NEURAL TUBE DEFECTS PROGNOSIS OUTCOME
Category: Child Health
Abstract : OUTCOME AND PROGNOSIS OF CHILDREN WITH NEURAL TUBE DEFECTS Major issues in
evaluating the outcome of children with myelomeningocele are hydrocephalus,
intellect, ambulation, continence, orthopedic problems, and employment and
independent living status Treatment of NTDs in neonates has evolved over the
past half century. Historically, there was a period when neonates with NTDs were
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OUTCOME AND PROGNOSIS OF CHILDREN WITH NEURAL TUBE DEFECTS
Major issues in
evaluating the outcome of children with myelomeningocele are hydrocephalus,
intellect, ambulation, continence, orthopedic problems, and employment and
independent living status Treatment of NTDs in neonates has evolved over the
past half century. Historically, there was a period when neonates with NTDs were
either left untreated or selectively treated.
The natural history of neonates
with NTDs left untreated is poor. Most died of meningitis, hydrocephalus, and
sepsis. Laurence described a cohort of 290 children with spina bifida (mostly
myelomeningoceles) left untreated in Wales during the 1950s and 1960s. Only 11%
of those children lived past the first decade of life. Lorber and Salfield
reported their results with selected treatment of neonates with
myelomeningocele. More than 80% of the selected neonates lived, whereas 97% of
the neonates denied treatment died in the first year of life. The tremendous
ethical implications of selected neonatal treatment led to its
abandonment.
In the United States during the 1960s, most children with
myelomeningocele were treated, which resulted in a higher survival rate (>80%
for the first decade) than that in Great Britain. Recognized causes of death
include shunt malfunctions, seizures, infections, and uncontrolled brainstem
symptoms from CM II and/or hydrocephalus. During the past 3 decades, aggressive
treatment of neonates with myelomeningocele has been pursued in almost all
pediatric centers in the United States.
Intellect
Cognitive ability
is, in part, influenced by hydrocephalus, CNS infections, and degree of
impairment. In most series, 60-70% of the children with myelomeningocele had
intelligence quotients (IQs) greater than 80; the others had IQs in the delayed
or severely delayed range. In the McLone series, children who had CNS infections
such as ventriculitis, or shunt infections fared worse than those who did not.
Children with myelomeningocele without hydrocephalus had an average IQ of 102;
those with hydrocephalus had an average IQ of 95. However, the average IQ
dropped to 73 when a CNS infection complicated the picture. Children with
moderate physical impairments, in most series, have a better intellectual
outcome than those with significant sensory levels and paraplegia. The reasons
most likely are multifactorial.
Continence
Only 10-15% of all children
with myelomeningoceles are continent of urine. This issue often causes the
children to be separated from their peers, which, in turn, leads to other
neuropsychologic deficits. Despite the development of catheters and Crede
manipulation (pushing on the pelvis over the bladder to engender urination),
children with NTDs still experience a high rate of infections, vesicoureteral
reflux, kidney failure, hydronephrosis, and obstruction. Clean intermittent
catheterization (CIC) has led to a marked improvement of the lifestyles and
lifespan of these children. CIC can make more than 75% of these children
socially continent and significantly decreases the rate of urosepsis. As a
result of CIC, urinary diversions are less commonly performed. Use of
anticholinergic drugs combined with CIC has resulted in a better self-image and
greater educational and vocational opportunities for children with NTDs. Bowel
continence is achieved with a combination of medication, diet control, manual
disimpaction, and enemas. Most patients with NTDs can be continent of stool with
these measures.
Ambulation
The ability to ambulate is influenced by
the level of the neural lesion, hydrocephalus, pelvic anatomy, limb deformities,
tethered cord, scoliosis, kyphosis, and syringomyelia, and varying degrees of
ambulation exist. Strong hip flexors, adductors, and quadriceps are required to
be ambulatory. Some children can ambulate in the community, some only in the
home, others can only stand but not walk, and the rest are wheelchair bound.
However, many children with NTDs, such as lumbar myelomeningocele, lose their
ability to ambulate as they get older. In general, patients with a sacral lesion
can ambulate, those with a thoracic lesion cannot.
Independent living,
vocation, education
Steinbok noted that about 60% of children with NTDs
attended normal classes, while 40% were in special classes or operated below
their grade level. Approximately 10-40% of children with myelomeningocele are
probably employable at some level, depending on the patient's intellectual
abilities, ambulation status, and environmental influences.
Latex
allergies
Over the past 2 decades, allergy to latex has been recognized in an
increasing number of children with myelomeningocele. Up to 50% of children with
myelomeningocele may be latex sensitive. This appears to be a result of a
massive immunoglobulin E (IgE) response to the antigen in latex that is derived
from the Heva brasiliensis plant. Most patients with myelomeningocele should be
treated with latex precautions when undergoing surgery. Surgeons and health care
providers should work with latex-free gloves and plastics so that they can avoid
latex-induced anaphylaxis, which can be life threatening. Medications such as
corticosteroids, Benadryl, bronchodilators, and epinephrine should be available
as a precaution during surgery on these children.
Late
complications
Neurosurgeons need to be wary of late-life neurologic
deterioration in children and adults. The most common deterioration seen is from
a tethered spinal cord. A routine MRI reveals a spinal cord that ends in the
lumbar or sacral regions in almost all patients with myelomeningocele. This is
normal in many patients without any new neurologic complaints. Despite careful
surgical closure of the original neural placode, approximately 20% or more of
all patients with myelomeningocele require an untethering of their spinal cord
later in their life. They may present with gait difficulty, back pain, leg
weakness, sensory loss, a new foot deformity, or simply a change in their
urodynamic data or urinary continence. These patients require a surgical
exploration to free the neural placode and nerve roots from the dorsal surface
of their dura. Patients with tethered cords on MRI but no new complaints do not
require reexploration.
Diastematomyelia can be diagnosed using MRI or
CT/myelogram. An enlarging syringomyelia can be the result of a symptomatic CM
II or retethering of the spinal cord. Many functional deteriorations result from
progressive orthopedic deformities such as scoliosis, pelvic obliquity, and limb
deformities. An orthopedic surgeon well versed in the care of patients with NTDs
is required to execute a reasonable plan to repair or stabilize treatable
disorders. In general, a multidisciplinary team consisting of neonatologist,
pediatrician, pediatric neurosurgeon, pediatric urologist, pediatric orthopedic
surgeon, physical therapist, nurse, nutritionist, psychologist, and teacher are
required to direct the care of children with NTDs.
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