Congenital Disorders Flashcards
Key Events in Embryonic Stage
3-6 weeks
outpouching of foregut into mesoderm
Form non-cartilagenous trachea, lobar/segmental bronchi
Vascular connections w/ atria are established
Key Events in Pseudoglandular Stage
braching to terminal bronchioles, pulm vasculature develops parallel to bronchi
Key Events in Canalicular Stage
Branching to alveolar ducts, epithelial differentiation, capillaries surround tubules to form gas exchange region
Key Events in Saccular Stage
Growth of acini, capillary development, differentiated epithelial cells function (surfactant production)
Key Events in Alveolar Stage
Alveoli develop up until adolescence
Bronchopulmonary dysplasia (definition, risk factors, prevention)
RDS infants with long-term abnormalities of airways/parenchyma
Def: on O2 at day 28 of life still
RF: low gestational age, low birth weight, infections, positive pressure ventilation
Prevention: Antenatal Steroids (accelerates epithelial development), ALEC (infant surfactant therapy)
Laryngomalacia (Definition, CM, Dx)
Softening/Floppiness of Larynx
CM: stridor
Dx: Bronchoscopy - omega shaped epiglottis
Tracheo-Bronchomalacia (definition, CM, Dx)
Softening of trachea or main stem bronchi
CM: noisy breathing
Dx: Bronchoscopy
Tracheoesophageal Fistula (definition, CM, Dx)
Abnormal connection b/w trachea and esophagus
CM: Drooling, choking on feedings
Dx: inability to pass gastric tube, or radiographic evaluation (swallow barium and see esophagus atresia)
Pulmonary Sequestration (Definition, Types, Dx)
Normal, non-functioning lung tissue - no connection to bronchi but blood connection from systemic circulation (thoracic aorta)
1. Intralobar - completely surrounded by normal lung tissue/pleura (more common)
2. Extralobar - accessory lung (less common)
3. Bronchopulmonary Foregut Malformation: lung connected to GI tract (very rare)
Dx: CXR
Congenital Pulmonary Airways Malformation
Most common lung malformation
Abnormal cystic and non-cystic lung masses
Congenital Lobar Emphysema
Overinflation and Distention of 1+ pulmonary lobes
Intrinisic: weakness of bronchial cartilage
Extrinsic: mass causes obstruction
Dx: CXR
Vascular Rings, Slings, Other Things
Dx: Barium Swallow with indentations
Sling: Aberrant L PA or R Subclavian - wraps b/w trachea/esophagus or behind esophagus (1 Ba indent)
Ring: Double Aortic Arch - surrounds esophagus/trachea (2 indents)
things: many variants
Pulmonary Arteriovenous Malformations (CM, Dx, Tx)
Abnormal Connection between PA and PV
CM: pulm hemorrhage or hemoptysis
Dx: CXR, Angio
Tx: embolize abnormal capillary bed
