Charlie Stolar
808 statements · 4 topics · summaries given as host listed separately
Featured statements
The problem is not that the bowel is up in the chest. The problem is that there's been a growth arrest of the lungs. And just there's nothing miraculous about getting the bowel out of the chest.
quote ·
Congenital Diaphragmatic Hernia
If you drive it out the subclavian artery, you're going to have a very well perfused hand, and you think your preductal sats are looking really good, but the baby's not seeing the oxygen.
quote ·
Congenital Diaphragmatic Hernia
this business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
quote ·
CICU / Post-op CHD Care
if you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
quote ·
CICU / Post-op CHD Care
ECMO is a drug delivery system for oxygen. So if the end organs are getting enough oxygen, then you're doing fine.
quote ·
Pulmonary Hypoplasia
Don't start something you can't finish.
quote ·
Pulmonary Hypoplasia
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Charlie's statements about CICU / Post-op CHD Care 110 statements
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Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
the most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
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
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
Open
Statement
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
Open
Statement
The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
Open
Statement
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
Open
Statement
Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
Open
Quotation
if you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
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
Open
Statement
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
Open
Statement
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
Open
Statement
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
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Statement
ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
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Statement
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
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Statement
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
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Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
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Statement
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
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Statement
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
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Statement
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
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Quotation
I often joke that when people say, how do you know when to set up an ECMO circuit with a diaphragmatic hernia, it's when they get out the oscillator.
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Statement
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
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Statement
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
Open
Statement
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
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Statement
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
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Statement
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
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Statement
The broad principle for ECMO candidacy is: do you have a reversible condition? Can you get out with reasonable confidence once you start?
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Statement
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
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Statement
VV ECMO is for less sick patients who aren't in extremis; most CDH babies are sicker and do better on VA ECMO
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Statement
VA bypass is basically dialing in a PaO2; VV has mixing issues, cannula position concerns, and is more annoying to manage
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Statement
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
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Statement
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
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Statement
Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
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Statement
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
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Statement
In the first hours on ECMO, hyperkalemia can cause cardiac arrest if blood isn't washed; just perfuse through it and give calcium
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Statement
ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
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Statement
Target VA ECMO flow is 100-125 cc/kg/min, which is about 80% of cardiac output assuming an open duct
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Statement
At target ECMO flow, preductal saturation will be good and mixed venous (from venous drainage) will come up to 65-70%
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Statement
Regular cardiac echos during ECMO weaning are important to assess right heart function, dilation, tricuspid regurgitation, and pulmonary outflow tract jet
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Statement
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
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Statement
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
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Statement
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
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) are making clot; platelet thrombus lifespan is 48-72 hours
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Statement
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
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Statement
Operating off ECMO means the baby will get stiff post-operatively, pulmonary hypertension may relapse, and you risk needing a second ECMO run
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Statement
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
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Statement
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
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Statement
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
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Statement
Use the infant ventilator instead of anesthesia machine intraoperatively; infant anesthesia machines have high dead space and aren't very compliant
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Statement
Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
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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
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Statement
Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
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Statement
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
Open
Statement
In Dr. Stolar's series of 35 thoracoscopic CDH repairs in stable babies, recurrence rate was about 25% in under a year; APSA outcomes committee meta-analysis reached similar conclusions
Open
Statement
Laparoscopic CDH repair is problematic because the scaphoid abdomen has loss of abdominal domain; insufflating just puts air up in the chest
Open
Quotation
I was very disappointed at our own data because I thought the scope approach was gorgeous.
Open
Statement
For open repair, key is adequate subcostal incision (not a small two-finger incision); rotate liver out of chest and abdomen to see the defect
Open
Statement
Critical repair points are all medial where structures with names are located; mobilize posterior leaflet by unrolling it like a window shade down to the body wall until you feel rib
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Statement
The medial repair is hardest because sometimes the esophagus or aorta hangs out with nothing to sew to; use an upside-down U-shaped pericardial flap rotated down to where the diaphragm would be to begin the repair
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike braided suture like Vicryl
Open
Statement
For patches, favor non-biologic material like 1mm Gore-Tex; laterally, anchor the patch to the ribs by getting a needle around the rib and into the patch
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out with a deep breath; over time it gets incorporated into fibrous tissue
Open
Statement
For thoracoscopic repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and reduce spleen last as it obturates the hole and keeps bowel in the belly
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Statement
Thoracoscopically, mobilizing the posterior leaflet is difficult because it's rolled into the abdomen and hard to see around the corner
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Statement
For thoracoscopic patch placement, use extracorporeal-intracorporeal suture technique: come from outside through skin around rib into patch and back out, tie externally, and bury the knot under the skin
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Statement
If you can see through the diaphragm (it's just pleura and peritoneum with no muscle), resect it back to something that looks like muscle; plication of see-through tissue will fail
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The ipsilateral lung is small and not as big as the pleural space; that's how God made this lung. You won't inflate the lung to fill the chest
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The pleural space will be obliterated either slowly by lung growth over 3-4 years or by filling with fluid; it's not a pneumothorax under pressure, it's pneumothorax ex vacuo
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Statement
Chest tube on water-seal suction will distort the mediastinum and precipitate a pulmonary hypertensive crisis; there's no reason to put in a chest tube unless there's active air leak or bleeding
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Statement
Typical stable post-op CDH baby will be unstable for about a day with increased ventilator requirements, then improve and get extubated in 4-5 days
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Statement
CDH is a field defect affecting the entire foregut from pharynx to ligament of Treitz; these kids have disordered motility throughout
Open
Statement
All CDH patients should have a GI series showing dilated, ectatic, abnormal esophagus; manometry and impedance show abnormal esophageal and gastric motility and emptying
Open
Quotation
this business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
The foregut problem is not really reflux; calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture
Open
Statement
CDH babies do well with continuous feedings slowly condensed to bolus; surgical intervention for foregut issues is unusual and should be approached as palliation
Open
Statement
Nissen fundoplication is a poor operation for CDH kids (like for esophageal atresia) because their motility is abnormal; if surgery is needed, use a partial wrap with gastrostomy and real drainage procedure (Jaboulay-Finney type, not Heineke-Mikulicz)
Open
Statement
Dr. Stolar's multidisciplinary CDH clinic follows 450-500 patients for life, addressing heart, lung, foregut, nutrition, neurodevelopmental, and axial skeleton issues that emerge over time
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Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis; he recommends lifelong proton pump inhibitors and regular endoscopic surveillance
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CDH patients have increased incidence of attention deficit disorders and autism; early intervention for neuropsychiatric issues is recommended
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Statement
As a field defect, CDH causes asymmetric chest growth leading to pectus-like distortion; some patients need Nuss-type operations to rebuild chest wall
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Statement
Some CDH girls have no breast development on the hernia side as teenagers; combined Nuss operation and breast implant procedures have been performed
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Statement
CDH patients develop thoracolumbar scoliosis (not idiopathic scoliosis, as it's mostly in boys); early bracing programs help minimize this
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Statement
CDH recurrence is related to tension on tissues and tissue quality; it's a tough problem whether tissue-to-tissue or prosthetic repair
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Statement
Recurrence is more common with left-sided CDH than right because the liver plugs up the hole on the right
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Statement
For right-sided CDH, the most important consideration is determining where hepatic veins drain; not infrequently they enter directly into the right atrium, not the suprahepatic IVC
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Statement
If hepatic veins enter the right atrium directly, attempting to reduce the liver into the abdomen will cause a troublesome moment; you should not try to put that liver in the abdomen
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Hepatopulmonary fusion actually exists in right-sided CDH; the liver and lung are fused and cannot be separated surgically
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Most patients with hepatopulmonary fusion don't survive; they often have associated severe congenital heart disease and IVC interruption with azygous continuation
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For right-sided CDH, prep the baby for both thoracic and abdominal incisions; often need to be on both sides to figure out what's going on because the liver is in the way
Open
Statement
For right-sided CDH, can put a scope through an open thoracotomy to see around corners where the liver might come up against the heart
Open
Charlie's statements about Congenital Diaphragmatic Hernia 201 statements
Open the Congenital Diaphragmatic Hernia collection →
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
The most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach.
Open
Statement
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies managed by community obstetricians.
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
CDH diagnosis is usually made at the 20-week anatomy scan when ultrasonographers see the stomach in the same cross-sectional plane as the heart.
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side.
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation.
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best.
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability.
Open
Statement
In single-center experiences, presence of liver in the chest is of no prognostic value for CDH.
Open
Statement
Lung-to-head ratio (LHR) is of limited prognostic value except when very low (less than 0.8).
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis in CDH.
Open
Quotation
If you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
Steroids have tremendous value for preterm labor under 35 weeks but their role in near-term CDH babies (37-39 weeks) is arguable.
Open
Statement
Exit-to-ECMO for CDH is essentially moving the goalposts and deck chairs around on the Titanic for non-viable babies.
Open
Quotation
What you're now doing is putting what in my view is almost certainly a non-viable baby on a heart lung machine and basically moving the goalposts and moving the deck chairs around on the Titanic.
