Orthognathic Surgery

1141
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
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Orth
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E
F
E, Frontal views in repose before and after reconstruction. F, Frontal views with smile before and after
reconstruction.
• Figure 28-15, cont’d 
Continued

1142 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orth
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Orth
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Orth
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Orth
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G
H
G, Left oblique facial views before and after reconstruction. H, Right oblique facial views before and after
reconstruction.
• Figure 28-15, cont’d

J
K
Orth
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r ery; J.C. Posnick
I
I, Occlusal views with orthodontics in progress and after reconstruction. J, Frontal facial and
computed tomography scan views after reconstruction. K, Profile computed tomography scan views before and after
reconstruction that indicate mandibular malformation and costochondral graft reconstruction.
• Figure 28-15, cont’d

1144 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
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• Figure 28-16  A newborn with a bilateral form of hemifacial microsomia that is more severe on the left side than the
right and that involves the facial soft tissues and the jaws. The upper facial skeleton and the adnexal structures were
essentially normal. The patient had an adequate airway and feeding ability at the time of birth and throughout childhood.
At another institution when she was 7 years old, she underwent bilateral external distraction of the mandible. The procedure
was unsuccessful for improving the mandibular dysmorphology or the occlusion. When she was 11 years of age, at another
institution, the patient underwent a second mandibular distraction procedure with an internal device. This was also unsuc­
cessful for improving the mandibular malformation or malocclusion. The patient had also undergone bilateral external ear
reconstruction with a suboptimal result, and she wore external hearing aids. She presented to this surgeon when she was
15 years old with orthodontics in progress including 4 bicuspid extractions. She had a lifelong history of obstructed nasal
breathing and deformities of the maxilla and the mandible. There was a marked Class II anterior open-bite malocclusion.
An assessment was carried out that included an evaluation of the cervical spine, a complete computed tomography scan,
and evaluation by a speech pathologist and an otolaryngologist. Reconstruction was executed and included septoplasty
and inferior turbinate reduction; Le Fort I osteotomy (horizontal advancement, vertical adjustment, and counterclockwise
rotation); bilateral ramus osteotomies (horizontal advancement and counterclockwise rotation) with interpositional grafting;
and osseous genioplasty (vertical shortening and horizontal advancement). A, Facial views at 1 year of age, before any
intervention. B, Facial views at 7 years of age, with bilateral mandibular distractors in place.

1145
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
• Figure 28-16, cont’d 
C
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C, Facial views at 8 years of age (i.e., 1 year after mandibular distraction) that indicate residual maloc­
clusion and deformities of the maxilla and the mandible. D, Facial view at 11 years of age with bilateral mandibular distractions
in place.
Continued

1146 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
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E
E, Facial view, computed tomography scan, and occlusal views at 15 years of age at the time of referral to this
surgeon.
• Figure 28-16, cont’d

1147
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
G
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F
Distraction osteogenesis altered ramus of mandible
F, Profile and computed tomography scan views at time of referral to this surgeon. G, Articulated dental casts that
indicate analytic model planning with the “mandible first” technique (see Chapter 14).
• Figure 28-16, cont’d 
Continued

1148 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
• Figure 28-16, cont’d 
Orth
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H
I
H, Facial views with smile before and after reconstruction. I, Oblique facial views before and after
reconstruction.

1149
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
K
Prior to definitive jaw surgery after 4 bicuspid extractions
After treatment
J
J, Occlusal views with orthodontics in progress and after reconstruction. K, Lateral cephalometric
radiographs before and after reconstruction.
• Figure 28-16, cont’d 
NOTE: In this patient with HFM, the lack of effectiveness of the two mandibular distraction procedures
carried out in the mixed dentition is documented.
future growth of the reconstructed condyle–ramus unit. It
is currently impossible to estimate the optimal amount of
cartilage to be preserved in each individual case. Neverthe­
less, clinical and experimental studies support the notion
that, by including only a “small amount” of cartilage in the
CCJ/rib graft, less postoperative growth (i.e., overgrowth)
can be expected.
Peltomaki examined the growth of the mandible after
CCJ/rib graft condyle replacement in marmoset monkeys.
Interestingly, in adult animals, the amount of cartilage did
not make any difference with regard to the postoperative
mandibular measurements. In the growing animal, a post­
operative gradual deviation of the midline to the unoper­
ated side occurred. The greater the amount of cartilage left
in the graft at the time of transplantation, the greater the
amount of midline deviation observed over time.
For a series of human patients, Peltomaki reviewed the
histology of surgically removed overgrown CCJ/rib grafts
that were initially placed during childhood. On the basis of
the data, he believes that the unpredictable growth of the
constructed condyle–ascending ramus is a common occur­
rence. Clinical and histologic examination revealed that the
clinical type of overgrowth (i.e., linear versus exuberant) was
not related to any specific microarchitecture, which in itself
showed considerable variation. This suggested that other
local factors (e.g., mandibular movement, loading of the
reconstructed condyle) may also have an effect on the sub­
sequent and eventual growth and overgrowth.

1150 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
in patients with HFM have been carried out. The transposi­
tion of a pedicled upper eyelid skin–muscle flap to the lower
eyelid’s deficient region is not technically difficult, but the
resulting adnexal scarring inevitably results in an operated
look that generally detracts from any positive advantages of
the procedure. For this reason, it may be best for the patient
and the family to consult an experienced pediatric oculo­
plastic surgeon when considering even minor eyelid surgical
procedures for the patient with HFM. Decisions about the
removal of epibulbar or bulbar dermoid cysts from the
adnexal region are based on eye function and eyelid
aesthetics.
The inferior displacement of the lateral canthus in a
patient with HFM is a reflection of both orbital dystopia
and hypoplasia of the lateral canthi and other components
of the eyelid structures. Correction of the orbital dystopia
through osteotomies and bone grafting and the completion
of a direct lateral canthopexy are helpful and should be
carried out when indicated, but these procedures will not
fully correct hypoplasia of the lateral canthal complex.
When temporal fossa hollowing is clinically noticeable in
the patient with HFM, it is a reflection of multilevel hypo­
plasia that may include that of the skin, the temporoparietal
fascia, the temporalis muscle, and the squamous portion of
the temporal bone. Attempts to fill the soft-tissue defect
with local pericranial or temporoparietal flaps are generally
not effective, because these tissues are part of the deficiency.
The intrinsic hypoplasia of these flaps (i.e., of the temporo­
parietal fascia and the temporalis muscle) also explains why
they have limited utility for adnexal region subcutaneous
augmentation.
For many patients, the extent of soft-tissue hypoplasia in
the preauricular–cheek region will be distinctly noticeable at
conversational distance, even after effective skeletal recon­
struction.61,86,89,91,117,123,124,149,204,205,244,245,258,268,269 This results
from multilevel hypoplasia of the subcutaneous tissue; the
fat; the muscles of mastication and facial expression; and
the parotid gland. For these patients, a well-designed soft-
tissue “free” vascularized composite flap should be consid­
ered. When meticulously designed and expertly executed,
the transfer of soft-tissue flaps from other body regions
(with microvascular reanastomosis) may be the alternative
of choice. Parascapular free flaps have become the work­
horse for the reestablishment of facial soft-tissue volume
since this process was first described by Dos Santos.40 Siebert
and colleagues found that the parascapular flap allows for
the reasonable correction of contour defects and yields
improved facial aesthetic results in selected patients with
HMF while minimizing scarring by providing vascularized
soft tissue in the subcutaneous plane.244,245 Fortunately,
hypoplasia of the centrally located soft tissues of the face
(i.e., the nose, the medial aspects of the upper and lower
lips, and the submental region) is not seen in patients with
HFM. The soft-tissue reconstruction requires an experi­
enced microvascular surgeon working closely with a maxil­
lofacial and a auricular surgeon to coordinate the flap inset;
to minimize recipient-site morbidity; and to coordinate
In general, for a first-stage mandibular procedure that is
carried out during childhood to be considered successful in
a patient with HFM, the growth of the reconstructed side
of the mandible should equal or at least be close to that of
the contralateral normal mandible. Unfortunately, despite
decades of surgical experience with the use of the CCJ/rib
graft as a reconstructive option for the missing condyle–
ascending ramus in the growing patient with HFM, this
option has not passed the test. Overgrowth is a frequent
and serious consequence. The mechanism that causes this
postsurgical growth disturbance is now clear. Predictable
methods of overcoming this complication while still allow­
ing the CCJ/rib graft to be effective have not been
realized.
Osteodistraction of the Residual Mandibular
Deformity after Costochondral Graft
Placement during Childhood
Wan and colleagues reported their findings for the recon­
struction of Type IIB and III mandibular malformations in
patients with HFM.279 Their basic approach was to recon­
struct the ipsilateral mandible with a CCJ/rib graft (N = 30
grafts, 27 patients) during the mixed dentition (mean age,
9.9 years ± 4.1 years). As a result of a high failure rate, this
was followed by DO treatment of the residual deficient
mandible. CCJ/rib graft failure that required regrafting
occurred in 7 out of 30 cases (23%). Undergrowth that
required DO during the mixed dentition occurred in 17
cases (57%). Overgrowth with severe distortion that required
surgical correction occurred in 3 of the 30 cases (10%). For
the majority of study patients who later required CCJ/rib
graft DO, the complication rate was exceedingly high. In
addition, all study patients (N = 27) required definitive
orthognathic surgery at growth maturity, despite earlier pro­
cedures having been carried out.
Corcoran and colleagues presented a series of 8 patients
with HFM who underwent mixed dentition costochondral
reconstruction and who then submitted to DO.33 They
found a 62.5% complication rate. Stelnicki and colleagues
similarly reported on 9 patients who underwent mixed den­
tition CCJ/rib graft reconstruction and then required
distraction.252A They reported a 33% rate of fibrous non­
union as well as other significant complications and limited
ultimate success. On the basis of current published studies,
it must be concluded that first-stage mandibular reconstruc­
tion with the use of CCJ/rib grafts during the mixed
dentition for patients with Type IIB and Type III HFM
deformities is fraught with complications and achieves
limited success. The theory that a mandible reconstructed in
this way can be reliably “distracted’ later if it does not grow
normally has proved to be false.
Facial Soft-Tissue Reconstruction
A variety of well-intentioned surgical attempts to correct
the soft-tissue deficiencies of the eyelid adnexal regions

