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Clinical Med (Year 2) > Myelodysplasia (Spina Bifida) - (Exam 2) > Flashcards

Myelodysplasia (Spina Bifida) - (Exam 2) Flashcards

1
Q

What is the general definition of Myelodysplasia?

A
  • Defective development of any part (especially the lower segments) of the spinal cord
  • Congenital neural tube defect (NTD)
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2
Q

What is the pathophysiology of Myelodysplasia?

A
  • Embryo (20 days after conception) neural groove –> neural crest –> closure of neural tube
  • Day 23 completely closed except for hole at top –> brain & hole at bottom –> spinal cord
  • Also errors in development of vertebral architecture
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3
Q

The degree of dysfunction of Myelodyplasia is related to what?

A

Anatomic level of the defect

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4
Q

Patients with Myelodysplasia usually present with loss of (BLANK) below the level of the lesion

A

Neurologic function (sensory & motor)

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5
Q

What is the etiology of Myelodysplasia?

A

Possible environmental & genetic factors:
- Folic acid deficiency
- Lower SES
- Teratogenic agents
- Hyperthermia during early pregnancy
- Vitamin A deficiency
- Rh factor
- Alcohol ingestion
- Genetic Link? (parent w/ one child with myelodysplasia have 50x higher chance of having a sibling)

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6
Q

How is Myelodysplasia detected pre and postnatally?

A

Pre-natal:
- Ultrasonic scanning is able to detect NTD prenatally
- Serum alpha- fetoprotein (AFP) testing

Post-natal:
- Observation (except occulta)

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7
Q

Myelodysplasia - Occulta is a result from what?

A

Failure of one or more vertebral arches to meet and fuse in 3rd month of development

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8
Q

In Myelodysplasia - Occulta (BLANK) & (BLANK) are unharmed and remain in vertebral canal
- (BLANK) defect is covered by skin

A

In Myelodysplasia - Occulta Spinal Cord & meninges are unharmed and remain in vertebral canal
- Bony defect is covered by skin

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9
Q

Where is Occulta most common at?

A

in Lumbosacral area

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10
Q

Occulta has no disturbance in (BLANK) or (BLANK) function

A

Occulta has no disturbance in neurological or musculoskeletal function

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11
Q

What physical features may be present with Occulta?

A
  • Depression or dimple
  • Cafe au lait spot
  • Soft fatty deposit
  • Tuft of hair midline over area
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12
Q

How does Aperta-cystica occur?

A

Neural tube & vertebral arches fail to close appropriately

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13
Q

In Aperta- cystica there is a (BLANK) protrusion of material through (BLANK)

A

In Aperta- cystica there is a cystic protrusion of material through defective arches

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14
Q

What are the two types of aperta-cystica?

A
  • Meningocele
  • Myelomeningocele
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15
Q

Describe Meningocele

A
  • Protrusion of meninges and CSF into cystic sac
  • Spinal cord remains within vertebral column
  • May exhibit some abnormalities but less common
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16
Q

What is Myelomenigocele?

A

Protrusion of both spinal cord & meninges into cystic sac

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17
Q

What is a closed Myelomenigocele?

A

Covered with a combination of skin & membranes

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18
Q

Where are meningoceles and closed myelomeningoceles most common?

A

At thoracic & lumbosacral areas

19
Q

What is an open myelomenigocele?

A

Nerve roots & spinal cord may be exposed with dura & skin at edge of lesion

20
Q

Where do open Myelomenigocele occur?

A

2/3 of open type occur at T-L junction

21
Q

What is Myelodyplasia: Lipoma?

A

Fatty tumor on spinal cord

22
Q

What is Myelodyplasia: Myelocytocele?

A

Cystic like tumor of spinal cord

23
Q

What is Myelodyplasia: Anencephaly?

A
  • Failure of closure of cranial end of neural tube
  • Some brain tissue may be evident but forebrain usually absent
  • Lack of sustained life
24
Q

What is the clinical picture of Occulta?

A
  • Usually does not cause neurologic dysfunction
  • Occasional disturbances in bowel & bladder function or foot weakness (Most common in LS area)
25
Q

What is the clinical picture of Myelomeningocele?