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth.
Open
Quotation
ECMO is a drug delivery system for oxygen. So if the end organs are getting enough oxygen, then you're doing fine.
Open
Statement
All therapy for CDH should be guided by preductal oximetry, not postductal, because all babies have some degree of pulmonary hypertension with shunting.
Open
Statement
If preductal saturation is 90% (PaO2 of 65 torr), the brain is doing fine because this is fetal hemoglobin.
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today because they trash lungs in a heartbeat.
Open
Statement
CDH babies should not be paralyzed and should have minimal sedation to maintain spontaneous breathing.
Open
Statement
Most CDH babies don't tolerate conventional ventilator settings (rate 40, peak pressure 25-28, PEEP 5) and require unconventional high-rate (100 breaths/min) low-pressure ventilation.
Open
Statement
High-frequency oscillatory ventilation (HFOV) as rescue therapy rarely spares CDH babies from ECMO.
Open
Quotation
When I see them getting out the oscillator, I'll go prime a circuit. That's just how I think about it.
Open
Statement
Nitric oxide is a waste of money for CDH babies; meta-analyses show it's terrific for premature babies with immature lung disease but of no value in CDH.
Open
Statement
ECMO should not be used in babies under 36 weeks gestational age initially, though this has been pushed down to 35 weeks, maybe 34 weeks, with intracranial hemorrhage rates taking off below 32 weeks.
Open
Statement
The smallest ECMO arterial perfusion cannula available is about 8 French, and resistance is related to both length and diameter, making adequate flow difficult in very small babies.
Open
Statement
For ECMO candidacy, the real risk for intracranial hemorrhage is the germinal matrix, which is usually OK by 35-36 weeks gestation, so it's gestational age that matters, not size.
Open
Statement
The broad principle for ECMO candidacy is whether you can get out of ECMO with reasonable confidence if you get in—don't start something you can't finish.
Open
Statement
VV ECMO is terrific if the heart works, but in CDH the heart function is often depressed and it's hard to get the cannula in with the shifted mediastinum.
Open
Statement
VA bypass is essentially dialing in a PaO2, while VV ECMO is much more annoying with mixing, cannula position issues, and CDH babies are generally too unstable for VV.
Open
Statement
Echo guidance during ECMO cannulation is really helpful to prevent driving the arterial cannula out the subclavian artery or the venous cannula into the innominate vein.
Open
Statement
If the arterial cannula goes out the subclavian artery, you'll have a very well-perfused hand and think preductal sats look good, but the baby isn't seeing the oxygen.
Open
Quotation
If you drive it out the subclavian artery, you're going to have a very well perfused hand, and you think your preductal sats are looking really good, but the baby's not seeing the oxygen.
Open
Statement
The first few hours on ECMO can be unstable; hyperkalemia cardiac arrest can occur if blood isn't washed, but you just perfuse through it and give calcium.
Open
Statement
ECMO flow should be slowly increased over 45 minutes to 1.5 hours to about 100-125 cc/kg/min (about 80% of cardiac output) to reduce intracranial hemorrhage incidence.
Open
Statement
The hyperoxia test—turning FIO2 up to 1.0 on the ventilator while on ECMO—gives courage to begin weaning if the PaO2 rises, showing the baby can use their lungs.
Open
Statement
For babies stuck on ECMO at 2 weeks, ensure they are maximally dried out (bone dry, eyes sucked into back of head, turned into a prune) before considering on-ECMO repair.
Open
Statement
The problem in CDH is not that bowel is in the chest; the problem is growth arrest of the lungs that happened at 14-15 weeks gestation. Getting bowel out of the chest is not miraculous.
Open
Quotation
The problem is not that the bowel is up in the chest. The problem is that there's been a growth arrest of the lungs. And just there's nothing miraculous about getting the bowel out of the chest.
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) make clot on ECMO. A platelet thrombus lasts 48-72 hours, giving a window to operate and get off ECMO before bleeding starts.
Open
Statement
Preferred approach is to wean ECMO down to 20 cc/kg/min, do the operation, accept 1 day of post-op edema, then have 2-3 days to get off ECMO before bleeding starts.
Open
Statement
If you take a baby off ECMO then operate, every patient gets stiff after surgery, pulmonary hypertension relapses, and you're talking about a second ECMO run.
Open
Statement
When operating on ECMO, load with Amicar preoperatively, do it as an abdominal operation, use a patch with low threshold to avoid tension, place a Jackson-Pratt drain under the patch, and place a chest tube.
Open
Statement
The typical stable CDH baby not requiring ECMO takes 3-4 days to wean to minimal ventilator settings (FIO2 0.4, conventional settings) before repair.
Open
Statement
Use the infant ventilator instead of an anesthesia machine intraoperatively because infant anesthesia machines have high dead space and are not very compliant.
Open
Statement
For repair timing, three variables matter: pre/post-ductal gradient on pulse ox, right ventricle dilation on echo, and RV pressure versus LV pressure (RV should be no more than systemic).
Open
Statement
The thoracoscopic approach for CDH is gorgeous with a sparkling view, but the recurrence rate is really high—about 25% in under a year in Dr. Stolar's series of 35 stable kids.
Open
Statement
The APSA outcomes committee meta-analysis came to a very similar conclusion about high thoracoscopic CDH recurrence rates.
Open
Statement
For open CDH repair, need a real subcostal incision (not a small two-finger incision), rotate the liver out of chest and abdomen, eviscerate bowel, and mobilize the posterior leaflet like unrolling a window shade down to rib.
Open
Statement
The medial part of CDH repair is hardest because sometimes the esophagus or aorta is hanging out with nothing to sew to.
Open
Statement
When there's no diaphragm to sew to medially, make an upside-down U-shaped incision on the pericardium and rotate that down to where the diaphragm would be to begin the repair.
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike Vicryl which saws tissue.
Open
Statement
Favor non-biologic material like 1mm Gore-Tex for patches, and anchor the patch to the ribs laterally by getting a needle around the rib.
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out taking a deep breath; over time it gets incorporated into fibrous tissue.
Open
Statement
For thoracoscopic CDH repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and put solid organs (spleen or left liver lobe) in last to plug the hole and keep bowel down.
Open
Statement
If you can see through the diaphragm tissue (it's nothing but pleura and peritoneum), resect it back to muscle for a fresh edge to sew together, or the plication will fail.
Open
Statement
There is no indication for a chest tube in CDH except for active air leak or anticipated/active bleeding. The ipsilateral lung is small and won't fill the pleural space—that's how God made this lung.
Open
Statement
A pneumothorax in CDH is a pneumothorax ex vacuo, not air under pressure. Putting in a chest tube on water seal suction will just distort the mediastinum and precipitate a pulmonary hypertensive crisis.
Open
Statement
The typical stable CDH baby will be crummy for about a day postoperatively, then get better and be extubated in 4-5 days and go home.
Open
Statement
CDH is a field defect affecting the whole foregut from pharynx to ligament of Treitz. Everything—esophagus, stomach, gastric emptying—has abnormal motility.
Open
Statement
If you do a GI series on CDH kids, the esophagus will be very dilated, ectatic, and abnormal-looking. Manometry and impedance manometry are abnormal.
Open
Statement
It's not really reflux in CDH—calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture.
Open
Quotation
This business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
CDH kids generally do well with continuous feedings slowly condensed to bolus. It's unusual to need surgical intervention for foregut dysmotility.
Open
Statement
When surgery is needed for CDH foregut dysmotility, approach it as palliation: some sort of fundoplication (not Nissen—it's fully competent on an abnormal-motility esophagus), combined with gastrostomy and a real drainage procedure like Jaboulay-Finney, not Heineke-Mikulicz.
Open
Statement
Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis and now recommends lifelong proton pump inhibitors and regular scoping for all CDH patients.
Open
Statement
Dr. Stolar's 1989-1990 paper showed most CDH kids turn out OK unless the mother had an 8th grade education and the child is a boy.
Open
Statement
There is an incidence of attention deficit disorders and autism in CDH survivors, prompting early intervention for neuropsychiatric issues.
Open
Statement
Because CDH is a field defect, there's asymmetric chest growth leading to pectus-like distorted chest wall growth requiring Nuss-type operations in some patients.
Open
Statement
Some CDH girls as teenagers have no breast development on the side of the hernia, requiring Nuss operation and breast implant.
Open
Statement
CDH patients can develop non-idiopathic scoliosis (mostly in boys, not the typical idiopathic scoliosis in girls), requiring early bracing to minimize thoracolumbar scoliosis.
Open
Statement
For right-sided CDH, the most important consideration is to ask echocardiographers where the hepatic veins drain, because they often enter directly into the right atrium, not the suprahepatic cava.
Open
Statement
Hepatopulmonary fusion actually exists in right-sided CDH. The liver and lung are fused and cannot be separated surgically. Most of these patients don't survive and often have severe congenital heart disease and IVC discontinuation.
Open
Statement
For right-sided CDH, prep the baby for both thoracic and abdominal incisions because you often need to be on both sides. Start in the abdomen, but if the liver is in the way, make a counter-incision in the chest.