1151
CHAPTER 28  Hemifacial Microsomia: Evaluation and Treatment
foreign substances are more susceptible to infection, soft-
tissue wound dehiscence, and minor trauma, even decades
after reconstruction. Techniques of tissue engineering with
bovine cartilage cells continue to be tested; these cells can
be grown in the laboratory and seeded on a synthetic bio­
degradable ear template that is then implanted beneath the
skin of an immunocompetent mouse. Unfortunately, until
tissue engineering evolves beyond the currently encountered
immunogenic problems, sculpted autogenous rib cartilage
will remain the material of choice for the surgical repair of
the ear.
Before completing microtia reconstruction, a decision
must be made about whether middle-ear surgery will even­
tually be carried out. Auricular construction should precede
any middle-ear surgery; after an atretic ear canal is opened,
the chances of obtaining a satisfactory auricular reconstruc­
tion are severely compromised as a result of scarring of the
soft-tissue envelope. Consideration should also be given to
the need for condyle-ascending ramus reconstruction (i.e.
Type IIB and Type III malformations). If CCJ/rib graft
harvesting will also be required then the location of each
donor site becomes even more important.
External Auditory Canal and
Middle-Ear Reconstruction
Occasionally, neurosensory hearing loss is present in the
patient with HFM, but hearing loss is generally attributable
to external auditory canal stenosis or atresia, hypoplasia
of the middle-ear cavity, or ankylotic or missing ossi­
cles.17,18,223,280 Ankylotic or non-functioning ossicles limit
hearing conduction to the same extent that would occur if
the ossicles were absent. Generally, attempts to reconstruct
the external auditory canal and the bones of the middle ear
for patients with HFM are not carried out. As long as
adequate hearing is present in the contralateral ear, clinical
problems generally relate only to the patient’s ability to
locate the origin of the sound. For most patients, attempts
to restore middle-ear function involve residual conductive
hearing loss to the extent that “stereo” hearing is rarely
achieved.
Bone-Anchored Hearing Aid Option
A bone-anchored hearing aid (BAHA) consists of a perma­
nent titanium fixture that is surgically implanted into the
skull bone behind the ear and a small detachable sound
processor that clips onto the fixture. BAHAs are suitable for
people with conductive or mixed hearing loss who cannot
adequately benefit from or who prefer not to use conven­
tional hearing aids.
Colquitt and colleagues completed a systematic review
and economic evaluation of BAHAs for people who are
bilaterally deaf. 29 Previous prospective studies of adults or
children with bilateral hearing loss were eligible for review.
Twelve studies were included; there were seven cohort
skeletal and soft-tissue reconstruction. The timing of soft-
tissue preauricular–cheek region reconstruction is generally
planned to follow the skeletal reconstruction.
Advocates of the DO technique for mandibular recon­
struction in patients with HFM had hoped that the soft
tissues (i.e., the skin, subcutaneous tissue, and muscle)
would increase in bulk when this method is used. However,
this hoped-for advantage has not been documented by clini­
cal studies. Of importance is that effective skeletal recon­
struction by whatever means will favorably influence the
aesthetic appearance of the soft-tissue envelope; however, it
will not directly increase the soft-tissue volume.
Dermal fat grafts, autogenous fat injections, and other
forms of soft-tissue augmentation have gained in popularity.
The results of autogenous fat injection are variable and
considered technique sensitive, but are generally not harmful
and clearly promising. Tanna and colleagues investigated
the use of serial autologous fat grafting to restore soft-tissue
contour in patients with HFM.259 Patients with moderate
to severe HFM were divided into two groups. Group I
included those who were undergoing microvascular free-
flap reconstruction (i.e., inframammary extended circum­
flex scapular flaps, n = 10). Group II included patients who
were undergoing multiple staged autogenous fat grafting (n
= 21). The two patient groups had similar OMENS scores
(2.4 and 2.3, respectively) and similar pre-reconstruction
facial symmetry scores (74% and 75%, respectively). During
the final evaluation, facial symmetry scores were 121% for
the microvascular free-flap group and 99% for the fat-
grafting group. Furthermore, no statistically significant dif­
ference between the microvascular and fat graft groups with
regard to either patient or physician rating of overall satis­
faction was noted.67,107,259
External Ear Reconstruction
In the hands of a few experts, surgical reconstruction of the
external ear can be satisfactorily achieved with a staged
approach.8,10-15,54,260,261 The methods described by Brent
continue to represent the reference standard for auricular
reconstruction.10-15 The successful placement of a well-
sculpted autogenous cartilage framework is the foundation
for a sound auricle repair. Brent prefers to wait until the
patient is at least 6 years old, when rib cartilage is adequate
for the reconstruction in most children. The synchondrotic
region of the sixth and seventh ribs will then provide an
ample cartilage block to use to form a framework for the
ear. With the use of a prefabricated template of the contra­
lateral ear and preoperatively determined measurements of
the face, the ear’s position and size are chosen, and a small
preauricular incision is made. Other stages of auricular con­
struction include lobule transposition, the detachment of
the ear with a skin graft, hairline management, and tragus
construction. Other options for external ear reconstruction
include the use of alloplastic and homologous frameworks.
Alloplastic materials currently in use include silicone or
Medpor (Porex Surgical, Inc, College Park, Ga). These

