1190 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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• Figure 30-2 A child who was born with
Apert syndrome underwent a bilateral lateral
canthal advancement procedure when she
was 6 weeks old; this was carried out by a
neurosurgeon working independently. When
she was 18 months old, she returned with
turricephaly and a constricted anterior cranial
vault that required further cranio–orbital
decompression and reshaping. When she was
5 years old, she underwent anterior cranial
vault and facial bipartition osteotomies with
reshaping. As part of the staged reconstruc
tion, she will require orthognathic surgery and
orthodontic treatment during her teenage
years. A, Illustrations of craniofacial morphol
ogy before and after the facial bipartition oste
otomies and reconstruction. B, Frontal views
before and after facial bipartition reconstruc
tion. C, Oblique facial views before and after
facial bipartition reconstruction.
1191
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
F
D
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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D, Profile views
before and after facial bipartition recon
struction. E, Worm’s-eye views before
and after facial bipartition reconstruc
tion. F, Intraoperative views after facial
bipartition and anterior cranial vault
reconstruction.
• Figure 30-2, cont’d
Continued
1192 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
G
H
G and H, Computed tomography scan views before and early after
reconstruction.
• Figure 30-2, cont’d
1193
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
I
J
I, Axial computed tomography scan views through the zygomas before and
after reconstruction. J, Axial computed tomography scan views through the midorbits before and after
reconstruction indicating relief of proptosis.
• Figure 30-2, cont’d
1194 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
B
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A
©Elsevier Inc.
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• Figure 30-3 A 5-year-old girl with Apert
syndrome who underwent lateral canthal
advancements when she was 6 months old
and in the care of another surgeon. She then
presented to this surgeon with residual cranio
facial deformities that required anterior cranial
vault and facial bipartition osteotomies with
reshaping. She will require orthognathic
surgery and orthodontic treatment during her
teenage years to complete the reconstruction.
A, Illustrations of preoperative craniofacial
morphology. The planned cranial vault and
facial bipartition osteotomies and the planned
reshaping are also shown. B, Frontal facial
views before and after anterior cranial vault
and facial bipartition reconstruction. C, Profile
views before and after facial bipartition
reconstruction.
1195
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
E
F
D
D, Intraoperative views
after anterior cranial vault and facial bipartition
reconstruction. E, Axial computed tomography
scan views through the midorbits before and
after reconstruction that demonstrate improve
ment in orbital hypertelorism and orbital depth
with diminished eye proptosis. F, Axial com
puted tomography scan views through the
cranial vault 1 week after facial bipartition (note
the dead space in the retrofrontal region) and
at 1 year (note that the initial retrofrontal dead
space has been resolved by brain expansion).
A (right), C (left), E, From Posnick JC: Cranio
facial dysostosis. Staging of reconstruction
management of the midface deformity, Neuro
surg Clin North Am 2:683-702, 1991.
• Figure 30-3, cont’d
1196 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 30-4 An 8-year-old girl who was born with Apert syndrome. She had undergone cranio–orbital surgery on two occasions and an attempted
Le Fort III osteotomy at another institution. The previous procedures had resulted in complications, including infection and traumatic encephalocele.
She was referred to this surgeon with cranial vault dysplasia, orbital dystopia, hypertelorism, midface deficiency, and skull defects. She then under
went anterior cranial vault, facial bipartition, and additional segmental orbital osteotomies with reshaping and reconstruction. She will require
orthodontic treatment and orthognathic surgery during her teenage years to complete the reconstruction. A, Frontal views before and after anterior
cranial vault and facial bipartition reconstruction. B, Oblique facial views before and after facial bipartition reconstruction.
1197
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
D
C
C, Intraoperative views after anterior cranial vault and facial bipartition reconstruction.
Encephalocele and skull defects have also been repaired. D, Computed tomography scan views before and early
after reconstruction. From Posnick JC: Craniosynostosis: surgical management in infancy. In Bell WH, ed: Orthog
nathic and reconstructive surgery, vol 3, Philadelphia, 1992, W.B. Saunders, p 1839.
• Figure 30-4, cont’d
1198 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
B
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©Elsevier Inc.
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osnicick
• Figure 30-5 A child born with a mild form of Apert syndrome. When he was 10 years old, he was referred to this surgeon and underwent
anterior cranial vault and monobloc osteotomies with advancement. He will require orthognathic surgery combined with orthodontic treatment
to complete the reconstruction. A, Facial views during infancy. B, Views of the hands and feet that indicate complex compound syndactyly.
1199
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Frontal facial views before and after anterior cranial vault and monobloc osteotomies with advancement. D, Profile views
before and after monobloc reconstruction.
• Figure 30-5, cont’d
Continued
1200 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
canals, and cochlear dysplasia.285 Otitis media is common,
and it is possibly related to the presence of cleft palate and
accompanying eustachian tube dysfunction.
Oral and maxillofacial anomalies can also be present.
The palate can be highly arched and constricted with a
median furrow in up to 94% of patients. The hard palate
is often shorter than normal, whereas the soft palate is
longer and thicker than normal. Clefting of the soft palate
occurs in 41% of patients, and a bifid uvula is present in
35%. Dental anomalies include severely delayed eruption
(68%), ectopic eruption (50%), and shovel-shaped incisors
(30%). Dental crowding is more severe in the maxilla than
in the mandible. Common dental deformities include
anterior open bite (73%), posterior crossbite (63%), and
mandibular overjet (81%).46,48
Ocular findings include hypertelorism, proptosis
(often asymmetric), and down-slanting palpebral fissures.
The presence of V-pattern strabismus, which is marked by
divergent upgaze and esotropic downgaze, is common
and secondary to structural abnormalities of the extraocu
lar muscles (i.e., the absence of the superior rectus
muscle). Hyperopia, myopia, and astigmatism can also
be present. Strabismus and significant refractive errors
can sometimes cause amblyopia. Proptosis in Apert syn
drome may require tarsorrhaphies to prevent exposure
keratitis.131
Hearing loss occurs in 90% of these patients, with 80%
of cases resulting from conductive pathology. Inner-ear
anomalies are found in all patients; these most commonly
present as dilated vestibules, malformed semicircular
E
F
E, Axial computed tomography scan views through the midorbits before and
after reconstruction indicating relief of proptosis. F, Axial computed tomography scan views through
the zygomatic arches before and after reconstruction.
• Figure 30-5, cont’d
1201
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
*References 9, 12, 27, 51-53, 59, 61, 78, 83, 84, 90, 95, 98, 100, 118,
126, 127, 130, 163, 224, 225, 237, 238, 270, 271
Crouzon Syndrome
Crouzon syndrome is characterized by craniosynostosis,
maxillary hypoplasia, shallow orbits, and ocular proptosis.*
(Figs. 30-6 through 30-11). Most cases are associated with
brachycephaly secondary to bicoronal synostosis; however,
trigonocephaly, scaphocephaly, and cloverleaf skull have
also been noted. Cranial malformation depends on the
order and rate of progression of the sutural synostosis. The
cranial volume of patients with Crouzon syndrome is much
smaller than that found in patients with Apert syndrome.
Central nervous system findings include shunted hydro
cephalus (25%), chronic tonsillar herniation (72%), jugular
foramen stenosis with venous obstruction (30%), seizures
(10.5%), frequent headaches (29%), and mental deficiency
(3%).46,48 As a result of severe maxillary deficiency, patients
should be monitored for snoring and excess daytime som
nolence. If this is severe during infancy, tracheostomy may
be the only option. Older children should be initially
worked up with a polysomnographic study. Treatment
options range from CPAP therapy to midface advancement
on the basis of the severity level.46
Cervical vertebral anomalies are found in 25% of patients
with Crouzon syndrome, and all involve C2 and C3.
Anomalies should be assessed before the patient undergoes
anesthesia for any craniofacial procedure. Patients with
Crouzon syndrome already have problematic airways as a
result of their relatively inflexible necks; thus, cervical
anomalies will compound the issue.46
Ophthalmologic abnormalities include hypertelorism,
pronounced ocular proptosis, exotropia, exposure keratitis,
poor vision, and optic atrophy. Luxation of the eye globes
may be observed in some instances. Although emergency
reduction followed by tarsorrhaphies may be necessary,
some patients can luxate and reduce all by themselves.131
Mild to moderate conductive hearing deficit occurs in
55% of patients, and atresia of the external auditory meatus
is found in 13%. Otitis media is common, and middle-ear
disease may be related to the presence of cleft palate and
associated eustachian tube dysfunction
Oral findings include lateral palatal swellings (50%),
obligatory mouth breathing (32%), bifid uvula (9%), and
cleft palate (9%). The maxillary dental arch is shortened
and associated with a posterior crossbite, dental crowding,
and ectopic eruption of the maxillary first molars. Anterior
open bite, mandibular overjet, and crowding of the man
dibular anterior teeth are also common.46
Pfeiffer Syndrome
Pfeiffer syndrome is characterized by craniosynostosis;
midface deficiency; broad thumbs, great toes, or both;
brachydactyly; variable soft-tissue syndactyly; and other
anomalies (Figs. 30-12 through 30-15).188,231 Three clinical
subtypes have been proposed. Type 1 is representative of the
aforementioned description. Type 2 is characterized by clo
verleaf skull, elbow ankylosis or synostosis, and a cluster of
unusual anomalies in addition to the Type 1 characteristics.
Type 3 involves a very short cranial base, severe ocular
proptosis, elbow ankylosis or synostosis, and an assortment
of unusual anomalies. Types 2 and 3 also include an
increased risk for neurodevelopmental difficulties. A favor
able outcome can be achieved in some cases with aggressive
medical and surgical management; however, normal
outcome is not the rule, and neurodevelopmental outcome
and life expectancy prognoses remain guarded in most
cases.44 Although these subtypes are useful, they have limited
nosologic status, and some clinical overlap does occur.
Craniofacial features include brachycephaly as a result of
bicoronal synostosis, hypertelorism, ocular proptosis, and
midface deficiency.44 Distortion ventriculomegaly, progres
sive hydrocephalus, and cerebellar herniation are common.
