Caring For Patients With
This complex neural tube defect causes a variety of impairments that can impact the delivery of dental treatment.
By Faizan Kabani, RDH, MHA, MBA
After reading this course, the participant should be able to:
1. Discuss the prevalence, incidence, and ramifications of spina bifida.
2. Define the risks and protective factors associated with the development of this neural tube defect.
3. Differentiate characteristics between various types of spina bifida.
4. List common impairments and complications associated with spina bifida.
5. Identify oral health care considerations for patients with spina bifida.
Spina bifida is a neurogenetic disorder
with a complex etiology that involves genetic and environmental factors.1
It is part of a larger group of congenital birth defects termed neural tube
defects. These congenital birth defects affect various parts of the central
nervous system, such as the brain, spine, and spinal column.2 Spina
bifida, the most common neural tube defect, is a largely preventable,
noncommunicable, and permanently disabling congenital condition that results in
the incomplete development of the brain, spinal cord, and/or meninges.3
Health disparities continue to exist for individuals with neural tube defects
in both racial/ethnic and sex distribution. Hispanic individuals are at higher
risk than non-Hispanics.4 Non-Hispanic whites are at higher risk
than non-Hispanic blacks. In all racial and ethnic communities, women are at
higher risk than men.4
The United States Centers for Disease Control and Prevention's (CDC)
National Spina Bifida Patient Registry and Surveillance System estimates the
national prevalence of spina bifida at 166,000 individuals.4 Researchers
estimate the national annual incidence of spina bifida to be approximately
1,500 live births.4
There are significant social and economic costs for individuals with this
neural tube defect. Multiple studies have tracked national expenditure trends
in populations with spina bifida. Children with spina bifida had an approximate
13-fold increase in health care expenditures compared to children without
neural tube defects, whereas adults with spina bifida experienced a three-fold
to six-fold increase in health care expenditures compared to adults without
spina bifida.5 The medical care and surgical costs for all
individuals with spina bifida exceed
$200 million annually, with the lifetime cost of care for each child
born with spina bifida estimated at more than half a million dollars.5
Diagnostic screenings for the presence of spina bifida begins in the first
trimester of pregnancy at 28 days of gestation. To detect spina bifida, the
mother's maternal serum alpha-fetoprotein (MSAFP) levels can be measured, a
thorough ultrasound examination can be performed, or an amniocentesis can be
Multiple risk factors are associated with the development of spina
bifida: familial history of neural tube defects; mother's consumption of
anticonvulsant medication; and mother's systemic health status, such as
presence of obesity and/or diabetes mellitus type 2.4 Clinical and
public health studies have indicated that the proper intake of folic acid is
the most significant preventive method in reducing the risk of spina bifida.3
Folic acid is a water soluble B-9 vitamin that is available in supplements or through
dietary sources, such as asparagus, avocado, beans, milk, orange juice, salmon,
soybeans, and spinach.6 In 1992, the US Public Health Service
published the mean recommended dosage to minimize risk for pregnancies with
spina bifida at 0.4 mg (400 mcg).7 The CDC estimates that
approximately 50% of all pregnancies in the US are unplanned; therefore, it is
important for all childbearing age women to take the recommended dosage of
folic acid daily.4 Women at increased risk, such as mothers with
affected children, typically require a 10-fold prescriptive dosage of 4 mg
folic acid per day at least 1 month to 3 months before conception and
continuing through the first trimester of pregnancy to minimize risk for neural
Research has been conducted on the polymorphic mutational impact of the
methylenetetrahydrofolate reductase (MTHFR) gene in the incidence of spina
bifida.9 The polymorphic mutation in the MTHFR gene modifies the
innate ability of the subsequent MTHFR enzyme to process folate in the body.9
Further research is needed on the role of the MTHFR gene and enzyme on the
incidence of neural tube defects.
