Pediatric Plastic and Reconstructive Surgery

4 Craniofacial Microsomia
Patrick A. Gerety, Albert K. Oh, and Jesse A. Taylor
Sum m ary
Management of Mandibular Hypoplasia in Pediatric Unilateral
Craniofacial Microsomia attempts to succinctly describe the
major principles of the surgical treatment of mandibular asym-
metry in patients with unilateral craniofacial or hemifacial
microsomia. This chapter also delves into some of the impor-
tant controversies regarding the surgical management of man-
dibular hypoplasia, particularly in regards to long-term results.
Keywords: craniofacial or hemifacial microsomia, mandibular
hypoplasia/asymmetry, distraction
osteogenesis, graft,
reconstruction
4.1 Introduction
Hemifacial or craniofacial microsomia (CFM) represents a wide
group of malformations primarily involving structures derived
from the first and second branchial arches. With reported inci-
dences ranging from 1 in 3,000 to 1 in 20,000, this heterogene-
ous disorder of craniofacial morphogenesis represents the
second most common craniofacial anomaly after cleft lip/palate.
Although the etiology of CFM is unclear, two major theories
include vascular disruption of the stapedial artery that supplies
the first and second pharyngeal arches and abnormal migration
of key neural crest cells during embryogenesis. The latter
theory provides a more plausible explanation for the bilateral
craniofacial features and extracraniofacial findings often seen in
expanded-spectrum CFM.
4.2 Diagnosis
With the ongoing advancements in ultrasonography and prena-
tal magnetic resonance imaging (MRI), CFM can be diagnosed
prenatally. However, it is much more common for clinical suspi-
cion to arise postnatally. Abnormalities of the external ear,
facial asymmetry, and facial paralysis are most likely to be iden-
tified by neonatologists and pediatricians.
4.2.1 Classification System
Although several systems exist for classifying CFM, the OMENS-
Plus classification is the most frequently utilized. The OMENS-
Plus classification (Fig. 4.1) succinctly describes and grades
the five major craniofacial abnormalities of CFM, specifically
involving the (1) orbit, (2) mandible, (3) ear, (4) nerve (facial),
and (5) soft tissue deficit. Scores range from 0 (normal) to 3
(most severe). The plus designation applies to extracraniofacial
anomalies, with the most common associated abnormalities
involving the central nervous, skeletal, and cardiac systems.
Patients with more severe CFM (i.e., those with higher OMENS
score) have significantly greater risk of having noncraniofacial
anomalies, lending further support to the theory of neural crest
involvement in the etiopathogenesis of CFM. The clinical
features and their severity will be discussed as follows in the
order of the OMENS-Plus classification system.
4.2.2 Orbit
The orbit is a ected in a minority (20–30%) of patients with
CFM. Orbital manifestations of CFM include a reduction in the
dimensions (O1), inferior positioning resulting in vertical orbi-
tal dystopia (O2), and, in severe cases, a severely constricted
orbit (O3) featuring microphthalmia and hypoplastic lids.
Patients with microphthalmia often do not have vision in the
a ected eye ( Fig. 4.2).
4.2.3 Mandible
Mandibular hypoplasia is the hallmark of CFM and a ects 90 to
100% of patients. The mandibular component of CFM has
received the most attention in the literature. This is related to
surgical innovations (distraction osteogenesis [DO]) and the
degree of facial normalization that mandibular surgery can pro-
duce. The morphology of the CFM mandible is quite variable
(Fig. 4.3), and there has been a vigorous debate about the best
way to classify the patients. Although there are several classifi-
cation schemes, the Kaban-modified Pruzansky’s system is the
most commonly used: type I is a small mandible with normal
morphology; type IIa is a mandible with a ramus that is abnor-
mal in size and shape but with a normal temporomandibular
joint (TMJ); and type IIb is a mandible with an abnormal ramus
and TMJ. Type III deformity is defined by an absent ramus, con-
dyle, and TMJ.
In general, the CFM mandible is smaller than the contrala-
teral normal mandible. Much of this size di erence is seen in
the condyle–ramus portion of the mandible. In mild cases, the
e ect on the ramus is largely vertical shortening, resulting in
decreased posterior facial height and occlusal canting upward
on the a ected side. In severe cases, specific portions of the
mandible, including the condyle, the ramus, the glenoid fossa,
and the TMJ, may be absent or unrecognizably distorted. An
important observation is that the a ected condyle and ramus
are shifted medially, contributing to decrease in transverse
facial width.
Because moderately and severely a ected mandibles are so
morphologically a ected, classification systems have been
found to be unreliable, even among expert raters. Hypoplasia of
the maxilla was long thought to be a part of CFM, but vigorous
examination has not shown this to be true. The maxilla appears
to be normal in volume but malpositioned secondary to the
abnormal CFM mandible.
4.2.4 Ear
As with all other manifestations of CFM, ear anomalies range
from mild hypoplasia to complete anotia. From the OMENS clas-
sification, E1 represents mild hypoplasia, often with cupping of
the a ected ear; E2 represents absent auditory canal and
Craniofacial Disorders
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Fig. 4.1 OMENS classification. The severity of the clinical features of craniofacial microsomia is described in this classification. The specifics of each
category can be found in their respective text in this chapter.
Craniofacial Microsomia
35

hypoplastic abnormal appearing ear remnant, and E3 repre-
sents severe microtia with all but a small remnant missing (e.g.,
lobular type). Preauricular remnants are also commonly found
anterior to the tragus. Studies demonstrate that the ear is
a ected in 80 to 90% of patients, with as many as 50% of
patients having microtia (E3). Hearing impairment is common
on the a ected side in CFM, occurring in approximately 75% of
patients. Nearly all of those patients have conductive hearing
loss; however, about 10% of all patients with CFM have sensori-
neural hearing loss as well.
4.2.5 Nerves
Anomalies of the nervous system, including aberrant brain
anatomy, may been seen in CFM. However, the most common
finding is partial facial nerve (CN VII) palsy. Facial nerve
involvement is found in approximately 40% of patients with
CFM. This palsy is more likely to a ect the lower face (buccal,
marginal mandibular, and cervical branches) than the upper
face (zygomatic and frontal branches). A small percentage of
patients with CFM have complete facial palsy. The OMENS clas-
sification describes facial palsy as either upper (N1), lower (N2),
or total (N3).
Clinical manifestations of facial paralysis include inability to
close the eyelid, lack of animation of the forehead, lack of active/
symmetric smile, drooling, and asymmetry of lip depressors.
4.2.6 Soft Tissue
All layers of the soft tissue on the a ected side of a patient with
CFM may be deficient. This includes skin, subcutaneous fat, and
muscles of animation and mastication. In moderate and severe
cases, this deficiency immediately draws the attention of an
observer to the asymmetry of the face. The most notable areas
of deficiency are in the temporal fossa, in the malar/zygomatic
region, and along the lower mandibular border. The severely
a ected CFM m andible contributes to the appearance of
soft tissue deficiency, because the condyle and ram us are
shifted m edially, robbing the face of transverse w idth. The
OMENS classification describes this soft deficiency as m ild
(S1), m oderate (S2), or severe (S3). Macrostom ia, or clefting
at the oral com m issure, also known as a Tessier 7 cleft,
occurs in approxim ately 25% of patients w ith CFM. The
orbicularis oris is discontinuous and can contribute to poor
oral competence and abnorm al appearance in both repose
and anim ation.
4.2.7 Noncraniofacial Manifestations
Noncraniofacial anomalies were long thought to identify a pa-
tient with “syndromic CFM,” usually Goldenhar’s syndrome.
The current understanding is that the error in neural crest cell
migration responsible for facial manifestation of CFM is also the
cause of noncraniofacial problems. The most common extracra-
niofacial anomalies occur in the heart (25% of patients with
CFM), kidneys (10% of patients with CFM), skeleton (40% of
patients with CFM), and central nervous system (CNS). Com-
mon cardiac defects include atrial and ventricular septal
defects. Skeletal anomalies are most often scoliosis and overt
vertebral abnormalities. The most common CNS problem is
hydrocephalus. One of the pathognomonic findings is epibulbar
dermoids of the eyelid.
4.2.8 Di erential Diagnosis
A number of di erent diagnoses produce facial asymmetry that
could resemble CFM to the untrained eye. Often, these other
diagnoses can be excluded based on their timing of onset and
specific phenotype. Examples of these diagnoses include Parry–
Romberg disease, TMJ ankylosis, and radiation-induced tissue
atrophy. Severe Tessier 7 can mimic CFM as well.
Facial asymmetry may be caused by hypoplasia or hyperpla-
sia. Hemifacial hypertrophy and an infiltrating lipomatosis are
rare conditions that create facial asymmetry. Condylar hypopla-
sia and hyperplasia should also be considered.
Treacher Collins syndrome (TCS) may be confused for
bilateral CFM. Treacher Collins syndrom e is a heritable
(autosom al dom inant) syndrome characterized by panfacial
bone hypoplasia, bilateral Tessier clefts, and severe antimon-
goloid palpebral slant, culm inating in a very characteristic
phenotype.
One recently identified diagnosis, condylar–coronoid col-
lapse, is believed to mimic CFM in producing mandibular con-
formation similar to mild to moderate CFM. It is noted to have a
deep sigmoid notch, but the patients demonstrate no other
OMENS-related features of CFM.
Fig. 4.2 Orbital deformity in craniofacial microsomia—microphthalmia.
This figure demonstrates the severity of the deformity that may occur
in the orbit. This patient had been previously treated for craniosynos-
tosis.
Craniofacial Disorders
36

Fig. 4.3 Spectrum of mandibular deformities in craniofacial microsomia on CT scan. (a) CT demonstrates a smaller right mandibular ramus with an
occlusal cant. The effect on the mandible is relatively mild. (b) CT demonstrates highly abnormal left hemimandible with absent ramus, condyle, and
coronoid.
Craniofacial Microsomia
37

4.2.9 Natural History
The natural history of CFM is poorly understood. One funda-
mental question is whether the CFM deformity will worsen as a
child ages. Furthermore, will interventions such as mandibular
reconstruction mitigate or exacerbate age-related change? This
has been studied most intensively pertaining to the mandible,
which can be measured radiographically. Through a number of
di erent methods, the a ected mandible has been shown to
grow but at a rate less than the rate of growth of the una ected
side. Given this, the phenotype of a growing patient is likely to
worsen slightly as he or she grows (i.e., occlusal cant and facial
soft tissue asymmetry).
4.2.10 Evaluation
Multidisciplinary craniofacial team care is critical in patients
with CFM. This ensures both that the patients will be treated in
an institution with the expertise and experience to handle such
a complex constellation of abnormalities and that it will be
done in a coordinated way. The team should include a geneti-
cist, audiologist, speech pathologist, otolaryngologist, ortho-
dontist, ophthalmologist, and craniofacial plastic surgeon.
4.2.11 Workup
Patients with CFM often receive a comprehensive workup.
Extracraniofacial anomalies may necessitate a cardiac, skeletal,
renal, and cerebral workup with the appropriate imaging
modality and specialist consultation. In patients with significant
mandibular deformity, visible retrognathia, or any observed
breathing di culty, a formal sleep study (polysomnogram)
should be obtained to evaluate for obstructive sleep apnea. A
formal endoscopic examination of the upper and lower airways
may also be valuable if concerns arise about airway obstruction.
Evaluation of cranial anatomy is most often done using three-
dimensional computed tomographic (3D CT) scan, which allows
detailed examination of the entire craniofacial skeleton.
4.3 Operative Treatment
Surgical management of patients with CFM may begin immedi-
ately after birth and is likely to continue until skeletal maturity.
Indications for, and timing of, surgery are not absolute, and the
decision for reconstruction must be tailored to each individual’s
needs and desires. Fig. 4.4 demonstrates the treatment algo-
rithm of the Children’s Hospital of Philadelphia for patients
with CFM.
4.3.1 Algorithm
Airway and nutrition are the top priorities when a new baby
with CFM is born. Mild cases of CFM may go unnoticed in
the newborn nursery, whereas severe cases may necessitate
emergent intubation for airway compromise. Neonatal manage-
ment of the impaired CFM airway may include endoscopic air-
way examination to rule out other anomalies such as choanal
atresia and tracheomalacia. Endoscopy may also reveal or rule
out tongue-based upper airway obstruction (TBUAO). In severe
cases of bilateral CFM, it may be necessary to place a tracheos-
tomy, especially if there is not enough bone stock for considera-
tion of mandibular DO (MDO). In cases of TBUAO with su cient
bone stock, MDO may provide an alternative to tracheostomy. It
is also important in patients with CFM to ensure proper nutri-
tion—this may include nasoenteric or surgically placed gastro-
stomy tubes. The algorithm for the treatment of all anomalies
of CFM throughout the life of a patient is complex and contro-
versial. Fig. 4.4 attempts to condense this treatment as a time-
line. It is worth noting that many steps overlap, and this is an
important consideration for the psychosocial well-being of
patients with CFM.
Fig. 4.4 Timing of treatment in craniofacial microsomia (CFM). This flow sheet represents the age at which the various CFM abnormalities are treated.
DO, distraction osteogenesis; DAO, depressor anguli oris.
Craniofacial Disorders
38

4.3.2 Macrostomia Repair/Commissuro-
plasty
Much like cleft lip repair, repair of the clefted oral commissure
(macrostomia) can be performed in early infancy. This repair
involves repositioning the commissure to an anatomic position
that mirrors the contralateral side. This position is typically
identified by observing the transition from cleft to noncleft
tissue as well as by measuring commissure distances on the
una ected side (in unilateral cases). The principles of macro-
stomia repair are (1) repair of the clefted orbicularis oris
muscle, (2) repositioning of the commissure to an anatomic
and symmetric position, and (3) cutaneous scar rearrange-
ment (i.e., Z-plasty) to avoid scar contracture and lateral drift
of the commissure.
4.3.3 Orbital Reconstruction
Periorbital reconstruction includes the correction of colobomas
as well as extraocular muscle imbalance (amblyopia) and is
often done in consultation with an ophthalmologist. Correction
of the orbit itself for vertical dystopia or severe constriction is
much less common. Discrepancies in vertical height, if signifi-
cant, can be corrected via cranial bone graft or alloplastic
implant. The severely constricted orbit with microphthalmia
may be treated with an orbital expander to allow for lid expan-
sion and adequate space for a prosthesis. In cases where these
techniques fail, it may be necessary to surgically expand the
orbit, based on its anatomy—this can include zygomatic and/or
supraorbital repositioning.
4.3.4 Mandible Reconstruction
The techniques for reconstruction of the CFM mandible can
be divided into the following groups: DO, importation of
additional bone (graft and flap), correction of TMJ ankylosis,
and final conventional orthognathic surgery at maturity. The
goals of treatment are to level the occlusal plane and create
bony mandibular symmetry. Distraction osteogenesis of the
hypoplastic mandible is currently the most popular form of
reconstruction, particularly during the period of mixed denti-
tion. It relies on the principles popularized by Ilizarov for lower
extremity lengthening after osteotomy and gradual distraction
of the bony segments, with concomitant bone formation in the
resultant gap.
Costochondral grafting is the most popular technique for TMJ
reconstruction in CFM. Although some reports have highlighted
the unpredictability of costochondral grafts, others have docu-
mented reliable symmetric growth in the majority of pediatric
patients requiring TMJ reconstruction for CFM. Free tissue
transfer in the form of a free fibula may be required when con-
dyle/ramus and body must be reconstructed. In general, less
severely a ected patients can be treated at a later age and with
fewer surgical stages. The Kaban-modified Pruzansky’s classifi-
cation provides a conceptual framework for understanding the
timing and type of treatment.
4.3.5 Treatment by Subtype
Patients with type I deformity are often successfully treated
with orthodontia or single-stage orthognathic surgery at skele-
tal maturity. Distraction osteogenesis in early permanent denti-
tion may be o ered when a significant dental cant exists, but
this is an exception rather than a rule. The decision to avoid or
delay mandibular procedures until skeletal maturity is often
based on the degree of psychosocial distress that is experienced
by these patients. In patients with minimal deformity, anxiety
and stress associated with facial asymmetry can usually be miti-
gated or temporized without major mandibular procedures
(Fig. 4.5).
Fig. 4.5 Vertical mandibular distraction for vertical mandible. (a) 3D CT scan demonstrating small left mandible with occlusal cant and vertically
shortened ipsilateral maxilla. (b) Distractor in place but before distraction. (c) Full distraction of 20 mm with orthodontic closure of open bite during
distraction activation phase.
Craniofacial Microsomia
39

Distraction osteogenesis with or without rib graft reconstruc-
tion is the most common treatment modality for type II mandib-
ular hypoplasia. Unlike type I mandibular hypoplasia, the facial
asymmetry in type II patients is often significant enough to
result in functional problems with occlusion as well as in anxiety
and psychosocial distress. Surgical intervention can provide both
functional and aesthetic improvements, at least in the short-
term follow-up. Type IIa mandibular hypoplasia can often be
addressed with mandibular DO alone, whereas the abnormal
TMJ in type IIb mandibular hypoplasia may necessitate costo-
chondral grafting, with or without subsequent distraction.
Similar to type IIb mandibular hypoplasia, type III deformity
may benefit from surgical intervention during mixed dentition,
thereby mitigating the functional and aesthetic concerns associ-
ated with such severe deformities, at least during the formative
childhood and early adolescent years. Costochondral grafting
with or without mandibular DO is the most common form of
treatment. As stated previously, growth of the costochondral
graft may be unpredictable, with some reports describing lack
of grow th, whereas other reports have documented over-
growth. Several centers have reported satisfactory results with
osteocutaneous free tissue transfer to treat severe type IIB and
III mandibles, as well.
It is critical to recognize that all patients with CFM may bene-
fit from finishing conventional orthognathic surgery at skeletal
maturity. This will be technically more challenging because of
the magnitude of the deformity, the presence of scar, and the
anatomic abnormality of the reconstructed mandible. The use
of computer-based presurgical planning can be invaluable in
these situations ( Fig. 4.6 and Fig. 4.7).
4.3.6 Ear Reconstruction
Ear reconstruction must compete with all other reconstructive
needs in patients with CFM. The timing of ear reconstruction is
typically after jaw correction and before hearing restoration
(auditory canaloplasty or bone-anchored hearing aid place-
ment). Ear reconstruction would ideally be performed at a
young age, before a child’s peers have developed cruel tenden-
cies and before significant psychological harm has been done.
The challenge of early ear reconstruction is that the template
for this reconstruction, namely the contralateral ear, has not yet
reached adult size and the building material for a new ear,
namely rib cartilage, is not large or firm enough to create an
anatomic ear. Because of these competing interests, rib carti-
lage–based ear reconstruction is often done at around 8 to 10
years of age ( Fig. 4.7). Many experienced surgeons have noted
disappointment and unpredictability of carved rib cartilage ear
reconstructions. In addition, the donor site can be quite
painful and morbid. An alternative to this is a synthetic ear
construct (porous polyethylene). These ear implants can be
performed earlier in life at around 5 to 6 years of age. Use of a
synthetic ear reconstruction requires coverage with a tempor-
oparietal fascial flap to cover the construct in its entirety. This
flap is then covered with full-thickness skin grafts. There is
now high-quality long-term evidence that these synthetic ears
are durable.
Additional alternative include prosthetic ears, which are
attached with either adhesive or bone anchored magnets.
Insurance often does not cover prosthetic reconstruction, which
can cost in excess of $8,000 and have a limited life span.
Fig. 4.6 Computer-aided surgical planning of final orthognathic surgery. This patient with craniofacial microsomia (CFM) underwent mandibular
distraction osteogenesis once in childhood and presents at skeletal maturity for correction of recurrent occlusal cant and facial asymmetry. Final
orthognathic surgery in CFM necessitates complex, large-magnitude movements. 3D computer planning can make this process much more efficient.
Of note, in this case, the patient had previously had a genioplasty to mask asymmetry, and the operated/abnormal CFM mandible may require rigid
fixation, as seen here.
Craniofacial Disorders
40

