Exam 1: Pediatric Anesthesia pt 3 (pg 41-59)

Question 1

Neural tube defects is a group of birth defects that describe a common failure of the neural tube to develop properly during the ____ stage

Answer 1

embryonic

pg 41

Question 2

Neural tube defects can be genetic or enviornmental, with 10% being cuased by chromosomal abnormalaties. What are some non-genetic environmental factors that can cause neural tube defects?

Answer 2

• Folate deficiency (folic acid decreases the incidence of this)
• Maternal anti-epileptic drugs (valproic acid, phenytoin, and carbamazepine)
• Retinoins (Accutane)
• Maternal diabetes

pg 41

Question 3

____ _____ is characterized by abnormal or incomplete formation of midline structures over the back.

Answer 3

Spina Bifida

pg 41

Question 4

Spina Bifida:
* _____, ____, and _____ elements can be involved individually or in combination with each other
* Can be associated with ____ anomalies
* Not always detected at birth, some forms are identified later in childhood

Answer 4

• Skin, bone and neural elements can be involved individually or in combination with each other
• Can be associated with brain anomalies
• Not always detected at birth, some forms are identified later in childhood

pg 41

Question 5

What are characteristics of spina bifida occulta?

Answer 5

• Overlying tissue appears normal
• Possible hairy patch
• Sacral dimple
• Lipoma
• spinal cord can end lower than usual

pg 42

Question 6

What spina bifida is most common? occulta or aperta?

Answer 6

aperta
occurs in 1/1000 live births

pg 43

Question 7

What are characteristics of spina bifida aperta?

Answer 7

• Obvious lesion on back

Either meningocele or myelomeningocele.

pg 43

Question 8

Differentiate a meningocele and a myelomeningocele.

Answer 8

• Meningocele contains CSF without spinal tissue
• Myelomeningocele contains CSF and spinal nerves

pg 43

Question 9

symptoms of spinal bifida

Answer 9

pg 43

Question 10

Flip for picture of Spina Bifida sub-types.

Answer 10

pg 43

Question 11

What is happening here?

Answer 11

• a hair patch or dermal sinus which may inidicate o Absence of herniation of neural tissue or coverings

pg 44

Question 12

What is this?

Answer 12

• Sacral dimple which is likely to be assd with spina bifida occulta
• Usually >5mm diameter and 2.5cm above the anus
• Closer to the anus are less at risk sacral dimple. Higher up is more at risk for spina bifida.

pg 45

Question 13

What is this?

Answer 13

failure of closure of vertebral arches despite outside appearance being normal.

pg 45

Question 14

Hydrocephalus is more common with which category of spina bifida?

Answer 14

Spina Bifida Aperta

pg 45

Question 15

What type of malformation is common in spinal bifida aperta?

Answer 15

Type II Chiari (Arnold Chiari malformation)

pg 45

Question 16

Do nerve roots below the level of the lesion function for myelomeningocele?

Answer 16

No

pg 45

Question 17

when should a myelomenigocele be repaired?

Answer 17

must be repaired w/n the first few day or hours of birth to prevent infection or further trauma

pg 47

Question 18

Spina Bifida Aperta:
* ____ leaks or ____ ruptures can occur

Answer 18

CSF leaks or dural ruptures can occur

pg 47

Question 19

What additional surgery is necessary for spina bifida w/ concomitant hydrocephalus.

Answer 19

VP shunt

pg 47

Question 20

What are some long term complications for spina bifida aperta?

Answer 20

• Paraparesis
• Neurogenic bowel or bladder
• Renal insufficiency
• Trophic limb changes
• Joint contractures
• Scoliosis requiring surgical intervention

(The higher up the SC, the more involvement)

pg 47

Question 21

What is each one depicting?

Answer 21

pg 47

Question 22

Spina bifida anesthetic considerations:
* Assess pre-op ____ and ____ defects
* Positioning for intubation and surgical procedure (may need support with foam donuts and towels to optimize)
* Preserve function and further injury
* May need to avoid ____ if renal dysfunction
* No need to avoid ____
* Can be considerable blood loss
* ____ measures in place

Answer 22

Spina bifida anesthetic considerations:
* Assess pre-op motor and sensory defects
* Positioning for intubation and surgical procedure (may need support with foam donuts and towels to optimize)
* Preserve function and further injury
* May need to avoid NSAIDs if renal dysfunction
* No need to avoid Succs
* Can be considerable blood loss
* Warming measures in place

pg 48

* An example of intubation of an infant with spina bifida aperta-myelomeningocele

Question 23

Why is increased ICP a concern for pts with spina bifiday?

