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Resp Exam > Congenital Lung Anomalies > Flashcards

Congenital Lung Anomalies Flashcards

1
Q

What is (Pierre) Robin sequence?

A

Micrognathia, glossoptosis, airway obstruction.

This is the original description. There may or may not be cleft palate. Main issues: airway obstruction and feeding.

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2
Q

Most common type of congenital lung anomaly?

A

CPAM - congenital pulmonary airway malformation

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3
Q

Second most common type of congenital lung anomaly?

A

Pulmonary sequestration

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4
Q

What is the most common cause of stridor in newborns?

A

Laryngomalacia

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5
Q

What is the second most common cause of stridor in newborn?

A

Vocal cord paralysis

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6
Q

What are the symptoms of unilateral versus bilateral vocal cord paralysis?

A

Unilateral: no airway obstruction, but issues with aspiration and phonation
Bilateral: issues with airway obstruction, no issues with phonation of aspiration

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7
Q

Is congenital vocal cord paralysis usually bilateral or unilateral? Is further work up required?

A
  • Bilateral
  • MRI brain for look for chiari malformation or hydrocephalus, meningocele
    another cause: myasthenia gravis
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8
Q

Is acquired vocal cord paralysis usually unilateral or bilateral? Causes?

A
  • Unilateral and more often left sided since longer course of recurrent laryngeal nerve
  • Often post op: cardiac surgery, TEF repair , thyroid surgery
  • can happen post birth trauma (congenital) or if mediastinal mass is present
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9
Q

What is the treatment for a newborn with vocal cord paralysis?

A
  • Unilataral: may medialize the cord, feeding interventions since risk of aspiration, but no permanent interventions for the first year since there can be spontaneous recover
  • Bilateral: MOST need tracheostomy, 1/2 of those with an idiopathic cause will spontaneously recover by end of first year (Trach and wait)
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10
Q

Which muscle separates vocal cords for normal breathing?

A

Posterior cricoarytenoid

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11
Q

What type of flow volume is seen with vocal cord paralysis?

A

Variable extrathoracic obstruction

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12
Q

What type of flow volume loop is seen with tracheomalacia?

A

Variable extrathoracic obstruction

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13
Q

What can cause fixed extrathoracic airway obstruction?

A

Tracheal stenosis
Goitre
Subglottic stenosis
Tracheostomy tube

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14
Q

Examples of vascular rings? Most common?

A

Most common: double aortic arch
Second most common: right aortic arch with aberrant left subclavian
Others:
- left aortic arch with aberrant right subclavian and ductus arteriosus
- circumflex aorta

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15
Q

Symptoms of vascular ring

A

More often respiratory than GI presentation

  • Esophageal symptoms don’t usually present till patients begin solid food
  • Respiratory symptoms: stridor, wheeze, apnea, noisy breathing, barking cough, frequent respiratory infections, cyanosis
  • GI symptoms: slow eating, extension of neck during feeds, food being stuck in throat
  • Double aortic arch: often presents in first month of life
  • Right aortic arch with aberrant left subclavian: usually presents at 1-6 months of age
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16
Q

Anomaly associated with pulmonary artery sling?

A

Complete tracheal rings

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17
Q

Pattern of indentation associated with vascular rings?

A
  • Double aortic arch: anterior trachea and posterior esophagus, both with bilateral compression
  • right arch with aberrant left subclavian: bilateral anterior trachea and posterior esophagus compression, but compression is greater on right than left side
  • Pulmonary artery sling: compress anterior esophagus and posterior trachea (compression of right main bronchus and trachea displaced to right)
  • Aberrant right subclavian: posterior esophageal indentation, generally normal trachea on bronch
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18
Q

Describe genetics of AAT

A
  • M = normal allele
  • PI*MM = homozygous for the normal gene
  • PI*ZZ = homozygous for the Z allele, which is the most common mutation. Eg. Z mutation corresponds to a point mutation of Glu342Lys
  • PI*SZ = two different mutation of the gene, increased risk for emphysema
  • PI*SS = no increased risk of emphysema
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19
Q

Indications for genetic testing of AAT

A
  • If a child is symptomatic from liver disease of no clear etiology–>grade A recommendation
  • Surpisingly, adolescent with persistent airflow obstuction–?grade B recommendation, though for adults this ends up being a grade A recommendation
  • Sibling of an individual with AAT deficiency–>grade A
  • Offspring of an individual with AAT deificency–>grade B recommendation
  • WHO: early onset COPD or adult onset asthma
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20
Q

Follow up for AAT, but asymptomatic

A
  • spirometry every 6-12 months
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21
Q

AAT level that would signify deficiency?

