Myelodysplasia

Question 1

What medical issue must be kept in mind when treating myelodysplasia patients?

Answer 1

IgE mediated anaphylactic latex allergy in 20-70%

Question 2

What level of myelodysplasia is a good predictor for independent ambulation?

Answer 2

L5

Most L3 and above confined to wheelchair

Question 3

What are the types of myelodysplasia?

Answer 3

1) spinal bifida oculta-
defect in vertebral arch with confined cord and meninges
2) meningocele- protruding sac without neural elements
3) myelomeningocele- protruding sac with neural elements
4) rachischisis- neural elements exposed with no covering

Question 4

What lab test is drawn during pregnancy to test for myelodysplasia?

Answer 4

AFP drawn in second trimester; elevated in 75% of those with open spina bifida

Question 5

What is the incidence of scoliosis in myelodysplasia with thoracic level?

Answer 5

100%

Curves that quickly progress must have tethered cord ruled out

Question 6

How does surgical treatment of scoliosis differ in pts with myelodysplasia?

Answer 6

1) fuse anterior as well d/t dysplastic posterior elements
2) fuse to pelvis
3) high infection rate 15-25%
High pseudoarthrosis rate

Question 7

What is the treatment for a dislocated hip in a L1 level pt with spina bifida?

Answer 7

Observation; poor outcomes with open reduction in L3 or above

Question 8

What are risk factors for myelodysplasia?

Answer 8

1) folate deficiency; 70% risk decrease with supplementation
2) maternal hyperthermia
3) maternal diabetes
4) valorous acid