The most frequent arterial supply of a sequestration
What is the thoracic aorta?
100
recurrent pulmonary infections and/or hemoptysis
What is pulmonary sequestration?
100
Most common type of CCAM, consists of large cystic spaces within a single pulmonary lobe
What is type I? (55% of cases)
200
By week 4 the lung buds have formed and there is separation of the trachea from the esophagus
What is the embryonic phase?
200
TEF without esophageal atresia
What is type E (H type)?
200
most common venous return of sequestration
What is the pulmonary veins?
200
feeding difficulty, excessive salivation, cyanosis, or recurrent infections
What is tracheobronchial esophageal fistula?
200
Type of CCAM consisting of numerous small cysts lass than 20 mm in diameter
What is Type II? (40%)
300
Complete development of the bronchial tree and pulmonary arteries
What is the pseudoglandular phase?
300
Esophageal atresia without TEF
What is Type A?
300
This percentage of sequestrations are extralobar (ELS)
What is 25%?
300
usually asymptomatic, often found incidentally on CXR
What is bronchogenic cyst?
300
mediastinal shift, pulmonary hypoplasia, polyhydramnios, and hydrops in the fetus most often occurs with this CCAM Type
What is Type I?
400
Development of the respiratory bronchioles and alveolar ducts. Ends with development of the pulmonary venous system
What is the Cannicular phase?
400
Esophageal atresia with proximal TEF
What is Type B?
400
90% of extralobar sequestrations are here
What is at the base of the left lung? (just above the dome of the diaphragm)
400
Present with exertional wheezing or stridor after birth
What is congenital tracheal stenosis?
Type 1 - entire trachea
Type 2 - funnel shape of upper, lower, or entire trachea
Type 3 - segmental stenosis of lower trachea
400
The underlying mechanism for the development of CCAM
What is segmental bronchial atresia? (perhaps from disruption of fetal lung bud development or disruption of fetal bronchial circulation)
500
Primitive alveoli proliferate while becoming intimately involved with the surrounding capillaries
What is the terminal sac phase?
500
Esophageal atresia with proximal and distal TEF
What is Type D?
500
Intralobar sequestrations communicate with normal lung via these
What are the pores of Kohn?
500
may present prenatally as polyhydramnios or as neonatal acute respiratory distress with multiple air fluid levels on CXR
What is Congenital Cystic Adenomatoid Malformation (CCAM)?
500
malignancies reported in association with CCAM (name at least 2)
What is pleuropulmonary blastoma,
rhabdomyosarcoma,
squamous cell carcinoma
bronchioalveolar carcinoma (the most common to arise in CCAM, almost always in Stoker type I lesions)