A

<20 micromol/L (ATS 2003 statement(

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22
Q

If you are suspicious for AAT, what are the series of steps for diagnosis?

A
  • Start with AAT level in blood, then genetic testing (start with testing common variants, before pursuing gene sequencing). This approach is similar to CF, where we would start with the functional test of the sweat chloride
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23
Q

Does PI*MZ have an increased risk for COPD?

A

If never smoker, then no increased risk for COPD

If they are a smoker, then increased risk for COPD

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24
Q

When considering genetic testing in asymptomatic relatives of patient with AAT, what should you consider?

A
  • genetic counselling to discuss risks/benefits (eg. pyschological effect, genetic discrimination)
  • informed consent
  • consider testing the other parent before testing a child who is unable to consent
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25
Q

Inheritance of AAT?

A

Autosomal co-dominant (but it basically behaves like autosomal recessive)

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26
Q

What are associated anomalies you should consider in a TEF patient?

A

Often associated abnormalities (2/3 of cases), though less likely to have associated abnormalities fo H type

  • Think about cardiac anomalies
  • Associated with VACTERL and CHARGE syndrome:
    • Vertebral, anal, cardiac, tracheoesophageal, renal, radial limb
    • Coloboma, heart, choanal atresia, retardation of growth, genital hypoplasia, ear
    • Associated with trisomy 21, 13, 18
  • Can have associated tracheobronchial anomalies: tracheomalacia, lung agenesis, ectopic bronchi
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27
Q

Long term complications of TEF?

A

Even after repair, the trachea and esophagus are still abnormal:

  • Reflux
  • Aspiration
  • Esophageal stricture or obstruction
  • tracheomalacia
  • bronchospasm
  • recurrence of fistula
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28
Q

What is the most common type of TEF?

A

Type C - proximal atresia and distal fistula

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29
Q

Which type of TEF does not have esophageal atresia?

A

H type (which is type E)

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30
Q

Which type of TEF does not have a fistula?

A

Type A - esophageal atresia, but no fistual

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31
Q

What are the stages of lung development?

A 
Embryonic (3-6 weeks)
Pseudoglandular (6-16 weeks)
Canalicular (16-26 weeks) 
Saccular (26-36 weeks) 
Alveolar (36 weeks to adolescence) 
(Every pulmonologist can see alveoli)
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32
Q

Describe the key points of what happens are each stage of lung development

A

Embryonic: basic branching architecture till 6th generation. Trachea and esophagus are separating
Pseudoglandular: form up to terminal bronchiole and primitive acinar structure, but there is no capacity for gas exchange
Canalicular: formation of respiratory bronchioles and acinar tubules. Formation of alveolar capillary membrane. If there is preterm birth, then gas exchange can be supported if surfactant is given. (There is a small amount of surfactant production and giving glucocorticoids will increase this)
○ saccular (26- 36 weeks)- surfactant ,thinning of alveolocapillary membrane
○ alveolar(from 36 weeks)-septation, proliferation, and thinning of the alveolar walls and tubular elongation of airways

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33
Q

Give examples of lung malformation that can happen at different stages of lung development

A 
Embryonic: 
o   Laryngotracheal malformation
o   TEF
o   Esophageal atresia, stenosis
o   Tracheal stenosis
o   Defects in pulmonary lobe formation
o   Pulmonary hypoplasia, agenesis
o   Bronchogenic cyst
o   Ectopic lobe
o   Extrapulmonary sequestration