Progressive hydrocephalus is much more common among
patients with Pfeiffer and Crouzon syndromes than it is
among those with Apert syndrome. Gyral abnormalities
have been observed in some cases.44 Vertebral anomalies
occur in 70% of patients, particularly at C2 and C3, but
such anomalies may involve other cervical vertebrae as well
as the lumbar vertebrae in some cases. 46 As in Apert and
Crouzon syndrome, OSA can affect patients with Pfeiffer
syndrome. For a further understanding of diagnosis and
therapy, please refer to the relevant discussions in the
sections of this chapter about the Apert and Crouzon syn
dromes. Other anomalies have been noted in some cases,
including cardiovascular defects, gastrointestinal issues, and
genitourinary anomalies.44
Saethre–Chotzen Syndrome
Saethre–Chotzen syndrome is characterized by a heteroge
neous phenotypic presentation that involves craniosynosto
sis, a low-set frontal hairline, facial asymmetry, ptosis of the
eyelids, a deviated nasal septum, brachydactyly, partial soft-
tissue syndactyly of the second and third fingers, and various
skeletal anomalies.4,33,40,234 Craniosynostosis is facultative
and not obligatory (i.e., some patients do not have it).
When it is present, the time of onset and the degree of
craniosynostosis can be quite variable. Brachycephaly is
found most commonly, and this is followed by synostotic
anterior plagiocephaly. Frontal bossing, parietal bossing,
and occipital flattening can also accompany the cranial
deformity. Large late-closing fontanelles and large parietal
foramina have also been reported. Ptosis of the eyelids,
hypertelorism, and strabismus are common. The ears may
be low set, small, and posteriorly angulated, and mild con
ductive hearing loss is common. Oral anomalies include a
narrow or highly arched palate, a cleft palate on occasion,
malocclusion, and supernumerary teeth.40
Text continued on p. 1226
1202 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
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©Elsevier Inc.
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©Elsevier Inc.
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g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
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©Elsevier Inc.
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• Figure 30-6 An 8-year-old boy who was born with
Crouzon syndrome and who underwent a first-stage cranio–
orbital procedure when he was 6 weeks old by a neurosur
geon who was working independently. He was referred
to this surgeon and then underwent anterior cranial vault
and monobloc osteotomies with advancement. A, Illustra
tion of craniofacial morphology before and after anterior
cranial vault and monobloc osteotomies with advancement.
B, Profile views before and after reconstruction. C, Frontal
views before and after anterior cranial vault and monobloc
reconstruction.
1203
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
D
E
F
D, Occlusal views before and after reconstruction. Note the improvement but without a full correction of
the malocclusion; this will await orthognathic correction during the patient’s teenage years. E, Intraoperative view of disimpaction
after monobloc osteotomy with the use of nasomaxillary forceps. F, Intraoperative view of monobloc showing tenon extension
and zygomatic arch advancement before and after plate and screw fixation.
• Figure 30-6, cont’d
Continued
1204 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
H
G
G, After monobloc advancement and fixation, dead space in the retrofrontal region with communication
into the nasal cavity can be seen. Pericranial flaps are elevated from under the surface of the scalp for the closure of cranio–nasal
communication. H, Axial computed tomography scan views through the midorbits before and after reconstruction. Reconstruction
resulted in increased intraorbital depth and decreased proptosis. A, B, C ,D, E, H, From Posnick JC: Craniosynostosis: surgical
management of the midface deformity. In Bell WH, ed: Orthognathic and reconstructive surgery, vol 3, Philadelphia, 1992, W.B.
Saunders, p 1889.
• Figure 30-6, cont’d
Orthognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 30-7 A 6-year-old girl who was born with Crouzon syndrome and who underwent anterior cranial vault and monobloc osteotomies with
advancement. A, Frontal facial views in repose before and after anterior cranial vault and monobloc reconstruction. B, Frontal views with smile
before and after monobloc reconstruction.
Continued
1206 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Profile views before and after monobloc reconstruction. D, Worm’s-eye views before and after monobloc
reconstruction.
• Figure 30-7, cont’d
1207
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
F
G
E
E, Occlusal views before and after reconstruction. Note the improvement but without the correction of
the malocclusion; this will await orthognathic surgery during the patient’s teenage years. F and G, Computed tomography scan
views immediately after reconstruction that indicate advancement with increased volume in the anterior cranial vault and orbits.
A, C, D, From Posnick JC: Craniosynostosis: surgical management of the midface deformity. In Bell WH, ed: Orthognathic and
reconstructive surgery, vol 3, Philadelphia, 1992, W.B. Saunders, p 1888.
• Figure 30-7, cont’d
1208 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
A
©Elsevier Inc.
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©Elsevier Inc.
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• Figure 30-8 A child who was born with Crouzon syndrome and who underwent bilateral
coronal suture release when she was 3 months old. Additional craniotomy and cranial vault
reshaping were completed when she was 9 months old. When the patient was 2 years old,
she underwent a Le Fort III midface osteotomy and forehead advancement procedure
through an intracranial approach. She presented to this surgeon when she was 14 years
old with residual deformity for which she underwent simultaneous anterior cranial vault,
monobloc, Le Fort I, and chin osteotomies with differential advancements. A, Profile facial
views at 1 year of age and at 2 years of age after Le Fort III and anterior cranial vault
advancement.
1209
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
C
B
Anterior cranial vault
15-mm advancement
Monobloc
12-mm advancement
Le Fort I
17-mm advancement
Genioplasty
5-mm advancement
©Elsevier Inc.
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B, Illustration of planned and completed anterior cranial vault, monobloc, Le Fort I, and chin osteotomies.
C, Profile view and lateral cephalometric radiograph before surgery. Articulated dental casts after model planning that indicate that 17-mm
advancement is required at the occlusal level. The amount of advancement required at the supraorbital ridge level was just 12 mm. Note
the absence of permanent molars in maxilla; this is the result of the Le Fort III osteotomy carried out when the patient was 2 years old.
The pterygomaxillary disjunction destroyed the developing teeth.
• Figure 30-8, cont’d
Continued
1210 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
D
E
F
©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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gnatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
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©Elsevier Inc.
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C Pos
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D, Frontal facial views before
and after reconstruction. E, Oblique facial views
before and after reconstruction. F, Profile views
before and after reconstruction.
• Figure 30-8, cont’d
1211
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
G
H
I
Molars injured during
early LeFort III
G, Occlusal views before and after reconstruction and orthodontic treatment. H, Lateral cephalometric
radiographs before and after reconstruction. I, Soft-tissue lateral cephalometric radiographs before and after reconstruction.
• Figure 30-8, cont’d
Continued
1212 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
J
K
J, Axial computed tomography scan views through the midorbits before and
after reconstruction confirm relief of proptosis. K, Axial computed tomography scan views through
the zygomatic arches before and after reconstruction. A, B (right), D, G, H (left), I, J, From Posnick
JC: Craniosynostosis: surgical management of the midface deformity. In Bell WH, ed: Orthognathic
and reconstructive surgery, vol 3, Philadelphia, 1992, W.B. Saunders, p 1888.
• Figure 30-8, cont’d
1213
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
©Elsevier Inc.
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A
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• Figure 30-9 A 12-year-old boy with unrepaired Crouzon syndrome was referred to this surgeon for evaluation. He underwent total cranial vault
and monobloc osteotomies with reshaping and advancement. A, Facial and computed tomography scan views before surgery. B, Profile facial
view before surgery. Illustrations of the patient’s craniofacial morphology with planned osteotomy locations indicated. A second illustration is shown
after the proposed osteotomies were completed, with reshaping and advancement.
Continued
1214 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Frontal facial views before and after reconstruction. D, Worm’s-eye views before and after reconstruction.
• Figure 30-9, cont’d
1215
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
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E, Profile views before and after reconstruction. F, Intraoperative views before
and after total cranial vault and monobloc osteotomy with reshaping and advancement. G, Computed
tomography scan views before and immediately after reconstruction. A (right), B (left, right), C (right),
D (right), E (right), F, G, From Posnick JC: Craniosynostosis: surgical management of the midface defor
mity. In Bell WH, ed: Orthognathic and reconstructive surgery, vol 3, Philadelphia, 1992, W.B. Saunders,
p 1888.
• Figure 30-9, cont’d
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 30-10 A 15-year-old girl with Crouzon syndrome that is characterized by mild to moderate midface deficiency with retrusion of the
infraorbital rims, the zygomatic buttresses, and the maxilla. The midface hypoplasia results in increased scleral show, nasal obstruction, and maloc
clusion. The cranial vault and the upper orbits have normal morphology. Orthodontic camouflage treatment was in progress, including mandibular
bicuspid extractions with retraction. The patient was referred to this surgeon for evaluation and treatment. She then underwent a Le Fort III extra
cranial osteotomy with advancement. A, Frontal facial views before and after reconstruction. B, Profile views before and after reconstruction.
1217
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
D
C
Orth
r ognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
C, Worm’s-eye views before and after reconstruction. D, Occlusal views before and after
reconstruction.
• Figure 30-10, cont’d
Continued
1218 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
G
E
F
E, Intraoperative view.
Note the potential for unsightly step-off at the
lateral orbital rim after advancement. F, Cranial
bone graft is placed in the frontonasal region.
Note the unavoidable lengthening of the nose
and the potential for the flattening of the
nasofrontal angle that occurs as part of the Le
Fort III advancement. G, Lateral cephalometric
radiographs before and after reconstruction.
A, B (right), C, D, E, F (left), G, from Posnick JC:
Craniosynostosis: surgical management of the
midface deformity. In Bell WH, ed: Orthognathic
and reconstructive surgery, vol 3, Philadelphia,
1992, W.B. Saunders, p 1888.
• Figure 30-10, cont’d
1219
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
C
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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• Figure 30-11 A 19-year-old boy who was born
with Crouzon syndrome. When he was 11 years old,
he underwent a Le Fort III osteotomy with advance
ment via an extracranial approach by another
surgeon. He presented to this surgeon during his late
teenage years with asymmetric and dystopic orbits,
zygomatic asymmetry, a retrusive upper jaw, an
asymmetric lower jaw, and a vertically long chin. He
underwent a combined orthodontic (upper bicuspid
extractions) and surgical approach. The procedures
included Le Fort I osteotomy (horizontal advance
ment); bilateral sagittal split ramus osteotomies (cor
rection of asymmetry); and osseous genioplasty
(vertical reduction and horizontal advancement). He
also underwent the reopening of a coronal scalp
incision with the harvesting of split cranial grafts and
the recontouring and augmentation of the orbits and
the zygomas. A, Frontal views before and after
reconstruction. B, Oblique facial views before and
after reconstruction. C, Profile views before and after
reconstruction.
Continued
1220 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
E
Pre surgery with maxillary bicuspid extractions
After treatment
D
D, Occlusal views before and after reconstruction. E, Articulated dental casts that indicate analytic model
planning.
