Sie sind auf Seite 1von 34

Spina Bifida

Jessica Kinsey

Spina Bifida Overview1


Failure of fusion of caudal neural
tube
Etiology
Folic acid deficiencies
Chromosome abnormalities
Single gene disorders

Prevention: 70%
Maternal folic acid
supplementation
Image from: http://wiki.cns.org/wiki/index.php/Spina_bifida

Image from: http://www.daviddarling.info/encyclopedia/S/spina_bifida.html

Myelomeningocele
Neural Tube Defects1
Anecephaly, encephalocele, spina bifida

Neurulation: Formation of neural tube1


Defect of primary neurulation- brain and most of
spinal cord formed

Defects occur between 3 and 4 weeks of


gestational age2,3
Occurs 1/1000 live births3

Complications
Anecephaly: Failure of fusion in cranial region of
neural tube4
Termination, stillbirths, survive hours-days

Open Spina Bifida: Spinal cord exposed at birth,


requiring surgery5
Hydrocephalus: Extra fluid in and around the
brain, causing ventricles to swell4
Requires shunt: Hollow tube that drains fluid
from brain and protects from pressure and
damage

Neural Tube Defects Overview4

Etiology
Occurs at birth, more common in
girls than boys2,3
NTD2,3
High Risk: Hispanic, Caucasian
Low Risk: African American, Asian

Age: Small Effect 1


Risk elevated in very old or very
young mothers

Family History1
Siblings of affected individuals 3-8%

Drugs1
Valproic acid and Carbamazepine

Image from:
http://blog.amsvans.com/
adorable-spina-bifidacover-girl-lights-upparents-magazine/

Etiology
Inadequate folic acid1
Increases risk by 2-8x

MTHFR Deficiency2
10% of whites

Obesity & Maternal Diabetes2


BMI >29: 2x greater risk
Diabetes increases risk by 2-10x
Glucose has teratogenic effect and
may alter expression of genes
involved in development

Recurrence risk: 1-5% 3


2.5x greater than general
population

Image from:
http://www.mountainside
-medical.com/naturesbounty-folic-acid400mcg-tablets.html

Genetics2

Risk Factors1

Prevention/Nutrition Diagnosis 2,5,6


Folate Intake: 4oo mcg/day
For 1 month prior to pregnancy and first 3 months of
pregnancy

Education/Health Promotion Programs


Encourage women to take folic acid supplements
periconceptually and increase folate rich foods

Folate Fortification: 20-30% decrease


1998: Folate supplementation in fortified cereal
grains required
1999: Over 100 products fortified including cereal,
fruit juice, and milk

Labs/Parameters 1,7
Blood tests
Alpha-fetoprotein

Two-dimensional ultrasound
Ability to detect in 79% of cases

Magnetic Resonance Imaging


Assesses malformations of CNS

Amniocentesis
Remove sample of amniotic fluid
to assess AFP

Diagnostic Tests3
Blood pressure for hypertension for adults if
blood pressure is higher than 120/80
Bone mineral density for osteoporosis
Pressure sore evaluation if necessary
Hematocrit or hemoglobin levels, if anemia is
potential problem
Blood urea nitrogen and other renal issues
evaluated
Glucose tolerance and lipid disorder tests if
overweight

Treatment 1
No treatment until after birth
Caesarean birth may be safer

Closure of spinal lesion within 48 hours of birth


Ventriculoperitoneal Shunt: 80%
1 year survival 87%
17 years survival 78%
Physiotherapy
Occupational therapy
Support
Image from:
http://www.sbccincy.org/?fuseaction=cms.page&id=1269

Surgical Treatment
https://www.youtube.com/watch?v=YG4nrRnAlcc

Images from: https://awordtothemany.wordpress.com/2012/10/08/spina-bifida-awareness-day-8/


http://www.glogster.com/shannamiller/spina-bifida/g-6lpa06ageaht71bruq8f9a0

Common Associations3
Epilepsy occurs in approximately 20%
Urinary tract infections and bowel dysfunction
Due to paralysis and renal problems

Swallowing problems, vocal cord paresis causing


stridor, neck pain, facial weakness, increased
weakness in lower extremities, cognitive
dysfunction if Chiari malformation of brain
Tethered cord
Progressive deterioration in lower extremity function,
changes in urinary tract function, progressive
scoliosis, pain

