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?

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?

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
What is pulmonary sequestration?
Aberrant non-functional masses of normal lung tissue that do not communicate wit the normal tracheobronchial tree and have a systemic vascular supply.
26
What is empyema?
Collection of pus outside of the lungs due to infection (acquired pathology)
27
Where is oxygenation coming from in the fetus?
From the placenta (NOT he lungs!)
28
How does blood circulate in the fetus?
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
Why might a lung congenital defect not become apparent until some weeks after birth?
Because it takes some times after birth for the fetal circulation to transition to the neonatal circulation (blood starts circulating towards lungs)
30
What is a congenital diaphragmatic hernia?
Failure of development of the posterolateral segment of either hemidiaphragm
31
How do we diagnose a congenital diaphragmatic hernia?
32
What is eventration of the diaphragm?
It can be congenital or acquired, and can be asymptomatic or cause respiratory distress due to paradoxical diaphragmatic motion.....
33