Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438

Difficult questions facing the craniofacial team

Derek A. Bruce, MB, ChBa,b,*
a
Center for Neuroscience and Behavioral Medicine, Pediatric Neurosurgery, Children’s National Medical Center,
111 Michigan Avenue NW, Washington, DC 20010-2970, USA
b
Department of Neurosurgery and Pediatrics, George Washington University, Washington, DC, USA

Many factors influence where and how infants
and children with craniosynostosis and craniofacial
syndromes are treated. This article attempts to iden-
tify these factors, establish how the decisions con-
cerning treatment are made, what support is available
for these decisions, and whether the best decisions
are being made for the children involved.
The major diagnostic criteria are cranial and facial
appearance. Cranial shape varies with race and
geography, and what is an acceptable shape of the
cranium is often a social decision. For thousands of
years, many different human societies have sought to
distort the shape of the cranium to enhance beauty,
social status, or perceived aspects of character
(eg, honesty) [1]. Some of these desired shapes from
the past we currently perceive as unacceptable when
they occur naturally. Surgery is applied to bnormalizeQ
the shape of the cranium because, in current societal
terms, the deformities are the result of a pathologic
process that is associated with premature cranio-
synostosis. It must be realized that the social accept-
ability of cranial shape depends on the social setting
in which it occurs.
Craniosynostosis can affect more than just the
shape of the head. There is good evidence that at least
in infants and children with multiple suture synostosis,
raised intracranial pressure can occur with resultant
blindness. Whether there are deleterious effects of
pressure on other aspects of brain function in children
with craniofacial syndromes is less clear and is
* Center for Neuroscience and Behavioral Medicine,
Pediatric Neurosurgery, Children’s National Medical Center,
111 Michigan Avenue NW, Washington, DC 20010-2970.
E-mail address: derfran@comcast.net
discussed. Minimal evidence exists that single suture
synostosis can or has produced brain dysfunction,
although sagittal synostosis can present in late child-
hood with papilledema and visual loss, because
usually many sutures are fused by this time. The
major issues to be addressed are (1) why we operate on
single or multiple suture synostosis, (2) what the de-
sired outcomes are, (3) what operations best approx-
imate the desired goals, (4) where these operations are
performed most safely and successfully, (5) the best
time for the surgery, (6) how we measure the outcome,
and (7) what major factors affect that outcome. Have
we convincingly established the case for surgery
versus no surgery in some or all cases of cranio-
synostosis, and can surgery alone be considered
adequate therapy for the child and family?
Craniosynostosis has been recognized since the
time of Vesalius in 1543. Since the late nineteenth
century, the concept of fused sutures causing raised
intracranial pressure has been acknowledged, and
surgery has been recommended to permit increased
room for the brain. The earliest reports of surgery
involved children with microcephaly. The mortality
rate was high (14 of 33 children) and the improve-
ment rate was low (2 of 33 children). It is likely that
most of these children had primary microcephaly and
that the surgery was not indicated. Presumably the
surgeons who operated had good intentions but at that
time did not have adequate information or experience
to recognize true craniosynostosis.
The mistaken surgical treatment of abnormal head
shape is not relegated to the past. During the late
1980s and the early 1990s, there was a substantial
increase in the amount of cranial reconstructive
surgery being performed in Denver, Colorado. An in-
quiry undertaken by the Centers for Disease Control

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430 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
concluded that an epidemic of craniosynostosis was
occurring. Children with true craniosynostosis and
hundreds of infants were operated on for irritability
that was wrongly diagnosed as being caused by
cranial synostosis and a small frontal fossa. Deaths
occurred and there was significant morbidity in these
children, many of whom were misdiagnosed as having
craniosynostosis. Was this another case of well-
intentioned surgeons being biased by their own beliefs
and seeing a new subtle form of craniosynostosis that
did not have radiographic proof of suture closure or—
in some cases—any cosmetic skull abnormality? Was
there a financial incentive for all involved—hospital
included—that prevented the appropriate review of
these cases and permitted the unnecessary surgery to
continue for years? Several factors clearly contrib-
uted, including parental acceptance of the need for
surgery. Many of the children were seen by other
pediatric neurosurgeons and craniofacial surgeons
who told the families that surgery was unnecessary,
yet many families returned to Denver for their
children to undergo a surgical procedure. They were
convinced by the information that brain damage could
occur if surgery was not performed. Was there any
hard evidence on which the threats of possible brain
damage were based? Why did the surgeons who saw
the children and believed that they did not have
craniosynostosis fail to convince the parents that their
children were normal? Were they less zealous in their
delivery of the information than the individuals who
promoted surgery? These cases leave unanswered
questions for all of us involved in cranial and cra-
niofacial surgery.