Open
Statement
Even with an open thoracotomy, you can put a scope in to see around a corner without ugly retractors.
Open
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
the most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
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
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
Open
Statement
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
Open
Statement
The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
Open
Statement
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
Open
Statement
Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
Open
Quotation
if you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
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
Open
Statement
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
Open
Statement
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
Open
Statement
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
Open
Statement
ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
Open
Statement
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
Open
Statement
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
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
Open
Statement
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
Open
Statement
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
Open
Statement
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
Open
Quotation
I often joke that when people say, how do you know when to set up an ECMO circuit with a diaphragmatic hernia, it's when they get out the oscillator.
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
The broad principle for ECMO candidacy is: do you have a reversible condition? Can you get out with reasonable confidence once you start?
Open
Statement
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
Open
Statement
VV ECMO is for less sick patients who aren't in extremis; most CDH babies are sicker and do better on VA ECMO
Open
Statement
VA bypass is basically dialing in a PaO2; VV has mixing issues, cannula position concerns, and is more annoying to manage
Open
Statement
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
Open
Statement
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
Open
Statement
Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
Open
Statement
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
Open
Statement
In the first hours on ECMO, hyperkalemia can cause cardiac arrest if blood isn't washed; just perfuse through it and give calcium
Open
Statement
ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
Open
Statement
Target VA ECMO flow is 100-125 cc/kg/min, which is about 80% of cardiac output assuming an open duct
Open
Statement
At target ECMO flow, preductal saturation will be good and mixed venous (from venous drainage) will come up to 65-70%
Open
Statement
Regular cardiac echos during ECMO weaning are important to assess right heart function, dilation, tricuspid regurgitation, and pulmonary outflow tract jet
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) are making clot; platelet thrombus lifespan is 48-72 hours
Open
Statement
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
Open
Statement
Operating off ECMO means the baby will get stiff post-operatively, pulmonary hypertension may relapse, and you risk needing a second ECMO run
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
Use the infant ventilator instead of anesthesia machine intraoperatively; infant anesthesia machines have high dead space and aren't very compliant
Open
Statement
Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
Open
Statement
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
Open
Statement
Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
Open
Statement
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
Open
Statement
In Dr. Stolar's series of 35 thoracoscopic CDH repairs in stable babies, recurrence rate was about 25% in under a year; APSA outcomes committee meta-analysis reached similar conclusions
Open
Statement
Laparoscopic CDH repair is problematic because the scaphoid abdomen has loss of abdominal domain; insufflating just puts air up in the chest
Open
Quotation
I was very disappointed at our own data because I thought the scope approach was gorgeous.
Open
Statement
For open repair, key is adequate subcostal incision (not a small two-finger incision); rotate liver out of chest and abdomen to see the defect
Open
Statement
Critical repair points are all medial where structures with names are located; mobilize posterior leaflet by unrolling it like a window shade down to the body wall until you feel rib
Open
Statement
The medial repair is hardest because sometimes the esophagus or aorta hangs out with nothing to sew to; use an upside-down U-shaped pericardial flap rotated down to where the diaphragm would be to begin the repair
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike braided suture like Vicryl
Open
Statement
For patches, favor non-biologic material like 1mm Gore-Tex; laterally, anchor the patch to the ribs by getting a needle around the rib and into the patch
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out with a deep breath; over time it gets incorporated into fibrous tissue
Open
Statement
For thoracoscopic repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and reduce spleen last as it obturates the hole and keeps bowel in the belly
Open
Statement
Thoracoscopically, mobilizing the posterior leaflet is difficult because it's rolled into the abdomen and hard to see around the corner
Open
Statement
For thoracoscopic patch placement, use extracorporeal-intracorporeal suture technique: come from outside through skin around rib into patch and back out, tie externally, and bury the knot under the skin
Open
Statement
If you can see through the diaphragm (it's just pleura and peritoneum with no muscle), resect it back to something that looks like muscle; plication of see-through tissue will fail
Open
Statement
The ipsilateral lung is small and not as big as the pleural space; that's how God made this lung. You won't inflate the lung to fill the chest
Open
Statement
The pleural space will be obliterated either slowly by lung growth over 3-4 years or by filling with fluid; it's not a pneumothorax under pressure, it's pneumothorax ex vacuo
Open
Statement
Chest tube on water-seal suction will distort the mediastinum and precipitate a pulmonary hypertensive crisis; there's no reason to put in a chest tube unless there's active air leak or bleeding
Open
Statement
Typical stable post-op CDH baby will be unstable for about a day with increased ventilator requirements, then improve and get extubated in 4-5 days
Open
Statement
CDH is a field defect affecting the entire foregut from pharynx to ligament of Treitz; these kids have disordered motility throughout
Open
Statement
All CDH patients should have a GI series showing dilated, ectatic, abnormal esophagus; manometry and impedance show abnormal esophageal and gastric motility and emptying
Open
Quotation
this business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
The foregut problem is not really reflux; calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture
Open
Statement
CDH babies do well with continuous feedings slowly condensed to bolus; surgical intervention for foregut issues is unusual and should be approached as palliation
Open
Statement
Nissen fundoplication is a poor operation for CDH kids (like for esophageal atresia) because their motility is abnormal; if surgery is needed, use a partial wrap with gastrostomy and real drainage procedure (Jaboulay-Finney type, not Heineke-Mikulicz)
Open
Statement
Dr. Stolar's multidisciplinary CDH clinic follows 450-500 patients for life, addressing heart, lung, foregut, nutrition, neurodevelopmental, and axial skeleton issues that emerge over time
Open
Statement
Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis; he recommends lifelong proton pump inhibitors and regular endoscopic surveillance
Open
Statement
CDH patients have increased incidence of attention deficit disorders and autism; early intervention for neuropsychiatric issues is recommended
Open
Statement
As a field defect, CDH causes asymmetric chest growth leading to pectus-like distortion; some patients need Nuss-type operations to rebuild chest wall
Open
Statement
Some CDH girls have no breast development on the hernia side as teenagers; combined Nuss operation and breast implant procedures have been performed
Open
Statement
CDH patients develop thoracolumbar scoliosis (not idiopathic scoliosis, as it's mostly in boys); early bracing programs help minimize this
Open
Statement
CDH recurrence is related to tension on tissues and tissue quality; it's a tough problem whether tissue-to-tissue or prosthetic repair
Open
Statement
Recurrence is more common with left-sided CDH than right because the liver plugs up the hole on the right
Open
Statement
For right-sided CDH, the most important consideration is determining where hepatic veins drain; not infrequently they enter directly into the right atrium, not the suprahepatic IVC
Open
Statement
If hepatic veins enter the right atrium directly, attempting to reduce the liver into the abdomen will cause a troublesome moment; you should not try to put that liver in the abdomen
Open
Statement
Hepatopulmonary fusion actually exists in right-sided CDH; the liver and lung are fused and cannot be separated surgically
Open
Statement
Most patients with hepatopulmonary fusion don't survive; they often have associated severe congenital heart disease and IVC interruption with azygous continuation
Open
Statement
For right-sided CDH, prep the baby for both thoracic and abdominal incisions; often need to be on both sides to figure out what's going on because the liver is in the way
Open
Statement
For right-sided CDH, can put a scope through an open thoracotomy to see around corners where the liver might come up against the heart
Open
Charlie's statements about Pulmonary Hypoplasia 203 statements
Open the Pulmonary Hypoplasia collection →
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
The most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach.
Open
Statement
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies managed by community obstetricians.
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
CDH diagnosis is usually made at the 20-week anatomy scan when ultrasonographers see the stomach in the same cross-sectional plane as the heart.
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side.
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation.
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best.
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability.
Open
Statement
In single-center experiences, presence of liver in the chest is of no prognostic value for CDH.
Open
Statement
Lung-to-head ratio (LHR) is of limited prognostic value except when very low (less than 0.8).
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis in CDH.
Open
Quotation
If you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
Steroids have tremendous value for preterm labor under 35 weeks but their role in near-term CDH babies (37-39 weeks) is arguable.
Open
Statement
Exit-to-ECMO for CDH is essentially moving the goalposts and deck chairs around on the Titanic for non-viable babies.
Open
Quotation
What you're now doing is putting what in my view is almost certainly a non-viable baby on a heart lung machine and basically moving the goalposts and moving the deck chairs around on the Titanic.
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth.
Open
Quotation
ECMO is a drug delivery system for oxygen. So if the end organs are getting enough oxygen, then you're doing fine.
Open
Statement
All therapy for CDH should be guided by preductal oximetry, not postductal, because all babies have some degree of pulmonary hypertension with shunting.
Open
Statement
If preductal saturation is 90% (PaO2 of 65 torr), the brain is doing fine because this is fetal hemoglobin.
Open
Quotation
If you ever wanted to find out if somebody tried to invent a neonatal ventilator today, they would throw it out as a lethal device because they will trash in the lungs in a heartbeat.
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today because they trash lungs in a heartbeat.
Open
Statement
CDH babies should not be paralyzed and should have minimal sedation to maintain spontaneous breathing.
