PEDIATRIC/CRANIOFACIAL

Raised Intracranial Pressure in Apert Syndrome

Damian D. Marucci, Ph.D.,
Background: Raised intracranial pressure is a well-known complication of Apert
F.R.A.C.S. syndrome. The current policy in the authors’ unit is to monitor these patients
David J. Dunaway, and only perform surgery when raised intracranial pressure has been diagnosed.
F.D.S.R.C.S., F.R.C.S. The authors present their experience with this protocol, as it allows a more
Barry M. Jones, M.S., F.R.C.S. accurate picture of the natural history of raised intracranial pressure in Apert
Richard D. Hayward, F.R.C.S. syndrome.
London, United Kingdom Methods: The records of 24 patients, aged between 7 and 14 years, with Apert
syndrome who had been managed expectantly (i.e., with no routine “automatic”
early surgery) were reviewed. Data were collected on the incidence, timing, and
management of raised intracranial pressure.
Results: Twenty of 24 patients (83 percent) developed raised intracranial pres-
sure. The average age of the first episode was 18 months (range, 1 month to 4
years 5 months). Raised intracranial pressure was managed with surgery in 18
patients, including two patients who underwent shunt procedures for hydro-
cephalus. Two patients had their raised intracranial pressure treated successfully
by correcting coexisting upper airway obstruction alone. Seven of the 20 patients
(35 percent) developed a second episode of raised intracranial pressure, on
average 3 years 4 months later (range, 1 year 11 months to 5 years 9 months).
Conclusions: In Apert syndrome, there is a high incidence of raised intracranial
pressure, which can first occur at any age up to 5 years and may recur despite
initial successful treatment. Causes of raised intracranial pressure include
craniocerebral disproportion, venous hypertension, upper airway obstruction,
and hydrocephalus. Careful clinical, ophthalmologic, respiratory, and radio-
logic monitoring will allow raised intracranial pressure to be diagnosed accu-
rately when it occurs and then treated most appropriately. (Plast. Reconstr. Surg.
122: 1162, 2008.)

A
pert syndrome is a rare autosomal domi-
nant condition comprising craniosynosto-
sis, midfacial hypoplasia, and limb syndac-
tyly. If left untreated, the incidence of raised
intracranial pressure has previously been reported
as 45 percent.1 Untreated intracranial hyperten-
sion may result in insidious optic atrophy, visual
loss, and possible developmental delay.2– 4
Many craniofacial centers advocate early surgery
for patients with syndromic craniosynostosis (often
frontoorbital advancement) in an attempt to pre-
vent the development of raised intracranial pressure
and improve the children’s subsequent cognitive
outcome.1,4,5 Various surgical timetables for treating
these patients have been published.1,6 – 8 The hope is
that the release of prematurely fused sutures will
allow the cranial vault to expand to accommodate
the growing brain.3 However, craniocerebral dispro-
portion is not the only cause of raised intracranial
pressure in patients with syndromic craniosynostosis.9
Previous studies have demonstrated that patients
with Apert syndrome have a larger intracranial
volume than normal controls.10,11 Other impor-
tant factors in the development of raised intracra-
nial pressure include abnormal intracranial ve-
nous drainage,12–14 hydrocephalus,1,15 and airway
obstruction.16
Given the multifactorial etiology of raised
intracranial pressure in syndromic craniosynosto-
sis, it is clear that not all patients require preemp-
tive vault expansion surgery. It has been the prac-
tice of our institution for the past 15 years to

From the Craniofacial Unit, Great Ormond Street Hospital

for Children.
Received for publication July 20, 2007; revised February 5,
2008.
Copyright ©2008 by the American Society of Plastic Surgeons
DOI: 10.1097/PRS.0b013e31818458f0
Disclosure: None of the authors has a financial
interest in any of the products, devices, or drugs
mentioned in this article.

