The Journal of the Arkansas Medical Society Med Journal Jan 2020 | Page 13

AFMC: A CLOSER LOOK AT QUALIT Y
discharge. Refer the family to their
states’ Early Intervention program (in
Arkansas — First Connections https://
dhs.arkansas.gov/dds/firstconnec-
tionsweb/#fc-home). This is vital in
getting therapy and teaching under-
way. Some families may use outpa-
tient therapy and/or a daycare that
emphasizes developmental therapy.
Children often have associated
intracranial abnormalities. Most will
have hydrocephalus although the
rates are lower with intrauterine
repairs. Hydrocephalus may require
surgical intervention, typically a
shunt that drains into the abdomen
(VP shunt). Programmable shunts
require periodic recalibration by the
neurosurgical team (particularly after
MRIs). Approximately 80–90% of chil-
dren with spina bifida will also have
Chiari II malformation. This hindbrain
herniation can be severe enough
to require surgical decompression.
Signs of herniation in need of neu-
rosurgical evaluation include poor
suck, swallow dysfunction, difficulty
breathing and speech problems. 7
As children grow, the spinal cord
may develop complications including
tethered cord or syringomyelia. Both
complications can manifest with
changes in bowel or bladder function,
loss of motor or sensory control, back
pain and progressive orthopaedic
deformities. These complications
should always be managed by spe-
cialists familiar with NTDs. 8
Children with spina bifida his-
torically had early mortality related
to urologic complications, urinary
tract infections (UTIs) or renal failure.
The use of surgical and nonsurgical
techniques (including catherization)
to regularly empty the bladder and
bowel has improved this. Functional
continence has been linked with
future income-earning potential and
improved quality of life. Because of
the methods used to maintain func-
tional bladder continence, the blad-
der may be colonized with bacteria.
Therefore, UTIs should not be treated
unless there are symptoms (fever,
increased leaking, etc.). Cultures
should always be collected as part
of urinalysis. Spina bifida patients
should be followed by a urologist
throughout their life. 7
Given the abnormal spinal
structure and innervation present,
orthopaedic concerns are frequent
and may include scoliosis, clubfoot
deformity and hip dysplasia. X-rays
and gait studies can guide treatment
plans. There are numerous equip-
ment needs to consider; conse-
quences of inappropriate devices can
be disastrous. A physical medicine
and rehabilitation specialist can help
with selection of appropriate bracing,
adaptive devices or wheelchair while
considering comorbid neurosurgical,
orthopaedic, developmental and
urologic factors. Standardized serial
exams (including PT, OT and neuro-
psychology) should be performed
regularly to determine if there are
clinically significant changes.
Children with spina bifida are
vulnerable to skin disorders, pressure
sores and latex allergy. Avoid latex
products and pressure over bony
prominences. Sunscreen and insect
repellant should be worn while
outdoors. Certain antibiotics used to
treat UTIs may make sunburns worse.
Medical conditions associated
with spina bifida include precocious
puberty, short stature, diabetes,
obesity and osteoporosis. A nephrol-
ogist may be involved if hyperten-
sion or kidney damage is related
to neurogenic bladder. Central and
obstructive apnea with poor sleep
hygiene contribute to sleep disor-
ders that are present in more than
60% of patients. This may require
collaboration with psychology,
pulmonology and otolaryngology.
Seizure disorders are more com-
mon and can be life threatening.
Children with spina bifida score
lower in assessments of social
functioning, attention and executive
function. They show lower levels of
participation in social and physical
activities. Participation in group
activities that specifically address
these deficits (such as Spina Bifida
Camp) can improve adaptive scores
and quality of life. s
Dr. Hobart-Porter is assistant professor
and medical director, Spinal Cord
Disorders Program and Concussion Clinic,
UAMS and Arkansas Children’s Hospital.
REFERENCES
1. CDC. 8-30-02. www.cdc.gov. Retrieved
9-30-19
2. Spina Bifida Assoc. 11-30-18. www.spinab-
ifidaassociation.org/guidelines, Retrieved
9-30-19
3. Ziring P. (1999). Care Coordination: Inte-
grating Health and Related Systems of Care
for Children with Special Healthcare Needs.
Pediatrics, 104 (4), 978-981
4. American Academy of Pediatrics. (1999).
Policy Statement. Pediatrics, 103 (3), 686-693
5. McLone D, Knepper P. (1989). The cause
of Chiari II malformation: a unified theory.
Pediatr Neurosci, 15 (1), 1-
6. Copp A, Stanier P, Greene N. (2013). Neural
tube defects: recent advances, unsolved
questions, and controversies. Lancet Neurol,
12 (8), 799-81
7. Alexander M, Matthews D. (2015). Pediatric
Rehabilitation: Principles and Practice. New
York, NY: Demos Medical
8. Bowman R, Mohan A, Ito J, et.al. (2009).
Tethered cord release: a long-term study in
114 patients. J Neurosurg Pediatr, 3, 181-187
AFMC WORKS COLLABORATIVELY WITH PROVIDERS,
COMMUNITY GROUPS AND OTHER STAKEHOLDERS TO
PROMOTE THE QUALITY OF CARE IN ARKANSAS THROUGH
EDUCATION AND EVALUATION. FOR MORE INFORMATION
ABOUT AFMC QUALITY IMPROVEMENT PROJECTS,
CALL 1-877-375-5700 OR VISIT AFMC.ORG.
JANUARY 2020
Volume 116 • Number 7
January 2020 • 161