RC 290
Occurrence
1-2% of all births 10% of all premature births
Greatest occurrence is in the premature and low birth weight infant
Pathophysiology
Surfactant deficiency Decreased FRC Atelectasis Increased R-L shunt Increased W.O.B. Hypoxemia and eventually hypercapnia because of V/Q mismatch
Pathophysiology (cont.)
Atelectasis keeps PVR high Increased PAP Lung hypoperfusion R-L shunting may reoccur across the Ductus Arteriosus and the Foramen Ovale
The cycle continues until surfactant levels are adequate to stabilize the lung
Symptoms usually appear 2-6 hours after birth
Why not immediately?
Disease peaks at 48-72 hours Recovery usually occurs 5-7 days after birth
Decreased breath sounds with crackles Cyanosis on room air Hypothermia Hypotension
CXR: Normal
Surfactant instillation!!!
May cause a sudden drop in elastic resistance!
Prognosis/Complications
Prognosis is good once infant makes it past the peak (48-72 hours) Complications possible are: Intracranial Bleed BPD (Bronchopulmonary Dysplasia) PDA (Patent Ductus Arteriosus)
Maternal Diabetes
Increased chance of C-section due to LGA
TTN Pathophysiology
Primary problem = retained lung fluid Fluid not expelled from airways because of Csection Poor absorption of remaining fluid by pulmonary capillaries and lymphatics If retained fluid is in interstitial spaces, compliance and TC are decreased If retained fluid is in airways,airway resistance and TC are increased TTN can be restrictive , obstructive, or both! Fluid usually clears by itself after 24-48 hours after birth
Clinical Signs
Tachypnea (usually rate is greater than seen in RDS) Minimal (if any) nasal flaring or expiratory grunting ABGs: mild hypoxemia. PaCO2 depends on whether problem is restrictive or obstructive
TTN CXR
Coarse peri-hilar streaks Prominent lung vasculature Flattened diaphragms if fluid is causing obstruction/air-trapping
Prognosis/Complications
Prognosis is very good Main complication is pneumonia
Often initial diagnosis
Occurrence
1 per 2000 term babies 30-50% of RDS babies
Hypoxia
Decreasing PaO2 allows it to re-open for up to three weeks after birth
Thus, a PDA can occur in a premature infant who is NOT hypoxic or in a term baby who is hypoxic
Worst case is a premature infant who is hypoxic!
Pathophysiology
D.A. fails to close or it re-opens Then shunting occurs between the pulmonary artery and the aorta The direction of the shunt depends on which vessel has the higher pressure
A PDA can cause L-R shunting or R-L shunting!
Clinical Signs
Tachypnea, bounding pulses, hyperactive pre-cordium Decreased breath sounds and possibly some crackles Possible murmur over left sternal border
Murmur is loudest when D.A. just starts opening or when it is almost closed
PDA Treatment
Basic NTE, O2, may require CMV if not already on the ventilator Medical
L-R shunt that fails to close: Indomethacin (Indocin) R-L shunt: Priscoline (Tolazoline) to decrease PVR; also nitric oxide
Prognosis/Complications
Good prognosis when baby responds to medical treatment May develop : Shock CHF Necrotizing Enterocolitis (NEC)
Meconium Aspiration Syndrome -MASSyndrome of respiratory distress that occurs when meconium is aspirated prior to or during birth
Occurrence
10-20% of ALL births show meconium staining
10-50% of stained babies may be symptomatic
Cord compression
Pathophysiology (cont.)
V/Q mismatch leads to hypoxia and acidosis which increases PVR TC increases because it increases airway resistance Meconium is usually absorbed in 24-48 hours; there are still many possible complications
Clinical Signs
Respiratory depression or distress at birth Hyperinflation Pallor Meconium stained body
Possible cyanosis on room air Moist crackles ABGs hypoxemia with combined acidosis CXR coarse, patchy infiltrates with areas of atelectasis and areas of hyperinflation
May see flattened diaphragms if obstruction is severe
M.A.S. Treatment
Amnioinfusion artificial amniotic fluid infused into uterus to dilute meconium Proper resuscitation at birth(clear meconium from trachea before stimulating respiration) Oro-gastric tube NTE O2 NaHCO3 if severe metabolic acidosis Broad spectrum antibiotics Bronchial hygiene May need mechanical ventilation
Slow rates and wide I:E ratios because of increased TC Low level of PEEP may help prevent check valve effect May need HFO
Failure to make the transition from fetal to neonatal circulation or a reversion back to the condition where pulmonary artery pressure exceeds aortic pressure
Results in R-L shunting across the D.A. and the Foramen Ovale
Occurrence
Usually term and post-term babies Females more often than males Symptoms may take 12-24 hours after birth to develop
Pathophysiology
Primary problem is pulmonary artery hypertension
Infants arterial walls are thicker and they are more prone to vasospasm
If pulmonary artery pressure gets high enough, blood will shunt R-L across the D.A. and Foramen Ovale
Remember, conditions that drive up PAP usually make the D.A. open
Clinical Signs
Refractory hypoxemia and cyanosis Shock and tachypnea Murmur possible Pre-ductal PaO2 > post-ductal PaO2
Hypoxemia with combined acidosis
PPHN Treatment
NTE and O2 Nitric Oxide
Often in conjunction with HFO
Priscoline, Indocin may also be used If completely unresponsive to therapy ECMO may be tried
Wilson Mikity Syndrome -Pulmonary DysmaturityRespiratory distress that develops after the first week of life and presents with definite CXR changes
Occurrence
Usually in <36 weeks gestational age and birth weight <1500 grams After first week of life
No prior symptoms
Pathology
Immature alveoli and T-B tree causes V/Q mismatch Areas of atelectasis and hyperinflation develop
Pathology (cont.)