As a result of the bback to sleepQ campaign, in the
early 1990s there was a dramatic increase in the
incidence of posterior skull flattening and associated
deformity of the ipsilateral forehead and ear in infants
[2 – 5]. In many pediatric centers these children un-
derwent operation for bimpendingQ suture closure.
The incidence of true lambdoid synostosis is 1% to
3% of all synostosis. In one children’s hospital, the
number of posterior operations went from 3 per year
to 200 per year as a result of the increase in positional
plagiocephaly. When the entity was recognized as not
being caused by synostosis, the number of posterior
cases returned to two to three per year. The radiologic
and CT studies of the children with posterior
plagiocephaly were read as showing bsticky sutures,Q
bimpending closure,Q or bpartial synostosisQ in many
hospitals. Was this an unconscious effort to support
the surgical opinion, a true lack of knowledge, or an
inability to escape the established paradigm? Most of
these children had unfused sutures and did not and do
not need a surgical operation. The rush to surgical
therapy was predicated on the false belief that an
abnormal skull shape had to be the result of a sutural
abnormality, because that was the paradigm of the
time. What other incentives were at play? What was
the economic incentive, and what role did it play in
the decision to operate on these children? We
currently know that over the first year of life, many
of these children have spontaneous correction of
posterior plagiocephaly. Most of the others in whom
the deformity is more marked can be treated
satisfactorily with a molding helmet, and complex
and dangerous surgery almost always can be avoided.
Why was a diagnosis of craniosynostosis, which led
to multiple unnecessary surgeries, made in some
centers and not in others? If a multidisciplinary team
rather than the neurosurgeons or craniofacial sur-
geons had evaluated these children, would the num-
ber who underwent surgery have been different? My
impression is that most of the cases of posterior
plagiocephaly were handled by neurosurgeons alone
rather than by craniofacial teams. These episodes from
the last 15 years highlight the potential dangers of
advocating surgery for correction of an abnormal
head shape without really thinking about the actual
cause of the deformity of the skull.
Through the twentieth century, strip craniotomy
was popularized for the treatment of cranial defor-
mities and the relief of raised intracranial pressure in
children with premature craniosynostosis [6]. This
surgical approach also was used for children with
craniofacial syndromes, although it became apparent
that freeing of the sutures of the skull did not improve
the growth of the face. Tessier’s 1967 publication of a
technique for repair of hypertelorism established the
possibility of performing facial osteotomies with
good results [7]. His later paper on midface advance-
ment in Apert and Crouzon syndromes established
the era of craniofacial surgery [8]. He also proposed
and championed the need for a multidisciplinary
team to oversee and deliver care to the children with
craniofacial syndromes. In 1977, there were fewer
than 12 craniofacial centers and teams in North
America; by 2003, the American Cleft Palate Cra-
niofacial Association directory listed 148 centers, 114
of which had performed intracranial surgical proce-
dures that year. The reported number of intracranial
procedures was 2711. This figure does not include
procedures performed outside these centers. We are
discussing potentially life-threatening surgery on at
least 3000 children a year in the United States alone.
It is appropriate to examine the basis of support for
this surgery and question whether the results are
acceptable. It is also reasonable to evaluate how
outcome has been measured and whether it could be
 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
measured better and highlight which of the aspects of
the care of the child and family have the greatest
impact on the life the children lead as they mature and
take their places in the larger society.
