1. Paige Terrien Church, MD
Neonatologist & Developmental Behavioral Pediatrician
Assistant Professor, Paediatrics
University of Toronto

2. Disclosures
I have no actual or potential conflict of interest in relation to this program.
I also assume responsibility for ensuring the scientific validity, objectivity, and
completeness of the content of my presentation.
I do have a personal interest in this program.

3. Objec.ves
 Review Neural Tube Defects (NTD’s)
 Review medical impact of NTD’s in adolescence
 “The most complex congenital malformation
compatible with life.” G. Liptak, MD
 Review medical issues
 Review psychosocial issues at adolescence
 Discuss considerations with family planning

4. Terminology
Spinal Dysraphism
Occult Spinal Spina Bifida
Spina Bifida Aperta Occulta
Dysraphism (20% of population)

5. Epidemiology
 Prevalence in Canada
 2.6/10000 births
 Prevalence in US
 6‐7/10000 births

8. Neurosurgical Issues
 Hydrocephalus
 2 peaks to malfunction (infancy and adolescence)
 Signs of shunt malfunction may be insidious
 Tethered cord syndrome
 Progressive damage to spinal cord which is immobilized
 Initial release versus repeat releases
 Headaches common
 Unknown (35%)*
 Shunt blockage (25%)

9. Bladder & Bowel
 Urologic:  Bowel
 Tethered cord changes  DIET!
 Bladder irritability  Medication
 Surgical follow up
 Increased risk for
bladder cancer

10. Bladder & Bowel
 Achieving continence is
critical to self‐esteem
 Studies have
demonstrated:
 Greater likelihood of
intimacy
 Greater success in
workplace

11. Pelvic Floor
 Sling of muscles
 Keep organs in optimal
position to work
 At risk for early
weakness

12. Pelvic Floor
 Weakening may result in shifting of pelvic
organs
 May cause bladder irritability or constipation
 Impact of hormones on bladder
 Possible management with oral contraceptives

13. Orthopedic

14. Orthopedic
 Rotator cuff critical to
maintain strength
 Maintenance of strength
and function critical
 Critical muscle group:
Hip adductors

15. Medical Issues
 Weight management critical
 Percentage of body not as metabolically active
 Obesity increases all complications!
 EXERCISE

16. Learning Profile
 Individuals with SB have a different learning profile
 Characterized by consistent inconsistency!
 Scattered areas of strengths and weaknesses in learning,
intelligence, attention, and organizational abilities
 Adult providers may not recognize weaknesses

17. Psychosocial Issues
 Independence critical to self‐esteem
 Increased risk for depression, anxiety, and low self‐
esteem
 Factors involved in life satisfaction:
 Social factors (friends, family, spouse)
 Financial independence
 Sex life

18. Sexuality
 Sexual Orientation
 Danger of presumption
 Sexual Education
 1/4 without awareness
of contraception or
conception

19. Sexuality
 Extent of sensation and intact mechanics depends on
lesion
 Minimal research on SB specifically
 Silendafil (Viagra) can improve erectile dysfunction
 Rings, pumps

20. Sexuality
 Reproductive Health Education
 Hygiene
 Sexually transmitted infections
 Contraception considerations
 Dexterity with hands
 Risk of a blood clot
 Latex free condoms (now more affordable!)

21. Family Planning
 Genetic counseling needed
 Pregnancy is possible
 Considerations in terms of assisted reproductive technology
 Risks during pregnancy
 Bladder instability, UTI
 UTI’s may lead to preterm labor
 Decreased mobility
 Pressure ulcers

22. Medical Checklist
 Family doctor/provider
 Urology
 Neurosurgery
 Physiatry/Physical Medicine and Rehabilitation
 Orthopedics
 Obstetrics/Gynecology/Urogynecology
 Physiotherapy
 Occupational therapy

23. Conclusion
 NTD’s with life long complications
 Education needed
 Avoid disenfranchising
 Protect and maintain optimal health
 Approach Folic Acid from prevention and minimizing of
morbidity

24. Holland Bloorview SB/SCI Team
 Coordinator  Social Work
 Novlette Douglas  Gert Montgomery
 Nursing  Psychology
 Elaine Mckay  Andrea Snider
 Julia L’Abbe  Orthopedics
 Carolyn Wilson  Jim Wright, MD
 Occupational Therapy  Urology staff
 Beth Morgan
 Transition
 Physiotherapy  Dr. Berbrayer
 Kim Moody
 Kelly Brewer
 My gratitude to an
 SLP
amazing team!
 Sukaina Hameer