4.3.7 Facial Reanimation
Reanimation of the paralyzed face is a technically demanding
field. The patient and the surgeon must strike a balance
between many important factors, including realistic expecta-
tions, volitional versus natural animation, one stage versus mul-
tiple stages, and no donor site versus multiple donor sites. Early
in life, critical attention must be paid to corneal protection. If
there is concern for lagophthalmos, the inability to close the
eye completely, then a regimen of frequent lubrication must be
started. Temporary tarsorrhaphy may also be performed. In
general, consistent lid closure is obtained with the insertion of
a gold weight into the upper eyelid. However, in neonates,
infants, and small children, the eye must be closely monitored
for exposure keratitis.
Another goal of facial reanimation is to improve social interac-
tions and acceptance by creating an animated smile. In children,
this can be accomplished by means of mimicking a smile when a
nerve or muscle of mastication is used. Alternatively, this can be
accomplished spontaneously by powering animation from the
contralateral functioning facial nerve. The most commonly used
strategy is a two-stage facial reanimation, combining a sural
nerve graft with neurotized free gracilis transfer to allow a spon-
taneous and coordinated smile. Similarly, the temporalis muscle
can be transferred from its insertion on the coronoid process of
the mandible to the modiolus (the Labbe’s procedure).
4.3.8 Soft Tissue Augmentation
Patients with CFM are often deficient of subcutaneous facial
volume. After the advent of microsurgical tissue transfer (free
flaps), the most common choice for augmentation was the free
parascapular flap (Fig. 4.8). This flap was tailored to the
morphology of the deficiency and placed via a preauricular
incision with anastomosis to the facial vessels. This procedure
would often be a finishing procedure, as any surgical treatment
for mandibular asymmetry, facial palsy, and microtia should
be performed first. In the age of microfat injection, with
Coleman’s technique, a new paradigm has arisen. In mature
patients with severe soft tissue volume deficiency, there is still
a role of one-stage free tissue transfer. However, fat grafting
now o ers the opportunity to slowly build facial volume, with
minimal morbidity and downtime ( Fig. 4.7). In fact, facial fat
grafting can be done at the time of nearly all other corrective
procedures for CFM. Fat is harvested where available (abdo-
men, inner thigh, and buttocks) and tailored to the deficiency.
On average, 10 to 25 mL can be placed, understanding that, at
most, two thirds will be revascularized and retained. Fat graft-
ing typically requires three to five or more stages ( Fig. 4.7
and Fig. 4.8).
Fig. 4.7 Female with right craniofacial microsomia who has undergone one round of unilateral mandibular distraction in childhood, multiple rounds of
fat grafting, rib-based ear reconstruction at an outside institution, and final two-jaw orthognathic surgery at skeletal maturity. (a,b) 9 years:
Predistraction with evident occlusal cant and facial asymmetry. (c,d) 13 years: Preorthognathic surgery. (e,f) 17 years: Post-Le Fort I, bilateral sagittal
split osteotomy, and advancement genioplasty.
Craniofacial Microsomia
41

4.4 Complications
4.4.1 Mandible Reconstruction
Complications related to mandible reconstruction include tooth
bud injury, lip numbness, partial facial palsy, and malocclusion.
Hardware can fail. Free flaps and rib grafts may fail via resorp-
tion, nonunion, or fracture. If present, the TMJ may become
ankylosed or a condylar reconstruction may fuse to the tempo-
ral bone.
Many complications from mandible reconstruction are spe-
cific to the modality that is used. A critical consideration is to
anticipate that procedures performed in early childhood will
likely be insu cient for the entirety of the child’s growth
period (i.e., skeletal maturity). With DO, early postoperative
complications include wound infection/abscess and device
failure. Technical complications include improper vector of dis-
traction and partial facial palsy, when using a submandibular
approach. Other important late complications include bony
nonunion and relapse. Distraction of the mandible unilaterally
can result in persistent malocclusion (unilateral open bite)—in
children younger than 10 years, maxillary down growth is typi-
cally obtainable with orthodontics after the maxilla has been
o oaded.
Fig. 4.8 A male patient with severe right craniofacial microsomia and a grade III hemimandible. (a,b) Patient at the age of 7 years before any surgical
intervention. (c,d) Patient at the age of 20 years after undergoing rib grafting, right free parascapular flap, formal orthognathic surgery with
genioplasty, and multiple rounds of fat grafting.
Craniofacial Disorders
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Complications related to large reconstruction depend on
technique. Free bone flap transfer can be complicated by flap
loss. A long-term complication from free fibula use can be lack
of growth, which leads to future asymmetry. Another impor-
tant complication can be ankylosis of the proximal condylar
segment to the temporal bone; this necessitates a future TMJ
release. Donor-site complications can occur at both free fibula
harvest and a costal bone harvest.
4.4.2 Ear Reconstruction
In the short term, extrusion, infection, and exposure of either
rib or synthetic ear constructs are the most concerning compli-
cations. Superficial wound infections can be treated with
antibiotics, whereas deep space infections require operative
debridement. Exposure of the ear construct is treated with
operative washout; debridement of necrotic, infected tissue;
and flap closure. Small extrusions of synthetic ear constructs
have successfully been treated in this way—soft tissue coverage
is often provided by an occipital fascial flap.
Long-term complications are largely related to aesthetics.
Microtia reconstruction with rib cartilage is often accompanied
by poor definition of the complex 3D details. This is related to
scar formation. Synthetic ear constructs are thought to have
enhanced and improving definition over time. However, there
have been reports of late synthetic ear extrusion necessitating
removal. The other important aesthetic complications to note
are malposition and size mismatch.
4.4.3 Facial Reanimation
Complications of facial reanimation surgery include loss of
muscle flaps, injury to other neurovascular structures during
facial dissection, and dehiscence of inset muscle. It is a multi-
staged operation, with complete failure as a possibility at any
stage. In the end, it may produce underwhelming results, with
the lack of a symmetric, spontaneous smile. In addition,
although poor animation of the smile is not a complication per
se, it may be seen as such by patients and parents. This high-
lights the importance of preoperative counseling and creation
of realistic expectations.
4.4.4 Soft Tissue Augmentation
For free parascapular flap reconstruction ( Fig. 4.8), complete
or partial flap loss is a known complication that occurs in
approximately 1 to 5% of patients in high-quality series. Partial
flap loss can present as a loss in volume as well as hardened,
calcified areas of fat necrosis or oil cysts. Coleman’s fat grafting
should retain approximately two thirds of the delivered volume
in the long term. These grafts too can be complicated by oil cyst
formation and poor take with little volume retention. Donor-
site complications can occur in both flaps and fat graft harvest.
4.5 Conclusion
Craniofacial microsomia represents a significant reconstructive
challenge for plastic craniofacial surgeons. Each of the OMENS
categories can represent a significant surgical undertaking, and
taken as a whole, it represents many surgeries throughout the
life of a patient with CFM.
The authors would like to acknowledge the contributions of
Scott P. Bartlett, MD, to this chapter.
4.6 Key Points
Craniofacial microsomia represents an embryologic error in
neural crest cell migration, a ecting the first and second bran-
chial arches primarily.
● Its clinical manifestations are succinctly organized in the
OMENS-Plus classification system—orbit, mandible, ear,
(facial) nerve, and facial soft tissue hypoplasia, plus assorted
noncraniofacial anomalies.
● The spectrum of presentation is highly variable, from mild
facial asymmetry to severe presentations with microtia,
hypoplastic mandible, obvious facial asymmetry, and facial
paralysis.
● Surgical treatment is customized to the severity of the
deformity and to the anatomic region—multiple stages,
relapse, and surgical treatment from early childhood to skele-
tal maturity are the rule.
● Distraction osteogenesis for the mandible and particulate fat
grafting for soft tissue deficiency represent paradigm shifts in
the management of these patients.
Suggested Readings
Tuin AJ, Tahiri Y, Paine KM, Paliga JT, Taylor JA, Bartlett SP. Clarifying the rela-
tionships among the di erent features of the OMENS+classification in cra-
niofacial microsomia. Plast Reconstr Surg. 2015; 135(1):149e–156e
Horgan JE, Padwa BL, LaBrie RA, Mulliken JB. OMENS-Plus: analysis of cranio-
facial and extracraniofacial anomalies in hemifacial microsomia. Cleft Palate
Craniofac J. 1995; 32(5):405–412
Shetye PR, Grayson BH, Mackool RJ, McCarthy JG. Long-term stability and
growth following unilateral mandibular distraction in growing children with
craniofacial microsomia. Plast Reconstr Surg. 2006; 118(4):985–995
Tanna N, Wan DC, Kawamoto HK, Bradley JP. Craniofacial microsomia soft-tis-
sue reconstruction comparison: inframammary extended circumflex scapu-
lar flap versus serial fat grafting. Plast Reconstr Surg. 2011; 127(2):802–811
Nagy K, Kuijpers-Jagtman AM, Mommaerts MY. No evidence for long-term
e ectiveness of early osteodistraction in hemifacial microsomia. Plast
Reconstr Surg. 2009; 124(6):2061–2071
Carvalho GJ, Song CS, Vargervik K, Lalwani AK. Auditory and facial nerve dys-
function in patients with hemifacial microsomia. Arch Otolaryngol Head Neck
Surg. 1999; 125(2):209–212
Polley JW, Figueroa AA, Liou EJ, Cohen M. Longitudinal analysis of mandibular
asymmetry in hemifacial microsomia. Plast Reconstr Surg. 1997; 99(2):328–
339
Bauder AR, Mitchell BT, Swanson J, Taylor JA, Bartlett SP. Long-Term Growth
of Costochondral Rib Grafts in Mandibular Reconstruction for Craniofacial
Microsomia. Plast Reconstr Surg. 2015; 136(4) Suppl:44–45
Craniofacial Microsomia
43

5 Orbital Hypertelorism
Robin Yang, Srinivas M. Susarla, and Anand Kumar
Sum m ary
Hypertelorism remains a clinical finding associated with a vari-
ety of congenital conditions. The severity of hypertelorism can
be based on the interdacryon distance and can be used in con-
junction with the shape of the frontal-orbital region to better
characterize the orbital dysmorphology. The correction of
hypertelorism is not a life saving procedure and is mainly an
aesthetic procedure when intracranial pressure is normal. Thus,
a carefull risk benefit analysis will be required for each case
with the treatment plan tailored to each specific deformity and
patient related variable. The appropriate choice depends on the
relationship between the orbits and the surrounding craniofa-
cial structures:
If the maxillary arch is narrow and the corresponding denti-
tion shows apergnathia with the incisors more cranial than the
molars, a bipartition is the operation of choice to improve the
maxillary arch and the orbits in the same procedure.
If a unidirectional repositioning is needed in less severe
(IOD <35 mm) cases, a box osteotomy is satisfactory. If the
orbits are asymmetric with laterally and downwardly oblique
positioning, a bipartition is preferred.
Bipartition is the procedure of choice in the most severe
cases.
If the nasal structures are narrow, a bipartition and medial-
ization of the upper face can improve the airways by enlarge-
ment of the lower face.
An encephalocele associated with a midline cleft can easily be
accessed by a bipartition or orbital box procedure. The amount
of periobital and midface well can be quite significant. Without
tracheostomy, we prefer 72 hours of post surgical intubation
and sedation. This allows maximal swelling to occur and begin
resolution and ensures adequate hemostasis prior to extuba-
tion. Early extubation has led to serious mal-outcomes in our
experience and thus our abandonment of this practice. When
the patient meets clinical parameters for extubation, it should
be performed within 5 days of surgery to assist with patient
mobilization and enteral feeding. Blindness, diplopia, and anos-
mia are uncommon complications, but should be discussed
with the patients.
Keywords: telorbitism, midline cleft defect, Tessier 0/14 cleft,
horizontal dystopia
5.1 Introduction
Orbital dystopia can be defined as the abnormal displacement
of the orbit and its contents in relation to the adjacent craniofa-
cial skeleton or more specifically in the vertical and/or horizon-
tal planes. Orbital dystopia in the horizontal plane has been
termed orbital hypertelorism. Hypertelorism is a symptom or
associated finding that can be accompanied by a multitude of
craniofacial conditions that are not genetically or epigenetically
related. Tessier had classically stressed the fact that hypertelor-
ism is the displacement of the orbital cones in their entirety,
manifested by expansion of the central ethmoid sinuses and
widening of the sphenoid wing angle to more than 110 degrees.
The severity of hypertelorism can be based on the interdacryon
distance and can be used in conjunction with the shape of the
frontal–orbital region to better characterize the orbital dysmor-
phology. Patients presenting with orbital hypertelorism will
require multidisciplinary specialty evaluation and coordinated
care. The specialties include but are not limited to ophthalmol-
ogy, oculoplastic surgery, psychology, social work, pediatrics,
neurosurgery, pediatric dentistry, craniofacial orthodontics,
and craniofacial surgery.
5.2 Diagnosis
Orbital hypertelorism is the physical manifestation of excessive
and widened ethmoidal sinuses. This orbital anomaly is not
pathognomonic of any particular condition but can present in a
variety of conditions, including craniofacial clefts, craniofacial
dysplasia, and facial craniosynostosis syndromes. Although
the term hypertelorism is frequently misused to describe an
increased intercanthal distance following trauma or tumors,
this process does not cause a change in position of the lateral
wall of the orbit or sphenoid wing angle. Hence, most causes of
traumatic increase in intercanthal distance are properly termed
telecanthus.
The distance between the two medial canthi is known as the
intercanthal distance. The distance between the two lateral canthi
is known as the outer canthal distance. Interpupillary distance is
the space between the two midpupillary points in forward gaze
(Fig. 5.1). All three distances (interpupillary distance, intercan-
thal distance, and outer canthal distance) are increased in patients
with hypertelorism. In patients who su er from a trauma (naso-
orbito-ethmoid fractures), there is usually an increase only in the
intercanthal distance, resulting in telecanthus.
In the preoperative setting, a computed tomographic (CT)
scan is recommended as the first-line diagnostic tool. This can
detect any previous or ongoing congenital brain malformations.
Any malformations identified should be treated by a neurosur-
geon in a collaborative and, often, staged reconstructive strat-
egy to optimize outcome. A high-resolution CT scan with thin
1-mm slices will also aid in accurate three-dimensional (3D)
reconstruction by limiting the e ects of volume averaging of
the craniofacial skeleton. In many cases, it can also reveal where
certain vital structures are located (e.g., maxillary tooth buds if
a Le Fort osteotomy is planned in addition to the orbital repair).
Patients with orbital dystopia may present with a variety of
anatomical di erences in the anterior cranial base. The ethmoid
sinuses, nasal cavity, and sphenoid wings are usually abnor-
mally lengthened. The crista galli and cribriform plates are usu-
ally displaced inferiorly, especially in patients with facial clefts.
These anatomical abnormalities can cause defects in the ante-
rior cranial base. Appropriate preoperative planning can avoid
surgical complications and subcranial strategies such as the Le
Fort III osteotomy are the best surgical options. Virtual surgical
planning (VSP) has become the de facto standard of care
Craniofacial Disorders
44

regarding osteotomy design and planning. The cost of VSP has
largely been absorbed into the cost of the craniofacial fixation
system or distraction system, thus facilitating more widespread
adoption. Similar in process to VSP for orthognathic surgery, a
more commonly performed procedure, VSP for orbital deform-
ities has added in accurate measurement of the interdacryon
distance and thus measurement of the central segment of bone
required for optimal correction of the orbits ( Fig. 5.1). We
prefer to use an interdacryon distance of 18 mm as the final dis-
tance and split the di erence equally in symmetric cases or one
third to two thirds in asymmetric cases, commonly seen in the
X-linked cranial frontal nasal dysplasia syndrome.
It is also important to have the patient evaluated by an ortho-
dontist who is preferably trained for craniofacial deformities.
Many craniofacial teams have appropriately trained orthodont-
ists who are comfortable with the management of the di cult
occlusion that may present in a patient with hypertelorism as a
sequel of his or her craniofacial deformity. Frequently, abnormal
position of the orbital cones can directly impact the maxilla.
Apertognathia is often seen in these patients, because the
smaller ethmoid bones limit the normal caudal rotation of the
maxillary ridge in development. In addition, a constricted max-
illa with a high arched palate can often be encountered. We
prefer the use of the facial bipartition to address the constricted
maxillary arch when both orbital constriction and maxillary
expansion are needed. In contrast, the orbital box osteotomy is
more optimal when only orbital expansion needs correction.
Facial advancement and the correction of sleep apnea along
with orbital pathology can be accomplished only with the facial
bipartition, thus making this operation our preferred and most
versatile corrective procedure.
5.2.1 Nonoperative Treatment
The correction of hypertelorism is not a life-saving procedure
and is mainly an aesthetic procedure when intracranial pres-
sure is normal. Thus, a careful risk–benefit analysis will be
required for each case, with the treatment plan tailored to each
specific deformity and patient-related variable. Patients with
severe noncorrected congenital heart disease or severe devel-
opmental delay who are noncommunicative or vegetative are
not considered candidates for surgery because of the unfavora-
ble risk-to-benefit ratio.
5.2.2 Operative Treatment
Corrective surgical procedure for hypertelorism is primarily indi-
cated when the deformity is thought to cause significant facial
distortion, resulting in significant poor self-esteem and body
image and is in conjunction with a comprehensive approach to
the craniofacial skeleton. The upper face and orbits need to be
set in their proper position before traditional orthognathic sur-
gery in most patients for optimal restoration of facial balance
and harmony. Many patients will have normal mental and phys-
ical development despite their often-disfiguring appearances,
and this remains a major reason for comprehensive surgical cor-
rection of hypertelorism. The goals of surgical corrections of
hypertelorism, regardless of cause, should be to:
● Normalize the anatomical distance between the orbital cones.
● Narrow the nasal width and create an appropriate nasal
projection.
● Correct any soft di use deformities or clefts as well as nor-
malize hair-bearing areas (widow’s peaks or eyebrows).
Fig. 5.1 The facial bipartition can be easily
designed by using virtual surgical planning. This
oblique image demonstrates the segmentation
of face and central wedge resection in a patient
with Apert’s syndrome. The central face con-
cavity can be readily corrected by bowing the
face anteriorly after bipartition. The cutting guide
in place aids in the precise marking of the central
osteotomy.
Orbital Hypertelorism
45

5.2.3 Operative Timing
All pediatric craniofacial reconstructions are a fine balance
between managing appropriate grow th of the craniofacial skel-
eton and optimizing psychosocial development and societal
integration. The advantage of operating earlier in life is to
address the psychosocial aspects as the developing child
becomes more self-aware. The best and most stable results for
correction of hypertelorism are obtained after the grow th of
the cranium and upper one-third of the face or orbits has com-
pleted. By the age of 6 years, the majority of the interzygomatic
width is established and there is adequate descent of the maxil-
lary tooth buds to facilitate an osteotomy below the infraorbital
nerve. Before the age of 5 years, the orbital bones are thin and
fragile, which can make the operation more di cult and a ect
the stability of the fixation, leading to relapse. Most craniofacial
centers proceed with hypertelorism correction between the
ages of 6 and 8 years. Our preferred age of treatment is 8 years,
a time when the upper third of the facial skeleton has com-
pleted the growth, but before the development of the frontal
sinus, which is typically seen at the age of 10 years.
5.2.4 Operative Selection
Before the landmark work of Paul Tessier in the late 1960s, cor-
rection of hypertelorism was not possible. The combined intra-
and extracranial technique allowed appropriate repositioning of
the entire orbital cones. The gold standard of correction is a
choice between orbital shift using either box osteotomies or facial
bipartition. The appropriate choice depends on the relationship
between the orbits and the surrounding craniofacial structures:
● If the maxillary arch is narrow and the corresponding denti-
tion shows apertognathia, with the incisors more cranial than
the molars; a bipartition is the operation of choice to improve
the maxillary arch and the orbits in the same procedure
(Fig. 5.2).
● If a unidirectional repositioning is needed in less-severe
(IOD <35 mm) cases, a box osteotomy is satisfactory. If the
orbits are asymmetric, with laterally and downwardly oblique
positioning, a bipartition is preferred.
● Bipartition is the procedure of choice in the most severe
cases.
● If the nasal structures are narrow, a bipartition and medial-
ization of the upper face can improve the airways by enlarge-
ment of the lower face.
● An encephalocele associated with a midline cleft can easily be
accessed by a bipartition or orbital box procedure.
5.2.5 Orbital Box Osteotomy
The orbital box osteotomy allows for mobilization of the entire
orbital cones into a more normalized position. This procedure is
routinely used to correct vertical orbital dystopia. However, as
stated earlier, some craniofacial surgeons still use this proce-
dure in patients with hypertelorism and a normal occlusion.
Most craniofacial teams prefer the frontal approach, which
allows the surgeon to have an unobstructed view of the anterior
cranial fossa. This exposure also allows the neurosurgeon to
repair any dural tears that are recognized. The design of the
frontal craniectomy must be carefully planned. Surgical prefer-
ence usually dictates the lower limit of the frontal craniectomy.
Fig. 5.2 After facial bipartition and central bone
resection, a fixation guide can be used to mark
optimal orbital translocation and temporary
fixation by using a wire before plate and screw
fixation. Note the large central gap between the
central incisors after proper central orbital trans-
location.
Craniofacial Disorders
46