The population of children who have craniofacial
or cranial anomalies covers a broad societal band, and
the professional teams who deal with these children
and their parents face many complex decisions. Cost
and reimbursement are a factor in obtaining care. The
ability to deliver free care is severely limited, but all
centers deliver some free care. The reimbursement
from governmental and insurance agencies has de-
creased, and it is a constant fight to obtain adequate
reimbursement for a team’s time and effort to care for
the children. It is impossible to ignore the economic
aspects of craniofacial care, but they must not be
allowed to become a limiting factor in the equation
for delivering care. Many children with craniofacial
syndromes end up on state or federal aid programs,
which plays a role in where they ultimately receive
their care. To perform an initial operation when a
child has insurance coverage and then refer the child
to a craniofacial team when the financial compensa-
tion has been used up never should be acceptable
behavior. This behavior implies that the financial
reward and ego override the clinical knowledge that a
surgeon has about a patient and his or her disease! If a
surgeon is not able to deliver full comprehensive care
to the child and family, he or she should not begin a
process that he or she cannot complete.
The need for a multidisciplinary approach to care
appears less for children with single suture synos-
tosis, and in some places these children are consid-
ered separately from syndromic children. We are
becoming increasingly aware that there are functional
problems in children with single suture synostosis
and that they may be better taken care of with a team
approach [9].
The largest step forward in our understanding of
diseases associated with craniosynostosis has been
the identification of genetic abnormalities that relate
directly to the molecular biology of growth and de-
velopment. In most of the syndromic cases (eg, Apert,
Crouzon, Jackson-Weis, Pfeifer’s, Saethre-Chotzen),
the abnormality involves the fibroblastic growth
factor receptors or closely related genes [10 – 19]. In
as yet a small percentage of cases of single suture
synostosis, abnormal genes have been identified
[20 – 25]. This genetic information has caused a
perceptual leap in our understanding of the patho-
physiology underlying this group of diseases. The
simple concept that a suture closes and the resultant
deformity is caused by the resulting growth limitation
is no longer tenable. It seems more likely that because
431
of the growth failure there is no longer stress on the
suture and thus it fuses. This genetic information also
allows us to envision that the involved areas have a
congenital growth limitation and that opening a
suture cannot resolve all future growth problems.
After a forehead expanding operation, the bones tend
to stay where they were put rather than grow as
normal tissue in an uninvolved area. These concepts
are particularly true of the syndromic entities, but it is
likely that most cases of synostosis will be shown to
be caused by a primary growth defect and that sim-
ple suturectomy will be shown to be an inadequate
therapy. Currently, surgeons who perform strip
craniectomies and predominantly use the endoscope
as a lighted retractor do not rely on the bone resection
but use some form of head shaping band to modify
future growth. As a larger database of patients is
obtained and DNA screening becomes more readily
available, it is hoped that the next few years will
provide greater insight into the frequency of DNA
anomalies in children with single suture problems.
Initial diagnosis
The entities covered by craniofacial surgery are
relatively rare. Single suture synostosis occurs in
approximately 1/3000 births [26,27] and syndromic
synostosis occurs in approximately 1/20,000 births
[28 – 31]. It is not surprising that the average pe-
diatrician or family physician does not immediately
recognize these unusual syndromes. There is a role
for increasing the amount of education given to
primary care physicians from craniofacial specialists
to ensure that the diagnosis (eg, craniofacial anomaly)
is recognized and that the primary care physician
knows enough to refer the family and child to an
appropriate center or physician. The availability of
information has been facilitated by parent groups
supplying Websites and written material that are
readily available to families who seek information in
the early stage of a child’s life, assuming that an
appropriate diagnosis has been made. Continued edu-
cation in the craniofacial and neurosurgery specialties
is also required to ensure increased understanding of
the genetic origins of these diseases and the
limitations of the outdated concept of simply opening
the suture. It is common for children with craniofacial
anomalies to be referred to a busy craniofacial unit
after several cranial surgeries have been performed,
often with areas of loss of bone and underlying
cerebral injury. Incisions may be less than ideally
placed. This combination impedes the ability to
obtain the best possible result for the child and
432 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
represents less-than-ideal care. It would be expected
that a prudent neurosurgeon or craniofacial surgeon
would understand the complexity of any given child’s
care and not begin a course of therapy that he or she
was unable to complete.
