU anomaly?
1. no UB
2. oligohydramnios
3. facial anomalies
4. clubfoot
potter syndrome
on US, what structures may mimic the renals? why?
adrenal glands – larger in the fetus
GU anomalies are associated with polyhydramnios. T/F?
false – oligohydramnios
with unilateral renal agenesis, everything in the fetus will look normal as long as there is no ___
obstruction
what is fetal hydronephrosis?
when the renal pelvis is dilated > 8 mm
what is the most common cause of fetal hydronephrosis?
UPJ obstruction
what is a frequent cause of UPJ obstruction?
double collecting system
which part of the population does posterior urethral valve syndrome more commonly occur in?
males
PUV is unilateral. T/F?
false
cause of PUV
extramembranous folds obstruct the distal fetal urethra
5 US appearances of PUV?
- keyhole sign (dilated urethra)
- enlarged UB
- thick UB wall
- hydroureters
- oligohydramnios
another name for prune belly triad?
eagle barrett syndrome
what part of the population does eagle barrett syndrome more commonly occur in?
97% males
3 signs of prune belly triad
- hypoplastic/agenesis of ABD musculature
- cryptoorchidism
- PUV & urinary ascites
why is eagle barrett syndrome called prune belly?
the abdomen of fetus is distended dt UB –> wrinkled when hydronephrosis is decompressed after birth
IPKD is autosomal recessive. T/F?
true
4 types of IPKD?
- perinatal
- neonatal
- infantile
- juvenile
what is perinatal IPKD?
when renals fail in the fetal period
what is neonatal IPKD?
renals start failing in the 1st month of life – death in the 1st year
what is infantile IPKD?
renals start failing within 3-6 months of life
what is juvenile IPKD?
renal issues between 1-5 yo & there is less renal involvement
why are the renals hyperechoic with IPKD?
many microscopic cysts give acoustic enhancement
US appearance of IPKD?
- bilateral enlarged homogenous hyperechoic kidneys
- severe oligohydramnios
- UB not seen
what is the most common renal dysplasia?
multicystic dysplastic kidney
multicystic dysplastic kidney is unilateral. T/F?
true
is multicystic dysplastic kidney autosomal recessive or dominant?
dominant
how do the lungs and amniotic fluid look with multicystic dysplastic kidney?
normal – one kidney is functioning properly
associations of multicystic dysplastic kidney?
an abnormality with the contralateral kidney
US appearance of multicystic dysplastic kidney?
many large anechoic cysts
largest cyst is not centered
malformed kidney shape
differential dx of multicystic dysplastic kidney?
hydronephrosis (with frozen image)
what is fetal hydrocele?
fluid around fetal testes
GI tract abnormalities are associated with poly or oligohydramnios?
polyhydramnios
what is duodenal atresia?
there is a blockage of the duodenal lumen, leading to dilation of duodenum & stomach, backing up amniotic fluid
US appearance of duodenal atresia?
double bubble sign
3 conditions duodenal atresia is associated with?
- VACTERL
- down’s syndrome (21)
- bowel malrotation (annular pancreas)
definition of fetal hydrops?
excessive accumulation of clear, watery fluid in any tissues/cavities
US signs for fetal hydrops? (AAPPPPHHS)
ascites
anasarca
pleural effusion
pulmonary effusion
polyhydramnios
placentamegaly
hepatosplenomegaly
hydropic umbilical cord
scalp edema
2 types of fetal hydrops?
- non-immune fetal hydrops
- immune fetal hydrops
most common cause of NIFH?
cardiovascular abnormalities
2nd most common cause of NIFH?
chromosomal abnormalities
the outcome for NIFH depends on ___
underlying causes
what is the most common cause of IFH?
Rh disease/fetal blood incompatibility/Rh incompatible/rhizoimmunization
are most people Rh+ or Rh-?
Rh+
with Rh disease, mom has Rh_ & baby has Rh_
mom = Rh-
fetus = Rh+
what happens with IFH
if there is a mixing of blood bw mom & fetus –> mom exposed to Rh antigen –> mom makes Ab against it –> attacks fetus RBC through placenta –> fetal anemia, CHF, hydrops
5 effects to the fetus with IFH
- jaundice
- anemia
- brain damage
- heart failure
- death
if the fetus has mild hemolytic disease, what is the treatment?
no prenatal tx needed
if the fetus has moderate hemolytic disease, what is the tx?
1 prenatal transfusion & neonatal tx with multiple transfusions
if the fetus has severe hemolytic disease, what is the tx?
multiple prenatal transfusions until 26-32 weeks
& aggressive neonatal tx