Some surgeons prefer to keep a frontal bandeau intact at the
lower part of the forehead. Usually, a frontal bandeau of
approximately 1 cm is maintained between the frontal bone
flap and the osteotomies of the supraorbital rim. The supraorbi-
tal rim to be preserved is also usually 1 cm in height, and the
lower limit of the frontal craniotomy should be 2 cm above the
orbits. Most craniofacial surgeons do not preserve the horizon-
tal bandeau that was originally described by Tessier since the
advent of rigid internal fixation. The horizontal bone segment
was typically used to anchor wire fixation of the mobile bone
segments. The lower limit of the craniotomy is kept 1 cm above
the orbits. The frontal craniotomy approach allows access to the
anterior cranial fossa.
5.2.6 Operative Details
Patient Positioning and Preparation
An oral endotracheal tube is placed and secured to the lower
central incisor teeth by using a 24-gauge wire. The upper and
lower eyelids can be closed with a tarsorrhaphy suture and
placement of ointment to protect the cornea. A small strip of
hair is trimmed and saved along the planned coronal incision.
The authors’ choice of local anesthesia is 0.5% lidocaine with
1:100,000 epinephrine. This is injected into the planned coro-
nal incision, the maxillary vestibule, and adjacent to the
infraorbital and supraorbital nerves. The patient is placed in
neck extension into a standard headrest, where the entire
head, face, and ears are prepped and exposed into the surgi-
cal field.
Surgical Exposure
A subperiosteal coronal incision is performed, and the dissec-
tion is carried out over the supraorbital rims. Using a small 2-
mm osteotome, a small osteotomy is usually performed to care-
fully release supraorbital nerve from its foramen. The tempora-
lis muscle is left attached to the cranium, and dissection is
performed deep to the temporoparietal fascia, until the zygo-
matic arch is encountered. An incision is performed onto the
zygomatic bone at this level to prevent fat atrophy and future
temporal hollowing. The lateral rim attachment of the tempora-
lis muscle is released to provide access for the bur holes needed
for craniotomy as well as the inferior oblique fissure. A careful
dissection of the orbital cavity and the nasal processes of the
frontal bone are performed carefully to not damage the optic
nerve. The periorbita is released circumferentially along the
orbital cavity. The anterior ethmoid arteries serve as an ana-
tomical landmark and are identified. Meticulous dissection is
performed around the medial canthal tendon attachment as
well as the nasolacrimal apparatus to avoid detachment or
injury to either structure. Appropriate surgical exposure will
include bilateral zygomatic arches, superior orbital rims, supe-
rior aspect of the nasal processes of the frontal bone, medial
wall of the orbit, and the lateral orbital rim. An upper maxillary
sulcus incision is performed to expose the nasal aperture, pyri-
form rims, zygomatic–maxillary buttresses, and inferior orbital
rims. The infraorbital nerves should be identified to avoid
injury, but care should be taken to minimize soft tissue strip-
ping of the midface.
Frontal Craniotomy
It should be emphasized that in this patient population, anoma-
lies are frequently encountered (thick or bifid crista galli and
deep groove for the longitudinal sinus). The craniotomy bur
holes are typically made in the pterional region. After the fron-
tal flap is elevated, dissection of the dura from the orbital roofs,
greater wing of the sphenoid, and the neighboring temporal
fossa is performed. In cases of a large medial resection or if the
olfactory grooves run close to the medial walls of the orbit, sac-
rifice of the olfactory nerves may be unavoidable. We prefer to
place our medial osteotomy anterior to the crista galli to avoid
injury to the olfactory system. A pericranial flap is useful to
assist with nasal cranial separation at the completion of the
osteotomies. A meticulous repair of any dura tears is manda-
tory. A dural patch using pericranium or deep temporal fascia is
often used to reinforce the closure as needed.
Orbital Osteotomy
A reciprocating saw through a temporalis muscle tunnel is used
to osteotomize the lateral orbital wall, starting at the inferior
oblique fissure. Malleable retractors are used to protect the
periorbita and its contents. Malleable retractors are also useful
for retraction of the brain to expose the anterior central cranial
base anterior to the crista galli. The supraorbital and upper
third of the medial orbit osteotomies are performed transcrani-
ally. The medial thirds of the orbital floor are completed by
using a 2-mm osteotome. These are performed at the centrum
of the globe, typically 2 cm posterior to the orbital rim, to facili-
tate appropriate translocation of the globes. The zygomatic
bones (including the zygomatic arch and its attachment to the
maxilla) are osteotomized with a reciprocating saw, through
the gingivobuccal and coronal incisions. The maxillary osteot-
omy is made typically 5 mm below the infraorbital nerve. The
canine tooth bud remains the only major structure to avoid in
the maxilla. Care to place the medial maxillary cut below the
nasolacrimal duct is necessary to avoid postsurgery epiphora.
Anterior Cranial Base Osteotomy
A V- or U-shaped osteotomy of the nasofrontal region is based
on the desired movement of the orbits and zygoma. The osteot-
omy is performed transcranially in the midline, extending
anteriorly to the crista galli. The paranasal osteotomies are
performed vertically, with a slight divergent angle. The frontal
bone, ethmoid, and nasal septum are removed en bloc. Inspec-
tion of nasal perforations is also performed, and these are
repaired.
Mobilization of the Orbits
Once all of the osteotomies are performed and hemostasis is
achieved, the orbits can be mobilized. Smith spreaders can be
used in a methodical fashion, starting at the lateral orbital rims
and walking them forward. This allows the operator to visualize
any areas that are not fully osteotomized. Attention should be
paid to the pyriform rims as well as to any remaining nasal sep-
tum or posterior ethmoid cells that need to be removed for full
mobilization. The orbits are than passively mobilized medially,
and a 24-gauge wire is used for temporary anchorage. The
Orbital Hypertelorism
47

interorbital distance should be close to 18 mm and may account
for 2 to 5 mm of overcorrection. Virtual surgical planning has
assisted with accurate orbital movement in asymmetric cases; a
contralateral normal side or a control normal side can be super-
imposed and used to accurately measure planed orbital move-
ments (Fig. 5.3 and Fig. 5.4).
Skeletal Fixation and Soft Tissue Draping/
Closure
Rigid skeletal fixation is applied to the midline, zygoma, and
pyriform regions. Often, the bone dust from the burr holes and
bone fragments from the resections can be used to fill in any
vault gaps. Cranial bone grafts are generally necessary to fill the
gaps of larger defects as well as for nasal reconstruction. Bilat-
eral medial canthopexy is performed, as needed, to augment
the medial canthal tendon and the surrounding soft tissue. Typ-
ically, a soft tissue bolster is all that is needed if the medial can-
thus is left attached. The coronal incision is closed in a two-
layer fashion. The oral mucosa can be closed with an absorbable
interrupted suture.
5.2.7 Facial Bipartition
As discussed previously, timing of correction of hypertelorism
is a balance of physiology and psychology. Generally, after 4
years of age, the skull is thick enough to perform stable fixation
and mobilization of the segments. In skeletally mature patients,
Kawamoto describes the use of a facial bipartition with a Le Fort
I osteotomy. This allows control of the maxillary dentition and
correction of any occlusal/skeletal discrepancies. In other clini-
cal scenarios (e.g., Apert’s syndrome), the bipartition can also
be combined with a Le Fort III advancement as part of a secon-
dary procedure following an initial anterior skull remodeling
performed at an earlier age. In general, the facial bipartition is
the preferred procedure to correct orbital hypertelorism.
Patient positioning, preparation, exposure, and craniotomy
are performed identical to the details discussed in the section
on orbital box osteotomy. Of note, when performing the facial
bipartition as an isolated procedure, small bilateral gingivobuc-
cal incisions are made to facilitate the use of the pterygomaxil-
lary osteotomies.
Osteotomies
Bilateral osteotomies are made anterior to the zygomatic arch,
lateral orbital wall, orbital roof, medial orbital wall, pterygo-
maxillary buttress, and the nasal septum. The lateral orbital
wall osteotomy is made with a reciprocating saw. The saw is
passed though the temporalis muscle tunnel. The saw blade is
oriented superiorly and directed toward the inferior orbital fis-
sure. The osteotomy is carried up to the orbital roof and then
across the anterior cranial base anterior to the cribriform plate
at the midline. A small (4-mm) osteotome is used to create the
“doughnut osteotomy” around the entire orbit. Areas of concern
include the medial canthus and the lacrimal apparatus. Osteot-
omies should be made behind the medial canthal attachments.
Intraoral use of the Kawamoto’s osteotomes to make the ptery-
gomaxillary osteotomies finalizes the craniofacial disjunction.
Before down fracture of the monobloc, the intradacryon dis-
tance is measured. A V-shaped resection is planned to leave 16
to 20 mm of bone between the medial canthi region. The
resected region includes the mid portion of the superior orbital
rim, the radix, and the nasal bone. A small intraoral gingivobuc-
cal incision is made to facilitate splitting of the palate.
The monobloc down fracture is performed with Rowe disim-
paction forceps. A double-pronged nasal septal osteotome is
also introduced and directed toward the junction of the hard
Fig. 5.3 The orbital deformity (green and blue)
and the position and shape of a normal orbit
(yellow). The exact distance at which the orbit
should be elevated and the exact dimension at
which the roof needs to be lowered can be
measured and the osteotomies planned as
depicted.
Craniofacial Disorders
48

and soft palates in order to cut the perpendicular plate of the
ethmoid bone. Full mobilization should allow a side-to-side
movement of the monobloc, without much resistance. The pre-
viously marked V segment is then resected with a reciprocating
saw, ensuring a stable bony attachment for the medial canthi.
The reciprocating saw is then used to split the hard palate. A
small 2-mm osteotome is placed between the two central inci-
sors to complete the palatal split. At this point, both halves of
the bipartition can be brought together at the midline and the
intracanthal distance can be measured and verified. Fixation
can be achieved with titanium plates or 24-gauge wire. At this
point, the distraction device can be placed or the Le Fort I
osteotomy can be performed if clinically indicated.
Closure/Soft Tissue Draping
A barrier should be created between the nasal sinus cavities
and the epidural space. A pericranial flap is the most useful and
first-line tool for proper nasal cranial separation. Fibrin glue
can also be placed to fully seal the area. The frontal craniotomy
can be fixated in the usual fashion with miniplates. Medial
canthopexies and medial canthal transnasal wiring can be per-
formed and bolstered externally with zeroform gauze, as
needed. Closure of the coronal flap will reveal the amount of
redundant glabellar skin between the eyebrows. A horizontal
mattress suture can be placed within the hair-bearing area of
the medial eyebrow, known as the Kawamoto’s stitch. The
suture is tightened to cause excess soft tissue to bundle in the
center of the forehead but position the eyebrows appropriately.
Over the following months, the excess tissue will tend to level
out and thus avoid a midline forehead scar. Alternatively, the
excess skin can be used during a second-stage procedure as a
forehead flap for nasal reconstruction seen in rare Tessier 1, 2,
or 3 clefts.
5.3 Complications
5.3.1 Intraoperative
Most common intraoperative complications include bleeding,
cerebrospinal fluid (CSF) leaks, and dural fistulas. The chances
of dural tears and adhesions are often encountered in revisional
Fig. 5.4 (a) The widened ethmoid bone that will be removed before orbital translocation has been marked in blue ink after coronal incision and
exposure. (b) The central segment of bone has been removed after completion of the orbital osteotomies and the bifrontal craniotomy. The nasal
ethmoid cells are easily identifiable in the central image between each medial orbital wall. (c) The space between the orbits has been obliterated by
mobilization of the entire orbit into close apposition of each other. This bone on bone contact is critical for adequate cranial–nasal separation.
Orbital Hypertelorism
49

or secondary procedures. Meticulous identification of tears or
adhesions can prevent most postoperative CSF complications.
Preoperative imaging is needed to evaluate any abnormal or
large venous sinuses that can potentially be encountered intra-
operatively. Visual disturbances can occur postoperatively due
to the mobilization of the orbits that results in imbalance of the
eye muscles.
5.3.2 Postoperative Management
Patients should be admitted to an appropriate intensive care
unit (ICU) postoperatively. Patients are monitored for any signs
of increased intracranial and intraorbital pressure. Careful con-
trol of intraoperative and postoperative fluid administration
will limit the amount of postoperative swelling. Perioperative
and postoperative use of steroids may have a role in the ma-
nagement of postoperative swelling as well. We prefer to avoid
routine steroid use and use steroids as an adjunct for extuba-
tion. We typically start steroid the day before extubation and
complete two additional doses after extubation for a total of 24
hours of steroid use. The amount of periorbital and midface
well can be quite significant. Without tracheostomy, we prefer
72 hours of postsurgical intubation and sedation. This allows
maximal swelling to occur and begin resolution and ensures
adequate hemostasis before extubation. Early extubation has
led to serious maloutcomes in our experience and thus our
abandonment of this practice. When the patient meets clinical
parameters for extubation, it should be performed within 5
days of surgery to assist with patient mobilization and enteral
feeding. Blindness, diplopia, and anosmia are uncommon
complications but should be discussed with the patients.
Postoperative imaging is performed to assess the new bony
reconstruction as well as any possible complications such an
intracranial hematoma or optic nerve injuries. Postoperative
complications for a facial bipartition are similar to those of the
box osteotomies.
5.4 Key Points
● Hypertelorism is a symptom or associated finding that can be
accompanied by a multitude of craniofacial conditions that
are not genetically or epigenetically related.
● Tessier had classically stressed the fact that hypertelorism is
the displacement of the orbital cones in their entirety, mani-
fested by expansion of the central ethmoid sinuses and wid-
ening of the sphenoid wing angle to more than 110 degrees.
● The gold standard of correction is a choice between orbital
shift using either box osteotomies or facial bipartition.
● Selection of treatment option should be based on the closure
pattern and the size of the remaining gap.
Suggested Readings
Kumar AR, Steinbacher D. Advances in the treatment of syndromic midface
hypoplasia using monobloc and facial bipartition distraction osteogenesis.
Semin Plast Surg. 2014; 28(4):179–183
Chan FC, Kawamoto HK, Federico C, Bradley JP. Soft-tissue volumetric changes
following monobloc distraction procedure: analysis using digital three-
dimensional photogrammetry system (3dMD). J Craniofac Surg. 2013; 24
(2):416–420
Greig AV, Britto JA, Abela C, et al. Correcting the typical Apert face: combining
bipartition with monobloc distraction. Plast Reconstr Surg. 2013; 131
(2):219e–230e
Cobb AR, Boavida P, Docherty R, et al. Monobloc and bipartition in craniofa-
cial surgery. J Craniofac Surg. 2013; 24(1):242–246
Bradley JP, Levitt A, Nguyen J, et al. Roman arch, keystone fixation for facial
bipartition with monobloc distraction. Plast Reconstr Surg. 2008; 122
(5):1514–1523
Bradley JP, Gabbay JS, Taub PJ, et al. Monobloc advancement by distraction
osteogenesis decreases morbidity and relapse. Plast Reconstr Surg. 2006; 118
(7):1585–1597
Craniofacial Disorders
50

6 Rare Craniofacial Clefts
Cassio Raposo, Rizal Lim, and Reza Jarrahy
Sum m ary
The treatment of rare craniofacial clefts starts with making an
accurate diagnosis, including an identification of all altered or
missing facial soft tissue and skeletal structures.
The ordered Tessier classification system facilitates in this
diagnostic process and in surgical planning.
Adherence to basic, established surgical principles of soft tis-
sue and skeletal reconstruction facilitate treatment of even the
most complex composite defects.
Surgical intervention is often staged and carried out over
many years to achieve a desirable end result.
The best approach to the longitudinal care of patients with
rare clefts is through close collaborative multidisciplinary
input.
Keywords: rare craniofacial clefts, rare Tessier clefts, facial clefts,
encephalocele, facial bipartition, hypertelorism/hypertelorbitism,
box osteotomy, cleft lip/cleft lip repair, nasal reconstruction
6.1 Introduction
“In the midst of chaos there is also opportunity” — Sun Tzu. As
surgeons, if we look for “common presentations of common dis-
eases” to facilitate our attempts to render accurate diagnoses,
develop appropriate treatment plans, and achieve predictable
surgical outcomes, then the spectrum of disorders collectively
referred to as the rare craniofacial clefts can frustrate even the
most skilled and resolute among us. The rare craniofacial clefts
represent aberrations of development that challenge our diag-
nostic skills and our technical abilities. However, like any other
complex surgical disease, the key to successful management is
making an accurate assessment of the problem, developing a
specific set of longitudinal surgical goals, and having an appre-
ciation for how sequential surgical results will evolve over time.
6.2 Classification and Diagnosis
6.2.1 Embryology
Although a comprehensive discussion of human craniofacial
embryology is well beyond the scope of this chapter, the com-
plexity of the rare facial clefts and their derivation from failures
of embryogenesis merit a brief review of the relevant aspects of
in utero facial development.
Much of the critical development of the craniofacial skeleton
occurs between the fourth and eighth weeks of gestation,
when the endoderm, mesoderm, and ectoderm layers of the
face migrate toward the midline. The frontonasal, maxillary,
and mandibular processes follow programmed patterns of
migration and fusion to create balanced facial features, includ-
ing the cranial base, forehead, orbits, zygomas, maxilla, nose,
stoma, and mandible (Fig. 6.1). Whether due to a failure of
fusion of these migrating processes or due to insu cient migra-
tion of mesenchymal cells into the planes of tissue fusion at the
leading margins of these processes—or a combination of both—
errors in embryogenesis involving these structures can mani-
fest as vertical, oblique, or transverse clefts.
When Paul Tessier presented his approach to classifying rare
craniofacial clefts to the second International Congress on Cleft
Palate in Copenhagen in 1973, he o ered a comprehensive and
concise way to categorize not only the obvious soft tissue
deformities but also the underlying aberrant bony anatomy. His
logical approach to a set of deformities that seemingly defy
logic has since helped us accurately diagnose and appropriately
manage these patients.
Dr. Tessier divided the face into various “zones” that are cen-
tered on imaginary axes running horizontally through the
orbits and vertically through the facial midline. Soft tissue and
bony clefts occurring below the orbits are numbered 0 through
8 and those above the orbits are numbered 9 through 14. This
numbering system encourages us to evaluate the entire height
of the face and thereby more readily identify coincident facial
and cranial clefts. When this occurs, the numbers of the individ-
ual clefts add up to 14 (e.g., 0–14, 1–13, and 2–12 clefts). Inter-
estingly, Tessier defined clefts of the lower lip, mandible, and
associated midline soft tissues of the lower face and neck as
number 30 clefts (Fig. 6.2).
These clefts may be characterized by tissue excess or defi-
ciency; may involve soft tissue, bone, or both; may be unilateral
or bilateral; and m ay be associated w ith num erous other
clefts of diverse orientation. Deconstructing complicated and
overlapping cleft patterns according to the diagnostic hall-
m arks of the individual component clefts, as described later,
helps establish accurate diagnoses and develop appropriate
treatment plans. In general, physical examination should be
complem ented by computed tom ography (CT) to identify
underlying bony abnorm alities and by magnetic resonance
im aging (MRI) to define the extent of intracranial disease, if
appropriate.
Number 0 Cleft
The zero cleft is a midline defect that may involve soft tissue
and bone from the central incisors up through the nasal cavity
and the perpendicular plate of the ethmoid bone. Mild presen-
tations may be limited to an isolated midline cleft of the lip.
With progressive severity, one may note a high and arched or
complete cleft palate with absent premaxilla and philtrum, cen-
tral cleft of the nose with absent columella and nasal septum,
hypoplasia of the nasoethmoid complex, arrhinia, and hypote-
lorbitism. Alternatively, midline tissue excess may result in
enlarged and broadened nasal bones, widened nasal septum,
and enlarged and laterally or superiorly displaced nasal carti-
lages. The isolated zero cleft is very rare; it is usually associated
with the number 14 Tessier cleft (Fig. 6.4).
Number 1 Cleft
The number 1 cleft is a vertical paramedian cleft characterized
by a cleft lip in the region of the Cupid’s bow and soft tissue
Rare Craniofacial Clefts
51

