BCC Vol.

68, November-December 2011

Craniosynostosis. II. AnaIysis of syndromic craniosynostosis and
different types of treatment
REVIEW ARTICLE
Bol Med Hosp nfant Mex 2011;68(6):377-385
Fernando Chico Ponce de Len
ABSTRACT
n this second part of the Review Article on craniosynostosis, different types of syndromatic craniosynostosis are analyzed along with clinical
and imaging aspects and, in known cases, embryogenetic alterations. Different types of treatments are also described for both syndromic
and nonsyndromic craniosynostosis. These range from the classic surgical treatments for achieving brain decompression, restoring the
anatomy and providing the highest degree of aesthetics for the child. Last, but not least, information on cutting-edge treatments such as
techniques in tissue engineering, use of bioabsorbable bone distractors and even endoscopic surgical systems are included. t is expected
that in the near future there should be a greater number of publications that report the success of these new techniques.
Key words: syndromic craniosynostosis, Crouzon's disease, Apert syndrome, Pfeiffer syndrome, cloverleaf skull syndrome, craniosynos-
tosis surgery.
Departamento de Neurociruga, Hospital nfantil de Mxico Federico
Gmez and Facultad de Medicina, Universidad Nacional Autnoma
de Mxico, Mxico D.F., Mxico
Correspondence:
Dr. Fernando Chico Ponce de Len
Departamento de Neurociruga
Hospital nfantil de Mxico Federico Gmez
Mexico, D.F., Mexico
E-mail: chico1204@prodigy.net.mx
Received for publication: 2-28-11
Accepted for publication: 8-9-11
INTRODUCTION
This second part oI the Review Article on Craniosynostosis
describes entities associated with a syndrome, also known
as syndromic.
Crouzon Syndrome
Dehnition and epidemiologv
This entity was frst described by Octave Crouzon in 1912
associated with a craniosynostosis (CS), usually bicoronal
and sagittal and at a later phase with hypoplasia oI Iacial
bones. This is the most Irequent syndromic CS Ior all
reviewed series.
1-4
Clinical and imaging characteristics
Mild Iacial malIormations are present at birth, making
this entity diIfcult to diagnose at that time. The disea-
se is usually diagnosed when the child is ~2 years old.
Although severe cases can be identifed at birth, they are
rare. Frequently, exorbitism appears aIter setback oI upper
maxillary and Iorehead bones. Severe cases oI Crouzon
syndrome may present exorbitism that compromises eye-
balls. Hypertelorism presents rarely; however, there is a
Irequent presentation oI strabismus with exotropia asso-
ciated with deIective insertion oI external ocular muscles.
Patients present class III malocclusion (mesiocclusion)
with prognathism or at least incisive teeth touching on
Iree borders, producing several types oI prognathism.
The nose is hooked (resembling a 'parrot) in some cases
(Figure 1). Sometimes these CS resemble scaphocephaly
at birth (Figure 2).
5,6

This entity evolves and early onset demonstrates the
most severe cases. In addition to compromising eyeball
integrity, patients oIten present respiratory insuIfciency
because oI narrow nostrils secondary to hypoplasia oI
Iacial bones, especially Irom upper maxillary bones and
choanae. This characteristic may lead to recurrent respi-
ratory inIections.
3,7,8
This is a severe malIormation where patients present
intracranial hypertension (ICH) (68.6), papillary edema
(17) and ophthalmic atrophy (3.4). Hydrocephaly,
BCD BoI Med Hosp Infant Mex
Fernando Chico Ponce de Len
possibly with venous etiology, presents in 25 oI cases
because oI narrow jugular Ioramen or compression oI
herniated cerebellar tonsil. Brain should be decompres-
sed beIore the frst year oI liIe to avoid a decrease oI
intellectual quotient because 80 oI cases with IQ ~90
may decrease to 65.6 oI cases. It can be associated with
cervical vertebrae anomalies (Klippel-Feil) and with acan-
thosis nigricans because oI mutation oI FGFR3 gene.
8-12
Apert Syndrome or Acrocephalosyndactyly
Dehnition and epidemiologv
This entity was described by Eugene Apert in 1906. This
is a severe malIormation associated with a Iaciocraniosy-
nostosis with syndactyly oI Iour limbs.
13
Stenosed sutures
oI the cranial vault are always coronal without compro-
mising metopic and sagittal sutures. There are reports
where coronal sutures are permeable in this entity.
