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SPINA BIFIDA
Presentation and Management of
Patients at Texas Scottish Rite
Hospital for Children
Dallas, TX
Susan McGuire, DDS
April 19, 2013
Assistant Professor, Department of Pediatric
Dentistry, Texas A&M University Baylor
College of Dentistry, Dallas, TX
Assistant Director, Special Needs Dental Clinic,
Texas Scottish Rite Hospital for Children,
Dallas, TX
SPINA BIFIDA
• Overview of Condition
• Texas Scottish Rite Hospital for Children
– Medical Management
– Dental Management
Spina Bifida
• One of most common congenital birth
defects
• 1-2 per 1000 births worldwide
• 1500-2000 births in USA annually
• Prevalence
– Hispanics>Caucasians>African Americans
– Girls>Boys
Overview
Formation of Neural Tube
• During the first 3-4 weeks, specific cells form a
narrow tube that becomes the foundation of the
spinal cord, brain bone and tissues.
• At 28 days of gestation,
neural tube closed.
• If the tube does not close
properly anywhere
along its entire length,
then a neural tube defect
will form at the open location.
Overview
Etiologies
• 95% do not have any family history
• Maternal
– Age
– Obesity
– Diabetes
– Seizure medications
• Folic acid deficiencies
– 0.4 mg/day
Overview
Diagnosis in utero
• Measurement of maternal serum alphafetoprotein (MSAFP)
– If elevated at 16-18 weeks of gestation can indicate
defects
• Ultrasound examination
– 18 weeks
• Amniocentesis
– Use amniotic fluid alpha fetoprotein (AFAFP) and
acetylcholinesterase (AFAChE) to confirm
Overview
Neural Tube Defects
• Affect the brain’s development and leave the
spinal cord vulnerable to damage
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• Paralyze or weaken
associated muscles
and affect organs
• Two Types
– Closed
– Open
Overview
Closed Neural Tube Defects
• Localized and confined to the spine; brain
rarely affected
• Neural tissue not exposed; spinal defect fully
covered by skin
• May remain undetected for years, especially in
absence of cutaneous markers
• Types:
– Lipomyelomeningocele
– Lipomeningocele
– Tethered cord
Overview
Open Neural Tube Defects
• Involve the entire CNS
• Neural tissue exposed with
associated CSF leakage
• Skin covering not intact
• Visible at birth; majority
discovered during pregnancy
Overview
Spina Bifida
• Characterized by the incomplete development of
the brain, spinal cord and/or meninges
• Nerves located below malformation are affected,
resulting in loss of sensation and paralysis.
• Complications may be minor physical problems
to severe physical and mental disabilities.
• Types:
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Anencephaly
Occulta
Meningocele
Myelomeningocele
Overview
Spina Bifida Occulta
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Mildest and most common form
One or more vertebrae are malformed
Opening in spine is covered by a layer of skin.
Rarely causes disabilities or symptoms
• Often undetected unless visible exterior sign is present
– Tuft of hair, dimple, birthmark, bulge at site of
malformation
Overview
Meningocele
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Meninges protrude from the spinal opening
May or may not be symptomatic
Noticeable bulge on back
Repaired surgically
Usually no resultant paralysis
Overview
Myelomeningocele
• Most severe and rarest form
• Occurs when the spinal cord is exposed through
the opening in the spine
• Results in partial or complete paralysis of the
parts of the body below the spinal opening
Overview
Treatment of Spina Bifida
• Surgery to close defect within 24 to 48 hours of
birth
Medical Management
Treatment of Spina Bifida
• Before surgery, the child must handled carefully
to reduce further damage to exposed spinal
cord
• Lifelong treatment for other organ system
disturbances secondary to damage to spinal
cord or spinal nerves
Medical Management
Team Approach to Management at
Texas Scottish Rite Hospital (TSR)
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Neurosurgeon
Developmental Pediatrician
Neurologist
Urologist
Orthopedic Surgeon
Dentist
Other services involved:
– Psychology, Child Life, Orthotics, PT, OT, Dietary,
Family Services
Medical Management
Associated Impairments
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Arnold-Chiari II malformation
Hydrocephalus
Seizure disorders
Bladder and bowel incontinence
Musculoskeletal Deformities
Learning disabilities
Altered wound healing
Latex allergy/risk
Medical Management
Arnold-Chiari II Malformation
• Downward displacement of the cerebellum,
cerebral tonsils, brainstem, and 4th ventricle
through the foramen magnum and into cervical
space
• Results in compression and elongation of nerves
and tissue responsible for vital functions
including respirations and protective reflexes
Medical Management
Arnold-Chiari II Malformation
Normal
Malformation
Medical Management
Arnold-Chiari II Malformation
• Symptomatic in about 33% of affected persons
– Difficulty swallowing
– Inspiratory stridor
– Stiffness or spasticity of arms or hands
– Poor balance and coordination
• Leading cause of death in spina bifida population
Medical Management
Hydrocephalus
• 80-90% develop hydrocephalus
• Abnormal accumulation of CSF
• Increased intracranial pressure
– Progressive enlargement of the head
– Convulsion
– Mental disability
Medical Management
Shunts
Hydrocephalus
• Placed to relieve
intracranial pressure
• Types:
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Ventriculoperitoneal (VP)
Ventriculoatrial (VA)
Ventriculopleural
Ventriculo-gall bladder
• VP shunts empty CSF directly into the
abdominal cavity, bypassing the venous
circulation.