Fig. 6.2 Tessier’s classification of rare craniofacial clefts. In his original
diagrams, Tessier correlated soft tissue deformities. Right: With their
underlying associated bony clefts.
Fig. 6.3 Rare Tessier 30 cleft.
Fig. 6.1 The paired facial processes migrate from lateral to medial during development, contributing to the symmetric features that define the human
face.
Craniofacial Disorders
52

notching through the dome of the nose that extends toward the
medial canthus and medial brow. The nasal defect may include
soft tissue fissures over the nasal dorsum or completely missing
upper and lower lateral cartilages. Heminasal atrophy or a pro-
boscis may be seen in severe forms. A bony cleft between the
central and lateral incisors may extend into the pyriform aper-
ture and cephalad to involve the ethmoid sinuses, the nasal
bone, and the frontal process of the maxilla, resulting in hyper-
telorbitism ( Fig. 6.5).
Number 2 Cleft
In its isolated form, the number 2 cleft is very rare. The cleft lip,
such as the number 1 cleft, occurs in the area of the Cupid’s
bow. The nose is characterized by flattening of the medial third
of the nostril, without true notching. The nasal dorsum may be
widened, and the septum may be deviated. The palpebral fis-
sure and medial brow are intact but may exhibit inferior dis-
placement or epicanthal folding. The bony cleft begins in the
Fig. 6.4 (a) Tessier 0 cleft. This cleft involves the midline caudal to the orbits, resulting in a bifid nasal tip and widened columella but normal
interorbital distance and forehead. (b) Tessier 0–14 cleft. The cranial extension of a number 0 cleft is referred to as a number 14 cleft. An
encephalocele with associated hypertelorbitism may be present, as shown in this patient. (c) The nasal deformity in this 0–14 cleft is characterized by
enlarged and broadened nasal bones, widened nasal septum, and laterally displaced nasal cartilages.
Fig. 6.5 Frontal and lateral photos of a patient
with isolated Tessier number 1 cleft. The dome is
severely notched, and the upper lateral cartilage
on the affected side is absent or other orbital
dystopia and the frontal process of the maxilla,
resulting in hypertelorbitism or other orbital
dystopia.
Rare Craniofacial Clefts
53

region of the lateral incisor, and the skeletal fissure extends
cephalad into pyriform aperture. The frontal process of maxilla
is broad and flat and can be notched. Ethmoid enlargement
contributes to hypertelorbitism.
Number 3 Cleft
The number 3 cleft has been described as an oblique cleft, due
to the vector of tissue involvement, and as an oro-naso-ocular
cleft, due to the facial cavities that it might involve. The soft tis-
sue cleft again begins in the area of the common cleft lip. It con-
tinues along the alar base and then obliquely cephalad toward
the medial canthus. The cheek tissue deficiency foreshortens
the distance from the nasal ala to the medial canthus, causing
inferior displacement of this structure. A lower-eyelid coloboma
medial to lacrimal punctum may be present, along with agene-
sis of part or all of nasolacrimal system. Microphthalmia,
anophthalmia, and epibulbar dermoids may be seen in this
cleft. The cleft in the alveolus occurs between the lateral incisor
and the cuspid and extends through the lateral border of the
pyriform into the nasal cavity. The bony defect may continue
cephalad along the nasomaxillary process to the level of the lac-
rimal bone and orbital rim and floor, resulting in vertical orbital
dystopia. In the most severe cases, absence of bone along this
axis results in a confluence of the orbital, maxillary, nasal, and
oral cavities ( Fig. 6.6).
Number 4 Cleft
This cleft is also oblique in nature, but unlike the numbers 0 to
3 clefts, the lip defect occurs laterally, between the philtral ridge
and the oral commissure. In addition, the defect moves onto the
cheek and largely spares the subunits of the nose, despite the
shortened alar–ocular distance. The medial canthus is largely
spared, as the cleft transitions onto the lower eyelid, lateral to
the punctum. The lacrimal drainage structures are therefore
intact but often dysfunctional. The globe can show anophthal-
mia, microphthalmia, or normal anatomy. The alveolar defect
also occurs between the lateral incisor and cuspid, but the pyri-
form is spared. The bony cleft extends through the maxilla
medial to the infraorbital foramen and then into the orbit. Her-
niation of orbital contents yields a hypoplastic and dystopic
orbit. In the complete form of the number 4 cleft, the oral, max-
illary, orbital cavities are confluent, for which reason this cleft
has been referred to as the oro-ocular oblique cleft ( Fig. 6.7).
Number 5 Cleft
The number 5 cleft is the rarest of the oblique facial clefts and is
rarely seen in isolation. Its clinical characteristics are similar to
those of the number 4 cleft. The maxillary defect occurs lateral
to the infraorbital foramen and then across the rim and onto
the floor of the lateral orbit.
Number 6 Cleft
The number 6, 7, and 8 rare craniofacial clefts often coincide as
Treacher Collins syndrome (TCS), and the isolated number
7 cleft underlies the findings associated with craniofacial micro-
somia. Both of these clinical entities are described in detail else-
where in this text and will therefore not be discussed in depth
here. However, there are relevant aspects of these individual
clefts that merit independent discussion.
In contrast to the syndromic presentation, which is charac-
terized by absence of the zygoma and its arch, the isolated
Fig. 6.6 Tessier number 3 cleft. The cleft lip is located in the region of
the Cupid’s bow. The alar base is involved, and the cleft runs toward
the medial canthus, which is inferiorly displaced. Note the associated
anophthalmia in this case.
Fig. 6.7 Bilateral Tessier number 4 cleft. The nose is largely spared, as
is the medial canthus on the patient’s right, in this bilateral asymmetric
case. Note the left-sided medial canthal distortion, anophthalmia, and
orbital dystopia.
Craniofacial Disorders
54

number 6 cleft is defined by a present but hypoplastic zygo-
matic body and an intact zygomatic arch. Soft tissue manifesta-
tions are similar to those seen in patients with TCS but are
milder in nature ( Fig. 6.8).
Number 7 Cleft
In addition to its role in TCS, the number 7 cleft is the most
common isolated rare craniofacial cleft. The numerous ways in
which it has been historically referred to indicate the variability
in its clinical manifestation: hemifacial microsomia, craniofacial
microsomia, microtia, otomandibular dysostosis, first and sec-
ond branchial arch syndrome, and oromandibuloauricular syn-
drome. Macrostomia is the hallmark feature of the cleft, but soft
tissue involvement can be di use, ranging from small preauric-
ular skin tags to significant deficiency and distortion of the
auricle, cheek, tongue, soft palate, parotid gland, and facial mus-
culature. The cardinal bony defects are centered on the zygoma-
ticotemporal suture, with resultant hypoplasia or absence of
the zygoma or mandible (Fig. 6.9).
Number 8 Cleft
An isolated number 8 cleft is a rare entity. Centered on the
zygomaticofrontal suture, the cleft begins at the lateral palpe-
bral fissure and extends to the temporal region. Soft tissue fur-
rows, discontinuity of the orbicularis oculi ring, colobomas, and
dermatoceles occur in the region of the lateral canthal tendon
insertion. The bony defect manifests as bone loss of the lateral
orbital rim at the level of the suture.
Number 9 Cleft
An isolated number 9 cleft is extremely rare. The cleft is located
at the superolateral angle of the orbit. The upper eyelid may
exhibit full-thickness tissue loss between its median and lateral
thirds. This defect may extend through the brow and obliquely
Fig. 6.8 Isolated Tessier number 6 cleft. (a) This
cleft is considered by some to represent an
incomplete form of Treacher Collins syndrome.
Lower-eyelid colobomas are seen, but the lids and
lateral canthi are less severely dystopic compared
with the syndromic presentation (b) due to
greater underlying skeletal support.
Fig. 6.9 Bilateral Tessier number 7 cleft. Macrostomia is the hallmark of
the isolated number 7 cleft. This patient demonstrates asymmetric soft
tissue and mandibular involvement.
Rare Craniofacial Clefts
55

toward the hairline and may involve the underlying orbit and
the forehead ( Fig. 6.10).
Number 10 Cleft
This cleft represents the cranial extension of the number 4 cleft
and may manifest as a coloboma of median third of the eyelid,
with continuation to the hairline. A whorl of hair-bearing scalp
may be seen in the frontal region in the region of the cleft. The
underlying skeletal defect can include absence of the median
orbital rim, roof, and adjacent frontal bone, with resultant
fronto-orbital encephalocele. In this setting, the orbit is laterally
and inferiorly rotated ( Fig. 6.11).
Number 11 Cleft
The number 11 cleft typically occurs with the number 3 facial
cleft. It passes through the medial third of the upper eyelid and
eyebrow, extending to the frontal hairline. It can involve the
orbit, causing a defect of the medial orbital rim, or the ethmoid
complex, resulting in orbital hypertelorbitism ( Fig. 6.12).
Number 12 Cleft
The number 12 cleft represents the cranial extension of the
number 2 cleft. It passes either through the frontal process
of the maxilla or between this process and the nasal bone.
Because of the increased width of the ethmoid complex,
Fig. 6.10 Tessier number 9 cleft.
Fig. 6.11 (a) Isolated Tessier number 10 cleft
with associated encephalocele. The upper eyelid
and brow are distorted, and the hypertelorbitism
is characterized by lateral and inferior displace-
ment of the orbit. (b) By comparison, this
number 10 cleft has an associated incomplete
form of a number 4 cleft, with a previously
repaired cleft lip, mild perinasal deformity,
shortened naso-ocular distance, and inferiorly
displaced medial canthus. (c) The number
10 cleft can be associated with massive ence-
phaloceles, as in this case with a complex pattern
of multiple severe coincident bilateral clefts.
(d) CTscan shows the large fronto-orbital defect.
Craniofacial Disorders
56

hypertelorbitism is a hallmark characteristic. The olfactory
organs and cribriform plate are typically structurally normal.
The overlying soft tissue may include brow irregularities imme-
diately lateral to its medial edge.
Number 13 Cleft
Displacement of an otherwise-uninterrupted medial brow is
characteristic of a number 13 cleft. The underlying skeletal
anomaly is consistent with cranial extension of the number
1 cleft, passing between the nasal bone and the frontal process
of the maxilla through the ethmoids and cribriform plate,
which may be inferiorly displaced in the setting of a concurrent
encephalocele. Collectively, these skeletal components can
result in severe hypertelorbitism (Fig. 6.13).
Number 14 Cleft
The zero cleft continues onto the central forehead as the number
14 cleft, which has a variety of presentations. If the cleft is char-
acterized by tissue agenesis, clinical manifestations can include
hypotelorbitism, cyclopia, ethmocephaly, or cebocephaly. Associ-
ated holoprosencephaly heralds a poor overall prognosis and
limited life expectancy. When, by comparison, the cleft is charac-
terized by incomplete migration of the frontonasal process, a
resulting bifid cranium or frontal encephalocele may maintain
the orbits in a lateralized position, with resultant hypertelorbi-
tism. In this setting, an enlarged or duplicated crista galli, wid-
ened olfactory grooves, inferiorly displaced cribriform plates,
and enlarged ethmoids are also characteristic (Fig. 6.4).
Number 30 Cleft
Finally, the number 30 cleft encompasses clefts of the lower lip
and mandible that fall on the same axis as the number 0 cleft.
These may involve the tongue, hyoid bone, and midline struc-
tures of the neck to the level of the sternum.
6.3 Nonoperative Management
Definitive management of rare craniofacial clefts is surgical in
nature. Nonsurgical care of patients with rare craniofacial clefts
is generally supportive in nature and helps facilitate successful
surgical outcomes. Under ideal circumstances, patients are
treated in a multidisciplinary craniofacial clinic, with close
coordination among all involved caregivers.
6.3.1 Feeding and Nutrition
In the immediate postnatal period, the rare facial clefts that
involve the upper lip and/or palate may pose challenges to
Fig. 6.12 (a) A rare bilateral Tessier number
11 cleft involves the medial portions of the upper
eyelid and brows, extending toward the frontal
hairline. (b) More commonly, the number 11 cleft
occurs with the number 3 cleft as a Tessier 3–
11 cleft. In this asymmetric bilateral case, in-
volvement of the lip, nose, medial canthus, orbit,
brow, and forehead is more severe on the
patient’s right side.
Fig. 6.13 (a) A Tessier 1–13 cleft with mild nasal
deformity but significant hypertelorbitism with
associated encephalocele. (b) In this case, the 1–
13 cleft is coincident with an ipsilateral number
10 cleft, with associated anophthalmia and orbital
deformity.
Rare Craniofacial Clefts
57

feeding and potentially contribute to dehydration and malnutri-
tion. Parents must be educated and guided on techniques and
materials that can facilitate adequate oral feeds. Consultation
with an occupational therapist who is experienced in the ma-
nagement of children with facial clefts may be indicated. This
support should coincide with a larger more holistic approach to
providing longitudinal psychosocial support to patients and
their families.
6.3.2 Neurologic Assessment
Children with rare craniofacial clefts with any suggestion of
encephalocele, holoprosencephaly, or other structural cranial
deformity should undergo neurologic and neurosurgical assess-
ments. These evaluations should include radiographic examina-
tion of the craniofacial skeleton via CT scan and of the brain via
MRI.
6.3.3 Dentistry/Orthodontia
Virtually all patients with rare craniofacial clefts will require
attention from a pediatric dentist and an orthodontist during
their growth and development. Orthodontic treatment should
be tailored to each individual’s needs, as hypodontia, alveolar
misalignment, malocclusion, and even orthodontic sequelae of
surgical interventions can have aesthetic and functional conse-
quences on occlusal relationships.
6.3.4 Speech
Cleft patients may have di culties with speech, which stems
from structural abnormalities or from learned misarticulations.
The speech pathologist plays a critical and ongoing role in iden-
tifying undesirable speech patterns early in language develop-
ment, providing speech therapy, and monitoring speech
patterns throughout childhood and young adulthood.
6.4 Operative Management
Because of the wide breadth of clinical diversity that is inherent
to rare craniofacial clefts, it is impossible to define specific algo-
rithms or protocols for any one specific cleft entity. However,
Tessier’s ordered classification system allows us to draw upon
well-established basic principles of cleft, craniofacial, and gen-
eral plastic surgery to address the complex defects that we
encounter in these patients. Described later is, first, a summary
of general approaches to surgical management, followed by
some cleft-specific considerations. Although not comprehen-
sive, this overview provides a template for developing surgical
treatment plans.
6.4.1 Soft Tissue Reconstruction
Perioral Reconstruction
Perioral reconstruction is fundamental to all of the facial clefts
that involve the soft tissues of the upper lip (and, in the case
of the number 30 cleft, the lower lip). Repair follows the princi-
ples of common cleft lip repair that are discussed at length
elsewhere in this textbook, including the use of presurgical
nasoalveolar molding devices. The goals of surgery are to estab-
lish continuity of the three tissue layers of the lip—skin, mucosa,
and muscle—with reconstitution of the muscular sphincter, cre-
ation of the philtral unit, shaping of the nose, and aesthetic
placement of scars. Cleft palate repair in patients with rare
facial cleft should follow the common principles of three-layer
intravelar veloplasty and two-layer closure of the hard palate.
Periorbital Reconstruction
The upper and lower eyelids and medial and lateral canthal ten-
dons are involved to varying degrees in rare cleft deformities
and are particularly vulnerable to deformation in the lateral
facial oblique clefts. Eyelid coloboma repair follows conven-
tional principles. Complete colobomas with associated risk for
corneal exposure and keratopathy must be approached with
greater urgency than partial defects with more limited func-
tional deficit. Caudal displacement of the medial and lateral
canthi calls for a variety of corrective measures that include
canthopexy and canthoplasty, Z-plasty, and other interdigitat-
ing local tissue transpositions. Microphthalmia should be man-
aged in coordinated fashion by a pediatric ophthalmologist.
Nasal Reconstruction
The treatment of rare facial clefts demands a thorough under-
standing of the concept of the aesthetic units of the face,
described by Gonzalez-Ulloa. Burget and Menick applied this
concept to develop a comprehensive approach to nasal recon-
struction, including the need for trilaminar nasal reconstruc-
tion, the use of local and regional tissue rearrangement for
lining and coverage of defects, and the placement of scars
between distinct anatomical areas to optimize scar aesthetics.
Microsurgical techniques may also be indicated in complex
defects where the supply of local tissue is scarce.
6.4.2 Bone Reconstruction
In addition to the standard techniques that one would use to
consolidate the maxillary alveolus or reconstruct orbital defects
with autologous bone graft, certain aspects of complex skeletal
reconstruction deserve specific attention, as they relate to the
management of rare craniofacial clefts.
Hypertelorbitism Repair
Hypertelorbitism is a potential trait of any of the rare clefts
associated with skeletal defects above the horizontal axis of the
orbit. Clefts 9 through 14 can present with either symmetric or
asymmetric hypertelorbitism, in which case the major goal of
surgical correction is medialization of the orbit(s). The com-
monly practiced techniques of hypertelorbitism repair are well
described in the literature, as are varying opinions on the ideal
age to perform the surgery to avoid long-term relapse of the
repositioned orbit. In general, older patients with adequate
occlusion are good candidates for an orbital box osteotomy pro-
cedure. By comparison, skeletally immature patients and
patients with inverted-V maxillary morphology benefit from a
facial bipartition procedure. Other considerations that impact
the choice of technique include the amount of cribriform
prolapse into the ethmoid complex and the degree of
Craniofacial Disorders
58

hypertelorbitism, as originally classified by Günther and Tessier.
For patients with an intact calvarium and skull base, hyperte-
lorbitism may be treated by medialization of the orbits via a
subcranial Le Fort III osteotomy.
Box Osteotomy
In this combined intracranial/extracranial approach, coronal
and gingivobuccal sulcus incisions are used to obtain subperios-
teal exposure of the frontal bones, orbits, and midface. A bifron-
tal craniotomy is performed. The interdacyron distance is
measured with calipers. Circumferential orbital osteotomies are
made, with preservation of the medial canthal insertions. The
zygomaticomaxillary and nasomaxillary buttresses are also cut.
Following adequate intracranial midline dissection and retrac-
tion, a central frontoethmoidal segment is resected. The width
of this resection is determined by the planned amount of
correction. The orbits are translocated medially, and the vertical
buttresses and nasofrontal process are rigidly fixed with tita-
nium plates and screws. A pericranial flap is raised and sutured
down to the cranial base in the midline before closure
(Fig. 6.14).
Facial Bipartition
In contrast to the box osteotomy technique, the zygomatico-
maxillary and nasomaxillary buttresses are left intact in a facial
bipartition. Pterygomaxillary, septal, and median palatal osteot-
omies are added to the bone cuts, described earlier, to allow
complete midface mobilization. Rowe disimpaction forceps
are used to down fracture the midface following completion of
the osteotomies. A wedge of central nasal, frontal, and ethmoid
bone is removed, and the hemifacial segments are rotated
toward the midline. Rigid fixation is applied, and autologous
bone grafts are used to stabilize the advanced lateral orbital
rims and zygomatic arches. When indicated, a cantilever nasal
bone graft is placed and rigidly secured ( Fig. 6.15).
Treatment of Encephalocele
When the cleft is associated with an encephalocele, a feature
that is commonly found in the number 10 cleft but is also seen
in number 9 and 11 through 14 clefts, neurosurgical interven-
tion must be incorporated into the longitudinal surgical plan.
The encephalocele can be treated by excision of abnormal,
nonfunctioning brain tissue and simultaneous restoration of
calvarial integrity with autologous bone grafts. Secondary
hypertelorbitism correction, as described earlier, is planned for
a later stage (Fig. 6.16).
The general principles discussed earlier can be applied to any
applicable defect that falls on the spectrum of rare craniofacial
clefting. An elaboration of some specific considerations that
apply to individual clefts is discussed as follows.
Median and Paramedian Clefts: 0–14, 1–13,
and 2–12 Clefts
Surgical treatment depends on the extent of the deformity.
Treatment of the facial and cranial components should be
staged, starting with reconstruction of the cleft lip deformity at
approximately 3 months of age according to standard cleft lip
repair principles. Subsequent repair of the nose can be accom-
plished by a combination of local and regional tissue advance-
ment, rotation, and transposition flaps, in combination with
composite or cartilaginous auricular grafts for support. More
complex reconstructions are necessary in clefts with cranial ex-
tension that a ect the orbits and forehead, following the guide-
lines for management of hypertelorbitism with or without
encephalocele, as described earlier. When there is significant
deformity of the nose, requiring an extensive amount of soft
tissue for coverage, initial correction of the hypertelorbitism
may provide additional mobile tissue to recruit for nasal
reconstruction.
Numbers 3–11 Cleft
The overall goals of care are to provide protective soft tissue
coverage to the globe; restore function to the nasolacrimal
structures; correct the position of the involved medial canthi;
establish acceptable nasal form and function; increase the fore-
shortened naso-ocular distance; and restore bony separation
between the oral, nasal, and orbital cavities. The initial care of
the patient should focus on ocular protection and minimization
of infection from a blind-ending nasolacrimal system. Cleft lip
and nose repair, reconstruction of eyelid colobomas, and clo-
sure of the soft tissue portions of the naso-orbitomaxillary cleft
can begin at 3 months of age. The deficient naso-ocular distance
can be addressed with multiple interdigitating Z-plasties,
regional tissue transfer, or undermining and advancement of
Fig. 6.14 Box osteotomy.
Rare Craniofacial Clefts
59

the superficial musculoaponeurotic system of the face as a Mus-
tarde’s flap. Lower eyelid and medial canthal reconstruction,
also using adjacent or local tissue transfer, can be performed
concurrently or deferred until a later stage.
Bony reconstruction typically follows soft tissue correction.
This may include bone grafting for orbitomaxillary defects,
hypertelorbitism correction, or both. Sources of autologous
bone graft include iliac crest, split calvarium, and rib. Bone graft
resorption is well described, regardless of the graft source, and
may necessitate secondary bone grafting.
Numbers 4–10 Cleft
The principles governing soft tissue reconstruction of the 4–
10 cleft are very similar to those described for the 3–11 cleft,
including goals, techniques, and timing. Ocular protection is a
critical early priority. One significant di erence in the cleft lip
repair technique of the number 4 cleft is to discard the normal
lip tissue that falls between the cleft laterally and the ipsilateral
philtral column medially. Although it is seemingly counterintui-
tive to discard normal tissue in the setting of a disease charac-
terized by severe tissue deficit, this maneuver is necessary to
place the scar within the appropriate facial subunit. Cheek
advancement, lid switch, nasolabial flap, and other regional
flaps, all facilitate cheek, lower eyelid, and medial canthus
reconstruction.
Orbital reconstruction in cases of anophthalmia is a staged
process. The first stage involves the creation of a palpebral
fornix by lining the inner surface of the chosen eyelid’s recon-
structive flap with a buccal or septal mucosal graft. The volume
of the hypoplastic orbit must then be progressively increased in
anticipation of an ocular prosthetic. This can be accomplished
with implanted tissue expanders or with serial placement of
orbital conformers of increasing size.
Management of encephaloceles, which can be particularly
large in number 10 clefts, follows the previously described prin-
ciples. Repair typically requires a combined intracranial/extrac-
ranial approach and adheres to the standard paradigm of three-
layer reconstruction of dura, bone, and skin. A facial bipartition
can be performed at the same time to derotate the orbit. Cor-
rection of the encephalocele and hypertelorbitism usually pro-
vides redundant soft tissue that can be used in secondary
repairs or revisions of the original cheek, eyelid, canthus, or
nasal reconstructions.
Numbers 5–9 Cleft
Similar to the other oblique facial clefts, neonatal corneal expo-
sure should be carefully managed to avoid permanent ocular
injury. As noted earlier, soft tissue considerations take early
precedence. W- or Z-plasty of the cheek is a useful approach to
reconstruction of the soft tissue furrow caudal to the eyelid.
The lower-eyelid coloboma can be repaired with local tissue
rearrangement such as the Fricke’s flap, upper eyelid switch
flap, or advancement of the uninvolved portions of the lower
lid. Abnormalities of the lateral canthal tendon that are
Fig. 6.15 Facial bipartition.
Craniofacial Disorders
60

associated with the cleft should be treated with lateral cantho-
pexy at the time of lower-lid repair. All the principles detailed
earlier regarding management of anophthalmia, microphthal-
mia, orbital defects, hypertelorbitism, and encephalocele in 4–
10 and 3–11 clefts all apply to 5–9 clefts.
6.4.3 Lateral Clefts
Number 6 Cleft
Correction of the cheek soft tissue furrow and eyelid coloboma
that overly the deficient orbitomalar skeleton can be obtained
early in childhood with a combination of Z-plasties and upper-
eyelid transposition flaps. Bone grating to the zygoma, zygo-
matic arch, and orbit can be performed later in childhood when
adequate sources of autologous bone are available. Secondary
soft tissue augmentation is accomplished using standard autol-
ogous structural fat-grafting techniques.
Number 7 Cleft
Initial macrostomia repair should not only match the transverse
length and wet/dry vermilion orientation of the lip on the unin-
volved side but also reorient the zygomaticus major, risorius,
and depressor anguli oris muscles to create the absent modio-
lus. Both straight-line and Z-plasty skin closures are acceptable.
Fig. 6.16 (a) Transillumination of a patient with the complex number 10 cleft, shown previously in Figure 10, shows the magnitude of the
encephalocele defect and the tenuous coverage. (b,c) Combined intracranial/extracranial approach allows repair of the encephalocele and dura,
assessment of the calvarial defect, and repair. In this case, autologous cranial bone and resorbable mesh plates were used to reconstruct the forehead
and provide protective coverage to the brain. (d) Immediate and (e) short-term follow-up before secondary surgical corrections.
Rare Craniofacial Clefts
61

