Congenital abnormalities

Question 1

what are the embryologic stages in order?

Answer 1

embryonic>pseudoglandular>canalicular>saccular>alveolar

Question 2

embryonic stage

Answer 2

foregut outpouches into mesoderm and branching begins (trachea,mainstem, lobar,segmental) under guidance of mesynchymal tissue. Early vascular connections w/ atria

Question 3

pseudoglandular stage

Answer 3

terminal bronchioles, epithelium begins to differentiate, vasculature developes

Question 4

canalicular stage

Answer 4

alveolar ducts and acinar tubules, secretory and lining cells, capillaries surround acinar tubules for gas exchange

Question 5

saccular stage

Answer 5

acini continue, cells differentiate (type 1 &2)

Question 6

alveolar stage

Answer 6

alveoli continue to develop, continues into adolescence

Question 7

Respiratory Distress Syndrome

Answer 7

not enough surfactant, ground glass appearance on radiograph, often in premature infants

Question 8

Bronchopulmonary Displasia

Answer 8

same as Chronic Lung disease of Infancy
Infants who have RDS. If on O2 at day 28, assess O2 need at either 36 wks post menstrual age (or equal to 32wksGA). Room air=mild, 30%FIO2 or PPV=severe

Question 9

Factors that increase and decrease risk of BPD

Answer 9

Increase=infections, ppv, O2 toxicity

Decrease=antenatal steroids(for all women at risk of birthing before 34wks), surfactant therapy

Question 10

Laryngomalacia

Answer 10

Stridor. Omega epiglottis, short aryepiglottic folds, prolapse arytenoids

Question 11

Tracheo-Bronchomalacia

Answer 11

Alterations of cartilage and aggrecans, Trachea just kinda collapses

Question 12

Tracheoeophageal Fistula

Answer 12

drooling, choking when feeding attempted, usually first 24 hrs of life. Incomplete separation of esophagus from larygotracheal tube. Esophagus is blind pouch w/ stomach usually connected to distal trachea

Question 13

Intralobar pulmonary sequestration

Answer 13

nonfxnal tissue completely covered by normal lung tissue. Congenital or acquired. No lobar predominance or gender predilection. Venous drainage via pulm veins. Blood supply via systemic

Question 14

extralobar pulmonary sequestration

Answer 14

separated from fxnal lung. Congenital. Left lower lobe. Males. Venous drainage via systemic (azygos or portal)

Question 15

CPAM

Answer 15

congenital pulmonary airways malformation.
Overgrowth of primary bronchioles. huge cysts usually in lower lobes. Can be confused with congenital diaphragmatic hernia

Question 16

Congenital lobar emphysema

Answer 16

overinflation of a lobe. Intrinsic causes-absence of bronchial cartilage. Extrinsic-mass effect. Bronchial narrowing w/ air trapping. Hypo or Polyalveolar based on number of alveoli. LUL.

Question 17

Rings, slings, other things

Answer 17

compression of esophagus and trachea. Sling-aberrant left pulm artery (ant. compression of esoph) or right subclavian(post. comp. of esoph). Ring-double aortic arch (ant and post comp. of esoph)

Question 18

Pulmonary arteriovenous malformations (AVM)

Answer 18

abnormal artery->vein connection. Presents as hypoxia, pulm hemorrhage, hemoptysis. Rx w/ embolization.

Question 19

Hereditary hemorrhagic telangectasia

Answer 19

Mutations in ALK1, ENG, SMAD4 cause AVM’s all over body