Neonatal Lung Disease and Development Flashcards

1
Q

Pediatric airway compared to adults?

A

smaller, more anterior, epiglottis is floppier, larger tongue, larger occiput, narrowest point is the cricoid

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2
Q

Stomodeum?

A

the developing mouth, connected to gut tube

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3
Q

Nasal placode?

A

develops above the Stomodeum, connects back to the gut tube

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4
Q

What arch does upper airway develop from?

A

Pharyngeal arch I

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5
Q

Choana?

A

opening nasal cavity to nasopharnyx

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6
Q

What membrane breaksdown in development?

A

buccopharyngeal membrane

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7
Q

Choanal Atresia?

A

failure in reabsoption of buccopharyngeal membrane during embryonic development
can be membranous or bony, usually mixed
more common in females

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8
Q

Diagnose/manage Choanal Atresia?

A

endoscopy or CT
inital- oropharyngeal ariway
surgery is definitive

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9
Q

Pierre Robin Sequence?

A

multiple related developmental defects

  • Micrognathia
  • Glossoptosis
  • Celft palate
  • Macroglossia

Problem in development of the first pharyngeal arch

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10
Q

Treat Pierre Robin Sequence?

A

most cases- insert of nasopharyngeal airway, severe can require trachesostomy
other-forms of mandibular distraction, release of the floor of the mouth

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11
Q

Micrognathia?

A

small underveloped chini, cause airway obstruction

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12
Q

Congenital airway masses?

A

many causes:

cysts, vascular (hemangiomas), infectious (papillomas), other (Teratomas, Hetertopic brain)

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13
Q

Papillomas cause in newborn?

A

often caused by HPV, can be picked up during childbirth, cause airway obstuction on vocal cords, debreaded can come back

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14
Q

Forms the lining of the airway?

A

endoderm, blebs off from the gut tube

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15
Q

How is the respiratory diverticulum connected to the the foregut?

A

through the laryngeal orifice

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16
Q

How does the respiratory diverticulum seperate from the foregut?

A

tracheoesophageal ridges that partition the tubes

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17
Q

What happens when the epithelium in the area of the laryngeal orifice grows too quickly?

A

it recloses the laryngeal orfice, this is normal

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18
Q

The epithelium later does what to open and its evidence remaining is called what?

A

re-canalized (opens the laryngeal orifice)
leaving the false (vestibular) fold and laryngeal ventricle

the tongue develops on the floor of the mouth

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19
Q

The respiratory diverticulum forms?

A

the primary bronchi, secondary, tertiary

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20
Q

Laryngeal cleft?

A

developmental failure of the primitive tracheoesophageal septum
cause a split like opening between the posterior larynx and the esophagus

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21
Q

Laryngeal cleft presents?

A

recurrent aspiration or cyanotic spells with feeding

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22
Q

Traceheosophageal fistula?

A

always have a component of associated tracheomalacia
Rare H type can present with recurrent aspiration
asses with pressure barium esophogram

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23
Q

Laryngomalacia?

A

Floppiness of the larynx

Most common congetial anomaly of the upper airway

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24
Q

Laryngomalacia present?

A

supine worse than prone (positional)

inspiratory stridor and/or stertor (heavy snoring) within the first few weeks of life

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25
Q

Laryngeal webs or atresias result from?

A

failure of recanalization of the larynx during embryogenesis

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26
Q

where do most lesions occur?

A

the glottic area and extend into subglottic

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27
Q

Subglottic stenosis?

A

can be congenital or aquired

3rd most common congenital laryngeal anomaly

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28
Q

Congential Subglottic stenosis?

A

result from incomplete recanalization of the laryngotracheal tube during the 3rd month of gestation

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29
Q

Risk factors for aquired Subglottic stenosis?

A

prolong intubation, aspiration, prolonged or severe infections, previous airway surgery

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30
Q

Tracheomalasia?

A

weakness (floppiness) of the tracheal wall

segmental/generalized, congenital/aquired

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31
Q

Tracheomalasia preset?

A

recurrent wheezing or barky cough with illness or activity
tends to impair cough efficiency
coarse, low pitch, monophonic wheeze on exam

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32
Q

Thing to watch with Tracheomalasia?

A

Beta agonist can worsen

important because asthma is very common and presents with monophonic wheeze

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33
Q

Bronchogenic cysts?

A

single cyst arising anywhere in bronchial tree, variable in size, abnormal budding of the ventral foregut endoderm

85% mediastinal, most cases located in right parathracheal or canial region
malignant transformation has been reported

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34
Q

Clinical presentation of Bronchogenic cysts?

