Congenital and Pediatric D/O Flashcards
When are the trachea, main stem, lobar and segmental bronchi formed during embryogenesis?
the embryonic stage
In the embryonic stage, what is absent from the trachea and bronchi?
cartilage, smooth muscle, nerves
At what stage does the embryonic tissue begin to differentiate into pneumocytes and other tissue?
pseudoglandular stage (6 to 16 w)
When does the primitive acinar structure form?
pseudoglandular stage (6 to 16 w)
When are vascular connections with the atria established?
embryonic stage
When does the pulmonary vasculature develop parallel to the bronchi?
pseudoglandular stage (6 to 16w)
When do type I and type II pneumocytes begin to differentiate?
Canalicular stage ( 16 to 26 w)
When is the embryo capable of gas exchange?
the end of the canalicular stage (26 w)
When does the embryo switch from branching morphogenesis to the process of alveolarization?
saccular stage (26 to 36 w)
When are epithelial cells fully differentiated?
the saccular stage
How does the alveolar stage progress, to make alveoli?
septae invaginate into exisiting spaces
Define respiratory distress syndrome
newborn without surfactant and difficulty breathing due to increased surface tension
Decreased lung volume, reticulogranular ground glass in air bronchograms, and unclear heart and lung borders are characteristic CXR findings of what congenital condition?
RDS
When are babies diagnosed with bronchopulmonary dysplasia (BPD)
Any infant who required oxygen at 28 days of life
*not graded at this stage
Define mild BPD
breathing room air at 36 weeks post menstrual age if under 32 weeks gestation
or
breathing room air at 56 days oflife if over 32 weeks gestation
Define moderate BPD
breathing less than 30% FiO2 at 36 weeks post menstrual age or 56 days of life
Define severe BPD
breathing more than 30% FiO2 or requiring positive pressure ventilation at 36 weeks post menstrual age
Lower birth weight, lower gestational age, infections, oxygen toxicity and positive pressure ventilation all increase a babies risk of what condition?
BPD (and therefore, RDS by definition)
What 2 treatments are established for patients with BDP in addition to nutrition and ventilation support?
- corticosteroids prior to premature birth
2. surfactant after birth
A baby presents with stridor. Upon bronchoscopy, they are found to have an omega shaped epiglottis, short aryepiglottic folds, and prolapsed arytenoids. What do they have?
laryngomalacia
What are two risks for a laryngomalacia baby that must be evaluated, even if they don’t need surgery?
aspiration and reflux
A baby presents with noisy breathing. Upon bronchoscopy, they have a collapsed trachea due to lack of C shaped cartilage, large membranous spaces, and difficulty breathing. What do they have?
tracheo-bronchomalacia
How do you treat tracheo bronchomalacia?
support, surgery if very severe
What is a fistula?
an abnormal connection between two structures
When does a tracheoesophageal fistula present/
First day of life
On DOL 1, a baby is drooling, has difficulty with feeding due to choking, and a feeding tube is unsuccessful . What does he or she have? How would you confirm?
tracheoesophageal fistula
confirm with barium CXR
What kind of atresia is most common with a tracheoesophageal fistula?
esophageal atresia with a distal tracheoesophageal fistula.
esophagus ends in blind pouch, trachea and esophagus resume further down and do not connect to upper structure
When can pulmonary sequestration present?
as a neonate (RDS), prenatal (ultrasound), or older (recurring infections)
In pulmonary sequestration, where does the nonfunctioning lung tissue get its blood supply? be specific
systemic circulation, i.e. descending thoracic aorta or abdominal aorta
What types of pulmonary sequestration are there? (3)
intra lobar (most common, 3;1)
extralobar
bronchopulmonary foregut malformation
What is a bronchopulmonary foregut malformation type pulmonary sequestration?
abnormal lung tissue connects to GI
How is an intra lobar pulmonary sequestration defined?
extra lung tissue is completely covered by normal lung tissue or visceral pleura
what are the possible etiologies of intralobar pulmonary sequestration?
congenital (lung bud) or acquired (bronchial obstruction -> pneumonia -> parasitization)
How are most intralobar pulmonary sequestrations drained?
into pulmonary veins
What is an extralobar pulmonary sequestration?
an accessory lung that is covered by visceral pleura and separated from the functioning lung
What lobe is most commonly affected by extralobar sequestration? What is the etiology?
LLL
congenital
What is the difference between venous drainage in intra v. extralobar sequestration?
intra = pulmonary veins extra = systemic venous system (azygos or portal)
Which kind of pulmonary sequestration is most common in males?
extralobar
Which condition is defined by overgrowth of the primary bronchioles and formation of cystic lung masses in communication with the abnormal bronchi, which lack cartilage?
congenital pulmonary airway malformation
Where in the lungs is CPAM usually found?
lower lobes, usually only one
How do you classify CPAM?
Stocker classification (0-4 by cysts and level of involvement)
What is congenital lobar emphysema?
overinflation or distention of pulmonary lobes due to either intrinsic or extrinsic causes
what are the intrinsic causes of congenital lobar emphysema?
weakness or absence of underlying bronchial cartilage
What are the extrinsic causes of congenital lobar emphysema?
mass effects
How is congenital lobar emphysema histologically defined?
by number of alveoli - poly or hypo
Which lobe is usually affected in congenital lobar emphysema? is it usually bilateral? is it multifocal?
left upper lobe
unilateral
unifocal
What are the three types of mediastinal vasculature abnormalities that we studied?
slings rings and things
What part of the pulmonary system is affected by rings/slings?
the bronchial tree is compressed
A baby presents with trouble swallowing, stridor, RDS, recurrent pneumonia, and episodes where they stop breathing. What do they have?
rings/slings
What is an aberrant pulmonary left artery or right subclavian usually an example of in congenital pulmonary abnormalities?
A sling
What is the effect of a double aortic arch (usually) in congenital pulmonary abnormalities?
a ring
How do you diagnose a ring or sling?
clinical suspicion followed by barium swallow looking for indentations of esophagus
A baby presents with asymptomatic hypoxia which progresses to hemoptysis and signs of pulmonary hemorrhage. What should be on your differential?
pulmonary arteriovenous malformations (MVA)
* don’t know if it is actually progressive, but any one of these things in isolation should also make you think of an MVA
How do you treat a large AVM that is causing symptoms?
embolization
A baby has a mutation in activin receptor kinase (ALk1) endoglin, or SMAD4. How might they present?
They have hereditary hemorrhagic telangectasia. They can have telangectasias of the nose, mouth, hands, lung, GI, or brain
