NEO RESP

Question 1

what are the differentials for respiratory distress that is airway?

Answer 1

Choanal atresia
Tracheal stenosis
Laryngeal web
Vascular Ring

Question 2

what are the differentials for respiratory distress that is thoracic?

Answer 2

Ribcage anomalies
(Jeune syndrome –
asphyxiating thoracic
dystrophy)
Air leak syndromes
Chylothorax

Question 3

What are the differentials for respiratory distress that is of neuromuscular origin?

Answer 3

Neuromuscular
Spinal cord injury
Spinal muscular
atrophy
Phrenic nerve palsy
Myasthenia gravis
Muscular dystrophy

Question 4

What are the differentials for respiratory distress that are nonspecific?

Answer 4

Other
Diaphragm eventration
Pain
Sepsis
Methemoglobinemia
Anemia

Question 5

cyanotic with feeds,
with resolution during crying–> dx?

Answer 5

choanal atresia

Question 6

what syndromes are associated with choanal atresia?

Answer 6

CHARGE association, Apert’s syndrome, DiGeorge syndrome, trisomy 18, Treacher Collins syndrome, and camptomelic dysplasia.
Approx 50% patients have other anomalies,
most commonly CHARGE syndrome

Question 7

whats more common u/l or b/l choanal atresia?

Answer 7

u/l 2/3 and R>L
* Bilateral CA present early, more commonly
associated with other anomalies, unilateral
usually isolated

Question 8

more choanal atresia in f vs m? or m>f?

Answer 8

F>M

Question 9

what injury can you have that leads to respiratory distress with shoulder dystocia? waiters tip / erbs palsy

Answer 9

phrenic nerve palsy
Phrenic nerve injury – most commonly on right side after birth trauma

• Etiology – lateral neck
  hyperextension at birth, affects
  C3‐C5
• Approximately 75% have
  associated injuries (brachial
  plexus, Horner’s)

Question 10

what does the cxr show with phrenic nerve palsy?

Answer 10

CXR with elevated hemidiaphragm with atelectasis
* Fluoroscopy diagnostic – paradoxical
movement of diaphragm – elevation with inspiration, descent with expiration
* Improvement over 2‐3 weeks, further over next few months
* If failure of improvement in respiratory status, may require diaphragm plication

Question 11

what kind of nerve injury with breech with neck hyperextension?

Answer 11

spinal cord injury

Question 12

brainstem injury what happens?
at or above c3?
Below C3?

Answer 12

Level of injury:
* Brain stem – can be fatal
* At or above C3 – phrenic nerve is also involved
* Below this level – Phrenic nerve is intact and diaphragm contracts vigorously to compensate for weak intercostal and abdominal muscles

Question 13

• Vigorous use of diaphragmatic muscles in a hypotonic infant – rule out what?

Answer 13

• Vigorous use of diaphragmatic muscles in a hypotonic infant – rule out spinal cord injury

Question 14

decreased risk for
development of pneumothorax?

Answer 14

Surfactant admin vs
Positive pressure ventilation
Frequent suctioning
Prematurity

Question 15

incidence of ptx?
Risk factors?
when does a ptx usually resolve?

Answer 15

Pneumothorax
* Spontaneous pneumothorax in ~1‐2% of live births
* Risk factors include meconium aspiration, respiratory
distress syndrome, pulmonary hypoplasia
* Usually resolve in 1‐2 days

Question 16

what is the sail sign, when do you see it?

Answer 16

Pneumomediastinum
* ‘sail sign’ – elevation of thymus from pericardial silhouette

Question 17

heart sounds, pericardial rub, narrow pulse pressure - dx?

Answer 17

pneumopericardium

Question 18

PIE - what causes it?
seen mostly in?
Is it diffuse? b/l?

Answer 18

Alveolar overdistension leading to rupture
* Primarily seen in premature ventilated patients
* Diffuse bilateral disease
* Localized – one or more lobes and mediastinal shift

Question 19

You are passing by the bedside for a newborn infant on CPAP respiratory
distress and hear biphasic stridor as they are changing the mask.
Respiratory status worsens and stridor becomes louder. What is the most likely etiology?

Answer 19

Subglottic stenosis

Question 20

inspiratory stridor due to what type of obstruction?

Answer 20

Inspiratory

Extrathoracic or upper airway obstruction
Large tongue, laryngomalacia, laryngeal cyst, congenital

tracheal stenosis (tracheal rings)

Question 21

biphasic stridor due to what type of obstruction?