A

often aymptomatic noted incidentally on chest imaging
depends on location- airway compression, wheeze, respiratory distress
hemorrhage or peptic ulceration in cysts containing gastric mucosa

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35
Q

Bronchogenic cysts management?

A

resected due to risk of malignant transformation
removed thoracoscopically
does not result in loss of any functional lung tissue

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36
Q

Primary bronchial lesions?

A

Bronchomalacia

Bronchial stenosis/atresia

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37
Q

Bronchomalacia?

A

monophonic wheezing
symptoms may persist past age 2, not symptomatic past school age
impair clearance of mucous clearance can lead to recurrent pneumonias
beta agonists worsen
associated with other anatomic anomalies

38
Q

Bronchial stenosis/atresia?

A

can both cause and result from recurrent infection

39
Q

Congenitcal Cystic Adenomatoid Malformation (CCAM)?

A

discrete intrapulmonary mass that contains cysts of variable size
defect in dev of terminal bronchioles leading to structures that resemble a cystic collection bronchioles
malignant transformation known to occur

40
Q

CCAM clinical presentation?

A

Antenatally
-polyhyraminos if lesion compresses the esophagus or hydrops fetalis if it compresses the heart or great vessels
neonatal
-may cause respiratory distress as a result of mediastinal shift and atalectasis (mass effect), or even pneumothorax

often noted incidentally on CXR

41
Q

CCAM management?

A

large antenatal lesions are candidates for surgery, EXIT procedure,etc
most favor resection
resection can be delayed until child reaches appropriate size

42
Q

Bronchopulmonary sequestration?

A

Tissue mass with abnormal communication with tracheobronchial treet
anomalous arterial supply
intrapulmonary, meaning within the normal lunch pleural, extrapulmonary

43
Q

Bronchopulmonary sequestration (BPS) clinical presentation?

A
neonatal
-usually asymptomatic
childhood
-recurrent pneumonias in same location
present even late, noted incidentally on imaging
44
Q

BPS management?

A

managed expectantly if asymptomatic
followed with full PFTs and instructed in airway clearance techniques
Surgical resection recommended for symptomatic cases

45
Q

Congenital large hyperlucent lobe (CLHL)?

A

massive overinflation of one or more lobes
-postnasally following intiation of respiration
abnormal developed bronchus
almost half are located in upper left lobe
right middle and right upper lobe next most common
assoc with congenital heart disease

46
Q

CLHL clincal presentation?

A

neonatal:
-mild to moderate respiratory distress
mediastinal shift may be preent with hyperresonace and decreased breath sounds on involved side

child:

  • recurrent pneumonia, respiratory inection
  • chronic cough, wheez, dyspnea
  • incidental noted on chest imaging
47
Q

CLHL management?

A

observe asymptomatic
gradual resolution
historical- resected due to concern for hyperinflation compressing surrounding lung tissue

48
Q

Development of smallest airways?

A

endorermal cells of the bronchi associate with the vessels of the presumptive lung and flatten

49
Q

flat cells lining the respiratory pathway?

A

become type I pneumocytes

allows for normal gas exchange

50
Q

Type II pneumocytes?

A

begin secreting surfactant, not reach sufficient level until near term
released into the amnion as the fetus, “breathes” fluid

51
Q

Purpose of surfactant?

A
after birth, the amnion is expelled and leaves surfactant to decrease surface tension on the alveoli
prevents atelactasis (collapse of alveoli) and allow easy expansion of lungs
52
Q

Respiratory distress syndrome (RDS)?

A

most common cause of respiratory failure in newborns

lack of mature surfactant production

53
Q

Complications from deficient surfactant?

A
increased surface tension in the alveoli
increased atelectasis
decreased lung compliance
V/Q mismatch
hypoxia
respiratory distress
54
Q

Respiratory distress (inspection)?

A

tachpnea
nasal flaring, head bobbing, grunting
retractions
cyanosis

55
Q

Respiratory distress (ascultation)?

A

decreased air movement

crackles, wheezing, stridor

56
Q

RDS radiographic?

A

reticular granular or ground glass appearances
air bronchograms
poor lung expansion

57
Q

Treat RDS?

A

direct delivery of exogenous surfactant and ventilatory support
long term- bronchopulmonary dysplasia (BPD) and chronic lung disease

58
Q

Meconium Aspiration Syndome?

A

under stress meconium happens in the womb, gets into airway, cause aspiration

59
Q

Meconium?

A

first intestinal discharge from newborns composed of intestinal epithelial cellls, lanugo, mucus, and intestinal secretions (bile)

60
Q

Aspiration leads to disruption of normal lung function?

