StayCurrentMD · Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
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Podcast82 min·Published Jan 2017Older

Congenital Diaphragmatic Hernia with Dr. Charlie Stolar

With Dr. Charlie Stolar · hosted by Dr. Todd Ponsky & Dr. Avi Schlager · StayCurrentMD
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What the experts said95 expert statements
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies
EpidemiologicalCharlie Stolar
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
ClinicalCharlie Stolar
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
ClinicalCharlie Stolar
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
ClinicalCharlie Stolar
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
ClinicalCharlie Stolar
CDH is a medical physiologic emergency but not a surgical emergency
ClinicalCharlie Stolar
The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
GuidelineCharlie Stolar
Antenatal interventions for CDH are no better than investigational and experimental at best
OpinionCharlie Stolar
Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
GuidelineCharlie Stolar
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
ClinicalCharlie Stolar
Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
ClinicalCharlie Stolar
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
ClinicalCharlie Stolar
If shown 100 children with CDH, 80-85% will survive to become teenagers
EpidemiologicalCharlie Stolar
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
ClinicalCharlie Stolar
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
OpinionCharlie Stolar
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
ClinicalCharlie Stolar
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
ClinicalCharlie Stolar
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
ClinicalCharlie Stolar
ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
ClinicalCharlie Stolar
The best way to assess end-organ function is urine output
ClinicalCharlie Stolar
ECMO indication is typically oxygenation index in excess of 40 for 4 hours or more
GuidelineCharlie Stolar
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
ClinicalCharlie Stolar
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
ClinicalCharlie Stolar
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
OpinionCharlie Stolar
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
ClinicalCharlie Stolar
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
ClinicalCharlie Stolar
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
ClinicalCharlie Stolar
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
ClinicalCharlie Stolar
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
OpinionCharlie Stolar
The best drug for CDH is oxygen
OpinionCharlie Stolar
ECMO gestational age limit has been pushed from 36 weeks down to 35, maybe 34 weeks; below 32 weeks the intracranial hemorrhage rate takes off and neurologic outcomes become poor
ClinicalCharlie Stolar
The smallest ECMO arterial cannula is about 8 French; getting adequate flow out of small cannulas is problematic due to resistance related to both length and diameter
ClinicalCharlie Stolar
For ECMO candidacy, it's the gestational age that matters for intracranial hemorrhage risk (germinal matrix), not the size; size becomes an issue only when cannulas don't fit
ClinicalCharlie Stolar
The broad principle for ECMO candidacy is: do you have a reversible condition? Can you get out with reasonable confidence once you start?
ClinicalCharlie Stolar
VV ECMO is terrific if the heart works but problematic in CDH because heart function is often depressed and it's hard to get the cannula in with the shifted mediastinum
ClinicalCharlie Stolar
VV ECMO is for less sick patients who aren't in extremis; most CDH babies are sicker and do better on VA ECMO
ClinicalCharlie Stolar
VA bypass is basically dialing in a PaO2; VV has mixing issues, cannula position concerns, and is more annoying to manage
ClinicalCharlie Stolar
Echo guidance during ECMO cannulation is really helpful with the shifted mediastinum; the arterial cannula can go out the subclavian artery or the venous cannula into the innominate vein
ClinicalCharlie Stolar
If the arterial cannula goes out the subclavian artery, you'll have a well-perfused hand and think preductal sats look good, but the baby isn't seeing the oxygen
ClinicalCharlie Stolar
Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
ClinicalCharlie Stolar
Use 3 stay sutures (5-0 Prolene, rubber-shod) to lift the venotomy and arteriotomy open to avoid shearing off the intima and creating a false passage
ClinicalCharlie Stolar
In the first hours on ECMO, hyperkalemia can cause cardiac arrest if blood isn't washed; just perfuse through it and give calcium
ClinicalCharlie Stolar
ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
ClinicalCharlie Stolar
Target VA ECMO flow is 100-125 cc/kg/min, which is about 80% of cardiac output assuming an open duct
ClinicalCharlie Stolar
At target ECMO flow, preductal saturation will be good and mixed venous (from venous drainage) will come up to 65-70%
ClinicalCharlie Stolar
Regular cardiac echos during ECMO weaning are important to assess right heart function, dilation, tricuspid regurgitation, and pulmonary outflow tract jet
ClinicalCharlie Stolar
The hyperoxia test (turning ventilator FiO2 up to 1.0) demonstrates if the baby can use their lungs; if PaO2 rises, it gives courage to begin weaning ECMO
ClinicalCharlie Stolar
For babies unweanable from ECMO after 2+ weeks at high flow, ensure they are maximally dried out (bone dry, eyes sucked into head), making good urine, with good labs and chest X-ray before considering on-ECMO repair
ClinicalCharlie Stolar
Repairing the hernia on ECMO is unusual to get you out of trouble; the problem is lung growth arrest at 14-15 weeks gestation, not that bowel is in the chest
ClinicalCharlie Stolar
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) are making clot; platelet thrombus lifespan is 48-72 hours
ClinicalCharlie Stolar
If operating on ECMO, you have about 48-72 hours to decannulate before bleeding starts; operate when nearly weanable (20 cc/kg/min), expect 1 day of post-op deterioration, then 2-3 days to get off
ClinicalCharlie Stolar
Operating off ECMO means the baby will get stiff post-operatively, pulmonary hypertension may relapse, and you risk needing a second ECMO run
ClinicalCharlie Stolar
When operating on ECMO, load with Amicar preoperatively, do abdominal approach, use low threshold for patch to avoid tension, place Jackson-Pratt drain under patch, and place chest tube
ClinicalCharlie Stolar
Futility on ECMO begins to rear its head after 2-3 weeks; it becomes easier to discuss withdrawal if there's been a CNS event like intracranial hemorrhage
ClinicalCharlie Stolar
For non-ECMO babies, repair timing is when they're on minimal ventilator settings (FiO2 0.4, conventional settings), which typically takes 3-4 days
ClinicalCharlie Stolar
Use the infant ventilator instead of anesthesia machine intraoperatively; infant anesthesia machines have high dead space and aren't very compliant
ClinicalCharlie Stolar
Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
ClinicalCharlie Stolar
Pre-repair assessment includes pre/post-ductal gradient, echo showing RV not dilated, RV pressure no more than systemic (not super-systemic), acceptable tricuspid regurgitation, and acceptable pulmonary outflow tract acceleration times
ClinicalCharlie Stolar
Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
ClinicalCharlie Stolar
The thoracoscopic approach provides a gorgeous view and often the bowel reduces with chest insufflation, but only rock-stable kids are candidates because you're creating a tension pneumothorax in a potentially hypercapnic baby
ClinicalCharlie Stolar