Class 17: GI & GU Anomalies

Question 1

what is bilateral renal agenesis?

Answer 1

no renals – not compatible with life

Question 2

another name for bilateral renal agenesis?

Answer 2

potter syndrome

Question 3

which part of the population does potter syndrome more commonly occur in?

Answer 3

males

Question 4

what is the most severe GU anomaly?

Answer 4

bilateral renal agenesis

Question 5

what weeks will severe oligohydramnios be seen with potter syndrome?

Answer 5

16-28 weeks

Question 6

potter syndrome will lead to oligohydramnios, which will lead to __ ___

Answer 6

pulmonary hypoplasia

Question 7

which part of pregnancy is directly related to the fetal production of urine?

Answer 7

2nd half of pregnancy

Question 8

the following are secondary signs of which GU anomaly?
1. no UB
2. oligohydramnios
3. facial anomalies
4. clubfoot

Answer 8

potter syndrome

Question 9

on US, what structures may mimic the renals? why?

Answer 9

adrenal glands – larger in the fetus

Question 10

GU anomalies are associated with polyhydramnios. T/F?

Answer 10

false – oligohydramnios

Question 11

with unilateral renal agenesis, everything in the fetus will look normal as long as there is no ___

Answer 11

obstruction

Question 12

what is fetal hydronephrosis?

Answer 12

when the renal pelvis is dilated > 8 mm

Question 13

what is the most common cause of fetal hydronephrosis?

Answer 13

UPJ obstruction

Question 14

what is a frequent cause of UPJ obstruction?

Answer 14

double collecting system

Question 15

which part of the population does posterior urethral valve syndrome more commonly occur in?

Answer 15

males

Question 16

PUV is unilateral. T/F?

Answer 16

false

Question 17

cause of PUV

Answer 17

extramembranous folds obstruct the distal fetal urethra

Question 18

5 US appearances of PUV?

Answer 18

1. keyhole sign (dilated urethra)
2. enlarged UB
3. thick UB wall
4. hydroureters
5. oligohydramnios

Question 19

another name for prune belly triad?

Answer 19

eagle barrett syndrome

Question 20

what part of the population does eagle barrett syndrome more commonly occur in?

Answer 20

97% males

Question 21

3 signs of prune belly triad

Answer 21

1. hypoplastic/agenesis of ABD musculature
2. cryptoorchidism
3. PUV & urinary ascites

Question 22

why is eagle barrett syndrome called prune belly?

Answer 22

the abdomen of fetus is distended dt UB –> wrinkled when hydronephrosis is decompressed after birth

Question 23

IPKD is autosomal recessive. T/F?

Answer 23

true

Question 24

4 types of IPKD?

Answer 24

1. perinatal
2. neonatal
3. infantile
4. juvenile

Question 25

what is perinatal IPKD?

Answer 25

when renals fail in the fetal period

Question 26

what is neonatal IPKD?

Answer 26

renals start failing in the 1st month of life -- death in the 1st year

Question 27

what is infantile IPKD?

Answer 27

renals start failing within 3-6 months of life

Question 28

what is juvenile IPKD?

Answer 28

renal issues between 1-5 yo & there is less renal involvement

Question 29

why are the renals hyperechoic with IPKD?

Answer 29

many microscopic cysts give acoustic enhancement

Question 30

US appearance of IPKD?

Answer 30

- bilateral enlarged homogenous hyperechoic kidneys - severe oligohydramnios - UB not seen

Question 31

what is the most common renal dysplasia?

Answer 31

multicystic dysplastic kidney

Question 32

multicystic dysplastic kidney is unilateral. T/F?

Answer 32

true

Question 33

is multicystic dysplastic kidney autosomal recessive or dominant?

Answer 33

dominant

Question 34

how do the lungs and amniotic fluid look with multicystic dysplastic kidney?

Answer 34

normal -- one kidney is functioning properly

Question 35

associations of multicystic dysplastic kidney?

Answer 35

an abnormality with the contralateral kidney

Question 36

US appearance of multicystic dysplastic kidney?

Answer 36

many large anechoic cysts largest cyst is not centered malformed kidney shape

Question 37

differential dx of multicystic dysplastic kidney?

Answer 37

hydronephrosis (with frozen image)

Question 38

what is fetal hydrocele?

Answer 38

fluid around fetal testes

Question 39

GI tract abnormalities are associated with poly or oligohydramnios?

Answer 39

polyhydramnios

Question 40

what is duodenal atresia?

Answer 40

there is a blockage of the duodenal lumen, leading to dilation of duodenum & stomach, backing up amniotic fluid

Question 41

US appearance of duodenal atresia?

Answer 41

double bubble sign

Question 42

3 conditions duodenal atresia is associated with?

Answer 42

1. VACTERL 2. down's syndrome (21) 3. bowel malrotation (annular pancreas)

Question 43

definition of fetal hydrops?

Answer 43

excessive accumulation of clear, watery fluid in any tissues/cavities

Question 44

US signs for fetal hydrops? (AAPPPPHHS)

Answer 44

ascites anasarca pleural effusion pulmonary effusion polyhydramnios placentamegaly hepatosplenomegaly hydropic umbilical cord scalp edema

Question 45

2 types of fetal hydrops?

Answer 45

1. non-immune fetal hydrops 2. immune fetal hydrops

Question 46

most common cause of NIFH?

Answer 46

cardiovascular abnormalities

Question 47

2nd most common cause of NIFH?

Answer 47

chromosomal abnormalities

Question 48

the outcome for NIFH depends on ___

Answer 48

underlying causes

Question 49

what is the most common cause of IFH?

Answer 49

Rh disease/fetal blood incompatibility/Rh incompatible/rhizoimmunization

Question 50

are most people Rh+ or Rh-?

Answer 50

Rh+

Question 51

with Rh disease, mom has Rh_ & baby has Rh_

Answer 51

mom = Rh- fetus = Rh+

Question 52

what happens with IFH

Answer 52

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

Question 53

5 effects to the fetus with IFH

Answer 53

1. jaundice 2. anemia 3. brain damage 4. heart failure 5. death

Question 54

if the fetus has mild hemolytic disease, what is the treatment?

Answer 54

no prenatal tx needed

Question 55

if the fetus has moderate hemolytic disease, what is the tx?

Answer 55

1 prenatal transfusion & neonatal tx with multiple transfusions

Question 56

if the fetus has severe hemolytic disease, what is the tx?

Answer 56

multiple prenatal transfusions until 26-32 weeks & aggressive neonatal tx