1162 www.PRSJournal.com
Volume 122, Number 4 • Apert Syndrome
frequently monitor patients with syndromic cra-
niosynostosis for evidence of raised intracranial
pressure and treat it in the most appropriate fash-
ion if and only when it develops. In the past, we
have used transcranial intracranial pressure mon-
itoring to determine intracranial pressure in all
patients, in addition to the usual ophthalmic as-
sessment of optic disc appearance and visual acu-
ity. Over the past decade, however, we have in-
creasingly used noninvasive serial pattern reversed
visual evoked potentials, which have been shown
to provide early and reliable evidence of postreti-
nal dysfunction, indicating raised intracranial
pressure in these patients.17 We present our ex-
perience with this protocol, as we believe it rep-
resents an accurate picture of the natural history
of raised intracranial pressure in Apert syndrome.
PATIENTS AND METHODS
After approval from our institutional ethics
board, the records of 36 consecutive patients
with Apert syndrome born between July of 1992
and December of 2000 were reviewed. These
patients were identified on our craniofacial da-
tabase to be aged between 7 and 14 years at the
time of the study. Data were collected on the age
at referral; the timing, nature, and reason for
any transcranial surgery; the incidence of raised
intracranial pressure as determined by fundus-
copy, pattern reversed visual evoked potentials,
or transcranial intracranial pressure monitor-
ing; and the management of raised intracranial
pressure.
Excluded from further analysis were five chil-
dren who had undergone surgery at another in-
stitution before referral, five patients who under-
went early vault expansion surgery for cosmetic
reasons (and could therefore be described as hav-
ing been treated in the “traditional” manner), one
patient whose care was transferred to another in-
stitution at the age of 5, and one patient who died
at 13 months of age as a result of respiratory arrest,
possibly following a seizure. Neither of these last
two patients had had any episodes of raised intra-
cranial pressure.
The remaining 24 patients were managed ex-
pectantly (i.e., surgery only if indicated for func-
tional concerns such as raised intracranial pres-
sure) and are the subjects of this review. All
parents were made aware of and consented to the
expectant management protocol for syndromic
craniosynostosis at Great Ormond Street Hospital
for Children. There were 15 male and nine female
patients. Nine patients (38 percent) had a cleft
palate. The average age at referral to our institu-
tion was 1.4 months (range, 0 to 6 months).
All patients, including those who had early
vault expansion surgery for cosmetic reasons, un-
derwent multidisciplinary assessment on referral,
including computed tomographic scanning, fun-
duscopy, and polygraphic cardiorespiratory sleep
studies to detect obstructive sleep apnea. Multi-
disciplinary assessment including funduscopy with
retinal photography was performed at ages 3, 6, 9,
12, and 18 months of age, and then at 3, 4, 6, and
10 years of age. Funduscopy was performed more
frequently if there were any concerns. In the
1990s, transcranial intracranial pressure monitor-
ing was routinely performed before the patient
was 12 months of age to assess for the presence of
raised intracranial pressure and also frequently to
confirm the presence of raised intracranial pres-
sure in the presence of papilledema. Mean pres-
sures of greater than 15 mmHg over 24 hours, or
more than three plateau waves in 24 hours, indi-
cated an abnormal trace. In the late 1990s to the
present, we have increasingly relied on serial pat-
tern reversed visual evoked potentials for the de-
tection of postretinal dysfunction and as an indi-
rect measure of raised intracranial pressure.
Previous work from our institution has validated
visual evoked potentials as an effective noninvasive
tool for detecting raised intracranial pressure
when compared with funduscopy and visual
acuity.17 Visual evoked potentials were performed
at the time of funduscopic assessment as outlined
above, and more frequently if there were any con-
cerns. A deterioration in visual evoked potentials
was indicative of postretinal dysfunction and prob-
able raised intracranial pressure. The degree of
deterioration of visual evoked potentials does not
necessarily correlate with a particular intracranial
pressure. After the detection of raised intracranial
pressure, (whether by funduscopy, a deterioration
in visual evoked potentials, or transcranial intra-
cranial pressure monitoring), the etiologic factor
was sought. In particular, computed tomographic
scanning was performed to exclude hydrocepha-
lus, and polygraphic cardiorespiratory sleep stud-
ies were used to diagnose obstructive sleep apnea.