The diagnosis of craniofacial syndromes and
craniosynostosis is made on the basis of the clinical
examination. In the neonatal period, neuroimaging
studies are rarely required but are obtained later to
confirm the diagnosis. Neuroimaging studies are an
important aspect of pre- and postsurgical investiga-
tions, and in many cases CT and MRI scans are
obtained to evaluate the patient accurately during the
diagnostic phase and plan the surgical correction.
These studies are most useful when conducted close
to the time of surgery and in the institution in which
the surgery will be performed. Education of insurance
payers is required so that they understand the im-
portance of appropriate referral before the onset of
therapy and the necessity for more than one type of
neuroimaging and its ideal timing.
Most appropriate care setting
We who are involved in the team care of children
with craniofacial problems assume that we do a better
job caring for the multiple needs of children and their
families than do individual practitioners, whatever
their specialty. We also assume that the surgical re-
sults as measured by morbidity, mortality, and clinical
results are better when performed in an appropriate
center. Unfortunately, we have failed to determine
objective criteria by which to measure the clinical
results, or we have established criteria that are not
available to many practitioners (eg, anthropometric
measurements) [27,32 – 52]. Parents have no access to
standards that allow them to compare the outcome of
their surgical team with the bbest available results.Q
Published results on morbidity and infection rates
from many of the large centers are available, but few
data from individual practitioners or small centers are
available [34 – 36,44,49,53 – 63]. The risks of com-
plications quoted to patients and parents are those in
the literature from the best centers, although they may
not reflect the real numbers in the hands of teams or
individuals who have not published their results. This
behavior is not unique to craniofacial surgery and is a
large problem for parents when they are trying to
gather information about the care of their children.
We must try to ensure that part of residency training
is to imbue the realization that quoting outcome
statistics that are not one’s own is misleading to
patients and families.
Centers have failed to prove that the long-term
outcome for the patient is significantly better if their
care is centralized from the beginning, largely
because of the absence of good grading scales and
the paucity of information published by individual
practitioners. Also at some point in their treatment,
most, if not all, children with craniofacial syndromes
are assigned to a craniofacial team. Comparative
results from different centers are hard to find, and
comparing different surgical techniques [64] gener-
ally has failed to demonstrate a clear benefit of one
over the other, except in sagittal synostosis [32,34,
35,39,43 – 45]. The paucity of strong outcome data
with which to compare results leaves the centers in a
weak position to argue that the care of children with
craniosynostosis always should be centralized. It
seems obvious that the availability of a coordinated
team, including the specialists who may be needed
for a child’s care, must result in a better outcome for
the child. Having input from all the specialists from
the beginning and being able to obtain easily every
aspect of care for a child and family should at least
make the complexity of care easier for the family. The
measurement of favorable results cannot be simply
the subjective comparison of pictures, parental
opinions, or anthropologic or neuroimaging data; it
must be a multifaceted view of each child’s outcome
as measured by progress at different ages. This type
of information also might answer the question as to
what aspect of care has the largest impact on the
child’s future. Is it surgical correction, development
in the child of a strong sense of self, the ability to
overcome other handicaps that some of the children
have (eg, hand problems of a child with Apert
syndrome)? It is likely that these factors play some
role and that the most important factor may vary from
child to child. There is a clear need for better devised
objective outcome studies in the population of
patients with craniofacial anomalies. They would be
expensive and difficult to design, yet a way must be
found to measure outcomes so that comparisons can
be made.
Despite the lack of easily recordable, objective
measures of outcome, no strong arguments can be
made against the importance of the multispecialty
approach for children with craniofacial syndromes.
No single specialty can supply the care required
to maximize the functioning of these children and
their families.
It is harder to argue for this kind of centralized
care for children with single cranial suture growth
problems. The latter group has been assumed to have
normal brains, and the goal of therapy has been con-
sidered to be avoidance of intracranial hypertension
 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
caused by reduced intracranial volume and correction
of the abnormal cranial shape. The cephalic index is
less than normal in these children pre- and post-
operatively [32], but no evidence supports the theory
that intracranial volume is reduced. Preoperative
volume and growth rates are normal [47], and
delayed fusion and raised intracranial pressure can
result after surgery [65]. The frequency with which
the stated goal of a bnormal head shapeQ is achieved
by the many practitioners who perform single suture
synostosis surgery is unavailable, as is the incidence
of death or serious morbidity. Most authors report
excellent results regardless of the type of surgery
[6,57,58,66 – 69]. The mortality and serious morbidity
rates from sagittal synostosis surgery are significant,
even using bminimal invasive techniques,Q and are
approximately 1%. Malpractice suits suggest that this
figure is even higher. The author’s personal experi-
ence of more than 28 years involves one death and
two serious residual brain injuries in 450 cases of
sagittal synostosis. I do know of at least three other
deaths and at least 12 cases of severe brain damage,
however. Accurate figures for national mortality and
morbidity would allow comparison with the results
from the craniofacial centers, and if the results from
the centers were better, then a strong argument could
be made for centralization of the care of all children
with craniosynostosis and craniofacial anomalies.
