Myelodysplasia Flashcards
1
Q
What is spina bifida?
A
- Disorders of spinal bony and neural formation
- “Split spine”
2
Q
What is myelodysplasia?
A
Displacement of some tissue in a sac protruding through posterior opening of spinal vertebra
3
Q
What is the biggest risk factor of spina bifida?
A
Folate acid deficiency
4
Q
Spina bifida occulta characterstics
A
- Minimal skin signs
- Hair tuft
- Vertebral fusion defect
- Normal spinal cord
5
Q
Occult spinal dysraphism signs
A
- Tethered cord
- lipoma
- Diastematomyelia
- Dermal sinus tract
- Dimple
- hemangioma
- Tuft of hair or small lumps
6
Q
What is occult spinal dysraphism associated with?
A
- Syringomyelia and Chiari I malformation
- Blocks ventricular CSF flow, causes fluid backup increasing pressure in brain or spinal cord
- Tethered cord can cause syringomyelia
7
Q
What is tethered cord?
A
- Limited movement of spinal cord within spinal canal, growing spine will cause damage
- Any conus caudal to the mid-body of L2 is considered abnormally low
8
Q
What is Chiari I malformation?
A
- Extension of cerebellar tonsils into foramen magnum
- May not caused symptoms, so its noticed in adulthood or adolescence
- Only one that can be acquired due to thoracic or lumbar CSF leak
9
Q
What is Chiari II malformation?
A
- Extension of both cerebellum and brain stem into foramen magnum
- Usually accompanied by myelomeningocele
- Arnold-Chiari is specific to this type
10
Q
What is Chiari III malformation?
A
- Cerebellum and brain stem herniate through foramen magnum and into SC
- Part of 4th ventricle may protrude as well
- Considered most severe
11
Q
What is Chiari IV malformation?
A
Incomplete or underdeveloped cerebellum
12
Q
Treatment for Chiari malformation
A
- Decompression by removal of small portion of posterior skull
- Skull, C1, C2 stabilization
- Shunt syrinx or for hydrocephalus
- Shrinkage of tonsils within electrocautery - allows better CSF flow from 4th ventricle
13
Q
What is a meningocele?
A
- Meninges outside, but SC inside vertebral defect
- Little or no SC damage
- Possible motor or sensory deterioration later in life
14
Q
What is a myelomeningocele?
A
- SC and meninges outside vertebral defect
- Nerve damage causes motor and sensory impairment
15
Q
What is myeloschisis?
A
- Complete exposure of SC
- 4th week gestation
- Caudal neuropore does not close
- Most severe form, requires surgical closure
16
Q
Prenatal diagnosis of spina bifida
A
- Maternal Alpha-fetoprotein levels - detect open defects
- Ultrasound
- Amniotic fluid analysis
- Acetylcholinesterase levels - detect closed defets
17
Q
What is “banana sign” on ultrasound?
A
Chiari II displacing cerebellum
18
Q
What is “lemon sign”?
A
Frontal bone scalloping
19
Q
What is present in all children with spina bifida?
A
- Chiari II malformation, primary cause of hydrocephalus
- Symptomatic in 33% with spina bifida
20
Q
Why do babies with open spinal defects need to be born via C-section?
A
Decreased trauma and lower likelihood of infection
21
Q
What is an intervention for spina bifida before birth?
A
- Intrauterine surgery to close open defect
- Minimizes damage to SC
- Decreased need for shunting of CSF
22
Q
What is the primary cause of cognitive deficit in children with spina bifida?
A
- Hydrocephalus
- Requires ventriculoperitoneal shunt
23
Q
What are signs of shunt malfunction?
A
- Increased head circumference
- Bulging fontanel
- Unusual irritability
- Nausea and vomiting
- Weak cry
- Stridor
- Sleepiness
- Fever unrelated to illness
- Medical emergency
24
Q
Symptoms of tethered cord
A
- Changes in bowel/bladder function
- Back/leg pain with activity
- Weakness
- Scoliosis
- Gait deterioration
- LE spasticity
- Foot deformity
25
Symptoms of syringomyelia
* UE spasticity
* Weakness
* Parasthesia
26
Cognitive skills in children with spina bifida
* Most have normal IQ
* Higher lesion and hydrocephalus may result in lower IQ
* Verbal > nonverbal skills
* Converse like adults but don't understand what they are saying
27
MSK anomalies with spinia bifida
* Club foot
* Congenital hip dislocation
* Muscle imbalance
* Joint contracture
* Pathological fractures
* Postural changes - crouched gait
28
What corrects clubfoot?
* Ponseti casting
* Denis Browne splint - high top open-toed shoes attached at ends of bar
29
GI concerns for spina bifida
* Sparing of S2-3 has potential for bowel and bladder control
* Bowel training - cleanliness training with suppositories or enemas
30
Integumentary concerns with spina bifida
* Ischemia - excessive pressure
* cast or orthosis
* Urine/stool
* Friction/shear
* Burns
31
Urological concerns for spina bifida
* Timed toileting
* Clean intermittent catheter
* Vesicostomy - bladder stoma midway between umbilicus and pubic bone
32
What allergy do many children with spina bifida have?
Latex allergy
33
What should PT intervention focus on for spina bifida?
* ROM and strength
* Developmental skills
* Orthoses and assistive devices
* Mobility
* Monitor signs of tethered cord, shunt malfunction, skin breakdown
34
How is level of spina bifida determined?
* Established by International Myelodysplasia Study Group
* Not always same as adult with SCI
* Evaluate lower than apparent lowest level
35
How is sensation different with spina bifida?
* May not correlate with motor level
* May skip within a determatome
* Vibration may be present below light touch and pinprick
36
Strongest muscle groups for spina bifida
* Quads and iliopsoas
* Glute med and max
* Ant tib
* Medial hamstrings
* Weakness most frequent in gastrco-soleus
37
Expected functional level for T12 and above spina bifida
* Walk short distances with HKAFOs or RGOs
* Walk longer with walkers or crutches
* Mostly use W/C
38
Expected functional level for L1-3 spina bifida
* KAFOs and crutches
* W/C for longer distances
39
Expected functional level for L4 spina bifida
* AFOs or KAFOs
* Crutches or canes
* May use W/C for distances or when older
40
Expected functional level for L5-S spina bifida
* AFOs or less
* May need crutches or canes