If present, hydrocephalus and obstructive sleep
apnea were treated first. If hydrocephalus and ob-
structive sleep apnea were not present or had been
successfully treated and the patient had raised
intracranial pressure, a procedure to expand the
intracranial volume (vault expansion, fronto-
orbital remodeling, or bipartition distraction) was
then performed. The correction of raised intra-
cranial pressure was confirmed by changes in fun-
1163
 Plastic and Reconstructive Surgery • October 2008

duscopy, visual evoked potentials, and/or trans-
cranial intracranial pressure monitoring in the
weeks after intervention in all cases. If posttherapy
funduscopy, visual evoked potentials, or transcra-
nial intracranial pressure monitoring did not
demonstrate a correction of intracranial pressure,
further treatment measures were taken as appro-
priate.
RESULTS
Of the 24 patients managed expectantly, 20 (83
percent) developed raised intracranial pressure at
some stage. The average age of the first episode of
raised intracranial pressure was 18 months (range, 1
month to 4 years 5 months) (Fig. 1). The first epi-
sode of raised intracranial pressure was managed
surgically in 18 patients (90 percent), as shown in
Table 1. Surgical procedures performed to treat
the first episode of raised intracranial pressure
in this group included posterior and/or bipa-
Fig. 1. Histogram of age at first presentation of raised intracra-
nial pressure for the 20 Apert patients in whom it developed.
rietal vault expansions in 11 patients, fronto-
orbital remodeling in four patients, shunt pro-
cedures in two patients who had hydrocephalus
(11 percent), and a facial bipartition distraction
procedure in one patient who had coexisting
problems with upper airway obstruction and cor-
neal exposure. Two patients who had under-
gone fronto-orbital remodeling had coexisting
airway obstruction that was treated with a com-
bination of nasopharyngeal airway, adenoton-
sillectomy, and/or continuous positive airway
pressure. Two patients (10 percent) had their
raised intracranial pressure managed successfully
without surgery, through correction of their airway
obstruction alone, using a combination of nasopha-
ryngeal airway, adenotonsillectomy, and/or contin-
uous positive airway pressure.
Seven of the 20 patients (35 percent) whose
initial episode of raised intracranial pressure was
treated successfully developed a second episode of
raised intracranial pressure, on average 3 years 4
months later (range, 1 year 11 months to 5 years
9 months) (Table 2). Both patients who had ini-
tially undergone shunt procedures to treat hydro-
cephalus as the cause of their first episode of
raised intracranial pressure developed further
raised intracranial pressure and were treated with
posterior vault expansions. One patient whose ini-
tial episode of raised intracranial pressure was suc-
cessfully treated by optimizing upper airway func-
tion developed a second episode of raised
intracranial pressure 5 years 9 months later, which
was treated with a posterior vault expansion.
Three patients whose first episode of raised intra-
cranial pressure was treated with surgery devel-
oped a second episode of raised intracranial pres-

Table 1. Management of First Episode of Raised Table 2. Management of Second Episode of Raised
Intracranial Pressure (n ⴝ 20) Intracranial Pressure
No. of Treatment Modality Time between
Treatment Modality Patients Average Age First and
Second Second
Transcranial surgery First Episode of Episode of Episodes of
Vault expansion (posterior Patient Raised ICP Raised ICP Raised ICP
and/or biparietal) 11 2 yr 2 mo
FOR* 4 1 yr 9 mo 1 NPA/AT/CPAP PVE 5 yr 9 mo
Facial bipartition with distraction 1 4 yr 5 mo 2 AT/FOR CPAP 4 yr 1 mo
Endoscopic third 3 VP shunt PVE 3 yr 1 mo
ventriculostomy† 1 8 mo 4 ETV/VP shunt PVE 4 yr 3 mo
VP shunt 1 1 yr 10 mo 5 PVE NPA/AT 2 yr
Correction of obstructive sleep 6 PVE CPAP 1 yr 11 mo
apnea (NPA, AT, CPAP)* 2 6 mo 7 FOR/NPA VP shunt/AT 2 yr 5 mo*
FOR, fronto-orbital remodeling; VP, ventriculoperitoneal; NPA, ICP, intracranial pressure; AT, adenotonsillectomy; FOR, fronto-
nasopharyngeal airway; AT, adenotonsillectomy; CPAP, continuous orbital remodeling; VP, ventriculoperitoneal; NPA, nasopharyngeal
positive airway pressure. airway; CPAP, continuous positive airway pressure; PVE, posterior
*Two patients had both correction of obstructive sleep apnea and vault expansion; ETV, endoscopic third ventriculostomy.
