Congenital Disorders

Question 1

Lung Morphogenesis Stages

Answer 1

Embryonic:            3-8 weeks
Psuedo-Glandular: 5-17 weeks
Canalicular:          16-26 weeks
Saccular:              24-38 weeks
Alveolar:               36 weeks - 3 years

Question 2

How many alveoli do you have at birth and at maximum?

Answer 2

100-150 million alveoli at birth
200-600 million at 3-8 years
Increased size thereafter

Question 3

Types of Congenital Lung Disease and Examples

Answer 3

Embryonic
Pseudoglandular
Diaphragm development

Embryonic:
Tracheal, laryngeal stenosis
Pulmonary agenesis
Tracheo-oesophageal fistula

Pseudoglandular:
Pulmonary sequestration
Cystadenomatoid malformation
Cyst formation

Diaphragm:
Pulmonary hypoplasia
Persistent Pulmonary hypertension
Eventration
L>R(?)

Question 4

Functional Changes in Lung at Birth

Answer 4

Change from fluid secretion to fluid absorption

Pulmonary vasodilation

Question 5

When is Surfactant produced?

Answer 5

Between weeks 24-28

By week 35, surfactant should be sufficient to prevent lung collapse

Question 6

Examples of Neonatal Lung Disease?

Answer 6

Surfactant deficiency
(Hyaline membrane disease)

Chronic Neonatal Lung Disease - increased severity bronchiolitis

Question 7

What is a Fletcher Peto diagram?

Answer 7

Graph showing FEV1 change over time.

Susceptible smokers deteriorate faster, cessation returns to original rate of decline.