Microtia repair by using autologous rib cartilage, porous poly-
ethylene implants, or osseointegrated prostheses are all well
described.
The technique and timing of skeletal reconstruction of the
deficient mandible depend on the degree of hypoplasia, man-
dibular growth, and secondary e ects on the maxilla. Options
include rib grafting for the congenitally absent mandible, dis-
traction osteogenesis for the significantly shortened mandibu-
lar body or ramus, and conventional orthognathic correction for
skeletally mature patients with mandibular asymmetry and
malocclusion. Free vascularized bone grafts have also been
described for mandibular agenesis.
Number 8 Cleft
Soft tissue reconstruction involves excision of the cleft tissue
and repair with tarsopalpebral flaps and laterally based cutane-
ous flaps. A lateral canthoplasty is performed and covered with
adjacent soft tissue.
6.5 Complications
In any complex disease pattern requiring multiple intricate sur-
gical interventions, adverse events are a reality; these may be
related either to bone or to soft tissue reconstruction. Encepha-
locele and hypertelorbitism repair are associated with numer-
ous complications that are inherent to combined intracranial
and extracranial procedures. These include death, stroke, hem-
orrhage, cerebrospinal fluid leak, meningitis, vascular injury,
and osteomyelitis. Orbital translocation carries the additional
risks of optic nerve injury and inadvertent medial canthal avul-
sion. Frontoethmoidal dissection and resection carry the risk of
olfactory sensory loss. Closely coordinated multidisciplinary
perioperative planning and meticulous intraoperative techni-
que mitigate these risks.
Soft tissue complications may occur more frequently but are
less severe in nature. Partial or total flap necrosis, soft tissue
infections, cicatricial deformities, and recurrent asymmetries
may all contribute to the need for surgical revision.
6.6 Conclusion
The craniofacial skeleton is a three-dimensional anatomical
structure that is constantly evolving within the fourth dimen-
sion of time. In patients with rare craniofacial clefts, specific
anatomical elements of the involved soft tissues or skeleton
may be absent, malformed, or misplaced. Appreciation of the
embryologic theories underlying pathogenesis helps us appre-
ciate the severity of the deformities that we encounter. The
Tessier classification system allows us to evaluate patients sys-
temically and logically. A multidisciplinary and systematic
approach can yield excellent results. However, the ever-chang-
ing nature of facial tissues renders construction of missing ele-
ments or reconstruction of aberrant structures much more
di cult: if the surgeon’s goal is to establish long-lasting balance
and harmony in a face that is devoid of either, we must predict
not only how our results will appear 1 month or 1 year after
surgery but also how those results will evolve over subsequent
decades. Although we have some control over our surgical
interventions, we have little control over how our patients heal
and how scarring contributes to undesirable secondary e ects
on facial growth and function. A forward-thinking perspective
and consistent longitudinal care improve our chances of obtain-
ing long-term success.
The care of patients with rare craniofacial clefts is challenging
and often humbling. When faced with seemingly impossible
reconstructive challenges, we must rely on fundamental surgi-
cal principles, collaboration, and persistence to provide the
level of care that these children require and deserve. Although
we consistently strive to improve our surgical results with
new and innovative techniques, we must not forget that a far
more important measure of success than improvement in the
patient’s physical appearance is whether our involvement in
their care has served to, in the words of Tagliacozzi, “buoy up
the spirit, and help the mind of the a icted.”
6.7 Key Points
● The treatment of rare craniofacial clefts starts with making an
accurate diagnosis, including an identification of all altered or
missing facial soft tissue and skeletal structures.
● The ordered Tessier classification system facilitates in this
diagnostic process and in surgical planning.
● Adherence to basic, established surgical principles of soft tis-
sue and skeletal reconstruction facilitates the treatment of
even the most complex composite defects.
● Surgical intervention is often staged and carried out over
many years to achieve a desirable end result.
● The best approach to the longitudinal care of patients with
rare clefts is through close collaborative multidisciplinary
input.
Suggested Readings
Allam KA, Lim AA, Elsherbiny A, Kawamoto HK. The Tessier number 3 cleft: a
report of 10 cases and review of literature. J Plast Reconstr Aesthet Surg.
2014; 67(8):1055–1062
Alonso N, Freitas RdaS, de Oliveira e Cruz GA, Goldenberg D, Dall’oglio Tolazzi
AR. Tessier no. 4 facial cleft: evolution of surgical treatm ent in a large series
of patients. Plast Reconstr Surg. 2008; 122(5):1505–1513
Cek DI, Güvenç BH, Uzunismail A, Günay Y. Tessier type VI-VII cleft combina-
tion associated with congenital bimaxillary fusion and anophthalmia. J Cra-
niofac Surg. 1997; 8(1):54–57
Fuente-del-Campo A. Surgical correction of Tessier number 8 cleft. Plast
Reconstr Surg. 1990; 86(4):658–661, discussion 662–663
da Silva Freitas R, Alonso N, Shin JH, Busato L, Dall’Oglio Tolazzi AR, de Olive-
ria e Cruz GA. The Tessier number 5 facial cleft: surgical strategies and out-
comes in six patients. Cleft Palate Craniofac J. 2009; 46(2):179–186
Kawamoto HK , Jr. The kaleidoscopic world of rare craniofacial clefts: order
out of chaos (Tessier classification). Clin Plast Surg. 1976; 3(4):529–572
Kumar A, Helling E, Guenther D, Crabtree T, Wexler AW, Bradley JP. Correc-
tion of frontonasoethmoidal encephalocele: the HULA procedure. Plast
Reconstr Surg. 2009; 123(2):661–669
Longaker MT, Lipshutz GS, Kawamoto HK , Jr. Reconstruction of Tessier no.
4 clefts revisited. Plast Reconstr Surg. 1997; 99(6):1501–1507
Pittet B, Jaquinet A, Rilliet B, Montandon D. Simultaneous correction of major
hypertelorism, frontal bone defect, nasal aplasia, and cleft of the upper lip
(Tessier 0–14). Plast Reconstr Surg. 2004; 113(1):299–303
Resnick JI, Kawamoto HK , Jr. Rare craniofacial clefts: Tessier no. 4 clefts. Plast
Reconstr Surg. 1990; 85(6):843–849, discussion 850–852
Craniofacial Disorders
62

7 Facial Fractures
S. Alex Rottgers and Joseph Edward Losee
Sum m ary
Treatment of facial fractures in pediatric patients di ers from
adults. Di erent mechanisms of injury, dental anatomy, facial
proportions, and bone quality result in unique injury patterns
not seen in older patients. Treatment plans must account for
the possibility that the injury and/or operative treatment can
alter future facial growth. Additionally, with future growth
and dental eruption, the pediatric facial skeleton is adaptive
and minor malocclusions and subtly displaced factures may
improve with time. Subsequently, pediatric facial fractures are
treated less frequently with open reduction and internal fixa-
tion, and are more frequently treated conservatively. Open
treatment is sometimes required. In these instances, careful
considerations are needed to protect immature dental struc-
tures and to avoid or remove permanent implants that may
restrict grow th. Basic principles of patient assessment and
operative/non-operative management of pediatric craniofacial
trauma are discussed with special attention brought to the pat-
terns of growth seen in the facial skeleton and how the anatomy
at di erent stages of development should a ect a surgeon’s
clinical decision making when managing facial trauma in young
patients. Unique fracture patterns, fixations techniques, and
potential complications seen only in the pediatric population
are discussed.
Keywords: pediatric, facial fractures, craniofacial trauma, orbital
fracture, nasal fracture, naso-orbital-ethmoid fracture, Le Fort
fracture, zygomaticomaxillary complex fracture, mandible frac-
ture, facial grow th, facial development
7.1 Introduction
Management of facial fractures in pediatric patients di ers from
that of their adult counterparts. Variations in anatomy, di erent
daily activities, and the presence of ongoing grow th account for
these di erences. Facial trauma is more common in children
than in adults, but the facial skeleton is fractured less fre-
quently. Pediatric patients account for 15% of facial fractures.
When fractures occur, they a ect areas of the face with di er-
ent frequencies, occur in di erent patterns, and are more com-
monly greenstick fractures. The treatment goal is not simply to
reestablish normal anatomy and function but to do so without
disrupting future grow th. The plasticity of the growing skull
allows significant remodeling with growth, so more conserva-
tive modalities are often successful. When open reduction
and fixation is necessary, surgeons must avoid injury to
developing dental structures and growth sites, consider the
e ect that wide subperiosteal undermining may have on
future growth, and recognize that fixation hardware has the
potential to restrict growth or migrate into the bone or cra-
nium. The specter of the fourth dimension haunts every sur-
geon caring for these injuries and must weigh on every
management decision.
7.2 Patterns of Craniofacial Growth
Understanding facial development is key to understanding frac-
ture patterns in pediatric patients and anticipating how they
might a ect future growth. At birth, the cranium is significantly
larger than the face, with a proportion of 8:1. With pneumati-
zation of the paranasal sinuses and eruption of the dentition,
this proportion changes over time. At 5 years of age, this ratio
shifts to 4:1 and reaches a ratio of roughly 2:1 at skeletal matur-
ity. The majority of cranial growth is completed by the age of 3
years. The brain’s volume triples in the first year of life. The
neurocranium is 25% of its adult volume at birth, 75% at 2 years
of age, and 95% at 10 years of age. Growth is much more
delayed in the facial skeleton, with the face achieving only 65%
of its adult proportions by 10 years of age.
The cranium and the upper face grow as a result of brain and
ocular expansion. During the early rapid expansion of the cra-
nium, growth is dependent on bone deposition at the cranial
sutures. As this process slows, further growth results more from
appositional bone deposition. Although some grow th remains,
expansion of the cranium and orbits are nearly completed by 6
years of age.
The maxilla and midface continue to grow until roughly 12
years of age. Flattening of the cranial base with early brain
development, grow th at cranial base synchondroses, transduc-
tion of forces from nasal septal growth via the septovomerian
ligament, and vertical alveolar elongation with dental eruption,
all account for the forward and downward displacement of the
maxilla. In addition, pneumatization of the paranasal sinuses
contributes to maturation of the midface. All of the primordial
paranasal sinuses have formed at the time of birth, but only the
maxillary sinus is radiographically visible and clinically rele-
vant. Ethmoid air cells begin to pneumatize in the first 2 years
of life and continue to enlarge thereafter. The sphenoid and
frontal sinuses are not radiographically apparent until after the
age of 6 years and will continue to enlarge in early adulthood.
The mandible begins as two separate bones joined by a carti-
laginous suture at the symphysis, which fuses during the first
year of life. Mandibular growth continues with the rest of the
face during childhood. The mandible continues to grow until
skeletal maturity. Grow th occurs both at the condylar head,
which functions as a growth site, and through centripetal
expansion. Condylar growth contributes to vertical and antero-
posterior (AP) enlargement of the mandible. Eruption of denti-
tion also adds to the vertical height of the alveolar segments, as
it does in the maxilla.
7.3 Varied Fracture Characteristics
in Children versus Adults
The high craniofacial proportion in early life and the lack of
pneumatized sinuses account for the di erent fracture patterns
seen in younger patients. In children younger than 6 years, the
Facial Fractures
63

most common fractures are orbital and cranial fractures. These
structures are proportionally larger and have greater promi-
nence in the immature facial skeleton. Without a frontal sinus
to absorb the energy of impact, fractures are prone to propaga-
tion into the frontal bone and along the orbital roof toward the
apex. Fractures may also extend in an oblique trajectory into
the midface, crossing either the nasal cavity or the buttresses
surrounding the zygoma (Fig. 7.1).
The midface is shielded from trauma and is more elastic and
resistant to fracture in young children. Younger patients rarely
exhibit the classic Le Fort pattern fractures seen in adults and
adolescents. Immature bone is more cancellous and is less min-
eralized. The midface of younger patients is void of sinuses and
is filled with dental follicles. Without aerated maxillary sinuses,
force on the maxilla is transferred to the alveolus and results in
dentoalveolar trauma. The palatal suture remains patent into
adolescence and results in a higher incidence of palatal split
fractures in children.
The lower mineral content and greater percentage of cancel-
lous bone often result in deformations and greenstick fractures
in children. When fractures require open reduction, they may
require osteotomy to allow mobilization and proper reduction.
In addition, dental eruption and remodeling can often over-
come minor deformities and occlusal irregularities. Fractures
that would clearly require open treatment in adults can be
successfully managed with conservative measures and subse-
quent orthodontic correction in children. A new balance must
be found while treating pediatric facial fractures when deciding
what constitutes an operative case.
7.3.1 Diagnosis
Initial Assessment and Physical Examination
Evaluation of pediatric patients with facial trauma should begin
with a comprehensive evaluation according to the principles of
Advanced Trauma Life Support (ATLS) to avoid missing concur-
rent significant or life-threatening injuries. Another serious
injury is present in 56% of patients. The most common associ-
ated injuries are neurologic, which occur in 47% of patients. Half
of these injuries are concussions. Patients must be adequately
screened and referred for appropriate follow-up. A low thresh-
old for evaluation of the cervical (C)-spine and ophthalmologic
consultation must be maintained as well. There is a 2.3 to 10%
concurrence rate between facial fractures and C-spine injuries.
Blindness has been reported in as high as 3% of orbital fractures,
and 12% of orbital fractures have concurrent ocular injuries.
Diagnosing facial fractures in pediatric patients may be di -
cult. Unless the patient is being evaluated immediately after the
injury, swelling will have begun to set in. This may mask subtle
physical findings and asymmetries. If a clear indication for
operative intervention is not apparent, a delayed reevaluation
after the swelling is resolved is necessary to appreciate the
resultant deformity. Examination of the available patient pho-
tographs, dental records, and occlusal models is helpful.
A comprehensive craniofacial examination from the vertex to
the clavicles should be completed for all patients. The patient
must be rolled, and the entire face and scalp must be examined
and palpated for lacerations, ecchymosis, swelling, hematomas,
and step-o s. Lacerations should be irrigated and probed for
fractures prior to closure. A complete examination includes oto-
scopic examination of the tympanic membrane, ophthalmo-
logic evaluation, examination of the C-spine, and palpation of
the neck for sub-Q subcutaneous emphysema and expanding
hematoma. If concern is raised for airway stability, the patient
should be intubated. This may be complicated by the presence
of a C-collar and/or midface or mandibular instability. Pediatric
anesthesia may be of assistance in complicated cases, and
tracheostomy and cricothyrotomy are always means of estab-
lishing a definitive airway in an emergency setting. Further
evaluation includes a full cranial nerve examination.
Signs of orbital trauma such as swelling, ecchymosis, subcon-
junctival hemorrhage, enophthalmos, exophthalmos, diplopia,
limited extraocular mobility, altered visual acuity, irregularity
or asymmetry of the pupil, a relative a erent pupillary defect
(RAPD), and medial or lateral canthal malposition should be
noted. Careful intra- and extranasal evaluations should be car-
ried out. Palpation of fractured nasal bones may reveal irregu-
larities, crepitus, or mobility. Loss of nasal projection may
indicate a complex nasal bone and septal injury, or it may indi-
cate the presence of a naso-orbito-ethmoid (NOE) fracture
when the intracanthal distance is increased. The nasal septum
must be carefully evaluated for the presence of a septal hema-
toma, as failure to drain this may result in septal necrosis and a
subsequent saddle-nose deformity.
Fig. 7.1 Oblique craniofacial fracture. Midface factures with Le Fort
pattern are uncommon when patients are in primary and early mixed
dentition. Young children lack pneumatization of the perinasal sinuses
and have proportionally larger orbits and crania. Cranial fractures are
more common in these patients. Injuries may propagate through the
orbital roof and into the buttresses surrounding the pyriform aperture
and malar prominence. These fractures typically follow on oblique
trajectory, such as that seen in this patient with a left frontal bone
fracture that extended into the orbital roof, ipsilateral nasomaxillary
buttress, and the contralateral inferior orbital rim and zygomatico-
maxillary buttress.
Craniofacial Disorders
64