fronto-orbital remodeling. *This patient developed further episodes of raised intracranial pres-
†Third ventriculostomy was followed by ventriculoperitoneal shunt. sure related to ventriculoperitoneal shunt malfunctions.

1164
Volume 122, Number 4 • Apert Syndrome
sure related to upper airway obstruction that was
treated successfully with adenotonsillectomy
and/or continuous positive airway pressure. One
patient developed more than two additional epi-
sodes of raised intracranial pressure, all of which
were related to repeated ventriculoperitoneal
shunt malfunctions.
Five patients (average age, 10 years; range, 7
years 9 months to 13 years) had not required any
transcranial surgery at the time of this study. Four
of these patients had not developed raised intra-
cranial pressure and one patient who did develop
raised intracranial pressure had it managed suc-
cessfully through the correction of airway obstruc-
tion. All patients will be offered facial bipartition
with or without distraction as teenagers to correct
the hypertelorism, brachycephaly, proptosis, and
maxillary hypoplasia associated with Apert syn-
drome. All patients will be offered definitive or-
thognathic correction of any residual improper
occlusal relationships at skeletal maturity.
The average number of major transcranial
procedures per patient in this cohort of patients
was one (i.e., excluding the single endoscopic
third ventriculostomy and seven shunt-related
procedures). In total, 29 transcranial intracranial
pressure monitoring procedures were performed,
giving an average of 1.2 such procedures per pa-
tient (range, 0 to 4). Eighteen patients (75 per-
cent) had at least one transcranial intracranial
pressure monitoring procedure performed dur-
ing the course of this study. Because of our in-
creasing reliance on visual evoked potentials, the
numbers of transcranial intracranial pressure
monitoring performed during the period evalu-
ated declined so that only two patients in this
cohort underwent transcranial intracranial pres-
sure monitoring in the past 5 years.
Of the five patients who underwent early
surgery for cosmetic deformity in the “tradi-
tional” manner, who were not part of the cohort
of 24 patients managed expectantly, four had
posterior vault expansions and one had a total
frontal craniectomy, all before the age of 12
months. Three of these patients subsequently
developed episodes of raised intracranial pres-
sure requiring intervention.
DISCUSSION
The syndromic craniosynostoses represent a
heterogenous group of conditions. The inci-
dences of hydrocephalus, herniation of the cere-
bellar tonsils, and raised intracranial pressure, for
example, differ in the various syndromes.1,14,15 It
used to be our policy to recommend some sort of
vault expansion surgery for all children presenting
with, in particular, Apert, Crouzon, and Pfeiffer
syndromes, to treat raised intracranial pressure
when present, or to prevent it1,14,15 when not. How-
ever, as experience with the ophthalmic diagnosis
and monitoring of raised intracranial pressure
grew, we altered our protocol so that we now only
treat raised intracranial pressure if and when it
occurs. The abandoning of a “one-size-fits-all” pol-
icy of early vault expansion provides an opportu-
nity to study the natural history of raised intracra-
nial pressure in Apert syndrome. The purpose of
this study was to unravel some of the complexities
of raised intracranial pressure in Apert syndrome
in a cohort of children treated only when raised
intracranial pressure was diagnosed. We have not
reported functional outcomes for these children,
as that is the subject of a separate communication.
