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TAS Neonatal Medicine ✅ > Developmental Abnormalities of the Respiratory System ✅ > Flashcards

Developmental Abnormalities of the Respiratory System ✅ Flashcards

1
Q

What can congenital anomalies in the lungs be classified into?

A

Malformations in;

  • Tracheobronchial tree
  • Distal lung parenchyma
  • Pulmonary arterial and venous trees and the lymphatics
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2
Q

At what stage of development do most pulmonary malformations arise?

A

During the embryonic and pseudo glandular stages

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3
Q

Can insults during the neonatal period lead to pulmonary malformations?

A

Yes

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4
Q

How can insults during the neonatal period cause pulmonary malformations?

A

Acute lung injury may alter subsequent alveolar and airway growth and development

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5
Q

What are the most common pulmonary and extra-pulmonary malformations?

A
  • Congenital diaphragmatic hernia
  • Congenital pulmonary airway malformations
  • Tracheo-oesophageal fistula
  • Bronchopulmonary sequestaration
  • Congenital hydrothorax
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6
Q

What is the incidence of congenital diaphragmatic hernia (CDH)?

A

1 in 2000-3000 births

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7
Q

In what proportion of cases is CDH an isolated lesion?

A

2/3

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8
Q

When might CDH not be an isolated lesion?

A

Can be part of a syndrome including chromosomal anomalies

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9
Q

What does CDH result from?

A

Failure of normal development of the diaphragm during the first trimester

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10
Q

What are the types of CDH?

A
  • Posterior lateral defect
  • Anterior or central portion defect
  • Complete absence of diaphragm
  • Eventration
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11
Q

Are posterior lateral CDHs more common on left or right?

A

Left

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12
Q

Which of the types of CDH is not a true hernia?

A

Eventration

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13
Q

What does eventration result from?

A

Failure of muscle development in the primitive diaphragm

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14
Q

How severe is CDH?

A

Produces spectrum of severity, from very mild pulmonary hypoplasia causing minimal clinical compromise, to severe cases that are incompatible with life

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15
Q

How does CDH lead to respiratory compromise?

A

The compression of fetal lungs results in lung hypoplasia

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16
Q

On which side is the lung hypoplasia more severe in CDH?

A

Lung ipsilateral to the defect

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17
Q

What can be compromised in severe cases of CDH?

A

Cardiac function

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18
Q

What happens to infants with CDH following delivery?

A

Can be further cardiorespiratory compromise

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19
Q

What causes further cardiorespiratory compromise following delivery in infants with CDH?

A

Gaseous distention of the intrathoracic gut

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20
Q

When is gaseous distention causing further compromise in CDH a particular hazard?

A

In babies given bag-valve-mask ventilation

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21
Q

What commonly develops as a result of CDH?

A

Pulmonary hypertension of the newborn (PPHN)

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22
Q

Why does PPHN commonly develop in CDH?

A

Due to pulmonary hypoplasia (including abnormalities of the pulmonary vasculature) and poor oxygenation following delivery

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23
Q

When might the presentation of CDH be delayed?

A

In mild cases

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24
Q

What treatment option is used for severe CDH?

A

Fetal surgery

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25
Q

What fetal surgical option is used in severe CDH?

A

Fetal endoscopic tracheal occlusion (FETO)

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26
Q

What is the purpose of FETO in severe CDH?

A

Promote lung growth and limit pulmonary hypoplasia

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27
Q

How is FETO performed?

A

Using fetal tracheoscopy, a balloon is inserted

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28
Q

When is FETO ideally performed for CDH?

A

26-28 weeks

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29
Q

When is the occlusion reversed in FETO surgery for CDH?

A

34 weeks

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30
Q

What is the limitation of FETO for CDH?

A

It offers little benefit, and its use should be limited to clinical trial

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31
Q

How is CDH managed at delivery if diagnosed antenatally?

A
  • Avoid face mask ventilation
  • Intubation and artificial ventilation as soon as possible
  • Continuous drainage (decompression) of gas from abdomen is started
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32
Q

Why is face mask ventilation avoided at birth in CDH?

A

It distends the GI tract

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33
Q

What should be minimised in CDH?

A

Factors that could precipitate PPHN and lung injury

34
Q

How is PPHN managed if present in CDH?

A

Pulmonary vasodilators

35
Q

Is surfactant useful in CDH?

A

No evidence to support its use, except in premature infants with co-existing surfactant deficiency

36
Q

What is the definitive management for CDH?

A

Surgical repair

37
Q

When should surgical repair of CDH be done?

A

When the baby is stable

38
Q

Is ECMO useful in CDH?

A

Able to provide stability and control PPHN, but benefit in CDH is unclear

39
Q

What is the incidence of tracheo-oesophageal fistula?

A

1 in 3000-4500 live births

40
Q

What does a tracheo-oesophageal fistula result from?

A

Failure of the process of separation of the primitive foregut into the respiratory and gastrointestinal tract at 3-6 weeks gestation

41
Q

What is tracheo-oesopahgeal fistula usually found in combination with?