Answer 21

Biphasic
Glottic or subglottic

Subglottic stenosis, subglottic hemangioma

Question 22

expiratory stridor due to what type of obstruction?

Answer 22

Expiratory
Intrathoracic airway obstruction
Arch anomalies, vascular rings, tracheomalacia of intrathoracic
segment of trachea

Question 23

Most common cause of inspiratory stridor in a neonate?

Answer 23

Laryngomalacia

Question 24

what are pulmonary causes of respiratory distress?

Answer 24

Acquired Pulmonary etiology
* Transient Tachypnea of the Newborn
* Pneumonia
* Pneumothorax
* Meconium aspiration syndrome

Congenital malformations of the lung
* Congenital Pulmonary airway malformation
* Congenital Pulmonary lymphangiectasia
* Congenital lobar emphysema
* Mediastinal tumors

Question 25

what congenital anomalies happen during the embryonic phase?

Answer 25

TE fistula, pulm sequestration, cong lobar emphysema

Question 26

what congenital anomalies happen during the Pseudoglandular phase?

Answer 26

CDH, CPAM, bronch cyst, bronchopulm sequestration

Question 27

what congenital anomalies happen during the canicular phase?

Answer 27

RDS, alveolar capillary dysplasia, pulmonary hypoplasia

Question 28

what congenital anomalies happen during the saccular phase?

Answer 28

RDS, BPD, pulmonary hypoplasia

Question 29

what congenital anomalies happen during the alveolar phase?

Answer 29

TTN, MAS, pneumonia, PPHN, emphysema

Question 30

A full‐term neonate is delivered by c‐section, and demonstrates tachypnea, grunting, and hypoxemia necessitating NICU care. At birth, activation of which intracellular channel aids in reabsorption of lung fluid?

Answer 30

Epithelial sodium channels

Question 31

fetal lung fluid - how much is made towards end of gestation?

Answer 31

30‐50ml/kg fluid in fetal lamb lungs towards end of gestation * Fetal lung fluid encompasses 90‐95% total lung weight toward term, decreases few days prior to delivery

Question 32

what ion does the pulmonary epithelium secrete into the alveoli? what ion follows?

Answer 32

* Pulmonary epithelium secretes chloride into alveoli * Na follows Cl through paracellular pathways, maintaining lung fluid

Question 33

labor onset what happens in lung with fluid?

Answer 33

Labor onset leads to increased circulating epinephrine, stimulating epithelial sodium channels (ENaC) Na moves into interstitium through Na/K channels with chloride and water passively following * Fluid then passes to pulmonary circulation primarily, lymphatics secondary Fluid also exits via squeeze of thorax during vaginal birth

Question 34

what are the risk factors for TTN?

Answer 34

C Section, maternal Diabetes, male sex, macrosomia, maternal asthma, delivery of preemie or late preterm

Question 35

Incidence of TTN at 33-34 wga, 35 - 36 wga, and in term?

Answer 35

Incidence: 3.6‐5.7/1000 births * 10% pts 33‐34wks * 5% pts 35‐36wks * 1% term infants

Question 36

Symptoms within first 6 hours of delivery * Tachypnea, grunting Dx?

Answer 36

TTN

Question 37

imaging in ttn shows?

Answer 37

Imaging * Fluid in fissure * Perihilar streaking * Congestion

Question 38

mx of ttn and when does it resolve?

Answer 38

Management * Oxygen supplementation, positive pressure support * Usually resolves by 48‐72 hours

Question 39

Chylothorax - which is more common congenital or acquired? what syndromes are the congenital assoc with?

Answer 39

10% Primary/Congenital – Trisomy 21, Noonan’s, Turner 90% Secondary/Acquired –most commonly associated with thoracic surgery, other associations include venous thrombosis leading to increased SVC pressure M> F

Question 40

how many newborns with chylothorax are sx within 24 hrs and how many within 7 days?

Answer 40

50% within 24 hrs 75% within 7 days

Question 41

how to dx chylothorax?

Answer 41

Fluid cell count > 1000 μL Lymph >80% TG >110 mg/dL Pleural fluid: serum cholesterol < 1

Question 42

how to mx chylothorax? when does it resolve?

Answer 42

Supportive, may take 4‐6 weeks for resolution, up to 80% will resolve without surgery * Fat free diet utilizing medium chain triglycerides (MCT) – absorbed by portal venous system * Will need essential fatty acids if prolonged utilization of MCT * If persistent, surgical options include pleurodesis, thoracic duct ligation, pleuroperitoneal shunt

Question 43

Dilation of lymphatics that results in obstruction and fluid leakage - what is dx?