• Figure 30-11, cont’d
1221
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
B
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A
©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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• Figure 30-12 A 7-year-old boy who was born with
Pfeiffer syndrome was referred for evaluation. He
previously had undergone bilateral lateral canthal
advancements when he was 3 months old; these
were performed by a neurosurgeon who was working
independently. The patient then required the place
ment of a ventriculoperitoneal shunt. He presented
to this surgeon with total cranial vault dysplasia,
orbital dystopia, and midface deficiency. He under
went cranial vault and monobloc osteotomies with
reshaping and advancement. A, An illustration of the
proposed cranial vault and monobloc osteotomies
and reconstruction is also shown. B, Frontal facial
views in repose before and after cranial vault and
monobloc reconstruction. C, Frontal facial views with
smile before and after reconstruction.
Continued
1222 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
F
D
E
©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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©Elsevier Inc.
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D, Profile views before
and after reconstruction. E, Worm’s-eye views
before and after reconstruction. F, Lateral
cephalometric radiographs before and after
reconstruction. A (left), B, D, from Posnick JC:
Craniofacial dysostosis: staging of reconstruc
tion and management of the midface deformity,
Neurosurg Clin North Am 2:683-702, 1991.
• Figure 30-12, cont’d
1223
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
• Figure 30-13 A 6-year-old girl who was
born with Pfeiffer syndrome. She had under
gone cranio–orbital reshaping earlier during
her childhood. She presented to this surgeon
with a constricted anterior cranial vault, orbital
dystopia, and midface deficiency. She under
went anterior cranial vault and monobloc oste
otomies with reshaping and advancement.
A, Frontal facial views before and after recon
struction. B, Profile views before and after
reconstruction. She still requires orthodontic
treatment and orthognathic surgery, which are
planned for her teenage years. C, Axial com
puted tomography scan views through the
midorbits before and after reconstruction.
C
A
B
©Elsevier Inc.
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Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 30-14 A 12-year-old girl who was born with Pfeiffer syndrome was referred to this surgeon for evaluation. She underwent anterior cranial
vault and facial bipartition osteotomies with reshaping. She is shown before and after reconstruction. Quantitative analysis of the presenting deformity
on the basis of computed tomography scan views confirmed the abnormal cranial vault length (85% of normal), medial orbital wall length (68% of
normal), and zygomatic arch length (83% of normal) as well as the extent of globe protrusion (140% of normal). All measurements demonstrated
horizontal deficiency in the upper face and the midface. In addition, the anterior interorbital distance (136% of normal), the mid-interorbital distance
(129% of normal), the lateral orbital distance (121% of normal), and the intertemporal distance (132% of normal) all indicated a degree of upper-
face hypertelorism. The patient then underwent anterior cranial vault and facial bipartition osteotomies for reconstruction. She achieved improved
horizontal facial depth, and the upper face hypertelorism was also improved. A, Frontal facial views in repose before and after reconstruction.
B, Frontal facial views with smile before and after reconstruction.
1225
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C
D
C, Oblique facial views before and after reconstruction. D, Profile views before and after reconstruction.
• Figure 30-14, cont’d
Continued
1226 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
F
E
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
low-set ears, and a short neck. The mandible may be hypo
plastic, and the palate may be narrow or highly arched.39
Neurologic Aspects of Craniosynostosis
Generally, craniosynostosis tends to restrict intracranial
volume. The earlier synostosis occurs, the more dramatic
the effect on subsequent cranial growth and development.
The later synostosis occurs, the less the effect on cranial
growth and development. Growth restriction also worsens
with increasing sutural involvement; complex craniosynos
tosis with two or more affected sutures demonstrates
higher growth restriction as compared with single sutural
involvement. There are two exceptions to this observation:
scaphocephaly and Apert syndrome. With scaphocephaly,
Carpenter Syndrome
Carpenter syndrome is characterized by craniosynostosis
(commonly, but not always); preaxial polydactyly of the
feet; brachydactyly, clinodactyly, or both; congenital heart
defects (these are seen in 33% of patients), such as ventricu
lar septal defect, atrial septal defect, patent ductus arterio
sus, pulmonary stenosis, and tetralogy of Fallot; short
stature, with a height that is usually below the 25th centile;
obesity; and mental deficiency in some cases.28,39 Cranio
synostosis involves the sagittal and lambdoid sutures first,
with the coronal suture being the last to close. The head is
usually broad, but it is variable in shape, and asymmetry
may be a feature. Other features include dystopia cantho
rum, down-slanting palpebral fissures, epicanthal folds,
E, Worm’s-eye views before and after reconstruction. F, Occlusal views before and after reconstruction.
Note that the occlusion is improved but that the patient will require orthognathic surgery and orthodontic treatment during her
teenage years. From Posnick JC, Waitzman A, Armstrong D, Pron G: Monobloc and facial bipartition osteotomies: quantitative
assessment of presenting deformity and surgical results based on computed tomography scans, J Oral Maxillofac Surg 53:358-
367, 1995.
• Figure 30-14, cont’d
1227
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
• Figure 30-15 A 12-year-old boy with Noonan syndrome. When he was 5 years old, he underwent bilateral total orbital osteotomies with
medial translocation for the correction of orbital hypertelorism; this was carried out by another surgeon. When he was 12 years old, he presented
to this surgeon with a retruded irregular forehead, shallow orbits, residual proptosis, and midface deficiency. He underwent anterior cranial vault
and monobloc osteotomies with horizontal advancement. An occult cerebral spinal fluid leak with drainage through the nose occurred. Eventu
ally, there was erosion through the mid-forehead skin and resorption of a segment of the anterior and lateral cranial vault. One year after the
monobloc procedure, the patient was taken back to the operating room for the repair and closure of the cerebral spinal fluid fistula and a
cranioplasty that consisted of autogenous split-rib grafts. The cranial vault reconstruction was successful, without further infection or long-term
sequelae. A, Frontal view of the upper face 6 months after the initial surgery, with a cerebral spinal fluid fistula through central forehead. B and
C, Intraoperative views of the anterior cranial vault documenting the region of bone loss. D, Intraoperative lateral view that shows the area of
bone loss. E, Intraoperative lateral view after autogenous split-rib graft reconstruction. F, Frontal view of the upper face 1 year after dura repair
and the split-rib reconstruction of the skull defects. G, Computed tomography scan views before and after successful split-rib graft
cranioplasty.
G
A
B
C
D
E
F
mechanical forces produce a large head circumference; with
Apert syndrome, brain weights have been shown to be above
the 97th centile, regardless of age.46,48
Mental deficiency is correlated with the number of fused
sutures, and it occurs more frequently with two or more
sutural involvements than with single-suture synostosis. In
addition, mental deficiency occurs more commonly with
coronal synostosis than with other single-suture craniosyn
ostoses. With single-suture craniosynostoses (other than
coronal), mental deficiency may be more attributed to a
brain malformation rather than to growth restriction. This
may occur in patients with trigonocephaly and even in some
cases of sagittal synostosis.25
In the section about Apert syndrome, a distinction was
made between benign megalencephaly or distortion mega
lencephaly and progressive hydrocephalus. The mechanism
of hydrocephalus may vary. Although communicating
hydrocephalus with obstruction at the level of the basal
cisterns is most common, aqueductal stenosis is fairly
frequent. Chronic tonsillar herniation occurs in 73% of
1228 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
radiograph, head computed tomography (CT) scan, brain
magnetic resonance imaging, initial assessment for hydro
cephalus, and formal assessment for hearing. An ophthal
mologic examination is mandatory. Depending on the
findings, a 12- to 24-hour pressure recording should be
considered. Whenever possible, a formal developmental or
newborn psychologic battery should be administered before
surgery. The family should be assessed for psychosocial
problems, especially when a complicated course of surgical
interventions is contemplated. Follow up by a pediatrician
or pediatric neurologist at regular intervals is essential.
Assessments at monthly intervals for the first 12 months,
every 3 months during the second year, and every 6 months
until 6 years of age seem appropriate.25
Effects of Midface Deficiency on the
Upper Airway
All newborn infants are obligate nasal breathers. Most
infants who are born with syndromic craniosynostosis have
moderate to severe hypoplasia of the midface. They have
diminished nasal, nasopharyngeal, and retropalatal airspaces
with resulting increased upper airway obstruction. The
affected child is thus forced to breath solely through the
mouth. For a newborn infant to ingest food through
the mouth, he or she must suck from a nipple to achieve
negative pressure and have an intact swallowing mechanism.
The neonate with severe midface hypoplasia is unable to
accomplish this task while breathing adequately through the
nose at the same time. Complicating this clinical picture
may be an abnormal palate, enlarged tonsils, and enlarged
adenoids. Thus, the compromised infant expends significant
energy on respiration, possibly pushing the child into a
catabolic state (i.e., a negative nitrogen balance). Failure to
thrive can occur in the absence of nutritional support (e.g.,
nasogastric tube, feeding gastrostomy). It is also essential to
establish a secure airway, typically via a tracheostomy, which
may require air tubing at night. It is also essential to obtain
evaluations from a pediatrician, a pulmonologist, a pediatric
otolaryngologist, and a feeding specialist with craniofacial
experience to help distinguish minor feeding difficulties
from those that require more aggressive treatment.
Sleep apnea may be central, obstructive, or of mixed
origin; proper workup and assessment are crucial to the
establishment of the correct diagnosis and treatment.*
Central apnea may result from intracranial hypertension. If
so, the condition should improve upon brain decompres
sion by appropriate cranio–orbital or posterior cranial vault
expansion or when effective shunting of the hydrocephalus
is accomplished.
OSA has already been addressed in the sections about
the Apert, Crouzon, and Pfeiffer syndromes.105,138,149,190
patients with Crouzon syndrome as compared with only
1.9% of patients with Apert syndrome.62 Premature synos
tosis of the lambdoid suture may be responsible for this
finding. It has also been suggested that the obstruction of
the venous drainage of the brain may play a role by narrow
ing the jugular foramina. Some patients are able to com
pensate well for their hydrocephalus.25
Clinicians must maintain a high index of suspicion for
hydrocephalus in all patients with craniosynostosis, particu
larly those with complex or syndromic types.77,91,108,170,174
Among the syndromic craniosynostoses, shunted hydro
cephalus is found in 26.6% of patients with Crouzon syn
drome and in 27.8% of patients with Pfeiffer syndrome but
in only 6.5% to 8% of patients with Apert syndrome.46
Increased intracranial pressure in craniosynostosis may be
the result of a mismatch in cranial volume to brain growth
or of hydrocephalus. Increased intracranial pressure is found
most commonly in patients with multiple suture synosto
ses.9,26,49,58,122,191,222,226,227-230,245,266 However, recent studies
have indicated that increased pressure may also occur in
patients with single-suture synostosis. In one report, 3 out
of 18 children with scaphocephaly had increased intracra
nial pressure during 12- to 24-hour recordings.265
When pressure is low grade, clinical symptoms can be
subtle or absent. Headache is the classic symptom that is
associated with increased intracranial pressure of any cause.