Pseudoglandular:

  • Tracheomalacia
  • Bronchomalacia
  • Intralobar bronchopulmonary sequestration
  • Congenital pulmonary airway malformation
  • Acinar dysplasia
  • Alveolar capillary dysplasia +/- misalignment of pulmonary veins
  • Congenital pulmonary lymphangiectasia
  • Pulmonary vascular malformation
  • Diagphragmatic hernia

Cannalicular:
Alveolar dysplasia, alveolar capillary dysplasia, pulmonary hypoplasia of various causes (CDH, thoracic mass, renal agenesis, oligo)

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34
Q

CCHS gene mutation?

A

PHOX2B

Inheritance: autosomal dominant

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35
Q

Monitoring for CCHS patient?

A

All patients need annual PSG, neurocog assessment, echo, holter, optho evaluation (frequency not specified), clinical assessment for hirscupsrung disease
For patient with PARM 28-33/20: annual CXR/abdo U/S to look for neural crest tumor
For patients with NPARM: abdo U/S and urine catecholamine q3 month till 2 years, then every 6 months till 7 years

(If less than 28-33 PARMs, there actually isn’t any additional screening for neural crest tumors)
Purpose of echo is for pulmonary hypertension

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36
Q

Which nerve is responsible for unilateral vocal cord paralysis and which root does it come form?

A
  • Recurrent laryngeal nerve, which comes from vagus nerve (CN 10)
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37
Q

What is a pulmonary sequestration?

A
  • “accessory lung” because pulmonary tissue that is separate from rest of lung by having a systemic vascular supply. It may or may not be connected to the tracheobronchial tree.
  • The systemic vascular supply can come anywhere off the aorta
  • Intralobar versus extralobar
  • Intralobar is more common: within the visceral pleura of the lung, more common, usually detected in adolescence, usually located in posterior segment of LLL, usually presents with recurrent infection/pneumonia
  • extralobar: below LLL, often in abdomen, more common in infants and there can be other associated malformations (eg. cardiac, CDH). usually presents as either incidental finding or respiratory distress due to mass effect
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38
Q

How does pulmonary sequestration present?

A
  • Mass of unclear etiology, mediastinal shift
  • Superinfection - recurrent pneumonia, pseudomonas, aspergillus
  • high output cardiac failure since there is a systemic vascular supply
  • shunting
  • pulmonary hypertension
  • malignancy
39
Q

Treatment for pulmonary sequestration?

A
  • surgery or embolization

- want to delineate the vasuclar supply before doing a procedure

40
Q

What is the relationship between the great vessels and the trachea?

A
  • Left half of trachea and left mainstem bronchus: aorta goes on top of and behind
  • Left pulmonary artery is on top of left main bronchus
  • Right pulmonary artery is on top of right bronchus
41
Q

Where do vascular rings tend to cause airway compression?

A
  • usually at the level of trachea as opposed to mainstem bronchi
  • (pulmonary artery sling can cause mainstem bronchi compression as well)
  • compression of just mainstem bronchi could be due to either pulmonary artery being enlarged. Left mainstem bronchus compression could be due to cardiomegaly.
42
Q

best test to order if you are suspicious of a vascular ring?

A
  • CT chest with contrast
43
Q

Why is it important to differentiate patients with tracheomalacia from those with asthma?

A

Be careful with use of beta agonist, which can relax airway smooth muscle and worsen the tracheomalacia

44
Q

Short term complications post TEF repair?

A
  • Anastomotic leak
  • Re-fistula
  • Aspiration
  • Espophgeal stricture
  • malacia
45
Q

Long term complications?

A
  • tracheomalacia, recurrent LRTI

bronchospasm - 30% rate of airway hyper-reactivity
*
recurrent TEF—>symptoms: coughing/choking/cyanosis with feeds, recurrent pneumonia
*
Recurrent pneumonia
*
Recurrent wheezing episodes, probably an increased risk of asthma, though not all wheezing episodes were ventolin Aspiration due to esophageal stricture
GERD due to poor esophageal peristalsis
responsive

46
Q

PFTs of a patient post TEF repair?