Functional problems
Past reports and recent data suggest that functional
problems occur in children with single suture fusion
and that an operation in isolation of appropriate long-
term, multidisciplinary care to identify or hopefully
avoid or minimize these problems is no longer the
appropriate standard of care. Although the IQ is
generally normal regardless of surgery [39,70,71], the
incidence of learning disabilities in children with
sagittal synostosis may be as high as 50%, regardless
of whether they have surgery [9,72,73]. The inci-
dence of diplopia and amblyopia in children with
unicoronal synostosis has been reported to be as high
as 30% [74]. If performed after 6 months of age,
treatment of trigonocephaly is less often associated
with adequate expansion in the region of the nasion
and correction of the nasal base displacement. There
is also an increasing number of reports of genetic
abnormalities in children with single suture growth
failure. Experimental data from molecular investiga-
tion of axis and organ development in the embryo
show many areas of overlap between the genes that
have been identified as abnormal in children with
433
craniosynostosis and early neurogenesis. Accumulat-
ing evidence suggests that there may be functional
and morphologic concerns in children with single
suture synostosis and that the use of the multi-
disciplinary craniofacial team may be the best setting
in which to follow these children. Increased informa-
tion regarding the functional problems associated
with single suture synostosis is the result of these
children being followed in craniofacial clinics.
Children with craniofacial syndromes have a host
of functional problems. The early treatment of these
children is directed toward improving function as the
primary goal and morphology as a secondary goal.
As the children age, there is more of a balance
between the functional and morphologic goals. Early
functional problems are related to incompetent,
narrowed airway and breathing difficulties because
of facial hypoplasia or choanal atresia. A small num-
ber of infants still require early tracheostomy to sur-
vive. Some of the children have severe exorbitism
at birth and require surgery to protect the corneas and
preserve vision. Children with kleeblatschädel have
raised intracranial pressure and may require emer-
gency shunting or cranial expansion in the first few
days to weeks of life to prevent blindness or cerebral
ischemia. Because of the abnormalities of the anat-
omy and shape of the orbits, many of the infants
require ophthalmologic care to prevent amblyopia
and to try to develop binocular vision.
Surgical treatment
It is interesting to try to identify the effects of
craniofacial centers on the treatment of single suture
synostosis. By the late 1970s, most of the large
pediatric neurosurgical centers in the United States
and Canada had realized that the results of strip
craniectomies for single suture synostosis, even if
performed in the first few months of life, were not
producing the desired morphologic corrections in a
high enough percentage of children. As a result of
this dissatisfaction, the extent of the surgery increased
and the standard operations became extensive cranial
vault remodeling, anterior, posterior or total for
sagittal synostosis and orbital bar advancement, uni-
lateral or bilateral for coronal synostosis. Because of
increased blood loss and the need for bony recon-
struction, the timing for surgery was delayed to after
3 months of age for the sagittal synostosis and
approximately 6 months for coronal synostosis. This
more extensive surgical correction did seem to be
associated with better morphologic correction of head
shape. The average hospital stay has diminished in
434 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
centers that reported it despite the increase in the
extent of surgery. The more extensive surgical tech-
niques also have led to greater efforts to control blood
loss in surgery and increase hemoglobin before
surgery [75]. The net result is a decrease in trans-
fusions, at least in the centers that report their results.