A
  • Motor dysfunction
  • Sensory Loss
  • Skeletal deformity
  • Hydrocephalus
  • Tethered cord
  • Bowel & Bladder incontinence
  • Latex allergy
  • ID (low percentage)
  • LD (high percentage)
26
Q

What are some of the skeletal deformities in a patient with Myelomenigocele?

A
  • Scoliosis
  • Hip subluxation/dislocation
  • Bone & joint deformities
  • Muscle contractures
27
Q

What is Myelomeningocele motor function prognosis?

A
  • Depends on level of lesion (complete paralysis of LE or m weakness)
  • Patient presents as if he/she has a diagnosis and presentation consistent with patient who has a SCI
28
Q

What is medical management of Myelodysplasia?

A
  • Team of physicians & health care providers
  • Surgical repair (pre or post natal)
29
Q

In regards to medical management of Myelodyplasia how is post natal surgical repair performed?

A
  • Closure usually within 72 hours
  • Place neural tissue into vertebral canal
  • Cover/repair vertebral defect
  • Achieve a flat, watertight closure of thecal sac
30
Q

What are the surgical repair outcomes of Myelodysplasia?

A
  • 10% infants recover after surgery & are d/c without further complications
  • 90% begin to develop hydrocephalus from next few days to weeks
  • Can develop tethered cord down the road as a result could experience similar signs & sxm as Arnold Chiari (also require surgery)
31
Q

What is hydrocephalus?

A

Abnormal accumulation of CSF in cranial vault

32
Q

What can Hydrocephalus be caused by?

A
  • Overproduction of CSF
  • Failure of absorption of CSF
  • Obstruction in normal flow of CSF through brain & spinal cord
33
Q

In Hydrocephalus, accumulation of CSF creates what? Which can lead to what?

A

Increased ICP
Lead to: Cerebral damage & cellular death

34
Q

What are some signs and symptoms of hydrocephalus?

A
  • Full, bulging, tense soft spot (fontanel) on top of the child’s head
  • Large prominent veins on scalp
  • Setting sun sign
  • Behavior changes
  • High pitched cry
  • Seizures
  • Vomiting or change in appetite
35
Q

What is setting sun sign?

A
  • Child appears to only look down
  • The whites of the eyes are obvious above the colored portion (iris) of the eyes
36
Q

What is the management of Hydrocephalus?

A

Ventriculoperitoneal (most common) or Ventriculo-atrial shunt

37
Q

Describe the shunt for management of hydrocephalus?

A

Thin tube with small pump attached, which diverts CSF from lateral ventricles to a location where the fluid can be managed

38
Q

What are some signs and symptoms of shunt malfunction?

A
  • Firm fontanels
  • Listlessness, drowsiness, irritability
  • Vomiting, change in appetite
  • Increasing head circumference
  • Swelling along the trunk
  • Disturbance of bowel & bladder problems
  • Seizures
39
Q

What are some signs & symptoms of shunt malfunction in older child/adult?

A
  • headaches
  • blurring vision
  • seizures
  • decrease in school performance
  • decrease in sensory & motor functions
40
Q

Describe Arnold-Chiari Malformation

A
  • Brainstem is displaced through the foramen magnum
  • Defect in formation of the brainstem
  • May result from myelomeningocele
  • Frequently contributes to development of hydrocephalus
41
Q

What are some signs of Arnold Chiari Malformation?

A
  • Brainstem dysfunction & cranial nerve involvement
  • Feeding difficulties
  • Choking
  • Pooling of secretions
  • Repeated aspirations, apnea, neck pain, vocal cord paralysis
  • Stridor breathing (noisy breathing)
  • UE weakness, spasticity, weakness, incoordination
42
Q

What are signs and symptoms of Tethered Cord?

A
  • Scoliosis
  • Increased spasticity
  • Increase asymmetrical postures or movements
  • Altered gait pattern
  • Decreased UE coordination
  • Changes in muscle strength (at or below lesion)
  • Back pain
  • Incontinence
43
Q

What is the intervention for Tethered Cord?

A

Surgical intervention

Clinical Med (Year 2) (17 decks)

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