A
  • variable intrathoracic obstruction

- BD reactivity, air trapping

47
Q

General features of CPAM?

A
  • 5 subtypes, 0-4
  • 0: congenital acinar dysplasia so lethal
  • 1: large cysts, most common. Associated with mucinous adenocarcinoma
  • 2: multiple small cysts, sponge like, also common
  • 3: solid, not common
  • 4: peripheral thin walled cysts, not common, associated with pleuropulmonay blastoma
  • complications:
  • 3% risk of infection, air leak or bleeding
  • 4% risk of malignancy
  • surgical management in asymptomatic is controversial, but reasonable given these complications
48
Q

Features of PPB?

A
  • Bilateral or multisegmental cysts

- Dicer 1 mutation

49
Q

If congenital lobar emphysema is asymptomatic, does it need to be removed?

A
  • No, which is in contrast to CPAM and pulmonary sequestration
50
Q

Is a vascular ring more likely to present with respiratory or GI symptoms?

A
  • Respiratory symptoms b/c GI symptoms won’t develop till the infant is on solid foods
    Respiratory symptoms: stridor, wheeze, apnea, noisy breathing, barking cough, frequent respiratory infections, cyanosis
  • GI symptoms: slow eating, extension of neck during feeds, food being stuck in throat
51
Q

From what embryonic layer do lungs develop?

A

Endoderm

52
Q

At what stage of lung development is there production of surfactant?

A
  • cannalicular or saccular (kendig’s says different things in different locations)–>late cannalicular/saccular
  • giving steroids to preterm babies in cannalicular stage will stimulate surfactant production
53
Q

List the stages of lung development, timing, major activities at each stage and developmental problems?

A

EPCSA

Embryonic: development of lung bud, separation of trachea and esophagus, developing basic branching architecture and mainstem bronchi, segmental bronchi. Timing: 3-6 weeks. Problems: TEF, laryngeal cleft, tracheal stenosis, bronchogenic cyst, pulmonary agenesis, defect in lobe formation, extrapulmonary sequestration

Pseudoglandular: 6-17 weeks, ongoing branching including subsegmental bronchi and up to terminal bronchiole, pleuroperitoneal cavity closes. Problems: CDH, CPAM, congenital lymphangiectasis

Cannalicular, 16-26: developmental of respiratory bronchioles and acinus, development of type 1 and type 2 alveolar epithelial cells, some surfactant production at the end of this stage. Problems: prematurity, acinar dysplasia, alveolar capillary dysplasia. Capacity for gas exchange at end of this stage

Saccular, 26-36 weeks: subdivision of alveolar saccules, production of surfactant

Alveolar, 36 weeks to adolescence: secondary alveolar septa, growth of capillary network, thinning of respiratory epithelium. This stage continues till adolescence

54
Q

Diagnosis of tracheobronchomalacia?

A
  • bronchoscopy in an free breathing child is ideal, though technically no gold standard, but this is what the guideline committee does
  • > 50% decrease in tracheal diameter
55
Q

Complications of stent in tracheobronchomalacia? Other treatment options?

A

granulation tissue, migration, encrustation with mucous, stent fracture, vascular erosion, can get airway obstruction from granulation tissue and mucous plugging

Other options:
o Chest physio: often prescribed, but poor evidence basis
Airway clearance is often used, unclear if it reduces the frequency of respiratory infections, few studies looking at airway clearance and mucolytic; they mention use of CPAP to improve peak cough flow (this theoretically makes more sense since the issue is more about central airway clearance than peripheral airway clearance)
o CPAP
o Bronchodilators: theoretical reasons for why bronchodilators may worsen airway obstruction, but bronchodilator non-responsive wheeze should be a clue that something else is going on
o Mucoactive: Cochrane review found likely harm from pulmozyme (I’m not sure what the harm because they just comment on lack of benefit from pulmozyme)
o Antibiotics: theoretical predisposition to PBB (due to vibration and irritation of the airway and reduced mucociliary clearance), lower threshold for antibiotics