3 Stages Stage 1
1-5 weeks after birth Diffuse areas of atelectasis and hyperinflation
Stage 2
1-5 months after birth Cystic (hyperinflated) areas coalesce and cause flattening of the diaphragms
Stage 3
5-24 months after birth Cystic areas start to clear up
Clinical Signs
Tachypnea Cyanosis on room air Some retractions and/or nasal flaring Decreased breath sounds with crackles ABGs respiratory acidosis with hypoxemia CXR consistent with the stage of the disease
Bronchopulmonary Dysplasia -BPDA result of RDS and/or its treatment that results in areas of fibrosis, atelectasis, and hyperinflation
Pathology: 4 Stages
Stage 1
Acute phase of RDS
Stage 4
1 month after the onset of RDS Diaphragms start to flatten Interstitial fibrosis evident on CXR PPHN may start to develop O2 dependency develops
Stage 2
4-10 days after the onset of RDS Areas of atelectasis and hyperinflation
Stage 3
2-3 weeks after RDS Hyperinflated areas start to coalesce Fibrosis starts to develop
Clinical Signs
Tachypnea Persistent retractions A-B spells Cyanosis on room air Decreased breath sounds with crackles ABGs respiratory acidosis (may be compensated) with hypoxemia CXR consistent with stage of disease
BPD Treatment
Prevention is best! Use the least amount of intervention for the least amount of time! Supportive care
O2, NTE, bronchial hygiene, maintain fluids/electrolytes Diuretics if needed to prevent fluid overload and heart failure
Possibly vitamin E
Diaphragmatic Hernia
Congenital malformation of the diaphragm that allows abdominal viscera into the thorax
Occurrence
Pathology
Usually occurs during the 8-10th week of gestation 80% occur on the left at the Foramen of Bochdalek Abdominal viscera enters thorax and compresses developing lung As baby attempts to breathe after birth, the stomach and bowel fill with air and cause further compression of the lung
Severe restriction!
Clinical Signs
Cyanosis Severe respiratory distress with retractions and nasal flaring Bowel sounds in chest Uneven chest expansion Decreased breath sounds on affected side ABGs profound hypoxemia with combined acidosis CXR loops of bowel in chest with shift of thoracic structures towards unaffected side, eg dextrocardia
4 Main Types
Pneumothorax Pneumomediastinum Pneumopericardium PIE (Pulmonary Interstitial Emphysema)
Gas from ruptured alveoli dissects along perivascular and interstitial spaces Causes airway compression (obstruction) and alveolar compression (restriction) May lead to pneumothorax, pneumomediastinum, or pneumopericardium
Occurrence
1-2% of all births
Clinical Signs
Sudden cyanosis (except with PIE) Respiratory distress Mediastinal shift Sudden hypotension (except with PIE)
Crepitus (if sub-Q emphysema develops) Unequal chest expansion Decreased breath sounds and hyperressonance ABGs hypoxemia with respiratory acidosis Transillumination
Transillumination
Transillumination
CXR: Pneumothorax
CXR: Pneumomediastinum
Note how air does NOT outline the apex of the heart
CXR: Pneumopericardium
CXR: PIE
Occurrence
20% of all premature births Males = Females Most common in low birth weight babies who experience perinatal distress
Pathology
Intestinal ischemia due to hypoperfusion, eg shock, or vascular occlusion, eg, clot from umbilical artery catheter Bacterial colonization after ischemia starts necrosis Early formula feeding may provide substrate needed for further bacterial growth and further necrosis
Clinical Signs
Abdominal distention Poor feeding Blood in fecal material Lethargy Hypotension Apnea Decreased urine output Bile stained emesis CXR bubbles in intestinal wall
NEC Treatment
NPO and NG suction IV hydration and hyperalimentation Broad spectrum antibiotics
Ampicillin, Gentamycin
Main complication is sepsis Infants who have bowel resection may develop malabsorption syndrome
Tetralogy of Fallot
VSD Over-riding aorta Pulmonary valve stenosis Right ventricular hypertrophy Significant cyanosis because of R-L shunt
Truncus Arteriosus
Aorta and pulmonary artery are the same vessel Large VSD Requires MAJOR surgical repair Mortality is 40-50%