1152 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
provide audiologic benefit as compared with unilateral
BAHAs (i.e., improved thresholds for tones, speech in quiet
and in noise, improved localization/lateralization, and
patient’s perceived subjective benefit). Disadvantages of
bilateral BAHAs included 1) listening in noise in some
condition 2) presumed additional cost and 3) presumed
increasing adverse event risk. The authors concluded that
bilateral BAHAs provided additional objective and subjec­
tive benefit as compared with unilateral BAHAs.
Conclusions
Hemifacial Microsomia generally occurs sporadically and
without a familial pattern. It is characterized by variable
deficiencies of the skeletal and soft-tissue structures within
the nasal placode and the first and second branchial arches,
primarily on one side. Considerations during infancy and
early childhood center on airway, feeding, hearing, vision,
and the need for psychosocial family support. The degree
of malformation present at the time of birth in the patient
with HFM is believed to be relatively stable and non-
progressive with age.
The current approach to the correction of the malforma­
tions associated with HFM is to stage the reconstruction to
coincide with facial growth patterns, visceral functions, and
psychosocial development. A precise morphologic analysis
of each patient’s anomalies followed by a broad-based recon­
structive plan serves to clarify for the clinicians and the
family the objectives of each stage and method of treatment.
As we continue to define our rationale for the timing,
methods, and extent of interventions and then to objec­
tively evaluate the functional, morphologic, and psychoso­
cial outcomes, we will improve the outlook for individuals
who are affected by HFM.
pre-post studies and five cross-sectional audiologic compari­
son studies. Comparisons were made between BAHAs and
the following: 1) conventional hearing aids, which are also
known as air-conduction hearing aids (ACHAs) 2) bone-
conduction hearing aids (BCHAs) and 3) unaided hearing
and ear surgery. The study also looked at 4) unilateral versus
bilateral BAHAs. Outcomes reviewed included hearing
measures, validated measures of quality of life, adverse
events, and measures of cost-effectiveness.
There appeared to be some audiologic benefits of BAHAs
for this group of patients including 1) improvements in
speech as compared with BCHAs and 2) understanding and
noise as compared with ACHAs (however, ACHAs may
produce better audiologic results for other outcomes). In
addition, 3) hearing was found to be improved with the
use of BAHAs as compared with unaided hearing and 4)
studies that compared unilateral with bilateral BAHAs sug­
gested the benefits of bilateral BAHAs in many situations.
However, prospective case series of BAHAs reported
rates of 6.1% to 19.4% for the loss of the implants over
time. In addition, the financial analysis suggests that BAHAs
may not be a cost-effective option in terms of hearing gain
and probability of needing alternative aids. Interestingly, the
greater the benefit to the individual from aided hearing and
the greater the difference in the proportion of people using
the hearing aid for more than 8 hours per day, the more
likely the BAHAs were a cost-effective option.
Janssen and colleagues completed a systematic review
of bilateral BAHAs for bilateral permanent conductive
hearing loss.88 This was a review of the literature from
between 1977 and 2011, and it included studies in which
subjects of any age had permanent conductive hearing loss
and bilateral implanted BAHAs. Eleven studies met the
criteria for data analysis. Bilateral BAHAs were found to
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1159

29
Binder Syndrome: Evaluation
and Treatment
JEFFREY C. POSNICK, DMD, MD
• Current Approach to Reconstruction
• Skeletal Stability after Orthognathic
Reconstruction
• Controversies and Unresolved Issues
• Conclusions
the anterior maxilla including the pyriform rim region.26,33
When viewing the nose–upper lip complex from the worm’s-
eye perspective, typical variations from normal are described
as a retracted columella–lip junction, a lack of normal tri­
angular flare at the nasal base, a perpendicular alar–cheek
junction, a convex flat nasal tip with a wide and shallow
philtrum, crescent-shaped nostrils without a distinct sill,
and a stretched and shallow cupid’s bow.27,41 Striking profile
characteristics of the nose include vertical shortening of the
columella, a lack of tip projection, perialar flattening, and
an acute nasolabial angle.11,28 The dentition and occlusion
in an untreated individual will typically demonstrate the
proclination of the maxillary incisors, “peg” laterals, and a
canine Angle class III negative overjet tendency.21
Anthropometric explanations for these findings were first
suggested by Zuckerkandl in 1882, when he described an
anomaly in the anterior nasal floor in which the normal
crest that separates the nasal floor from the anterior surface
of the maxilla was absent.56 Instead, a small pit—the fossa
praenasalis—constituted the pyriform aperture.18,24 Other
investigators have pointed out that the premaxilla of normal
Caucasian individuals is incorporated into the upper arch.2,3
This results in a prominence or projection of the base of the
nose. By contrast, when the premaxilla (i.e., the primary
palate) is not incorporated into the arch (i.e., in higher
primates, certain racial groups, persons with Binder syn­
drome, and individuals with bilateral clefted alveolar ridges
and lips), there will be flattening of the premaxillary region
and of the base of the nose.6 This is more commonly seen
among African and Asian people.10
In an attempt to reconstruct the skeletal deformities
associated with Binder syndrome, clinicians have sug­
gested a spectrum of procedures, including Le Fort I
osteotomy, Le Fort II osteotomy, Le Fort III osteotomy,
and a combination of Le Fort I and II osteoto­
mies.4,8,12,17,19,20,22,23,31,36,39,42,44,49,51,55 Augmentation of the
infraorbital rims, the pyriform rims (paranasal), and the
anterior maxilla with the use of a spectrum of materials
have all been tried.7,25,40,50 Suggested nasal reconstructive
In 1939, Noyes first described a patient whose face was
characterized by a flat nasal tip and a retruded maxillary–
nasal base.32 He did not recognize this as an entity unique
from other known forms of maxillary retrusion. It was not
until 1962 that von Binder came to recognize the specific
entity of nasomaxillary hypoplasia that is now called Binder
syndrome.53 He described physical findings of nasomaxillary
hypoplasia, a convex lip, a vertical (short) nose, a flat fron­
tonasal angle, an absent anterior nasal spine, limited nasal
mucosa, and hypoplastic frontal sinuses. von Binder postu­
lated that the hypoplasia was the result of a disturbance
of the prosencephalic induction center at a critical phase
during development. In 1989, Sheffield and colleagues
reviewed 103 cases of chondrodysplasia punctata (CDP)
seen in Melbourne, Australia, over a 20-year period.48 They
concluded that Binder syndrome should be classified as a
mild form of CDP. In 1991, Sheffield and colleagues
pointed out that most patients with Binder syndrome seek
medical attention during adolescence.47 By this age, the
confirmatory diagnostic radiologic features of CDP have
disappeared, so the diagnosis of CDP is often not consid­
ered. Older patients may show terminal phalangeal hypo­
plasia of the hand and variable anomalies of the vertebrae
(i.e., vertebral clefting). Associated malformations of the
cervical spine primarily affect the atlas and the axis without
known clinical sequelae.35,43 Familial recurrence has been
reported, and inheritance may occur as an autosomal reces­
sive trait with incomplete penetrance.34 The syndrome may
also be of a threshold character with a genetically multifac­
torial background.5,14,45
The physical findings of Binder syndrome result from
hypoplasia of the anterior nasal floor (fossa praenasalis), and

1160 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
patient and family. The most gratifying long-term functions
(i.e., occlusion and breathing) and facial aesthetics are gen­
erally achieved when carrying out the reconstruction after
the completion of growth and before the individual’s gradu­
ation from high school (Fig. 29-1 through 29-5). Ortho­
dontic treatment should be coordinated with consideration
of orthognathic correction and premaxillary augmentation
to be followed by definitive nasal reconstruction. The ideal
orthodontic treatment often includes maxillary first bicus­
pid extractions with retraction and alignment of the ante­
rior teeth to produce ideal incisor inclination within solid
basal bone. Unfortunately, many individuals with Binder
syndrome will have already been treated with an orthodon­
tic camouflage approach. During the process, the maxillary
incisors are tipped facially, and the mandibular incisors are
often retroclined. This will typically achieve successful neu­
tralization of the occlusion (i.e., the correction of overjet),
but it will leave the patient looking as if he or she has a
maxillary deficiency. Attempts at surgical augmentation
after orthodontic camouflage are generally suboptimal.
When indicated, definitive orthognathic reconstruction is
planned to idealize the vertical, transverse, and horizontal
midface proportions. A Le Fort I osteotomy (horizontal
advancement and a variable degree of vertical lengthening)
is frequently combined with an osseous genioplasty (vertical
shortening and horizontal advancement). Sagittal split
ramus osteotomies are often needed to avoid the limitations
inherent to mandibular autorotation. Further reconstruc­
tion involves the application of a crafted bone graft to the
deficient premaxillary and pyriform rim regions. This is
best accomplished simultaneously with the orthognathic
procedures.
options have included autogenous, homogenous, and allo­
genic bone and cartilage grafts that extend up the colu­
mella (i.e., from the base of the maxilla to the nasal tip)
and over the nasal dorsum (i.e., from the radix to the
tip).1,9,13,15,19,29,30,38,39,46,52 Suggested soft-tissue procedures
include septal cartilage and mucosal flaps, upper lip to
nasal skin flaps to lengthen the columella, and a variety
of subcutaneous augmentation procedures and fillers.37,54
Compensating orthodontic or dental restorative work may
also be undertaken.
With Binder syndrome, a degree of hypoplasia of the
premaxilla and of the quadrangular cartilage of the nasal
septum is a consistent finding. This author agrees with
Holmstrom and colleagues that, with this anomaly, there is
a local shortage of bone in the premaxillary region and an
absence of cartilage in the anterior septum.18-20 However, as
Tessier pointed out, there is no significant shortage of soft
tissue available.50-51 Furthermore, modification of the unin­
volved orbits, zygomas, and upper nasal dorsum (i.e., the
nasal bones) is rarely indicated or advantageous. The
observed facial features in an individual with Binder syn­
drome are dependent on the degree of hypoplasia of the
anterior nasal floor (fossa paranasalis) present at the time of
birth. The deformities are believed to be non-progressive
with age.
Current Approach to Reconstruction
The approach to the correction of the Binder syndrome
deformity is to plan for a staged reconstruction to coincide
with facial and dental growth patterns and psychosocial
needs.16,39 An analysis of each patient’s morphology is fol­
lowed by a review of the reconstructive options with the
©Elsevier Inc.
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A
• Figure 29-1  A 17-year-old boy, who was born with Binder syndrome was referred
by his orthodontist for surgical evaluation. When he was 7 years old he underwent
nasal augmentation with Silastic implants with another surgeon. Orthodontic treatment
including maxillary first-bicuspid extractions and orthognathic and nasal surgery was
chosen. The patient’s procedures included a Le Fort I osteotomy (horizontal advance­
ment); the removal of nasal implants; osseous genioplasty (horizontal advancement);
and nasal reconstruction (corticocancellous iliac graft). A, Facial views at 10 years old
with Silastic implants in place.
Text continued on p. 1177

