Developmental Pathology 2

Question 1

Define hydrops fetalis.

Answer 1

severe diffuse edema of of tissues and multiple effusion in body cavities

Question 2

What are the major causes of immune and non-immune (3) hydrops?

Answer 2

immune: classically associated with Rh factor incompatibility (erythroblastosis fetalis/ HDON)

non-immune: cardiovascular defects, chromosomal anomalies (Turners 45X) and non-immune fetal anemia (parvo B19, hemoglobinopathy)

Question 3

What is cystic hygroma?

Answer 3

‘posterior cervical hygroma’
fluid accumulation in the neck

associated with multiple causes, essentially a focal form of fetal hydrops, U/S dx may indicate underlying lethal condition

Question 4

Contrast omphalocele and castroschesis.

Answer 4

omphalocele is a large *umbilical cord defect through which intestines protrude, covered in a membranous sac

gastrocschesis is a paraumbilical *abdominal wall defect with extruded free loops of bowel, not covered by a sac

Question 5

How is volume of amniotic fluid regulated?

Answer 5

fetus largely regulates amniotic fluid: amniotic fluid is produced by fetal urine and removed by fetal swallowing

Question 6

Give examples of what cases oligohydramnios and polyhydramnios.

Answer 6

oligohydramnios: urethral obstruction (prune belly), renal anomalies (potter sequence)
polyhydramnios: esophageal obstruction (atresia)

Question 7

Describe the association of symptoms that occur with Prune Belly Syndrome (sequence).

Answer 7

resulting from urethral obstruction occurring mainly in males

prune-like belly with wrinkled skin, thin or absent abdominal musculature, dilated bladder, hydroureters, hydronephrosis, renal dysplasia

cryptorchidism (lack of descent), hypo plastic prostate

Question 8

What is intrauterine growth restriction?

Answer 8

fetus is small for gestational age based on ultrasound measurements (less than 10th percentile weight for gestational age)

Question 9

Contrast the causes of symmetric (proportional) and asymmetric FRG/ IUGR.

Answer 9

symmetric: fetal head and body equally small due to fetal factors ie. chromosomal defect/infections- a global insult
asymmetric: head size is normal but body and other organs are small often due to placental or maternal factors (ie. uteroplacental insufficiency, abnormal cord, placenta prevue or abruption)

Question 10

What are the structures of a normal umbilical cord?

Answer 10

two arteries and a single vein (if only a single artery can signal CV troubles)

Question 11

What are the detrimental effects of an ascending infection? What is typically the etiology?

Answer 11

ascending infections are often bacterial, they cause chroioamnionitis and premature birth, not IUGR

Question 12

Define chronic villititis and what is usually suggests.

Answer 12

chronic inflammatory cells of the placental villi suggest chronic transplacental infection (TORCHES) or villitits of unknown etiology (VUE) or autoimmune process

Question 13

What is the syndrome associated with transplacental parvovirus B19 infection?

Answer 13

has a predilection for RBC, causing anemia, hydrops and extramedulary hematopoeisis

Question 14

What do PPROM and PROM stand for?

Answer 14

PPROM: preterm premature rupture of membranes

PROM: premature rupture of membranes refers to rupture of membranes prior to labor but after 37 weeks

Question 15

What are causes/associations with premature delivery?

Answer 15

prior preterm labor or premature birth
multiple gestation
structural abnormalities of uterus, cervix or placenta
smoking, drugs, alcohol
poor maternal nutrition
chronic maternal disease
pre-pregnancy weight outside the normal range
prior history of spontaneous abortions
trauma/ injury during pregnancy
**ascending infections

Question 16

______ infections may cause premature rupture of membranes due to inflammation and premature rupture of membranes increases the risk of ______ infections.

Answer 16

transcervical can cause PROM

ascending infection risk increased with PROM

Question 17

Contrast chroiomaniotis and fetal vasculitis/ funisitis.

Answer 17

chroioamniotis is acute inflammation of fetal membranes due to *maternal immune response (maternal PMN), can cause PPROM and complications of prematurity

fetal vasculitis/funistis is the fetal inflammatory response causing acute inflammation of the umbilical cord, indicates neonatal infection

Question 18

Organisms including gram negatives and Group B strep can cause fetal/ neonatal infection in the form of _____, ______ and ______.

Answer 18

pneumonia, meningitis and sepsis

Question 19

Which bugs cause early onset vs. late onset perinatal infection?

Answer 19

early (first week of life): E. coli, Group B strep

late (later than 1 week after birth): Listeria, Candida

Question 20

What are the 3 most common causes of neonatal meninigitis?

Answer 20

EGL:
E. coli
Group B strep
Listeria

Question 21

Describe the problems seen in the following organ systems if an infant is born prematurely.

Answer 21

brain: hemorrhages (germinal matrix, intraventricular)
GI tract: necrotizing enterocolitis
Heart: PDA
Liver: physiologic jaundice (risk of kernicterus)
Lungs: hyaline membrane disease

Question 22

What should be administered to mothers if preterm delivery is expected, to better prepare neonatal lungs for breathing?

Answer 22

glucocorticoids (steroids) will induce surfactant synthesis, alveoli do not develop until 26-32 weeks and there is usually a deficiency in surfactant

Question 23

Describe the events that occur with neonatal respiratory distress syndrome.

Answer 23

atelectasis of small alveoli
hypoxemia, CO2, and acidosis
poor pulmonary perfusion and cellular damage
leakage of plasma and fibrin
deposition of fibrin membranes (fibrin, fibrinogen and cell debris)

(develops only after birth)

Question 24

What are the possible complications of neonatal Respiratory distress syndrome (hyaline membrane disease) and how is it treated?

Answer 24

treated with assisted ventilation and high oxygen levels and prophylactic exogenous surfactant

complications include chronic lung disease, retinopathy and bronchopulmonary dysplasia (epithelial hyperplasia, interstitial fibrosis and alveolar wall thickening) due to oxygen toxicity and/or barotrauma from respiratory