Spina Bifida (Myelomeningocele) Flashcards

1
Q

Description

A

Failure of fusion of the neural fold

During neurulation phase of embryonic development

Embryologic defect of bone, meninges, and spinal cord

Spectrum from anencepahly to spina bifida occulta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Etiology

A

Multifactorial

Folate deficiency?

Environmental: socioeconomic, maternal age, hyperthermia, teratogens, seasonal

Familial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Incidence

A

0.6/1000 in US

Prenatal diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

High incidence of Arnold Chiari II

A

Consists of downward displacement of the cerebellar tonsils through the foramen magnum (opening at the base of the skull)

As a result of obstruction, non-communicating hydrocephalus can result

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Hydrocephalus

A

Abnormal accumulation of CSF in the ventricles of the brain

Causes increased intracranial pressure

In non communicating, due to obstruction of CSF outflow

Can cause headaches, dizziness, impaired coordination, and in severe cases, paralysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Shunt hydrocephalus

A

in 90%

50% obstruct and require revision in 1st year of life

Risk of shunt infection 12%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Other Symptoms

A

Neurogenic bladder and bowel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Site of Defect, Percentages

A

Cervical level 1%

Upper Thoracic 1%

Lower Thoracic and Upper Lumbar 6%

Mid Lumbar 27%

Lumbosacral 42%

Sacral 21%

Entire lumbosacral 2%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Neurologic Impairment, Percentages

A

Cervical and upper thoracic 1%

Lower thoracic 27%

Intact hip flexion but impaired quadriceps 23%

Lower lumbar and quadriceps sparing 45%

Lower sacral, leg strength, B&B abnormal 4%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Neurological impairment

A

Typically flaccid paralysis

May have

Spasticity

Mixed paraplegia and spasticity

Asymmetry

Intact regions of voluntary control below regions of paralysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Hydromyelia

A

50-80%

abnormal widening of the central canal of the spinal cord

Creates cavity in which cerebrospinal fluid can accumulate

Build up of CSF can put pressure on spinal cord

Damage nerve cells

Weakness of upper limbs, spasticity, ascending motor changes

Scoliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Tethered cord

A

11-15%

Traction of conus medullaris and cauda equina after repair

Progressive neurologic deficits

Impair strength, spasticity, back pain, B&B changes, scoliosis

May require surgical release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Musculoskeletal deformity of the hip

A

Occurs in majority

Hip Extensors and Abductors most affected

Muscle imbalance leads to hip flexion contracture

50% subluxed or dislocated hips

May require surgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Musculoskeletal deformity of the knee

A

Flexion contracture most common

May be due to joint stiffness, spasticity in the knee flexors

Or Progressive crouched posture with weak quadriceps

More common L3 or higher

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Musculoskeletal deformity of the foot

A

Deformities occur in 85%

Abnormal bony cartilaginous growth

Lack of sensation and vasomotor instability
- skin injury and poor healing

Equinovarus

Pure Equinus

Pes Cavus

Cock-up toe deformity at great toe

May require surgery as well as bracing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Musculoskeletal deformity of the spine

A

Scoliosis with lordosis

Kyphosis

Rigid congenital malformation

17
Q

Prevalence of scoliosis in spina bifida

A

100% with thoracic lesions develop scoliosis of at least 45 degrees

L4 lesions, 60%

18
Q

Scoliosis in spina bifida

A

high paraplegia, develops early, C-shaped, always progresses

with hydromyelia, usually S-shaped

kyphosis almost always progressive

may have rigid apex

treat to decrease pressure on abdomen, diaphragm and skin

treat before 180

19
Q

Other Complications

A

Fractures

Pressure Sores

Obesity

Latex Allergy