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Integrating the Spina Bifida Patient

Into the General Dental Practice

JoAnn Campoli Scofield, RDH, BAAS
Patricia Regener Campbell, RDH, MS

Neural tube defects, including spina bifida, affect one out children and adults with SB.4 This article is intended to
of every 1,000 newborns each year. Due to advances in educate the dental health professional to the special needs
medical technology, the life expectancy of these patients of the SB patient, in order to integrate this population into
is rising annually, and the dental community should be pre- the general dental practice.
pared to treat them. This article discusses how, with proper
precautions and a little effort, patients with spina bifida can Types of Spina Bifida
be treated in the general dental practice. Among SB patients, 1% of the vertebral defects occur in the
cervical region, 1% in the thoracic region, and 98% between
Introduction the lumbar and sacral vertebrae.5 There are also differences
The term neural tube defect (NTD) refers to any one of a in the severity of the condition (Figure 1). Spina bifida occulta
group of malformations involving defects of the skull and/or is a bony defect in which one or more of the vertebrae
spinal cord that occur during embryonic development.1 fails to fully develop, but the skin is left intact. Detection
Neural tube defects affect one out of every 1,000 American of this type of defect requires radiographic examination.2,6
newborns each year.1 Spina bifida (SB) is a type of NTD Meningocele is a more severe type of defect, where
in which the neural tube and posterior vertebrae do not the meninges and cerebral spinal fluid protrude into the
completely close during the first month in utero. Prior to opening of the spinal canal, forming an external sac filled
the advent of antibiotics and the advances in surgery in the with cerebrospinal fluid. The sac is covered with skin, and
past several decades, the life expectancy for individuals nerve tissue is not involved.2,6
with SB was brief, but currently, 85% to 90% of children The most severe form of SB, myelomeningocele (MSB),
born with the most severe form of SB survive into adult- is the one to which people most commonly refer. This
hood.2,3 In the US alone, there are an estimated 40,000 defect occurs when both the meninges and the spinal cord
protrude into an external sac (Figures 1 and 2). The sac con-
tains portions of the spinal cord, cord membranes, and spinal
JoAnn Campoli Scofield, RDH, BAAS, is a graduate dental fluid without benefit of skin coverage.2,5 Neuromuscular
hygiene student at the Caruth School of Dental Hygiene at function is affected below the level of the lesion, which is
Baylor College of Dentistry, a member of the Texas A&M indicated by a decrease or absence of sensation (eg, pain,
University Health Science Center, Dallas, Texas. She is also Pro- pressure, friction, temperature) in the lower body.5
fessor of Dental Hygiene at Collin County Community College, The cause of SB is unknown, although environmen-
McKinney, Texas and has been a registered dental hygienist tal factors (eg, infections, drugs, repeated x-ray exposure)
for more than 25 years. and genetics have been implicated.7 Folic acid (0.4 mg per
Patricia Regener Campbell, RDH, MS, is an Associate Professor day) and multivitamins taken by the mother before and
and Clinical Coordinator at the Caruth School of Dental during pregnancy, as well as foods rich in foliates (eg, dark
Hygiene at Baylor College of Dentistry, a member of the Texas green leafy vegetables, orange juice, dried peas, beans,
A&M University Health Science Center, Dallas, Texas. She has lentils) have been shown to reduce NTD.1-3,7,8 The Centers
been a practicing hygienist for more than 25 years and has pub- for Disease Control and Prevention (CDC) estimate that
lished several articles on patient assessment. women taking the recommended dosage of folic acid

May/June 2001 27
The Spina Bifida Patient

in Bone

Normal Spine Spina Bifida Occulta With Meningocele With Myelomeningocele

Figure 1. A comparison of a normal spine to the types of spina bifida (differing in severity).