Intraoral examination must also be carried out, with atten-
tion paid to dental trauma, intraoral lacerations, widened inter-
dental distances, ecchymosis, and hematomas. These may
indicate the presence of alveolar, maxillary, or mandibular frac-
tures. Bimanual manipulation of the maxilla may indicate the
presence of midface instability and a Le Fort fracture. A com-
plaint of subjective malocclusion is sensitive but not specific for
detecting fractures. This history is often unattainable in chil-
dren and is therefore even less useful. A unilateral or anterior
open bite may indicate the presence of condylar fracture. Tris-
mus, or di culty in opening the mouth, may be caused by a
mandibular fracture, impingement by a depressed zygomatico-
maxillary complex (ZMC) or zygomatic arch fracture, or swel-
ling in the muscles of mastication from direct trauma. The
mandibular condyle can be palpated by moving the jaw while
placing one finger in the external auditory canal (EAC) and one
finger in front of the ear. Laceration of the EAC on otoscopic
examination may indicate a condylar fracture as well as a tem-
poral bone fracture.
Radiographic Evaluation
Fine-cut (1–2 mm) maxillofacial computed tomographic (CT)
scans have become the gold standard for assessing fractures of
the craniofacial skeleton. Plain radiographs lack sensitivity, and
adequate interpretation of these studies is becoming a lost art.
The one possible exception to this is the pantomogram, or Pan-
orex. This panoramic study provides a comprehensive, two-
dimensional (2D) survey of the anatomy of the maxilla and
mandible with low costs and radiation doses. It has comparable
sensitivity to current CT scanners in identifying fractures of the
mandible but has less inter-interpreter agreement when ruling
out the presence of a fracture. Furthermore, it lacks the three-
dimensional (3D) information obtainable from a CTstudy. These
3D reformats have become invaluable for characterizing frac-
ture patterns and optimizing exposures and fixation strategies.
When caring for pediatric patients, surgeons must be
thoughtful of the possible e ects radiations have on the cogni-
tive development of the growing brain and of the oncologic risk
from such exposures. In a large retrospective study of the Brit-
ish National Health Service, obtaining two to three head CTs in
patients younger than 15 years could lead to a threefold
increase in the incidence of brain tumors. The absolute risk is
less staggering. This number translates to only 1 additional
tumor for every 10,000 scans performed. This risk is small, and
although it must be considered, it should not dissuade a sur-
geon from obtaining studies needed for good clinical
management.
7.4 Cranial/Frontal Sinus Fractures
7.4.1 Diagnosis
With the increased craniofacial proportions observed in small
children, skull fractures are common and diminish in preva-
lence with age. In the authors’ series, they represented the sec-
ond most common fracture type in children younger than 6
years. These injuries typically present as linear fractures that
may extend into the orbital roof, skull base, nasal capsule, or
zygoma in oblique trajectories. In children younger than 5
years, accompanying dural lacerations may result in a growing
skull fracture, as pulsations cause fracture widening and her-
niation of cranial contents. This occurs in less than 1% of skull
fractures. The parietal bone is the most common location, but it
can occur in the frontal bone or orbital roof. It then results in
frontal swelling or pulsatile exophthalmos (Fig. 7.2).
Patients with frontal sinus, cribriform plate, and other cranial
base fractures should be interrogated for cerebrospinal fluid
(CSF) leaks. In compliant patients with a cleared C-spine, they
should be asked to sit forward and observed for clear-liquid
draining from the nose. They may complain of salty fluid in the
pharynx. Edema, rhinorrhea, epistaxis, an unstable spine, or
altered mental status may complicate this assessment. Cerebro-
spinal fluid will demonstrate a “ring sign” when bloody drain-
age is blotted on an absorbent surface. Clear CSF will migrate
further than blood when absorbed. In addition, when available,
a β-2 transferrin assay is highly sensitive in di erentiating CSF
from other body fluids. Otorrhea may indicate a temporal bone
fracture with CSF leak. Otoscopic examination is necessary to
detect this and other signs of temporal bone trauma such as
hemotympanum and laceration of the EAC.
7.4.2 Nonoperative Management
The goals of managing cranial and frontal bone injuries are pro-
tection of the neurocranium, repair of dural injuries, resolution
of CSF leak, prevention of infection, and correction of aesthetic/
contour deformities. In the absence of significant displacement
or step-o , nasofrontal outflow obstruction, pneumocephalus
indicating dural injury, gross contamination, or underlying neu-
rotrauma, nonoperative management is preferred. Owing to the
rapid cranial expansion seen in the first 3 years of life, signifi-
cant remodeling can be expected, and depressed skull fractures
are more likely to be successfully managed nonoperatively in
small children. Most CSF leaks will resolve spontaneously. Typi-
cally, a course of bed rest and head elevation is adequate. If this
fails, a period of CSF diversion via a lumbar drain or external
ventricular drain is indicated before surgery.
In patients with a frontal sinus, patency of the nasofrontal
duct is paramount. Delayed abscess and mucocele may develop
years after an injury if this structure is injured. In the setting of
a patent duct and minimally displaced fractures, nonoperative
management is warranted, but serial examination and repeat
imaging 3 to 5 years later are necessary. Serial follow-up is nec-
essary in younger patients with frontal fractures to clinically
rule out the development of a growing skull fracture.
7.4.3 Operative Management
Surgical treatment of skull fractures in children is rarely
needed. In the senior author’s series of isolated cranial frac-
tures, 86% were managed nonoperatively; 7.5% required neuro-
surgical interventions such as external ventricular drain (EVD)
placement, hematoma evacuation, and decompressive craniec-
tomy; 6.5% underwent operative repair of the fracture, open
reduction internal fixation (ORIF), frontal sinus repair, and frac-
ture debridement. Frontal bone injuries were more likely to
necessitate operative intervention due to the presence of the
frontal sinus, orbital complications due to orbital roof involve-
ments, and greater cosmetic sensitivity in this location.
Facial Fractures
65

Frontal bone injuries can be approached directly through
extensive local lacerations or via a coronal approach. Any child
with remaining cranial growth potential must have fractures
fixated with absorbable hardware, as metallic implants are
known to translocate through the skull with future appositional
growth ( Fig. 7.3). These plates and screws may become
imbedded in dura, brain parenchyma, or dural sinuses and
present significant potential morbidity. Growing skull fractures
are repaired with craniotomy to allow access and repair of the
underlying dural injury. Reconstruction then requires reconsti-
tution of the normal anatomy, with cortical bone grafts in a
configuration that places a contiguous piece of cortical bone
overlying this repair.
When a frontal sinus is present, the algorithm developed by
Rodriguez and Manson represents the most evidence-based
approach to managing these injuries. The essential decision-
making factor is the presence of trauma and obstruction of the
nasofrontal outflow complex. When the nasofrontal outflow
Fig. 7.3 Transcranial migration of metallic hard-
ware. The use of metallic implants should be
avoided for osteosynthesis in the growing cra-
nium. In addition to growth at the sutures, the
cranium grows through concurrent appositional
growth, with deposition of new bone on the
external cortex and resorption of the internal
cortex. This results in transmigration of perma-
nent implants into the skull. Hardware may
become imbedded in the dura, brain parenchy-
ma, or venous sinuses. The use of suture fixation
or absorbable hardware made with poly(glycolic
acid) (PGA) or poly(lactic-co-glycolic acid) (PLGA)
is suggested.
Fig. 7.2 Growing skull fracture. (a) A 10-year-old child with a growing skull fracture of the left forehead and orbit after trauma. (b) Three-dimensional
CT scan revealing the fracture. (c) Intraoperative view of the growing skull fracture. (d) Reconstructed fracture. (e) One-year follow-up, with normal
forehead and orbital morphology.
Craniofacial Disorders
66

complex is uninjured, displaced anterior wall fractures may be
managed with simple ORIF, as long as duct patency can be
documented intraoperatively. If the drainage pathway is either
obstructed or likely to become obstructed due to scaring from
an injury, the sinus must be defunctionalized. This requires
complete removal of the sinus mucosa by burring the entire
cortical surface, as epithelial cells are known to be present along
draining venous sinuses in the bone and can serve as a tissue
source for reepithelialization. The sinus duct should be filled
with a well-fitting cortical bone plug to promote ossification.
Management of the remaining sinus depends on the fracture
patterns. In the setting of significantly displaced posterior table
fractures, it is best to cranialize the sinus. The posterior table is
removed, dural injuries are repaired, the obliterated sinus duct
is covered with vascularized tissue such as a pericranial or
galeal frontalis flap, and the cranial contents are allowed to
expand into the frontal sinus space. When the posterior sinus
wall is intact, the sinus may be managed by obliteration, in
which the defunctionalized sinus is filled with various graft
materials, including fat, fascia, and bone. Osteoneogenesis—a
technique in which the duct is obliterated, the sinus is demuco-
salized, and the empty sinus is allowed to fill with bone and
fibrous tissue over time—has also been described. Higher com-
plication rates have been shown with the use of fat to fill the
sinus dead space and with osteoneogenesis, and the authors’
preferred technique is to utilize bone, harvested locally from
the cranium or the iliac crest. In a patient with a developing
frontal sinus, the authors have demonstrated that even signifi-
cantly displaced anterior table fractures have the potential of
remodeling with time and continued pneumatization of the
frontal sinus ( Fig. 7.4).
7.5 Orbital, Zygomaticomaxillary,
and Naso-Orbito-Ethmoid
Fractures
7.5.1 Diagnosis
The orbit represents the intersection between the cranium and
the facial skeleton. In the authors’ series, orbital fractures repre-
sent the most common fractures in pediatric patients of all ages,
representing 29.8% of fractures. In children aged 5 years or
younger, orbital fractures represented 56.4% of fractures. This is
likely due to the high cephalic-to-facial ratio and di erences in
midface anatomy, discussed previously. According to the
authors’ findings, as children aged, the incidence of orbital frac-
tures remained high but decreased as children began to su er
more classic adult patterns of injury.
Because of the propensity for various fracture patterns to
transgress the orbit in pediatric injuries, the senior author has
described a classification system for pediatric orbital fractures.
Type 1 injures represent pure orbital fractures, without involve-
ment of adjacent structures. Included within this group are iso-
lated orbital roof fractures, which are more common in early
childhood and are replaced by floor fractures as the child
matures. Type 2 fractures represent oblique craniofacial frac-
tures involving the frontal bone, orbit, and, possibly, midface.
Finally, type 3 fractures represent more common adult patterns
of complex midface trauma, including ZMC and NOE fractures.
Orbital examination is critical in all patients with facial frac-
tures. The authors recommend an ophthalmologic consultation
for all patients with orbital fractures to rule out ocular trauma
and document a baseline visual acuity. This is especially impor-
tant for nonverbal patients and in any patient for whom
operative intervention is considered. With fractures extending
toward the superior orbital fissure, ptosis and ophthalmoplegia
may indicate a diagnosis of superior orbital fissure syndrome
(SOFS), with trauma to the third, fourth, and six cranial nerves.
Diminished visual acuity and an RAPD indicate traumatic optic
neuropathy (TON). If SOFS and TON are present together, the
correct diagnosis is orbital apex syndrome. Involvement of oph-
thalmology in these cases is critical, as vision is threatened and
management is nuanced. Surgical decompression may be indi-
cated if an o ending anatomic lesion is identified. The use of
mega-dose steroids is controversial, with little evidence dem-
onstrating their e cacy. Their use should be on an individual-
ized basis in the setting of an interdisciplinary approach.
Visual inspection of the eyelids for lacerations and injuries to
the lacrimal apparatus is important. Lacerations of the eyelid
medial to the punctum indicate a lacrimal disruption, until pro-
ven otherwise. Integrity of the medial canthal tendon must be
confirmed with a bowstring test. The bowstring test is per-
formed by the examiner by placing a finger over the insertion
of the medial canthal tendon while exerting lateral traction on
the eyelids. Resistance to lateral traction and an intact medial
tendon insertion will be appreciated. A mobile medial canthus,
or asymmetric canthal position, relative to the midline,
Fig. 7.4 Anterior table frontal sinus fracture
remodeling. (a) A patient with depressed anterior
table frontal sinus fracture, without a posterior
table fracture or injury of the nasofrontal outflow
tract, who presented for plastic surgical evalua-
tion 4 weeks after the injury. The family wished
to avoid surgery, and a conservative follow-up
course was adopted. (b) One-year follow-up CT
scan revealed remodeling of the anterior table
without contour irregularity and the absence of
retained fluid in the sinus. An additional follow-up
with repeat imaging 5 years after the injury was
recommended to ensure that the patient had a
“safe sinus.”
Facial Fractures
67

indicates either a canthal avulsion or an NOE fracture. Asymme-
try of the lateral canthus with inferior or posterior displace-
ment may indicate a displaced fracture of the zygoma. Note
should be made of subconjunctival hemorrhage, as this tissue
plane is continuous with the periorbita and, in the setting of
significant trauma, is diagnostic of a periorbital fracture, until
proven otherwise. The surgeon should also look for signs of
enophthalmos, a finding that will often be masked by swelling
in the acute setting. After resolution of swelling, narrowing of
the palpebral fissure, asymmetry of the supratarsal crease, and
deprojection of the globe are diagnostic. Exophthalmos may
also be present in the setting of an impacted zygoma, fronto-
temporal orbital fracture, or orbital roof “blow-in” fracture. Pul-
satile exophthalmos can develop in traumatic carotid cavernous
fistulas and delayed presentation of a growing skull fracture.
Restricted extraocular motility may have multiple causes,
including neurologic lesions such as SOFS; edema and hema-
toma in an extraocular muscle; globe malposition, altering the
functional advantage of the extraocular muscle; or muscle
entrapment. Extraocular muscle entrapment may be indicated
by radiographic findings on a CT scan (Fig. 7.5) and is diag-
nosed clinically. It is rare for large orbital defects to cause
muscle entrapment, though septae connecting the periorbita to
the extraocular muscles may become snared on bony edges.
This mechanism is more common in adults. In young children,
greenstick, linear trapdoor fractures of the orbital floor have a
propensity to close and trap herniated periorbital fat or muscle,
once the o ending compressive force is removed from the orbit.
On physical examination, an individual will have diplopia and
pain on superior gaze and a firm stopping point for ocular
excursion confirmed with a forced duction test. These patients
typically present with increased parasympathetic tone due to
an ocular–cardiac reflex and may be bradycardic and nauseated.
This diagnosis is a true emergency, as the entrapped muscle
may easily become ischemic, resulting in potential scarring and
impaired excursion in the future. This can cause disabling
diplopia.
ZMC fractures typically present with displacement of the lat-
eral canthus, flattening of the malar prominence, trismus from
zygomatic arch impingement on the temporalis muscle, and
subconjunctival and buccal sulcus hematomas. Exophthalmos
may be present in cases where significant impaction is present;
and, enophthalmos may develop postoperatively if reduction of
the fracture is performed without recognizing that reduction of
the impacted segment often results in a significant orbital floor
defect.
NOE fractures are the least common injury in the pediatric
facial skeleton. Fractures of the nasofrontal buttress, nasomaxil-
lary buttress, and medial orbital wall can result in traumatic tel-
ecanthus and loss of dorsal nasal support. The medial orbital
wall segment, or “central fragment,” bears the insertion of the
medial canthus and if displaced, results in traumatic telecan-
thus. Fractures are classified based on the degree of comminu-
tion present. Type 1 fractures include a single fragment; type 2
fractures are comminuted but the canthal tendon remains
attached to a substantial bony fragment, which can be reduced
and fixed in position; and type 3 fractures are severely com-
minuted with an avulsed canthal attachment. Displacement of
bony segments may result in swelling or trauma to the central
lacrimal apparatus. Typically, this is corrected with proper
reduction of the fractures.
7.5.2 Nonoperative Management
Indications for operative treatment of orbital fractures are less
common in pediatric patients. In adults, clear indications such
as blow out fractures with a greater than 1 cm 2 defect, greater
than 50% orbital wall displacement, or bony impingement caus-
ing SOFS or TON are clear-cut. The authors believe that the peri-
orbita and supporting structures of the pediatric globe are
more resilient than those of the adult. Pediatric patients, with
even large bony defects following trauma, often do not develop
enophthalmos, vertical orbital dystopia (VOD), or diplopia. For
this reason, isolated pediatric orbital fractures are best observed
in the absence of acute enophthalmos, VOD, or muscle
entrapment.
In the senior author’s series, type 1 pure orbital fractures
were managed nonoperatively in 88% of cases. Oblique
Fig. 7.5 Inferior rectus entrapment in an orbital floor “trap door” fracture. (a) Coronal and (b) sagittal CT images demonstrating entrapment of the
inferior rectus muscle in a linear, nondisplaced, “trap door” fracture of the orbital floor. Findings of CTscan may be highly suggestive and illustrate the
mechanism of entrapment well, but the diagnosis of entrapment must be made by clinical examination. Patients will typically have diplopia, limited
excursion of the affected eye when gazing upward, and pain with eye motion; they may also have nausea and bradycardia. A forced duction test will
reveal a firm stop preventing upward eye mobility.
Craniofacial Disorders
68

craniofacial injuries, or type 2 fractures, were conservatively
followed unless a clear operative indication was encountered;
and, only 17% ultimately required intervention. Type 3 injuries,
which represent impure orbital fractures involving the sur-
rounding skeleton (inferior orbital rim, ZMC, or NOE), were
more likely to require operation. In the author’s series, 72% of
impure orbital fractures underwent operative repair. These
were the least common injuries, representing 25.9% of cases.
7.5.3 Operative Management
Ophthalmologic evaluation is recommended prior to operative
treatment of periorbital facial fractures. In the setting of pene-
trating globe trauma, retinal pathology, TON, or traumatic glau-
coma a delay in surgery may be warranted. This decision
should be made in conjunction with a trained ophthalmologist.
Acute orbital muscular entrapment with a positive forced duc-
tion test is an operative emergency in the pediatric population.
Often, muscle and/or fat is caught in the fracture line. This tissue
may become ischemic and the best results are achieved with
prompt exploration and release. Other indications for operative
treatment include acute enophthalmos, presentation of clinically
significant enophthalmos after swelling resolution, vertical orbi-
tal dystopia, and impingement from boney fragments.
Access to the orbital floor can easily be achieved through a
transconjunctival approach in either a preseptal or a retroseptal
plane, with limited risk of ectropion and lid malposition. If
needed, this approach can be augmented with a transcaruncu-
lar incision to achieve exposure of the medial orbit and/or a
lateral canthotomy to gain access to the lateral orbital wall.
Reconstruction of the orbital floor and walls requires subper-
iosteal dissection 360 degrees around the perimeter of the
defect. Malpositioned orbital contents are returned to the orbit,
and an implant is placed to partition the two cavities. This
implant must sit on all edges of the defect, including the poste-
rior margin so that it does not settle into the sinus and cause
postoperative enophthalmos. While dissecting to reach the pos-
terior ledge of the defect, the surgeon must remember that the
optic nerve is 40 to 45 mm from the orbital rim; however, these
dimensions will be smaller in younger patients. Orbital defects
may be reconstructed with autologous split calvarial bone, or
alloplastic materials such absorbable mesh, titanium, and poly-
ethylene. These constructs should be rigidly fixed to the orbital
rim to prevent implant migration. Autologous bone reconstruc-
tion is ideal, but one must weigh the morbidity of a graft donor
site against the likelihood of infection and implant migration.
ZMC fractures are rare in children due to the lack of an aer-
ated maxillary sinus. When they occur, they are likely to be sig-
nificantly displaced and require operative reduction. Some
authors have described open reduction via a buccal sulcus inci-
sion alone, with fixation at the orbital rim and zygomaticomax-
illary buttress. However, the authors feel that this is inadequate,
as the best location to assess reduction of the fracture is at the
lateral orbital wall along the zygomaticosphenoid suture. Expo-
sure of the orbital rim and floor can be achieved via a transcon-
junctival approach, but a cantholysis is necessary to assess the
lateral rim through this incision. Alternatives include augment-
ing the transconjunctival approach with a lateral blepharo-
plasty incision or substituting a subciliary approach with lateral
canthal extension in a crow’s foot crease. Care must be taken to
avoid extending the lid incision into the temporal area where
the scar will be more noticeable. Some authors have reported
that the subciliary approach carries a slightly higher risk of
ectropion than the transconjunctival approach; however, the
senior author has found that a carefully planned and executed
subciliary approach with lateral canthal extension into a crow’s
foot crease, combined with robust soft tissue resuspension, can
be an acceptable approach.
Adequate reduction of the ZMC fracture is achieved at the
zygomaticofrontal suture, infraorbital rim, and zygomaticomax-
illary buttress. Typically, reduction and fixation are achieved in
that order. Ensuring that the lateral orbital wall is aligned is the
best way to confirm that the fracture is reduced in all three
dimensions of space. Exploration of the orbital floor is neces-
sary to confirm that the configuration of the orbital floor has
not been altered after reduction and floor reconstruction is not
warranted. Exposure through these three combined approaches
necessitates wide undermining of the entire malar prominence.
Soft tissues must be resuspended to the orbital rim to prevent
cheek and lid ptosis.
Significant NOE fractures are characterized by posterior and
lateral displacement of the nasal bone and medial orbital walls.
Reduction typically requires a combination of coronal, trans-
conjunctival, and buccal sulcus incisions. Anatomic reduction
of a type 1 or 2 fracture should place the canthal tendon in a
proper location. Type 3 fractures often need bone grafting and
transnasal wiring with a posterior–superior vector to correct
the traumatic telecanthus. In severe injuries, overcorrection of
the medial canthus and soft tissue adaptation with bolsters are
often needed. Despite anatomic reduction, handling the soft tis-
sue often determines the result. Bone grafts may be needed to
reconstruct the medial orbital wall and the nasal dorsum.
Children must be observed postoperatively following orbital
surgery, with visual acuity documented. Head elevation, cold
compresses, and steroids may help alleviate swelling and che-
mosis. After significant manipulation of the lower eyelid, the
surgeon should consider placing a Frost stitch to support the
lower eyelid during early healing and edema resolution and to
minimize eyelid malposition. Any change in visual acuity or
unexplained pain should prompt radiographic evaluation. In
the setting of an orbital fracture and reconstruction, a retrobul-
bar hemorrhage resulting in an orbital compartment syndrome
is exceptionally rare; however, it should always be considered.
7.6 Nasal Fractures
7.6.1 Diagnosis
Nasal fractures are the second most common pediatric facial
fracture. Their incidence increases with age. The diagnosis can
often be made clinically. Radiographs are often unnecessary;
however, with significant injuries, a CT scan is helpful in delin-
eating pathologic anatomy, particularly of the nasal septum.
External inspection and endonasal examination for nasal devia-
tion, swelling, lacerations, and septal hematoma are key.
7.6.2 Nonoperative Management
Most practitioners are reticent to perform open manipulation
or resection of the septum, or osteotomies of the nasal bones,
Facial Fractures
69