However, children seem to experience this inconsistently,
and infants are not developed enough to communicate
their symptoms. The most common physical finding is pap
illedema, although this may not be as apparent during
infancy or early childhood, when bulging may occur at the
anterior fontanel instead. Longstanding papilledema may
result in optic atrophy and eventual blindness.25
Regardless of the cause, increased intracranial pressure
tends to rise during rapid eye movement sleep, presumably
as a result of increased cerebral blood flow. Indeed, plateaus
of increased intracranial pressure that last approximately
10 to 120 minutes have been observed during sleep. Con
versely, intracranial pressure may be normal while the
patient is awake or between periods of rapid eye movement
sleep. Thus, the diagnosis of increased intracranial pressure
may be missed by a single lumbar puncture measurement.
Reasonably safe ways of long-term, direct measurement of
increased intracranial pressure are possible with the use of
an epidural pressure transducer to record pressure for at least
12 hours. In this way, the significance of increased intracra
nial pressure as a contributing factor to mental deficiency
can be examined.25
With these considerations in mind, the neurologic assess
ment of patients with craniosynostosis can be performed as
follows. The medical history should concentrate on genetic
factors as well as the on child’s development. The physical
examination should include a careful search for associated
anomalies, with scrupulous attention paid to each of the
cranial nerves (and particularly to cranial nerves II and
VIII). Head circumference should be plotted on the stan
dard growth curve. Investigations should include a skull
*References 2, 35, 79, 92, 101, 105, 106, 114, 135, 141, 162, 172, 173,
175, 181, 185, 187, 190, 275, 284
1229
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
decannulation. In one patient, a tracheostomy was required
6 months after midface advancement for severe and recur
rent OSA.
Twenty-four months after MB advancement, Witherow
and colleagues documented the significant improvement of
airway obstruction on polysomnography in all patients who
had been diagnosed with Apert, Crouzon, or Pfeiffer syn
drome.280 Before MB advancement, 14 patients were
managed with tracheostomy or CPAP therapy for severe
OSA. Of these 14 patients who were undergoing MB
advancement for severe OSA, only 43% (n = 6) had resolu
tion of their OSA; 8 of 14 patients (57%) remained depen
dent on tracheostomy or CPAP despite MB advancement.
Nelson and colleagues studied 18 patients with cranio
synostosis syndromes who also had coronal synostosis and
midface deficiency.172 Eighty-three percent (16 of 18
patients) were treated with tracheostomy or CPAP for
known OSA before midface advancement. After the midface
advancement procedures, five of the tracheostomy patients
were decannulated; in six others, CPAP was no longer
required. This represents a 73% (11 of 15 patients) initial
success rate for the relief of OSA. The mean postsurgical
follow up was 3 years.
In patients with syndromic craniosynostosis who were
undergoing midface advancement, the lack of universal
success for the relief of OSA could result from a combina
tion of factors, including inadequate midface advancement;
midface relapse after initial advancement; other anatomic
sites or conditions of airway obstruction not addressed by
midface advancement (e.g., septum, turbinates, soft palate,
tonsils, adenoids, or mandible; tracheomalacia); a central
sleep apnea component; or inadequate contraction of the
pharyngeal dilator muscles during sleep. The anatomy of
the upper airway is different for each syndrome and also
widely variable within each syndrome. Patients with a cra
niosynostosis syndrome and OSA who are non-responders
to an effective midface advancement may have pharyngeal
wall collapse caused by compromised pharyngeal dilator
muscle function during sleep. Although a tendency for
airway collapse can be overcome by midface advancement,
the patient must also have the sufficient ability to control,
contract, and maintain the tone of the pharyngeal muscles
while asleep.
Morphologic Considerations
Examination of the patient’s entire craniofacial region should
be meticulous and systematic. The skeleton and the soft
tissues are assessed in a standard way to identify all normal
and abnormal anatomy.65,66,68,202,205,206,211,212,216,217,272,273 Spe
cific findings tend to occur in particular malformations, but
each patient is unique. The achievement of symmetry,
normal proportions, and the reconstruction of specific
aesthetic units is essential to forming an unobtrusive face
in a child who is born with one of the craniosynostosis
syndromes.
OSA in a child who is affected by one of these conditions
is frequently treated with tracheostomy. It may also be
treated by adenoidectomy, tonsillectomy, midface advance
ment, or CPAP. If left untreated or ineffectively treated,
OSA will result in disabilities that include failure to thrive,
recurrent upper respiratory infection, cognitive dysfunc
tion, developmental delay, cor pulmonale, and sudden
death.173 Midface hypoplasia in the setting of a craniosyn
ostosis syndrome is often a primary cause of OSA. If fea
sible, surgical midface advancement is the preferred biologic
treatment approach; even with such treatment, some
patients will still require a tracheostomy or CPAP therapy.
Clinical signs of OSA may include loud snoring; observed
apnea during sleep; excessive perspiration during sleep
(except in patients with Apert syndrome, as described previ
ously in this chapter); difficulty during daytime breathing;
and daytime somnolence. Polysomnography is essential for
accurately diagnosing the type of obstruction (i.e., central,
obstructive, or mixed). In the pediatric age group, apnea is
defined as the absence of airflow for more than two breaths,
whereas hypopnea is defined as a reduction of at least 50%
of nasal flow signal amplitude. The apnea–hypopnea index
(AHI) is the number of obstructive apneas in combination
with hypopneas (followed by desaturations) per hour. The
oxygen–desaturation index is the number of desaturations
(i.e., ≥4% decrease with respect to the baseline) per hour.
A score of less than 1.0 in a child is considered normal. The
severity of the condition in children is defined as follows:
between 1 and 5 events per hour is considered mild OSA;
6 to 25 events per hour constitute moderate OSA; and more
than 25 events per hour results in severe OSA. In the child
with a craniosynostosis syndrome and a midface deficiency
with documented OSA, neither tonsillectomy nor adenoid
ectomy generally provides sufficient opening of the upper
airway.
Al-Saleh and colleagues completed a retrospective review
of children with syndromal craniosynostosis who were
referred to the Hospital for Sick Children in Toronto,
Canada, between 1996 and 2008 for initial polysomnogra
phy to rule out sleep-related disordered breathing.2 The
research confirmed a high prevalence of this type of breath
ing in children with Crouzon, Apert, Pfeiffer, and Saethre–
Chotzen syndromes (N = 35). Despite a spectrum of
surgical and non-surgical interventions having been carried
out, the complete resolution of the sleep-related disordered
breathing could not be routinely achieved.
Ishii and colleagues used lateral cephalometric radio
graphs to evaluate the nasopharyngeal airway after Le Fort
III advancement in patients with Apert or Crouzon syn
drome and OSA.114 The researchers documented improve
ment in the nasopharyngeal airway space after successful
midface advancement surgery. Arnaud and colleagues
reviewed respiratory improvement in patients with syn
dromic craniosynostosis and OSA after monobloc (MB)
advancement.8 Eighty-eight percent of patients (n = 16)
showed improvement as measured by oxygen level during
sleep; 4 of 6 patients underwent successful tracheostomy
1230 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
lobes of the brain and horizontally short (i.e., retruded);
this results in a shallow, hyperteloric, “reverse curved”
upper midface. Surgically advancing the midface without
simultaneously addressing the increased transverse width
and the “reverse curve” will not adequately correct the
dysmorphology.
Maxillary/Nasal Base Aesthetic Unit
In the patient with a craniosynostosis syndrome and a
midface deficiency, the upper anterior face is vertically short
(i.e., from the nasion to the maxillary incisor), and there is
a lack of horizontal anteroposterior projection. These find
ings may be confirmed through cephalometric analysis,
which indicates the presence of a sella–nasion–A point angle
that is less than the mean value as well as a short upper
anterior facial height (i.e., from the nasion to the anterior
nasal spine). The width of the maxilla in the dentoalveolar
region is generally constricted, with a highly arched palate.
To normalize the maxillary/nasal base region, multidirec
tional surgical expansion and reshaping are generally
required. The abnormal maxillary lip-to-tooth relationship
and the occlusion are improved through Le Fort I segmental
osteotomies and orthodontic treatment as part of the staged
reconstruction. The mandible and the chin are frequently
secondarily involved and will benefit from surgical reposi
tioning as part of the orthognathic correction.
Surgical Approach to
Craniosynostosis Syndromes
Historical Perspectives
As early as 1890, Lannelogue134 and then Lane133 described
a surgical approach to the treatment of craniosynostosis.
Lannelogue’s aim was to remove the fused suture (i.e., strip
craniectomy) in the hope of controlling the problem of
brain compression within a congenitally small cranial vault.
By the turn of the century, Harvey Cushing, who was the
most prominent neurosurgeon of his day, suggested that
surgical intervention for the problem of craniosynostosis
was misdirected and that more attention should instead be
given to the schooling of these children.55 Shillito disagreed
with Cushing and enthusiastically supported the concept of
surgical intervention to improve the outlook for these chil
dren.243 He believed that the linear “strip” craniectomy of
the fused sutures would “release” the skull and allow the
cranium to reshape itself and continue to grow in a normal
and symmetric fashion. The strip craniectomy procedures
were supposed to allow for a new suture line at the site of
the previous synostosis. With the realization that this goal
was not biologically feasible, attempts were made to remove
portions of the cranial vault surgically and leave large open
areas or to use the removed segments as free grafts to refash
ion the cranial vault shape. Problems with these methods
included uncontrolled postoperative skull molding,
Fronto–Forehead Aesthetic Unit
The fronto–forehead region is dysmorphic in an infant with
a craniosynostosis syndrome. Establishing the normal posi
tion of the forehead is critical for overall facial symmetry
and balance. The forehead may be considered as two sepa
rate aesthetic components: the supraorbital ridge/lateral
orbital rim region and the superior forehead. The supraor
bital ridge/lateral orbital rim unit includes the nasofrontal
process and the supraorbital rim extending inferiorly down
each frontozygomatic suture toward the infraorbital rim and
posteriorly along each temporoparietal region. The shape
and position of the supraorbital ridge/lateral orbital rim
region is a key element of upper facial aesthetics. In a
normal forehead, at the level of the nasofrontal suture, an
angle that ranges from 90 to 110 degrees is formed by the
supraorbital ridge and the nasal bones when the face is
viewed in profile. In addition, the eyebrows, which overlie
the supraorbital ridge, should be anterior to the cornea.