1161
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
C
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orth
r ognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
B
B, Frontal views with smile before and after reconstruction. C and D, Profile views before
and after reconstruction.
• Figure 29-1, cont’d 
Continued

1162 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
D
E
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
E, Worm’s-eye views before and after reconstruction.
• Figure 29-1, cont’d

1163
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
Prior to treatment
Prior to surgery after maxillary bicuspid extractions
After treatment
F
F, Occlusal views before and after reconstruction.
• Figure 29-1, cont’d 
Continued

1164 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
H
G
I
H
G, Articulated dental casts that indicate analytic model planning. H, Intraoperative views of removed
Silastic nasal implants. The crafted iliac corticocancellous bone graft before inset is also shown. I, Lateral cephalometric radio­
graphs before treatment and after reconstruction. A (right), B, C, F, H, I, From Posnick JC, Tompson B: Binder syndrome: Staging
of reconstruction and skeletal stability and relapse patters after Le Fort I osteotomy using miniplate fixation, Plast Reconstr Surg
99:967, 1997.
• Figure 29-1, cont’d

1165
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
A
B
• Figure 29-2  A 16-year-old girl who was born with Binder syndrome was referred by her orthodontist for surgical
evaluation. She agreed to orthodontic treatment that included maxillary first bicuspid extractions and orthognathic
and nasal surgery. The patient’s procedures included Le Fort I osteotomy (horizontal advancement); bilateral sagittal
split ramus osteotomies; osseous genioplasty; and nasal reconstruction (autogenous rib graft). A, Frontal views in
repose before and after reconstruction. B, Frontal views with smile before and after reconstruction.
Continued

1166 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
C
D
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
Orthognathic Surgery; J.C. Posnick
C, Oblique facial views before and after reconstruction. D, Profile views before and after
reconstruction.
• Figure 29-2, cont’d

1167
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
E
©Elsevier Inc.
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Prior to surgery after maxillary bicuspid extractions
After treatment
F
G
Maxillary
clockwise
rotation
Mandibular
counter-
clockwise
rotation
E, Worm’s-eye views before and after reconstruction. F, Occlusal views before and after recon­
struction. G, Articulated dental casts that indicate analytic model planning.
• Figure 29-2, cont’d 
Continued

1168 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
I
J
H
H, Intraoperative views of open rhinoplasty exposure. The rib bone (dorsal strut) and rib
cartilage (caudal strut) grafts are shown before inset. I, Computed tomography scan and intraoperative views
that confirm premaxillary hypoplasia consistent with Binder syndrome. J, Lateral cephalometric radiographs
before and after reconstruction. B, F (top center and bottom center), H, From Posnick JC, Tompson B: Binder
syndrome: Staging of reconstruction and skeletal stability and relapse patters after Le Fort I osteotomy using
miniplate fixation, Plast Reconstr Surg 99:965, 1997.
• Figure 29-2, cont’d

1169
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
C
©Elsevier Inc.
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O hththththththththththththththththththththththththththththththththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatathicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicick
A
B
A
• Figure 29-3  A 16-year-old girl who was
born with Binder syndrome was referred
by her orthodontist for surgical evaluation.
She agreed to orthodontic treatment that
included maxillary first bicuspid extractions
and orthognathic surgery. The patient’s proce­
dures included Le Fort I osteotomy (horizontal
advancement); osseous genioplasty (horizon­
tal advancement); and nasal reconstruction.
The nasal reconstruction (cranial graft) was
complicated by pressure necrosis of the nasal
tip skin, which necessitated the removal of
the distal aspect of the graft. Six months
later, through an open rhinoplasty approach,
autogenous rib cartilage (a caudal strut)
was used to revise the nasal tip. A, Frontal
views in repose before and after reconstruc­
tion. B, Profile views before and after recon­
struction. C, Worm’s-eye views before and
after reconstruction.
Continued

1170 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
E
Maxillary
clockwise
rotation
F
Prior to surgery after maxillary bicuspid extractions
After treatment
D
D, Occlusal views during orthodontics and after reconstruction. E, Articulated dental casts that indicate
analytic model planning. F, Lateral cephalometric radiographs before and after reconstruction.
• Figure 29-3, cont’d

1171
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gnatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatathicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicici k
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O thththththththththththththththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g
tatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatathicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicicicicicicicicicicicicicicicicicicicicick
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatathicicicicicici Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicici k
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orththththththththththththththththththththththththththththththththththththththththththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicicicici k
A
B
• Figure 29-4  A 17-year-old boy who was born with Binder syndrome was referred for surgical
evaluation. He agreed to orthodontic treatment that included maxillary first bicuspid extractions and
orthognathic and nasal surgery. The patient’s procedures included Le Fort I osteotomy (horizontal
advancement) and nasal reconstruction (autogenous rib graft). The nasal reconstruction was carried
out through an open (columella splitting) technique. The skin of the columella was stretched but not
directly lengthened. Autogenous rib bone (dorsal strut) and rib cartilage (caudal strut) were harvested,
crafted, and then inset and secured in place. A, Frontal views in repose before and after reconstruc­
tion. B, Frontal views with smile before and after reconstruction.
Continued

1172 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
Orthognathicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
o nicicicicicicicicicicicicick
Orthognathicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C Pos
os
os
osnicicicicicicicicicicicicick
Orththththththththththththththththththt ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnick
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththththththththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicicic Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicick
C
D
C, Close-up profile views before and after reconstruction. D, Worm’s-eye
views before and after reconstruction.
• Figure 29-4, cont’d

1173
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
Prior to treatment
After treatment with maxillary bicuspid extractions
F
E
E, Intraoperative views of open rhinoplasty exposure. A Kirschner wire has been inserted into the base of the
maxilla. The Kirschner wire extends out of bone. The rib cartilage (caudal strut) will pierce the Kirschner wire and extend to the nasal
tip. The crafted rib bone (dorsal strut) is shown before inset. It will join to the caudal graft to form the nasal tip. F, Occlusal views
before and after reconstruction.
• Figure 29-4, cont’d 
Continued

1174 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
G
H
G, Articulated dental casts that indicate analytic after model
planning. H, Lateral cephalometric radiographs before and after reconstruction.
• Figure 29-4, cont’d

1175
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
C
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O thththththththththththththththththogn
gn
gn
gn
gnatatatatathicic Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C Posnick
©Elsevier Inc.
Or
Or
Or
Or
Orththththththththththththththt ogn
gn
gn
gn
gn
gn
gnatatatathicicicic Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C Posnick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O ththththththththththththththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatathicicicicicicicicicicicicici Su
Su
Su
Su
Su
Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
o nicicicicicicicicicicicicicicicicicicicicicicicicicicicici k
©Elsevier Inc.
Or
Or
Or
Or
Or
O ththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatathicicicicicicicicic Su
Su
Su
Su
Su
S rgery
ry
ry; J.C.
C.
C.
C.
C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
o nicicicicicicicicici k
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Orthththththththththththththogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatatata hicicicicicicicicicicicicicicicicici Su
Su
Su
Su
Su
Surgery
ry
ry
ry
ry
ry
r ; J.C.
C.
C.
C.
C.
C.
C.
C.
C. Pos
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
os
osnicicicicicick
©Elsevier Inc.
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
Or
O thththththththththththththththththththththththth
r ogn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
gn
g atatatatatatatatatatatatatatatatatata hicicicicicicicicicicic Su
Su
Su
Su
Su
S rgery
ry
ry
ry
ry
ry
ry
ry
ry
ry; J.C.
C.
C.
C.
C.
C.
C Pos
os
os
os
os
os
os
os
os
os
o nicicicicicicicicicicicicicick
A
B
• Figure 29-5  A 16-year-old girl who was born with Binder
syndrome underwent Le Fort I osteotomy and septal carti­
lage graft to the dorsum of the nose with another surgeon.
A residual nasal deformity remained. She was referred to
this surgeon and underwent nasal reconstruction. Through
a coronal scalp incision, a full-thickness cranial bone graft
was harvested, crafted, and stabilized in place. The graft
extended from the radix to the nasal tip. The lower lateral
cartilages were then sutured over the top of the graft to form
the new nasal tip. A, Close-up profile views before and after
nasal reconstruction. B, Close-up frontal views before and
after nasal reconstruction. C, Close-up worm’s-eye views
before and after nasal reconstruction.
Continued