14
As
in Crouzon`s disease, this entity presents upper maxillary
hypoplasia (Figure 2). In Apert syndrome, this condition
is more severe and triggers a type II dental malocclusion

(mesiocclusion) with prognatic bite, wide Iace, aquiline
nose and constant hypertelorism with external eyelid
commissures pointing downwards and antimongolic tilt
oI external eyelid commissures (Figure 1). Palate is ogival
and 27 present a cleIt palate. The mouth is kept open
because oI malIormation and the need to breathe through
it, secondary to choanae atresia. Skin is thick, greasy and
generally presents acne.
8,14-16
Clinical and imaging characteristics
Syndactyly is severe and disabling. Some oI these pre-
sentations are at skin level and others also involve bones.
They usually aIIect the second, third and Iourth digit on the
Iour limbs (type 1); more severe presentations are type 2
where the fIth fnger (pinky) is Iused or type 3 where the
fve fngers are Iused. Prognosis worsens as the number oI
Iused fngers increases. Some cases present a single nail
sinanchy. Prognosis is poorer depending on the number
oI digits that are Iused. Nail beds can also be Iused and
this condition is known as synonychia (Figure 3). There
are several bony malIormations involving vertebrae and
other bones.
8,14,17-19
Brain anomalies are Irequent with an almost-constant,
nonprogressive ventricular dilation that rarely requires
cerebrospinal fuid diversion. Patients present several
types oI cortical dysplasias, neuron migration Iailures,
corpus callosum and septal anomalies.
20,21
The presence oI
a cyst between septal laminae indicates a poorer prognosis,
as well as a dysIunctional impact on the Iamily. Onset
oI mental retardation has been reported in most cases;
patients with IQ ~90 (45.5) drop to 7.4 when they
are not decompressed beIore the frst year oI liIe. There
is hearing loss in 56 oI cases and ICH in 45 oI cases
Irom CHUMP series (Centre Hospitalier Universitaire des
EnIents Malades Necker de Paris).
8,11,12,14,22

Figure 1. Apert syndrome. (A) Lateral view. (B) Frontal view. Fre-
quently patients present brachycephaly trending to turricephaly ( ),
forehead is bulging (A), several eye proptosis levels can be observed
(), pterional and temporal regions bulge (), antimongoloid fold in
external commissure of eyelids (!), hypertelorism present at different
levels (>), upper maxillary and malar hypoplasia ("), open mouth
and inverted bite with setback of upper maxillary teeth, which lies
behind lower teeth (#).
Figure 2. Cranial tomography (3D reconstruction, lateral view).
(A) Apert. (B) Crouzon. Hypertelorism (*), less evident in Crouzon,
upper maxillary hypoplasia with several choanae atrophy levels (*),
dental articulation inversion because of upper maxillary setback (!).
(A) Shows persistent sagittal suture (+), bulging of pterional and
parietal areas () and bulging of frontal areas (A) because they are
generally brachycephaly crania (!").( B) Shows that generally
both coronal and sagittal sutures close (+) and brachycephaly is
less frequent (!").
BCE Vol. 68, November-December 2011
Craniosynostosis. . Analysis of syndromic craniosynostosis and different types of treatment
Saethre-Chotzen syndrome
Dehnition and epidemiologv
This entity was described by two German authors, Sae-
thre (1931) and Chotzen (1932), where there is a varible
craniosynostosis that may aIIect any suture, although it is
prevalent on coronal sutures.
Clinical and imaging characteristics
Both coronal sutures are usually closed, resulting in a fat
Iorehead and a straight nose in contrast with Crouzon`s
syndrome where patients present an aquiline nose. There
is palpebral ptosis, either unilateral or bilateral with hyper-
telorbitism at diIIerent levels. External ears are small and
round with crux cymba (helix continues in a transversal
Iold that crosses concha). Limbs are short and may pre-
sent a thickened thumb without pathological deviations.
A membranous syndactyly is Irequently Iound between
the Iorefnger and middle fnger and between the second
and third toes. Patients present hallux valgus and there is
a distal deIect in the terminal phalanges. IQ is compro-
mised when there is no cranial decompression beIore the
frst year oI liIe.
8,12,16,23
Pfeiffer syndrome
Dehnition and epidemiologv
This syndrome was recently described in 1964 by PIeiIIer
and is associated with brachycephaly, stenosis oI coronal
and sagittal sutures as well as membranous syndactyly in
hands and Ieet. This entity presents enlarged thumbs and
big toes with a clear varus deviation. These abnormalities
are because oI the triangular shape oI frst phalange and
hypertrophy oI frst metacarpal and metatarsal bones.
All oI the above are accompanied by brachydactyly and
elbow synostosis.