When the supraorbital ridge is viewed from above, the rim
should arc posteriorly to achieve a gentle 90-degree angle at
the temporal fossa with a center point of the arc at the level
of each frontozygomatic suture. The superior forehead com
ponent, which is about 1.0 to 1.5 cm up the supraorbital
rim, should have a gentle posterior curve of about 60
degrees, thus leveling out at the coronal suture region when
the face is viewed in profile.
Posterior Cranial Vault Aesthetic Unit
The symmetry, form, and adequate intracranial volume of
the posterior cranial vault are closely linked. Posterior
cranial vault flattening may result from unilateral or bilat
eral lambdoid synostosis, which is rare; previous craniec
tomy with reossification in a dysmorphic flat shape, which
is frequent; or postural molding as a result of intrauterine
positioning or repetitive supine sleep positioning. A short
anteroposterior cephalic length may be misinterpreted as
an anterior cranial vault (forehead) problem when the
occipitoparietal (posterior) skull represents the primary
region of the abnormality. Careful examination of the entire
cranial vault is essential to defining the dysmorphic region
so that, when indicated, appropriate cranial vault expansion
and reshaping may be carried out.
Orbito–Naso–Zygomatic Aesthetic Unit
In the craniosynostosis syndromes, the orbito–naso–zygo
matic regional abnormality is a reflection of the cranial base
malformation. In patients with Crouzon syndrome, when
bilateral coronal suture synostosis is combined with skull
base and midface deficiency, the orbito–naso–zygomatic
region will be dysmorphic and consistent with a short
anteroposterior and wide transverse anterior cranial base. In
patients with Apert syndrome, the nasal bones, orbits, and
zygomas—like the anterior cranial base—are transversely
wide as a result of the anterolateral bulging of the temporal
1231
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
advanced as a separate element in conjunction with the Le
Fort III complex below and the frontal bones above.256
Seven years later, Ortiz-Monasterio and colleagues refined
the MB osteotomy to advance the orbits and midface as one
unit; this was combined with frontal bone (anterior cranial
vault) repositioning to correct the upper and midface defi
ciency associated with Crouzon syndrome.178,179 In 1979,
Van der Meulen described the medial fasciotomy for the
correction of midline facial clefting.267 Van der Meulen split
the MB osteotomy vertically in the midline, removed central
nasal and ethmoid bone, and then moved the two halves of
the facial skeleton together for the correction of the orbital
hypertelorism. To correct the midface dysplasia and the
associated orbital hypertelorism in patients with Apert syn
drome, Tessier refined the vertical splitting and reshaping
of the midline split MB segments, thereby correcting the
midface deformity in three dimensions via a procedure that
is now known as facial bipartition. Wolfe and colleagues,281,282
Kawamoto,19 and Posnick and colleagues198,207,208,220 have all
independently documented the advantages of Tessier’s facial
bipartition technique for the correction of the upper and
midface abnormalities associated with Apert syndrome. The
widespread use of autogenous cranial bone grafting has
virtually eliminated the need for rib and hip grafts when
bone replacement is required during cranio–orbito–zygo
matic procedures.213 This represents another of Tessier’s con
tributions to craniofacial surgery.255
In 1968, Luhr introduced the use of small metal
(vitalium) plates and screws to stabilize maxillofacial frac
tures and then osteotomies (see Chapter 2). In current
practice, the use of internal miniplate and microplate and
screw (titanium) fixation of various sizes is the preferred
form of rigid fixation when the stability and three-
dimensional craniomaxillofacial reconstruction of multiple
osteotomized bone segments and grafts are required. In
infants and young children, resorbable materials are now
generally used for the stabilization of non–load-bearing
osteotomy segments (e.g., the cranial vault).139,196,199,221 This
avoids the issues or uncertainty regarding growth restriction
and brain trauma from retained non-resorbable hardware.
Philosophy Regarding the Timing of Surgery
In considering both the timing and type of intervention,
the experienced surgeon will take several biologic realities
into account, including the natural course of the malforma
tion (i.e., progressively worsening dysmorphology versus a
nonprogressive craniofacial abnormality); the tendency for
growth restriction in operated skeletally immature bones
(i.e., akin to the maxillary hypoplasia that occurs following
cleft palate repair); the relationship between the underlying
developing viscera (i.e., brain) and the congenitally affected
and/or surgically altered skeleton (i.e., brain compression if
the cranial vault is not expanded); and the child’s airway
needs (i.e., midface deficiency resulting in OSA).
To limit impairment while simultaneously achieving
long-term preferred facial aesthetics and head and neck
reossification into dysmorphic configurations, and the
occurrence of large residual skull defects.
The concept of the simultaneous release of the fused
suture in combination with more meticulous cranial vault
reshaping in infants was initially suggested by Rougerie
and colleagues232 and then refined by Hoffman and Mohr
in 1976 for children who were born with unilateral coronal
synostosis.107 In 1977, Whitaker and colleagues proposed
a more formal anterior cranial vault and orbital reshaping
procedure for unilateral coronal synostosis.279 Marchac
and Renier published their experience with a “floating
forehead” technique to manage craniosynostosis during
infancy; this consisted of simultaneous unilateral coronal
and bilateral coronal suture release and forehead and
upper orbital osteotomies with advancement.144 Unfortu
nately, the “floating forehead” technique resulted in unpre
dictable reossification of the open cranial vault areas.145 In
addition, bitemporal constrictions and bulging of the skull
concavities were frequent occurrences as reossification
occurred. In any case, the hope for midface growth did not
materialize.
During the 1950s, Gillies and Harrison reported their
experience with an extracranial Le Fort III osteotomy to
improve the anterior projection of the midface in an adult
with Crouzon syndrome. The initial Gillies procedure
was actually carried out in 1942.89 Gillies mobilized the
midface via a variety of osteotomies performed through skin
incisions directly over each osteotomy site. The midface was
mobilized and advanced, and intermaxillary fixation was
applied. After the removal of the intermaxillary fixation
(i.e., 2 weeks after the operation) a metal cast-cap dental
splint was attached to a plaster headcap and maintained for
3 weeks. There was no evidence that Gillies used bone grafts
to bridge the surgical gaps that were created. The early
enthusiasm for this technique later turned to discourage
ment when the patient’s facial skeleton relapsed to its pre
operative status. Relapse occurred at the maxillary incisor
level and also resulted in ocular proptosis.
Dr. Tessier was aware of the previous work of Gillies and
its accompanying difficulties. In 1967, Tessier described a
new intracranial–cranial base approach to the management
of Crouzon syndrome.242 This work was first presented in
France at a meeting in Montpellier in 1966 and then again
the following year at the International Plastic Surgery
Meeting in Rome.252-254 Tessier’s landmark presentations
and publications were the beginning of modern craniofacial
surgery (see chapter 2). 252-264 To overcome the earlier prob
lems encountered by Gillies, Tessier developed an innova
tive basic surgical approach that included new locations
for the Le Fort III osteotomy, a combined intracranial–
extracranial (cranial base) approach, the use of a coronal
skin incision to expose the upper facial bones, and the use
of fresh autogenous bone graft. He also applied an external
fixation device to help maintain bony stability until healing
had occurred.
In 1971, Tessier described a single-stage frontofacial
advancement in which the fronto–orbital bandeau was
1232 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
children, the use of chromic gut on the skin may obviate
the need for postoperative suture or staple removal.
Management of the Ventricular System
In the patient with a craniosynostosis syndrome who is to
undergo intracranial volume expansion with the concurrent
management of hydrocephalus as part of the reconstruction,
the potential for morbidity increases.25,77,91,108,170,174 Compli
cations may arise from either excessive cerebrospinal fluid
drainage (overshunting) or inadequate shunting that leads
to increased intracranial pressure. With overshunting there
is decreased central nervous system mass to fill any surgically
created retrofrontal dead space. Uncontrolled hydrocepha
lus may result in raised intracranial pressure, which leads to
its own set of problems. In either situation, fronto–facial
advancement via an intracranial approach to the midface or
isolated cranial vault expansion procedures should be
thoughtfully staged with ventriculoperitoneal shunt man
agement. We believe that the presence or absence of a ven
triculoperitoneal shunt is not in itself a major factor in the
success of a fronto–facial advancement procedure. An
important aspect is the satisfactory physiologic function of
the ventricular system, with or without the placement of a
shunt. Ultimately, the decision regarding the need for and
sequencing of shunting is based on the patient’s neurologic
findings and the neurosurgeon’s judgment. In a patient with
a ventriculoperitoneal shunt in place before the surgery,
experienced neurosurgical evaluation, including imaging
studies of the ventricular system, is carried out to confirm
satisfactory physiologic function.
Upper Midface Reconstruction Options
The osteotomy selected (i.e., extra cranial Le Fort III or
intracranial, monobloc or facial bipartition) to manage the
upper midface dysmorphology in the individual with a cra
niosynostosis syndrome should reflect the presenting skel
etal deformities and provide a realistic opportunity for long
term upper midface (naso-orbito-malar) region aesthetic
enhancement.
A main objective of this phase of reconstruction is to
“normalize” the orbits, zygomas, and cranial vault. Correc
tion of the maxillo-mandibular deformity will require
orthognathic surgery including at a minimum a separate Le
Fort I osteotomy. The patient specific dysmorphology will
depend on the original malformation, the previous proce
dures carried out, and the effects of ongoing growth.
When evaluating the upper and midface morphology in
the mixed dentition child or young adult born with Crouzon
syndrome, one should note: 1) if the supraorbital ridge is in
good position when viewed from the sagittal plane (are the
depth of the upper orbits adequate?); 2) if the midface and
forehead have an acceptable arc of rotation in the transverse
plane (is the midface arc concave?); and 3) if the root of the
nose and orbits are of normal width (is there orbital hyper
telorism?). If these structures are confirmed to be with
acceptable morphology, then there is no need to further
reconstruct the forehead and upper orbits. In those few,
function, the surgeon must ask an essential question,
“during the course of craniofacial development, does the
operated-on facial skeleton of a child with a craniosynostosis
syndrome tend to grow abnormally, resulting in further distor
tions and dysmorphology, or are the initial positive skeletal
changes achieved (at operation) maintained during ongoing
growth?” Unfortunately, the proposed theory that craniofa
cial procedures carried out in early infancy will “unlock
growth” has not been documented through the scientific
method.194,200-206,218,219
Final reconstruction of the upper midface deformities
in those born with a craniosynostosis syndrome can be
managed as early as 7 to 10 years of age. By this age, the
cranial vault and orbits normally attain approximately 85%
to 90% of their adult size. Whenever feasible, waiting until
the maxillary first molars have erupted is also preferred.
