Myelodysplasia

Question 1

What is myelodysplasia?

Answer 1

Defective development of any part of the spinal cord. Posterior arch of the vertebral column doesn’t form as it should during embryogenesis.

Question 2

Aparta

Answer 2

Open/visible

Question 3

Occulta

Answer 3

hidden/skin covered. May have dimple, darkened skin, harry patch - typically does not cause disabilities, may go undiscovered

Question 4

Meningocele

Answer 4

fluid filled sac (no nerve compromise)

Question 5

Myelomeningocele

Answer 5

Nerve involvement. Paralysis depending on level of lesion.

Question 6

When is the spinal cord typically formed?

Answer 6

28 days gestation

Question 7

What is the cause?

Answer 7

Not definitive -
Genetic: assoc. with other genetic abnormalities
Environmental: use of certain medications (valproic acid), excessive alcohol consumption
Nutritional: lack of folic acid, obesity, diabetes

Question 8

Is this progressive in nature?

Answer 8

Yes. due to dysplasia and tethered cord.

Question 9

What is dysplasia in this case?

Answer 9

Abnormal tissue growth that may cause paralysis later in life (or further paralysis)

Question 10

What is tethered cord?

Answer 10

Ischemia and progressive neural dysfunction as a result of improper growth of connective tissue around the cord.

Question 11

Associated orthopedic impairments

Answer 11

Spinal Deformities
Rotational/torsional deformities at the hip/subluxation
Club foot/equinovarus

Question 12

Associated Neuro components

Answer 12

Chiari II Malformation
Hydrocephalus
Tethered Cord
Seizures
Neurogenic B&B
Abnormal muscle tone
UMN/LMN

Question 13

What is Chiari II Malformation/Arnold Chiari?

Answer 13

Caudal displacement on pons, medulla and fourth ventricle through foramen magnum

Question 14

S/S Chiari II

Answer 14

Changes in breathing pattern, weakness in arms, quick downward eye movements, swallowing problems/gagging, tethered cord, hydrocephalus

Question 15

When does Chiari II become symptomatic

Answer 15

infancy/early childhood

Question 16

What is hydrocephalus?

Answer 16

Build up of CSF in ventricle puts pressure on brain = impairments in brain function

Question 17

Whats the relationship between hydrocephalus and arnold chiari?

Answer 17

The opening in the spinal cord results in loss of the fluid surrounding the nervous system. This causes the brain to be positioned further down into the upper spinal column than normal, which is called an Arnold Chiari II malformation. When this happens, the normal flow of fluid out of the brain is obstructed, causing Hydrocephalus. After birth, most children with Hydrocephalus need to have the extra fluid shunted out of the brain into the abdomen via a ventriculoperitoneal shunt.

Question 18

S/S shunt malfunction

Answer 18

headache, vomiting, seizures, bulging fontanelle, lethargy, irritability, confusion, decreased school performance, edema along shunt tract.

Question 19

What percentage develop tethered cord?

Answer 19

20-50% (can cause LE weakness, foot deformities, back pain, scoliosis, etc.)

Question 20

PT examination (infancy)

Answer 20

newborn pre & post op
6, 12, 18, 24 months
Annual 
Growth Spurts
Identify hip dislocation (barlow & ortolani) 
Joint alignment (hip and knee flexion contractures)
Muscle function
Sensation

Question 21

PT examination (toddler/Pre-K)

Answer 21

Differentiate b/t flexible and fixed orthopedic abnormalities, and contractures. 
Functional observation
Sensation to light touch
proprioception
Functional Activities Assessment

Question 22

PT Interventions (infancy)

Answer 22

EDUCATION!

pt positioning, stretching, interaction with environment, adapted equipment

Question 23

PT Interventions (toddler/preschool)

Answer 23

Family Education (stretching, positioning for play, skin integrity)
Gait training the child