• Hypersensitivity to loud noises
Medical Management
Hydrocephalus
Seizure Disorders
• Occur in approx 15% of patients
• Usually generalized tonic-clonic type
• Responds well to anticonvulsant medication
• Recurrent seizure activity may indicate shunt
blockage or infection.
Medical Management
Hydrocephalus
Learning Disabilities
• At least 80% have
normal intelligence
• Cognitive dysfunctions correlated to:
– Hydrocephalus and related complications
– Level of lesion
• Upper level lesions associated with mental
retardation
Medical Management
Hydrocephalus
Learning Disabilities
• Dysfunctions include:
– Short attention span
– Decreased arm and
hand function
– Poor eye-hand coordination
– Memory deficits
Medical Management
Bladder and Bowel Incontinence
• Urinary complications
- Hydronephrosis, incomplete emptying of the
bladder, urinary reflux, infections and incontinence
- May lead to renal damage
- Patients frequently use catheters and antibiotics.
• Altered GI motor and sensory function
– Impairs peristalsis
– Leads to constipation, impaction and bowel
incontinence
– Patients frequently require added fiber to diet.
Medical Management
Musculoskeletal Deformities
• Paralysis may lead to deformities
• 90% of patients with defect above sacral level
develop:
– Scoliosis
– Kyphosis
• Surgery often required
to correct spinal
curvatures.
• Loss of muscle strength and inactivity may lead
to pathological fractures.
Medical Management
Obesity
• 40% of patients
• Due to
– Impaired mobility
– Decreased energy expenditure
– High carbohydrate
food “rewards”
Medical Management
Altered Wound Healing
• Altered skin integrity over spine and cord
• Altered sensory function below the level of the
lesion results in risk of skin breakdown
throughout life
• Common risk factors
– Reduced mobility
– Nutritional status
– Bowel and urinary
incontinence
Medical Management
Latex Allergy/Risk
• 70% exhibit symptoms
• Predisposition to latex allergy unknown
– Increased need for
health care
shunts
other allergies.
Medical Management
Dental Management
As dentists, how do we manage all of these medical
complications in providing dental care to the
spina bifida patient?
Dental Management
Obtain Medical History
• Medical conditions
• Allergies
– Latex, Drugs
• Past surgical history
– Shunt
– Spinal instrumentation
• Medications
– Antibiotics, anticholinergics, sympathomimetics,
anticonvulsants and stool softeners
• Family history
• Appropriate consults
Dental Management
Dental Implications of Spina Bifida
Associated Impairments
• Latex Allergy/Risk
– Requires latex-free environment
• Medications
– Anticonvulsants
• Make sure patient has taken Rx to minimize risk of
seizure
– Antibiotics
• May need to switch ABX if required for shunt (VA)
or spinal instrumentation
Dental Management
Dental Implications of Spina Bifida
Associated Impairments
• Shunt
– Avoid putting pressure on shunt while treating patient
– May exhibit sensitivity to loud noises
• Seizures
– Make sure anticonvulsant medication taken
• Nausea, drowsiness
• Gingival hyperplasia
– Anxiolysis/Sedation medications may need to be
reduced
– Know proper protocol to manage seizure
• Broken teeth, tongue lacerations
Dental Management
Dental Implications of Spina Bifida
Associated Impairments
• Bladder and Bowel Incontinence
– Empty prior to dental treatment
• Scoliosis/Kyphosis
– Spinal instrumentation requires ABX
• Paralysis
– Postural hypotension likely, best not to treat patient
supine
– If chair-bound, treat in chair, tilt chair back
Dental Management
Dental Implications of Spina Bifida
Associated Impairments
• Obesity
– For anxiolysis/sedation, use ideal weight for patient
• Feeding/Swallowing Issues
– Gastrostomy tube: Increased calculus formation
– Tracheostomy: Protect airway
if patient unable to respond
protectively
• Minimize use of water
• Use suction judiciously
Dental Management
Oral Complications
• Poor oral hygiene
– Involuntary movements
– Lack of motor skills
– Vomit reflex during brushing
• Dental caries
– Poor nutrition
– Reduced salivary flow
– Long-term use of medications
Dental Management
Oral Complications
• Gingivitis
– Increased plaque