When the “upper midface” reconstruction is carried out
after this age, the objective is to attain adult morphology in
the cranio-orbito-zygomatic region with the expectation of
a stable result (no longer influenced by growth) once healing
has occurred. Psychosocial considerations also support the
age 7 to 10 years time frame for the upper midface proce
dure. When a successful reconstruction is achieved at this
age, the child may progress through school with an oppor
tunity for a healthy body image and self-esteem.*
Management of the Upper Midface Deformity
in Children and Young Adults
Incision Placement and Soft-Tissue Management
For the exposure of the craniofacial skeleton above the Le
Fort I level, the approach used is the coronal skin incision.
This allows for relatively camouflaged access to the anterior
and posterior cranial vault, the orbits, the nasal dorsum, the
zygomas, the upper maxilla, the pterygoid fossa, and the
temporomandibular joints. For added cosmetic advantage,
the placement of the coronal incision more posteriorly on
the scalp and with postauricular rather than preauricular
extensions is useful.214 When the exposure of the maxilla at
the Le Fort I level is required, a circumvestibular maxillary
intraoral incision is used. Unless complications occur that
warrant unusual exposure, no other incisions are required
to manage any aspect of reconstruction in patients with a
craniosynostosis syndrome. These incisions (i.e., coronal
[scalp] and maxillary [circumvestibular]) may be reopened
as needed to further complete the individual’s staged
reconstruction.
A layered closure of the coronal incision (i.e., the galea
and the skin) optimizes healing and limits scar widening.
Resuspension of the midface periosteum to the temporalis
fascia may facilitate the redraping of the soft tissues. Each
lateral canthus should be reattached in a superoposterior
direction to the newly repositioned lateral orbital rim. In
*References 8, 10, 11, 23, 32, 36, 56, 57, 63, 69, 70-72, 89, 97, 109,
110, 113, 117, 120, 119, 136, 149-151, 155-158, 169, 171, 176, 180,
189, 192, 195, 197, 215, 235, 239, 241, 244, 277
1233
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
orbital depth. This frequently results in either residual prop
tosis or enophthalmos. Simultaneous correction of upper
face (orbital) hypertelorism and the concave midface arc-of-
rotation typical in Apert syndrome is also not possible with
the Le Fort III procedure. Excessive lengthening of the nose,
accompanied by flattening of the nasofrontal angle, will also
occur if the Le Fort III osteotomy is selected when the
skeletal dysmorphology favors an MB or facial bipartition.
Unfortunately, it is not possible to later correct the elon
gated nose or the flattened naso-frontal angle. To avoid
these short comings, it is not a matter of becoming more
proficient at the Le Fort III osteotomy or simply “managing
the overlying soft tissues” in a different way (i.e., canthope
ties or midface lift). The Le Fort III osteotomy is not con
sistent with the presenting dysmorphology in most
craniosynostosis syndrome patients and, therefore, will not
provide the opportunity to achieve the desired aesthetic
result. Nevertheless, the Le Fort III is often considererd the
“go to” approach by surgeons as it is: 1) an extracranial
procedure; 2) requires less surgeon skill/experience; 3) is less
likely to result in significant blood loss; and 4) is less likely
to result in perioperative complications (i.e., cranionasal
fistula, intracranial abscess, bone resorption).
In most syndromal craniosynostosis patients, a subopti
mal aesthetic result will occur if the surgeon attempts to
simultaneously adjust the orbits and idealize the occlusion
using the Le Fort III, MB or facial bipartition osteotomy
without completing a separate Le Fort I osteotomy. The
degree of horizontal deficiency observed at the orbits and
at the maxillary dentition are rarely uniform. If a Le Fort I
osteotomy to separate the “lower midface” from the “upper
midface” complex is not carried out, excess advancement at
the orbits with enophthalmos is likely to occur as the
surgeon attempts to achieve a positive overjet at the incisors.
The Le Fort I osteotomy is generally not performed at the
time of the “upper midface” procedure. This will await
skeletal maturity and is combined with orthodontic treat
ment. Until then, a degree of an Angle class III anterior
open bite negative overjet malocclusion will remain. When
the mature teenage or adult patient presents for surgical
correction and requires both “upper midface” (i.e.,
naso-orbito-zygomatic) and “lower midface” (i.e., maxilla)
management the procedures may be carried out
simultaneously.
Monobloc and Facial Bipartition Osteotomies
(Intracranial Approach to the Orbits and the Midface)
Cranial vault reconstruction in the child with a craniosyn
ostosis syndrome should provide space for the compressed
brain to expand. Immediately after completing cranial vault
and monobloc (MB) or facial bipartition (FB) osteotomies
with advancement, extradural (retrofrontal) dead space
remains in the anterior cranial fossa above the skull base gap
created by the osteotomy.208 The skull base gap is in direct
communication with the nasal cavity. Therefore, the post
operative recovery may be complicated by cerebrospinal
fluid leakage across the skull base gap followed by infection,
craniosynostosis syndrome patients, in whom the residual
deformity is only in the lower half of the orbits, the zygo
matic buttress, and the maxilla an extracranial Le Fort III
osteotomy is likely to be effective treatment.
If the supraorbital region, anterior cranial base, zygomas,
root of the nose as well as the lower orbits, and maxilla all
remain deficient in the sagittal plane (horizontal retrusion),
then an MB is indicated. In these patients, the forehead is
also generally flat and retruded and will require reshaping
and advancement. If upper midface hypertelorism (increased
transverse width) with midface flattening (horizontal retru
sion) and a concave facial curvature (reverse facial arc) is
also present, then the MB unit is split vertically in the
midline (facial bipartition). A wedge of interorbital (nasal
and ethmoidal) bone is removed, and the orbits and zygomas
are repositioned medially while the maxillary arch is
widened. A facial bipartition is rarely required in Crouzon
syndrome but the MB is. When a monobloc or facial biparti
tion osteotomy is carried out as the “upper midface” proce
dure, additional segmentation of the upper and lateral
orbits may also be required to normalize the morphology
of the orbital aesthetic units.
For almost all Apert syndrome patients, facial bipartition
osteotomies combined with further cranial vault reshaping
permits a better correction of the dysmorphology than can
be achieved through any other upper midface procedure
(i.e., MB or Le Fort III). When using facial bipartition
osteotomies, correction of the concave midface arc of rota
tion is also possible. This further reduces the stigmata of the
Apert “flat, wide and retrusive” facial appearance. The facial
bipartition allows the orbits and zygomas to shift to the
midline (correction of hypertelorism) as units while the
maxilla is simultaneously widened (i.e., relief of the V-shaped
face). Horizontal advancement of the reassembled upper
midface complex is then possible to improve orbital depth
and zygomatic length. The forehead is generally flat, tall,
and retruded, with a constricting band just above the supra
orbital ridge. Reshaping of the anterior cranial vault is also
simultaneously carried out. A Le Fort III osteotomy is virtu
ally never adequate for an ideal correction of the residual
upper midface anomalies documented in Apert syndrome.
The study by McCarthy confirms that the Le Fort III
osteotomy is not effective as an aesthetic option to manage
the upper midface deformity in the majority of craniosyn
ostosis syndrome patients (see section later in this chapter
concerning Le Fort III option).276 By anatomic design, the
Le Fort III prevents management of the whole orbital aes
thetic unit during one operative setting. Therefore a major
aesthetic shortcoming of the Le Fort III osteotomy, when
its indications do not fit the presenting dysmorphology, is
the creation of irregular step-offs in the lateral orbital rims.
This will occur even when only a moderate Le Fort III
advancement is carried out. These lateral orbital step-offs
will be visible to the casual observer as unattractive at con
versational distance and surgical attempts at modification
performed later will be suboptimal. Another problem with
the Le Fort III osteotomy is the difficulty in judging an ideal
1234 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
dura by 6 to 8 weeks after surgery. Specific intraoperative
maneuvers were undertaken by the surgeons to close the
nasofrontal communication, including flaps, fibrin glue,
bone grafts, and Gelfoam. After surgery, care was taken to
limit a pressure gradient across the communication (via the
repair of dural tears, sinus precautions, and nasal stenting),
with the objective of providing time for nasal mucosa
healing. The infection rate in this study group was limited
to 2 of 23 patients (9%). In both patients who developed
an infection, a retrofrontal (extradural) fluid collection was
noted, with drainage across the residual nasofrontal com
munication into the nose. Both patients healed without
significant comorbidity (i.e., brain or eye injury) but did
require further reconstruction of the resorbed portions
of the anterior cranial vault and the supraorbital ridges
(see Fig. 30-15).
Wolfe completed a critical analysis of 81 MB advance
ments carried out over a 27-year period.281 This was a ret
rospective chart analysis of a series of patients who
underwent either MB (frontofacial) advancement (MFFA)
or FB (MFFA plus FB). The procedures were carried out at
seven different craniofacial centers and included 49 MFFA
and 32 MFFA plus FB. The MFFA and MFFA plus FB
osteotomies were either placed in their preferred location in
the operating room (standard approach) or gradually dis
tracted (DO technique) with internal or external devices.
Complications included two deaths (cardiac arrest in one
patient and complications arising from hypovolemia in the
other). One case was aborted as a result of a large-volume
blood loss; there were three infections or sequestrations and
one persistent cerebrospinal fluid leak (no meningitis).
There were significant complications documented in the
DO group and fewer in the non-DO group. Blood loss and
operative time were equivalent for both standard and DO
techniques. Interestingly, the incidence of infection and
cerebrospinal fluid leaks was not diminished with the use
of the alternative distraction DO approach. The author also
concluded that, for the majority of patients, the standard
approach offered improved morphologic results. The author
then compared the morphologic results of the MMFA and
MMFA plus FB with those of the extracranial Le Fort III
option and concluded that the Le Fort III was less favorable.
Regardless of the technique used, all patients required
orthognathic surgery (at the Le Fort I level) to complete the
reconstruction.