A previous study did not find any correlation be-
tween raised intracranial pressure and intellectual
development in patients with Apert syndrome, al-
though the numbers of patients in each subgroup
were small.5
This study has demonstrated a high incidence
of raised intracranial pressure (83 percent) in un-
treated patients with Apert syndrome. This figure
is higher than older studies but in keeping with
more recent reports of intracranial pressure in
syndromic craniosynostosis using transcranial in-
tracranial pressure monitoring.1,9 Intracranial hy-
pertension occurred as early as 1 month of age,
and no patients developed their first episode of
raised intracranial pressure after 4 years 5 months.
This validated our examination of patients older
than 7 years of age with respect to the develop-
ment of raised intracranial pressure. Of the five
patients who underwent early surgery in the
“traditional” manner, three (60 percent) subse-
quently developed raised intracranial pressure.
The incidence of raised intracranial pressure fol-
lowing early surgery was higher in this closely mon-
itored cohort than reported in previous studies.2,3,6,18
The noninvasive detection of raised intracra-
nial pressure in children with syndromic cranio-
synostosis can be difficult. Irreversible visual loss
and even blindness may occur without any other
symptoms of raised intracranial pressure.4 Optic
appearance (i.e., papilledema) has been shown to
be a specific indicator of raised intracranial pres-
sure in children with craniosynostosis younger
than 8 years, but it lacks sensitivity.19 Radiologic
and computed tomographic findings also lack sen-
sitivity for detecting raised intracranial pressure in
craniosynostosis.20 Although transcranial intracra-
nial pressure monitoring remains the standard
1165
 Plastic and Reconstructive Surgery • October 2008
method for detecting raised intracranial pressure
in these children, it is an invasive procedures with
recognized complications including mechanical
malfunction, cerebrospinal fluid leak, intracranial
hemorrhage, and infection.8,9 Furthermore, a nor-
mal intracranial pressure at, say, 1 year of age does
not, as we have shown, preclude the onset of raised
intracranial pressure 2 or 3 years later. We do not
consider it acceptable to subject children with syn-
dromic forms of craniosynostosis to serial trans-
cranial intracranial pressure monitoring now that
a satisfactory alternative is available.
Reversed pattern visual evoked potentials is a
reproducible noninvasive method of monitoring
neuronal processing of the visual pathway with
millisecond resolution. Visual evoked potentials
have been validated as an accurate measure of
postretinal dysfunction in patients with syndromic
craniosynostosis.17 Visual evoked potentials as part
of a regular ophthalmic assessment have largely
replaced transcranial intracranial pressure moni-
toring at our institution and have greatly facili-
tated the monitoring, early detection, and treat-
ment of raised intracranial pressure.
Raised intracranial pressure in syndromic cra-
niosynostosis is multifactorial in origin. Cranioce-
rebral disproportion was traditionally thought to
be the most common cause of raised intracranial
pressure in patients with craniosynostosis. How-
ever, Apert syndrome is associated with an in-
crease in intracranial volume compared with nor-
mal controls, and previous studies have noted a
lack of predictable correlation between intracra-
nial volume and intracranial pressure.10 –12 Clearly,
other factors are involved in the development of
raised intracranial pressure in Apert syndrome,
and we now know it to be a complex interaction
between venous hypertension, airway obstruction,
and cerebrospinal fluid circulation dynamics.9,14
Venous hypertension may develop in patients
with syndromic craniosynostosis because of abnor-
mal intracranial venous drainage.14 There is a high
incidence of significant stenosis or occlusion of
the sigmoid-jugular sinus complex with anoma-
lous venous collateral channels in patients with
syndromic craniosynostosis.12 The disordered ve-
nous anatomy may be attributable to bony con-
striction of the normal venous outflow pathways,
a primary disorder of venous anatomy related to
the genetic mutation or persistence of the fetal