Clinical and imaging characteristics
Patients present upper maxillary hypoplasia, hypertelo-
rism, antimongolic tilt oI external eyelid commissures,
exorbitism (may be severe), with compromised eyelid
closure and strabismus secondary to exotropia due to
the same causes present in Crouzon`s syndrome. Pa-
tients also present several types oI brain problems such
as hydrocephaly, low-set cerebellar tonsils and venous
return anomalies because oI narrow jugular Ioramen.
Patients present low-set ears with upper maxillary hypo-
plasia, sometimes with choanal atresia, calcifcation oI
tracheal rings and vertebral, cervical and sacrococcygeal
malIormations.
PIeiIIer syndrome has been classifed according to three
subtypes: 1) Type 1, classic presentation, sporadic or au-
tosomal dominant; 2) Type 2, cloverleaI skull, Irequently
accompanied by brain malIormations; and 3) Type 3,
similar to type 2 but without cloverleaI skull.
8,24
Cloverleaf Skull
Dehnition and epidemiologv
This is a severe CS with an onset at birth where most
cranial vault sutures are stenosed, narrowing the temporo-
and Irontoparietal joints, bulging temporal, parietal and
occipital regions producing a cloverleaI skull (Figures
4-5).
25
Sometimes this CS is accompanied by a reticular
skull with bone spicules in the brain sulci producing severe
ICH (Figure 6).
A precise, prenatal diagnosis is recommended when
possible to evaluate surgical options that should be ca-
rried out as early as possible.
26
Early radical treatment
reduces sequelae produced by this malIormation. It can
be associated with Crouzon`s disease in its most severe
presentations as well as with Saethre-Chotzen, PIeiIIer,
Figure 3. Syndactyly in Apert syndrome. (A-C) Type 1: only three
central digits are fused both in hands and in feet.
BDF BoI Med Hosp Infant Mex
Fernando Chico Ponce de Len
Apert syndromes and with thanatophoric dysplasia; the
latter is incompatible with liIe.
27,28

Treatment
A multidisciplinary medical team must be responsible
Ior these patients and include neurosurgeons, plastic
surgeons, anesthesiologists, pediatricians, geneticists,
psychiatrists, psychologists, neurologists and neurora-
diologists specialized in cranioIacial malIormations. It
is also important to include ophthalmologists, maxillo-
Iacial surgeons, otorhinolaryngologists, orthodontists
and odontologists.
4,29,30
Team mandates should be to a) obtain a precise diag-
nosis with the clearest understanding oI dysmorphia;
b) understand Iunctional alterations as completely
as possible. Alterations may resemble others during
their early stages, such as scaphocephaly resembles
Crouzon`s disease; c) detect associated malIormations
(when a brain malIormation is associated, special
care should be exercised and classiIied); d) prepare
decompression and corrective surgery and e) prevent
intellectual and visual deterioration associated with
these entities.
29,31-33
SurgicaI Treatment
The purpose oI surgical treatment is to achieve brain de-
compression associated with most CS because oI chronic
ICH and, sometimes, papillary edema and atrophy as well
as neurological development retardation, decrease oI IQ
and mental disorders in some cases.
11,16,34,35
Physical-aes-
thetic aspect is also important because it will infuence the
child`s emotional development and correction oI physical
deIects will allow a better integration oI the patient in
society. Also, a correct cranial aspect will Iavor a positive
cranium-brain relationship.
36,37
Classic surgical treatment
Surgery is the elective treatment Ior most cranioIacial
malIormations and outcome will improve as diagnosis
is more accurate. Also, it is necessary to obtain a precise
clinical status, evaluating ICH, mental development and
ophthalmologic condition. AIIected organs should also be
assessed (mainly brain malIormations), especially Ior non-
syndromic CS and those associated with these problems
such as trigonocephaly and monosuture CS. Syndromic CS
may produce several types oI brain involvement.
Figure 4. Cloverleaf skull. This is a severe craniosynostosis where
all cranial vault sutures close and can be present as a single entity
or associated with Crouzon or Apert syndromes. This is a surgical
emergency because of brain compromise. Sunken parts match
stenosed sutures (#$) and there are several levels of exorbitism .
Figure 5. Cloverleaf skull CT (3D reconstruction). (A) Lateral view.
(B) Frontal view. Constraints can be observed at suture levels,
producing the characteristic cloverleaf shape (#$); stenosed sutures
can be identifed at constricted areas (%&%&%). Cloverleaf skull may
also present hypertelorism (*).
BDG Vol. 68, November-December 2011
Craniosynostosis. . Analysis of syndromic craniosynostosis and different types of treatment
Technical details
The Iollowing goals should be kept in mind:
Brain decompression.
Brain growth helps bone shaping within the Iirst 2-3
years oI age where cranium reaches ~80 oI adult
size.
38
Restore anatomy as close to normal as possible.