Open
Statement
Most CDH babies don't tolerate conventional ventilator settings (rate 40, peak pressure 25-28, PEEP 5) and require unconventional high-rate (100 breaths/min) low-pressure ventilation.
Open
Statement
High-frequency oscillatory ventilation (HFOV) as rescue therapy rarely spares CDH babies from ECMO.
Open
Quotation
When I see them getting out the oscillator, I'll go prime a circuit. That's just how I think about it.
Open
Statement
Nitric oxide is a waste of money for CDH babies; meta-analyses show it's terrific for premature babies with immature lung disease but of no value in CDH.
Open
Statement
ECMO should not be used in babies under 36 weeks gestational age initially, though this has been pushed down to 35 weeks, maybe 34 weeks, with intracranial hemorrhage rates taking off below 32 weeks.
Open
Statement
The smallest ECMO arterial perfusion cannula available is about 8 French, and resistance is related to both length and diameter, making adequate flow difficult in very small babies.
Open
Statement
For ECMO candidacy, the real risk for intracranial hemorrhage is the germinal matrix, which is usually OK by 35-36 weeks gestation, so it's gestational age that matters, not size.
Open
Statement
The broad principle for ECMO candidacy is whether you can get out of ECMO with reasonable confidence if you get in—don't start something you can't finish.
Open
Statement
VV ECMO is terrific if the heart works, but in CDH the heart function is often depressed and it's hard to get the cannula in with the shifted mediastinum.
Open
Statement
VA bypass is essentially dialing in a PaO2, while VV ECMO is much more annoying with mixing, cannula position issues, and CDH babies are generally too unstable for VV.
Open
Statement
Echo guidance during ECMO cannulation is really helpful to prevent driving the arterial cannula out the subclavian artery or the venous cannula into the innominate vein.
Open
Quotation
If you drive it out the subclavian artery, you're going to have a very well perfused hand, and you think your preductal sats are looking really good, but the baby's not seeing the oxygen.
Open
Statement
If the arterial cannula goes out the subclavian artery, you'll have a very well-perfused hand and think preductal sats look good, but the baby isn't seeing the oxygen.
Open
Statement
The first few hours on ECMO can be unstable; hyperkalemia cardiac arrest can occur if blood isn't washed, but you just perfuse through it and give calcium.
Open
Statement
ECMO flow should be slowly increased over 45 minutes to 1.5 hours to about 100-125 cc/kg/min (about 80% of cardiac output) to reduce intracranial hemorrhage incidence.
Open
Statement
The hyperoxia test—turning FIO2 up to 1.0 on the ventilator while on ECMO—gives courage to begin weaning if the PaO2 rises, showing the baby can use their lungs.
Open
Statement
For babies stuck on ECMO at 2 weeks, ensure they are maximally dried out (bone dry, eyes sucked into back of head, turned into a prune) before considering on-ECMO repair.
Open
Statement
The problem in CDH is not that bowel is in the chest; the problem is growth arrest of the lungs that happened at 14-15 weeks gestation. Getting bowel out of the chest is not miraculous.
Open
Quotation
The problem is not that the bowel is up in the chest. The problem is that there's been a growth arrest of the lungs. And just there's nothing miraculous about getting the bowel out of the chest.
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) make clot on ECMO. A platelet thrombus lasts 48-72 hours, giving a window to operate and get off ECMO before bleeding starts.
Open
Statement
Preferred approach is to wean ECMO down to 20 cc/kg/min, do the operation, accept 1 day of post-op edema, then have 2-3 days to get off ECMO before bleeding starts.
Open
Statement
If you take a baby off ECMO then operate, every patient gets stiff after surgery, pulmonary hypertension relapses, and you're talking about a second ECMO run.
Open
Statement
When operating on ECMO, load with Amicar preoperatively, do it as an abdominal operation, use a patch with low threshold to avoid tension, place a Jackson-Pratt drain under the patch, and place a chest tube.
Open
Statement
The typical stable CDH baby not requiring ECMO takes 3-4 days to wean to minimal ventilator settings (FIO2 0.4, conventional settings) before repair.
Open
Statement
Use the infant ventilator instead of an anesthesia machine intraoperatively because infant anesthesia machines have high dead space and are not very compliant.
Open
Statement
For repair timing, three variables matter: pre/post-ductal gradient on pulse ox, right ventricle dilation on echo, and RV pressure versus LV pressure (RV should be no more than systemic).
Open
Statement
The thoracoscopic approach for CDH is gorgeous with a sparkling view, but the recurrence rate is really high—about 25% in under a year in Dr. Stolar's series of 35 stable kids.
Open
Statement
The APSA outcomes committee meta-analysis came to a very similar conclusion about high thoracoscopic CDH recurrence rates.
Open
Statement
For open CDH repair, need a real subcostal incision (not a small two-finger incision), rotate the liver out of chest and abdomen, eviscerate bowel, and mobilize the posterior leaflet like unrolling a window shade down to rib.
Open
Statement
The medial part of CDH repair is hardest because sometimes the esophagus or aorta is hanging out with nothing to sew to.
Open
Statement
When there's no diaphragm to sew to medially, make an upside-down U-shaped incision on the pericardium and rotate that down to where the diaphragm would be to begin the repair.
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike Vicryl which saws tissue.
Open
Statement
Favor non-biologic material like 1mm Gore-Tex for patches, and anchor the patch to the ribs laterally by getting a needle around the rib.
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out taking a deep breath; over time it gets incorporated into fibrous tissue.
Open
Statement
For thoracoscopic CDH repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and put solid organs (spleen or left liver lobe) in last to plug the hole and keep bowel down.
Open
Statement
If you can see through the diaphragm tissue (it's nothing but pleura and peritoneum), resect it back to muscle for a fresh edge to sew together, or the plication will fail.
Open
Statement
There is no indication for a chest tube in CDH except for active air leak or anticipated/active bleeding. The ipsilateral lung is small and won't fill the pleural space—that's how God made this lung.
Open
Statement
A pneumothorax in CDH is a pneumothorax ex vacuo, not air under pressure. Putting in a chest tube on water seal suction will just distort the mediastinum and precipitate a pulmonary hypertensive crisis.
Open
Statement
The typical stable CDH baby will be crummy for about a day postoperatively, then get better and be extubated in 4-5 days and go home.
Open
Statement
CDH is a field defect affecting the whole foregut from pharynx to ligament of Treitz. Everything—esophagus, stomach, gastric emptying—has abnormal motility.
Open
Statement
If you do a GI series on CDH kids, the esophagus will be very dilated, ectatic, and abnormal-looking. Manometry and impedance manometry are abnormal.
Open
Statement
It's not really reflux in CDH—calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture.
Open
Quotation
This business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
CDH kids generally do well with continuous feedings slowly condensed to bolus. It's unusual to need surgical intervention for foregut dysmotility.
Open
Statement
When surgery is needed for CDH foregut dysmotility, approach it as palliation: some sort of fundoplication (not Nissen—it's fully competent on an abnormal-motility esophagus), combined with gastrostomy and a real drainage procedure like Jaboulay-Finney, not Heineke-Mikulicz.
Open
Statement
Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis and now recommends lifelong proton pump inhibitors and regular scoping for all CDH patients.
Open
Statement
Dr. Stolar's 1989-1990 paper showed most CDH kids turn out OK unless the mother had an 8th grade education and the child is a boy.
Open
Statement
There is an incidence of attention deficit disorders and autism in CDH survivors, prompting early intervention for neuropsychiatric issues.
Open
Statement
Because CDH is a field defect, there's asymmetric chest growth leading to pectus-like distorted chest wall growth requiring Nuss-type operations in some patients.
Open
Statement
Some CDH girls as teenagers have no breast development on the side of the hernia, requiring Nuss operation and breast implant.
Open
Statement
CDH patients can develop non-idiopathic scoliosis (mostly in boys, not the typical idiopathic scoliosis in girls), requiring early bracing to minimize thoracolumbar scoliosis.
Open
Statement
For right-sided CDH, the most important consideration is to ask echocardiographers where the hepatic veins drain, because they often enter directly into the right atrium, not the suprahepatic cava.
Open
Statement
Hepatopulmonary fusion actually exists in right-sided CDH. The liver and lung are fused and cannot be separated surgically. Most of these patients don't survive and often have severe congenital heart disease and IVC discontinuation.
Open
Statement
For right-sided CDH, prep the baby for both thoracic and abdominal incisions because you often need to be on both sides. Start in the abdomen, but if the liver is in the way, make a counter-incision in the chest.
Open
Statement
Even with an open thoracotomy, you can put a scope in to see around a corner without ugly retractors.
Open
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
the most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
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
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
Open
Statement
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
Open
Statement
The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
Open
Statement
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
Open
Statement
Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
Open
Quotation
if you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
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
Open
Statement
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
Open
Statement
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
Open
Statement
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
Open
Statement
ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
Open
Statement
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
Open
Statement
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
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
Open
Statement
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
Open
Statement
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
Open
Statement
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
Open
Quotation
I often joke that when people say, how do you know when to set up an ECMO circuit with a diaphragmatic hernia, it's when they get out the oscillator.
