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Pulmonology > Congenital and Pediatric D/O > Flashcards

Congenital and Pediatric D/O Flashcards

1
Q

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

A

the embryonic stage

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2
Q

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

A

cartilage, smooth muscle, nerves

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3
Q

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

A

pseudoglandular stage (6 to 16 w)

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4
Q

When does the primitive acinar structure form?

A

pseudoglandular stage (6 to 16 w)

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5
Q

When are vascular connections with the atria established?

A

embryonic stage

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6
Q

When does the pulmonary vasculature develop parallel to the bronchi?

A

pseudoglandular stage (6 to 16w)

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7
Q

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

A

Canalicular stage ( 16 to 26 w)

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8
Q

When is the embryo capable of gas exchange?

A

the end of the canalicular stage (26 w)

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9
Q

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

A

saccular stage (26 to 36 w)

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10
Q

When are epithelial cells fully differentiated?

A

the saccular stage

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11
Q

How does the alveolar stage progress, to make alveoli?

A

septae invaginate into exisiting spaces

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12
Q

Define respiratory distress syndrome

A

newborn without surfactant and difficulty breathing due to increased surface tension

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13
Q

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

A

RDS

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14
Q

When are babies diagnosed with bronchopulmonary dysplasia (BPD)

A

Any infant who required oxygen at 28 days of life

*not graded at this stage

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15
Q

Define mild BPD

A

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

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16
Q

Define moderate BPD

A

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

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17
Q

Define severe BPD

A

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

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18
Q

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

A

BPD (and therefore, RDS by definition)

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19
Q

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

A
  1. corticosteroids prior to premature birth

2. surfactant after birth

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20
Q

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?

A

laryngomalacia

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21
Q

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

A

aspiration and reflux

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22
Q

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?

A

tracheo-bronchomalacia

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23
Q

How do you treat tracheo bronchomalacia?

A

support, surgery if very severe

24
Q

What is a fistula?

A

an abnormal connection between two structures

25
Q

When does a tracheoesophageal fistula present/

A

First day of life

26
Q

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?

A

tracheoesophageal fistula

confirm with barium CXR

27
Q

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

A

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

28
Q

When can pulmonary sequestration present?

A

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

29
Q

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

A

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

30
Q

What types of pulmonary sequestration are there? (3)

A

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

31
Q

What is a bronchopulmonary foregut malformation type pulmonary sequestration?

A

abnormal lung tissue connects to GI

32
Q

How is an intra lobar pulmonary sequestration defined?

A

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

33
Q

what are the possible etiologies of intralobar pulmonary sequestration?

A

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

34
Q

How are most intralobar pulmonary sequestrations drained?

A

into pulmonary veins

35
Q

What is an extralobar pulmonary sequestration?

A

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

36
Q

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

A

LLL

congenital

37
Q

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

A 
intra = pulmonary veins
extra = systemic venous system (azygos or portal)
38
Q

Which kind of pulmonary sequestration is most common in males?

A

extralobar

39
Q

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?

A

congenital pulmonary airway malformation

40
Q

Where in the lungs is CPAM usually found?

A

lower lobes, usually only one

41
Q

How do you classify CPAM?

A

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

42
Q

What is congenital lobar emphysema?

A

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

43
Q

what are the intrinsic causes of congenital lobar emphysema?

A

weakness or absence of underlying bronchial cartilage

44
Q

What are the extrinsic causes of congenital lobar emphysema?

A

mass effects

45
Q

How is congenital lobar emphysema histologically defined?

A

by number of alveoli - poly or hypo

46
Q

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

A

left upper lobe
unilateral
unifocal

47
Q

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

A

slings rings and things

48
Q

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

A

the bronchial tree is compressed

49
Q

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

A

rings/slings

50
Q

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

A

A sling

51
Q

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

A

a ring

52
Q

How do you diagnose a ring or sling?

A

clinical suspicion followed by barium swallow looking for indentations of esophagus

53
Q

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

A

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

54
Q

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

A

embolization

55
Q

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

A

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

Pulmonology (35 decks)

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