There are four
types of spina bifida: occulta; closed neural tube defect; meningocele; and
myelomeningocele, with the closed neural tube defect being the most rare.3,4
Occulta is the mildest and most common form, in which the vertebrae are
malformed.3 Occulta is present in approximately 10% to 20% of the
affected population, rarely causes disability, and is typically asymptomatic.3
Closed neural tube defect consists of a diverse group of vertebral defects
marked by malformation of fat, bone, or membranes, and can cause neurogenic
bowel and bladder.3
Meningocele and myelomeningocele manifest with a physiological protrusion
through an abnormal vertebral opening. In myelomeningocele, however, the spinal
cord and neural elements in the physiological protrusion are exposed and
vulnerable to symptomatic paralysis.3 Table 1 outlines the commonly
associated impairments and considerations in patients with spina bifida.10
or fluid in the brain, is caused by excessive accumulation of cerebral spinal
fluid (CSF), resulting in increased pressure in the ventricular environment.11
Approximately 80% of patients with spina bifida experience hydrocephalus.6
An excessive accumulation of CSF presents as an abnormally rapid increase in
cranial circumference or an unusually large head size.12 The
abnormal widening of the craniofacial structure creates potentially harmful pressure
on the cranial meninges, increasing risk for abnormal gait, altered
coordination, visual impairment, developmental delay, and/or convulsive
seizures.11 The typical medical protocol for hydrocephalus is the
surgical placement of a biocompatible shunt to redirect excessive cerebral
fluid from the ventricles into alternative vital organs for systemic excretion.10
Artificial shunts placed for the management of hydrocephalus include the
ventriculoperitoneal (VP) shunt, ventriculoatrial shunt, ventriculopleural
shunt, or the ventriculocholecyst shunt.12 The VP shunt is the most
The American Academy of Pediatric Dentistry and the American Heart
Association recognize that patients with certain types of complex medical
conditions are at an increased risk for bacteremia-induced infections, such as
infective endocarditis.13 Patients with shunted hydrocephalus that
have a ventriculoatrial shunt, ventriculocardiac shunt, or ventriculovenus
shunt to drain excessive CSF are at a significantly increased risk for
bacteremia-induced infections. As such, they should be prescribed prophylactic
antibiotic premedication for invasive dental procedures, as should those at
risk for infective endocarditis.13 Oral health care procedures that
engage in "manipulation of gingival tissues, periapical regions of teeth, or
perforation of oral mucosa" are invasive and susceptible to increasing the risk
for infective endocarditis.13 The use of prophylactic antibiotic
premedication decreases but does not eliminate the risk for bacterial-induced
infections.13 Clinicians should be judicious and selective in
prescribing antibiotics based on the individual needs of the patient to
minimize risk for antibiotic resistance and allergic sensitization.
Patients with impaired autonomic nerve function can manifest with
neurogenic bowel and bladder.11 The permanent nerve damage secondary
to spina bifida can result in interrupted voluntary communication between the
sphincter muscles, spinal column, and bowel/bladder function.14
Unfortunately, these patients experience both frequent urinary tract infections
and/or frequent fecal impactions. The typical medical management of neurogenic
bowel and bladder includes intermittent catheterization; urinary diversion,
such as vesicostomy or bladder augmentation; and pharmaceutical interventions.4
There are multiple oral health considerations for populations affected
with neurogenic bowel and bladder, including attentiveness to scheduling
appointments, provider sensitivity to patients' needs to use the restroom, and
xerostomia management.14 Patients with neurogenic bowel and bladder
should be encouraged to use the restroom before the appointment and clinicians
should frequently suggest restroom breaks during lengthy appointments.14
Oral health professionals should suggest methods to mitigate decreased salivary
flow, such as using over-the-counter glycerin-based mouthrinses and toothpastes
with a neutral pH, saliva substitutes, and oral lubricants.14
Patients with xerostomia are also at an increased risk for dental caries and,
therefore, need close monitoring and, if indicated, topical fluoride therapy.14
Some patients with spina bifida also manifest with the Arnold-Chiari II
malformation complex. This presents as an inferior migration of the cerebellum
directed proximally toward the foramen magnum; thus, the cerebellum's depth of
protrusion through the foramen magnum categorizes the stage of Arnold-Chiari
malformation complex.3 Radiologists can detect the depth of the
malformation through magnetic resonance imaging or computed tomography
diagnostic imaging. The second stage of the complex, known as Arnold-Chiari II
malformation, occurs when the cerebellar tonsils herniate through the foramen
magnum.3 Approximately one in every 1,000 live births and one-third
of patients with myelomeningocele have Arnold-Chiari II malformation.3,10