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
The broad principle for ECMO candidacy is: do you have a reversible condition? Can you get out with reasonable confidence once you start?
Open
Statement
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
Open
Statement
VV ECMO is for less sick patients who aren't in extremis; most CDH babies are sicker and do better on VA ECMO
Open
Statement
VA bypass is basically dialing in a PaO2; VV has mixing issues, cannula position concerns, and is more annoying to manage
Open
Statement
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
Open
Statement
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
Open
Statement
Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
Open
Statement
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
Open
Statement
In the first hours on ECMO, hyperkalemia can cause cardiac arrest if blood isn't washed; just perfuse through it and give calcium
Open
Statement
ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
Open
Statement
Target VA ECMO flow is 100-125 cc/kg/min, which is about 80% of cardiac output assuming an open duct
Open
Statement
At target ECMO flow, preductal saturation will be good and mixed venous (from venous drainage) will come up to 65-70%
Open
Statement
Regular cardiac echos during ECMO weaning are important to assess right heart function, dilation, tricuspid regurgitation, and pulmonary outflow tract jet
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) are making clot; platelet thrombus lifespan is 48-72 hours
Open
Statement
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
Open
Statement
Operating off ECMO means the baby will get stiff post-operatively, pulmonary hypertension may relapse, and you risk needing a second ECMO run
Open
Statement
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
Open
Statement
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
Open
Statement
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
Open
Statement
Use the infant ventilator instead of anesthesia machine intraoperatively; infant anesthesia machines have high dead space and aren't very compliant
Open
Statement
Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
Open
Statement
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
Open
Statement
Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
Open
Statement
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
Open
Statement
In Dr. Stolar's series of 35 thoracoscopic CDH repairs in stable babies, recurrence rate was about 25% in under a year; APSA outcomes committee meta-analysis reached similar conclusions
Open
Statement
Laparoscopic CDH repair is problematic because the scaphoid abdomen has loss of abdominal domain; insufflating just puts air up in the chest
Open
Quotation
I was very disappointed at our own data because I thought the scope approach was gorgeous.
Open
Statement
For open repair, key is adequate subcostal incision (not a small two-finger incision); rotate liver out of chest and abdomen to see the defect
Open
Statement
Critical repair points are all medial where structures with names are located; mobilize posterior leaflet by unrolling it like a window shade down to the body wall until you feel rib
Open
Statement
The medial repair is hardest because sometimes the esophagus or aorta hangs out with nothing to sew to; use an upside-down U-shaped pericardial flap rotated down to where the diaphragm would be to begin the repair
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike braided suture like Vicryl
Open
Statement
For patches, favor non-biologic material like 1mm Gore-Tex; laterally, anchor the patch to the ribs by getting a needle around the rib and into the patch
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out with a deep breath; over time it gets incorporated into fibrous tissue
Open
Statement
For thoracoscopic repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and reduce spleen last as it obturates the hole and keeps bowel in the belly
Open
Statement
Thoracoscopically, mobilizing the posterior leaflet is difficult because it's rolled into the abdomen and hard to see around the corner
Open
Statement
For thoracoscopic patch placement, use extracorporeal-intracorporeal suture technique: come from outside through skin around rib into patch and back out, tie externally, and bury the knot under the skin
Open
Statement
If you can see through the diaphragm (it's just pleura and peritoneum with no muscle), resect it back to something that looks like muscle; plication of see-through tissue will fail
Open
Statement
The ipsilateral lung is small and not as big as the pleural space; that's how God made this lung. You won't inflate the lung to fill the chest
Open
Statement
The pleural space will be obliterated either slowly by lung growth over 3-4 years or by filling with fluid; it's not a pneumothorax under pressure, it's pneumothorax ex vacuo
Open
Statement
Chest tube on water-seal suction will distort the mediastinum and precipitate a pulmonary hypertensive crisis; there's no reason to put in a chest tube unless there's active air leak or bleeding
Open
Statement
Typical stable post-op CDH baby will be unstable for about a day with increased ventilator requirements, then improve and get extubated in 4-5 days
Open
Statement
CDH is a field defect affecting the entire foregut from pharynx to ligament of Treitz; these kids have disordered motility throughout
Open
Statement
All CDH patients should have a GI series showing dilated, ectatic, abnormal esophagus; manometry and impedance show abnormal esophageal and gastric motility and emptying
Open
Statement
The foregut problem is not really reflux; calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture
Open
Quotation
this business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
CDH babies do well with continuous feedings slowly condensed to bolus; surgical intervention for foregut issues is unusual and should be approached as palliation
Open
Statement
Nissen fundoplication is a poor operation for CDH kids (like for esophageal atresia) because their motility is abnormal; if surgery is needed, use a partial wrap with gastrostomy and real drainage procedure (Jaboulay-Finney type, not Heineke-Mikulicz)
Open
Statement
Dr. Stolar's multidisciplinary CDH clinic follows 450-500 patients for life, addressing heart, lung, foregut, nutrition, neurodevelopmental, and axial skeleton issues that emerge over time
Open
Statement
Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis; he recommends lifelong proton pump inhibitors and regular endoscopic surveillance
Open
Statement
CDH patients have increased incidence of attention deficit disorders and autism; early intervention for neuropsychiatric issues is recommended
Open
Statement
As a field defect, CDH causes asymmetric chest growth leading to pectus-like distortion; some patients need Nuss-type operations to rebuild chest wall
Open
Statement
Some CDH girls have no breast development on the hernia side as teenagers; combined Nuss operation and breast implant procedures have been performed
Open
Statement
CDH patients develop thoracolumbar scoliosis (not idiopathic scoliosis, as it's mostly in boys); early bracing programs help minimize this
Open
Statement
CDH recurrence is related to tension on tissues and tissue quality; it's a tough problem whether tissue-to-tissue or prosthetic repair
Open
Statement
Recurrence is more common with left-sided CDH than right because the liver plugs up the hole on the right
Open
Statement
For right-sided CDH, the most important consideration is determining where hepatic veins drain; not infrequently they enter directly into the right atrium, not the suprahepatic IVC
Open
Statement
If hepatic veins enter the right atrium directly, attempting to reduce the liver into the abdomen will cause a troublesome moment; you should not try to put that liver in the abdomen
Open
Statement
Hepatopulmonary fusion actually exists in right-sided CDH; the liver and lung are fused and cannot be separated surgically
Open
Statement
Most patients with hepatopulmonary fusion don't survive; they often have associated severe congenital heart disease and IVC interruption with azygous continuation
Open
Statement
For right-sided CDH, prep the baby for both thoracic and abdominal incisions; often need to be on both sides to figure out what's going on because the liver is in the way
Open
Statement
For right-sided CDH, can put a scope through an open thoracotomy to see around corners where the liver might come up against the heart
Open
Charlie's statements about Single Ventricle / HLHS 294 statements
Open the Single Ventricle / HLHS collection →
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Quotation
The most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach.
Open
Quotation
The most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach.
Open
Statement
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies managed by community obstetricians.
Open
Statement
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies managed by community obstetricians.
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Quotation
The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
Open
Statement
CDH diagnosis is usually made at the 20-week anatomy scan when ultrasonographers see the stomach in the same cross-sectional plane as the heart.
Open
Statement
CDH diagnosis is usually made at the 20-week anatomy scan when ultrasonographers see the stomach in the same cross-sectional plane as the heart.
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side.
Open
Statement
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side.
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation.
Open
Statement
At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation.
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best.
Open
Statement
Antenatal interventions for CDH are no better than investigational and experimental at best.
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability.
Open
Statement
Babies with CDH should be born at a full-service children's facility with ECMO capability.
Open
Statement
In single-center experiences, presence of liver in the chest is of no prognostic value for CDH.
Open
Statement
In single-center experiences, presence of liver in the chest is of no prognostic value for CDH.
Open
Statement
Lung-to-head ratio (LHR) is of limited prognostic value except when very low (less than 0.8).
Open
Statement
Lung-to-head ratio (LHR) is of limited prognostic value except when very low (less than 0.8).
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis in CDH.
Open
Statement
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis in CDH.
Open
Quotation
If you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Quotation
If you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
Open
Statement
Steroids have tremendous value for preterm labor under 35 weeks but their role in near-term CDH babies (37-39 weeks) is arguable.
Open
Statement
Steroids have tremendous value for preterm labor under 35 weeks but their role in near-term CDH babies (37-39 weeks) is arguable.
Open
Statement
Exit-to-ECMO for CDH is essentially moving the goalposts and deck chairs around on the Titanic for non-viable babies.
Open
Statement
Exit-to-ECMO for CDH is essentially moving the goalposts and deck chairs around on the Titanic for non-viable babies.
Open
Quotation
What you're now doing is putting what in my view is almost certainly a non-viable baby on a heart lung machine and basically moving the goalposts and moving the deck chairs around on the Titanic.
