SM 166 Congenital and Pediatric Disorders

Question 1

What is Bronchopulmonary Dysplasia?

Answer 1

Term given to infants who had RDS and still require 30% FiO2 at 36 weeks post-menstrual age if < 32 weeks gestational age or at 56 days of life if > 32 weeks gestational age

Question 2

How are Tracheo-bronchomalacia and Laryngomalacia differentiated?

Answer 2

The sounds they make

Question 3

How does Laryngomalacia present?

Answer 3

Stridor, Omega shaped Epiglottis and prolapsed arytenoids

Question 4

How are pulmonary arteriovenous malformations treated?

Answer 4

Embolization, to block off the abnormal connection

Question 5

What are factors that decrease the risk of VPD?

Answer 5

Ventilation management, antenatal steroids to mother prior to delivery, surfactant therapy

Question 6

How does Tracheo-bronchomalacia present?

Answer 6

Noisy breathing, but not stridor

Question 7

How do pulmonary arteriovenous malformations present?

Answer 7

Pulmonary hemorrhage or hemoptysis

Question 8

What causes rings and slings?

Answer 8

A developmental abnormality of the aortic arch that leads to compression of the esophagus and trachea, either partial (sling) or completely (ring)

Question 9

How is lobar emphysema treated?

Answer 9

Like all 3 congenital parenchymal abnormalities, with surgery

Question 10

When does the Pseudoglandular Stage occur?

Answer 10

6 - 16 weeks

Question 11

Are pulmonary sequestrations intralobar or extralobar?

Answer 11

Intralobar > extralobar, but both are possible

Question 12

When does the Canalicular Stage occur?

Answer 12

16 - 26 weeks

Question 13

Which lobe is most effected by extralobar pulmonary sequestration?

Answer 13

The Left Lower Lobe

Question 14

How is pulmonary sequestration treated?

Answer 14

Like all 3 congenital parenchymal abnormalities, with surgery

Question 15

How do the causes of intralobar and extralobar pulmonary sequestration compare?

Answer 15

Both can be congenital, but intralobar may also be acquired

Question 16

Are males or females more effected by extralobar pulmonary sequestration?

Answer 16

Males

Question 17

What is congenital lobar emphysema?

Answer 17

Overinflation and distension of one or more pulmonary lobes

Question 18

How is pulmonary sequestration diagnosed?

Answer 18

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation

Question 19

What occurs during the Alveolar stage

Answer 19

Alveoli continue to mature and develop

Question 20

How can a ring or a sling be seen on CXR?

Answer 20

Indentation or “grooves” in the esophagus

Question 21

What causes congenital lobar emphysema?

Answer 21

Intrinsic or extrinsic bronchial narrowing leading to air trapping

Question 22

Does the Canalicular Stage have surfactant?

Answer 22

No, the Type II Pneumocytes have not differentiated

Question 23

What are factors that increase the risk of Bronchopulmonary Dysplasia?

Answer 23

Low birth weight, low gestational age, ventilation

Question 24

What causes intralobar pulmonary sequestration?

Answer 24

May be congenital or due to bronchial obstruction in older patients

Question 25

When does the Alveolar Stage occur?

Answer 25

36 weeks onward

Question 26

How many cysts do congenital pulmonary airway malformations cause?

Answer 26

Varied, from a single large cyst to several smaller cysts

Question 27

What are the 5 stages of lung development?

Answer 27

Every Pulmonologist Can See Alveoli Embryonic, Pseudoglandular, Canalicular, Saccular, Alveolar

Question 28

How is pulmonary airway malformation diagnosed?

Answer 28

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation

Question 29

What causes extralobar pulmonary sequestration?

Answer 29

Congenital

Question 30

How does congenital lobar emphysema present after birth?

Answer 30

Within hours, a worsening hyperlucent area can be seen on CXR

Question 31

How do rings and slings present?

Answer 31

Stridor, trouble swallowing, respiratory distress

Question 32

How is pulmonary airway malformation treated?

Answer 32

Like all 3 congenital parenchymal abnormalities, with surgery

Question 33

Which lobe is mostly frequently effected by lobar emphysema?