Answer 23

risk for N/V so RSI

pg 48

Question 24

• Spina bifida is repaired in a ____ position with chest and abdominal rolls.
• How is the head placed?
• Make sure to do what after positioning?

Answer 24

• prone
• Head can be turned to the side or placed in prone pillow
• Secure your airway
• Double check your IV’s and ETT after positioning

pg 49

Question 25

What is a herniation of neural tissue and meninges out of the skull through deficient skin and bone?

Answer 25

encephalocele | pg 50

Question 26

Encephalocele location can occur from occiput to frontal area. What is associated with anterior vs posterior encephaloceles?

Answer 26

* Anterior: can be associated with brain, orbits, and or pituitary gland * Posterior: associated with cerebral or cerebellar tissue | pg 50

Question 27

what kind of encephalocele is hard to detect?

Answer 27

ones that exist intranasally | pg 50

Question 28

Encephalocels are associated with high mortality, and surviors have what?

Answer 28

severe neurological developmental delay and hyrocephalus | pg 50

Question 29

what is the treatment for encephalocele?

Answer 29

Surgical repair is only treatment and can be done with a shunt if hydrocephalus is present | pg 50

Question 30

What disorder is characterized by cerebellar herniation results when the cerebellar tonsils herniate through the foramen magnum from the posterior fossa to the cervical space?

Answer 30

Chiari Malformations | pg 55

Question 31

What allowschiari malformations to occur?

Answer 31

bony abnormality in the posterior fossa and upper cervical spine | pg 55

Question 32

Chiarir malformations are often associated with ____. And can lead to what?

Answer 32

* myelomeningocele * leads to an obstruction of CSF flow and eeturally hyrocephalus results | pg 55

Question 33

what is hydrocephalus?

Answer 33

mismatch of CSF production and absorption that leads to an increased intracranial volume of CSF | pg 55

Question 34

hydrocephalus usually results from what?

Answer 34

some type of obstruction or imbalance of absorption and production | pg 55

Question 35

what are the classifications of hydrocephalus

Answer 35

* obstructive / noncommunicating * nonobstructive/ communicating * *depending on its ability to cover the spinal column* | pg 55

Question 36

what can happen if hydrocelphauls is left untreated?

Answer 36

intracranial hypertension | pg 55

Question 37

Chronic increasing of ____ is characteristic of chiari malformations.

Answer 37

ICP - Headache - Irritability - N/V | pg 56

Question 38

What are the four types of chiari malformations?

Answer 38

| pg 56

Question 39

What are the S/S of arnold chiari malformation?

Answer 39

* Vocal cord paralysis with stridor * Respiratory distress * Apnea * Abnormal swallowing * Aspiration * Opisthotonos: backward arching of the neck and spine * Cranial nerve deficits | pg 57

Question 40

How is chiari malformation repaired?

Answer 40

* Usually, a decompressive suboccipital craniectomy with cervical laminectomies * Repaired in posterior fossa craniotomy positioning * Head placed in pins or placed on a padded horseshoe shaped frame | pg 58

Question 41

Chiari malformation anesthetic considerations

Answer 41

* 2 Lg IVs and A-line * Nasal intubation may be preferred for prone positioning * Arfin to bilateral nares, nasal trumpet dilation, Magill’s for ETT advancement with DL * OGT placed and left to gravity during case * Eyes lubricated and clear Tegaderms placed over eyes. * Chest rolls and pelvic rolls to free abdomen compression or pressure on femoral nerve or genitalia * Secure your ETT well!!! * Avoid oral airway, sometimes a “soft” bite block is placed b/w lateral incisors especially if cortical motor potentials are used * Avoid excessive intra-op fluids * Maintain normal temp, the head is large BSA in infants. | pg 59

Question 42

if increased ICP is a concern in chiara malformation, what should you do?

Answer 42

* avoid ketamine (increases CMRO2) * Use controlled hyperventilation and narcotics to blunt response to laryngoscopy | pg 59