Provide the best aesthetic aspect according to pa-
tients` ethnic characteristics.
Surgery may be limited to only stenosed suture or a
Iull reshaping surgery can be carried out that will provide
a better outcome oI brain-cranium relationship and will
immediately improve the child`s physical aspect.
Extensive surgery is recommended because it has been
reported that aIter surgical correction oI malIormations
using wide reshaping techniques produces a substantial
intracranial volume increase.
33,39
In most cases, all CS (syndromic or nonsyndromic) will
require a Iorehead and orbit reshaping. We should have
special knowledge in regard to normal confgurations oI
these structures to shape them. Normal measurements,
according to age and aesthetic characteristics oI each
ethnic group, should be observed closely. Nowadays,
current tissue engineering techniques and the application
oI several materials allow a more eIfcient reshaping.
37,40-42

Bone fxation can be carried out using several elements
such as silk (not Irequently used because oI reactions) and
nylon and/or wire (more eIIective and better tolerated).
Metal plates and screws have also been used. Recently,
bioabsorbable systems have been successIully used.
43,44

Bone distraction systems both Ior simple CS as well
as Ior syndromic CS have been used as modern treatment
techniques.
40,41
Scaphocephaly
Surgical treatment
At Hospital InIantil de Mexico Federico Gomez (HIMFG) we
generally carry out ablation oI sagittal stenosed suture using
a 'bear skin technique also carrying out a devitalization
oI dura mater parallel to the sagittal sinus in order to Iavor
neosutures (Figure 7). This technique includes carrying out
trenches along coronal and lamboid sutures to normalize as
much as possible cranium shape, which will improve brain-
cranium relationship. Also, it is necessary to advance towards
the foor oI the temporal Iossa over the sphenoIrontal suture
at this level. When this zone is opened, a sphenoidal 'lock is
opened that allows a good postoperative evolution and a better
cranium reshaping. At the posterior part we reach asterion.
There are other techniques that have provided good
results with morbidity prophylaxis and a pleasing aspect.
Some authors propose suture resection with 3-cm margins
and relaxing incisures in parietal bones. In other cases,
simple linear craniectomy, a simple suturectomy is used
as well as calvariectomies.
29,31,32,45,46
Brachycephaly
Surgical treatment
Anteroposterior diameter should be enlarged through a
Ironto-orbital approach. Both Iorehead as well as upper
Figure 6. Cloverleaf skull x-ray. Reticular cranium is frequently ob-
served in cloverleaf skull cases. Reticula is a consequence of bone
disease and is complicated by severe intracranial hypertension that
may accompany these cases. Dotted line marks orbits. Constraints
at suture levels provide a characteristic cloverleaf shape (#$); some
fossas are circumscribed by reticula ( ).
*
BDH BoI Med Hosp Infant Mex
Fernando Chico Ponce de Len
section oI orbits are separated Irom the Iace and set at their
new position, advancing as necessary (usually 2 cm) as
shown in Figure 8. Pieces Irom this scaIIolding are joi-
ned as best as possible making an acceptable permanent
correction. This malIormation calls Ior urgent surgery
because oI Irequent ICH and its impact over eyeballs and
intellectual development.
34,35,47
Trigonocephaly
Surgical treatment
This entity is a special challenge Ior surgery. Movements
required by Iacial faps are due to malIormation confgu-
ration. Orbital edges are pulled backwards both in sagittal
and axial planes, producing pterional and anterior temporal
regions to set inwards. This should be corrected making
external ends oI orbital edges go Iorward at the same time,
tilted Iorward and downwards, accompanied by the middle
section oI the trigonocephalic area at the inIerior Irontal
bone, which is between orbits. In orbits, we should correct
hyptelorism and 'raccoon eyes, balancing the external
part oI the orbits downwards. The Irontal fap should co-
rrect the prominence by stenosed metopic suture, leaving
two faps in beetle-wing shape, which will be placed back
over orbit assembly (Figure 9).
29,48,49
Plagiocephaly
Surgical treatment
Correction should consider malIormation characteristics.
Orbit Irom stenosed side should be reset considering
that the malIormation presents at least three diIIerent
directions: orbit edge is misplaced on three spatial axes.
ModiIying articulations Irom both orbit edges will allow
proper adjustment oI orbit assembly and allow the brain
to slowly correct the malIormation. A discrete hyper-
correction is desirable.
50
MalIormation tends to relapse
postoperatively and, thereIore, this can be counterbalanced
using the aIorementioned hypercorrection. Partial outco-
mes with a certain degree oI malIormation necessitate
a waiting period oI 1-2 years beIore undergoing a new
surgery. Brain growth triggers, in most cases, cranial
reshaping aIter surgery (Figure 10).
