SM 166a - Congenital and Pediatric Disorders

Question 1

You suspect an airway abnormality in your 3 month old patient. You can hear stridor when they breathe, and bronchoscopy reveals an omega-shaped epiglottis.

What is the most likely diagnosis?

How is it treated?

Answer 1

Laryngomalacia

Evaluate for aspiration and reflux, treat if necessary

The laryngomalacia may resolve on its own, surgery may be necessary if severe

Question 2

What defines bronchopulmonary dysplasia?

How is severity defined?

Answer 2

Continued airway and parenchymal abnormalities in an infant with RDS.

Severity is defined by the amount of supplemental oxygen required at:

36 week post-menstrual age (if the baby was born at <32 weeks gestational age)

OR

56 DOL if (if the baby was born ≥32 weeks gestational age)

• Mild
  
  • Breathing room air
• Moderate
  
  • Breathing <30% F_iO2
• Severe
  
  • Breathing >30% F_iO2 or requiring positive pressure ventilation

Question 3

At what stage of embryonic development do alveolar cells differentiate into type 1 and type 2?

Answer 3

Saccular stage (24-36 weeks/birth)

Question 4

What causes laryngomalacia?

Answer 4

Floppiness/softening of the larynx

Any of the following are possible:

• Omega-shaped epiglottis
• Short aryepiglottic folds
• Prolapsed arytenoids

Question 5

What abnormality is shown in this image?

Answer 5

Vascular sling: aberrant left pulmonary artery

The arrow is pointing to the left artery that is sneaking between the esophagus and the trachea

Question 6

How is a vascular ring or sling diagnosed?

Answer 6

Barium swallow

Will show compression of the esophagus or bronchial tree

Question 7

What congenital lung malformation is shown in this x-ray of a 2-month old girl?

Answer 7

Congenital pumonary aiway malformation (CPAM)

The arrow is pointing to a large systic mass

Question 8

What congenital lung malformation is shown in these pictures?

Answer 8

Tracheo-Bronchomalacia

Question 9

What are the risk factors for bronchopulmonary dysplasia?

Answer 9

• Low birth weight
• Low gestational age
• Infections (maternal and infant)
• Positive pressure ventilation
• O2 toxicity

Question 10

What is the treatment for laryngomalacia?

Answer 10

Evaluate for aspiration and reflux, treat if necessary

The laryngomalacia may resolve on its own, surgery may be necessary if severe

Question 11

What is a bronchopulmonary foregut malformation?

Answer 11

Abnormal lung tissue is connected to the GI tract

Question 12

What are ongenital pulmonary airway malformations?

Answer 12

A heterogeneous group of congenital cystic and non-cystic lung masses characterized by extensivve overgrowth of the primary bronchioles in communication with the abnormal (lacks cartilage) bronchial tree

Question 13

Why are mothers at risk for preterm delivery prescribed steroids prior to 34 weeks?

Answer 13

To decrease the infant’s risk of bronchopulmonary dysplasia

Question 14

What is congenital lobular emphysema?

Answer 14

Overinflation or distension of one or more pulmonary lobes

May have intrinsic or extrinsic causes

Question 15

What congenital lung malformation is shown in this CT scan of a 2-month old girl?

Answer 15

Congenital pumonary aiway malformation (CPAM)

The arrow is pointing to a large systic mass

Question 16

What is an intralobular pulmonary sequestration?

Answer 16

Extra lung tissue si sompletely covered by normal lung tissue OR by a segment of visceral pleura of the associated lung lobe

May be congenital or acquired

Question 17

What is respiratory distress syndrome (RDS) in newborns?

Answer 17

A newborn has RDS if their lungs do not have enough surfactant

• Often seen in premature infants
• Surface tension increases
• Compliance decreases
• Breathing requires more effort

Question 18

Describe the clinical presentation of a pumonary AVM

Answer 18

Asymptomatic hypoxia

OR

Bleeding in the lungs (pumonary hemorrhage, hemoptysis

Question 19

List 3 congenital airway abnormalities

Answer 19

Laryngomalacia

Tracheo-bronchomalacia

Tracheoesophagel fistula (TEF)

Question 20

How is a pumonary AVM diagnosed?

Answer 20

chest x-ray or angiography

Question 21

How is a pulmonary AVM treated?

Answer 21

Embolization if it is large enough; the vessel is occluded so blood does not enter the AVM

Question 22

What is the treatment for tracheo-bronchomalacia?

Answer 22

Surgery if severe

Question 23

List the 3 major types of pulmonary sequestration

Answer 23

• Intralobular pulmonary sequestration (most common)
• Extralobular pulmonary sequestration
• Bronchopulmonary foregut malformation

Question 24

What are the etiologies of intralobular pulmonary sequestration?

