Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies
CDH diagnosis is typically made at the 20-week anatomy scan when ultrasonographers see the stomach in the same cross-sectional plane as the heart
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
CDH is a medical physiologic emergency but not a surgical emergency
The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
Cites a guidelineCharlie Stolar5:24
Antenatal interventions for CDH are no better than investigational and experimental at best
Expert opinionCharlie Stolar6:35
Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
Cites a guidelineCharlie Stolar7:23
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
If shown 100 children with CDH, 80-85% will survive to become teenagers
Antenatal steroids have tremendous value for preterm labor under 35 weeks but most CDH babies are near-term (37-39 weeks) where steroid role is arguable
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
Expert opinionCharlie Stolar12:06
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
Initial evaluation of newborn with CDH includes looking for morphology, associated anomalies, respiratory distress, pre- and post-ductal oxygen gradients, and early cardiac echo for right heart function
Not every baby with CDH needs or is a candidate for ECMO; approximately 5% have insufficient lung to support life based on inability to saturate preductal blood
ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
The best way to assess end-organ function is urine output
ECMO indication is typically oxygenation index in excess of 40 for 4 hours or more
Cites a guidelineCharlie Stolar18:21
All therapy is guided by preductal oximetry, not postductal; if preductal saturation is 90% (PaO2 ~65 torr), the brain is doing fine with fetal hemoglobin
When managing dropping preductal saturation, first ensure adequate hemoglobin and circulating volume, then increase FiO2 or ventilator pressure, but avoid trying to control PCO2 as this will destroy the lungs
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
Expert opinionCharlie Stolar21:38
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
Unconventional ventilation mode for CDH uses high rate (100 breaths/min), low peak pressure (turned down to zero due to stacking), and high gas flow rate with permissive hypercapnia
High-frequency oscillatory ventilation (HFOV) as rescue therapy rarely spares CDH babies from ECMO; when they get out the oscillator, it's time to prime an ECMO circuit
Nitric oxide is a waste of money for CDH; it's terrific for premature babies with immature lung disease but of no value in CDH
Expert opinionCharlie Stolar25:45
The best drug for CDH is oxygen
Expert opinionCharlie Stolar26:07