during the childbearing years and early in pregnancy can short attention span, visual perception problems, sensory
prevent up to 3,000 serious birth defects annually.8 processing difficulties, decreased arm and hand function,
poor eye-hand coordination, and memory deficits, are asso-
Medical Complications ciated with MSB and are complicated by hydrocephalus.
Children born with MSB have a 90% chance of also hav- A condition common to all children with MSB is
ing hydrocephalus, which is characterized by an accumu- Arnold-Chiari malformation, a congenital structural defect
lation of cerebrospinal fluid in the head that puts increased where the brainstem is pushed into the cervical spine due
pressure on the brain. Causes may include developmen- to pressure from increased cerebrospinal fluid in the fourth
tal anomalies, infection, trauma, or brain tumors.9 Surgical ventricle. Common symptoms include headache, scoliosis,
placement of a shunt helps to relieve the pressure by redi- difficulty with balance and coordination, low muscle tone,
recting the fluid buildup to the abdominal area.2,5,7 The voice alteration, decreased gag reflex, and respiratory diffi-
shunt is inserted subcutaneously behind the ear, runs down culties.5,12 Other complications include neurogenic bladder,
the side of the neck, and ends in the abdomen, where the neurogenic bowel, and kyphosis (hunchback).2,7,13 Only
excess spinal fluid is reabsorbed. In many instances, the 9% to 21% of those affected will need treatment for this
shunt needs to be revised or replaced as the child matures.5 condition,7,12 which involves the reduction of the pressure
There has been some disagreement about adverse against the brainstem and spinal cord through surgical
effects associated with shunts. According to Jansen et al, decompression, involving removal of small portions of bone.13
there is a correlation between hydrocephalus patients with
shunts and poor hand function.10 In contrast, a study by Nutrition and Growth Complications
Muen et al reports that poor fine motor skills may be a A combination of poor self-esteem, dependence on family
result of cerebellum abnormalities, and the lack of strength for care, and fears of maturity and independence put adoles-
in the small muscles of the hand may be due to cervical cent MSB patients at risk for eating disorders (eg, anorexia).14
cord abnormalities.11 Eating disorders may also be precipitated by caretakers
While most children with MSB are not mentally who urge the patient to lose weight.2,14 In addition, 50%
retarded, complications such as hydrocephalus or infections to 60% of children with MSB exhibit a disturbed growth
can lead to delays in learning.2,7 Dysfunctions, for example, pattern that may be a result of growth hormone deficiency.15

28 The Journal of Practical Hygiene


Dermatological Disorders
It has been reported that children with SB are 500 times
more likely to have a reaction to latex than the general
population.15 Other studies indicate 28% to 60% are affected
to varying degrees.16,17 Symptoms may include urticaria
(hives), conjunctivitis, and anaphylaxis.16 While some stud-
ies indicate that early and repeated exposure to latex from
multiple surgeries can cause sensitivity,2,8,17-20 others indi-
cate that the number of surgeries may not be the primary
cause. A high association between latex sensitivity and
foods such as avocados, bananas, and chestnuts has been
reported,16,20 indicating that various foods are risk factors
Figure 2. Patient placed in an oxygen tent at birth to to SB patients.16,20 A study by Szépfalusi et al suggests the
address respiratory problems. The presence of myelo-
meningocele is evident. propensity for latex sensitization by the SB population may
be caused by genetics.19
Body positioning and assistive devices (eg, wheel-
chairs and leg braces) may create pressure and cause irri-
tation to the skin. Due to the lack of sensation in the lower
body, blisters and bedsores may go unnoticed by the
patient. Skin infections also pose a potential danger to the
spinal cord, particularly in the MSB patient,2,7 therefore,
strict infection-control procedures are crucial. Bacteria do
not have to travel far to infiltrate the nerves of the spinal
column and cause irreparable harm. Efforts should be made
to reduce the potential of irritation to the gibbus (ie, hump)
when seating a SB patient in the dental chair (Figures 3
and 4). Pillows, a styrofoam ring (ie, donut), a mattress
Figure 3. Back view of the patient. pad, or a body-size beanbag can be used to protect the
gibbus and provide comfort for the patient. The patient
should be allowed to shift his or her weight every 20 min-
utes to maintain circulation and prevent pressure sores.
Common Items Containing Patient Management
Natural Rubber Latex
All female patients of childbearing age can benefit from
Latex gloves Shoes nutritional counseling regarding the use of multivitamins.
Elastic bandages Dental rubber dam Neural tube defects occur soon after conception, usually
Face mask with Dental bite block before a woman realizes she is pregnant, so the nutritional
elastic band Fingercot status of a woman at the time of conception is critical. As
Medication stopper Penrose drain reported by Butterworth and Bendich, 60% of women who
Anesthetic carpule Orthodontic elastics took multivitamins for three months prior to conception and
with rubber plunger
Wheelchair tires three months after conception were less likely to have
Disposable rubber cup
Elastic ligature thread infants with NTDs than those women who did not take a
prophylaxis angles
Induction mask supplement during the same time period.1
Blood pressure
cuff tubing Window insulation Problems with obesity and eating disorders among
Stethoscope tubing Underwear elastic SB patients make nutritional counseling a necessity.14 The
Adhesive Stamps dental hygienist can deliver such information in a non-
threatening manner, which can serve as reinforcement for
Rubber bands
existing knowledge.