1176 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
E
D
F
D, Harvested full-thickness cranial bone before and after crafting for nasal reconstruction. E, Intraoperative
close-up views of the nasofrontal process before and after the placement and stabilization of the cranial bone graft. F, Lateral cepha­
lometric views before and after nasal reconstruction. D, From Posnick JC, Seagle MB, Armstrong D: Nasal reconstruction with full-
thickness cranial bone grafts and rigid internal skeletal fixation through a coronal incision, Plast Reconstr Surg 86:894, 1990.
• Figure 29-5, cont’d

1177
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
aperture. All grafts to the upper jaw (five of seven patients)
were autogenous corticocancellous iliac bone. After the
titanium plates and screws were in place to stabilize the
osteotomy, corticocancellous bone grafts were crafted to
reconstruct the area of hypoplasia of the premaxilla. The
grafts were fixed with additional plates and screws. Simul­
taneous sagittal split ramus mandibular osteotomies were
carried out in three of seven patients. The majority of
patients (six of seven patients) also underwent osseous
genio­plasty with horizontal advancement and varying
degrees of vertical shortening. Six of seven patients under­
went autogenous grafting of the nose (costochondral grafts
in four patients, a cranial graft in one patient, and an iliac
graft in one patient). One patient underwent a coronal scalp
incision, the others (five of six patients) underwent an open
rhinoplasty (i.e., columella skin-splitting incision) approach.
Only one of the seven patients underwent simultaneous
orthognathic surgery and nasal reconstruction.
Results
The serial cephalometric radiographs for each patient were
analyzed, and the preoperative and immediate postoperative
tracings were superimposed. The end result was a Cartesian
coordinate system that illustrated the horizontal and vertical
directional changes of the maxilla at intervals after surgery.
In addition, the incisor overjet and overbite measurements
from the 1-year postoperative cephalogram were docu­
mented and compared with the measurements taken at the
final clinical assessment. All patients had a complete set of
longitudinal clinical and cephalometric records, and all were
available for late postoperative clinical reassessment at the
close of the study. Clinical follow-up ranged from 1.5 to
5.5 years (mean, 3 years) at the completion of the study.
Perioperative morbidity was unremarkable when patients
were reviewed for cardiopulmonary compromise, maxillo­
facial infection, hemorrhage, aseptic necrosis, loss of teeth,
and need for root canal therapy. All patients maintained a
positive overjet and overbite at the incisors as documented
on the 1-year postoperative clinical and cephalometric
examinations. The mean effective maxillary advancement
achieved was 6.0 mm, with 5.9 mm maintained 1 year later.
The mean anterior vertical change of the maxilla was
4.2 mm immediately after the operation and 3.1 mm after
1 year. The mean posterior vertical change of the maxilla
was 2.8 mm immediately after the operation and 2.2 mm
after 1 year. In review, the fixed and grafted Le Fort I oste­
otomies were able to maintain the horizontal advancement.
Although a degree of anterior vertical relapse occurred, the
long-term maintenance of satisfactory occlusion with a
positive overjet and overbite was documented in all patients.
Controversies and Unresolved Issues
The precise genetic cause of Binder syndrome has not
yet been confirmed. Gorlin and colleagues suggested
that maxillonasal dysplasia was a non-specific abnormality
Ideally, the nasal reconstruction is carried out after
the orthognathic and anterior maxillary augmentation
procedures (see Fig. 1-5).38,39 This is accomplished by recon­
structing the cartilaginous nasal deficiency. Stretching of the
soft-tissue envelope over the reconstructed underlying nasal
cartilage framework is accomplished at wound closure.
When first-stage early nasal augmentation was carried out
during childhood and before orthognathic correction, some
profile improvements maybe achieved, but a relief of the
stigma of Binder syndrome is not likely. Early nasal aug­
mentation also imposes limitations on the child’s physical
activity to prevent graft fracture or displacement. These
restrictions on the daily activities of childhood are likely to
be more psychosocially traumatic than the original nasal
deformity. For effective reconstruction, all aspects of the
deformity should be addressed with a coordinated long-
term approach in mind.
Skeletal Stability after Orthognathic
Reconstruction
Few authors have looked at the early or late skeletal stability
achieved when osteotomies are carried out to correct
the Binder syndrome midface deficiency. In a previously
published study, Posnick and colleagues prospectively
assessed initial and long-term skeletal stability after orthog­
nathic reconstruction including a Le Fort I osteotomy fol­
lowed by nasal reconstruction in a consecutive series of
skeletally mature Caucasian individuals born with Binder
syndrome.39
Patients and Methods
The medical and cephalometric records and late posttreat­
ment (surgical and orthodontic) clinical examination of a
consecutive series of patients with Binder syndrome (n = 7)
who underwent Le Fort I osteotomy by a single surgeon
(Posnick) during a 6-year period were reviewed. The patients’
ages at operation ranged from 16 to 20 years (mean, 17
years). For all study patients, the prospective protocol
required obtaining a lateral cephalogram preoperatively,
immediately (i.e., 3 to 7 days) after surgery, 6 to 8 weeks
after surgery, and 1 year after surgery. At the time of final
review, all patients had obtained their interval cephalograms
and were available for a final clinical evaluation.
All patients underwent orthodontic treatment, orthog­
nathic surgery, and nasal reconstruction. During the
initial evaluation, all study patients were judged to have
inadequate alveolar bone to retain the full complement of
maxillary teeth and to maintain long-term periodontal
health. Extraction of maxillary first bicuspids was carried
out to remove dental compensations and to establish normal
dentoalveolar arch form and incisor inclination. All patients
underwent a standard one-piece Le Fort I osteotomy
extended through the zygomatic buttress (below the malar
eminence) and anteriorly through the hypoplastic pyriform