Open
Quotation
What you're now doing is putting what in my view is almost certainly a non-viable baby on a heart lung machine and basically moving the goalposts and moving the deck chairs around on the Titanic.
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth.
Open
Statement
We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth.
Open
Quotation
ECMO is a drug delivery system for oxygen. So if the end organs are getting enough oxygen, then you're doing fine.
Open
Quotation
ECMO is a drug delivery system for oxygen. So if the end organs are getting enough oxygen, then you're doing fine.
Open
Statement
All therapy for CDH should be guided by preductal oximetry, not postductal, because all babies have some degree of pulmonary hypertension with shunting.
Open
Statement
All therapy for CDH should be guided by preductal oximetry, not postductal, because all babies have some degree of pulmonary hypertension with shunting.
Open
Statement
If preductal saturation is 90% (PaO2 of 65 torr), the brain is doing fine because this is fetal hemoglobin.
Open
Statement
If preductal saturation is 90% (PaO2 of 65 torr), the brain is doing fine because this is fetal hemoglobin.
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today because they trash lungs in a heartbeat.
Open
Quotation
If you ever wanted to find out if somebody tried to invent a neonatal ventilator today, they would throw it out as a lethal device because they will trash in the lungs in a heartbeat.
Open
Statement
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today because they trash lungs in a heartbeat.
Open
Statement
CDH babies should not be paralyzed and should have minimal sedation to maintain spontaneous breathing.
Open
Statement
CDH babies should not be paralyzed and should have minimal sedation to maintain spontaneous breathing.
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Statement
Most CDH babies don't tolerate conventional ventilator settings (rate 40, peak pressure 25-28, PEEP 5) and require unconventional high-rate (100 breaths/min) low-pressure ventilation.
Open
Statement
Most CDH babies don't tolerate conventional ventilator settings (rate 40, peak pressure 25-28, PEEP 5) and require unconventional high-rate (100 breaths/min) low-pressure ventilation.
Open
Statement
High-frequency oscillatory ventilation (HFOV) as rescue therapy rarely spares CDH babies from ECMO.
Open
Statement
High-frequency oscillatory ventilation (HFOV) as rescue therapy rarely spares CDH babies from ECMO.
Open
Quotation
When I see them getting out the oscillator, I'll go prime a circuit. That's just how I think about it.
Open
Quotation
When I see them getting out the oscillator, I'll go prime a circuit. That's just how I think about it.
Open
Statement
Nitric oxide is a waste of money for CDH babies; meta-analyses show it's terrific for premature babies with immature lung disease but of no value in CDH.
Open
Statement
Nitric oxide is a waste of money for CDH babies; meta-analyses show it's terrific for premature babies with immature lung disease but of no value in CDH.
Open
Statement
ECMO should not be used in babies under 36 weeks gestational age initially, though this has been pushed down to 35 weeks, maybe 34 weeks, with intracranial hemorrhage rates taking off below 32 weeks.
Open
Statement
ECMO should not be used in babies under 36 weeks gestational age initially, though this has been pushed down to 35 weeks, maybe 34 weeks, with intracranial hemorrhage rates taking off below 32 weeks.
Open
Statement
The smallest ECMO arterial perfusion cannula available is about 8 French, and resistance is related to both length and diameter, making adequate flow difficult in very small babies.
Open
Statement
The smallest ECMO arterial perfusion cannula available is about 8 French, and resistance is related to both length and diameter, making adequate flow difficult in very small babies.
Open
Statement
For ECMO candidacy, the real risk for intracranial hemorrhage is the germinal matrix, which is usually OK by 35-36 weeks gestation, so it's gestational age that matters, not size.
Open
Statement
For ECMO candidacy, the real risk for intracranial hemorrhage is the germinal matrix, which is usually OK by 35-36 weeks gestation, so it's gestational age that matters, not size.
Open
Statement
The broad principle for ECMO candidacy is whether you can get out of ECMO with reasonable confidence if you get in—don't start something you can't finish.
Open
Statement
The broad principle for ECMO candidacy is whether you can get out of ECMO with reasonable confidence if you get in—don't start something you can't finish.
Open
Statement
VV ECMO is terrific if the heart works, but in CDH the heart function is often depressed and it's hard to get the cannula in with the shifted mediastinum.
Open
Statement
VV ECMO is terrific if the heart works, but in CDH the heart function is often depressed and it's hard to get the cannula in with the shifted mediastinum.
Open
Statement
VA bypass is essentially dialing in a PaO2, while VV ECMO is much more annoying with mixing, cannula position issues, and CDH babies are generally too unstable for VV.
Open
Statement
VA bypass is essentially dialing in a PaO2, while VV ECMO is much more annoying with mixing, cannula position issues, and CDH babies are generally too unstable for VV.
Open
Statement
Echo guidance during ECMO cannulation is really helpful to prevent driving the arterial cannula out the subclavian artery or the venous cannula into the innominate vein.
Open
Statement
Echo guidance during ECMO cannulation is really helpful to prevent driving the arterial cannula out the subclavian artery or the venous cannula into the innominate vein.
Open
Statement
If the arterial cannula goes out the subclavian artery, you'll have a very well-perfused hand and think preductal sats look good, but the baby isn't seeing the oxygen.
Open
Quotation
If you drive it out the subclavian artery, you're going to have a very well perfused hand, and you think your preductal sats are looking really good, but the baby's not seeing the oxygen.
Open
Quotation
If you drive it out the subclavian artery, you're going to have a very well perfused hand, and you think your preductal sats are looking really good, but the baby's not seeing the oxygen.
Open
Statement
If the arterial cannula goes out the subclavian artery, you'll have a very well-perfused hand and think preductal sats look good, but the baby isn't seeing the oxygen.
Open
Statement
The first few hours on ECMO can be unstable; hyperkalemia cardiac arrest can occur if blood isn't washed, but you just perfuse through it and give calcium.
Open
Statement
The first few hours on ECMO can be unstable; hyperkalemia cardiac arrest can occur if blood isn't washed, but you just perfuse through it and give calcium.
Open
Statement
ECMO flow should be slowly increased over 45 minutes to 1.5 hours to about 100-125 cc/kg/min (about 80% of cardiac output) to reduce intracranial hemorrhage incidence.
Open
Statement
ECMO flow should be slowly increased over 45 minutes to 1.5 hours to about 100-125 cc/kg/min (about 80% of cardiac output) to reduce intracranial hemorrhage incidence.
Open
Statement
The hyperoxia test—turning FIO2 up to 1.0 on the ventilator while on ECMO—gives courage to begin weaning if the PaO2 rises, showing the baby can use their lungs.
Open
Statement
The hyperoxia test—turning FIO2 up to 1.0 on the ventilator while on ECMO—gives courage to begin weaning if the PaO2 rises, showing the baby can use their lungs.
Open
Statement
For babies stuck on ECMO at 2 weeks, ensure they are maximally dried out (bone dry, eyes sucked into back of head, turned into a prune) before considering on-ECMO repair.
Open
Statement
For babies stuck on ECMO at 2 weeks, ensure they are maximally dried out (bone dry, eyes sucked into back of head, turned into a prune) before considering on-ECMO repair.
Open
Statement
The problem in CDH is not that bowel is in the chest; the problem is growth arrest of the lungs that happened at 14-15 weeks gestation. Getting bowel out of the chest is not miraculous.
Open
Statement
The problem in CDH is not that bowel is in the chest; the problem is growth arrest of the lungs that happened at 14-15 weeks gestation. Getting bowel out of the chest is not miraculous.
Open
Quotation
The problem is not that the bowel is up in the chest. The problem is that there's been a growth arrest of the lungs. And just there's nothing miraculous about getting the bowel out of the chest.
Open
Quotation
The problem is not that the bowel is up in the chest. The problem is that there's been a growth arrest of the lungs. And just there's nothing miraculous about getting the bowel out of the chest.
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) make clot on ECMO. A platelet thrombus lasts 48-72 hours, giving a window to operate and get off ECMO before bleeding starts.
Open
Statement
Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) make clot on ECMO. A platelet thrombus lasts 48-72 hours, giving a window to operate and get off ECMO before bleeding starts.
Open
Statement
Preferred approach is to wean ECMO down to 20 cc/kg/min, do the operation, accept 1 day of post-op edema, then have 2-3 days to get off ECMO before bleeding starts.
Open
Statement
Preferred approach is to wean ECMO down to 20 cc/kg/min, do the operation, accept 1 day of post-op edema, then have 2-3 days to get off ECMO before bleeding starts.
Open
Statement
If you take a baby off ECMO then operate, every patient gets stiff after surgery, pulmonary hypertension relapses, and you're talking about a second ECMO run.
Open
Statement
If you take a baby off ECMO then operate, every patient gets stiff after surgery, pulmonary hypertension relapses, and you're talking about a second ECMO run.
Open
Statement
When operating on ECMO, load with Amicar preoperatively, do it as an abdominal operation, use a patch with low threshold to avoid tension, place a Jackson-Pratt drain under the patch, and place a chest tube.
Open
Statement
When operating on ECMO, load with Amicar preoperatively, do it as an abdominal operation, use a patch with low threshold to avoid tension, place a Jackson-Pratt drain under the patch, and place a chest tube.
