Term
| Congenital Diaphragmatic Hernia (CHD) accounts for what % of all major congenital anomalies? |
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Definition
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Term
| CHD ain't just about lungs and guts...what's the chance that the neonate has a concurrent heart condition? |
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Definition
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Term
| T or F: CHD previously had poor outcomes but mortality has been greatly reduced due to surgical and anesthetic improvements. |
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Definition
| F: perioperative mortality for CDH is 33-66% and has NOT improved for over 25 years! |
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Term
| What causes CHD to happen? |
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Definition
| Incomplete closure of the pleural and peritoneal canal with herniation of abdominal organs into the thorax at the 8th week of gestation. |
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Term
| Do we need to know what Frynn Syndrome is (besides that it's associated with CHD)? IDK, but here's something brief: |
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Definition
- usually a lethal autosomal recessive disorder
- often occurs with CHD
- digital defects
- coarse face
- webbed neck
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Term
| CHD can occur in the right or left foramen of Bochdalek or in the foramen of Margagni...where does it occur most commonly? |
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Definition
| LEFT foramen of bochdaleck (80%). The left normally closes after the right. |
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Term
| When the guts sit in the pulmonary cavity (CDH) describe the impact this has on the lungs. |
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Definition
- IPSILATERAL lung HYPOPLASIA
- Decreased number of bronchi
- Decreased number of alveoli
- Decrease in cross sectional area
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Term
| What are some signs that a neonate has CDH when they are born? |
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Definition
- Scaphoid abdomen
- Barrel shaped chest
- BOWEL SOUNDS IN CHEST!
- Respiratory distress
- arterial hypoxemia with cyanosis
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Term
| If your kid has a huge CDH, they might have a HYPOPLASTIC heart. Besides this problem, what else might be going on in their poor little hearts that is the result of their crappy pulmonary system? |
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Definition
- PDA
- PFO (patent foramen ovale)
Recall that the following factors are contributing to extremely HIGH PULMONARY PRESSURES/PULMONARY RESISTANCE:
- bowel content in chest
- loss of cross-sectional bronchial and alveolar area
- hypoxemia
The overall effect is to produce a RIGHT TO LEFT shunt and persistant fetal circulation via a the PDA AND PFO. |
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Term
| What is the preferred mode of intubation and subsequent ventilation in the CDH neonate? Why? |
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Definition
AWAKE INTUBATION: positive pressure masking needs to be avoided b/c it can inflate the gut and further impede lung expansion.
Ventilation should be done with LOW VOLUME and HIGH RATES: the contralateral lung is susceptible to a pneumothorax before, during, and after surgery (lung protective strategy). |
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Term
| If you wanted to insert a preductal A-line, where would it be placed? |
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Definition
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Term
| You MUST avoid conditions that cause pulmonary hypertension in the neonate with CDH. Give me a list of these factors that you can control (hint: most start with "H") |
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Definition
- hypoxemia
- hypothermia
- acidosis (H+ ion)
- hypercarbia
- Pain
- Noxious stimuli such as suctioning
- Nitrous oxide (increases pulmonary pressures and would be bad if you inadvertantly created a pneumo in the good lung.)
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Term
| You know the factors that cause pulmonary hypertension...what are some specific things you can do to make sure to avoid them or to treat them? |
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Definition
- avoid nitrous
- frequent ABGs to assess adequacy of ventilation
- sodium bicarb for acidosis (0.5-1 mEq/kg)
- hyperventilation to keep CO2 around 25-30 torr; pH 7.5
- Maintain their temp
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Term
| (In reference to CDH). If an infant's lungs respond to hyperventilation with a ________then they have a good profile for recovery. If not, they're in trouble. |
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Definition
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Term
| What type of maintenance fluid is used for the neonate with CDH? What's the baseline rate (I'm assuming this is the hourly rate for all neonates?)? What's the calculated value for fluid replacement for evaporative loss (hint: incision is often chest to gut)? |
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Definition
- D5.45 or D5.9Ns at 4ml/kg/hr (preservative free of course)
- 8-10ml/kg/hr
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Term
| Is it a good idea try to inflate the bad lung of the CDH neonate after they push the guts out of the way? |
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Definition
| No. You can pop the good one. The crappy one doesn't readily inflate. LaPlace's law and all that... |
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Term
| You should be monitoring electrolytes in your CDH patient because of all the fluid shifts in this big type of case. Should your blood transfusion trigger be high/middle/low? Why? |
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Definition
| High (meaning you don't want to let them bleed much before you give them blood). The infant is prone to hypoxemia. If they don't have the capacity to carry what oxygen they can get to the blood they will really be screwed. |
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Term
| Why don't they try to cram the guts into the abdominal cavity after they're taken out of the lungs during CDH surgery? |
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Definition
They won't fit.
- cephalad displacement of diaphragm
- reduced FRC
- compression of IVC
They leave the belly open and close gradually. |
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Term
| What condition manifests within the 2nd to 6th week of life as nonbilous, projectile vomiting that occurs about 30 minutes after feedings and occurs more frequently in males? |
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Definition
| Hypertrophic Pyloric Stenosis - hypertrophy of the muscular layer of the pylorus. Can often be palpated in the RUQ. |
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Term
| Chronic vomiting leads to 3 things in the neonate with hypertrophic pyloric stenosis...what are they? |
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Definition
- metabolic alkalosis (kidney dumps bicarb initially)-->don't hyperventilate the neonate!
- hypokalemia
- hypochloremia (sodium is also lost)
Note: the kidney eventually begins to save sodium and the patient will begin to develop a paradoxical aciduria (loss of more hydrogen ions)-->I'm not sure what pump does this, does it exchange sodium for H+ ions?...not sure, sorry! |
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Term
| Clinical Pearl: hypertrophic pyloric stenosis is a _______ emergency, never a _________ emergency! |
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Definition
MEDICAL never a SURGICAL....
the neonate needs to be "tuned-up" prior to surgery (electrolytes corrected, alkalosis corrected, etc.) |
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Term
What do you need to do prior to inducing your neonate with hypertrophic pyloric stenosis?
How will you intubate?
How will you extubate? |
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Definition
Decompress stomach with OG!!! (critical!)
RSIV or awake intubation (pulmonary aspiration is the major concern during induction)
Extubate FULLY awake |
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Term
| You're preparing your neonate for pyloromyotomy. In addition to making sure they are EUVOLEMIC what should your patient's sodium, potassium, chloride, and urine output be before you proceed? |
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Definition
Na>130mEq
K>3mEq
Cl>85
UO>1-2ml/kg/hr
Correcting sever dehydration and electrolyte abnormalities takes 24-48 hours...don't go along with a plan of correcting intraop! |
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Term
What fluids are typically used for pyloromyotomy (etiology: hypertrophic pyloric stenosis)?
Can you use LR? |
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Definition
0.45-0.9NS with D5W
Probably shouldn't use LR (cori cycle turns lactate into bicarbonate and glucose-->your patient is already most likely alkalotic and dumping bicarb) |
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Term
| How is a Nissen different from pyloromyotomy? |
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Definition
Nissen uses stomach to create a tighter lower esophageal sphincter.
Pyloromyotomy cuts the pyloric sphincter because it's too tight and does not allow the stomach to empty. |
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