Answer 24

• Congenital
  
  • ​Sequestration arises from the lung bud during development
• Acquired
  
  • ​Sequestration develops after bronchial obstruction
    
    • ex: pneumonia -> no perfusion if no ventilation -> parasitation of pulmonary arteries

Question 25

Describe the presentation of tracheo-bronchomalacia

Answer 25

* Noisy breathing caused by trachea and mainstem bronchi floppiness * Lack of C-shape to the airways

Question 26

What congenital lung abnormality is present in this CT scan of a 2-day old girl?

Answer 26

Congenital lobar emphysema Note: hyperinflated LUL with attenuated lung markings, mediastinal shift to the right side

Question 27

What abnormality is shown in this image?

Answer 27

**Double aortic arch** - a type of vascular ring around the esophagus and bronchial tree

Question 28

What is a pulmonary atriovenous malformation (AVM)?

Answer 28

A pulmonary AVM is an abnormal connection between arteries and veins that causes blood to skip the capillary bed (Basically a shunt)

Question 29

What congenital lung abnormality is present in this x-ray of a 2-day old girl?

Answer 29

Congenital lobar emphysema Note: hyperlucency in the LUL

Question 30

Where are extralobular pulmonary sequestrations most commonly located?

Answer 30

Left lower lobe Some (~10%) are below the diaphragm

Question 31

What is pulmonary seqestration? (congenital lung abnormality)

Answer 31

Normal, non-functioning lung tissue w/o a connection to the bronchial tree Recieves blood from the systemic circulation

Question 32

What factors decrease an infant's risk of bronchopulmonary dysplasia?

Answer 32

* Antenatal steroids given to the mother prior to delivery * Surfactant therapy for the infant * Nutritional support to increase weight * Ventilator management * Don't overventilate! Positive pressure ventilation and O2 toxicity increase an infant's risk of BPD

Question 33

What is the difference between a vascular ring and a vascular sling?

Answer 33

* Ring * Completely surrounds the esophagus and bronchial tree * Squishes teh trachea and esophgaus together * Sling * Wraps around the esophagus and bronchial tree * Must sneak back between the trachea and the esophagus

Question 34

What congenital lung malformation is pictured?

Answer 34

Tracheoesophagel fistula (TEF)

Question 35

What lung abnormalityis indicated by the arrow?

Answer 35

Intralobular pulmonary sequestration

Question 36

Describe the clinical presentation of a vascula ring or sling

Answer 36

* Trouble swallowing * Stridor * Respiratory distress * Recurrent pneumonea * Apnea

Question 37

Describe the circulation of an intralobular pulmonary sequestration Blood Supply: Blood Drainage:

Answer 37

Blood supply: Systemic circulation (ascending or abdominal aorta) Blood Drainage: Pulmonary veins

Question 38

How are vascular rings and slings treated?

Answer 38

Surgery

Question 39

Describe the circulation of an extalobular pulmonary sequestration Blood Supply: Blood Drainage:

Answer 39

Blood supply: Systemic circulation (ascending or abdominal aorta) Blood Drainage: Systemic venous circulation (azygous or portal)

Question 40

What causes tracheo-bronchomalacia?

Answer 40

Floppiness in the trachea and mainstem bronchi Lack of C-shape to the airways

Question 41

How is tracheoesophageal fistula diagnosed?

Answer 41

* A tube cannot pass from the mouth to the stomach * Radiography: no shadow from the esophagus

Question 42

What is the etiology of extralobular pulmonary sequestration?

Answer 42

Usually congenital

Question 43

What congenital lung malformation is shown in this CT scan of a 1-day old boy?

Answer 43

Congenital pumonary aiway malformation (CPAM)

Question 44

What is a vascular ring or sling?

Answer 44

Abberations in the mediastinal vasculature caused by alterations in the **aortic arch system** that cause **compression of the bronchial tree.**

Question 45

Which lobe is most frequently affected by congenital lobar emphysema?

Answer 45

Upper left lobe Congenital lobar emphysema is rarely bilateral or multifocal

Question 46

Describe the presentaton of tracheoesophageal fistula (TEF)

Answer 46

At DOL 1: * Infant can't handle secretions: they drool a lot * Choking with feeding

Question 47

Describe the presentation of laryngomalacia

Answer 47

* Stridor caused by an omega-shaped epiglottis * Short aryepiglottic folds * Prolapsed arytenoids Overall floppiness/softness of the larynx

Question 48

What is an extralobular pulmonary sequestration?

Answer 48

An **accessory lung** covered by visceral leura, separated from the functional lung

Question 49

What is the treatment for tracheoesophageal fistula?

Answer 49

Surgery to disconnect the trachea and esophagus

Question 50

What causes congenital lobar emphysema?

Answer 50

* Intrinsic * Weakness/absence of underlying bronchial cartilage causes the lobe to collapse * Extrinsic * Mass effect: pushing on the bronchi means that air cannot get out Either etiology can lead to bronchial narrowing, air trapping, and overinflation/distension fo the lung

Question 51

Name 2 congential lung malformations that are vascular abnormalites

Answer 51

* Vascular rings/slings * Pulmonary atriovenous malformations