on growing patients. For this reason, corrective septorhino-
plasty for secondary nasal deformities is typically deferred until
midface skeletal maturity. Operative treatment should be con-
sidered for children with nasal fracture, in the form of a closed
reduction and external fixation. Patients with nondisplaced
injuries require no intervention other than limiting activity for
6 weeks to protect the nose. If significant swelling is present,
the patient should be reevaluated after several days to deter-
mine if a deformity is present. If displacement is noted, all med-
ically stable children benefit from a closed reduction under
anesthesia, to at least lessen the deformity, if not correct it, as
they will not be candidates for formal septorhinoplasty until
they have reached the age of puberty and midface skeletal
maturity.
7.6.3 Operative Management
Initial treatment of nasal fractures consists of closed reduction
under anesthesia, with a protected airway. An elevator is placed
intranasally to elevate depressed nasal bones, and manual
manipulation can straighten the dorsum and improve symme-
try. Ash forceps can be used to straighten a fractured septum.
The nasal complex is immobilized with tape and an external
splint, whereas Doyle splints are used to stabilize the septum.
Nasal packing may be used, when required, to maintain reduc-
tion of a freely mobile and collapsed nasal bone. Antibiotics
are needed while internal splints are in place. Doyle splints
and nasal packing can be removed in 5 to 7 days, and external
splints can be removed after 1 to 2 weeks.
Nasal and septal fractures may result in development of a
crooked nose, dorsal hump, and residual deformities—commonly
seen after closed reduction and external fixation. Residual septal
distortion or scarring may contribute to functional nasal
obstruction. These deformities can be addressed in a delayed
fashion by using traditional septorhinoplasty techniques, but
these techniques should be deferred until adolescence.
7.7 Maxillary and Dentoalveolar
Fractures
7.7.1 Diagnosis
Maxillary fractures are less common in young children and
become more prominent as the maxillary sinus develops and
the permanent dentition erupts. At younger ages, impacts are
absorbed by the cranium and orbital region. Blows to the mid-
face are dissipated by the flexible bone and deflected to the
alveolar ridge. Palatal splits are more common due to a patent
midpalatal suture.
Important attention must be paid to diagnosing malocclusion
in patients with maxillary trauma. This can be di cult in chil-
dren with mixed dentition (6–12 years old). The presence of
mamelons indicates a tooth that has never been in occlusion.
When available, dental records and occlusal models are invalu-
able in determining the preoperative occlusion. These can be
used to fabricate an occlusal splint for use in the operating
room. Bimanual manipulation of the midface is key to deter-
mine if a mobile Le Fort segment is present, which requires
fixation.
Attention must be paid to the dentition. Mobile, missing, and
fractured teeth must be noted. Diastasis between teeth may
indicate a palatal fracture. A pediatric dentist is a valuable ally
in evaluating and triaging injuries to the dentition and alveolar
segment.
7.7.2 Nonoperative Management
The growing maxilla has a great deal of plasticity with growth
and dental eruption. Orthodontics can correct many minor
occlusal irregularities caused by fractures. Open reduction and
internal fixation also places unerupted dental follicles within
the maxilla at risk of injury. For this reason, it is often best to
accept minor malocclusions in young children and manage
them nonoperatively. Dental eruption is typically completed at
around 12 years of age, with the exception of the third molars.
When patients are treated conservatively, jaw rest with a
pureed diet until the fracture has healed is prescribed. Regular
follow-up visits to gauge compliance and to monitor for wor-
sening malocclusion are indicated.
7.7.3 Operative Management
Mobile dentition involved in dental alveolar fractures should be
stabilized using arch bars, occlusal splints, or bonding to the
adjacent dentition. Arch bars are helpful for maxillomandibular
fixation (MMF), but placement can be di cult in children.
However, the authors have reported the successful use of arch
bars in primary and mixed dentition. The primary dentition has
short roots and conical crowns that make it di cult to cinch
wires tightly. Crowding in mixed dentition can also complicate
their application. More inventive means of MMF are often
needed, such as Risdon’s cables and pyriform drop wires com-
bined with circummandibular wires. Palatal fractures can often
be handled conservatively with a palatal splint, with or without
MMF following reduction. Open fixation is sometimes war-
ranted. When MMF is applied in children, the course is typically
shortened due to rapid fracture healing and the propensity for
ankylosis of the temporomandibular joint (TMJ). One week or
less of rigid MMF can be followed by 2 to 3 weeks of elastics,
with close observation for recurrent malocclusion. When ORIF
is indicated, standard exposure and fixation of the zygomatico-
maxillary and nasomaxillary buttresses are indicated. Care
must be taken to avoid traumatizing unerupted tooth follicles.
In addition, if greenstick fractures prevent reduction, the frac-
ture may need to be completed to allow full mobilization and
placement of the segments in occlusion before plating.
7.8 Mandible Fractures
7.8.1 Diagnosis
Trismus, intraoral swelling, hematoma, dental trauma, and mal-
occlusion should warn the clinician of a possible mandible frac-
ture. Condylar and condylar head fractures represent the most
common location for pediatric fractures. The condyle is hyper-
vascular and less mineralized than the adult counterpart,
making it prone to fracture. Falls with a blow to the chin are
a common mechanism, and injuries such as this are often
the presumed etiology of mandibular asymmetry and TMJ
Craniofacial Disorders
70

ankylosis. Distinct patterns of malocclusion are commonly wit-
nessed with subcondylar fractures and should be identified. In
unilateral injuries, the fragments commonly override, causing
shortening of the condylar unit and premature dental contact
ipsilateral to the injury. An open bite is observed on the con-
tralateral side. Bilateral injuries will cause an anterior open bite
through the same mechanism.
7.8.2 Nonoperative Management
The mandibular condyle possesses a remarkable propensity for
remodeling and growth in the first decade of life. Prolonged
immobilization may predispose children for the development
of TMJ ankylosis. The presence of dental follicles and the infe-
rior displacement of the inferior alveolar nerve result in limited
real estate for the placement of fixation hardware. For these
reasons, conservative management of pediatric mandible frac-
tures is often favored. Nondisplaced or minimally displaced
fractures in young children can often be successfully managed
with immobilization by using a jaw bra or cervical collar, along
with a liquid diet. Minor malocclusions may be corrected later
with orthodontic manipulation.
Owing to the risk of ankylosis, intracapsular condylar head
injuries should not be immobilized. Typically, a short period of
mandibular rest with a liquid diet is followed by chewing as the
physical therapy to maintain mobility. In fact, the presence of a
condylar fracture should be considered an indication for ORIF
of concurrent fractures elsewhere in the mandible to avoid the
need for MMF. The most common combined fracture of the
pediatric mandible includes the symphysis/parasymphysis
along with a condylar (head or neck) fracture. The authors rec-
ommend the placement of arch bars, an ORIF of the symphysis/
parasymphysis fracture, and the postoperative use of elastics
and physical therapy of the TMJ as a treatment regimen. Con-
sultation with a pediatric dentist and/or craniofacial orthodont-
ist is also very helpful.
7.8.3 Operative Management
When managing pediatric mandible fractures, the goal is to
restore occlusion while limiting injury to dental follicles or
future grow th. Whenever possible, intraoral approaches or pre-
existing lacerations should be utilized for access while remain-
ing cognizant of fracture fragments’ blood supply. As in
maxillary trauma, placing children in MMF may be di cult,
and alternative methods may be needed.
When required, the period of MMF is abbreviated in pediatric
patients. Often, unilateral subcondylar fractures can be man-
aged with a short course of mandibular rest in MMF (5–7 days),
followed by 2 to 3 weeks of contralateral guiding elastics and
close observation for recurrent open bite. This contralateral
force is able to overcome the masticatory muscles on the side of
the injury, preventing overclosure of the fractured side and a
resulting contralateral open bite. Treatment algorithms for
bilateral injuries are more controversial. When posterior height
is lost with a resultant open bite, the youngest children will
require a course of MMF. In older children approaching skeletal
maturity, this scenario represents a reasonable indication for
ORIF of one or both sides, followed by MMF for the manage-
ment of an unrepaired condyle. Other indications for ORIF of
the condyle include foreign body in the temporomandibular
joint (TMJ), failure of closed reduction to obtain normal occlu-
sion, and displacement of the condyle into the middle cranial
fossa.
Displaced fractures of the angle, body, symphysis, and para-
symphysis require ORIF. Angle fractures can be managed with a
single superior border plate (Champy’s technique). Accepted
principles for the management of other fracture types include
an inferior border plate with bicortical screws, along with a
superior tension band. In children, hardware must be placed at
the inferior border and monocortical screws should be used to
protect tooth buds. Bicortical screws may be used after the sec-
ondary teeth have erupted. A bridle wire, arch bar, or small
plate with monocortical screws may be used as a tension band.
In small children, an inferior border interosseous wire, bridle
wire, and a brief course of MMF may be substituted. Metallic
fixation should be considered for later removal to prevent any
potential growth restriction. When approaching the symphysis
and parasymphysis, preservation of a cu of mentalis muscle
attached to the bone is necessary for resuspension during clo-
sure to prevent chin ptosis.
7.9 Panface Fractures
Open reduction and internal fixation of panfacial fractures
should proceed in a systematic manner. It is necessary to
expose all fractures through multiple approaches before initiat-
ing the sequence of fixation. All buttresses must be recon-
structed, with attention paid to restoring the width, height, and
projection of the face. Fracture fixation must start from a stable,
uninjured point. The sequence of fixation may proceed from
the cranium downward or from the mandible upward. Simi-
larly, fixation may begin centrally and progress laterally or may
begin laterally and progress centrally. The process must be
sequential and not haphazard. Cases with maxillary injuries
and mandibular condyle fractures represent an indication for
ORIF of the condyle to reestablish facial height. Similarly, in a
true panface fracture, ORIF of the zygomatic arch via a coronal
approach may serve as a reliable landmark to establish facial
width when all other buttresses are fractured and unreliable.
7.10 Outcomes
Owing to concern for altered growth, adequately defining out-
comes following pediatric facial fractures remains an elusive
goal. Reported complication rates vary from 2.1 to 32.2%. Serial
clinical and radiographic follow-up until skeletal maturity is
needed to completely evaluate the outcomes of a treatment
protocol. With this in mind, the authors have proposed the fol-
lowing classification scheme for outcomes following pediatric
facial fractures to facilitate thoughtful characterization and
study of these events ( Table 7.1). Type 1 adverse outcomes
directly result from the injury (i.e., blindness after orbital frac-
ture). Type 2 adverse outcomes directly result from the treat-
ment (surgical or nonoperative) of the fracture (i.e., ectropion
after subciliary approach to an orbital fracture). Type 3 adverse
outcomes result from interaction between the injury, its treat-
ment, and subsequent growth and development (i.e., late man-
dibular asymmetry). The true incidence and causative factors
Facial Fractures
71

leading to abnormal posttraumatic growth remain somewhat
elusive, and the role that hardware plays is similarly unclear. If
metallic implants are used in treatment, subsequent removal
should be considered, especially in younger patients and in
cases where dental eruption is impeded by the hardware.
7.11 Conclusion
The neonatal craniofacial skeleton is dramatically di erent from
its adult counterpart. Growth and development result in an
amazing transformation during the first two decades of life.
When treating a pediatric patient with facial trauma, the fourth
dimension must be considered in every treatment decision. The
goal is to ultimately recreate normal form and function, but the
surgeon must recognize that the injury and any subsequent in-
tervention may alter the course of normal grow th. Conservative
treatment strategies are often the best, but when surgery is
indicated, it must be done with extensive knowledge of the dif-
ferent anatomy and growth processes present in the patient.
7.12 Key Points
● Variations in anatomy result in di erent fracture patterns in
children. Young children exhibit more fractures of the cra-
nium and orbit. Complex facial trauma takes the form of obli-
que craniofacial fractures rather than fractures with Le Fort
pattern. As the craniofacial skeleton matures, more mature
patterns of injuries are seen in adolescents.
● With remaining grow th and dental eruption, the pediatric
facial skeleton is amazingly adaptive. It is often best to accept
minor malocclusions or minimally displaced fractures as
future growth, and remodeling may correct these.
● When operative intervention is warranted, care must be
taken to avoid trauma to the growth sites and dental follicles.
Suggested Readings
Grunwaldt L, Smith DM, Zuckerbraun NS, et al. Pediatric facial fractures:
demographics, injury patterns, and associated injuries in 772 consecutive
patients. Plast Reconstr Surg. 2011; 128(6):1263–1271
Moore MH, David DJ, Cooter RD. Oblique craniofacial fractures in children. J
Craniofac Surg. 1990; 1(1):4–7
Rodriguez ED, Stanwix MG, Nam AJ, et al. Twenty-six-year experience treat-
ing frontal sinus fractures: a novel algorithm based on anatomical fracture
pattern and failure of conventional techniques. Plast Reconstr Surg. 2008;
122(6):1850–1866
Bonfield CM, Naran S, Adetayo OA, Pollack IF, Losee JE. Pediatric skull frac-
tures: the need for surgical intervention, characteristics, complications, and
outcomes. J Neurosurg Pediatr. 2014; 14(2):205–211
Losee JE, Afifi A, Jiang S, et al. Pediatric orbital fractures: classification, ma-
nagement, and early follow-up. Plast Reconstr Surg. 2008; 122(3):886–897
Smith DM, Bykowski MR, Cray JJ, et al. 215 mandible fractures in 120 chil-
dren: demographics, treatment, outcomes, and early growth data. Plast
Reconstr Surg. 2013; 131(6):1348–1358
Rottgers SA, Decesare G, Chao M, et al. Outcomes in pediatric facial fractures:
early follow-up in 177 children and classification scheme. J Craniofac Surg.
2011; 22(4):1260–1265
Naran S, Keating J, Natali M, et al. The safe and e cacious use of arch bars in
patients during primary and mixed dentition: a challenge to conventional
teaching. Plast Reconstr Surg. 2014; 133(2):364–366
MacIsaac ZM, Naran S, Losee JE. Pediatric frontal sinus fracture conservative
care: complete remodeling with growth and development. J Craniofac Surg.
2013–40; 24(5):1838–1840
Ne A, Chossegros C, Blanc JL, et al. Position paper from the IBRA Symposium
on Surgery of the Head—the 2nd International Symposium for Condylar Frac-
ture Osteosynthesis, Marseille, France 2012. J Cranio-Maxillo-Fac Surg. 2014;
42(7):1234–1249
Table 7.1 Classification system for adverse outcomes in pediatric facial fractures
Definition

Examples
Type 1

Adverse outcomes related to the injury

Blindness resulting from an injury or tooth loss
Type 2

Adverse outcomes resulting from the treatment of the
fracture
Ectropion following subciliary approach, hardware failure, and
malocclusion after open reduction internal fixation
Type 3

Adverse outcomes resulting from a combination of the injury,
its treatment, and subsequent altered growth and develop-
ment
Progressive hypoplasia, facial asymmetry, or failure of dental
eruption
Craniofacial Disorders
72

8 Cranioplasty
Arin K. Greene and Gary F. Rogers
Sum m ary
The two most important variables to be considered for cranio-
plasty are whether the problem is related to contour (onlay) or
protection of the brain (inlay) and the age of the patient.
Because onlay cranioplasty generally is performed to improve
an aesthetic deformity, we prefer to intervene when children
are older and use an alloplastic material because there is less
resorption compared to bone grafts. We favor porous polyethy-
lene because it allows fibrovascular ingrowth and is easy to
contour. Large full-thickness cranial defects necessitate closure
to protect the brain. We allow spontaneous ossification to occur
during infancy and intervene after this time. Particulate bone
grafting/exchange cranioplasty is our first-line technique in
children and adults because the bone grows with the child and
completely osseointigrates. In adults with significant morbid-
ities we will consider inlay cranioplasty using alloplastic mate-
rials to limit the extent of the operation
Keywords: cranium, cranioplasty, grafts, alloplastic, osseointe-
grate, fibrovascular
8.1 Introduction
Cranioplasty involves either the augmentation (onlay) or
replacement (inlay) of cranial bone. Substances used for cranio-
plasty include (1) autologous bones (typically cranium, iliac
crest, and rib) and (2) alloplastic substances (hydroxyapatite,
demineralized bone, methylmethacrylate, porous polyethylene,
and titanium). Advantages of autologous bone grafts are that
they completely osseointegrate and thus provide strength and
durability and have minimal risk of infection. Disadvantages of
autologous grafts are limited availability, increased operating
room time, and donor-site morbidity.
Alloplastic materials have three advantages: (1) supply is
abundant, (2) there is no donor site, and (3) operative time may
be shortened compared with autogenous techniques. However,
these substances are not osteogenic (capable of new bone for-
mation) and tend to be expensive. Demineralized bone is
osteoinductive (able to induce transformation of undi erenti-
ated mesenchymal cells to osteoblasts). The remaining
substrates are either osteoconductive and provide a sca old
for bony ingrowth (e.g., hydroxyapatite and porous polyethy-
lene) or have no biological activity (e.g., methylmethacrylate
and titanium). Most bioactive alloplastic substances undergo
incomplete revascularization and osseointegration and are sus-
ceptible to latent infection, foreign body reaction, displacement,
and breakdown. The major variables that determine which
material is used for cranioplasty are (1) whether the recon-
struction is onlay or inlay and (2) the age of the patient.
8.2 Diagnosis
Most partial and full-thickness cranial defects can be diagnosed
by physical examination. Large areas are noticed visually,
especially if they involve a non–hair-bearing region. Moderate
to large bony deficiencies can be palpated. Generally, patients
who are planning on cranial reconstruction have imaging of
their defect to facilitate planning of the procedure. Computed
tomography (CT) is the most commonly used imaging modality
because it best delineates the bone. CT subjects the patient to
radiation and thus should be used sparingly. Although we typi-
cally obtain CT prior to an operation, we often do not obtain
postoperative CT to assess the reconstruction if the procedure
is successful clinically.
8.3 Nonoperative Treatment
Intervention for patients with osseous defects is not mandatory.
Those with partial-thickness deficits may su er psychosocial
morbidity if the deficiency is causing a visible deformity. A con-
cavity in a hair-bearing region can be camouflaged with a wig,
hat, scarf, etc. Patients with frontal bone deformities may be
able to grow their hair su ciently to cover the abnormality.
Full-thickness defects less than 1 cm in an adult often heal
spontaneously (critical-size defect). In children younger than 12
months, much larger areas of exposed dura may spontaneously
ossify. Consequently, it is best to wait several months in adults
with small full-thickness cranial defects or longer in infants
with larger areas to allow as much spontaneous ossification as
possible. Some individuals will no longer require intervention,
and if cranioplasty is required, the area to reconstruct will be
smaller.
Patients with small full-thickness defects often do not require
cranioplasty to protect the brain from trauma. However, indi-
viduals with larger deficits are at a greater risk for brain injury
from trauma. Helmeting can be used to protect the brain in
patients awaiting cranioplasty (usually children who are most
susceptible to incidental trauma).
8.4 Operative Treatment
8.4.1 Onlay Cranioplasty
Children
The primary indication for onlay cranioplasty is to correct a vis-
ual deformity to improve the patient’s self-esteem. Because the
cranium has achieved more than 80% of its growth by the age of
4 years, elective procedures to improve appearance should be
done after this time. In addition, a child’s self-esteem does not
begin to form until approximately 4 years of age. Onlay cranio-
plasty is often performed when patients are older and verbalize
that they are bothered by the deformity.
Although autologous bone grafts are preferred for full-thick-
ness defects in children, their use for onlay cranioplasty in the
pediatric population is uncommon. Because underlying osseous
continuity exists, there is less concern that an alloplastic sub-
stance will restrict cranial growth or become dislodged with
growth. Nevertheless, bone thickening will occur around the
implant, and loss of contour over time is not uncommon. Some
Cranioplasty
73

surgeons, however, prefer to use autologous bone grafts for
onlay cranioplasty in children. Split cranial bone is the best
autologous material. When harvested before 5 years of age, split
cranial bone yields limited bone thickness and can be chal-
lenging due to a poorly form ed diploic space. In situ harvest
is not generally recomm ended before 9 years of age. Split
graft is primarily cortical and heals by osteoconduction. The
recipient site should not be burred to punctate bleeding,
because enhanced vascularization will increase resorption of
the graft through greater osteoclast activity. Cancellous bone
graft from the iliac crest and particulate bone graft are not
indicated for onlay cranioplasty because they resorb. These
types of grafts require im mediate vascularization to survive,
which does not occur when they are placed on top of corti-
cal bone.
Drawbacks of split cranial bone for onlay cranioplasty are
donor-site morbidity (e.g., injury to the dura, bleeding, and
thinner bone at donor site) and resorption of the graft. Cortical
grafts may undergo significant resorption when placed over
bone, and thus, patients are at risk for an unfavorable outcome
and repeat cranioplasty. Most surgeons prefer alloplastic mate-
rials for onlay cranioplasty (even in older children), because
there is no donor-site morbidity and minimal resorption
(Fig. 8.1). However, nonautologous substances have a higher
infection rate than bone grafts.
Adults
Because the cranium is almost fully grown by 5 years of age,
concerns regarding dislodgement of alloplastic substances or
restriction of growth are not as relevant after this time. Conse-
quently, many surgeons prefer alloplastic substances instead of
autologous bone grafts, because they shorten operative time,
do not expose the patient to donor-site morbidity, and have
minimal resorption. Although there are numerous alloplastic
substances that are used for onlay cranioplasty, the most com-
mon are hydroxyapatite, methylmethacrylate, and porous poly-
ethylene (Medpore).
Porous polyethylene is our preferred alloplastic material. It
allows osseointegration/neovascularization at the periphery of
the implant and is easily moldable to fit the partial-thickness
defect. Although custom implants can be ordered based on CT
images, contouring standard pieces to the defect is easy and less
expensive. Bone substitutes (e.g., hydroxyapatite) allow some
osseointegration at their periphery but are typically harder to
contour than porous polyethylene and are more susceptible to
fracture and infection. Methylmethacrylate is not commonly
used for onlay cranioplasty, because it does not osseointegrate
and has a higher fracture and infection rates compared with
other materials.
8.4.2 Inlay Cranioplasty
Children
Inlay cranioplasty in children is challenging, because there is
limited autologous donor-site material, and split cranial graft
harvest may be challenging in very young patients. Although
the cranium has completed most of its growth by 5 years of age,
most surgeons prefer to use autologous bone graft until adoles-
cence because the graft will (1) fully osseointegrate and (2) not
restrict grow th or become dislodged as the cranium expands.
Bone grafts undergo less resorption when used for inlay cranio-
plasty compared with the onlay position. Dural-mediated
osteogenesis supersedes the resorptive e ect of revasculariza-
tion and leads to a gain in volume.
The cranium is the preferred donor site for cranioplasty,
because it is in the same field as the defect, there is less donor-
site morbidity, a large amount of material can be obtained, and
the bone undergoes minimal resorption compared with iliac or
rib grafts (Fig. 8.2). Donor-site problems from rib graft har-
vest include contour irregularity, pleuritic pain, and pneumo-
thorax. Iliac procurement causes discomfort and risks injury to
the lateral femoral cutaneous nerve. In contrast to iliac or rib
donor sites, which require a second incision, the cranium is
already exposed during the procedure. The cranial donor site
causes minor discomfort, and the scar is well concealed. Cranial
bone can be harvested as a full-thickness, partial-thickness, or
particulate graft. Although full-thickness bone is excellent for
structural purposes, most surgeons use split cranial bone graft
through the diploic space to obtain two pieces of mostly cortical
bone—one to perform the reconstruction and the other to
repair the donor site. Limitations of split cranial bone grafting
include (1) the segments over the donor and recipient sites do
not result in normal thickness, (2) the technique requires a
well-developed diploic space, and (3) certain areas of the cra-
nium (e.g., temporal bone) are not amenable due to a narrow or
absent diploic space.
Fig. 8.1 An adolescent girl with a partial-thickness parietal defect managed by onlay cranioplasty using porous polyethylene. (a,b) Preoperative CT
images. (c) Porous polyethylene. (d) Intraoperative view of defect (e) after only cranioplasty.
Craniofacial Disorders
74