A

airway obstruction, surfactant dysfunction, direct chemical pneumonitis, resultant presistant pulmonary hypertension

61
Q

Mortality for Meconium Aspiration Syndome?

A

still hight 20%, incidence decreasing

62
Q

Spaces (intraembryonic coelom) develop?

A

in lateral plate mesoderm

63
Q

Intraembryonic coelom then?

A

fuse and expand, eventually they enlarge and seperate the lateral plate mesoderm into a somatic layer and a visceral layer

64
Q

The visceral and the pariental layers?

A

seperate and fold anteriorly

fold to form the gut tube and mesentary

65
Q

The parietal layer, ectoderm, and amniotic cavity fold to?

A

completely surround the gut tube

66
Q

Forms the intraembryonic cavity?

A

somatic layers of the lateral plate mesoderm approach each other anteriorly, they fuse and form

this cavity will produce the pericardial, pleural, and peritoneal cavities

67
Q

Intraembryonic vaity then splits?

A

pleural and the pericardial cavities by the developing diaphragm
they communicate through the left and right pericardioperitoneal canals

68
Q

Pericardioperitoneal canals close as?

A

two pleuroperitoneal folds extend from lateral body to fuse with the septum transversum and esophageal mesentary

69
Q

Create remainder of diaphragm?

A

muscle from the body wall

70
Q

Congential diaphragmatic hernia?

A

abdominal viscera herniating into thorax
faulty development of the septum transversum, should seperate the body cavities during early gestation
typically left sided
ipsilateral lung is hypoplastic
contralateral lung may also be hypoplastic
lesion with significant lung hypoplasia are usually fatal, associated congenital abnromalities common

71
Q

Common weakness of diaphragm?

A

50% Hiatal
36% Posterolateral
7% Eventration

Left side more common

72
Q

Congential diaphragmatic hernia CXR?

A

air in gut tube in throax

73
Q

Clincal presentation Congential diaphragmatic hernia?

A

antenatally:
large CDH often now Dx on prenatal U/S

Neonatal:
babies with significant lung hypoplasia present with severe respiratory distress/failure on DOL1

Infancy/Childhood:
Chronic respiratory symptoms or decreased pulmonary reserve, feeding difficulty or GI symptoms
may be asymptomatic

74
Q

Congential diaphragmatic hernia management?

A

Antenatally
fetal surgery

Neonatal:
supportive care to maintain oxygenation, ECMO

Childhood
Surgical closure of diaphragmatic defect

75
Q

Pulmonary hypoplasia?

A

spectrum- incomplete development of lung tissure to complete lung aplasia

direct compression, low amnitoic fluid volume, fetal respiratory muscle failure

76
Q

diagnose Pulmonary hypoplasia?

A

lung biopsy
clincial suspicion raised by small or absent lung tissue on imagin in the setting of respiratory distress
severe- not compliable with life

77
Q

Eventration of the diaphragm?

A

manifests similarily to a mild congenital disphragmtaic hernia but pushes weakened diaphragm into the thoracic cavity

78
Q

Parasternal and esophageal hernias?

A

result in gut herniation from the abdomen into the thorax

79
Q

formation of the pleural cavity?

A

lungs take up space, push two pleuropericardial folds (phrenic nerves) ahead of them

80
Q

Heart enter embryonic pericardial cavity?

A

before the lungs

81
Q

What happens when the pleuropericardial folds fuse?

A

with each other to seperate the pleuarl cavity from the pericardial cavity anterior to it

82
Q

Extrapleural lesions?

A

Pneumothorax
Chylothorax (milk liquid from lymph)
Hemothorax

83
Q

Number of alveoli present at infancy?

A

as few as half the number of adult

84
Q

infant bronchi?

A

more collapsible, decreased elastin and collagen content

85
Q

Pores of Kohn?

A

Internalveolar canals, not well developed

decreased collateral ventilation, propensity for atelaectasis

86
Q

Ribs orientated horizontal in infants?

A

much more compliant chest wall than adults, much less outward recoil

87
Q

Glottic bakeing?

A

creates PEEP to balance inward elastic recoil, don’t breath out all the way

88
Q

Poiseuille’s law and edema?

A

resistance equals 1 over radius to the 4th
which means a smaller radius in an infant with the same amount of edema will dramatically affect infant more than an adults resistance

89
Q

How to infants breath?

A

nose breathers (inflammatory process causing nasal congestion lead to apnea)

90
Q

Compliance infant?

A

increased airway and chest wall compliance, increase propensity for atelectasis/collapse

91
Q

Airways of babies, size?

A

smaller, exponentially easier to obstruct