Congenital and Pediatric D/O

Question 1

When are the trachea, main stem, lobar and segmental bronchi formed during embryogenesis?

Answer 1

the embryonic stage

Question 2

In the embryonic stage, what is absent from the trachea and bronchi?

Answer 2

cartilage, smooth muscle, nerves

Question 3

At what stage does the embryonic tissue begin to differentiate into pneumocytes and other tissue?

Answer 3

pseudoglandular stage (6 to 16 w)

Question 4

When does the primitive acinar structure form?

Answer 4

pseudoglandular stage (6 to 16 w)

Question 5

When are vascular connections with the atria established?

Answer 5

embryonic stage

Question 6

When does the pulmonary vasculature develop parallel to the bronchi?

Answer 6

pseudoglandular stage (6 to 16w)

Question 7

When do type I and type II pneumocytes begin to differentiate?

Answer 7

Canalicular stage ( 16 to 26 w)

Question 8

When is the embryo capable of gas exchange?

Answer 8

the end of the canalicular stage (26 w)

Question 9

When does the embryo switch from branching morphogenesis to the process of alveolarization?

Answer 9

saccular stage (26 to 36 w)

Question 10

When are epithelial cells fully differentiated?

Answer 10

the saccular stage

Question 11

How does the alveolar stage progress, to make alveoli?

Answer 11

septae invaginate into exisiting spaces

Question 12

Define respiratory distress syndrome

Answer 12

newborn without surfactant and difficulty breathing due to increased surface tension

Question 13

Decreased lung volume, reticulogranular ground glass in air bronchograms, and unclear heart and lung borders are characteristic CXR findings of what congenital condition?

Answer 13

RDS

Question 14

When are babies diagnosed with bronchopulmonary dysplasia (BPD)

Answer 14

Any infant who required oxygen at 28 days of life

*not graded at this stage

Question 15

Define mild BPD

Answer 15

breathing room air at 36 weeks post menstrual age if under 32 weeks gestation
or
breathing room air at 56 days oflife if over 32 weeks gestation

Question 16

Define moderate BPD

Answer 16

breathing less than 30% FiO2 at 36 weeks post menstrual age or 56 days of life

Question 17

Define severe BPD

Answer 17

breathing more than 30% FiO2 or requiring positive pressure ventilation at 36 weeks post menstrual age

Question 18

Lower birth weight, lower gestational age, infections, oxygen toxicity and positive pressure ventilation all increase a babies risk of what condition?

Answer 18

BPD (and therefore, RDS by definition)

Question 19

What 2 treatments are established for patients with BDP in addition to nutrition and ventilation support?

Answer 19

1. corticosteroids prior to premature birth

2. surfactant after birth

Question 20

A baby presents with stridor. Upon bronchoscopy, they are found to have an omega shaped epiglottis, short aryepiglottic folds, and prolapsed arytenoids. What do they have?

Answer 20

laryngomalacia

Question 21

What are two risks for a laryngomalacia baby that must be evaluated, even if they don’t need surgery?

Answer 21

aspiration and reflux

Question 22

A baby presents with noisy breathing. Upon bronchoscopy, they have a collapsed trachea due to lack of C shaped cartilage, large membranous spaces, and difficulty breathing. What do they have?

Answer 22

tracheo-bronchomalacia

Question 23

How do you treat tracheo bronchomalacia?

Answer 23

support, surgery if very severe

Question 24

What is a fistula?

Answer 24

an abnormal connection between two structures

Question 25

When does a tracheoesophageal fistula present/

Answer 25

First day of life

Question 26

On DOL 1, a baby is drooling, has difficulty with feeding due to choking, and a feeding tube is unsuccessful . What does he or she have? How would you confirm?

Answer 26

tracheoesophageal fistula | confirm with barium CXR

Question 27

What kind of atresia is most common with a tracheoesophageal fistula?

Answer 27

esophageal atresia with a distal tracheoesophageal fistula. esophagus ends in blind pouch, trachea and esophagus resume further down and do not connect to upper structure

Question 28

When can pulmonary sequestration present?