29,33,36,51
DeIormed Iorehead can be managed by rotating a bone
fap or using the number oI required cuts, allowing brain
growth that will produce a better reshaping. Marchac et
al.
29
and Goodrich
36
recommend taking a skull Iragment
that contains the appropriate shape to rebuild the Iorehead,
using Marchac compass Ior this task.
Dura mater will receive the same treatment as in sca-
phocephaly, devitalizing the external sheet oI dura mater
with a mild coagulation at the stenosed suture site.
Other techniques have been described with unlatching
oI aIIected external orbit and its reposition aIter Irontal
craniotomy. Jimenez and Barone recommend endoscopic
surgery.
52,53,54
Syndromic Craniosynostoses: Crouzon and Apert
Syndromes
Surgical treatment
These malIormations pose a particular challenge frst
undertaken by Tessier in France and then continued in
Figure 7. Scaphocephaly: craniectomy in "bear-skin and perisin-
usoidal devitalization. (A) Scaphocephaly viewed from above. We
observe osteotomy in "bear-skin ( ) and neosutures (!). (B)
Scaphocephaly, lateral view. We observe osteotomy in "bear-skin
and cut sites over occipital bone in cases with a large protrusion
() and temporal fossa, especially over pterion ().
Figure 8. Brachycephaly: fronto-orbital advance technique. (A) Late-
ral view of brachycephaly. We observe decrease in antero-posterior
diameter (). B) Fronto-orbital advance planning. We observe cut
lines on bone (). (C) Advance. We observe frontal "bandeau and
obtained advance () can be viewed over nasal bridge as well as
the space that makes evident the advance of frontal concha () and
bone pieces that help keep the advance ().
BDB Vol. 68, November-December 2011
Craniosynostosis. . Analysis of syndromic craniosynostosis and different types of treatment
Mexico by Ortiz Monasterio and Fuente del Campo.
55

Whereas Tessier managed advances in two parts, Ortiz
Monasterio et al. proposed a monobloc technique that
revolutionized the approach to these malIormations. Cu-
rrently, monobloc bone cut is being used as well as bone
distractors, avoiding malIormation relapse because oI Iace
middle-third setback aIter surgery (Figure 11).
37,40,55,57-59
Endoscopic surgery
This approach has been used to manage nonsyndromic
CS, especially scaphocephaly. Published reports Irom the
end oI the 1990s make these techniques a saIe alternative
and with good quality outcomes.
12,23
However, more se-
rious studies need to be published to confrm that these
techniques are the most optimal to manage these entities.
Although these interventions require no blood transIusion
and a short hospital stay, adequate Iollow-up and relapse
percentage are necessary that will indicate with higher
certainty the eIIectiveness oI these techniques.
52-54
HeImets as Adjuvant Treatment
The use oI helmets to model a malIormed skull has been
another proposal to manage these entities. Although they
can be used as the primary treatment, they are usually
applied aIter surgery.
51
REFERENCES
1. Buchman SR, Muraszko KM. Syndromic craniosynostosis. n:
Lin KY, Ogle RC, Jane JA, eds. Craniofacial Surgery. Phila-
delphia: W.B. Saunders Company; 2002. pp. 252-271.
2. Crouzon A. Dysostose cranio-faciale hrditaire. Bull Mem
Soc Med Hop Paris 1912;33:545-555.
3. Posnick JC. Crouzon syndrome: evaluation and staging of
reconstruction. n: Posnick JC, ed. Craniofacial and Maxillofa-
cial Surgery in Children and Young Adults. Philadelphia: W.B.
Saunders Company; 2000. pp. 271-307.
Figure 10. Plagiocephaly. (A) Frontal and from above view (3D
reconstruction). Coronal suture absence ('''); "harlequin stenosed
orbit ("). (B) Surgical correction. Frontal fap: it is possible to turn it,
trying to fnd the best possible shape (A). Fronto-orbital advance on
affected side () should be pronounced, making a hypercorrection
that will usually even itself. This hypercorrection will be evident
on external pillar from affected orbit ((). Frontal bone fap can be
done using "daisy cuts (*). This fap joins the orbital correction
already performed ()). Orbit fap joins nasal bones (*) and keeps
its advance with the help of a bone scale (') that aids to keep its
side and provide strength.
Figure 11. Apert and Crouzon syndromes. (A) Before monobloc
advance. Cut lines for monobloc advance (*). Antimongolic fold
("). Setback and hypoplasia of the middle third (*). nverted bite
with lower teeth arc in front of upper teeth arc (*). (B) Surgical
correction with monobloc advance. Advance magnitude, trench
separating facial and cranial faps (). Frontal fap (A). Frontal
"Bandeau (). Monobloc piece, advanced (). Zygomatic bone
separated from malar bone (').
