74711_7b.spina bifida.doc

8/11/2019 74711_7b.Spina bifida.doc

1/8

Spina Bifida

Definition:

Spina bifida or myelodysplasia is a congenital abnormality, in which

there is a developmental defect in the spinal column with incomplete closure

of the vertebral canal due to a failure of fusion of the vertebral arches. The

primary developmental defect is thought to arise in the first few weeks of

gestation due to failure of the neural tube to close. Spina bifida is the

commonest one of the major congenital abnormalities, which affects the

neuro-musculoskeletal and genitourinary systems.

Etiology

Neural Tube defects may result from a combination of environmental

and genetic causes such as teratogens (any factor disturbing fetal growth

and nutritional deficiencies, notably folic acid deficiency.

Classification:

* Spina bifida occulta:

!n this defect, the vertebral arches are unfused" however there is no

herniation or displacement of neural tissues but there are skin changes over

the defect. Neurological signs may or may not be present. This form is

usually asymptomatic and most commonly involves #$and S%.

* Spina bifida cystica (meningocele):

!n this defect, the vertebral arches are unfused" however, there is

herniation of the meninges, producing a protrusion containing cerebrospinal

fluid (&S'. The sac is covered by skin or membrane. art of the cord or

nerve roots may be present in the sac" if so, they conduct impulses normally

%


2/8

* Myelomeningocele:

!t involves the spinal cord, nerve roots and meninges" all of which

may protrude through the vertebral defect. The defect can occur at any level

but most commonly involve the lumbosacral area.

Fig. (1):Neural Tube Defects

)


3/8

Classification of myelomeningocele according to level of lesion:

* Thoracic level:

- Signs of upper motor neuron lesion (*+N# can be seen if there is

damage to the cord.

- No function in the voluntary muscles crossing the hip joint.

- ip joints are usually not dislocated.

- atients are ambulatory only with high standing brace ('/.

- Scoliosis is the major problem.

* igh lumbar level:

- #% - #)0 ip fle1ion and adduction is present.

- 2islocation of the hip joint is the commonest orthopedic problem.

Treatment of hip dislocation depends on ambulation potential of the patient.

- &hild usually uses knee-ankle foot orthoses ('/s.

* !o"#lumbar level:

- #3- #$0 ips are not significantly affected.

- &alcaneus deformity of the ankle and foot ulceration.

- atient is usually mobile with ankle foot orthoses ('/s with walker or

sticks.

* Sacral level:

- atients are ambulators.

- 'oot and toes deformities.

- atients may re4uire shoe supports during walking.

5


4/8

Diagnosis and detection:

- *ltrasonography.

- +6!.

Prognosis:

Spina bifida is a static non-progressive defect, with worsening from

secondary problems. So, severity of symptoms depends on the level of

lesion, the degree and e1tent of neural involvement and associated problems.

Clinical features:

The commonest site of myelomeningocele is the lumbosacral region.

The clinical features evident in the infant and young child with a

lumbosacral myelomeningocele are0

- 'laccid paralysis (muscle weakness and wasting.

- 2ecreased or absent tendon refle1.

- 2ecreased or absent e1troceptive and7or proprioceptive sensation.

- 6ectal and bladder incontinence.

- aralytic and congenital deformities.

Secondary complications which mainly develop are:

- 6etarded physical development.

- Severe vasomotor changes.

- 8urn and pressure ulceration of the skin.

- /steoporosis.

- Soft tissues contractures.

- 8ony deformity.

- /besity.

3


5/8

mpairments associated with spina bifida:

# ydrocephalus:

Fig. ($):!ydrocephalus

# %rnold &hiari malformation:

There is cerebellar hypoplasia, with caudal displacement of the

hindbrain through the foramen magnum. !t is usually associated with

hydrocephalus. The manifestations usually consist of0

- &ongenital hip dysplasia.

- &ongenital feet deformity.

- &ognitive and perceptual problems.

- 9isual perceptual deficits.

$


6/8

Physical e"amination:

* 'y sight:

- Tuft of hair.

- !ncrease head si:e.

- 2eformities of the lower limbs.

- Skin ulceration.

* 'y palpation:

- +uscle tone.

- 8ony defect.

- Sensory test0 ccording to the level of lesion.

* 'y measurements:

- +uscle test.

- 'unctional test.

- Tape measurements0 ead, long and round measurement of the lower

limbs.

* ross motor assessment:

*sing 2enver;s developmental screening test (22ST.

Physical treatment:

Main goals:

- 'acilitation of weak muscles of the lower limbs and trunk.

-


7/8

#ethods of treatment:

* Facilitatory stimuli: 'aradic stimulation, brief ice application, e1troceptive

stimuli, 4uick stretch, brushing and tapping are indicated.

* Strengthening eercises: 'or children under 5 years old, functional

strengthening e1ercises are utili:ed. 'or older children, isolated

strengthening e1ercises using universal e1ercise unit, weights and pulleys

are used.

> ctive and passive range of motion.

> ydrotherapy.

> Stretching e1ercises.

> 6outine daily living activities.

> *sing splints, orthoses and assistive devices0

- #ong leg brace with pelvic band.

- nee ankle foot orthoses ('/s.

- nkle foot orthoses ('/s.

- +edical shoes.

- Stand frames.

- ?alkers.

- Sticks with wide base.

Surgical management:

Surgical closure of the back lesion is needed )3-3@ hours after birth,

with shunt insertion within = months in case of hydrocephalus.

A


8/8

Fig. ():E"amples of an$le foot orthoses

@

74711_7b.spina bifida.doc

Documents