Open
Statement
The typical stable CDH baby not requiring ECMO takes 3-4 days to wean to minimal ventilator settings (FIO2 0.4, conventional settings) before repair.
Open
Statement
The typical stable CDH baby not requiring ECMO takes 3-4 days to wean to minimal ventilator settings (FIO2 0.4, conventional settings) before repair.
Open
Statement
Use the infant ventilator instead of an anesthesia machine intraoperatively because infant anesthesia machines have high dead space and are not very compliant.
Open
Statement
Use the infant ventilator instead of an anesthesia machine intraoperatively because infant anesthesia machines have high dead space and are not very compliant.
Open
Statement
For repair timing, three variables matter: pre/post-ductal gradient on pulse ox, right ventricle dilation on echo, and RV pressure versus LV pressure (RV should be no more than systemic).
Open
Statement
For repair timing, three variables matter: pre/post-ductal gradient on pulse ox, right ventricle dilation on echo, and RV pressure versus LV pressure (RV should be no more than systemic).
Open
Statement
The thoracoscopic approach for CDH is gorgeous with a sparkling view, but the recurrence rate is really high—about 25% in under a year in Dr. Stolar's series of 35 stable kids.
Open
Statement
The thoracoscopic approach for CDH is gorgeous with a sparkling view, but the recurrence rate is really high—about 25% in under a year in Dr. Stolar's series of 35 stable kids.
Open
Statement
The APSA outcomes committee meta-analysis came to a very similar conclusion about high thoracoscopic CDH recurrence rates.
Open
Statement
The APSA outcomes committee meta-analysis came to a very similar conclusion about high thoracoscopic CDH recurrence rates.
Open
Statement
For open CDH repair, need a real subcostal incision (not a small two-finger incision), rotate the liver out of chest and abdomen, eviscerate bowel, and mobilize the posterior leaflet like unrolling a window shade down to rib.
Open
Statement
For open CDH repair, need a real subcostal incision (not a small two-finger incision), rotate the liver out of chest and abdomen, eviscerate bowel, and mobilize the posterior leaflet like unrolling a window shade down to rib.
Open
Statement
The medial part of CDH repair is hardest because sometimes the esophagus or aorta is hanging out with nothing to sew to.
Open
Statement
The medial part of CDH repair is hardest because sometimes the esophagus or aorta is hanging out with nothing to sew to.
Open
Statement
When there's no diaphragm to sew to medially, make an upside-down U-shaped incision on the pericardium and rotate that down to where the diaphragm would be to begin the repair.
Open
Statement
When there's no diaphragm to sew to medially, make an upside-down U-shaped incision on the pericardium and rotate that down to where the diaphragm would be to begin the repair.
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike Vicryl which saws tissue.
Open
Statement
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike Vicryl which saws tissue.
Open
Statement
Favor non-biologic material like 1mm Gore-Tex for patches, and anchor the patch to the ribs laterally by getting a needle around the rib.
Open
Statement
Favor non-biologic material like 1mm Gore-Tex for patches, and anchor the patch to the ribs laterally by getting a needle around the rib.
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out taking a deep breath; over time it gets incorporated into fibrous tissue.
Open
Statement
Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out taking a deep breath; over time it gets incorporated into fibrous tissue.
Open
Statement
For thoracoscopic CDH repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and put solid organs (spleen or left liver lobe) in last to plug the hole and keep bowel down.
Open
Statement
For thoracoscopic CDH repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and put solid organs (spleen or left liver lobe) in last to plug the hole and keep bowel down.
Open
Statement
If you can see through the diaphragm tissue (it's nothing but pleura and peritoneum), resect it back to muscle for a fresh edge to sew together, or the plication will fail.
Open
Statement
If you can see through the diaphragm tissue (it's nothing but pleura and peritoneum), resect it back to muscle for a fresh edge to sew together, or the plication will fail.
Open
Statement
There is no indication for a chest tube in CDH except for active air leak or anticipated/active bleeding. The ipsilateral lung is small and won't fill the pleural space—that's how God made this lung.
Open
Statement
There is no indication for a chest tube in CDH except for active air leak or anticipated/active bleeding. The ipsilateral lung is small and won't fill the pleural space—that's how God made this lung.
Open
Statement
A pneumothorax in CDH is a pneumothorax ex vacuo, not air under pressure. Putting in a chest tube on water seal suction will just distort the mediastinum and precipitate a pulmonary hypertensive crisis.
Open
Statement
A pneumothorax in CDH is a pneumothorax ex vacuo, not air under pressure. Putting in a chest tube on water seal suction will just distort the mediastinum and precipitate a pulmonary hypertensive crisis.
Open
Statement
The typical stable CDH baby will be crummy for about a day postoperatively, then get better and be extubated in 4-5 days and go home.
Open
Statement
The typical stable CDH baby will be crummy for about a day postoperatively, then get better and be extubated in 4-5 days and go home.
Open
Statement
CDH is a field defect affecting the whole foregut from pharynx to ligament of Treitz. Everything—esophagus, stomach, gastric emptying—has abnormal motility.
Open
Statement
CDH is a field defect affecting the whole foregut from pharynx to ligament of Treitz. Everything—esophagus, stomach, gastric emptying—has abnormal motility.
Open
Statement
If you do a GI series on CDH kids, the esophagus will be very dilated, ectatic, and abnormal-looking. Manometry and impedance manometry are abnormal.
Open
Statement
If you do a GI series on CDH kids, the esophagus will be very dilated, ectatic, and abnormal-looking. Manometry and impedance manometry are abnormal.
Open
Quotation
This business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
It's not really reflux in CDH—calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture.
Open
Statement
It's not really reflux in CDH—calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture.
Open
Quotation
This business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
Open
Statement
CDH kids generally do well with continuous feedings slowly condensed to bolus. It's unusual to need surgical intervention for foregut dysmotility.
Open
Statement
CDH kids generally do well with continuous feedings slowly condensed to bolus. It's unusual to need surgical intervention for foregut dysmotility.
Open
Statement
When surgery is needed for CDH foregut dysmotility, approach it as palliation: some sort of fundoplication (not Nissen—it's fully competent on an abnormal-motility esophagus), combined with gastrostomy and a real drainage procedure like Jaboulay-Finney, not Heineke-Mikulicz.
Open
Statement
When surgery is needed for CDH foregut dysmotility, approach it as palliation: some sort of fundoplication (not Nissen—it's fully competent on an abnormal-motility esophagus), combined with gastrostomy and a real drainage procedure like Jaboulay-Finney, not Heineke-Mikulicz.
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Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis and now recommends lifelong proton pump inhibitors and regular scoping for all CDH patients.
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Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis and now recommends lifelong proton pump inhibitors and regular scoping for all CDH patients.
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Dr. Stolar's 1989-1990 paper showed most CDH kids turn out OK unless the mother had an 8th grade education and the child is a boy.
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Dr. Stolar's 1989-1990 paper showed most CDH kids turn out OK unless the mother had an 8th grade education and the child is a boy.
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There is an incidence of attention deficit disorders and autism in CDH survivors, prompting early intervention for neuropsychiatric issues.
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There is an incidence of attention deficit disorders and autism in CDH survivors, prompting early intervention for neuropsychiatric issues.
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Because CDH is a field defect, there's asymmetric chest growth leading to pectus-like distorted chest wall growth requiring Nuss-type operations in some patients.
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Because CDH is a field defect, there's asymmetric chest growth leading to pectus-like distorted chest wall growth requiring Nuss-type operations in some patients.
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Some CDH girls as teenagers have no breast development on the side of the hernia, requiring Nuss operation and breast implant.
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Some CDH girls as teenagers have no breast development on the side of the hernia, requiring Nuss operation and breast implant.
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CDH patients can develop non-idiopathic scoliosis (mostly in boys, not the typical idiopathic scoliosis in girls), requiring early bracing to minimize thoracolumbar scoliosis.
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CDH patients can develop non-idiopathic scoliosis (mostly in boys, not the typical idiopathic scoliosis in girls), requiring early bracing to minimize thoracolumbar scoliosis.
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For right-sided CDH, the most important consideration is to ask echocardiographers where the hepatic veins drain, because they often enter directly into the right atrium, not the suprahepatic cava.
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For right-sided CDH, the most important consideration is to ask echocardiographers where the hepatic veins drain, because they often enter directly into the right atrium, not the suprahepatic cava.
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Hepatopulmonary fusion actually exists in right-sided CDH. The liver and lung are fused and cannot be separated surgically. Most of these patients don't survive and often have severe congenital heart disease and IVC discontinuation.
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Hepatopulmonary fusion actually exists in right-sided CDH. The liver and lung are fused and cannot be separated surgically. Most of these patients don't survive and often have severe congenital heart disease and IVC discontinuation.
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For right-sided CDH, prep the baby for both thoracic and abdominal incisions because you often need to be on both sides. Start in the abdomen, but if the liver is in the way, make a counter-incision in the chest.