Patients can be asymptomatic; therefore, surgical intervention remains deferred
until the patient is symptomatic.3 If the patient is symptomatic,
then a surgical posterior fossa decompression intervention alleviates pressure
at the compression site.3 The common signs and symptoms of
Arnold-Chiari II malformation include abnormal gait, altered coordination,
irregular respirations, dysphagia, increased aspiration risk, hypersensitive
gag reflex, and hypotonia in the arms.6 The development of symptoms
varies on the typology and severity of the spina bifida.10
Oral health care implications for patients with Arnold-Chiari II
malformation include preliminary evaluation of a patient's gait and
coordination to assess increased risk for orofacial trauma secondary to
falling.14 Patients with Arnold-Chiari II malformation should be
positioned upright to minimize concerns with airway obstruction and dysphagia.14
Furthermore, oral health professionals should have extra personnel to
assist with suctioning during procedures that use water to minimize the risk of
aspiration.14 Clinicians can employ alternative approaches to
provide customized care to patients with special health care needs, such as
interchanging between wet and dry gauzes to cleanse the oral cavity during
procedures instead of spraying the air/water syringe and suctioning with the
saliva ejector if patients experience dysphagia and are at an increased risk
Many patients with spina bifida have orthopedic concerns such as
clubfoot, dislocated hips, spinal dysplasia, and atypical musculoskeletal
contractures.6 An estimated 90% of patients with a neural tube defect
experience musculoskeletal deformities, such as scoliosis, kyphosis, or
kyphoscoliosis.4 Patients with significant abnormal spinal
curvatures undergo surgical rodding procedures that attempt to align the spinal
column to a near-normal curvature.14 Some orthopedic surgeons
require patients to obtain medical clearances from other clinicians, including
the patient's oral health professional, prior to spinal instrumentation. As
such, patients who are candidates for spinal surgery need to complete oral health
care treatment as soon as possible.14 Oral health professionals need
to maintain communication with the patient and his/her orthopedic surgeon
regarding the medical necessity for prophylactic antibiotic premedication for
routine and invasive dental procedures post-spinal instrumentation.14
Patients with spina bifida are at increased risk for orofacial
musculoskeletal concerns, such as Angles Class II malocclusions.15
Patients with malocclusions and crowding have difficulty maintaining adequate
oral hygiene and are at an increased risk for developing both dental caries and
periodontal diseases.15 Patients with complex malocclusions may want
to see an orthodontist for therapeutic realignment. Oral health professionals
need to help patients minimize their incidence of oral diseases by stressing
oral hygiene education, increasing preventive dental hygiene visits, placing
dental sealants, frequently applying topical fluoride, and providing referrals
to dental specialists, as necessary.
Patients with spina bifida are at an increased risk for a compromised
vertebral column; thus, affected patients often experience a permanent altered
sensory function.4 Most patients with spina bifida are limited in
ambulation and are likely to use assistive devices, such as wheelchairs.14
There are multiple oral health care considerations for patients with
limited ambulation and insensate skin. Clinicians need to identify if the
patient, based on comfort and safety, should remain in the wheelchair or
transfer to the dental chair for treatment. If the patient remains in the
wheelchair, then oral health professionals will need to use adaptive ergonomics
to complete the necessary treatment. Alternatively, if a patient transfer
scenario is indicated, clinicians need to ensure that the patient's receiving
platform is at a lower height than the initial platform to minimize risk for
patient injury. For example, if a patient transfer is from wheelchair to dental
chair, the clinician should lower the dental chair so that the patient transfer
is from a higher to lower plane. Conversely, if a patient transfer is from
dental chair to wheelchair, then the clinician should raise the dental chair
slightly so that the patient transfer is from a higher to lower plane.
Additional resources, such as extra personnel and patient transfer boards, can
assist the patient during transfers.
FIGURE 1. Treating a patient with spina bifida on a soft
beanbag may help minimize the risk of pressure wounds.
COURTESY OF THE DENTAL CLINIC AT TEXAS SCOTTISH RITE HOSPITAL FOR CHILDREN IN DALLAS
Patients with impaired sensory function are at increased risk for
developing sores, calluses, blisters, and bruises. Due to localized spatial
areas of insensate skin, patients with spina bifida are likely to be unaware of
their pressure-based wound.4 Affected dental patients can benefit
from the use of an intermediary device placed between the patient and the firm
dental chair, such as a soft beanbag (Figure 1). A beanbag adapts to the
patient's musculoskeletal structure and redistributes the bodily pressures.
