PEDIATRICS Section 5: CHEST (Congenital)

Question 1

Diagnosis? Describe

Answer 1

Pulmonary hypoplasia

This is the situation where the lung(s) look tiny or incompletely developed.

Question 2

What are the secondary causes of Pulmonary hypoplasia?

Answer 2

A. Things inside the chest pushing teh lungs (MC - Diaphragmatic hernia, sometimes - Neurbolastoma, Giant congenital heart or sequestration)

B. Things outside the chest pushing the lungs - fucked up skeleton/ribs - Jeune syndrome - “asphysxiating thoracic dystrophy”

C. Things outside the chest fucking with normal lung development - “Oligohydramios)
Anything that drops fluid (PROM, Potter sequence, REnal problems)

Question 3

Desribe Potter Sequence

Answer 3

Question 4

Most common Diaphragmatic hernia

Answer 4

Bochdalek - B in in the Back

Question 5

Diagnosis

Answer 5

Congenital Diaphragmatic Hernia (CDH) Bockdalek

Question 6

If CDH is on the right, it is associated with?

Answer 6

GBS pneumonia

Question 7

Mortality rate is related to?

Answer 7

Degree of pulmonary hypoplasisa

Question 8

Seen in kids. What is it?
Describe

Answer 8

Bronchogenic cysts`

They are generally solitary and unilocular. They typically do NOT communicate with the airway, so if they have gas in them you should worry about infection.

Question 9

“extra lung that is NOT connected to the airways or vasculature normally.”

Answer 9

Bronchopulmonary Sequestration

Question 10

Other names of Bronchopulmonary Sequestration

Answer 10

Accessory lung tissue or Bronchopulmonary Foregut malformations

Question 11

Diagnosis? Descibe

Answer 11

Bronchopulmonary Sequestration

• NO communication with the airway
• NO communication with the pulmonary arteries
• They - USUALLY have an Aortic Feeder Vessel

Question 12

What is the difference between Intralobar vs Extralobare Bronchopulmonary Sequestration

Answer 12

Question 13

Most common side of Intralobar sequestration?

Answer 13

Left lower lobe (60%)

Question 14

“recurrent pneumonia in the same area”

Answer 14

Intralobar sequestration

Question 15

you have bronchial pathology (maybe atresia depending on what you read), that leads to a ball-valve anomaly and progressive air trapping.

Answer 15

Congenital Lobar Emphysema

Question 16

Diagnosis?

Answer 16

Congenital Lobar EMphysema

Question 17

Diagnosis?

Answer 17

Congenital Cystic Adenomatoid Malformation (CCAM)

Question 18

What are the types of Congenital Cystic Adenomatoid Malformation (CCAM) or CPAM

Answer 18

Type I (Cystic - 70% - most common) and solid types

Question 19

CPAM communicate with the airway and spontaneously decrease when?

Answer 19

3rd Trimester (90%)

Question 20

CCAP Risks and treatment

Answer 20

Malignant transformation:
Pleuropulmonary blastoma
Rhabdomyosarcoma

Question 21

What ifyou see a systemic arterialfeeder (one coming offthe aorta) going to the CCAM ?

Answer 21

Then it’s not a CCAM, it’s a Sequestration. — mumble to yourself “nice try assholes”

Question 22

Left upper lobe:

Left lower lobe:

Answer 22

Question 23

Case

“New born with congenital heart disease”

Answer 23

Extralobar Sequestration

Question 24

Case

“10year old with recurrentpneumonia”

Answer 24

Intralobar Sequestration

Question 25

Give the age:

Intralobar sequestration is seen in?
Extralobar sequestration is seen in?
CLE is seen in?

Answer 25

**Intralobar is seen older kids,
**Extralobar is seen in infants with co-morbids
** CLE is in the upper lobe