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Spina Bifida

From SBAA. Spina Bifida. Hilary Suzawa Med/Peds May 2006. Spina Bifida—3 types. Spina bifida occulta Spina bifida cystica Meningocele Myelomeningocele. Epidemiology. ~1 out of 1,000 infants in US born with spina bifida 90-95% of babies born with SB are born to parents with no FMH of SB

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Spina Bifida

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  1. From SBAA Spina Bifida Hilary Suzawa Med/Peds May 2006

  2. Spina Bifida—3 types • Spina bifida occulta • Spina bifida cystica • Meningocele • Myelomeningocele

  3. Epidemiology • ~1 out of 1,000 infants in US born with spina bifida • 90-95% of babies born with SB are born to parents with no FMH of SB • More common in girls than boys • Most common Neural Tube Defect (NTD) • Most frequently occurring birth defect that results in permanent disability

  4. Risk Factors • Family history (RR 30) • If parents have 1 child with SB, the risk of having another child with SB are 1-5% • If one parent has SB, the changes of having a child with SB are 3-5% • If both parents have SB, the changes of having a child with SB are 15%

  5. Risk Factors • Folic acid deficiency—i.e. not taking supplement (RR 2-8) • Pre-gestational maternal diabetes (RR 2-10) • Anticonvulsants—Valproic acid and/or Carbamazepine (RR 10-20)

  6. Prevention • For the general population, taking 400 mcg per day of folic acid both prior to pregnancy and during first trimester, reduces incidence of SB by 75% • Women with SB should take 4000 mcg per day of folic acid for 1-3 months before pregnancy

  7. Diagnosis • Maternal serum alpha-fetoprotein • Screening at 16-18 weeks gestation (in the second trimester) • As a rule of thumb a value above 2.0-2.5 MoM is abnormal but depends on lab and maternal factors • Ultrasound • Follow-up with amniocentesis

  8. Ultrasound Findings From Up to Date

  9. Treatment • Closure of spinal lesion is usually done within 24-48 hours of birth. • If there are signs of hydrocephalus then may place VP shunt

  10. Prognosis • 1-year survival ~90% • ~80% of all individuals with SB survive to age 17 years • ~90% of babies born with spina bifida survive into adulthood • ~80% have normal IQ scores • ~75% are able to take part in sports and recreational activities

  11. Medical Complications • Neurologic • Urologic • GI • Orthopedic • Dermatologic • Sexuality and Fertility

  12. Neurologic • Most pt have normal intelligence • Most pt with SB have strong verbal skills but may have difficulties in other areas—Nonverbal Learning Disabilities • Memory • Understanding • Attention • Organization • Sequencing

  13. Neurologic • Hydrocephalus • Occurs in 70-90% of pt with myelomeningocele • Almost all pt with thoracic level lesions need a shunt • Less than 70% of pt with sacral level lesions need a shunt • ~90% of adults with SB will have a VP shunt • Pt with shunted hydrocephalus may have poor hand-eye coordination and may have difficulty “picturing” things in their head • Chiari II Malformation • herniation of the cerebellum and hindbrain that prevents drainage of CSF • Third ventriculostomy • Small hole made in the bottom of 3rd ventricle, one-time procedure offered to adolescents and young adults

  14. Neurologic • Tethered Cord Syndrome • May be asymptomatic until adulthood • Decreased strength, spasticity, back pain, decreased mobility, incontinence • Hydromyelia • Dilation of the central canal of spinal cord with increased fluid accumulation • Rapidly progressive scoliosis, weakness of UE, spasticity, ascending motor strength changes in the LE

  15. Urologic • UTIs • Neurogenic Bladder • Intermittent catheter program • Crede’s maneuver—applying pressure on the lower abdomen over the bladder • Urinary incontinence • Medications—anticholinergics,oxybutynin (Ditropan), tolterodine (Detrol) • Surgical procedures • More than 80% of adults are able to develop social bladder continence

  16. Urologic • Renal Ultrasound recommended at least QOY • Bladder augmentation surgery to allow easy access for self-cath • 10 year follow-up cystoscopy and then annually thereafter

  17. GI • Fecal incontinence • Constipation • Encourage fiber 25-30 grams/day • Encourage water 6-8 glasses/day • Stool softeners, laxatives, enemas • Goal of bowel management program is to have predictable bowel emptying at least q3 days

  18. GI • Obesity • Encourage healthy diet and regular exercise • Americans with Disabilities Act ensures that pt with disabilities have the right to participate in the same recreation activities as everyone else • At gym, check if machines have latex • Weight loss goal of ½-1 pound/week

  19. Orthopedic • Spasticity • Clubfoot • Dislocated hips • Arthrogryposis—prenatal fixation of joints in a flexed or contracted position • Impaired mobility • 50% of pt walking with braces at age 12 yrs stopped walking altogether by age 20 yrs • Charcot Joints • Overuse Syndromes • Osteoarthritis • Osteoporosis

  20. Dermatologic • Latex allergy • ~75% of pt are sensitive to latex • Cause of latex allergy unknown • Pt should carry non-latex gloves for emergencies

  21. Health Care Items Gloves Catheters Tourniquets Elastic bandages Ace wraps IV tubing Medication vials Adhesive tape/band aids Home Items Balloons Dental dams Rubber bands Elastic in clothing Condoms Art supplies Latex

  22. Dermatologic • Pressure ulcers and other skin breakdown • Encourage weight shifts (wheelchair pushups) every 10-15 minutes This is not actually a wheelchair push-up. Don’t worry, your pt doesn’t have to lift the wheelchair.

  23. Sexuality and Fertility • People with disabilities are 2-3x more likely to be sexually abused • No latex condoms • Animal skin condoms work for birth control but not effective prevention for STDs/AIDS • ~40% of people with spina bifida reported ability to achieve an orgasm

  24. Sexuality and Fertility: Men • Although most men are fertile, difficulty with erection and ejaculation may interfere with conception • 2 types of erections: psychogenic or reflexive • Lesion at or above L2 do NOT have psychogenic erections • Lesion between T11 and L2 usually have neither • Some men with SB will be able to maintain erection for intercourse w/o meds • Viagra improves erectile function in 80% of men with SB • Most men with S1-S5 lesions can ejaculate

  25. Sexuality and Fertility: Women • Most women have normal fertility • Encourage women to empty bladder before and after sex to avoid UTI • Orgasm • Lesions above T11 usually will not have orgasm • Lesions below T11 may have orgasm • Pt with SB have higher risk of having child with SB—should take folic acid 4,000 mcg daily 1-3 months pre-pregnancy • A woman with SB may not be able to feel labor contractions so may need a home monitor device to alert her when labor begins

  26. From Point A to Point B Spina Bifida Association at http://www.sbaa.org

  27. Bibliography • Barker E et al. Spina Bifida. RN 2002; 65 (12): 33-39. • Hochber L and Stone J. Etiology, prenatal diagnosis, and prevention of neural tube defects. Up to Date. • Klingbeil H, Baer H, and Wilson P. Aging With a Disability. Arch Phys Med Rehabil 2004; 85 (3): S68-S73. • Mitchell L et al. Spina Bifida. Lancet 2004; 364: 1885-1895. • Health Guide for Adults Living with Spina Bifida published by the Spina Bifida Association at http://www.sbaa.org

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