Bradley and colleagues completed a single-center retro
spective study that compared differences in morbidity in a
series of patients who were born with a craniosynostosis
syndrome and who then underwent MB osteotomy for the
correction of upper and midface anomalies or hypoplasia.19
The authors describe three different sequential treatment
groups that were followed during a period of 23 years.
Group I patients (1979 to 1989; n = 12) underwent MB
osteotomies without any special attention paid to the retro
frontal dead space or the communication through the skull
base between the anterior cranial fossa and the nasal cavity.
Group II patients (1989 to 1995; n = 11) underwent MB
fistula formation, and subsequent bone resorption in the
glabella region. After fronto–facial advancement by either
MB or FB, the communication between the nasal cavity
and the cranial fossa must be managed to limit these poten
tial complications. The most effective method to do so
remains unclear, but all agree that it is a critical aspect for
successful reconstruction. Technical aspects of management
include the following: 1) gentle tissue handling; 2) achiev
ing good hemostasis; 3) effective repair of dural tears; 4)
avoidance of over expansion of the cranial vault; 5)
maximum separation of dura and nasal mucosal tissue
planes (i.e., interposing tissue such as bone grafts, tissue
sealants, and flaps); 6) rigid plate and screw fixation of the
osteotomies and bone segments; 7) avoidance of pressure
gradients across the opening to facilitate nasal mucosa
healing; and 8) prevention of too much or too little ven
triculoperitoneal shunting.
After cranio–orbital reconstruction in the infant with a
craniosynostosis syndrome, rapid filling of the expanded
intracranial volume by the previously compressed frontal
lobes of the brain has been documented.208 This has also
been shown to occur after MB and FB advancement in
children and young adults.249 More gradual filling of the
space is thought to occur in older adults. At the time of the
MB or FB osteotomies, the nasal cavity can be sealed from
the cranial fossa by doing the following: 1) inserting peri
cranial tissue; 2) placing bone grafts to bridge the osteotomy
gaps; and 3) using tissue sealants. This provides time for the
re-epithelialization (i.e., healing) of the nasal mucosa. Until
the torn nasal mucosa heals, communication between the
nasal cavity and the anterior cranial fossa may result in the
transfer of air, fluid, and bacteria followed by infection and
naso–cranial fistula formation. The postoperative continu
ation of nasotracheal intubation for several days and
nasopharyngeal tube placement after extubation have also
proven useful to limit pressure gradients across the com
munication. The avoidance of positive pressure ventilation,
the enforcement of sinus precautions, and the restriction of
nose blowing further limits the reflux of air, fluid, and
bacteria early after surgery.
Four clinical studies clarify issues of morbidity related to
MB or FB osteotomies for the reconstruction of an indi
vidual with a craniosynostosis syndrome.
Posnick and colleagues studied the issues of retrofrontal
dead space; communication across the skull base osteotomy
gap; and associated morbidity in a consecutive series of
children in the mixed dentition and young adults in the
permanent dentition (n = 23) who were undergoing either
MB or FB osteotomies in combination with cranial vault
expansion.208 The procedures were carried out by a single
craniofacial surgeon (Posnick) and one of three neurosur
geons at a single tertiary care hospital from 1987 to 1991.
The extradural (retrofrontal) dead space was measured
from consistent CT scan images at specific postoperative
intervals (immediate, 6 to 8 weeks, and 1 year). The study
confirmed the presence of an immediate retrofrontal dead
space that generally filled in with the expanding brain and
1235
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
COMMENT: With an MB osteotomy, as much
aesthetic damage is done by overadvancement
(enophthalmos) as by underadvancement (residual eye
proptosis). In addition, the achievement of a “normal”
occlusion is not usually a treatment objective at the time
of MB advancement. Accomplishing an ideal occlusion
without creating enophthalmos generally requires a
separate Le Fort I osteotomy to differentially reposition
the maxilla.
cranial vault volume; 2) exposure of the eyes (i.e., corneal
irritation) as a result of shallow orbits; 3) airway obstruction
from a reduced upper airway space (i.e. OSA); and 4)
feeding difficulties.3 Each of the study patients underwent
fronto–facial (MB) advancement with the use of DO tech
niques. The mean age at operation was 18 months (range,
4 to 30 months). The mean advancement was 16.6 mm at
the forehead level and 17 mm at the midface level. Ocular
protection and the reduction of intracranial pressure (when
raised) was achieved in all children. At least a degree of
airway improvement was achieved in all but one child. The
authors subjectively state that there was marked improve
ment in every patient’s appearance. Complications included
cerebrospinal fluid leaks (2 of 12 patients [16.6%]); pin-site
infections (3 of 12 patients [25%]); external DO device
frame slippage that required replacement (2 of 12 patients
[16.6%]); and overadvancement with resulting enophthal
mos (1 of 12 patients [8.4%]). In addition, one patient died
9 months after the procedure in conjunction with tracheal
reconstruction. The published article was discussed by
Hopper,111 who recommends that clinicians consider a
less invasive approach and accept limited aesthetics objec
tives in an attempt to lower perioperative morbidity. The
approach that he suggests includes the following: 1) either
anterior cranial or posterior cranial vault expansion without
simultaneous midface advancement to relieve intracranial
pressure; 2) extracranial Le Fort III advancement without
simultaneous cranial vault expansion to open the upper
airway; 3) tarsorrhaphies to protect the eyes from exposure
rather than orbital expansion; and 4) continued tracheos
tomy for airway management rather than midface
advancement.
osteotomies with various attempts at closure of the skull
base gap with pericranial flaps and fibrin glue. Group III
(1995 to 2002; n = 24) patients underwent MB osteotomies
without immediate advancement. An internal distraction
device was placed across the osteotomized zygomatic arch
on each side. After a 7-day latency period, the advancement
of the MB and forehead unit was initiated at 1 mm per day
for approximately 2 to 4 weeks. The infection rate for
Group III patients was significantly lower (2 of 24 [8%])
than it was for those in Groups I and II. Neither of the two
infections in Group III resulted in bone loss. Group I
patients had the greatest morbidity likely as a result of the
limited fixation performed during the 1970s and 1980s.
As described by Bradley and colleagues, the DO tech
nique does allow more time for the brain to expand into
the retrofrontal dead space after the completion of an MB
osteotomy (i.e. delayed for 7 days) before advancing the
upper midface.19 In theory, this should facilitate early nasal
mucosa healing and thereby limit the communication of
fluid, air, and bacteria across the surgically created skull base
gap. This likely explains the drop in infection rate in the
Group III patients as compared with the Group I and II
patients. The rate of infection in Bradley’s Group III
patients, who were treated with a DO approach, essentially
matches that of the patients described by Posnick and col
leagues who were treated with a standard approach (8% and
9%, respectively).
Bradley and colleagues also described greater advance
ment in Group III (DO approach) patients as compared
with Group I and II patients.19 Confounding variables
may explain these differences, including increased surgical
experience during the later years of the study (i.e., group III
patients) and the impact of complications during the earlier
years of the study (i.e., extremely high rates of infection in
Groups I and II), which likely resulted in relapse and limited
the long-term midface advancement. More importantly,
there was no correlation between the number of millimeters
of MB advancement and either greater functional gains or
enhanced facial aesthetics.
COMMENT: The four studies reviewed and the
discussion by Hopper that followed demonstrate the
wide variation of opinions on how best to reconstruct
individuals with a craniosynostosis syndrome. In this
author’s opinion, for the majority of these children, only
the MB or facial bipartition osteotomies offer a realistic
opportunity to achieve close to normal morphology and
the opportunity to develop healthy self-esteem.
Favorable craniofacial function is expected to follow the
enhanced facial form. This includes cranial vault
expansion (i.e., relief of ICP); orbital expansion (i.e.,
relieve of proptosis and eye protection); and upper
airway expansion (i.e., relief of OSA and improved
breathing during the day). Gains in mastication, swallow
mechanism, speech articulation, breathing, vision, and
cognitive function are also expected.
A fourth clinical study sheds further light on this subject.
Ahmad and colleagues described a series of 12 children
who were born with a craniosynostosis syndrome who also
had multiple functional problems, including the following:
1) raised intracranial pressure as a result of a diminished
For the individual with a craniosynostosis syndrome, to
achieve the most favorable facial harmony and head and
neck function, the surgeon needs both an aesthetic sense
and the technical expertise to execute effective upper/
midface surgical procedures. Several key aspects include:
1236 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
Two different forms of fixation were used: Group 1’s treat
ment included intraosseous wires, bone grafts, suspension
wires, dental splint, and 8 weeks of intermaxillary fixation;
Group 2’s treatment involved titanium plates and screws,
bone grafts, dental splints, and elastics. Good stability of
the midface was documented in both groups at 1 year, 5
years, and 10 years. As has been found with previously
published studies, no significant horizontal growth occurred
in the midface after surgery. The form of fixation used was
not a factor (i.e., plate and screw fixation did not increase
incidence of growth restriction). The mandible was mea
sured at the pogonion and found to advance 5.72 mm and
7.32 mm at the 5- and 10-year time points, respectively.
This study confirms that, when the Le Fort III advancement
is carried out at a young age, the occurrence of jaw dishar
mony with ongoing growth is inevitable and will require
further staged reconstruction.
In a second publication, Shetye and colleagues evaluated
a larger group of syndromal craniosynostosis patients who
also underwent extracranial Le Fort III advancement
between the years of 1973 and 2006.238 Three different
stabilization techniques were sequentially used over the
study period: Group 1 (n = 20) was treated with intraosse
ous wires, suspension wires, bone grafts, dental splinting,
and 8 weeks of intermaxillary fixation; Group 2 (n = 20)
was treated with titanium plates and screws, bone grafts,
dental splinting, and elastics; and Group 3 (n = 20) was
treated with the extended use of an external DO device
during 3 months of healing. The stability of midface
advancement was assessed with the use of measurements
from 1- and 5-year postoperative lateral cephalometric
radiographs. Patients in Groups 1 and 3 showed equal levels
of skeletal stability, whereas Group 2 showed slightly less
stability at the A point. At the orbitale, all three groups
showed similar levels of stability. Interestingly, all three
methods of fixation for the extracranial Le Fort III advance
ments showed good levels of stability. The study also con
firmed that all study subjects who were in the mixed
dentition demonstrated progressive facial disharmony as a
result of ongoing mandibular growth, thereby necessitating
further skeletal reconstruction by the late teenage years (i.e.,
different forms of fixation had no bearing on the incidence
of growth restriction).