pattern of intracranial venous drainage in these
patients. It is thought that the contribution of
venous hypertension to raised intracranial pres-
sure is diminished after the age of 6 years because
of the opening of collateral venous channels or
1166
the persistence of bilateral drainage through the
transverse sinuses.14 Venous hypertension result-
ing from venous occlusion at the cranial base may
cause intracranial pressure to rise directly, or may
result in secondary hydrocephalus because of de-
creased cerebrospinal fluid absorption.15 Al-
though there have been isolated reports in the
literature of bypass procedures to treat intracra-
nial venous hypertension,21–23 the current treat-
ment of choice remains vault expansion once hy-
drocephalus and upper airway obstruction have
been excluded.14
Ventricular dilatation occurs in 40 to 90 per-
cent of Apert syndrome patients because of a con-
stricted posterior fossa and/or impaired cerebro-
spinal fluid absorption resulting from venous
hypertension.15 However, only a small number of
patients with Apert syndrome develop shunt-de-
pendent hydrocephalus. Two patients (8 percent)
in the current study required shunt procedures to
treat their first episode of raised intracranial pres-
sure. Both of these patients developed second ep-
isodes of raised intracranial pressure in the ab-
sence of shunt malfunction and required vault
expansion surgery. One patient developed shunt-
dependent hydrocephalus following previous
fronto-orbital remodeling as the cause of their
second episode of raised intracranial pressure.
This sequence of events following vault expansion
surgery has been described previously.24
Obstructive sleep apnea caused by midfacial hy-
poplasia is a common feature of Apert syndrome.16
Obstructive sleep apnea in adults without craniosyn-
ostosis can cause raised intracranial pressure in ad-
dition to papilledema and visual loss.4 Obstructive
sleep apnea is associated with raised intracranial
pressure in patients with syndromic craniosynostosis.16
Carbon dioxide is a potent cerebral vasodilator
and is retained in obstructive sleep apnea, leading
to raised intracranial pressure. Polygraphic car-
diorespiratory sleep studies were performed on all
our patients with Apert syndrome. If raised intra-
cranial pressure is detected in the presence of an
abnormal sleep study, our first priority is to correct
to upper airway obstruction with either nasopha-
ryngeal airway, continuous positive airway pres-
sure mask, adenotonsillectomy, midfacial surgery,
or tracheostomy. Should the raised intracranial
pressure persist after correction of the obstructive
sleep apnea, vault expansion surgery may be in-
dicated. Three patients in the current series had
clinically significant obstructive sleep apnea at the
time of their first episode of raised intracranial
pressure. In two patients, correction of obstructive
sleep apnea successfully treated the raised intra-
Volume 122, Number 4 • Apert Syndrome
cranial pressure without the need for transcranial
surgery. One of these patients developed a second
episode of raised intracranial pressure over 5 years
later that required a vault expansion.
The timing and sequence of transcranial sur-
gery to treat patients with Apert syndrome remains
controversial. Should vault expansion be deemed
necessary to treat raised intracranial pressure, our
preference is to perform a posterior procedure in
the first instance.25 This provides a generous in-
crease in intracranial volume and preserves the
fronto-orbital area for definitive correction at a
later stage. Our preferred procedure for the
frontofacial region is the facial bipartition with or
without distraction.25–27 This is designed to correct
the hypertelorism, midfacial retrusion, exorbit-
ism, abnormal maxillary occlusal plane, and
brachycephaly. It effectively removes the stigmata
of Apert syndrome from the face. One patient in
this series underwent this procedure at the age of
4 years because of coincident problems with raised
intracranial pressure, corneal exposure, and ob-
structive sleep apnea. All patients will be offered
this procedure in their early teenage years. Finally,
all patients are offered definitive orthognathic
correction of occlusion at skeletal maturity.