May/June 2001 29
The Spina Bifida Patient

Since patients with SB are considered at high risk for

latex allergy, they should all be treated in a latex-free
environment (Table). Powdered latex gloves have been
reported to increase the latex aeroallergen level by as much
as 115 times in areas where latex gloves are used, causing
a reaction in sensitive patients.16 Nelson et al reported a case
of anaphylactic shock by a dental patient, possibly caused
by radiograph packets contaminated with powder from
latex gloves.21

Radiation Exposure
As diagnostic radiographs of the spine, neck, and head are
important tools in long-term management, SB patients are
exposed to high amounts of radiation over time. Gaskill
and Marlin reported that excessive radiation exposure may Figure 4. Side view of the patient. Note the gibbus,
for which accommodation must be made so the patient
be the reason patients with SB are at increased risk to will be comfortable.
develop bladder and rectal carcinomas.4 Definitive conclu-
sions, however, regarding cumulative, long-term radiation The patient used a specially designed wheelchair with
exposure cannot be drawn at this time for the SB popula- support straps and steadied himself with his left hand on
tion. As additional SB patients grow into adulthood, more a stable surface when not strapped in the wheelchair or
precise conclusions may be determined. Based on current otherwise secured. To avoid pressure sores on the skin, the
knowledge, radiographs should be taken only if they will back of the chair had a cushion with an opening to allow
improve the ability of medical/dental professionals to care for the gibbus. The child’s mother reported that ulcerations
for the patient.4 occurred on the gibbus approximately three to four times
per year.
Case Report The patient brushed his teeth once or twice daily with
Patient History a soft-bristled toothbrush utilizing fluoridated toothpaste.
A seven-year-old white male patient presented for a rou- No other oral hygiene products were employed. His mother
tine oral prophylaxis. His medical history included myelo- reported that she occasionally brushed his teeth as a follow-
meningocele affecting the spine at T8, with kyphosis, up. The patient did not report any oral complications
hydrocephalus, and Arnold-Chiari malformation. The patient (eg, dry mouth, aphthous ulcers, sensitivity).
was born by cesarean section and had accompanying res-
piratory complications. Spina bifida correction surgery was Treatment
performed at the age of one week and again at two weeks. One hour before the scheduled procedure, the patient
A ventriculoperitoneal shunt was placed to reduce the pres- took 12 ccs of 250 mg/5 mL oral suspension amoxicillin
sure caused by the hydrocephalus. The shunt was exam- trihydrate. A beanbag pillow was placed on the dental chair
ined annually by a neurosurgeon and had not been replaced for the comfort of the patient (Figure 5), and he was trans-
or presented complications. Although both hips are dis- ferred from his wheelchair by his mother.
located, the parents and physician have decided not to A resident pediatric dentist performed an oral exam-
correct this condition since the patient will never be ambu- ination, and a dental assistant recorded the findings. The
latory. Decompression surgery was performed at the age oral examination revealed a Class I amalgam restoration
of two for the Arnold-Chiari malformation. Although the on T. A carious lesion was discovered on J. The patient
patient exhibits a neurogenic bladder and bowel, the child’s exhibited a Class I occlusal relationship on the right side
mother reported a history of only two urinary tract infec- and a Class II occlusal relationship on the left side. The
tions. The patient appeared to have difficulties with visual mother reported that she had a similar occlusal relation-
perception despite corrective lenses. When reading, he ship. There was a lack of interproximal spacing in the pri-
tilted his head to one side to see properly. The patient mary dentition. Due to the slow growth rate of the patient,
took 2 mL per day of senna, an over-the-counter peristaltic there was a possibility of crowding of the permanent den-
agent to prevent constipation. tition. The maxillary left buccal mucosa appeared slightly

30 The Journal of Practical Hygiene


SB patient in any general dental practice. Latex-avoidance

precautions, wheelchair access, limited radiation exposure,
and accommodations for comfort in the dental chair are
further examples of ways to integrate SB patients with safety
and comfort. Placing a beanbag pillow or an egg crate
mattress pad will provide additional comfort for patients
with back problems as well as the SB patient. Placing a
hand on the shoulder of the SB patient as the dental chair
is reclining will give the patient a feeling of stability. An alter-
native is to leave the patient in the wheelchair during the
procedure. Verbal as well as written self-care instructions
are also extremely beneficial, due to the patient’s visual and
perceptual difficulties. These simple procedures can easily
be adapted within the dental practice and can provide com-
Figure 5. A beanbag pillow, covered with a clean white fortable accommodations for physically challenged patients.
sheet, is placed under the patient during treatment.
The authors would like to thank Michael Webb, DDS, Chil-
erythematous. The mother mentioned that the patient
dren’s Medical Center, Dallas, TX, for his review of this article.
had a tendency to hold food in the left side of his mouth
before swallowing. References
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defects. Annu Rev Nutr 1996;16:73-97.
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1997;22(9):60-62, 65-72.
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May/June 2001 31