Congenital Disorders Of Lung

Question 1

CPAM

• define
• types
• Ca risk

Answer 1

Harmatomatous or dysplastic lung mixed with normal tissue confined to one lobe
1-4 per 100,000
Type 0 microcystic disease throughout lungs, poorest prog, least common
Type 1 (60%) single or several large cysts in one lobe, 30% mucus secreting cells, good prog
Type 2 (20%) microcystic, associated renal, CHD, CDH, poor prog
Type 3 (<10%) mixed microcystic & solid tissue
Type 4 (10%) macrocystic, lacks mucus cells, associated pleuropul blastoma

Sarcomatous or carcinoid degeneration

Question 2

Sequestration 
Define 
Blood supply 
Intra vs extra pulmonary 
Rx

Answer 2

Tissue in lung not connected to bronchus which does not participate in gas exchange and receives blood supply from aorta and venous drainage to right heart
Acts as a space occupying lesion
Intra: within lower lobe pleura. Px w infection hemoptysis
Extra: boys; left lung. Own pleura. Associated w CDH, colonic duplication, pulmonary hypoplasia, vertebrae anomalies
Surgery to remove

Question 3

Bronchogenic cysts
Arise when 
Commonly found where
Lined with
Sx
Rx

Answer 3

Abnormal budding before 16w
Lined with ciliated epithelium
Found on right near a midline structure eg trachea, oesophagus, carina
Fever, CP, productive cough sometimes dysphagia
Seen on xr; +- air fluid level
Excision

Question 4

Pulmonary lymphangectasia

Three causes

Answer 4

Pulmonary venous obstruction 
Generalised lymphangectasia (assoc Noonan syn)
Primary lymphangectasia: abnormal lymph development in lung 

Hydrops, resp distress in neonate, chylothorax

Supportive mgmt, correction of CHD