Answer 43

Congenital Pulmonary Lymphangiectasia (CPL)

Question 44

what is secondary congenital pulmonary lymphangiectasia associated with?

Answer 44

Secondary CPL Associated with cardiac defects – total anomalous pulmonary venous return, hypoplastic left heart syndrome with restricted atrial septum

Question 45

what is primary congenital pulmonary lymphangiectasia associated with?

Answer 45

Associated with Noonan’s, Ehlers‐Danlos, Ullrich‐Turner, Down’s syndrome

Question 46

how does primary CPL happen?

Answer 46

Failure in development of lymphatics – starts to develop in 6th week and lymphatic channels develop before 20 weeks * Localized vs general lymphatic development defect * Generalized tends to have less severe pulmonary involvement

Question 47

Respiratory failure, hypoxia at birth, often with chylothorax, hyperinflation seen in what dx? what are the differentials?

Answer 47

CPL differentials: On differential in a fetal patient with non‐immune hydrops and congenital chylothorax

Question 48

congenital pna what bugs? Early pna happens at what days? late pna at what days of life? what bugs?

Answer 48

Congenital – Herpes simplex, usually acquired during labor; disseminated HSV results in pneumonia in approximately 33‐54%. Early (1st 3‐7 days),– Group B streptococcus, E coli increasing Late late (after 7 days of life)– RSV most common viral cause of late‐ onset pneumonia

Question 49

systemic disease + pna what bugs?

Answer 49

Systemic Disease + Pneumonia – consider CMV, HSV, toxoplasma, disseminated candida (particularly in preterm)

Question 50

what is the pathophysiology of pna? both cellular and inflammatory

Answer 50

Cellular Injury Alveolar surface impairment leading to transudation of fluid (pulmonary edema), surfactant impairment Inflammatory response * Neutrophil recruitment * Bacterial and inflammatory debris leading to airway obstruction * Increases in pulmonary vascular resistance through vasoconstrictor and procoagulants bacterial toxin exposure or Viral exposure – CMV and HSV infection leads to lysis of pneumocytes, further viral growth lead to cellular injury and inflamm response

Question 51

what is the cdc definition of pna?

Answer 51

Signs/Symptoms in infants <1year old: Worsening Gas Exchange (Desats, increased vent settings) + 3 of the following: * Temperature instability * Leukopenia (≤4000 WBC/mm3 ) or leukocytosis (>15,000 WBC/mm3 ) and left shift (>10% band forms) * New onset of purulent sputum or change in character of sputum , or increased respiratory secretions or increased suctioning requirements * Apnea, tachypnea, nasal flaring with retraction of chest wall or nasal flaring with grunting * Wheezing, rales, or rhonchi * Cough * Bradycardia (<100 beats/min) or tachycardia (>170 beats/min)

Question 52

what will imaging show with pna?

Answer 52

Imaging: With underlying pulmonary or cardiac disease: 2 or more CXR with: New and persistent or Progressive and persistent Infiltrate, consolidation, cavitation or pneumatocele *IF no underlying pulm or CV disease, can diagnose from 1 xray

Question 53

You are called to a csection for a fetus with prenatal diagnosis of congenital lung malformation. Baby delivers via NSVD and is initially vigorous, crying, begins to quiet, and HR <100. Baby is then intubated and brought to NICU. Which is the most common type of congenital pulmonary airway malformation?

Answer 53

Type 1 – Macrocystic (cysts > 2cm) Incidence Rare, approximately 1/10000‐1/35000 live births Types * Congenital pulmonary airway malformation (CPAM) – 40% * Bronchopulmonary sequestration (BPS) – 10% * Congenital lobar emphysema (CLE) * Bronchogenic cysts (BC) Most common time for detection is a fetal ultrasound – many asymptomatic in the neonatal

Question 54

pulmonary sequestration happens in what phase of lung dev?

Answer 54

embryonic (pulm sequestration, TE fistula, congenital lobar emphysema)

Question 55

cpam in what phase of lung dev?

Answer 55

pseudoglandular (cdh, cpam, bronchogenic cyst)

Question 56

what is the pathophys of cpam?

Answer 56

Pathophysiology Malformation during pseudoglandular stage Macrocystic lesions (>5mm) better prognosis than microcystic (<5mm) Most often found in lower lobes, right = left