1178 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
study by Posnick and colleagues, bilateral sagittal split
osteotomies were also necessary to correct the secondary
mandibular dysmorphology or to prevent the inherent
shortcomings of mandibular autorotation.39 In none of the
patients was the mandible “set back” as a camouflage for
maxillary deficiency.39
Binder syndrome is not an orbital, zygomatic, nasal
bone, or cranial vault malformation. Over time, it has been
found that alteration of these regions is counterproductive.
A Le Fort III osteotomy would have the disadvantage of
flattening the nasofrontal angle, producing enophthalmos,
and resulting in distortions (step-offs) of the lateral orbital
rims. A Le Fort II osteotomy would also flatten the naso­
frontal angle, produce enophthalmos, and result in distor­
tions (step-offs) along the infraorbital rims. In addition,
neither procedure would directly address the nasal septal
cartilaginous vault malformation nor address the deficient
premaxillary region.
In my experience, corticocancellous iliac bone offers
a preferred volume, quality, and osteogenic potential to
reconstruct the anterior alveolus–floor of the nose–anterior
nasal spine region. Full-thickness cranial bone may lack the
necessary volume (thickness) for premaxillary construction.
The use of a rib graft is likely to be inadequate in both
volume and quality. When the nasal reconstruction follows
within 6 months after grafting and the new (bone-grafted)
pre­maxilla is used as a platform, less resorption is expected.
The use of artificial materials in this region circumvents the
issue of resorption and donor-site morbidity, but the long-
term concern of recipient-site infection and extrusion must
be considered.
Effective facial rehabilitation for the patient with Binder
syndrome must address the nasal deformity. Potential pit­
falls to the nasal reconstruction merit discussion. The open
rhinoplasty technique (i.e., the columella splitting incision)
allows ideal access to the area of the nose that requires
reconstruction. Whenever possible, the surgeon should
avoid other external nasal incisions carried out either for
exposure (e.g., coronal scalp flap, vertical incisions directly
over the nose) or in an attempt to directly lengthen the
columella (e.g., Cronin technique). In the Caucasian
patient, if a graft is placed from the radix to the nasal tip,
a technical error will be made unless the surgeon first
reduces (i.e., burs down) the bony dorsum from the fron­
tonasal suture to the caudal aspects of the nasal bones before
the augmentation procedure. If this is not done, flattening
of the nasofrontal angle occurs, and an unnatural facial
appearance will result. Achieving a flexible (or at least “non-
firm”) reconstructed nasal tip is preferred. A relatively
soft nasal tip with enough strength for tip projection is
best accomplished with the use of a rib cartilage graft. The
lower lateral cartilages are sutured over the top of the graft.
If a full-length dorsal bone graft is required (i.e., from the
radix to the tip), then stable fixation is necessary; this may
be accomplished in a number of ways (e.g., plates and
screws, lag screws, Kirschner wires [K-wires]), depending
on the clinical circumstances. Placing a protective external
of the nasomaxillary complex.14 Over time, it has become
apparent that many patients with Binder syndrome
ultimately have a form of chondrodysplasia punctate,
although genetic heterogeneity exists. Children with the
X-linked recessive for of chondrodysplasia punctate have
molecular alterations in the arylsulfatase E gene. Because of
the X-linked inheritance pattern, females with this form
of chondrodysplasia punctate tend to be much more mildly
affected than males. Molecular testing for alterations in
arylsulfatase E is clinically available and should be
tested for.
In retrospect, the features defined by von Binder were
not altogether typical of those found in most patients who
are currently categorized as having Binder syndrome. It is
also known that frequent ethnic characteristics of both
Asian and African individuals are a sloped backward and
vertically short anterior maxilla, pyriform rims, and a defi­
cient dorsum of the nose. This results in deficiency of alveo­
lar bone to house the maxillary incisors. The anterior nasal
spine is hypoplastic, but it is often present as a small ridge.
A lack of height of the dorsum of the nose (i.e., bone and
cartilage) with increased width is a frequent ethnic charac­
teristic of Asian and African individuals but not a finding
among patients with Binder syndrome. We believe that
many patients who are believed to have Binder syndrome
more accurately represent ethnic variation. This may well
encompass many of the Asian patients described and treated
by Goh and Chen in their review of nasal reconstruction
for Binder syndrome.13
Holmstrom and others support an orthognathic approach
and state that, in a series of Caucasian patients with Binder
syndrome (54% of the study group), a majority “suffered
a class III malocclusion.”18-20 Although an edge-to-edge
incisor relationship may be possible with orthodontics only,
it is generally accomplished with excessive maxillary incisor
proclination. This camouflage approach does not address
the flat midface appearance or the long-term orthodontic
retention needs, and it may lead to periodontal sequelae.
To correct the maxillary incisor inclination, an alveolar
space analysis will confirm the advantage of extracting
maxillary first bicuspids in many of these patients. With
molar anchorage, the orthodontist is able to retract the
anterior teeth into the space created by the bicuspid extrac­
tions, and ideal incisor positioning is accomplished. This
approach will further unmask the maxillary hypoplasia and
allow for an effective horizontal advancement at the Le Fort
I level. When this is combined with a premaxillary region
crafted bone graft reconstruction, maximum improvement
in the perialar and nasal base morphology is also accom­
plished. An orthodontic camouflage approach should only
be carried out with full disclosure to the patient and his or
her family.
The aesthetic advantage of a vertical reduction and an
advancement osseous genioplasty in the majority of indi­
viduals with Binder syndrome likely reflects the longstand­
ing mouth-breathing pattern and its effects on anterior
mandibular growth. In three of the seven patients in the

1179
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
When iliac bone is used at the time of the Le Fort I
osteotomy for augmentation of the premaxilla, addi­
tional graft may be harvested for simultaneous nasal
reconstruction, as described previously. This surgeon
has used this combined single-stage approach only in
unique and specific circumstances, because very few
deformities require upper dorsal reconstruction (see
Fig. 29-1). This approach also raises the level of com­
plexity of the procedure by involving the need for
sophisticated intraoperative airway management.
5. An L-shaped rib cartilage graft reconstruction has
become this author’s preference whenever the Binder
deformity allows. With this procedure, it is not neces­
sary to harvest the L-shaped rib cartilage graft in one
piece. Rather, two struts (dorsal and columella) are
crafted separately and then inset and sutured together
to form the new nasal tip. The columella strut graft
extends from the base of the maxilla to the nasal tip.
It is fixed in place at the base of the maxilla with a
buried K-wire (no. 32 threaded) to prevent slippage
or movement. The dorsal strut graft extends from the
caudal edge of the nasal bones to the new nasal tip,
where it is secured to the columella strut graft with
the use of non-resorbable suture material. Warping of
the cartilage graft can occur, but resorption is not a
problem. A separate K-wire (no. 32 threaded) may be
inserted through the spine of the dorsal graft before
inset to limit the risk of warping. The lower lateral
cartilages are sutured over the top of the grafts, and
the end result is a flexible nasal tip. This rib cartilage
reconstruction option is preferred whenever feasible
(see Chapter 34).
For the individual with Binder syndrome, early recon­
struction of the dysmorphic nose (i.e., between the ages of
5 and 12 years) to improve tip projection and to encourage
self-esteem is technically possible, but the surgeon should
proceed with caution. Disadvantages include the need for
additional nasal procedures at the completion of growth and
after definitive jaw reconstruction. The inherent periopera­
tive risks and expense; the creation of scar tissue within the
nasal soft-tissue envelope; and the need for the child to
avoid normal school-aged physical activities to prevent graft
dislodgement or fracture should also be considered (see Fig.
29-1, A).
Conclusions
The individual with Binder syndrome presents with hypo­
plasia of the anterior maxilla and the caudal aspect of the
cartilaginous nose. No genetic marker yet exists for this
condition, and diagnostic confusion with normal ethnic
variations of premaxillary and nasal anatomy is frequent. A
staged reconstructive approach is generally carried out
during the teenage years, before the patient graduates from
high school. In Caucasian patients, this often includes
orthodontic treatment, orthognathic procedures, anterior
nasal splint over the grafted region limits swelling and assists
with immobilization and overall healing. If dorsal bone
augmentation is carried out, union to the underlying nasal
bones is essential to achieve long-term facial aesthetic and
functional objectives.
Holmstrom reviewed the long-term results of nasal
reconstruction in a series of patients with Binder syndrome
and found overall good results with a variety of techniques.18-20
A review of nasal reconstruction options is listed below. The
option selected for a specific patient is dependent on
the unique presenting dysmorphology, the potential risks,
the patient-specific objectives, and the surgeon’s comfort
level with the particular technique.
1. A cranial graft placed through a coronal scalp incision
offers ideal access to the nasofrontal region for con­
touring and graft stabilization (i.e., plates and screws),
and it provides excellent bone volume and quality.
The lower lateral cartilage is then sutured over the
graft at the tip via a columella incision. This approach
generally requires craniotomy and a full-thickness
graft, depending on the volume of bone required.
Unfortunately, it results in a firm nasal tip that has a
tendency toward a degree of resorption and that is
prone to fracture with trauma. This approach is pre­
ferred only for the occasional patient in whom sig­
nificant reconstruction of the nasofrontal region and
augmentation of the bony dorsum is required (see
Fig. 29-5).
2. A costochondral (bone and cartilage) graft maybe
placed through a columella splitting incision. This
results in a relatively “non-firm” nasal tip with limited
resorption or risk of warping over time. If the rela­
tively thin and malleable rib graft is placed in a can­
tilever fashion (i.e., without a columella strut for
stabilization), there is a tendency for non-union with
the underlying nasal bones and dislocation or fracture
after minimal trauma. If the graft extends from the
radix, it may also flatten the nasofrontal angle in an
unfavorable way. This approach is generally not a first
choice (see Fig. 29-1).
3. If a costochondral graft is selected for dorsal recon­
struction (as described previously), a separate colu­
mella (rib cartilage) strut graft maybe crafted and
then secured with sutures to the dorsal costochondral
graft at the new nasal tip. Temporary stabilization
of the dorsal bone graft component may be accom­
plished with a transcutaneous K-wire. A second
short buried K-wire is placed to stabilize the rib
cartilage (caudal strut) at the base of the maxilla (see
Fig. 29-2).
4. An anterior iliac (corticocancellous) graft offers
another option, but this is only used when extensive
bone volume reconstruction is required. It is placed
as a crafted L-shaped strut that is stabilized at the base
of the premaxilla with titanium plates and screws and
over the mid dorsum with percutaneous K-wires.