Problems Associated with Age3


Problems may increase as an adult ages
Overweight and obesity
Short stature with high body mass index
Decreased energy needs due to reduced physical
activity and lean body mass
Anxiety and depression
Decreased muscle tone
Orthopedic problems due to paralysis, lesion level,
and congenital skeletal deformities

Problems Associated with Age3


Osteoporosis and fractures due to reduced
physical activity and low bone mineral density
Scoliosis
Skin breakdown and pressure sores due to
excessive weight and decreased sensation of pain
Constipation due to decreased physical activity,
hypotonia, high lesion, decreased bowel control,
low fiber, inadequate fluid intake
Latex allergy
Hypertension
End-stage renal disease with high lesion in adults

Referral to Dietitians3
All children should be seen before age 5
Weekly in infancy
Monthly after age 1 month
Every 3 months after age 1 year

If weight is above 35th percentile or below 5th


percentile or mobility loss is greater than 25%

Referral to Dietitians3
Mechanical feeding problems, allergies, diarrhea,
constipation, diabetes, obesity, hypertension, lipid
disorders, food or vitamin/mineral faddism, use of
alternative therapy
Obesity
Major concern after age 4 if the lesion from
myelomeningocele is high and child can no longer
walk

Eating disorders

Role of the Dietitian3


Anthropometric Measurements
Upper-arm length used to evaluate height
Growth charts for up to age 20 developed using 1,900
males and females with myelomeningocele
Skinfold measurements used to coincide with arm
muscle area and circumference to indirectly determine
lean body mass, which energy needs are based on

Food Recall
Indicates individuals ability to make independent
food choices, skill level for tasks like food preparation,
ability to shop, willingness to follow direction

General Nutrition Diagnosis8


Excessive energy intake
Increased body adiposity
Energy intake higher than estimated need
Reduced energy needs related to altered body
composition, short stature

Swallowing difficulty
Condition associated with malformation of brain

Altered gastrointestinal function


Constipation
Condition associated with diagnosis: neurogenic
bowel

Nutrition Treatment3,5
General, healthful diet of nutrient-dense foods
Low calorie
7 kcal/cm of height for minimally active
9-11 kcal/cm of height for highly active
Obesity

Increased fluids

Water preferred to limit extra energy, sodium,


phosphorus
8-10 cups per day
Bowel issues

Increased fiber
Bowel issues

Image from: Google Images

Nutrition Treatment3,5
Folic Acid
400 mcg/day depending on lesion and ambulation

Increased protein
Skin impairments and healing

Adequate vitamins and minerals


Skin impairments and healing

Image from: Google Images

Nutrition Treatment3
Bladder/Renal Issues
Acid ash diet

Latex Allergy
Avoid avocado, kiwi, bananas, water chestnuts,
chickpeas

Hypertension: Low sodium


Nutrition support usually not an issue except in
end-stage renal disease
Must be evaluated every 1-4 months

Recommended Foods3

Sample Menu: 12-year old patient


Breakfast:

1 cup steel-cut oats


2 scrambled egg whites
1 pear
1 cup skim milk
2 cups water

Lunch:
2 slices whole-wheat
bread, 3 ounces turkey
breast, 1 slice low-fat
cheese
1 cup broccoli
1 cup low-fat vanilla
yogurt
1 apple
3 cups water

Dinner:

1 grilled chicken breast


1 cup brown rice
cup black beans
1 cup spinach, cup
peppers, cup dried
cranberries, cup
walnuts, cup low-fat
shredded cheese, 2
tablespoons fat-free
Italian dressing
cup mixed berries
2 cups water

Snacks:
Celery and peanut
butter
Apple and carrot sticks
Granola bar
cup almonds
3 cups water

Image from: Google Images

Continuous Monitoring 3,6


Head circumference
Bladder function: UTI, renal function
deterioration
Bowel Movements: Suppositories, laxatives
Weight maintenance
Skin care
Mobility including feeding skills
Neuropsychological development
Biochemical values
Oral Cavity

Image from: Google Images

Medications9
Mother: Folate supplementation
Multivitamins
Antibiotics for UTI
Amoxicillin, Trimethoprim

