Pulm - Pediatric Disease

Question 1

What are the stages of pulmonary embryology?

Answer 1

1. Embryonic
2. Pseudoglandular
3. Cannalicular
4. Saccular
5. Alveolar

Question 2

Name the time periods of the following stages of pulmonary development:

1. Embryonic
2. Pseudoglandular
3. Cannalicular
4. Saccular
5. Alveolar

Answer 2

• (Follow the sixes)*
  1. Weeks 3 - 6
  2. Weeks 6 - 16
  3. Weeks 16 - 26
  4. Weeks 26 - 36
  5. Weeks 36 - adolescence

Question 3

What happens in the embryonic stage of pulmonary development?

(Embryonic –> Pseudoglandular –> Cannalicular –> Saccular –> Alveolar)

Answer 3

Outpouching of foregut;

development all the way to lobar bronchi

Question 4

What happens in the pseudoglandular stage of pulmonary development?

(Embryonic –> Pseudoglandular –> Cannalicular –> Saccular –> Alveolar)

Answer 4

Development all the way to terminal bronchioles

Question 5

What happens in the cannalicular stage of pulmonary development?

(Embryonic –> Pseudoglandular –> Cannalicular –> Saccular –> Alveolar)

Answer 5

Development of type I and type II pneumocytes

Question 6

What happens in the saccular stage of pulmonary development?

(Embryonic –> Pseudoglandular –> Cannalicular –> Saccular –> Alveolar)

Answer 6

Capillary bed development;

surfactant production increases

Question 7

What happens in the alveolar stage of pulmonary development?

(Embryonic –> Pseudoglandular –> Cannalicular –> Saccular –> Alveolar)

Answer 7

Alveolar invaginations

(increase in number)

Question 8

Gas exchange is first supported in which phase of pulmonary embryology?

Answer 8

Late cannalicular

(embryonic –> pseudoglandular –> cannalicular –> saccular –> alveolar)

Question 9

What is the term given to the lung disease in infants who have persistent respiratory distress syndrome and continued airway/parenchymal abnormalities?

Answer 9

Bronchopulmonary dysplasia

Question 10

Progression to bronchopulmonary dysplasia is assessed at day ____ in patients with persistent respiratory distress syndrome.

Answer 10

Progression to bronchopulmonary dysplasia is assessed at day 28 in patients with persistent respiratory distress syndrome.

Question 11

A newborn presents with inspiratory stridor due to a malformed, floppy laryngeal structure (causing airway collapse during inspiration).

What is the name of this condition characterized by an omega-shaped epiglottis, arytenoid collapse, and a short aryepiglottic fold?

Answer 11

Laryngomalacia

Question 12

A newborn presents with noisy breathing due to a malformed, floppy airway (causing airway collapse).

What is the name of this condition characterized by absence or abnormal development of tracheal cartilagenous C-rings?

Answer 12

Tracheobronchomalacia

Question 13

What is the most common subtype of tracheoesophageal fistula?

Answer 13

Esophageal Atresia with distal TEF (87%)

Question 14

Tracheoesophageal fistulas are incomplete outpouching of the primitive __________.

Answer 14

Tracheoesophageal fistulas are incomplete outpouching of the primitive foregut.

Question 15

What congenital lung disorder is characterized by normal, non-functioning lung tissue that is not connected to the bronchial tree?

(Note: may be bronchopulmonary foregut malformation, (abnormal lung tissue connected to GI tract), intralobar, and extralobar.)

Answer 15

Pulmonary sequestration

Question 16

Congenital pulmonary airway malformations (CPAM) make up 25-30% of all congenital lung malformations. What are they?

Answer 16

Cystic and non-cystic pulmonary masses

(developmental issues)

Question 17

Congenital lobar emphysema is caused by air trapping due to what?

Answer 17

Bronchial narrowing

Question 18

How do pulmonary arteriovenous malformations present?

Answer 18

Hemoptysis, hypoxia

(congenital abnormal connections between arteries and veins)

Question 19

What is here described?

Congenital issue: vessels that partially wrap around the esophagus + bronchial tree (e.g. aberrant left pulmonary artery, aberrant right subclavian)

Answer 19

Pulmonary vascular slings

Question 20

What is here described?

Congenital issue: vessels that completely encircle the esophagus + bronchial tree (e.g. double aortic arch)

Answer 20

Pulmonary vascular rings

Question 21

What are pulmonary vascular slings?

Answer 21

Congenital issue: vessels that partially wrap around the esophagus + bronchial tree (e.g. aberrant left pulmonary artery, aberrant right subclavian)

Question 22

What are pulmonary vascular rings?

Answer 22

Congenital issue: vessels that completely encircle the esophagus + bronchial tree (e.g. double aortic arch)

Question 23

How do pulmonary vascular slings and rings present?

Answer 23

Stridor,

respiratory distress,

recurrent pneumonia,

dysphagia,

apnea