-        Surgical:
o   Tracheostomy - last resort 
	* 
Aortopexy: 
	* 
usually done for short segment TM due to TEF, doesn't cure the tracheomalacia, but it just creates more room

o Posterior tracheopexy
o Tracheal resection of short affected segment
o Internal stent - lots of potential complications, for cases where there’s no other options and tracheostomy is not an option. Problems: encrustation with mucous, migration, development of granulation tissue, mucosal hyperplasia, vascular eriosion
o External airway splinting - lo

Managing co-morbidities: 
	* 
GERD 
	* 
Eosinophilic esophagitis 
	* 
immunizations, smoking, vaccines, exercise - general health maintenance 
Ventilatory Pressure: 
	* 
no management algorithms
	* 
can't maintain CPAP for 24 hours
	* 
consider if recurrent infection and sleep disordered breathing 
	* 
may have a role if recurrent infection and poor growth and not able to do other therapies
56
Q

What is narrowest part of airway in child versus adult?

A
  • Child - up till about 8 years of age, below the glottis (subglottic) at level of cricoid cartilage
  • Adult - narrowest part is at the glottis
57
Q

What is the treatment for subglottic stenosis?

A
  • mild acquired SGS may be asymptomatic or symptomatic and observation only is usually fine (grade 1-2)
  • Endoscopy options: radial incisions (cold steel or laser) though stenosis, laryngeal dilation (this is probably same as balloon dilatation), topical or injected steroids and topical mitomycin
  • Most severe (grade 3-4): open airway reconstruction with costal cartilage grafts
58
Q

Infant with upper airway obstruction (Eg. subglottic stenosis), why is there work of breathing?

A
  • “The more negative intrapleural pressure during inspiration against a high airway resistance leads to retraction of the pliable portions of the chest wall, including the intercostal and subcostal tissues and the supraclavicular and suprasternal fossae.”
  • due to very compliant chest wall, which is drawn inwards
59
Q

Which nerve supplies motor part of larynx and where does it come from?

A
  • recurrent laryngeal nerve, which supplies adductor and abductor function
  • it comes from the vagal nerve, CN 10
60
Q

Why is there a higher prevalence (30% versus population level of 8%) of bronchial hyper-reactivity in children with TEF?

A
  • GERD, aspiration, recurrent LRTI
61
Q

What are the indications for use of diuretics in infants with BPD?

A
  • short term improvements in lung compliance, but no sustained reduction in ventilator support, length of stay
    (only 1 small study showed improved abiliy to wean from ventilator with lasix)
  • Cochrane review on aldactazide:
  • improved lung compliance (after 4 weeks of treatment and when treatment initiated at >3 weeks of age)
  • decreased risk of death
  • decreased risk for remaining intubated after 8 weeks
62
Q

What is the role of steroids in BPD?

A
  • late systemic doses can help with earlier extubation (DART study)
    • inhaled steroids have NOT been shown to improve lung function
    • early use of steroids and prolonged use impairs head growth and cognitive development
    • using a short course, high dose (eg. 3-5 days) can help with an acute deterioration
63
Q

Cause of transient tachypnea of newborn?
Risk factors?
Why is there a higher probability with C/S versus vaginal?

A
  • ineffective clearance of fetal lung fluid due to decreased number/activity of epithalial sodium channels (ENAC) and aquaporins
  • relative inactivation of surfactant (until the fetal lung fluid can be absorbed)
  • risk factors: C/S birth with or without labor, male, infant of dibaetic mother, macrosomia, mother with asthma
  • vaginal delivery: spinal flexion and catecholamine surge
  • of note, the absorption of fetal lung fluid happens in third trimester with increased production of catecholmaines. Switch from secretion to absorption of lung fluid
64
Q

CXR findings in TTN?

A
  • fluid in fissure
  • hyperinflation
  • increased vascular markings
  • edema of interlobular septa - but i don’t think you would see this on CXR
65
Q

Imaging findings of RDS?

A

The chest radiographic features of RDS include a low lung volume and the classic diffuse reticulogranular ground glass appearance with air bronchograms

66
Q

What anomalies are associated with complete tracheal rings?

