Myelodysplasia (Spina Bifida) - (Exam 2) Flashcards
What is the general definition of Myelodysplasia?
- Defective development of any part (especially the lower segments) of the spinal cord
- Congenital neural tube defect (NTD)
What is the pathophysiology of Myelodysplasia?
- 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
The degree of dysfunction of Myelodyplasia is related to what?
Anatomic level of the defect
Patients with Myelodysplasia usually present with loss of (BLANK) below the level of the lesion
Neurologic function (sensory & motor)
What is the etiology of Myelodysplasia?
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)
How is Myelodysplasia detected pre and postnatally?
Pre-natal:
- Ultrasonic scanning is able to detect NTD prenatally
- Serum alpha- fetoprotein (AFP) testing
Post-natal:
- Observation (except occulta)
Myelodysplasia - Occulta is a result from what?
Failure of one or more vertebral arches to meet and fuse in 3rd month of development
In Myelodysplasia - Occulta (BLANK) & (BLANK) are unharmed and remain in vertebral canal
- (BLANK) defect is covered by skin
In Myelodysplasia - Occulta Spinal Cord & meninges are unharmed and remain in vertebral canal
- Bony defect is covered by skin
Where is Occulta most common at?
in Lumbosacral area
Occulta has no disturbance in (BLANK) or (BLANK) function
Occulta has no disturbance in neurological or musculoskeletal function
What physical features may be present with Occulta?
- Depression or dimple
- Cafe au lait spot
- Soft fatty deposit
- Tuft of hair midline over area
How does Aperta-cystica occur?
Neural tube & vertebral arches fail to close appropriately
In Aperta- cystica there is a (BLANK) protrusion of material through (BLANK)
In Aperta- cystica there is a cystic protrusion of material through defective arches
What are the two types of aperta-cystica?
- Meningocele
- Myelomeningocele
Describe Meningocele
- Protrusion of meninges and CSF into cystic sac
- Spinal cord remains within vertebral column
- May exhibit some abnormalities but less common
What is Myelomenigocele?
Protrusion of both spinal cord & meninges into cystic sac
What is a closed Myelomenigocele?
Covered with a combination of skin & membranes
Where are meningoceles and closed myelomeningoceles most common?
At thoracic & lumbosacral areas
What is an open myelomenigocele?
Nerve roots & spinal cord may be exposed with dura & skin at edge of lesion
Where do open Myelomenigocele occur?
2/3 of open type occur at T-L junction
What is Myelodyplasia: Lipoma?
Fatty tumor on spinal cord
What is Myelodyplasia: Myelocytocele?
Cystic like tumor of spinal cord
What is Myelodyplasia: Anencephaly?
- Failure of closure of cranial end of neural tube
- Some brain tissue may be evident but forebrain usually absent
- Lack of sustained life
What is the clinical picture of Occulta?
- Usually does not cause neurologic dysfunction
- Occasional disturbances in bowel & bladder function or foot weakness (Most common in LS area)
What is the clinical picture of Myelomeningocele?
- Motor dysfunction
- Sensory Loss
- Skeletal deformity
- Hydrocephalus
- Tethered cord
- Bowel & Bladder incontinence
- Latex allergy
- ID (low percentage)
- LD (high percentage)
What are some of the skeletal deformities in a patient with Myelomenigocele?
- Scoliosis
- Hip subluxation/dislocation
- Bone & joint deformities
- Muscle contractures
What is Myelomeningocele motor function prognosis?
- 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
What is medical management of Myelodysplasia?
- Team of physicians & health care providers
- Surgical repair (pre or post natal)
In regards to medical management of Myelodyplasia how is post natal surgical repair performed?
- Closure usually within 72 hours
- Place neural tissue into vertebral canal
- Cover/repair vertebral defect
- Achieve a flat, watertight closure of thecal sac
What are the surgical repair outcomes of Myelodysplasia?
- 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)
What is hydrocephalus?
Abnormal accumulation of CSF in cranial vault
What can Hydrocephalus be caused by?
- Overproduction of CSF
- Failure of absorption of CSF
- Obstruction in normal flow of CSF through brain & spinal cord
In Hydrocephalus, accumulation of CSF creates what? Which can lead to what?
Increased ICP
Lead to: Cerebral damage & cellular death
What are some signs and symptoms of hydrocephalus?
- 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
What is setting sun sign?
- Child appears to only look down
- The whites of the eyes are obvious above the colored portion (iris) of the eyes
What is the management of Hydrocephalus?
Ventriculoperitoneal (most common) or Ventriculo-atrial shunt
Describe the shunt for management of hydrocephalus?
Thin tube with small pump attached, which diverts CSF from lateral ventricles to a location where the fluid can be managed
What are some signs and symptoms of shunt malfunction?
- Firm fontanels
- Listlessness, drowsiness, irritability
- Vomiting, change in appetite
- Increasing head circumference
- Swelling along the trunk
- Disturbance of bowel & bladder problems
- Seizures
What are some signs & symptoms of shunt malfunction in older child/adult?
- headaches
- blurring vision
- seizures
- decrease in school performance
- decrease in sensory & motor functions
Describe Arnold-Chiari Malformation
- Brainstem is displaced through the foramen magnum
- Defect in formation of the brainstem
- May result from myelomeningocele
- Frequently contributes to development of hydrocephalus
What are some signs of Arnold Chiari Malformation?
- 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
What are signs and symptoms of Tethered Cord?
- 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
What is the intervention for Tethered Cord?
Surgical intervention
