Embryology - Clinical aspects of congenital embryonic abnormalities Flashcards

1
Q

What is the main determinant of the survival of newborn babies?

A

Their lung health

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2
Q

The esophagus gives rise to…

A

the tracheal bud (i.e. the trachea and the esophagus share a common ancestor)

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3
Q

In healthy development, the esophagus and the tracheal tube should…

A

completely separate (NO fusion)

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4
Q

The endoderm gives rise to…

A
  • airways epithelium
  • airway glands
  • pulmonary epithelium
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5
Q

The mesoderm gives rise to…

A
  • connective tissue
  • cartilage
  • smooth muscle
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6
Q

What is atresia?

A

When a luminal structure becomes blind-ended

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7
Q

85% of abnormalities of tracheal budding are…

A

???

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8
Q

What are the symptoms of esophageal atresia and tracheo-esophageal fistula?

A
  • excess salivation
  • respiratory distress
  • choking on feeds
  • maternal polyhydraminos
  • baby cannot swallow amniotic fluid
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9
Q

How is a fistula usually detected?

A

The baby cannot swallow amniotic fluid, and the stomach is smaller than normal (because the baby is not swallowing amniotic fluid)

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10
Q

What is a bronchopulmonary foregut malformation?

A

It is a cystic remnant of the budding process of the trachea. It is found in the mediastinum. It makes the baby prone to infection.

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11
Q

What are the symptoms of bronchopulmonary foregut malformation?

A

Can be asymptomatic or cause acute or chronic aero-digestive tract symptoms

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12
Q

Histologically, what do you see in a bronchopulmonary foregut cyst

A

Both GI muscle pattern and ciliated columnar epithelium typical of respiratory airways.

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13
Q

By 24 weeks how many orders of branches have formed in the lungs?

A

17

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14
Q

How long does the alveaolar development period continue?

A

From 32 weeks of development to 8 years of life!

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15
Q

What happens during the canalicular period of development?

A
  • Bronchi and terminal bronchioles become larger
  • Lung becomes highly vascular
  • Respiratory bronchioles and alveolar ducts develop
  • Terminal saccules (primordial cavities) are now capable of gas exchange
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16
Q

What happens during the terminal saccular period?

A
  • Increased number of terminal saccules
  • TS epithelium…
17
Q

What is the purpose of surfactant therapy?

A
18
Q

When is the alveolar period I? When is the alveolar period II?

A

Alveolar period I: 32 weeks-birth
Alveolar period II: birth - 8 years

19
Q

Factors necessary for normal lung development.

A
  • Adequate thoracic space for lung growth
  • fetal breathing movements
  • adequate amounts of amniotic fluid
20
Q

What is Potter’s sndrome?

A

No production of amniotic fluid, so lungs do not develop and the babies die of respiratory failure.

21
Q

What is an aplastic lung? What is an aplastic lobe?

A
22
Q

What is congenital lobar emphysema?

A

Failure of bronchial mesenchyme
Dysplastic bonchial cartilage collapses during expiration
Severe air trapping and hyperinflation of affected lobe

23
Q

What is congenital pulmonary airways malformation (CPAM)?

A

A segment of the lung arrests in development early (in the pseudoglandular period). There is an overgrowth of bronchioles and a deficiency in the number of alveoli in the segment. The affected segment is cystic and glandular (adenomatoid).

24
Q

Symptoms of CPAM are due to…

A

presence of a large non-functional mass or delayed infections

25
Q

What is pulmonary sequestration?

A

Aberrant non-functional masses of normal lung tissue that do not communicate wit the normal tracheobronchial tree and have a systemic vascular supply.

26
Q

What is empyema?

A

Collection of pus outside of the lungs due to infection (acquired pathology)

27
Q

Where is oxygenation coming from in the fetus?

A

From the placenta (NOT he lungs!)

28
Q

How does blood circulate in the fetus?

A

There is almost no blood flow to the lungs, because the pulmonary vascular resistance is very high so the blood is shunted from the lungs. A soon as the baby is born and takes its first breath, the resistance drops and blood begins to circulate towards the lung.

29
Q

Why might a lung congenital defect not become apparent until some weeks after birth?

A

Because it takes some times after birth for the fetal circulation to transition to the neonatal circulation (blood starts circulating towards lungs)

30
Q

What is a congenital diaphragmatic hernia?

A

Failure of development of the posterolateral segment of either hemidiaphragm

31
Q

How do we diagnose a congenital diaphragmatic hernia?

A
32
Q

What is eventration of the diaphragm?

A

It can be congenital or acquired, and can be asymptomatic or cause respiratory distress due to paradoxical diaphragmatic motion…..

33
Q
A