A

in 70% of cases, there is an associated anomaly
- CVS - pulmonary artery sling is most common, but could have another vascular ring (eg. double aortic arch), VSD, ASD, TGA
- tracheal bronchus
pulmonary hypoplasia/agenesis
- T21
- VACTERL

67
Q

Two types of CDH and where do they occur?

A
  • Bochdalek = back = more common–posterior, more common on left
  • morgagni = anterior, less common, more likely to be right sided
68
Q

What are the different types of TEF?

A
  • A: atresia, no fistula
  • B: proximal fistula, distal atresia
  • C: proximal atresia, distal fistula –>MOST common (80%)
  • D: proximal and distal fistula
    H-type fistula: no atresia
69
Q
  1. At which developmental stage do you think TEF occurs
A

Embryonic stage

70
Q

How do babies with TEF usually present?

A
  • Polyhydramnios if esophageal atresia (cause the fetus can’t swallow properly)
  • Esophageal atresia: drooling, respiratory distress, poor feeding, frothing, choking, cyanosis
  • Unable to pass orograstric tube
  • May or may not have gas in stomach, depending on type of fistula
  • Fistula: air from trachea into esophagus causing gastric distension + aspiration risk, recurrent respiratory infections
  • H type: no atresia so if defect is small can go undiagnosed for a while and present with recurrent respiratory infections. In one case series, H type was more commonly treated at 8 months of age
71
Q

Definitive treatment for TEF?

A

Surgical ligation of the fistula with anastomosis of the esophageal segments
Reanastomosis might not be possible if the esophageal remnants are far apart in which case a staged approach might be needed.

72
Q

Neonate with unilateral cystic lung lesion?

A
  • CDH
  • CPAM
  • PIE
73
Q

What side does CDH usually occur on?

A

Left side

74
Q

Two types of CDH and differences between them?

A 
Bochdalek:
Unilateral 
Left sided 
Postero-lateral 
More common 
Presents in neonatal period 
Association: Fryn syndrome, trisomy 13, 18
Defect in diaphragm 
Morgagni:
Bilateral 
If unilateral, then more commonly right sided 
Anterior 
Less common 
Presents later, not in neonatal period 
Cardiac, pentatology of cantrell 
Herniation through a potential space
75
Q

Definitive treatment and timing of surgery with CDH?

A 
- surgery is no longer considered a medical emergency 
Surgical repair (closing defect and reducing abdominal viscera back to abdomen; +/- patch)  - after medically stabilizing and controlling pulmonary hypertension
76
Q

Long term complications of CDH?

A
  • Motor and cognitive abnormalities
  • GERD
  • Foregut dysmotility
  • skeletal abnormalities
  • Uptodate:
  • respiratory infections, chronic lung disease
  • recurrent hernia
  • patch complication - eg. infection
  • GERD
  • intestinal obstruction from adhesions
  • neurodevelopmental abnormalities
  • MDK - pectus excavatum, carinatum, scoliosis
77
Q

Good and bad prognostic antenatal factors for CDH?

A

Good prognostic factors: left sided hernia, intra abdominal stomach, diagnosis after 24 weeks

Bad- Syndromic CDH, Prematurity, right CDH, bilateral hernia, associated major cardiac anomalies, presence of liver in thorax, lower observed/expected lung head ratio

For left CDH, LHR<25% is associated with poor outcome. For right CDH, LHR<40% is associated with poor outcome

78
Q

CDH post natal factor for higher mortality risk?

A

pulmonary hypertension

79
Q

PFT abnormalities on patient with CDH?

A
  • normal or obstructive or restrictive Kendig’s doesn’t commit to which of these is most common
  • bronchial hyper-reactivity (more related to airway dysfunction as opposed to inflammation)
80
Q

What is the risk of malignancy with CPAM?

A

4%

81
Q

Initial management for infant with CDH?

