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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
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Ep 1 · 2:13
quote
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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Ep 1 · 2:45
epidemiological
CDH occurs in approximately 1 out of every 3,000-4,000 pregnancies
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Ep 1 · 3:11
quote
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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Ep 1 · 3:37
clinical
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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Ep 1 · 4:14
clinical
CDH represents a growth arrest of both lungs, with the ipsilateral side more severely affected than the contralateral side
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Ep 1 · 4:23
clinical
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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Ep 1 · 4:39
clinical
CDH is a field defect; the most common neonatal comorbidity is foregut motility difficulty
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Ep 1 · 5:00
clinical
CDH is a medical physiologic emergency but not a surgical emergency
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Ep 1 · 5:24
guideline
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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Ep 1 · 6:35
opinion
Antenatal interventions for CDH are no better than investigational and experimental at best
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Ep 1 · 7:23
guideline
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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Ep 1 · 8:31
clinical
In single-center experiences, presence of liver in the chest or stomach in the chest is of no prognostic value
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Ep 1 · 8:42
clinical
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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Ep 1 · 9:07
clinical
Associated congenital heart disease and central nervous system abnormalities augur for poor prognosis
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Ep 1 · 10:13
epidemiological
If shown 100 children with CDH, 80-85% will survive to become teenagers
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Ep 1 · 10:13
quote
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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Ep 1 · 10:37
clinical
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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Ep 1 · 12:06
quote
I think that's nonsense.
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Ep 1 · 12:06
opinion
Exit to ECMO for CDH is nonsense; it moves the goalposts on almost certainly non-viable babies
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Ep 1 · 13:00
clinical
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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Ep 1 · 14:45
clinical
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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Ep 1 · 15:55
clinical
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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Ep 1 · 17:06
clinical
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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Ep 1 · 17:44
clinical
The best way to assess end-organ function is urine output
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Ep 1 · 18:21
guideline
ECMO indication is typically oxygenation index in excess of 40 for 4 hours or more
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Ep 1 · 19:06
clinical
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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Ep 1 · 20:25
clinical
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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Ep 1 · 21:38
opinion
Neonatal ventilators would be thrown out as lethal devices if someone tried to invent them today
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Ep 1 · 21:56
clinical
CDH babies are not paralyzed during ventilation; minimal sedation is used and babies should be awake and breathing spontaneously
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Ep 1 · 22:15
clinical
Initial conventional ventilator settings (IMV rate ~40, peak pressure 25-28, PEEP ~5) are not tolerated by most CDH babies
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Ep 1 · 22:53
clinical
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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Ep 1 · 24:30
quote
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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Ep 1 · 24:30
clinical
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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Ep 1 · 25:45
opinion
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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Ep 1 · 25:45
quote
Nitric oxide is a waste of money.
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Ep 1 · 26:07
opinion
The best drug for CDH is oxygen
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Ep 1 · 26:07
quote
The best drug for diaphragmatic hernia is oxygen.
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Ep 1 · 26:22
clinical
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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Ep 1 · 27:40
clinical
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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Ep 1 · 28:15
clinical
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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Ep 1 · 28:45
clinical
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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Ep 1 · 30:27
clinical
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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Ep 1 · 31:00
clinical
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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Ep 1 · 31:25
quote
there ain't nothing like VA bypass.
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Ep 1 · 31:53
clinical
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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Ep 1 · 32:17
clinical
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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Ep 1 · 33:01
clinical
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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Ep 1 · 33:23
clinical
Using a guide wire to position the venous cannula into the right atrium is helpful when the mediastinum is distorted
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Ep 1 · 33:55
clinical
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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Ep 1 · 34:47
clinical
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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Ep 1 · 34:55
clinical
ECMO flow must be increased slowly over 45-90 minutes to reduce intracranial hemorrhage incidence
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Ep 1 · 35:10
clinical
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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Ep 1 · 35:29
clinical
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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Ep 1 · 36:12
clinical
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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Ep 1 · 36:34
clinical
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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Ep 1 · 37:38
quote
I leave town after 2 weeks.