This study raises a number of issues regarding
the relationship between the development of
raised intracranial pressure and preemptive sur-
gery. Are we doing our patients a disservice by
allowing them to develop raised intracranial pres-
sure before intervening? Preemptive vault expan-
sion, it could be argued, has a protective effect in
terms of the development of raised intracranial
pressure. However, 21 percent of our patients
have not required any transcranial surgery (ex-
cluding cerebrospinal fluid diversion) for raised
intracranial pressure, sparing them the potential
morbidity of these procedures. Furthermore, be-
cause of the frequent monitoring of these pa-
tients, any episode of raised intracranial pressure
is detected and treated early, and subsequent oph-
thalmic assessments have confirmed restoration of
their previous visual status.17 Finally, by taking a
more holistic approach to the issue of raised in-
tracranial pressure in syndromic craniosynostosis,
the treatment of any episodes of raised intracra-
nial pressure is directed at the cause (airway ob-
struction, hydrocephalus) rather than automatic
vault expansion. We are currently analyzing the
functional outcomes of patients with Apert syn-
drome treated at our institution to assess the im-
pact of our expectant protocol.
CONCLUSIONS
There is a high incidence of raised intracranial
pressure in Apert syndrome that can be attribut-
able, singly or in combination, to craniocerebral
disproportion, venous hypertension, airway ob-
struction, or hydrocephalus, and whose manage-
ment may involve attention to any of these causes.
Raised intracranial pressure can first occur at any
age up to 5 years and may recur despite initial
successful treatment. It is our contention that such
complexity and uncertainty is not best treated by
routine vault expansion in the first year of life.
Instead, careful clinical, ophthalmologic, and re-
spiratory monitoring will allow raised intracranial
pressure to be treated in the most appropriate
manner only when it occurs.
Richard D. Hayward, F.R.C.S.
Great Ormond Street Hospital for Children
Great Ormond Street
London WC1N 3JH, United Kingdom
haywar@gosh.nhs.uk
ACKNOWLEDGMENTS
Dr. Marucci’s Fellowship was supported by the Hugh
Johnston Travel Grant from the Royal Australasian
College of Surgeons. The authors thank Edmund Fitzger-
ald O’Connor, who cowrote the craniofacial database
with the first author.
REFERENCES
1. Renier, D., Lajeunie, E., Arnaud, E., and Marchac, D. Man-
agement of craniosynostoses. Childs Nerv. Syst. 16: 645, 2000.
2. Gosain, A. K., McCarthy, J. G., and Wisoff, J. H. Morbidity
associated with increased intracranial pressure in Apert and
Pfeiffer syndromes: The need for long-term evaluation. Plast.
Reconstr. Surg. 97: 292, 1996.
3. Siddiqi, S. N., Posnick, J. C., Buncic, R., et al. The detection
and management of intracranial hypertension after initial
suture release and decompression for craniofacial dysostosis
syndromes. Neurosurgery 36: 703, 1995.
4. Bartels, M. C., Vaandrager, J. M., de Jong, T. H., and Simonsz,
H. J. Visual loss in syndromic craniosynostosis with papill-
edema but without other symptoms of intracranial hyper-
tension. J. Craniofac. Surg. 15: 1019, 2004.
5. Renier, D., Arnaud, E., Cinalli, G., et al. Prognosis for mental
function in Apert’s syndrome. J. Neurosurg. 85: 66, 1996.
6. McCarthy, J. G., Glasberg, S. B., Cutting, C. B., et al. Twenty-
year experience with early surgery for craniosynostosis: II.
The craniofacial synostosis syndromes and pansynostosis—
Results and unsolved problems. Plast. Reconstr. Surg. 96: 284,
1995.
7. Panchal, J., and Uttchin, V. Management of craniosynostosis.
Plast. Reconstr. Surg. 111: 2032, 2003.
8. Sgouros, S. Skull vault growth in craniosynostosis. Childs Nerv.
Syst. 21: 861, 2005.
9. Tamburrini, G., Caldarelli, M., Massimi, L., Santini, P., and
Di Rocco, C. Intracranial pressure monitoring in children
with single suture and complex craniosynostosis: A review.
Childs Nerv. Syst. 21: 913, 2005.