A
  • immediate intubation
  • gentle ventilation (minimize peak inspiratory pressure and avoid large tidal volumes to avoid ventilator associated lung injury)
  • judicious fluid and inotropic support
  • echo to assess for pulmonary hypertension, consider pulmonary vasodilator, iNO, prostaglandin or milrinone, may consider Ecmo
  • delay surgical repair until physiologically stable, but if delayed beyond 2 weeks then there should be a team discussion with the family
  • long term multiD surveillance including neurodevelopmental testing
82
Q

what is scimitar syndrome?

A

Scimitar syndrome — Scimitar syndrome is a variant PAPVC in which part or even the entire right lung is drained by right pulmonary veins that connect anomalously to the IVC (image 1 and movie 2). The affected lung and its associated airways, which are drained by the scimitar vein, are often hypoplastic or have unusual bronchial or vascular distribution patterns. Sequestration as well as aortopulmonary collateral vessels may also involve the affected lung. Other cardiac defects are commonly seen and often include hypoplasia of the left heart or aorta [9]. Left-sided scimitar syndrome has also been described, where some or all of the left pulmonary veins connect to the right-sided IVC [10].

A chest radiograph may show the shadow of these veins as they course and coalesce towards the diaphragm and the IVC, giving the characteristic “scimitar” appearance (image 2).

Symptomatic infants with this diagnosis tend to present with more severe disease and have a poorer prognosis than patients who present as adults or children [9]. At the time of diagnosis, most affected infants have pulmonary hypertension.

83
Q

What is the management of PIE?

A
  • If unilateral, then affected side down
  • Decrease PEEP and PIP to lowest possible values, while maintaining appropriate oxygenation and ventilation
  • HFOV or HFJV to minimize pressure swings, though this has not been definitely proven
  • selective intubation, but this may be challenging
  • (for pneumatoceles, would also want to reduce pressures and consider HFOV/HJV)
84
Q

Risk factors for RDS?

A
  • decreasing gestational age
  • antenatal steroids will lower the risk
  • males have higher risk, females have lower risk
  • race - African americans have lower risk
85
Q

Risk factors for TTN?

A
  • Late preterm—>every week beyond 35 weeks decreases the risk of TTN
    • C/S
		* 
C/S with no labor increases the risk by 3x 
	* 
Infant of diabetic mother
	* 
Macrosomia
	* 
Maternal history of asthma: B adrenergic stimulation active ENAC sodium channels and in infants of asthmatic mothers, they are less responsive to this stimulation
86
Q

Pathophysiology of TTN?

A
  • Ineffective clearance of fetal lung fluid
    • The extra lung fluid will inactivate the surfactant
    • Normal labor causes spinal flexion and catecholamine surge
    • inadequate sodium transport out of airways due to either low number or low function of ENAC sodium channels (there is active transport of sodium through these channels; water follow sodium through aquaporin channels)
    • Decreased number of aquaporins
    • The fact that TTN is more common in late preterm infants is because they have lower levels of surfactant and less Na excretion
87
Q

Imaging findings for BPD?

A
  • diffuse hazziness
    • hyperinflation, atelectasis
    • CT:
    • hyperexpansion, empysema, fibrosis, interstitial abnormalities, mosaic attenuation
88
Q

Post op cardiac surgery. What are some respiratory complications?

A
  • chylothorax
  • subglottic stenosis
  • airway compression from cardiac disease (Eg. atrial enlargemet)
  • vocal cord paralysis from recurrent laryngeal nerve injury
  • diaphragm paralysis
89
Q

Describe general anatomy of the lymphatic system.

A
  • extensive channels
  • lymphatic capillaries which coalesce into channels, like the cisternal chyli (which is below diaphragm) and thoracic duct
  • these channels are kind of like veins, they are important conduits, they can also pump the lymph
  • thoracic duct connects with left and right subclavian veins so all the lymphatic fluid gets back into systemic circulation
  • components of lymphatic fluid:
  • interstitial fluid
  • lymphocytes
  • chylomicrons, triglycerides from the intestinal lymphatics
90
Q

What would make you suspect chylothorax?