Figure 9. Trigonocephaly. (A) Frontal and from above view (3D
reconstruction) where we observe decrease in pterion diameter
("#) and stenosed metopic suture ()). (B) Surgical correction.
Ablation of stenosed suture (#), posterior fxation of frontal faps
in "beetle-wings (), skull bone band that holds advance (),
fxation of frontal faps to orbitary edge (*), orbitary fap fractured
in the middle and joined with angle correction for trigonocephaly ( ).
*
BDI BoI Med Hosp Infant Mex
Fernando Chico Ponce de Len
4. Urata M, Staffemberg DA, Kawamoto HK. Congenital facial
disorders. n: Goodrich JT, Staffenberg DA, eds. Plastic Tech-
niques in Neurosurgery. New York: Thieme Medical Publishers;
2004. pp. 94-110.
5. Kreiborg S, Cohen MM Jr. Ocular manifestations of Apert and
Crouzon syndromes: qualitative and quantitative fndings. J
Craniofac Surg 2010;21:1354-1357.
6. Limn-De Brown E, Ortiz-Monasterio F, Barrera G. Estrabismo
en enfermedad de Crouzon. Cir Plast berolatinoamericana
1979;5(suppl 1):209.
7. Renier D, Arnaud E, Marchac D. Les craniostnoses: physio-
pathologie. Neurochirurgie 2006;52:195-199.
8. Renier D, Arnaud E, Marchac D. Classifcation des craniost-
noses. Neurochirurgie 2006;52:200-227.
9. Cinalli G, Renier D, Sebag G, Saint-Rose C, Arnaud E, Pierre-
Kahn A. Chronic tonsillar herniation in Crouzon's and Apert's
syndromes: the role of the premature synostosis of the lamb-
doid suture. J Neurosurg 1995;83:575-582.
10. Cinalli G, Saint-Rose C, Kollar EM, Zerah M, Brunelle F, Chu-
mas P, et al. Hydrocephalus and craniosynostosis. J Neurosurg
1998;88:209-214.
11. Renier D, Arnaud E, Marchac D. Le retentissement fonctionnel
des craniostnoses. Neurochirurgie 2006;52:259-263.
12. Thompson DNP, Hayward RD. Craniosynostosis: pathophysio-
logy, clinical presentation, and investigation. n: Choux M, Di
Rocco C, Hockley A, Walker M, eds. Pediatric Neurosurgery.
London: Churchill Livingstone; 1999. pp. 275-290.
13. Apert E. De l'acrocphalosyndactylie. Bull Mem Soc Med Hop
Paris 1906;23:1310-1330.
14. Posnick JC. Apert syndrome: evaluation and staging of re-
construction. n: Posnick JC, ed. Craniofacial and Maxillofacial
Surgery in Children and Young Adults. Philadelphia: W.B.
Saunders Company; 2000. pp. 308-342.
15. Cohen MM Jr, Kreiborg S. Unusual cranial aspects of the Apert
syndrome. J Craniofac Genet Dev Biol 1994;14:48-56.
16. Renier D, Capon-Degardin N, Arnaud E, Marchac D. Diagnostic
des craniostnoses. Neurochirurgie 2006;52:238-245.
17. Hemmer KM, McAlister WH, Marsh JL. Cervical spine ano-
malies in the craniosynostosis syndromes. Cleft Palate J
1978;24:328-333.
18. Journeau P, Lajeunie E, Rnier D, Salon A, Guro S, Pouli-
quen JC. Syndactyly in Apert syndrome. Utility of a prognostic
classifcation. Ann Chir Main Memb Super 1999;18:13-19.
19. Kreiborg S, Barr M Jr, Cohen MM Jr. Cervical spine in the Apert
syndrome. Am J Med Genet 1992;43:704-708.
20. Saavedra-Ontiveros MD, Morn-Barroso VF. Perspectivas del
genoma humano en las malformaciones congnitas. . Aspec-
tos clnicos en craneosinostosis. Gac Md Mx 2003;139:236-
239.
21. cal E, Sun PP, Persing JA. Craniosynostosis. n: Albright
AL, Pollack F, Adelson PD, eds. Principles and Practice of
Pediatric Neurosurgery. New York: Thieme Medical Publishers;
1999. pp. 219-242.
22. Renier D, Arnaud E, Cinalli G, Sebag G, Zerah M, Marchac D.
Prognosis for mental function in Apert's syndrome. J Neurosurg
1996;85:66-72.