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Ep 1 · 38:18
clinical
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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Ep 1 · 39:05
clinical
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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Ep 1 · 39:39
quote
This is not a space occupying lesion is not the problem here
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Ep 1 · 40:40
clinical
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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Ep 1 · 41:07
clinical
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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Ep 1 · 41:51
clinical
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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Ep 1 · 42:39
clinical
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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Ep 1 · 44:11
clinical
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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Ep 1 · 45:42
clinical
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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Ep 1 · 46:07
clinical
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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Ep 1 · 46:28
clinical
Anesthesia can be all intravenous (muscle relaxants and narcotics); you don't need an anesthesia machine
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Ep 1 · 46:47
clinical
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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Ep 1 · 47:57
clinical
Perioperative antibiotics (typically ampicillin-gentamicin) are given because a patch may be placed in a newborn
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Ep 1 · 48:32
quote
The thoracoscopic approach is gorgeous. It is just a gorgeous view.
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Ep 1 · 48:32
clinical
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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Ep 1 · 49:37
clinical
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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Ep 1 · 50:21
opinion
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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Ep 1 · 53:01
quote
I was very disappointed at our own data because I thought the scope approach was gorgeous.
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Ep 1 · 54:58
clinical
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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Ep 1 · 55:31
clinical
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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Ep 1 · 56:11
clinical
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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Ep 1 · 57:16
opinion
Favor monofilament suture (like PDS) because it doesn't saw through tissue when pulled, unlike braided suture like Vicryl
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Ep 1 · 57:59
clinical
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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Ep 1 · 58:43
clinical
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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Ep 1 · 1:00:23
clinical
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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Ep 1 · 1:01:35
clinical
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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Ep 1 · 1:02:03
clinical
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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Ep 1 · 1:04:16
clinical
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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Ep 1 · 1:06:31
quote
neonatal nurses, they pee around their isolate to mark their territory.
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Ep 1 · 1:06:47
clinical
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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Ep 1 · 1:06:53
quote
That's how God made this lung.
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Ep 1 · 1:07:01
clinical
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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Ep 1 · 1:07:18
clinical
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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Ep 1 · 1:08:53
clinical
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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Ep 1 · 1:09:34
clinical
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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Ep 1 · 1:09:51
clinical
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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Ep 1 · 1:10:09
opinion
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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Ep 1 · 1:10:09
quote
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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Ep 1 · 1:10:28
clinical
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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Ep 1 · 1:10:40
opinion
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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Ep 1 · 1:12:11
clinical
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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Ep 1 · 1:13:18
clinical
Dr. Stolar has 4 CDH teenagers with Barrett's esophagitis; he recommends lifelong proton pump inhibitors and regular endoscopic surveillance
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Ep 1 · 1:13:57
clinical
CDH patients have increased incidence of attention deficit disorders and autism; early intervention for neuropsychiatric issues is recommended
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Ep 1 · 1:14:09
clinical
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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Ep 1 · 1:14:29
clinical
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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Ep 1 · 1:14:34
clinical
CDH patients develop thoracolumbar scoliosis (not idiopathic scoliosis, as it's mostly in boys); early bracing programs help minimize this
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Ep 1 · 1:15:16
clinical
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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Ep 1 · 1:17:35
clinical
Recurrence is more common with left-sided CDH than right because the liver plugs up the hole on the right
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Ep 1 · 1:17:51
clinical
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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Ep 1 · 1:18:09
clinical
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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Ep 1 · 1:18:29
clinical
Hepatopulmonary fusion actually exists in right-sided CDH; the liver and lung are fused and cannot be separated surgically
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Ep 1 · 1:19:11
clinical
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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Ep 1 · 1:19:48
clinical
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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Ep 1 · 1:20:12
clinical
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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