1167
 Plastic and Reconstructive Surgery • October 2008

10. Anderson, P. J., Netherway, D. J., Abbott, A. H., et al. Analysis
of intracranial volume in Apert syndrome genotypes. Pediatr.
Neurosurg. 40: 161, 2004.
11. Fok, H., Jones, B. M., Gault, D. G., Andar, U., and Hayward,
R. Relationship between intracranial pressure and intracra-
nial volume in craniosynostosis. Br. J. Plast. Surg. 45: 394,
1992.
12. Taylor, W. J., Hayward, R. D., Lasjaunias, P., et al. Enigma of
raised intracranial pressure in patients with complex cranio-
synostosis: The role of abnormal intracranial venous drain-
age. J. Neurosurg. 94: 377, 2001.
13. Rich, P. M., Cox, T. C., and Hayward, R. D. The jugular foramen
in complex and syndromic craniosynostosis and its relationship
to raised intracranial pressure. A.J.N.R. Am. J. Neuroradiol. 24: 45,
2003.
14. Hayward, R. Venous hypertension and craniosynostosis.
Childs Nerv. Syst. 21: 880, 2005.
15. Collmann, H., Sörensen, N., and Krauss, J. Hydrocephalus in
craniosynostosis: A review. Childs Nerv. Syst. 21: 902, 2005.
16. Gonsalez, S., Hayward, R., Jones, B., and Lane, R. Upper
airway obstruction and raised intracranial pressure in chil-
dren with craniosynostosis. Eur. Respir. J. 10: 367, 1997.
17. Liasis, A., Nischal, K. K., Walters, B., et al. Monitoring visual
function in children with syndromic craniosynostosis: A com-
parison of 3 methods. Arch. Ophthalmol. 124: 1119, 2006.
18. Pollack, I. F., Losken, H. W., and Biglan, A. W. Incidence
of increased intracranial pressure after early surgical treat-
ment of syndromic craniosynostosis. Pediatr. Neurosurg. 24:
202, 1996.
19. Tuite, G. F., Chong, W. K., Evanson, J., et al. The effectiveness
of papilledema as an indicator of raised intracranial pressure
in children with craniosynostosis. Neurosurgery 38: 272, 1996.
20. Tuite, G. F., Evanson, J., Chong, W. K., et al. The beaten
copper cranium: A correlation between intracranial pres-
sure, cranial radiographs, and computed tomographic scans
in children with craniosynostosis. Neurosurgery 39: 691, 1996.
21. Steinbok, P., Hall, J., and Flodmark, O. Hydrocephalus in
achondroplasia: The possible role of intracranial venous hy-
pertension. J. Neurosurg. 71: 42, 1989.
22. Sindou, M., Mercier, P., Bokor, J., and Brunon, J. Bilateral
thrombosis of the transverse sinuses: Microsurgical revascu-
larization with venous bypass. Surg. Neurol. 13: 215, 1980.
23. Niwa, J., Ohtaki, M., Morimoto, S., Nakagawa, T., and Hashi,
K. Reconstruction of the venous outflow using a vein graft in
dural arteriovenous malformation associated with sinus oc-
clusion. No Shinkei Geka 16: 1273, 1988.
24. Thompson, D. N., Jones, B. M., Harkness, W., Gonsalez, S.,
and Hayward, R. D. Consequences of cranial vault expansion
surgery for craniosynostosis. Pediatr. Neurosurg. 26: 296, 1997.
25. Jones, B. M., Dunaway, D. J., and Hayward, R. D. Surgery. In
R. D. Hayward, B. M. Jones, D. J. Dunaway, et al. (Eds.), The
Clinical Management of Craniosynostosis. London: Mac Keith
Press, 2004. P. 374.
26. Van der Meulen, J. C. Medial faciotomy. Br. J. Plast. Surg. 32:
339, 1979.
27. Tessier, P. Facial bipartition: A concept more than a proce-
dure. In D. Marchac (Ed.), Craniofacial Surgery. Berlin:
Springer-Verlag, 1987. P. 218.

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