spina bifida

defective embryonic neural tube closure during the 1st trimester of pregnancy results in various malformations of the spine.

generally the defect occurs in the lumbosacral area but maybe found in the sacral, thorcic, and cervical areas

spina bifida occulta

spina bifida w/meningocele

spina bifida w/ myelomeningocele

most common/least severe spinal cord defect

characterized by incomplete closure of one or more vertebrae w/o protrusion of the spinal cord or meninges

incomplete closure of one or more vertebrae causes protrusion of the meninges of CSF in an external sac or cystic lesion

also=meningomyelocele

incomplete closure of the vertebrae causes protrusion of the meninges, CSF, and a portion of the spinal cord or nerve roots in an external sac or cystic lesion.

varies w/ degree of accompanying neurologic deficit

myelomeninocele-least favorable prognosis

large open lesion, neurogenic bladders, increased infection, renal failure

prognosis better

many people may lead normal life w/ no complication

normally-20 days after conception the embroyo develops a nerual groove in the dorsal ectoderm

the groove deepens, edges fuse & become the neural tube

by day 23, the tube is completely closed except for an opening at each end

if the posterior portion of the neural tube fails to close by the 4th week of gestation, or if it closes but then splits open from a cause such as an abnormal increase in CSF later in the first trimester

a spinal defect results

• depression or dimple
• tufts of hair
• soft fatty deposits
• port wine nevi

any combination of these abnormalities on the skin cover the defect

all signs may be absent

usually does not cause neurological dysfunction

occasionally associated with foot weakness and bowel and bladder disturbances

structure protrudes over spine

rarely caused neurological deficts

sac contains CSF and meninges

saclike structure over spine

neurological deficits related to the level of the defect

permanent neurological dysfunction (flaccid/spastic paralysis, bowel/bladder incontinence, etc)

tropic skin disturbances (ulcerations, cyanosis)

mental retardation

clubfoot

knee contractures

hydrocephalus (in about 90%)

often overlooked

x-rays can show the bone defect

pin prick examination of legs and trunk (sensory evaluation)

need to identify sensory and motor levels

skull x-rays, head measurements, CAT scan (hydrocephalus)

amniocentesis

can only detect open defects (myelomeningocele and meningocele)

alpha-fetoprotein test-done at 14 weeks gestation-may indicate defects in the spinal cord (neural tube oopening)

usually requires no treatment

if neuromuscular problems occur with growth, surgery may be required

surgical closure of the protruding sac

continual assessment of growth and development

surgical repair of the sac-cover the flap

supportive measures to promote independence and prevent further complicatons (therapies, adaptive equipment )

parent/child bonding an issue

surgery cannot reverse neurological deficits

usually a shunt is necessary to relieve associated hydrocephalus rehabilitation

orthopedic appliances (crutches, braces, walkers, wheelchairs)

adaptive equipment

diet and bowel training

manage incontinence or colostomy

neurologenic bladder-catheter, crede(pressure on the bladder to go to the bathroom)

care of the child with severe spinal defects requires a team approach:

• neurosurgeon
• orthopedist
• urologist
• nurse
• social worker
• OT
• PT
• parents
• teachers,etc

psychological support to help parents accept the diagnosis and the child

positioning and handling

sensory stimulation

promote bonding

independence

working with the child and family

prevent complications

contractures-pressure ROM

decubiti ulcers

handle the infant carefully

do not apply pressure to the defect

can be minimized by PROM exercises

moderately abduct hips with a pad between knees

to help them cope with the infant's physical problems and to successfully reach the long-term goals