• Gingival hyperplasia
– Anticonvulsant medications
• Periodontal disease
– Increased calculus
Dental Management
Treating patients
• Wheelchair bound patients
– Can be left in wheelchair
• Sliding board can help with
supporting the patient’s head
• Wheelchair transfer to dental chair
• 2-person lift
– Under arms
– Legs
Dental Management
Anticipatory Guidance
• Chlorhexidine gluconate mouthwash for
gingival/periodontal issues
– May use as rinse or brush on tissues
• Fluoride for decreasing caries incidence
– Apply fluoride at all prophylactic appointments
• Preventive Restorations
– Sealants effective to reduce occlusal caries
Dental Management
Recall Schedule
• Individualized for patient’s needs
• Close observation of patients and regular dental
exams are important
• 2, 3 or 4 month recall schedule can be beneficial
Dental Management
Home Care
•Common Positions
– Child positioned in front of
adult. Adult cradles the child’s
head with one hand and uses
other to brush.
– Child in wheelchair. Adult sits
behind it. Lock the wheels and
tilt chair into the lap.
Dental Management
Oral Hygiene Tips
• Horizontal scrub toothbrushing
technique recommended.
– Electric toothbrush
– Customized toothbrush handles
– Toothettes
• Use of floss holders assists with
flossing
Dental Management
Behavior Management
• Behavior management techniques become
more important in these patients!
• Patients and their families generally exhibit
anxiety due to frequency of medical
interventions.
• It is important to spend additional time with
parent and child to establish rapport.
Dental Management
Behavior Management
• Parental Anxiety
– Talk to the parent and find out their expectations
– Tour the office
– Introduce staff
Dental Management
Behavior Management
• Patient Anxiety
– Schedule early in day
– Actively listen to patient
• Verbal/Non-verbal
– Speak at patient IQ level
– Keep appointments short
– Gradually progress to more complicated procedures
– Reward patient after successful completion of
procedures
Dental Management
Delivery of Care
• Immobilization
– Effective way to diagnose and
deliver dental care
• Helps protect patient, dentist, and
staff
• Make sure parents have clear
understanding of use
• Make sure you obtain proper
informed consent
• Includes parental assistance, extra
personnel, mouth props,
papoose board
Dental Management
Sedation
• Neurologically handicapped patients may be
unable to cope with dental treatment
• Individualized for each patient
• Review any changes in medical history
• Review allergies
• Review medications
– Confirm patients have taken
regular medications the
morning of procedure.
Dental Management
Sedation
• All medication doses determined by weight
– Obese patients, use ideal body weight/height
• Calculation: Height cm2 X 1.65 / 1000 = Kg
• Consider decrease in dosage of sedative agent by
1/3 for patients taking benzodiazepines,
barbiturates, valproic acid, MAO inhibitors,
elective serotonin re-uptake inhibitors
• Must include reversal agent and doses
Dental Management
General Anesthesia
• Sedation is contraindicated for ASA Type III and
IV patients, or any other patient not cleared by
his/her physicians.
• Amount of dental work
needed would require
more than 2 sedation
appointments.
• Failed sedation attempt
Dental Management
General Anesthesia
• Complete physical work-up for each patient
prior to general anesthesia
• Must follow NPO guidelines
• Benefits must outweigh risks!
Dental Management
General Anesthesia
• More aggressive treatment
– Ext vs. Endo therapy
– SSCs vs. Class IIs
– Amalgam vs. composite
– Limit occlusal anatomy
• Plaque accumulation
– Sealants
Dental Management
Summary
• Know your patient’s medical history
– Surgeries
– Medications
– Allergies
• Obtain necessary consults
– Need for ABX
• Be aware of patient’s anxiety level
– Consider sedation/GA
• Emphasize prevention.
• MOST OF ALL, be patient, understanding, and
compassionate.
Thank
Questions
you
References
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Ekmark EM. Risky Business: Preventing skin Breakdown in Children with Spina Bifida. Journal Pediatric
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