A

Various forms of oesophageal atresia

42
Q

What is the most commonly found combination of tracheo-oesophageal fistula and oesophageal atresia?

A

Oesophageal atresia with distal tracheo-oesophageal fistula

43
Q

What % of cases are of TOF are a combination of oesophageal atresia with distal trachea-oesophageal fistula?

A

91%

44
Q

What is it called when trachea-oesophageal fistula occurs without oesophageal atresia?

A

H-type fistula

45
Q

Are H-type fistulas common?

A

No, extremely rare

46
Q

When do H-type TOF present?

A

Usually after the neonatal period

47
Q

What is the problem in tracheomalacia?

A

Absence or softening in the cartilaginous rings causes the trachea to collapse on expiration

48
Q

What causes the softening of cartilaginous rings in tracheomalacia?

A

A reduction in the cartilage to soft tissue ratio

49
Q

Is the anomaly in tracheomalacia/bronchomalacia segmental or diffuse?

A

Can be either

50
Q

What is bronchopulmonary seqeusteriation?

A

Development of a mass of non-functioning lung tissues, not connected to the tracheo-bronchial tree

51
Q

How does the mass in bronchopulmonary sequestration receive its blood supply?

A

From one or more anomalous systemic arteries arising from the aorta

52
Q

Does bronchopulmonary sequestration occur inside or outside of the visceral pleural lining?

A

Can be either

53
Q

What is it called when bronchopulmonary sequestration occurs on the inside of the visceral pleural lining?

A

Intralobar

54
Q

What is it called when bronchopulmonary sequestration occurs on the outside of the visceral pleural lining?

A

Extralobar

55
Q

How does bronchopulmonary sequestration appear on CXR?

A

As triangular or oval-shaped lung tissue on one side of the chest

56
Q

On what side is bronchopulmonary sequestration more common?

A

Left

57
Q

How is a diagnosis of bronchopulmonary sequestration made?

A

CT or MRI scan of chest

58
Q

What does congenital lobar emphysema result from?

A

Malformation in the bronchial cartilage with absent or incomplete rings

59
Q

How does malformation in the bronchial cartilage result in congenital lobar emphysema?

A

It results in lobar or segmental over-inflation and therefore compression of the remaining ipsilateral lung or lobes

60
Q

What other fetal abnormalities can pulmonary hypoplasia occur secondary to?

A
  • Renal agenesis
  • Oligohydraminos
  • Diaphragmatic hernia
  • Large pleural effusion
  • Congenital anomalies of the neuromuscular system
61
Q

By what mechanisms can other fetal anomalies cause pulmonary hypoplasia?

A

They either restrict physical growth or expansion of the peripheral lung

62
Q

What was congenital pulmonary airway malformation formerly known as?

A

Congenital cystic adenomatoid malformation (CCAM)

63
Q

What is a congenital pulmonary airway malformation (CPAM)?

A

A pulmonary maldevelopment with cystic replacement of smaller airways and distal lung parenchyma

64
Q

What are CPAMs classified on the basis of?

A

Gross appearance and histological features

65
Q

What are CPAMs broadly divided into?

A
  • Macrocystic

- Microcystic

66
Q

What are the features of macrocystic CPAM?

A

Cysts more than 5mm in diameter and visible on fetal ultrasonography

67
Q

What are the features of microcystic CPAM?

A

Cysts smaller and mass has solid appearance

68
Q

What has a better prognosis, microcystic or macrocystic CPAM?

A

Macrocystic

69
Q

What features worsen the prognosis of microcystic CPAM?

A
  • Large mass
  • Mediastinal shift
  • Polyhydraminos
  • Pulmonary hypoplasia
  • Hydrops fetalis
70
Q

What are the cysts connected to in CPAM?

A

The airways

71
Q

What is the result of the cysts being connected to the airways in CPAM?

A

They fill with air

72
Q

What happens when the cysts fill with air in CPAM?

A

They cause compression of the adjacent lungs

73
Q

How can CPAM be treated?

A

Fetal surgery

74
Q

When is fetal surgery used in CPAM?

A

Severe cases associated with hydros

75
Q

What is the main concern with fetal surgery in CPAM?

A

Onset of preterm labour

76
Q

What is congenital pulmonary lymphangiectasis (CPL) characterised by?

A

Marked distention of pulmonary lymphatics

77
Q

Is the distention of pulmonary lymphatics generalised or localised in congenital pulmonary lymphangiectasis?

A

Can be either

78
Q

How does congenital pulmonary lymphangiectasis present?

A

Pleural effusion which is typically chylous

79
Q

How can congenital pulmonary lymphangiectasis be managed if identified antenatally?

A

A catheter shunt inserted between the chest and amniotic cavity

80
Q

What is congenital pulmonary lympheangiectasis associated with?

A

A number of syndromes, including Noonan’s and Down’s syndrome

TAS Neonatal Medicine ✅ (35 decks)

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