A
  • it’s the most common cause of neonatal pleural effusion
  • post op head or CV surgery, including scoliosis surgery
  • milky appearance
  • lymphocytes >80%
  • triglycerides >1.1 if on a fat containing diet
  • sterile
  • pH 7.4-7.8
  • protein content similar to plasma
  • sudan III positive, indicating chylomicrons
91
Q

Differential diagnosis for chylothorax?

A

Congenital:

  • Congenital lymphatic malformation:
  • lymphangiomatosis (isolated)–proliferation of lymphatic vessels, resulting in doughy mass
  • complex lymphatic abnormality–lymphangioma with multiorgan involvement (eg. cardiac, bone, liver, spleen). For lymphangiomatosis and complex lymphatic abnormalities, need to consider surgical resection and sirolimus
  • Lymphangiectasis (aka central conducting lymphatic abnormality) which can be primary or secondary
  • associated with syndrome–T21, noonan, turner
  • hydrops fetalis

Traumatic: head or neck surgery

High central venous pressure: thrombosis of SVC or subclavian vein, post fontan surgery

Compression of thoracic duct by lymph nodes: lymphoma, malignancy, TB, sarcoid, histoplasmosis

Transdiaphragmatic movement of chylous ascites

92
Q

What is a complex lymphatic malformation and examples?

A
  • Lymphangiomatosis = multiple lymphangiomas, which can be found in lung, liver, spleen, bone, mediastinum
      *  CXR: interstitial infiltrate
  *  CT: thickened interlobular septa and bronchovascular bundles, ground glass attenuation 
  *  Biopsy: increased number of dilated lymphatic channels 
  *  Natural history: lymphangiomas grow and compress vital structures 
  *  Management: surgical resection (but no possible if multiple lymphangiomas) or medical (eg. sirolimus, but this is less evidence based)
    • 4 CLAs:
    • Generalized lymphatic abnormality: diffuse lymphatic malformation, chylothorax + lytic bone lesions. Known as “vanishing bone disease”
    • Gorham-Stout: soft tissue mass/swelling, chylothorax
    • Key point: GLA and Gorham Stout have bone involvement
    • Central conducting lymphatic abnormality, also known as lymphangiectasia
    • Kaposiform lymphangiomatosis: soft tissue masses, consumptive coagulopathy, thrombocytopenia (this was the patient we talked about who had chylous pericardial effusion, mass on CT which bled in the OR)
93
Q

Investigations and management of chylothorax?

A
  • Medical management x 4 weeks
      *  Basis: try and decrease lymphatic flow to promote spontaneous healing of the thoracic duct 
  *  Low dietary consumption of long chain fatty acids, but are allowed to have medium chain fatty acids 

      *  MCTs are absorbed directly into portal system, so chylomicrons are not made 
  *  If no improvement with dietary intervention—>NPO, TPN 
  *  If patient is going to respond to conservative medical management, then they should respond within 3 weeks    *  Octreotide: 

  *  synthetic form of the hormone somatostatin, so it decreases splanchnic blood flow—>decreased chyle production    *  In the MAJORITY of cases (80%), medical therapy will work
  • localize site of possible lymphatic rupture with lymphangiography or lymphoscintigraphy
  • CT to look for signs of lymphangiectasis or lymphangioma

Surgical options:
*
Close the thoracic duct:

	*  Open or VATs  
	*  Pleurodesis 
			* 
Eg. intra pleural injection of povidone-iodine 
		* 
Fluoroscopically guided embolization of thoracic duct 
		* 
Radiotherapy 
	* 
Pleuroperitoneal shunt
94
Q

Early onset, severe respiratory distress in neonate?

A
  • ACD-MPV (FoxF1)
  • pulmonary hypoplasia/alveolar simplification
  • PIG
  • surfactant protein B deficiency
  • ABCA3 deficiency
  • TTF-1 (NKX2.1)
  • pulmonary hemorrhage syndrome
  • pulmonary lymphangiectasia
  • Other:
  • PPHN (+/- menconium aspiration)
  • cardiac - TAPVR with obstruction
  • sepsis
  • CDH

Resp Exam (16 decks)

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