Anticholinergic Drugs 10
Continence, prevention of infection, and preservation
of urinary tract

Life with Spina Bifida


Complications: obesity, leg weakness, paralysis,
sensory loss, bowel and bladder dysfunction,
orthopedic abnormalities3
Concerns5

Independence
Confidence, Self-esteem
Motility/Physical Activity
Urination/Bowel Movements: Incontinence, UTI
Skin complications: Blisters, sores, burns, calluses
Latex allergy

Living with Spina Bifida


Spina bifida on lower spine, near hips5
Crutches, braces, walkers

Spina bifida on spine, near head5


Paralyzed legs, wheel chairs

The Wheelies Club3


Racing wheelchairs for adults

Special Olympics3
Careers3
Encourage careers that do not require
extensive standing/walking

Image from:
http://www.cdc.gov/ncb
ddd/spinabifida/schoolage.html

References
1. Mitchell L, Adzick N, Melchionne J, Pasquariello P, Sutton L, Whitehead A. Spina
bifida. Lancet [serial online]. November 20, 2004;364(9448):1885-1895. Available
from: MEDLINE with Full Text, Ipswich, MA. Accessed October 13, 2014.
2. Frey L, Hauser W. Epidemiology of neural tube defects. Epilepsia [serial online].
2003;44 Suppl 3:4-13. Available from: MEDLINE with Full Text, Ipswich, MA.
Accessed October 15, 2014.
3. Academy of Nutrition and Dietetics. Nutrition Care Manual. Spina Bifida. 2014.
Available at:
http://www.nutritioncaremanual.org/topic.cfm?ncm_category_id=1&ncm_toc_id=2
55331&ncm_heading=Nutrition%20Care&ncm_content_id=110090#Overview.
Accessed November 11, 2014.
4. Mastroiacovo P, Leoncini E. More folic acid, the five questions: why, who, when, how
much, and how. Biofactors (Oxford, England) [serial online]. July 2011;37(4):272279. Available from: MEDLINE with Full Text, Ipswich, MA. Accessed October 15,
2014.
5. National Center on Birth Defects and Developmental Disabilities, Center for Disease
Control and Prevention. Living with Spina Bifida: Toddlers and Preschoolers,
Infants, Adolescents and Teens, Young Adults. September 18, 2014. Available at:
http://www.cdc.gov/ncbddd/spinabifida/toddler.html. Accessed October 15, 2014.

References
6. Bower C, Miller M, Payne J, Serna P. Promotion of folate for the prevention of neural tube
defects: who benefits?. Paediatric And Perinatal Epidemiology [serial online]. November
2005;19(6):435-444. Available from: MEDLINE with Full Text, Ipswich, MA. Accessed
October 15, 2014.
7. Araujo Jnior E, Nakano M, Moron A, et al. Comparison between 2D ultrasonography and
magnetic resonance imaging for assessing brain and spine parameters in fetuses with spina
bifida. Archives Of Gynecology And Obstetrics [serial online]. May 2013;287(5):845-849.
Available from: MEDLINE with Full Text, Ipswich, MA. Accessed October 15, 2014.
8. American Dietetic Association. Journal of the American Dietetic Association. Position of the
American Dietetic Association: Providing Nutrition Services for People with
Developmental Disabilities and Special Health Care Needs. February 2010. Available at:
http://www.eatright.org/search.aspx?search=spina%20bifida. Accessed November 11,
2014.
9. Zegers B, Winkler-Seinstra P, Uiterwaal C, de Jong T, Kimpen J, de Jong-de Vos van
Steenwijk C. Urinary tract infections in children with spina bifida: an inventory of 41
European centers. Pediatric Nephrology (Berlin, Germany) [serial online]. April
2009;24(4):783-788. Available from: MEDLINE with Full Text, Ipswich, MA. Accessed
October 15, 2014.
10. Ferrara P, D'Aleo C, Salvaggio E, et al. Plasma antidiuretic hormone levels in children with
spina bifida. Urologia Internationalis [serial online]. 2002;68(3):144-147. Available from:
MEDLINE with Full Text, Ipswich, MA. Accessed October 15, 2014.

Das könnte Ihnen auch gefallen