SM 166 Congenital and Pediatric Disorders Flashcards
What is Bronchopulmonary Dysplasia?
Term given to infants who had RDS and still require 30% FiO2 at 36 weeks post-menstrual age if < 32 weeks gestational age or at 56 days of life if > 32 weeks gestational age
How are Tracheo-bronchomalacia and Laryngomalacia differentiated?
The sounds they make
How does Laryngomalacia present?
Stridor, Omega shaped Epiglottis and prolapsed arytenoids
How are pulmonary arteriovenous malformations treated?
Embolization, to block off the abnormal connection
What are factors that decrease the risk of VPD?
Ventilation management, antenatal steroids to mother prior to delivery, surfactant therapy
How does Tracheo-bronchomalacia present?
Noisy breathing, but not stridor
How do pulmonary arteriovenous malformations present?
Pulmonary hemorrhage or hemoptysis
What causes rings and slings?
A developmental abnormality of the aortic arch that leads to compression of the esophagus and trachea, either partial (sling) or completely (ring)
How is lobar emphysema treated?
Like all 3 congenital parenchymal abnormalities, with surgery
When does the Pseudoglandular Stage occur?
6 - 16 weeks
Are pulmonary sequestrations intralobar or extralobar?
Intralobar > extralobar, but both are possible
When does the Canalicular Stage occur?
16 - 26 weeks
Which lobe is most effected by extralobar pulmonary sequestration?
The Left Lower Lobe
How is pulmonary sequestration treated?
Like all 3 congenital parenchymal abnormalities, with surgery
How do the causes of intralobar and extralobar pulmonary sequestration compare?
Both can be congenital, but intralobar may also be acquired
Are males or females more effected by extralobar pulmonary sequestration?
Males
What is congenital lobar emphysema?
Overinflation and distension of one or more pulmonary lobes
How is pulmonary sequestration diagnosed?
Like all 3 congenital parenchymal abnormalities, with radiographic evaluation
What occurs during the Alveolar stage
Alveoli continue to mature and develop
How can a ring or a sling be seen on CXR?
Indentation or “grooves” in the esophagus
What causes congenital lobar emphysema?
Intrinsic or extrinsic bronchial narrowing leading to air trapping
Does the Canalicular Stage have surfactant?
No, the Type II Pneumocytes have not differentiated
What are factors that increase the risk of Bronchopulmonary Dysplasia?
Low birth weight, low gestational age, ventilation
What causes intralobar pulmonary sequestration?
May be congenital or due to bronchial obstruction in older patients
When does the Alveolar Stage occur?
36 weeks onward
How many cysts do congenital pulmonary airway malformations cause?
Varied, from a single large cyst to several smaller cysts
What are the 5 stages of lung development?
Every Pulmonologist Can See Alveoli
Embryonic, Pseudoglandular, Canalicular, Saccular, Alveolar
How is pulmonary airway malformation diagnosed?
Like all 3 congenital parenchymal abnormalities, with radiographic evaluation
What causes extralobar pulmonary sequestration?
Congenital
How does congenital lobar emphysema present after birth?
Within hours, a worsening hyperlucent area can be seen on CXR
How do rings and slings present?
Stridor, trouble swallowing, respiratory distress
How is pulmonary airway malformation treated?
Like all 3 congenital parenchymal abnormalities, with surgery
Which lobe is mostly frequently effected by lobar emphysema?
The left upper lobe
How is Laryngomalacia treated?
No treatment unless aspiration or reflux occurs, in which case surgery is indicated
What occurs during the Embryonic Stage?
Outpouching of the foregut into the Mesoderm results in the formation of the Trachea and Bronchi
What is Respiratory Distress Syndrome?
RDS occurs when a newborn has lungs with insufficient compliance, making breathing difficult; typically require ventilation
When does the Saccular Stage occur?
26 - 36 weeks
How does RDS present on CXR?
Grey everywhere due to diffuse pulmonary edema from lack of surfactant
Are males or females more effected by intralobar pulmonary sequestration?
Equal among males and females
Do Alveoli stop maturing at birth?
No, continue to mature up to 8 years after birth
When does the Embryonic Stage occur?
3 - 6 weeks
What effect do antenatal steroids have on a premie?
Antenatal steroids given to mother promote surfactant production in the child
What are the congenital vascular abnormalities?
Vascular rings/slings
Pulmonary arteriovenous malformation
What occurs during the Canalicular Stage?
The primitive alveolar ducts form, and gas exchange can be supported after birth, but lack surfactant
How is Laryngomalacia diagnosed?
Bronchoscopy can diagnose Laryngomalacia
How is Tracheo-bronchomalacia diagnosed and treated?
Bronchoscopy + supportive or surgery if severe
How is tracheoesophageal fistula treated?
Surgical
What occurs during the Pseudoglandular Stage?
Airways continue to branch, with terminal bronchioles forming and epithelial cells differentiating
Pulmonary vasculature develops but is far from the Bronchi and Bronchioles
What causes Tracheo-bronchomalacia?
Etiology unknown, may involve cartilage
What is a pulmonary sequestration?
Normal, non-functioning lung tissue with no connection to the bronchial tree that receives blood supply from the systemic circulation (Aorta); intralobar > extralobar
What develops in the Saccular Stage?
Terminal sacs form, and Type I and II Pneumocytes differentiate
What is a pulmonary arteriovenous malformation?
An abnormal connection between arteries and veins
What are the 3 congenital parenchymal abnormalities?
Pulmonary Sequestration
Pulmonary Airway Malformation
Lobar Emphysema
What is a tracheoesophageal fistula?
An abnormal connection between the Trachea and the Esophagus
What causes Stridor?
A distortion in the airway at the level of the Larynx results in a high pitched inspiratory sound
How does tracheoesophageal fistula present?
Drooling and choking within the first 24 hours of life
What are the 3 congenital airway abnormalities?
LTT
Laryngomalacia, Tracheo-bronchomalacia, and Tracheoesophageal Fistula
What are congenital pulmonary airway malformations?
Congenital cystic and non-cystic lung masses that are due to overgrowth of primary bronchioles which communicate with an abnormal bronchial tree lacking cartilage
Which disease produces cartilage rings without a C shape?
Tracheo-bronchomalacia
How is tracheoesophageal fistula diagnosed?
Inability to pass a gastric tube
Which lobe is most effected by intralobar pulmonary sequestration?
Neither lobe predominates
What is an extralobar pulmonary sequestration?
An accessory lung separate from the main lung with no functionality
What is an intralobar pulmonary sequestration?
Normal non-functioning lung tissue completely covered by normal lung tissue
How is lobar emphysema diagnosed?
Like all 3 congenital parenchymal abnormalities, with radiographic evaluation