Because the dura has the ability to spontaneously ossify until
approximately 12 months of age, cranioplasty is generally not
performed in infants, to allow the area become as small as pos-
sible. Our preferred method of inlay cranioplasty in infants and
young children is cranial particulate bone grafting, because this
technique does not require a diploic space for harvest and is
simpler and safer than split cranial bone graft (Fig. 8.3). A 16-
mm Hudson’s brace and D’Errico’s craniotomy bit (Codman &
Shurtle , Inc) are used to harvest corticocancellous bone from
any area of the cranium. The manual drill prevents thermal
injury and ensures maximal graft viability. The material is mixed
with blood, applied to the dura that is at least 0.5-cm thick (or
to match the thickness of the surrounding bone), and covered
with fibrin glue. Particulate graft heals by osteogenesis and
works best when placed over normal or minimally scarred dura.
Over time, the graft becomes indistinguishable from the sur-
rounding bone and can even form a diploic space (Fig. 8.4). An
advantage of particulate graft is that the donor sites are of partial
Fig. 8.2 Resorption of rib graft used for inlay cranioplasty. Preoperative cranial defect (left). Resorption of graft 2 years postoperatively (right).
Cranioplasty
75

thickness and less than critical size. Thus, they reossify and can
be used again to harvest additional bone (reparative osteogene-
sis). Through cycles of harvest and healing, the craniofacial sur-
geon can access a nearly endless supply of autogenous cranial
bone. Particulate graft is di erent from bone dust harvested with
a high-speed burr—this material produces much smaller par-
ticles that lack viable cells and are usually completely resorbed.
Disadvantages of particulate graft are that it does not provide
immediate strength, is less amenable to correcting visible con-
tour deformities of the frontal bone, and has a greater
Fig. 8.3 Technique of particulate bone grafting. (a) Brace and bit with graft material. (b) Ectocortical harvest. Note partial-thickness donor-site
defects. (c) Endocortical harvest.
Fig. 8.4 Inlay cranioplasty using particulate bone graft in a 2-year-old child. (a) Venous malformation of the bone. (b) Defect following removal of the
lesion. (c) Hudson’s brace. (d) Particulate bone graft harvested from the ectocortical surface. (e) Graft placed over the dura. (f,g) CT images 6 weeks
postoperatively. (h,i) Healed graft 2 years postoperatively. Note that the graft is indistinguishable from the adjacent cranium and completely
osteointegrated, and the donor-site defects have healed.
Craniofacial Disorders
76

resorption when placed over scarred dura. These limitations
can be overcome by using exchange cranioplasty (Fig. 8.5 and
Fig. 8.6). This method involves harvesting a full-thickness
piece of parietal bone and placing it over the defect. The donor
site is then filled with particulate graft harvested from the adja-
cent ectocortex or endocortex of the parietal graft.
Almost any size defect can be managed with direct particu-
late bone grafting or exchange cranioplasty. An alternative to
particulate bone/exchange cranioplasty in older children with a
diploic space is split cranial bone grafting. A disadvantage of
this technique is that the donor- and recipient-site bones are
thinner than the surrounding cranium, and in older children,
these sites cannot be used in the future to harvest additional
bone graft. The thickness of split cranial bone can be aug-
mented with particulate graft harvested from the ectocortical
surface. The graft is placed under the cortical bone segment.
Particulate graft o ers some advantages over split cranial bone;
for example, neurosurgical assistance is not required and
particulate graft can also be harvested from those areas of the
cranium that would be di cult to split (e.g., temporal, greater
sphenoid wing, and occiput).
In our opinion, the use of nonautologous bone for inlay cra-
nioplasty in children should be avoided because of the risk of
dislodgement, infection, and growth restriction (Fig. 8.7). The
use of particulate graft has simplified the ability to perform
autologous cranioplasty in the pediatric population. We do not
advocate for the use of bone morphogenetic protein, because
this osteoinduction agent may increase the risk of malignant
transformation and craniosynostosis.
Adults
Many surgeons prefer autologous cranioplasty in adults. Unlike
synthetic material, the healed bone graft has the same proper-
ties as native bone: strength, growth potential, stability, and
resistance to infection. Split calvarial bone and/or particulate
graft/exchange cranioplasty are our first-line techniques. How-
ever, because the cranium is no longer growing in adults, the
use of nonautologous materials is much more prevalent com-
pared with that in children. Alloplastic materials shorten opera-
tive time, obviate donor-site morbidity, and can be custom-
made. These substances are particularly useful for patients with
significant comorbidities.
The most commonly used alloplastic materials for inlay
cranioplasty are titanium, hydroxyapatite, methylmethacrylate,
and porous polyethylene. Titanium has a low risk of infection
and is typically used for small defects, because it does not
provide adequate strength to protect the brain. It also can be
used as a temporary barrier to separate the brain from an over-
lying soft-tissue reconstruction ( Fig. 8.8). Hydroxyapatite,
methylmethacrylate, and porous polyethylene are commonly
employed but should not be placed in continuity with muco-
sae/sinuses, because they are at risk for infection. Hydroxyapa-
tite also has a higher likelihood of fracture/displacement when
used for large areas. Methylmethacrylate is usually not applied
for inlay cranioplasty, because the heat generated from prepar-
ing the material can injure the brain. Medpore is our preferred
nonautologous inlay cranioplasty material, because it can
be easily contoured to the defect and allows fibrovascular
ingrowth and some osseointegration.
Fig. 8.5 Exchange cranioplasty in a 3-year-old child with a frontal bone defect following removal of a dermoid cyst complicated by an infection.
Exchange cranioplasty was chosen instead of direct particulate bone grafting, because the dura at the frontal bone defect was scarred and the visible
area of the forehead was a favorable location for a cortical graft. (a,b) Preoperative appearance of the defect. (c,d) Full-thickness parietal bone graft
harvested and placed into frontal defect. (e,f) Particulate bone graft was harvested from the adjacent ectocortex and used to fill the parietal bone
graft site. (g,h) Healed recipient and donor sites. (With permission from Greene et al 2008.)
Cranioplasty
77

8.5 Complications
Although iliac and rib graft are rarely used for cranioplasty,
donor-site problems can include neuropathic pain, pneumotho-
rax, and deformity. When harvesting split cranial bone, the
dura (and less commonly a subdural vein) can be injured. When
a full-thickness piece of cranium is removed, a neurosurgeon
should be present. Cranial bone grafts used for onlay cranio-
plasty have a high rate of resorption and may require additional
augmentation procedures. Split cranial and particulate bone
grafts used for inlay cranioplasty may not completely heal the
defect and thus repeat grafting may be required. Infection of
autologous bone grafts is rare; the risk of infection, displace-
ment, and fracture of alloplastic substances is greater. If an
alloplastic material becomes infected, it cannot usually be sal-
vaged with antibiotic therapy and requires removal, clearance
of the infection, and then repeat cranioplasty.
8.6 Conclusion
The two most important variables to be considered for cranio-
plasty are (1) whether the problem is related to contour (onlay)
or protection of the brain (inlay) and (2) the age of the patient
(Fig. 8.9). Because onlay cranioplasty is generally performed
to improve an aesthetic deformity, we prefer to intervene when
children are older and use an alloplastic material, because there
is less resorption compared to bone grafts. We favor porous
polyethylene, because it allows fibrovascular ingrowth and is
Fig. 8.6 A 4-year-old child with a large cranial defect following a motor vehicle accident. (a) Appearance before undergoing exchange cranioplasty. (b)
Immediate postoperative CT images to rule out hydrocephalus and dural injury show full-thickness parietal graft over original defect and particulate
graft in the parietal donor site. (c) Healed grafts 3 months postoperatively.
Craniofacial Disorders
78

easy to contour. Large full-thickness cranial defects necessitate
closure to protect the brain. We allow spontaneous ossification
to occur during infancy and intervene after this time. Particu-
late bone grafting/exchange cranioplasty is our first-line techni-
que in children and adults, because the bone grows with the
child and completely osseointegrates. In adults with significant
morbidities, we will consider inlay cranioplasty using alloplas-
tic materials to limit the extent of the operation ( Table 8.1).
8.7 Key Points
● The cranium is the preferred autologous donor site for cranio-
plasty; there are three main types of grafts (full thickness,
split thickness, particulate).
● Alloplastic materials are a good choice for onlay cranioplasty,
especially in older children, because they undergo lesser
resorption than bone grafts.
Fig. 8.7 A 9-year-old child managed with inlay methylmethacrylate at an outside hospital presented at the age of 13 years with pain and an unstable
implant. (a,b) Preoperative image shows nonintegration of the implant with the adjacent cranium. (c–g). Patient was managed by removal of the
alloplastic material and cranioplasty using split parietal bone graft. (h) Osteointegration of the bone graft postoperatively.
Fig. 8.8 Inlay cranioplasty using titanium. (a) A 5-year-old child who suffered a traumatic injury from a motor vehicle accident. (b,c) The brain was
temporarily covered with titanium mesh to support a free flap to provide soft tissue coverage.
Cranioplasty
79

● Cranial bone grafts are better than alloplastic materials for
inlay cranioplasty, because they osseointegrate, grow with
the patient, and provide good strength and resistance to
infection.
● Our preferred onlay cranioplasty material is porous polyethy-
lene, because it allows fibrovascular ingrowth and is easy to
use.
● Our inlay cranioplasty methods are particulate bone grafting/
exchange cranioplasty and split cranial bone.
Suggested Readings
Koenig WJ, Donovan JM, Pensler JM. Cranial bone grafting in children. Plast
Reconstr Surg. 1995; 95(1):1–4
Chen NT, Glowacki J, Bucky LP, Hong HZ, Kim WK, Yaremchuk MJ. The roles
of revascularization and resorption on endurance of craniofacial onlay bone
grafts in the rabbit. Plast Reconstr Surg. 1994; 93(4):714–722, discussion
723–724
Paige KT, Vega SJ, Kelly CP, et al. Age-dependent closure of bony defects after
frontal orbital advancement. Plast Reconstr Surg. 2006; 118(4):977–984
Greene AK, Mulliken JB, Proctor MR, Rogers GF. Primary grafting with autolo-
gous cranial particulate bone prevents osseous defects following fronto-orbi-
tal advancement. Plast Reconstr Surg. 2007; 120(6):1603–1611
Zins JE, Moreira-Gonzalez A, Papay FA. Use of calcium-based bone cements in
the repair of large, full-thickness cranial defects: a caution. Plast Reconstr
Surg. 2007; 120(5):1332–1342
Greene AK, Mulliken JB, Proctor MR, Rogers GF. Pediatric cranioplasty using
particulate calvarial bone graft. Plast Reconstr Surg. 2008; 122(2):563–571
Gosain AK, Chim H, Arneja JS. Application-specific selection of biomaterials
for pediatric craniofacial reconstruction: developing a rational approach to
guide clinical use. Plast Reconstr Surg. 2009; 123(1):319–330
Clune JE, Mulliken JB, Glowacki J, et al. Inlay cranioplasty: an experimental
comparison of particulate graft versus bone dust. Plast Reconstr Surg. 2010;
126(4):1311–1319
Rogers GF, Greene AK, Mulliken JB, Proctor MR, Ridgway EB. Exchange cranio-
plasty using autologous calvarial particulate bone graft e ectively repairs
large cranial defects. Plast Reconstr Surg. 2011; 127(4):1631–1642
Rogers GF, Greene AK. Autogenous bone graft: basic science and clinical
implications. J Craniofac Surg. 2012; 23(1):323–327
Fig. 8.9 Management algorithm for cranial defects.
Table 8.1 Comparison of authors’ preferred materials used for cranioplasty
Particulate
graft
Split cranial graft

Porous
polyethylene
Advantages

Does not require a diploic space for harvest
Can harvest from any area of cranium
Bone becomes as thick as
surrounding cranium
Donor and recipient sites can be
reharvested
Simpler than split cranial graft
Heals over scarred dura
Immediate strength and contour
No donor-site morbidity
Reduced operative time
No resorption
Peripheral
fibrovascular ingrowth
Disadvantages

Less effective if placed over scarred dura
No immediate strength/contour
(Disadvantages are overcome by using
exchange cranioplasty)
Requires diploic space for harvest
Limited donor-site areas
Not as thick as surrounding cranium
Does not completely osseointegrate
Increased infection rate
Cranial growth restriction and
dislodgment if inlay in children
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9 Osseous Lesions of the Craniofacial Region
Salim Afshar
Sum m ary
Osseous lesions in the craniofacial region can be discovered in a
variety of clinical and radiological scenarios and often present as
facial asymmetry or as a disturbance in growth such as tooth
eruption. Having a broad understanding of the range of lesions
that can occur in this area of the body is critical given that benign
lesions can often be destructive and result in significant morbid-
ity. Early diagnosis and a multidisciplinary team approach is often
warranted in order to optimize results for the growing child.
Keywords: buccal, bifurcation, cyst, odontogenic, aneurysmal,
nasopalatine, cementoblastoma, odontoma, cherubism, osteo-
blastoma, myxoma, neurofibroma
9.1 Introduction
Osseous lesions in the craniofacial region can be discovered in a
variety of clinical and radiologic scenarios. Most commonly,
when a pediatric patient is found to have a facial asymmetry or
a mass, initial consultation is with a plastic surgeon or a maxil-
lofacial surgeon. Having a broad understanding of the range of
lesions that can occur in this area of the body is critical to
understanding appropriate workup and surgical management.
Because of the intimate association of lesions in this area with
vital structures such as the jaw, sinuses, orbits, and ears, a mul-
tidisciplinary team approach is often warranted.
In this chapter, we classify osseous lesions of the craniofacial
region broadly into cysts and tumors and further subdivide these
lesions into odontogenic and nonodontogenic cysts and tumors.
9.2 Odontogenic Cysts
Most commonly, odontogenic cysts and lesions are discovered
when there is radiographic investigation for asymmetric or
delayed eruption of adult teeth. It is important to know the tim-
ing of eruption, and if there is significant delay between the
right and left sides, then a radiologic examination is necessary.
The most common finding is a cyst associated with an impacted
and displaced tooth. Odontogenic cysts are broadly divided into
inflammatory and developmental cysts.
In the pediatric population, the most common developmental
cyst is a dentigerous cyst associated with an impacted canine,
a premolar, or a molar such as the third molar (Fig. 9.1a). On
a radiograph, there is a well-circumscribed, unilocular radio-
lucency associated with the crown of an unerupted tooth
attached at the cementoenamel junction (CEJ). The cyst can
cause tooth displacement, and the treatment consists of enu-
cleation of the cyst and, in most cases, removal of the impacted
tooth (Fig. 9.1b, c).
The odontogenic keratocyst and the adenomatoid odonto-
genic cyst were previously considered developmental cysts but
are no more considered tumors. They are now known as kerato-
cystic odontogenic tumor (KCOT) and adenomatoid odonto-
genic tumor (AOT) and will be discussed in the section on
odontogenic tumors.
9.2.1 Inflammatory Cysts
Inflammatory cysts are often associated with a nonvital
tooth and are painful on palpation and percussion. They can be
asymptomatic if the lesion is chronic in nature and are usually
associated with the roots of a nonvital tooth or a tooth with a
large carious lesion. Treatment often involves root canal therapy
of the tooth or extraction, with enucleation of the granulation
tissue within the cyst.
9.2.2 Buccal Bifurcation Cyst
The buccal bifurcation cyst (BBC) is inflammatory rather than
developmental and usually develops on the buccal region of
vital mandibular first or second molars ( Fig. 9.2). It is usually
seen in children between the age of 5 and 15 years. Treatment
options include curettage of the lesion with daily irrigation or
enucleation and removal of the tooth. They can become secon-
darily infected and may require antibiotics and surgical drain-
age of the surrounding tissue.
Fig. 9.1 (a) Large left maxillary cyst associated with an impacted premolar tooth. (b) Intraoperative view of lesion being removed from left maxillary
sinus. (c) Specimen: Large left maxillary dentigerous cyst.
Osseous Lesions of the Craniofacial Region
81

9.3 Nonodontogenic Cysts
The most common nonodontogenic cyst in childhood is the
traumatic or simple bone cyst (SBC). Typically, the patient has
an asymptomatic radiolucent lesion of the mandible, with no
clinical swelling or any other abnormalities on examination
(Fig. 9.3). The lesion favors the male population and presents
in the first two decades of life. The radiographic imaging war-
rants surgical exploration and curettage in order to rule out an
epithelial lined cyst or tumor. The clinical finding is an empty
bony cavity with very little tissue, if any, to submit to pathology.
If the lesion is large enough or near the condylar head, a bone
graft may be warranted, but typically, after curettage of the
bony wall, the lesion will fill in with normal bone over time.
9.3.1 Aneurysmal Bone Cyst
Another similar radiographic presentation to a traumatic bone
cyst is the aneurysmal bone cyst (ABC). This lesion tends to
favor the female population, and often, there can be jaw swel-
ling and pain. The most common type is the vascular type,
which can be rapid growing and destructive ( Fig. 9.4a, b).
Unlike a traumatic bone cyst, on entry of this lesion, you will
encounter brisk bleeding, which is controllable with pressure
and resolves with curettage of the lesion ( Fig. 9.4c–e). Aneur-
ysmal bone cysts tend to have a recurrence rate of 10% and are
part of a spectrum of vascular lesions; these cysts are associated
with jaw tumors.
9.3.2 Nasopalatine Duct Cyst
Finally, the nasopalatine duct cyst (NPDC) is a nonodontogenic
developmental cyst that is usually found incidentally in the
anterior maxilla in the area of the incisive canal. It can become
painful, especially if secondarily infected. Radiographically, on
an occlusal plain film radiograph, one finds a midline heart-
shaped unilocular radiolucency. The lesion often is symmetric
and is present in the anterior maxilla along the length of the
incisive canal (Fig. 9.5). Treatment comprises enucleation and
curettage, with recurrence being very rare.
9.4 Odontogenic Tumors
Odontogenic tumors are common in children because of the
natural course of tooth development of the adult teeth, leading
to eruption of the adult teeth and exfoliation of the deciduous
teeth. As a result, the most common clinical signs of an odonto-
genic lesion are asymmetric tooth eruption and alveolar bone
asymmetry.
Fig. 9.2 (a,b) Axial view of bilateral buccal bifurcation cysts associated with erupting first molars.
Fig. 9.3 Simple bone cyst of the left mandibular ramus.
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