When comparing the results of endoscopic-assisted
craniectomies with more standard techniques, the
factor of blood transfusion must be compared with
current results from good centers and not historical
controls. Each center that performs endoscopic-
assisted surgery must inform patients about that
center’s transfusion rate, because many patients
who undergo such surgery also receive blood trans-
fusions. Most single suture surgery is still performed
by neurosurgeons, often in isolation from a cranio-
facial team. In the early 1990s, an uncontrolled
survey by the American Association for Pediatric
Neurosurgery found that at least 50% of cranio-
synostosis surgery was performed by neurosurgeons
with no special pediatric training.
Psychosocial impact
We do not have adequate evidence to measure the
impact of craniostenosis and craniofacial syndromes,
excluding cleft lip and palate, on psychosocial
development in children [9,38,70,72,73,76 – 81].
Most studies suggest that the impact is greater with
age and that the early positive results on self-esteem
and confidence may fade with age. Most pre- and
postoperative studies show a positive effect on
perception of appearance in parents and children. It
is hard to extract that this effect has any long-term
positive effect on a child’s social outcome. More
longitudinal studies are required and more informa-
tion on older children must be collected. Currently, I
could not find sufficient data to support performing
craniofacial surgery in either single suture synostosis
or syndromic synostosis purely for the psycho-
social benefits that result from the improved appear-
ance. The syndromic children require psychological
follow-up and counseling as part of the multidis-
ciplinary approach.
Decision making
What factors come into play during a physician’s
decision-making process about whether to perform a
surgical procedure for craniosynostosis? Hippocrates
is reported to have proposed the dichotomy of the
physician being torn between a sense of philanthro-
pia, love of mankind, and philotechnica, love of
the art. This struggle is still present. Most neuro-
surgeons are exposed to the treatment of craniosynos-
tosis during their residency. The actual operative
procedure is not considered complex if a strip cra-
niectomy is being performed and is more complex if
extensive cranial reconstruction is performed. There
are some major differences between performing
surgery on the sutures of an infant and performing a
craniotomy in an older child or adult, however.
Meticulous control of blood loss is important from
the time of skin incision throughout the entire
procedure. The use of the Colorado needle has made
a big difference in the amount of blood lost during
opening and closing of the coronal scalp incision, yet
it is not in general use. The anatomy of the nor-
mal sutures at this age is such that the dura reflects
through the suture, and tearing the dura at the suture
line is a frequently encountered technical problem.
The vascularity of the bone is significant, and it is not
easy to stop all the oozing with bone wax because of
the small surface area of the bone edge. The surgeon
must consider these factors. The need for an anes-
thesiologist who has pediatric training and is familiar
with delivering anesthesia to infants is possibly the
most important of the intraoperative needs. Infants
cool easily, especially with the brain exposed, and
handle blood loss in a different manner than adults
or older children. The only signs of hypovolemia
may be tachycardia followed by a sudden precipi-
tous drop in blood pressure. These problems must
be understood and prevented or treated by the anes-
thesiologist. The most common time for the appear-
ance of hypovolemic shock is the first 1 to 2 hours
after surgery, which is often after the child has left
the recovery room. Because most hospitals do not
have a pediatric intensive care unit, usually the nurses
are less equipped to recognize and respond to such
an emergency.
The decision to operate should consider not only
the surgeon’s skills and experience but also the
availability of necessary other personnel to make
the procedure as safe as possible. Surgeons must be
honest with themselves and the family when consid-
ering the best location for surgery. It is often easier
for surgeons to consider that the surgery can be
performed more safely in another location with all the
personnel available, thus bypassing the question of
their own skills.
The fact that it is easier and more convenient for
the baby and family to have the surgery performed in
their home location is often brought up as a reason for
initial surgery being done outside a craniofacial center
or not in a children’s hospital. This is a factor to be
 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
considered, but most parents happily offset their own
convenience for the best care for their child. Rarely is
the surgery an emergency, and there is time for the
family to prepare for a trip to whatever location is
considered best for the surgery. Local physicians can
place more weight on the convenience of local care
than the quality of care if they are not fully aware of
not only the risks of surgery but also the dependence
of the operation’s success on site selection.