1180 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
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maxillary augmentation, and nasal reconstruction. The
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1181
CHAPTER 29  Binder Syndrome: Evaluation and Treatment
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1182

30
Syndromes with Craniosynostosis:
Evaluation and Treatment
JEFFREY C. POSNICK, DMD, MD
• General Considerations
• Mutations in Craniosynostosis and
Craniosynostosis Syndromes
• Complex Craniosynostosis
• Neurologic Aspects of Craniosynostosis
• Effects of Midface Deficiency on the Upper
Airway
• Morphologic Considerations
• Surgical Approach to Craniosynostosis
Sydromes
• Staging of Reconstruction for Crouzon
Syndrome
• Staging of Reconstruction for Apert
Syndrome
• Conclusions
fusion. Craniosynostosis may be of prenatal or perinatal
onset, or it may occur during infancy.14,74 The earlier syn­
ostosis occurs, the more dramatic the effect on subsequent
cranial growth and development; the later synostosis occurs,
the less effect on cranial growth and development.44
Active pediatric neurosurgical services generally see
a characteristic frequency, gender predilection, and
phenotypic
presentation
for
sutural
involve­
ment.6,15,55,125,133,154,161,193,232,238,243,278,283 Sagittal synostosis
occurs most commonly, with a higher incidence among
boys than girls. Patients typically present with a boat-shaped
skull (i.e., scaphocephaly) in combination with midline
ridging in the posterior half of the skull. Metopic synostosis
is currently the second most frequent form of synostosis.
Presentations occur along a spectrum that ranges from
metopic ridging to the presence of a triangular-shaped skull
(i.e., trigonocephaly). It should be noted that metopic
ridging is common during infancy and childhood and that
it is not necessarily associated with a diagnosis of craniosyn­
ostosis. Coronal synostosis occurs next most frequently;
girls slightly predominate or, in some surveys, boys and girls
have equivalent frequency.46 Unicoronal synostosis is
marked by frontal plagiocephaly, whereas bicoronal synos­
tosis is characterized by a short and wide skull (i.e., brachy­
cephaly). Multiple synostoses of various types are less
common than coronal synostosis. Lambdoid synostosis
occurs with least frequency, and it is marked by occipital
plagiocephaly.44
Craniosynostosis is etiologically heterogeneous and
pathogenetically variable. Overall, 8% of cases have a famil­
ial component, and this is generally marked by an autoso­
mal dominant inheritance pattern. Specifically, pedigrees
are familial in 14.4%, 6%, and 5.6% of coronal, sagittal,
and metopic synostoses, respectively.44 Although some pedi­
grees show synostosis of a specific suture, others may show
fusion of different sutures in affected relatives of the same
family. Different chromosomal aberrations have been linked
to craniosynostosis, with variable penetrance. In some con­
ditions the penetrance is high, such as with dup(3q),
del(7p), del(9p), and del(11q). In other cases, penetrance
General Considerations
Craniosynostosis, which is the premature fusion of cranial
sutures, affects approximately 1 in 2500 children. Patients
may present with a wide range of phenotypic and functional
deformities.94 Virchow’s law from 1851 states that the pre­
mature fusion of a cranial vault suture results in growth
arrest perpendicular to the affected suture and compensa­
tory growth parallel to the fused suture.16,164,269 This prin­
ciple is generally applicable to single sutures; multisutural
synostosis—such as the combination of sagittal and lamb­
doid synostosis (coup de sabre) or a cloverleaf skull—can
assume more complex patterns of cranial morphology.43,46,47
In addition to the type of affected suture, the eventual head
shape depends on the timing and order of cranial suture

1183
CHAPTER 30  Syndromes with Craniosynostosis: Evaluation and Treatment
can be very low, such as with dup(5p), del(6)(22q22.2-
q23.1), del(8q), and dup(15q).44
Many syndromes are associated with craniosynostosis,
and well over 100 are known.44 It is important to accurately
diagnose syndromal patients for three reasons.22 First, most
syndromes with craniosynostosis affect not only the cranial
vault but also the cranial base and the midface. Deficiencies
in these skeletal sites, which are variable in degree yet
commonly serious, must be addressed as part of the
staged reconstructive approach. Second, syndromes are
often genetic and familial, thus necessitating proper coun­
seling. Third, depending on the experience of the particular
surgeon, the molecular diagnosis should be made. Often,
the patient may be referred for molecular diagnosis and
counseling to avoid the possibility of litigation.37,43,46,47
Primary forms of craniosynostosis are most common and
include single- and multi-sutural synostosis. Some condi­
tions, however, result in secondary synostosis. These include
hyperthyroidism; rickets; mucopolysaccharidoses, such as
Hurler syndrome and Morquio syndrome; hematologic dis­
orders, such as thalassemia and sickle cell anemia; terato­
gens, such as diphenylhydantoin, retinoids, valproate, and
aminopterin; and certain malformations, such as holopros­
encephaly and microcephaly.44
Mutations in Craniosynostosis and
Craniosynostosis Syndromes
Mutations responsible for craniosynostosis have been iden­
tified in FGFR1, FGFR2, FGFR3, TWIST, MSX2, EFNB1,
RAB23, EFNA4, POR, and ALPL.44 These include Apert
syndrome (two common FGFR2 mutations; Ser252Trp,
Pro253Arg), Crouzon syndrome (more than 30 FGFR2
mutations), Pfeiffer syndrome (more than 30 FGFR2 muta­
tions), are known and at least 6 of them are the same as
those found with Crouzon syndrome; one Pfeiffer syn­
drome (mutation on FGFR1).* In addition, there are some
cases of FGFR2 mutations with isolated coronal synostosis
as well as with some other forms of non-syndromic synos­
tosis. A single known FGFR2 mutation is known for
Jackson-Weiss syndrome.1,41,182 Although this disorder is
distinctly uncommon, affected families are often large, with
variable phenotypic expression. Beare-Stevenson cutis gyrate
syndrome involves one of two possible mutations in the
transmembrane domain of FGFR2.37,42
Muenke syndrome is the most common craniosynostosis
syndrome that is currently known. It involves a single muta­
tion on FGFR3, and it is characterized most commonly by
unilateral coronal synostosis and less commonly by bicoro­
nal synostosis. About 6% of affected patients have macro­
cephaly without synostosis, and cloverleaf skull has been
reported in some instances.37,42 Therefore, all patients with
coronal synostosis should be tested for this very common
FGFR3 mutation; if they are negative, they should be tested
for FGFR2.
Crouzonodermoskeletal syndrome is characterized by
one specific FGFR3 mutation. The name indicates its char­
acteristic features: either a Crouzonoid or cloverleaf skull
appearance; acanthosis nigricans; and a decreased interpe­
diculate distance that is radiographically present but not
clinically significant. Serious decreased interpediculate
distance is a feature of the most common mutation for
achondroplasia, which is only 11 amino acids away from
the FGFR3 mutation for Crouzonodermoskeletal syn­
drome. To call this disorder “Crouzon syndrome with acan­
thosis” is unwarranted, because only one FGFR3 mutation
is responsible for all cases; this is in contrast with Crouzon
syndrome, which involves more than 30 different FGFR2
mutations.38
Rarely, patients with achondroplasia and hypochondro­
plasia (both of which involve mutations on FGFR3) may
also have craniosynostosis (there have been three reported
cases of hypochondroplasia and two reported cases of
achondroplasia). Saethre–Chotzen syndrome has TWIST
mutations inside or outside of the coding region that
result in haploinsufficiency.48 Craniofrontonasal syndrome
is caused by heterozygous loss-of-function mutations in
EFNB1. EFNA4 mutations rarely cause non-syndromal
coronal synostosis. Carpenter syndrome, which is an auto­
somal recessive disorder, is caused by RAB23 mutations.
Boston-type craniosynostosis, which is an autosomal domi­
nant disorder caused by MSX2 mutations, presents with
variable expressivity (coronal synostosis, frontal depression,
or cloverleaf skull). Antley–Bixler syndrome is caused by
POR mutations, and infantile hypophosphatasia is caused
by ALPL mutation.44,184
Complex Craniosynostosis
Background
Complex craniosynostosis, which involves the fusion of
multiple cranial sutures, occurs in about 5% of non-
syndromic cases.47 Two thirds of cases involve two affected
sutures, whereas one third of cases involve more than two
affected sutures.203 As the number of affected sutures
increases, so does the risk of intracranial hypertension and
associated mental deficiency. As demonstrated by Renier
and colleagues, the incidence of intracranial hypertension
rises from 14% to 47% when going from single to several
affected sutures.228,226,227,229,230 In addition, increasing sutural
involvement can be accompanied by worsened phenotypic
severity. In a series reported by Chumas and colleagues,34
59% of cases of sagittal and bilateral lambdoid synostosis
had an acute angulation of the posterior skull, which is
known in French as coup de serpe and means “cut with a
scythe.” In a similar fashion, marked anterior dysmorphism
with frontal bone hypoplasia was found in 23% of cases of
metopic and bilateral coronal synostosis.
*References 5, 42, 45, 81, 82, 95, 115, 116, 131, 143, 159, 160, 165-168,
177, 183, 223, 233, 236, 248, 250, 251