Answer 28

as a neonate (RDS), prenatal (ultrasound), or older (recurring infections)

Question 29

In pulmonary sequestration, where does the nonfunctioning lung tissue get its blood supply? be specific

Answer 29

systemic circulation, i.e. descending thoracic aorta or abdominal aorta

Question 30

What types of pulmonary sequestration are there? (3)

Answer 30

intra lobar (most common, 3;1) extralobar bronchopulmonary foregut malformation

Question 31

What is a bronchopulmonary foregut malformation type pulmonary sequestration?

Answer 31

abnormal lung tissue connects to GI

Question 32

How is an intra lobar pulmonary sequestration defined?

Answer 32

extra lung tissue is completely covered by normal lung tissue or visceral pleura

Question 33

what are the possible etiologies of intralobar pulmonary sequestration?

Answer 33

congenital (lung bud) or acquired (bronchial obstruction -> pneumonia -> parasitization)

Question 34

How are most intralobar pulmonary sequestrations drained?

Answer 34

into pulmonary veins

Question 35

What is an extralobar pulmonary sequestration?

Answer 35

an accessory lung that is covered by visceral pleura and separated from the functioning lung

Question 36

What lobe is most commonly affected by extralobar sequestration? What is the etiology?

Answer 36

LLL | congenital

Question 37

What is the difference between venous drainage in intra v. extralobar sequestration?

Answer 37

``` intra = pulmonary veins extra = systemic venous system (azygos or portal) ```

Question 38

Which kind of pulmonary sequestration is most common in males?

Answer 38

extralobar

Question 39

Which condition is defined by overgrowth of the primary bronchioles and formation of cystic lung masses in communication with the abnormal bronchi, which lack cartilage?

Answer 39

congenital pulmonary airway malformation

Question 40

Where in the lungs is CPAM usually found?

Answer 40

lower lobes, usually only one

Question 41

How do you classify CPAM?

Answer 41

Stocker classification (0-4 by cysts and level of involvement)

Question 42

What is congenital lobar emphysema?

Answer 42

overinflation or distention of pulmonary lobes due to either intrinsic or extrinsic causes

Question 43

what are the intrinsic causes of congenital lobar emphysema?

Answer 43

weakness or absence of underlying bronchial cartilage

Question 44

What are the extrinsic causes of congenital lobar emphysema?

Answer 44

mass effects

Question 45

How is congenital lobar emphysema histologically defined?

Answer 45

by number of alveoli - poly or hypo

Question 46

Which lobe is usually affected in congenital lobar emphysema? is it usually bilateral? is it multifocal?

Answer 46

left upper lobe unilateral unifocal

Question 47

What are the three types of mediastinal vasculature abnormalities that we studied?

Answer 47

slings rings and things

Question 48

What part of the pulmonary system is affected by rings/slings?

Answer 48

the bronchial tree is compressed

Question 49

A baby presents with trouble swallowing, stridor, RDS, recurrent pneumonia, and episodes where they stop breathing. What do they have?

Answer 49

rings/slings

Question 50

What is an aberrant pulmonary left artery or right subclavian usually an example of in congenital pulmonary abnormalities?

Answer 50

A sling

Question 51

What is the effect of a double aortic arch (usually) in congenital pulmonary abnormalities?

Answer 51

a ring

Question 52

How do you diagnose a ring or sling?

Answer 52

clinical suspicion followed by barium swallow looking for indentations of esophagus

Question 53

A baby presents with asymptomatic hypoxia which progresses to hemoptysis and signs of pulmonary hemorrhage. What should be on your differential?

Answer 53

pulmonary arteriovenous malformations (MVA) * don't know if it is actually progressive, but any one of these things in isolation should also make you think of an MVA

Question 54

How do you treat a large AVM that is causing symptoms?

Answer 54

embolization

Question 55

A baby has a mutation in activin receptor kinase (ALk1) endoglin, or SMAD4. How might they present?

Answer 55

They have hereditary hemorrhagic telangectasia. They can have telangectasias of the nose, mouth, hands, lung, GI, or brain