The question of reimbursement must be consid-
ered. Although few neurosurgeons or craniofacial
surgeons directly confront this issue, it does play a
role in the decision regarding where surgery is
performed and what surgery is performed. The
neurosurgeon who performs one or two such opera-
tions per year is aware of what he or she will be paid
for the procedure; this plays some role in deciding
whether to refer the child. In a children’s hospital or
craniofacial center, reimbursement is also a factor, but
most craniofacial centers are used to caring for the
indigent and insured populations. The weight given
to the issue of reimbursement is hopefully less in the
busy craniofacial centers. Monetary payment should
play no role in making the decision as to whether
surgery is required.
The selection of which operative procedure is ap-
propriate for any given child varies among the centers.
Some centers always perform the same operation—
the most extensive one—for a given diagnosis,
whereas most of the children require a more
individualized approach. Some centers automatically
perform two operations a few weeks apart for sagittal
synostosis—one posterior followed by one anterior—
although there is no evidence that this is necessary or
that the results are better than a single appropriate
operation. Does some economic incentive contribute
to this approach? Some centers have higher rates of
reoperation than others. Does reimbursement come
into the equation, or is this a difference in aesthetic
acceptance of what is a good result? Many of the
children are on state or federal aid programs, and the
reimbursement is small, so it is difficult to evaluate
exactly how large a factor reimbursement is in any of
the decisions made for the care of the children.
Advertising for patients is currently a part of
medical practice that invades the public domain. The
use of Websites to attract craniofacial or single suture
synostosis patients is increasing rapidly. These sites
rely entirely on physicians and other health care
professionals involved for their veracity. There is no
peer review; there is no external control over what is
placed on the Website. Parents have no way of
knowing whether the claims made are true. These
sites should be considered as part of the informed
435
consent procedure and should be controlled rigor-
ously to reflect true and current data from a specific
center. These sites can supply useful information to
parents that helps them to choose treatment. At best,
these sites furnish parents with an understanding of
their child’s problems. Parents who have the ability to
travel can visit some of the units and decide where
they will feel most comfortable receiving treatment
for their child. It is a surgeon’s responsibility and
moral duty to be truthful as to number of cases and
complications in their center and not tout one
operative technique over another to convince parents
to use his or her facility unless there is reliable class I
information that one technique is better than another.
In many cases, several operative procedures are
associated with similar results, and although the team
may feel that their particular approach is best, this
information should be passed on to parents as opinion
rather than hard fact.
Summary
Children born with growth problems that affect
the cranium or face require an initial correct diagnosis
of at least the area of their problem. Children with
syndromic craniosynostosis should be referred to an
appropriate center in which multiple personnel
needed to deliver the best care to the child and
family are available. The best timing for the first
operative procedure is not yet established. In general,
the younger the child at the time of the first opera-
tion, the greater the need for more surgery. Con-
versely, many children require surgery to preserve
function in the early months of life. Children with
single suture synostosis do have functional and
morphologic problems that may be cognitive, devel-
opmental, and visual. These problems must be cate-
gorized and treated as necessary. Although the
surgery in this group of children often may be per-
formed by a neurosurgeon alone, a comprehensive
and long-term follow-up is needed for the optimal
care of these children. There is no support for the
claim that brain damage as a result of intracranial
hypertension can occur in all of the children within
this group.
Craniofacial surgery is a necessary and effective
specialty that involves the operative treatment of
children with abnormal growth, deficient develop-
ment, or traumatic or postnatal damage to the cranium
or face. It requires the expertise of many specialists
and an adequate number of treated cases a year to
maintain expertise, although an exact number has not
been established scientifically. Although many dis-
436 D.A. Bruce / Oral Maxillofacial Surg Clin N Am 16 (2004) 429 – 438
agreements exist among the operative teams regard-
ing timing and exactly what operation may be best,
the mortality and morbidity rates should be available
in all centers that perform this type of surgery.
Infection occurs almost exclusively in the reopera-
tions, but brain ischemia from hypotension and death
can occur during any craniofacial procedure. The
reported mortality rates are below 1% but are not
zero. Surgical interventions for craniosynostosis—
regardless of whether it is syndromic—should not be
undertaken lightly because they are serious, major
operations in infants. There is insufficient evidence in
the literature that improving the morphology of an
infant’s head results in significant, long-term, psy-
chosocial benefit. This must be remembered and
presented to parents during the information-giving
process and is especially important when treating
children with single suture synostosis.
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