1184 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
syndrome, and patent sutures and synchondroses (except
for coronal synostosis) are present as well. The frequency of
progressive hydrocephalus is much lower than in it is with
Crouzon and Pfeiffer syndromes. Central nervous system
anomalies may include hypoplasia of the corpus callosum,
agenesis of the corpus callosum, agenesis of the septum
pellucidum, cavum septum pellucidum, dorsally displaced
hippocampi and hippocampal gyri, hypoplastic white
matter, and heterotopic gray matter.46,48
Skeletal abnormalities with Apert syndrome include
symmetric syndactyly of the hands and feet; abnormal
shoulders with significant limitation of motion; and abnor­
malities of the elbows, hips, knees, rib cage, and spine.46 It
should be carefully noted that the radiologic interpretation
of the hands, feet, and cervical spine as being affected by
so-called “progressive fusion” is very misleading. In actual­
ity, there is a failure of cartilage segmentation during embry­
onic and fetal life, so these abnormalities are preset (ab
initio). These failures in cartilage segmentation cannot be
readily observed radiographically at birth. When they do
become evident radiographically after birth, they are said to
be a “progressive abnormality,” which they are not. The
same can be said for the cervical vertebrae,46 as 68% of these
patients have cervical abnormalities, particularly involving
C5 and C6. Before considering a surgical procedure, mag­
netic resonance imaging of the brain, radiography of the
cervical spine, and the assessment of cardiovascular (10%
of patients) and genitourinary (9.6% of patients) anomalies
should be carried out.44,46,48
Patients with Apert syndrome also suffer from reduced
nasopharyngeal dimensions and reduced patency of the pos­
terior choanae; these features pose the risks of respiratory
embarrassment, obstructive sleep apnea (OSA), cor pulmo­
nale, and even sudden death. During infancy, patients may
be trialed on a nasopharyngeal airway, but tracheostomy
may be the only definitive treatment. Clinicians must
always maintain a high index of suspicion for OSA and
monitor patients for snoring or an unusual amount of
daytime somnolence. When either of these is apparent,
patients should be referred to a sleep center for proper
diagnosis and treatment. It should be carefully noted that,
although patients with Apert syndrome very commonly
have OSA, the excessive sweating that occurs with this
syndrome is not a sign of OSA but rather a consequence of
the increased number of sweat and sebaceous glands that
accompany the syndrome. Even those patients who have
surgical advancement of the midface in combination with
continuous positive airway pressure (CPAP) will still have
excessive sweating of the head at night and of the hands all
the time. Another consequence of an increased number of
sweat and sebaceous glands is a high frequency of conglo­
bate acne during adolescence, often with extension to the
forearm, the thighs, and the buttocks. The acne vulgaris of
Apert syndrome suggests exquisite end-organ responsive­
ness to steroid hormones.46
Cloverleaf skulls, which represent the extremes of
phenotypic severity, are pathogenetically variable (Fig.
30-1).30,204 Synostosis may involve the coronal, lambdoid,
and metopic sutures, which are marked by bulging of the
cerebrum through an open sagittal suture or, in some cases,
through patent squamosal sutures. Synostosis of the sagittal
and squamosal sutures with cerebral eventration through a
widely patent anterior fontanel may also be observed. A
trilobular skull shape may also occur, with complete synos­
tosis of all cranial sutures in some cases or with widely
patent sutures and no craniosynostosis at birth in other
instances.36,51 In addition to being pathogenetically variable,
cloverleaf skulls are also etiologically heterogeneous. This
condition most commonly occurs with type 2 thanato­
phoric dysplasia (i.e., in about 40% of cases) with a specific
FGFR3 mutation, but stillborn status or an early demise
during very early infancy precludes treatment. Isolated clo­
verleaf skull occurs in about 20% of cases. It also occurs in
about 15% of cases of type 3 Pfeiffer syndrome, and,
although the prognosis is guarded, these patients can be
treated aggressively.45,48
Plagiocephaly is defined as the asymmetric distortion of
the skull. Well-known types include synostotic anterior
plagiocephaly (unilateral coronal synostosis), synostotic
posterior plagiocephaly (unilateral lambdoid synostosis),
deformational anterior plagiocephaly, and deformational
posterior plagiocephaly.146,148,268 Among these types, defor­
mational posterior plagiocephaly is common, whereas uni­
lateral lambdoid synostosis is rare. Deformational posterior
plagiocephaly can be caused by intrauterine factors such as
hypotonia, fetal positioning, and prematurity. This can
produce asymmetric flattening of the occiput that becomes
favored by infants sleeping on their backs, thereby exag­
gerating the plagiocephaly. Deformational posterior plagio­
cephaly has also increased dramatically since the 1992
“Back to Sleep” campaign by the American Academy of
Pediatrics, which calls for supine infant sleeping to reduce
the risk of sudden infant death syndrome.44 Finally, rare
forms of plagiocephaly can be produced by unilateral fron­
tosphenoidal synostosis and unilateral frontozygomatic
synostosis.44
Apert Syndrome
Apert syndrome is characterized by craniosynostosis, mid­
face deficiency, symmetric syndactyly of the hands and
feet, and other abnormalities* (Figs. 30-2 through 30-5).
Most cases are associated with brachycephaly secondary to
bicoronal synostosis. Megalencephaly and increased cranial
height are characteristic of Apert syndrome; affected
children typically display head circumference, length, and
weight that are above the normal 50th percentile. Benign
distortion ventriculomegaly is also a feature of Apert
Text continued on p. 1200
*References 7, 13, 17, 54, 88, 98, 99, 104, 121, 123, 124, 128-130, 132,
142, 147, 152, 186

1185
CHAPTER 30  Syndromes with Craniosynostosis: Evaluation and Treatment
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• Figure 30-1  A child with a severe form of cloverleaf skull anomaly. At 10 months of age, she was referred to this surgeon and under­
went first-stage cranial vault and upper orbital decompression with reshaping. She then required a ventriculoperitoneal shunt for the
management of hydrocephalus. When the patient was 3 12 years old, posterior cranial vault decompression with reshaping to increase
the intracranial volume was performed. When she was 4 12 years old, facial bipartition osteotomies in combination with anterior cranial
vault reshaping and advancement were carried out. After the cranial vault and facial bipartition procedures, the patient’s airway improved,
and it was possible to remove the tracheostomy tube. The cranial vault reshaping expanded the intracranial volume, thereby providing
more space for the brain. The midface advancement improved the eye proptosis as well as the patient’s ability to chew and articulate
speech. As part of the staged reconstruction, she will require orthognathic surgery in combination with orthodontic treatment at the time
of early skeletal maturity. A, Frontal facial and computed tomography (CT) scan views at 10 months of age. B, Intraoperative views at 10
months of age after craniotomy and the removal of the cranial vault and the upper orbits. The “bandeau” is in place (3 cm advancement)
before the reconstruction of the overlying cranial vault. C, Profile views at 2 12 and then at 3 12 years of age with flattened posterior cranial
vault and severe midface deficiency.
Continued

1186 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
E
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D, Illustration of flattened posterior cranial vault with the ventriculoperitoneal shunt in place.
Illustration after posterior cranial vault reshaping is also shown. E, Intraoperative side view with the patient in the
prone position before and after the craniotomy and the reshaping of the posterior cranial vault. Arrows point to
the ventriculoperitoneal shunt, which remains intact and deep to the skull reconstruction.
• Figure 30-1, cont’d

1187
CHAPTER 30  Syndromes with Craniosynostosis: Evaluation and Treatment
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F, Profile view at 3 12 years of
age, before and after posterior cranial vault reconstruc­
tion. G, CT scan views before and just after posterior
cranial vault reshaping. H, Illustration before facial bipar­
tition. The second illustration indicates the planned
reconstruction.
• Figure 30-1, cont’d
H
Continued

1188 SECTION 4  Frequently Seen Malformations with Dentofacial Deformity
K
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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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©Elsevier Inc.
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©Elsevier Inc.
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o nicicicicicicicicici k
I, Oblique facial views
before and after facial bipartition reconstruc­
tion. The tracheostomy has been removed.
J, Facial views before and after facial biparti­
tion reconstruction. K, Profile views before and
after facial bipartition reconstruction.
• Figure 30-1, cont’d