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For right-sided CDH, prep the baby for both thoracic and abdominal incisions because you often need to be on both sides. Start in the abdomen, but if the liver is in the way, make a counter-incision in the chest.
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Even with an open thoracotomy, you can put a scope in to see around a corner without ugly retractors.
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Even with an open thoracotomy, you can put a scope in to see around a corner without ugly retractors.
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Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
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the most important understanding of the word doctor means to teach. It has nothing to do with healing, means to teach
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The discussion begins with telling parents that the chances are they're going to raise an obnoxious teenager. That's sort of how this story usually ends.
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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
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CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
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At birth, CDH lungs are affected by a mix of pulmonary hypoplasia and altered pulmonary vascular resistance with altered transitional circulation
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CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
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The diagnosis of CDH alone is not an indication for cesarean section; elective spontaneous vaginal delivery is recommended assuming no obstetric issues
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Antenatal interventions for CDH are no better than investigational and experimental at best
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Babies with CDH should be born at a full-service children's facility with ECMO capability; maybe 10-15% will benefit from ECMO
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In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
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Lung-to-head ratio is of limited prognostic value except when very low (less than 0.8), where prognosis is concerning
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Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
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if you show me 100 children with diaphragmatic hernia, 80 to 85% are going to turn into obnoxious teenagers, and that's what I tell them.
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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
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Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
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We are born with about 1/2 to 2/3 of our full complement of alveoli and can grow the balance sometime after birth
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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
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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
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ECMO is a drug delivery system for oxygen; indication is when end organs aren't getting enough oxygen despite best care
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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
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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
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Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
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CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
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Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
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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
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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
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I often joke that when people say, how do you know when to set up an ECMO circuit with a diaphragmatic hernia, it's when they get out the oscillator.
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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
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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
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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
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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
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The broad principle for ECMO candidacy is: do you have a reversible condition? Can you get out with reasonable confidence once you start?
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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
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VV ECMO is for less sick patients who aren't in extremis; most CDH babies are sicker and do better on VA ECMO
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VA bypass is basically dialing in a PaO2; VV has mixing issues, cannula position concerns, and is more annoying to manage
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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
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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
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Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
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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
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In the first hours on ECMO, hyperkalemia can cause cardiac arrest if blood isn't washed; just perfuse through it and give calcium
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ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
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Target VA ECMO flow is 100-125 cc/kg/min, which is about 80% of cardiac output assuming an open duct
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At target ECMO flow, preductal saturation will be good and mixed venous (from venous drainage) will come up to 65-70%
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Regular cardiac echos during ECMO weaning are important to assess right heart function, dilation, tricuspid regurgitation, and pulmonary outflow tract jet
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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
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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
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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
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Heparin inhibits conversion of fibrinogen to fibrin, so only platelets (beat-up ones) are making clot; platelet thrombus lifespan is 48-72 hours
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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
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Operating off ECMO means the baby will get stiff post-operatively, pulmonary hypertension may relapse, and you risk needing a second ECMO run
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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
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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
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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
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Use the infant ventilator instead of anesthesia machine intraoperatively; infant anesthesia machines have high dead space and aren't very compliant
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Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
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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
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Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
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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
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In Dr. Stolar's series of 35 thoracoscopic CDH repairs in stable babies, recurrence rate was about 25% in under a year; APSA outcomes committee meta-analysis reached similar conclusions
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Laparoscopic CDH repair is problematic because the scaphoid abdomen has loss of abdominal domain; insufflating just puts air up in the chest
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I was very disappointed at our own data because I thought the scope approach was gorgeous.
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For open repair, key is adequate subcostal incision (not a small two-finger incision); rotate liver out of chest and abdomen to see the defect
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Critical repair points are all medial where structures with names are located; mobilize posterior leaflet by unrolling it like a window shade down to the body wall until you feel rib
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The medial repair is hardest because sometimes the esophagus or aorta hangs out with nothing to sew to; use an upside-down U-shaped pericardial flap rotated down to where the diaphragm would be to begin the repair
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Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike braided suture like Vicryl
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For patches, favor non-biologic material like 1mm Gore-Tex; laterally, anchor the patch to the ribs by getting a needle around the rib and into the patch
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Make the patch somewhat balloon-shaped with redundancy so the baby doesn't rip sutures out with a deep breath; over time it gets incorporated into fibrous tissue
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For thoracoscopic repair, use 3 ports (4mm camera with 30-degree lens, 3mm neonatal instruments), insufflation peak pressure 5-7 cm (no more), and reduce spleen last as it obturates the hole and keeps bowel in the belly
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Thoracoscopically, mobilizing the posterior leaflet is difficult because it's rolled into the abdomen and hard to see around the corner
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For thoracoscopic patch placement, use extracorporeal-intracorporeal suture technique: come from outside through skin around rib into patch and back out, tie externally, and bury the knot under the skin
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If you can see through the diaphragm (it's just pleura and peritoneum with no muscle), resect it back to something that looks like muscle; plication of see-through tissue will fail
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The ipsilateral lung is small and not as big as the pleural space; that's how God made this lung. You won't inflate the lung to fill the chest
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The pleural space will be obliterated either slowly by lung growth over 3-4 years or by filling with fluid; it's not a pneumothorax under pressure, it's pneumothorax ex vacuo
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Chest tube on water-seal suction will distort the mediastinum and precipitate a pulmonary hypertensive crisis; there's no reason to put in a chest tube unless there's active air leak or bleeding
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Typical stable post-op CDH baby will be unstable for about a day with increased ventilator requirements, then improve and get extubated in 4-5 days
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CDH is a field defect affecting the entire foregut from pharynx to ligament of Treitz; these kids have disordered motility throughout
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All CDH patients should have a GI series showing dilated, ectatic, abnormal esophagus; manometry and impedance show abnormal esophageal and gastric motility and emptying
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Quotation
this business about calling it reflux has suckered all kinds of surgeons into doing fundoplications and pyloroplasties and all kinds of stuff in these kids that basically is torture.
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The foregut problem is not really reflux; calling it reflux has suckered surgeons into doing fundoplications and pyloroplasties that are basically torture
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CDH babies do well with continuous feedings slowly condensed to bolus; surgical intervention for foregut issues is unusual and should be approached as palliation
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Nissen fundoplication is a poor operation for CDH kids (like for esophageal atresia) because their motility is abnormal; if surgery is needed, use a partial wrap with gastrostomy and real drainage procedure (Jaboulay-Finney type, not Heineke-Mikulicz)
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Dr. Stolar's multidisciplinary CDH clinic follows 450-500 patients for life, addressing heart, lung, foregut, nutrition, neurodevelopmental, and axial skeleton issues that emerge over time
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Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis; he recommends lifelong proton pump inhibitors and regular endoscopic surveillance
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CDH patients have increased incidence of attention deficit disorders and autism; early intervention for neuropsychiatric issues is recommended
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As a field defect, CDH causes asymmetric chest growth leading to pectus-like distortion; some patients need Nuss-type operations to rebuild chest wall
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Some CDH girls have no breast development on the hernia side as teenagers; combined Nuss operation and breast implant procedures have been performed
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CDH patients develop thoracolumbar scoliosis (not idiopathic scoliosis, as it's mostly in boys); early bracing programs help minimize this
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CDH recurrence is related to tension on tissues and tissue quality; it's a tough problem whether tissue-to-tissue or prosthetic repair
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Recurrence is more common with left-sided CDH than right because the liver plugs up the hole on the right
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For right-sided CDH, the most important consideration is determining where hepatic veins drain; not infrequently they enter directly into the right atrium, not the suprahepatic IVC
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If hepatic veins enter the right atrium directly, attempting to reduce the liver into the abdomen will cause a troublesome moment; you should not try to put that liver in the abdomen
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Hepatopulmonary fusion actually exists in right-sided CDH; the liver and lung are fused and cannot be separated surgically
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Most patients with hepatopulmonary fusion don't survive; they often have associated severe congenital heart disease and IVC interruption with azygous continuation
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For right-sided CDH, prep the baby for both thoracic and abdominal incisions; often need to be on both sides to figure out what's going on because the liver is in the way
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For right-sided CDH, can put a scope through an open thoracotomy to see around corners where the liver might come up against the heart
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Summaries Charlie gave as host · 4 summaries
Recaps of other experts' statements, not Charlie's own clinical position.
Summaries Charlie gave as host · Congenital Diaphragmatic Hernia 2 summaries
Open the Congenital Diaphragmatic Hernia collection →
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Host summary
Charlie Stolar summarizing a resource: If you ever wanted to find out if somebody tried to invent a neonatal ventilator today, they would throw it out as a lethal device because they will trash in the lungs in a heartbeat.
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Summaries Charlie gave as host · Single Ventricle / HLHS 2 summaries
Open the Single Ventricle / HLHS collection →
Congenital Diaphragmatic Hernia with Dr. Charlie Stolar
Host summary
Charlie Stolar summarizing a resource: If you ever wanted to find out if somebody tried to invent a neonatal ventilator today, they would throw it out as a lethal device because they will trash in the lungs in a heartbeat.
Open