In the discussion, McCarthy, who was the senior inves
tigator, stated his personal preference for the management
of the midface deformity in syndromal craniosynostosis
with an extracranial Le Fort III advancement followed by
orthognathic surgery. When early Le Fort III advancement
is indicated in a growing child, he prefers distraction to
standard techniques. According to McCarthy, it is easier to
overcorrect the advancement with the use of DO, which is
one of his stated objectives for young children (i.e., those
who are <11 years old). Standard extracranial Le Fort III
advancements with plate and screw fixation and bone grafts
are preferred by McCarthy for older children and adults
(i.e., those who are >11 years old), because this allows for
1. The ability to remove, segment, reshape and then
stabilize the cranial vault
2. The ability to separate the orbits and midface as a unit
MB from the skull base
3. The ability to segment and reshape the upper orbits
of the MB including interposing bone grafts as needed
to reconstruct each orbital aesthetic unit during a
single operative setting.
4. The ability to separate the MB into halves (facial
bipartition) and then three-dimensionally reposition
and stabilize (plate and screw fixation) the two facial
halves to achieve the most favorable morphology in
all three planes (i.e., pitch, role, yaw orientation).
This often requires increasing the maxillary width
(i.e., arch expansion) and decreasing the upper face
width (i.e., correction of hypertelorism of the orbits,
zygomas, and bitemporal regions). Facial bipartition
also provides the ability to correct the transverse facial
arc of rotation. An example is changing the concave
facial arc of rotation (i.e., yaw orientation) in Apert
syndrome.
In current clinical practice when either a monobloc or
facial bipartition osteotomy is selected, the technique of
“distraction” to gradually advance the upper/midface, after
closure of the wounds is frequently used. Any potential for
reduced infection across the skull base using the DO tech
nique should be considered in light of limitations to achiev
ing the aforementioned aesthetic objectives (points 1 to 4
mentioned previously). At the same time, clinicians must
take into account the procedure specific morbidity associ
ated with the DO technique including: 1) pin tract infec
tion; 2) soft tissue scarring; 3) hardware loosening requiring
re-application; 4) brain trauma from the screw penetration;
and 5) the need for device removal. 6) In addition, effective
DO technique depends on the patient’s, the patient’s family,
and the clinician’s continued commitment to staying the
course after surgery (approximately 4 months). It is hoped
that in the future, clinicians will both master the known surgi
cal techniques and also find novel solutions to manage the
potential for morbidity associated with MB and facial biparti
tion osteotomies (i.e., CSF leak, intracranial abscess, and bone
resorption) while focusing to the aesthetic details so that indi
viduals with a craniosynostosis syndrome can more commonly
benefit from the procedures inherent advantages.
Le Fort III Osteotomy (Extracranial Approach to
the Midface)
Shetye and colleagues set out to examine long-term (10-
year) midface skeletal stability and the potential for maxil
lary growth after extracranial Le Fort III advancements in
children with craniosynostosis syndromes.242 Their study
group included 25 children (10 had Crouzon syndrome, 9
had Apert syndrome, and 6 had Pfeiffer syndrome) who
underwent extracranial Le Fort III advancement before they
were 11 years old (mean, 5.8 years; range, 3.8 to 10.9 years).
1237
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
reduced treatment time and less patient psychosocial issues
(i.e., there is no need to wear an external DO device for 3
months after surgery).
McCarthy and colleagues recently reviewed the long-
term midface growth and periorbital/malar (upper midface)
aesthetic results in individuals who were born with a cra
niosynostosis syndrome and who had undergone Le Fort
III advancement.276 The study represented a follow-up
period of more than 20 years for four identified patients
(one with Apert syndrome and three with Crouzon syn
drome) who were 4, 6, 14, and 20 years old at the time of
the Le Fort III advancement. Thus, this study represented
two young children and two skeletally mature individuals
who underwent Le Fort III midface advancement. The
authors’ review confirmed several points: 1) a Le Fort III
advancement carried out during early childhood results in
no further horizontal growth of the midface; 2) a Le Fort
III advancement carried out during early childhood results
in further vertical growth of the midface with clockwise
rotation of the mandible; and 3) a Le Fort III advancement
carried out in individuals with either Crouzon or Apert
syndrome—whether during childhood or adulthood—
does not effectively correct the presenting zygomatic,
orbital, and nasal malformations. The upper midface aes
thetic results as viewed through the overlying soft-tissue
envelope (i.e., the eyelid–cheek–malar region) were subop
timal after Le Fort III advancement. Furthermore, with
age, the upper midface aesthetic results became even more
unfavorable.
McCarthy’s findings of continued vertical growth of the
midface after Le Fort III osteotomy carried out during early
childhood is consistent with the inability to achieve ade
quate nasal airflow in the operated patient. In addition, even
if adequate nasal airflow is accomplished, at least some
individuals will have limited masticatory muscle tone. In
either case, when a child continues with an open-mouth
protrusive tongue posture, hypereruption (i.e., vertical
growth) of the maxillary dentition through the alveolar
bone with clockwise rotation of the mandible is expected
(see Chapter 4). A lack of horizontal growth of the maxilla
after either a Le Fort I, Le Fort III, or MB osteotomy is
carried out during childhood has been documented by a
number of independent investigators.31,50,75,76,102,103,112,192
This results from both intrinsic growth limitations of the
malformation and injury to the sutural growth centers from
the osteotomies that are carried out.
Monobloc and Facial Bipartition Osteotomies:
Step-by-Step Description of the Surgical
Technique
The lack of consensus about the ideal timing and techniques
for the management of complex upper midface malforma
tions and deformities in patients with craniosynostosis syn
dromes reflects not only uncertainty about the potential
results with any one approach to treatment but also
confusion about how to perform these technically sensitive
procedures.
This section provides the surgeon with a step-by-step
technical description for the MB and FB osteotomies as this
author has routinely performed them. The detailed opera
tive technique is illustrated through the case of a 12-year-
old Persian girl who was born with Pfeiffer syndrome and
who had never undergone surgery. The patient then under
went FB osteotomies in combination with anterior cranial
vault reshaping (see Fig. 30-14). A review of the October
1992 Plastic Surgery Education Foundation Teleplast
Conference: Monobloc and Facial Bipartition for Recon
struction of Craniofacial Malformations may serve as
an additional educational tool to complete the surgeon’s
understanding of the technical aspects of the procedures
that will be described ( Video 15).
Monobloc and Facial Bipartition Osteotomies:
Step-by-Step Approach
Step 1. Satisfactory airway management in a patient who
is undergoing an MB/FB osteotomy is essential.
A method that I have used effectively involves an
orotracheal tube that is secured adjacent to the
cusp edge of the mandibular incisors with a cir
cummandibular wire (Fig. 30-16, A). After the
MB/FB osteotomies and disimpaction are com
pleted, a nasotracheal tube is placed, and the
orotracheal tube is removed (Fig. 30-16, B).
Through this controlled approach, endotracheal
tube injury at the completion of the osteotomies
and dislodgement during disimpaction are pre
vented. In addition, direct contact between the
maxillary and mandibular teeth can be achieved
for the improved control of the occlusion. The
nasotracheal tube remains in place at the end of
the procedure to allow for nasal mucosal stenting
during the initial postoperative phase. Other
approaches to managing the airway have been
described and used effectively (e.g., tracheostomy,
submental intubation, orotracheal intubation
without exchange).
Step 2. When feasible, I recommend the placement of
Crawford tubes to protect the nasolacrimal appa
ratus during surgery (Fig. 30-17). The puncta are
dilated, and a probe is inserted through each
punctum to confirm entrance into the nose. The
Crawford tube is inserted through each punctum
and pulled through the nose. Within the nose, the
Silastic sheeting is stripped; the tubing is tied in
the nose, and the excess is cut in the nose with
scissors.
Temporary tarsorrhaphies are placed with 6-0
nylon suture from gray line to gray line just lateral
to the pupil of each eye. As an alternative, corneal
shields may be used to protect the cornea, but this
1238 SECTION 4 Frequently Seen Malformations with Dentofacial Deformity
• Figure 30-17 A through D, Step 2.
A
C
D
B
• Figure 30-16 A and B, Step 1. Illustrations of a 12-year-old girl with Pfeiffer syn
drome (see Fig. 30-14) before and after facial bipartition. The step-by-step approach
for the surgical technique that was carried out is described in the text. From Posnick
JC: Craniofacial dysostosis syndromes: a staged reconstructive approach. In Turvery
JA, Vig TWL, Fonseca RJ, eds: Facial clefts and craniosynostosis: principles and
management, vol 26, Philadelphia, 1995, W.B. Saunders, p 645.
A
B
1239
CHAPTER 30 Syndromes with Craniosynostosis: Evaluation and Treatment
have been described (e.g., Z-plasty in the tempo
ral regions). Lidocaine with epinephrine is injected
to facilitate hemostasis.
Step 6. The anterior scalp flap is then elevated by remain
ing deep to the superficial layer of the deep
temporal fascia over the temporalis muscles;
subperiosteal over the mid-forehead region; sub
periosteal down the lateral orbital rims; subperi
osteal with exposure of the anterior maxilla;
subperiosteal for exposure of the zygomatic aches;
prevents the direct examination of the pupils
during surgery.
Step 3. Surgical arch wires are generally applied (Fig.
30-18). A throat pack is placed, and the mouth is
cleansed. Erich arch bars are applied to the maxil
lary and mandibular teeth. Circum-mandibular
wires are placed to further stabilize the mandibu
lar arch bar, and the orotracheal tube is secured
with a wire to the symphyseal region of the surgi
cal arch bar.
Step 4. The patient is prepared and draped. The patient’s
head is placed in a Mayfield (horseshoe-shaped)
head holder in the neutral neck position. Cleans
ing of the entire scalp is performed with povidone–
iodine (Betadine) soap; the soap is then washed
out with sterile water. Next, the application of
povidone–iodine solution to the scalp, face, and
neck is carried out. The surgical field is draped to
expose the neck down to the clavicles; the full
face, including the external ears; and the anterior
scalp back to the planned incision. The separation
of the mouth/nose from the eyes/forehead is
achieved with an additional sterile towel drape;
this is done to limit the contamination of the
intracranial cavity with oral or nasal flora.
Step 5. A coronal (scalp) skin incision is completed (Fig.
30-19). The incision site is postauricular and pos
terior in the scalp. Other incision modifications
• Figure 30-18 A through E, Step 3.
A
B
D
E
C
• Figure 30-19 Step 5.