Patients placed on soft beanbags are seated both on adaptive, more secure
structures, reducing their risk for pressure wounds.14 Oral health
professionals should ideally schedule short appointments to minimize these
spina bifida are at increased risk of latex sensitivity.4 An
estimated 70% of patients with spina bifida exhibit physiological symptoms such
as pruritus, erythematous skin, itchy/watery eyes, dyspnea, wheezing, and
slurred speech, when exposed to latex.16 The exact etiology for
latex sensitivity in patients with spina bifida is idiopathic; however, many
sources trace the latex sensitivity to frequent exposures during both
post-natal medical and surgical interventions.17 Studies have also
demonstrated that latex sensitivities are associated with allergic reactions to
bananas, strawberries, and kiwi.18 Many health care institutions
have transitioned to nonlatex products when treating patients with spina bifida
to mitigate concerns with sensitivity. Oral health professionals need to note
that many products used in dentistry may contain latex, including, but not
limited to adhesive bandages; blood pressure cuffs; personal protective
equipment, such as gloves and masks; nitrous oxide reservoir bag; orthodontic
bands/elastics; rubber cup polish; rubber dams; and saliva ejectors.18
An adequate supply of nonlatex alternatives for commonly used armamentarium
should be maintained. Early morning clinical appointments are ideal for
patients with latex sensitivity to minimize aerosol exposure.18
Patients with special health care needs, like
spina bifida, are a particularly vulnerable and underserved population.19
Oral health professionals are valuable members of an interdisciplinary health
care team dedicated to improving the overall health of these patients. Spina
bifida is a complex neural tube defect with multiple associated impairments and
considerations. Proactive oral health professionals need to ensure their teams
are educated and clinically adaptive to the special health care needs of
patients with spina bifida.
- Fletcher JM, Brei TJ. Introduction: spina bifida–a multidisciplinary
perspective. Dev Disabil Res Rev. 2010;16:1–5.
- Medline Plus. Neural Tube Defects. Available at:
nlm.nih.gov/medlineplus/neuraltubedefects.html. December 28, 2016.
- National Institute of Neurological Disorders and Stroke. Spina Bifida
Fact Sheet. Available at: ninds.nih.gov/disorders/spina_bifida/detail_spina_bifida.htm.
Accessed December 27, 2016.
- Centers for Disease Control and Prevention. Spina Bifida. Available
at: cdc.gov/ncbddd/ spinabifida/data.html. Accessed December 27, 2016.
- Ouyang L, Grosse S, Armour B, Waitzman N. Health care expenditures of
children and adults with spina bifida in a privately insured U.S. population. Birth
Defects Res A Clin Mol Teratol. 2007;79:552–528.
- Spina Bifida Association. Overview. Available at:
spinabifidaassociation.org. Accessed December 27, 2016.
- Recommendations for the Use of Folic Acid to Reduce the Number of
Cases of Spina Bifida and Other Neural Tube Defects. MMWR Recomm Rep.
- Folic acid for the prevention of neural tube defects. American Academy
of Pediatrics. Committee on Genetics. Pediatrics. 1999;104:325–327.
- US National Library of Medicine. MTHFR Gene. Available at:
ghr.nlm.nih.gov/gene/MTHFR. Accessed December 27, 2016.
- Kabani F, Anderson M. Treating children with spina bifida. Dimensions
of Dental Hygiene. 2012;10(4):52–57.
- March of Dimes. Spina Bifida. Available at:
marchofdimes.org/baby/spina-bifida.aspx#. Accessed December 27, 2016.
- Mayo Clinic. Diseases and Conditions Spina Bifida. Available at:
Accessed December 27, 2016.
- American Academy on Pediatric Dentistry Clinical Affairs Committee.;
American Academy on Pediatric Dentistry Council on Clinical Affairs. Guideline
on antibiotic prophylaxis for dental patients at risk for infection. Pediatr
Dent. 2008-2009;30(7 Suppl):215–218.
- Kabani F. Special care for special needs populations: spina bifida.
Presented at Collin County Dental Hygiene Society; October 24, 2015; McKinney,
- McGuire S. Presentation and Management of Patients at Texas Scottish
Rite Hospital for Children: Spina Bifida. Presented at Special Care Dentistry
Conference; April 20, 2013; New Orleans.
- American Academy of Allergy, Asthma, & Immunology. Latex Allergy.
Available at: aaaai.org/conditions-and-treatments/allergies/latex-allergy.aspx.
Accessed December 27, 2016.
- American Latex Allergy Association. AANA Latex Protocol. Available
at: latexallergyresources.org/ articles/aana-latex-protocol. Accessed December
- Spina Bifida Association. Latex in the Hospital Environment.
Available at: spinabifidaassociation. org/project/latex-hospital. Accessed
December 27, 2016.
- Lo A, Polsek D, Sidhu S. Estimating the burden of neural tube defects
in low-and middle-income countries. J Glob Health. 2014;4:010402.
From Dimensions of Dental Hygiene. January 2017;15(1):48-51.