23. Tutino M, Chico F, Ortiz-Monasterio F. Endoscopic dissection
of dura and craniotomy with minimal trephines: a preliminary
series. J Craneofac Surg 1998;9:154-161.
24. Posnick JC. Pfeiffer syndrome: evaluation and staging of re-
construction. n: Posnick JC, ed. Craniofacial and Maxillofacial
Surgery in Children and Young Adults. Philadelphia: W.B.
Saunders Company; 2000. pp. 343-353.
25. Cohen MM Jr. Cloverleaf skulls: etiologic heterogeneity and pa-
thogenetic variability. J Craniofac Surg 2009;20(suppl 1):652-
656.
26. Zuccaro G, Dogliotti P, Bennum R, Monges J. Treatment of
cloverleaf skull syndrome. Childs Nerv Syst 1996;12:695-698.
27. Posnick JC. Cloverleaf skull anomalies: evaluation and staging
of reconstruction. n: Posnick JC, ed. Craniofacial and Maxi-
llofacial Surgery in Children and Young Adults. Philadelphia:
W.B. Saunders Company; 2000. pp. 354-366.
28. Rodrguez Nava P, Dena Espinoza EJ, Hernndez Garduo
A, Fuentes Garca M, Basile Lengue R, Amescua Herrera C,
et al. Craniosinostosis multisutural del tipo crneo en trbol
o Kleeblattschdel y otras craenoestenosis. Ann Radiologa
Mxico 2007;3:219-231.
29. Arnaud E, Marchac D, Renier D. Le traitement fonctionnel
des craniostnoses: indications et techniques. Neurochirurgie
2006;52:264-291.
30. Meyer P, Cuttaree H, Blanot S, Orliaguet G, Jarreau MM,
Charron B, et al. L'anesthsie-ranimation dans le traitement
des craniostnoses. Neurochirurgie 2006;52:292-301.
31. Bellew M, Chumas P, Mueller R, Liddington M, Russell J.
Pre- and postoperative developmental attainment in sagittal
synostosis. Arch Dis Child 2005;90:346-350.
32. Bellew M, Liddington M, Chumas P, Russell J. Preoperative
and postoperative developmental attainment in patients with
sagittal synostosis: 5-year follow-up. J Neurosurg Pediatr
2011;7:121-126.
33. Renier D, Arnaud E, Marchac D. Craniostnoses: rsultats
fonctionnels et morphologiques post-operatoires. Neurochi-
rurgie 2006;52:302-310.
34. Renier D, Saint-Rose C, Marchac D. ntracranial pressure in
craniostenosis. 302 recordings. n: Marchac D, ed. Craniofacial
Surgery. Berlin: Springer; 1987. pp. 110-113.
35. Renier D. ntracranial pressure in craniosynostosis: pre- and
postoperative recordings. Correlation with functional results.
n: Persing JA, Edgerton MT, Jane JA, eds. Scientifc Founda-
tions and Surgical Treatment of Craniosynostosis. Baltimore:
Williams Wilkins; 1989. pp. 263-269.
36. Goodrich JT. Craniofacial reconstruction for craniosynostosis.
n: Goodrich JT, Staffenberg DA, eds. Plastic Techniques in
Neurosurgery. New York: Thieme Medical Publishers; 2004.
pp. 56-93.
37. Ortiz-Monasterio F. Molina F. Ciruga esttica del esqueleto
facial. Mxico D.F.: Editorial Mdica Panamericana; 2005.
38. Blinkov SM, Glezer . The Human Brain in Figures and Tables.
A uantitative Handbook. New York: Plenum Press; 1968.
39. Navas-Aparicio MC, Segura-Valverde JL. Anlisis del creci-
miento craneal posterior a la correccin quirrgica en nios con
craneosinostosis en el Hospital Nacional de Nios "Dr. Carlos
Senz Herrera, Caja Costarricence de Seguro Social, durante
el perodo 1997-2004. Rev Chil Neurociruga 2009;32:11-15.
40. Fuente del Campo A. A simplifed bone distractor for induced
osteogenesis. Plast Reconstr Surg 2002;110:1485-1491.
41. Hollinger JO, Winn SR, Sfeir C, Calvert JW, Schmitz JP. Bone
regenerating options for the craniofacial skeleton. n: Lin KY,
BDJ Vol. 68, November-December 2011
Craniosynostosis. . Analysis of syndromic craniosynostosis and different types of treatment
A, et al. Plagiocefalia anterior: descripcin de tcnica quirr-
gica y revisin de la literatura de las opciones en el manejo
neuroquirrgico. Rev Hosp Jua Mex 2009;76:88-93.
