Spina Bifida

Sarah Winter, MD

Assistant Professor

Cincinnati Children’s

Division of Developmental Disabilities

Terminology

• myelomeningocele/meningomyelocele– /myelodysplasia

• meningocele

• lipomeningocele

• spina bifida vs. spina bifida oculta

When Does It Occur?

• Failure of closure of the neural tube occurs within the first 28 days of pregnancy.

Embryo at 21 Days

What is SPINA BIFIDA?

• One of the neural tube defects

• Abnormal development of the spinal cord and brain

Pictures of Spina Bifida

What Causes Spina Bifida?

• Nutrition? Folic acid supplements are protective of neural tube defects

• Genetics? families have higher risk if have had previous child with a neural tube defect

• multifactorial

Who Can Get Spina Bifida ?

• 0.6 out of every 1,000 children has a neural tube defect.

• Highest prevalence in certain ethnic populations.

• Slightly more girls than boys have spina bifida

• Occurs less in African Americans than in white individuals.

Detection of Neural Tube Defects

• Chorionic villous sampling 10-12 weeks

• Alphafetoprotein week 16

• Amniocentesis week 16

• Screening ultrasound

• High resolution ultrasound

Prenatal Intervention

• Genetic Counselling

• Prenatal consultation with neurosurgery and medical director of Spina Bifida clinic

• In-utero repair – approx. 22 - 25 weeks gestation, – normal foot and leg movement.

History of the Treatment of Children with Spina Bifida

• presence of infants with myelomeningocele detected 3000 years ago

• most left untreated until the development of methods to treat hydrocephalus (late1950’s)

• Lorber criteria accepted (1970’s)

• New England Journal of Medicine, 1985: “Early surgical care is now appropriate for most newborns with myelodysplasia”

Treatment

• Immediate repair of spinal defect (<48 hours)

• treatment of hydrocephalus as necessary – 85-90% of all children with SB

Spina Bifida

• Spinal Cord Defect

• Chiari II Malformation

Spinal Cord Defect

• paralysis below the level of the lesion

• neurogenic bladder

• neurogenic bowel

• insensate skin

• sexual dysfunction

• orthopedic deformities

Paralysis below the level of the lesion

Orthopedic concerns

Mobility

• Many people with spina bifida need something to help them move about.– crutches and walkers

– wheelchairs

– leg braces

Insensate Skin

Bladder and Bowel

• Nerves that control the bladder and bowel were damaged before the child with spina bifida was born– this means that the child may have trouble with

urination & bowel movements.– many children with spina bifida need to use

special medicines and treatments, such as catheterizations, to help them void.

Latex

Caution!• Art supplies: paints,

glue, erasers, fabric paints

• Balloons, Balls• Toys• Rubber Bands

Chiari II Malformation

Chiari II Malformation

• hydrocephalus

• learning disability

• swallowing difficulties

• vocal cord paralysis

• cognitive impairment

• central ventilatory dysfunction

Hydrocephalus- continued...

• A shunt is put in the child’s head to help drain the fluid from the brain to an open area around the stomach. Then it is absorbed back into the blood.

Shunt Malfunction

• Headache

• Vomiting

• Decreased level of alertness

• Decline in school performance

School• A lot of children with

spina bifida have trouble with certain parts of school– attention

– memory

– comprehension

– organizing

– writing

Strategies In School

• Early Intervention/Preschool

• Special Education as Needed / IEP

• Regular Classroom Supports

• Physical and Occupational Therapy

• Nursing Services-Catheterizations/Meds

• Adapted Physical Education

School Issues

• Social Isolation• Accessibility Issues• Peer Acceptance• Safety Issues• Recreational

Peer Acceptance

• Education about the Disability

• Differences/ Similarities

• Respect Issues

Encourage parents to allow their children with spina bifida

to experience life.Hold high expectations for their child and encourage them to continue to try new tasks to increase their feeling of

self-competence and confidence .

Children with spina bifida need to overcome many obstacles and deal with issues regarding peer, as well as personal acceptance.

What Does All This Mean cont...

• People with spina bifida can lead long and fulfilling lives.– graduate from high

school, go to college, have successful careers

– get married

– some may have children

Salim,

One Year Old

RESOURCES

• National and Local SBA• Bureau for Children with

Medical Handicaps/SSI• Therapeutic Recreation• Bureau for Vocational

Rehabilitation• Spinabilities• Children with Spina Bifida:A

parent’s Guide Woodbine House 1999

• UACCDD

Questions?