SFP: congenital lung disorders

Question 1

What is the most common congenital lung malformation?

Answer 1

CPAM

Question 2

Briefly describe congenital pulmonary airway malformation.

Answer 2

A cyst or mass in the chest appearing before birth that is made of abnormal benign lung tissue that doesn’t function properly but continues to grow.

Question 3

Describe type 1 CPAM.

Answer 3

There is a low number of larger cysts. This is the most common and usually doesn’t cause symptoms.

Question 4

Describe type 2 CPAM.

Answer 4

Many small cysts. It is often associated with other congenital abnormalities.

Question 5

Describe type 3 CPAM.

Answer 5

High number of large lesions that can cause organs in the chest to shift. This is the most serious one.

Question 6

What are the two types of pulmonary sequestrations?

Answer 6

Intralobar and extralobar.

Question 7

Describe pulmonary sequestration.

Answer 7

Rare congenital condition where a portion of lung tissue doesn’t develop correctly. The abnormal segment has its own blood supply. The nonfunctional tissue can have many small cysts or several large ones.

Question 8

Describe intralobar pulmonary sequestration.

Answer 8

Abnormal tissue forms within the normal lobe; it is the most common and normally requires surgery after birth.

Question 9

Describe extralobar pulmonary sequestration.

Answer 9

Abnormal tissue forms outside the lung. This can often be managed without surgery.

Question 10

Venous drainage from intralobar pulmonary sequestration goes to the ___

Answer 10

Pulmonary vein.

Question 11

In which part/which lung are intralobar pulmonary sequestrations more common?

Answer 11

Left lower lobe.

Question 12

In what case do we see intrapulmonary sequestration in the right lung?

Answer 12

Scimitar syndrome.

Question 13

Where are extralobar sequestrations often found?

Answer 13

Between the diaphragm and the lower lobes.

Question 14

How are extralobar sequestrations often found in women?

Answer 14

As a left retroperitoneal mass.

Question 15

What is the most common arterial supply for extralobar sequestrations?

Answer 15

Descending thoracic aorta.

Question 16

In what instances should pulmonary sequestration be considered in a fetus?

Answer 16

Fetal hydrops, pleural effusion, pulmonary mass, polyhydramnios.

Question 17

In what instances should pulmonary sequestrations be considered in an infant?

Answer 17

Infants with grunting, recurrent respiratory distress, feeding difficulty.

Question 18

In what instances should pulmonary sequestration be considered in adults and children?

Answer 18

Recurrent pneumonia, lung abscess, and hemoptysis.

Question 19

What is needed for a definitive diagnosis of pulmonary sequestration?

Answer 19

Establishment of a systemic arterial blood supply and venous draining of the sequestered tissue.

Question 20

What is on the differential in pulmonary sequestration?

Answer 20

1. CPAM
2. Bronchogenic cyst
3. Focal bronchiectasis
4. Congenital lobar emphysema
5. Retroperitoneal tumors.

Question 21

What factors of pulmonary sequestration indicate poor prognosis?

Answer 21

1. Fetal hydrodrops or pulmonary hypoplasia
2. Large pleural effusion.

Question 22

Which pulmonary sequestrations have better prognosis?

Answer 22

Extra lobar intraabdominal sequestrations; they usually aren’t actively pushing on anything. They do predispose to malignancy, so they’re often removed.

Question 23

What are complications of pulmonary sequestrations?

Answer 23

Recurrent infection in intralobar, hemoptysis in adulthood.

Question 24

What is the most common infectious agent causing infection in pulmonary sequestration?

Answer 24

Pseudomonas.

Question 25

Describe congenital lobar emphysema.

Answer 25

Anomaly of the lower respiratory tract characterized by hyperinflation of one or more pulmonary lobes. This leads to compressed lung tissue and herniation of the affected lobes and displacement of mediastinum.

Question 26

What breathing pattern may be seen in congenital lobar emphysema?

Answer 26

Tachypnea.

Question 27

What are clinical features of congenital lobar emphysema?

Answer 27

Progressive respiratory distress that develops rapidly or gradually, depending on the patient. The severity differs. Infants are normally tachypneic and may be cyanotic.

Question 28

How do we treat congenital lobar emphysema?

Answer 28

It depends on the symptoms. Surgery can be done to resect the impacted lobe.

Question 29

What is the most common clinical symptom of congenital lobar emphysema post-resection?

Answer 29

Wheezing.