51. Mottolese C, Szathmari A, Ricci AC, Ginguene C, Simon E,
Paulus C. Plagiocphalies positionnelles: place de l'orthse
crnienne. Neurochirurgie 2006;52:184-194.
52. Jimnez DF, Barone CM. Endoscopic techniques for craniosynos-
tosis. Atlas Oral Maxillofac Surg Clin North Am 2010;18:93-107.
53. Jimnez DF, Barone CM. Multiple-suture nonsydromic cranios-
ynostosis: early and effective management using endoscopic
techniques. J Neurosurg Pediatr 2010;5:223-231.
54. Jimnez DF, Barone CM. The role of endoscopy in craniofacial
surgery. n: Lin KY, Ogle RC, Jane JA, eds. Craniofacial Sur-
gery. Philadelphia: W.B. Saunders Company; 2002. pp. 173-
187.
55. Ortiz-Monasterio F, Fuente-Del Campo A, Carrillo A. Recons-
tructive surgery for Crouzon's disease and Apert's syndrome.
n: Converse JM, MacCarthy JG, Wood-Smith D, eds. Sympo-
sium on Diagnosis and Treatment of Craneofacial Anomalies.
New York: CV Mosby; 1979. pp. 370-384.
56. Tutino M, Chico F, Tutino MA, Goodrich JT, Ortiz-Monasterio
F. Endoscopic intracranial, craniofacial and monobloc osteo-
tomies with the aid of a malleable high-speed pneumatic drill:
a cadaveric and clinical study. Ann Plast Surg 2000;44:1-7.
57. Ortiz-Monasterio F, Fuente-Del Campo A, Carrillo A. Advan-
cement of the orbits and the midface in one piece, combined
with frontal repositioning, for the correction of Crouzon's
deformities. Plast Reconstr Surg 1978;61:507-516.
58. Ortiz-Monasterio F. Surgical correction of Crouzon's deformity.
n: Brent B, ed. Artistry of Reconstructive Surgery: Selected
Classic Case Studies. St. Louis, MO: CV Mosby; 1983.
59. Ortiz-Monasterio F, Fuente-Del Campo A. Refnements on
the monobloc orbitofacial in advancement. n: Caronni E, ed.
Craniofacial Surgery. Boston: Little-Brown; 1985. pp. 263.
Ogle RC, Jane JA, eds. Craniofacial Surgery. Philadelphia:
W.B. Saunders Company; 2002. pp. 113-131.
42. Molina F, Ortiz-Monasterio F. Hemifacial microsomia and
Goldenhar syndrome. n: Lin KY, Ogle RC, Jane JA, eds.
Craniofacial Surgery. Philadelphia: W.B. Saunders Company;
2002. pp. 272-287.
43. Fuente del Campo A, Garca-Gutirrez E. Tratamiento quirr-
gico de las craneodisostosis con mtodo de fjacin interna.
Bol Med Hosp nfant Mex 1993;50:225-233.
44. Muoz-Casado MJ, Romance A, Garca-Recuero J. Bioab-
sorbable osteofxation devices in craniosynostosis. Clinical
experience in 216 cases. Neurociruga (Astur) 2009;20:255-261.
45. Delgado-Hernndez C, Mendizabal-Guerra R, Muoz-Bellizzia
JA, Soto-Barraza JC, Ayala-Arcipreste A, Acosta-Garcs R, et
al. Escafocefalia: descripcin de la tcnica quirrgica y revisin
de la literatura de las opciones en el manejo neuroquirrgico.
Rev Hosp Jua Mex 2008;75:213-217.
46. Posnick JC. Scaphocephaly: sagittal synostosis. n: Posnick
JC, ed. Craniofacial and Maxillofacial Surgery in Children and
Young Adults. Philadelphia: W.B. Saunders Company; 2000.
pp. 199-230.
47. Posnick JC. Brachicephaly: bilateral coronal synostosis without
midface defciency. n: Posnick JC, ed. Craniofacial and Maxi-
llofacial Surgery in Children and Young Adults. Philadelphia:
W.B. Saunders Company; 2000. pp. 249-268.
48. Dhellemmes P, Pellerin P, Lejeune P, Lepoutre F. Surgical
treatment of trigonocephaly. Experience with 30 cases. Childs
Nerv Syst 1986;2:228-232.
49. Posnick JC. Trigonocephaly: metopic synostosis. n: Posnick
JC, ed. Craniofacial and Maxillofacial Surgery in Children and
Young Adults. Philadelphia: W.B. Saunders Company; 2000.
pp. 162-198.
50. Delgado-Hernndez C, Mendizabal-Guerra R, Delgado-Ochoa
MA, Muoz-Bellizzia JA, Jimnez-Jimnez M, Monroy Sosa