Answer 33

The left upper lobe

Question 34

How is Laryngomalacia treated?

Answer 34

No treatment unless aspiration or reflux occurs, in which case surgery is indicated

Question 35

What occurs during the Embryonic Stage?

Answer 35

Outpouching of the foregut into the Mesoderm results in the formation of the Trachea and Bronchi

Question 36

What is Respiratory Distress Syndrome?

Answer 36

RDS occurs when a newborn has lungs with insufficient compliance, making breathing difficult; typically require ventilation

Question 37

When does the Saccular Stage occur?

Answer 37

26 - 36 weeks

Question 38

How does RDS present on CXR?

Answer 38

Grey everywhere due to diffuse pulmonary edema from lack of surfactant

Question 39

Are males or females more effected by intralobar pulmonary sequestration?

Answer 39

Equal among males and females

Question 40

Do Alveoli stop maturing at birth?

Answer 40

No, continue to mature up to 8 years after birth

Question 41

When does the Embryonic Stage occur?

Answer 41

3 - 6 weeks

Question 42

What effect do antenatal steroids have on a premie?

Answer 42

Antenatal steroids given to mother promote surfactant production in the child

Question 43

What are the congenital vascular abnormalities?

Answer 43

Vascular rings/slings | Pulmonary arteriovenous malformation

Question 44

What occurs during the Canalicular Stage?

Answer 44

The primitive alveolar ducts form, and gas exchange can be supported after birth, but lack surfactant

Question 45

How is Laryngomalacia diagnosed?

Answer 45

Bronchoscopy can diagnose Laryngomalacia

Question 46

How is Tracheo-bronchomalacia diagnosed and treated?

Answer 46

Bronchoscopy + supportive or surgery if severe

Question 47

How is tracheoesophageal fistula treated?

Answer 47

Surgical

Question 48

What occurs during the Pseudoglandular Stage?

Answer 48

Airways continue to branch, with terminal bronchioles forming and epithelial cells differentiating Pulmonary vasculature develops but is far from the Bronchi and Bronchioles

Question 49

What causes Tracheo-bronchomalacia?

Answer 49

Etiology unknown, may involve cartilage

Question 50

What is a pulmonary sequestration?

Answer 50

Normal, non-functioning lung tissue with no connection to the bronchial tree that receives blood supply from the systemic circulation (Aorta); intralobar > extralobar

Question 51

What develops in the Saccular Stage?

Answer 51

Terminal sacs form, and Type I and II Pneumocytes differentiate

Question 52

What is a pulmonary arteriovenous malformation?

Answer 52

An abnormal connection between arteries and veins

Question 53

What are the 3 congenital parenchymal abnormalities?

Answer 53

Pulmonary Sequestration Pulmonary Airway Malformation Lobar Emphysema

Question 54

What is a tracheoesophageal fistula?

Answer 54

An abnormal connection between the Trachea and the Esophagus

Question 55

What causes Stridor?

Answer 55

A distortion in the airway at the level of the Larynx results in a high pitched inspiratory sound

Question 56

How does tracheoesophageal fistula present?

Answer 56

Drooling and choking within the first 24 hours of life

Question 57

What are the 3 congenital airway abnormalities?

Answer 57

LTT Laryngomalacia, Tracheo-bronchomalacia, and Tracheoesophageal Fistula

Question 58

What are congenital pulmonary airway malformations?

Answer 58

Congenital cystic and non-cystic lung masses that are due to overgrowth of primary bronchioles which communicate with an abnormal bronchial tree lacking cartilage

Question 59

Which disease produces cartilage rings without a C shape?

Answer 59

Tracheo-bronchomalacia

Question 60

How is tracheoesophageal fistula diagnosed?

Answer 60

Inability to pass a gastric tube

Question 61

Which lobe is most effected by intralobar pulmonary sequestration?

Answer 61

Neither lobe predominates

Question 62

What is an extralobar pulmonary sequestration?

Answer 62

An accessory lung separate from the main lung with no functionality

Question 63

What is an intralobar pulmonary sequestration?

Answer 63

Normal non-functioning lung tissue completely covered by normal lung tissue

Question 64

How is lobar emphysema diagnosed?

Answer 64

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation