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Pulmonology > SM 166 Congenital and Pediatric Disorders > Flashcards

SM 166 Congenital and Pediatric Disorders Flashcards

1
Q

What is Bronchopulmonary Dysplasia?

A

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

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2
Q

How are Tracheo-bronchomalacia and Laryngomalacia differentiated?

A

The sounds they make

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3
Q

How does Laryngomalacia present?

A

Stridor, Omega shaped Epiglottis and prolapsed arytenoids

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4
Q

How are pulmonary arteriovenous malformations treated?

A

Embolization, to block off the abnormal connection

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5
Q

What are factors that decrease the risk of VPD?

A

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

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6
Q

How does Tracheo-bronchomalacia present?

A

Noisy breathing, but not stridor

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7
Q

How do pulmonary arteriovenous malformations present?

A

Pulmonary hemorrhage or hemoptysis

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8
Q

What causes rings and slings?

A

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

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9
Q

How is lobar emphysema treated?

A

Like all 3 congenital parenchymal abnormalities, with surgery

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10
Q

When does the Pseudoglandular Stage occur?

A

6 - 16 weeks

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11
Q

Are pulmonary sequestrations intralobar or extralobar?

A

Intralobar > extralobar, but both are possible

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12
Q

When does the Canalicular Stage occur?

A

16 - 26 weeks

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13
Q

Which lobe is most effected by extralobar pulmonary sequestration?

A

The Left Lower Lobe

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14
Q

How is pulmonary sequestration treated?

A

Like all 3 congenital parenchymal abnormalities, with surgery

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15
Q

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

A

Both can be congenital, but intralobar may also be acquired

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16
Q

Are males or females more effected by extralobar pulmonary sequestration?

A

Males

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17
Q

What is congenital lobar emphysema?

A

Overinflation and distension of one or more pulmonary lobes

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18
Q

How is pulmonary sequestration diagnosed?

A

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation

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19
Q

What occurs during the Alveolar stage

A

Alveoli continue to mature and develop

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20
Q

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

A

Indentation or “grooves” in the esophagus

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21
Q

What causes congenital lobar emphysema?

A

Intrinsic or extrinsic bronchial narrowing leading to air trapping

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22
Q

Does the Canalicular Stage have surfactant?

A

No, the Type II Pneumocytes have not differentiated

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23
Q

What are factors that increase the risk of Bronchopulmonary Dysplasia?

A

Low birth weight, low gestational age, ventilation

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24
Q

What causes intralobar pulmonary sequestration?

A

May be congenital or due to bronchial obstruction in older patients

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25
Q

When does the Alveolar Stage occur?

A

36 weeks onward

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26
Q

How many cysts do congenital pulmonary airway malformations cause?

A

Varied, from a single large cyst to several smaller cysts

27
Q

What are the 5 stages of lung development?

A

Every Pulmonologist Can See Alveoli

Embryonic, Pseudoglandular, Canalicular, Saccular, Alveolar

28
Q

How is pulmonary airway malformation diagnosed?

A

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation

29
Q

What causes extralobar pulmonary sequestration?

A

Congenital

30
Q

How does congenital lobar emphysema present after birth?

A

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

31
Q

How do rings and slings present?

A

Stridor, trouble swallowing, respiratory distress

32
Q

How is pulmonary airway malformation treated?

A

Like all 3 congenital parenchymal abnormalities, with surgery

33
Q

Which lobe is mostly frequently effected by lobar emphysema?

A

The left upper lobe

34
Q

How is Laryngomalacia treated?

A

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

35
Q

What occurs during the Embryonic Stage?

A

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

36
Q

What is Respiratory Distress Syndrome?

A

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

37
Q

When does the Saccular Stage occur?

A

26 - 36 weeks

38
Q

How does RDS present on CXR?

A

Grey everywhere due to diffuse pulmonary edema from lack of surfactant

39
Q

Are males or females more effected by intralobar pulmonary sequestration?

A

Equal among males and females

40
Q

Do Alveoli stop maturing at birth?

A

No, continue to mature up to 8 years after birth

41
Q

When does the Embryonic Stage occur?

A

3 - 6 weeks

42
Q

What effect do antenatal steroids have on a premie?

A

Antenatal steroids given to mother promote surfactant production in the child

43
Q

What are the congenital vascular abnormalities?

A

Vascular rings/slings

Pulmonary arteriovenous malformation

44
Q

What occurs during the Canalicular Stage?

A

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

45
Q

How is Laryngomalacia diagnosed?

A

Bronchoscopy can diagnose Laryngomalacia

46
Q

How is Tracheo-bronchomalacia diagnosed and treated?

A

Bronchoscopy + supportive or surgery if severe

47
Q

How is tracheoesophageal fistula treated?

A

Surgical

48
Q

What occurs during the Pseudoglandular Stage?

A

Airways continue to branch, with terminal bronchioles forming and epithelial cells differentiating

Pulmonary vasculature develops but is far from the Bronchi and Bronchioles

49
Q

What causes Tracheo-bronchomalacia?

A

Etiology unknown, may involve cartilage

50
Q

What is a pulmonary sequestration?

A

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

51
Q

What develops in the Saccular Stage?

A

Terminal sacs form, and Type I and II Pneumocytes differentiate

52
Q

What is a pulmonary arteriovenous malformation?

A

An abnormal connection between arteries and veins

53
Q

What are the 3 congenital parenchymal abnormalities?

A

Pulmonary Sequestration
Pulmonary Airway Malformation
Lobar Emphysema

54
Q

What is a tracheoesophageal fistula?

A

An abnormal connection between the Trachea and the Esophagus

55
Q

What causes Stridor?

A

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

56
Q

How does tracheoesophageal fistula present?

A

Drooling and choking within the first 24 hours of life

57
Q

What are the 3 congenital airway abnormalities?

A

LTT

Laryngomalacia, Tracheo-bronchomalacia, and Tracheoesophageal Fistula

58
Q

What are congenital pulmonary airway malformations?

A

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

59
Q

Which disease produces cartilage rings without a C shape?

A

Tracheo-bronchomalacia

60
Q

How is tracheoesophageal fistula diagnosed?

A

Inability to pass a gastric tube

61
Q

Which lobe is most effected by intralobar pulmonary sequestration?

A

Neither lobe predominates

62
Q

What is an extralobar pulmonary sequestration?

A

An accessory lung separate from the main lung with no functionality

63
Q

What is an intralobar pulmonary sequestration?

A

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

64
Q

How is lobar emphysema diagnosed?

A

Like all 3 congenital parenchymal abnormalities, with radiographic evaluation

Pulmonology (22 decks)

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