4 Hereditary Developmental and Environmental Influences On The Formation of Dentofacial Deformities 2014 Orthognathic Surgery

4
Hereditary, Developmental, and

Environmental Influences on the
Formation of Dentofacial Deformities
JEFFREY C. POSNICK, DMD, MD
Development of the Head and Neck

Syndromes and Anomalies that Include
Dentofacial Deformities factors that influence facial skeletal growth and dental
Hereditary Tendencies in the Development of development (e.g., the muscles of the neck, tongue, and
Dentofacial Deformities lips; breathing patterns) is important. Factors that are
known to alter facial growth and cause jaw discrepancy can
Environmental and Neuromotor Effects on
be subdivided to include the following: 1) known syn-
Skeletal Growth
dromes and anomalies; 2) hereditary tendencies; 3) envi-
Effects of Trauma on Skeletal Growth ronmental and neuromotor effects; 4) effects of trauma; and
Effects of Tumors and Growths on 5) effects of tumors or growths.
Skeletal Growth
Conclusions Development of the Head and Neck
Skull
The skull is formed from the lateral plate mesoderm (the
The term dentofacial deformity is generally used to describe neck region), the paraxial mesoderm, and the neural crest
a significant disproportion of the jaws in association with (Fig. 4-1). The bony skull is formed by one of two mecha-
malocclusion. It is estimated that, at a minimum, 5% of the nisms: intramembranous ossification or endochondral ossifica-
population will have a discrepancy that falls into the devel- tion. Skull development is divided into two parts: the
opmental dentofacial deformity category. Another 45% of viscerocranium and the neurocranium. The viscerocranium
the population will fall into a much larger group of those forms the bones of the face, whereas the neurocranium
with malocclusion but without what is considered a forms the bones of the cranial base and the cranial vault.
notable jaw discrepancy component.2,3,5,56,65,72 After jaw The neurocranium can be divided into the membranous
growth is complete (i.e., 14 to 16 years of age in girls and neurocranium and the cartilaginous neurocranium. The
16 to 18 years of age in boys), the definitive reconstruction anterior fontanel (bregma) has a wide range of expected
of a dentofacial deformity can go forward. closure between 4 and 26 months of age. The posterior
Knowing the etiology of a patients jaw deformity can be fontanel (lambda) generally closes between 1 and 2 months
important to the fundamental understanding of how and of age.
when to treat the problem. Knowledge of normal facial From the first pharyngeal pouch are formed the maxillary
growth and development as well as the effect of any associ- process, the mandibular process, and the second pharyngeal
ated malformations (e.g., hemifacial microsomia with cervi- arch. Many bones are derived from the maxillary arch,
cal spine anomalies) is useful both to limit complications including the maxilla, the temporal bones, the zygoma, the
during treatment and for family planning. Understanding palatine bone, the lacrimal bone, the vomer, the nasal
the natural history of a condition that involves environmen- bones, and the inferior nasal concha. The mandibular process
tal influences may provide an opportunity to prevent or at forms the mandible, the sphenomandibular ligament, the
least lessen the impact of the deformity. Knowledge of malleus, and the incus. From the second pharyngeal arch are
69
70 S E C T I O N 1 Basic Principles and Concepts
Chondrocranium at 9 weeks
Orbitosphenoid (orbital, Optic foramen
or lesser, wing of future
sphenoid bone) (vision) Greater wing of future
Crista galli sphenoid bone
Nasal capsule (olfaction) Otic capsule (audition)
Meckels cartilage
Incus
Cartilaginous Styloid process
pharyngeal Hyoid cartilage Malleus
arch skeleton Thyroid cartilage
Cricoid cartilage
Pharyngeal arch
mesenchyme for
Membrane bones at 9 weeks viscerocranium Intramembranous
Interparietal ossification (both
part of Head mesenchyme from neural crest)
occipital bone for neurocranium
Frontal bone Cartilage from
Zygomatic
pharyngeal arches
bone
Nasal bone for viscerocranium
Squamous part and neck cartilages Endochondral
Maxilla of temporal bone ossification
Cartilage from somite
Chondrocranium sclerotomes and neural
Mandible crest anteriorly for base
of neurocranium
Membrane bones at 12 weeks Site of future anterior

fonticulus (fontanelle)
Site of future coronal suture
Frontal bone Parietal bone
Nasal bone Interparietal part of occipital bone
Lacrimal bone Greater wing of sphenoid bone

Chondrocranium
Maxilla
Squamous part and zygomatic
Zygomatic bone process of temporal bone
Mandible Tympanic ring of temporal bone
Figure 4-1 Illustrations of the development of the skull. From www.netterimages.com. Elsevier Inc. All rights reserved.
CHAPTER 4 Hereditary, Developmental, and Environmental Influences on the Formation of Dentofacial Deformities 71
Skull of Newborn
Lateral view Anterior fontanelle Parietal bone

Coronal suture Tuber (eminence)
Sphenoidal fontanelle
Frontal bone Squamous suture
Squamous part
Supraorbital notch (foramen) Posterior fontanelle
Lambdoid suture
Ethmoid bone
Occipital bone
Orbital plate
Mastoid fontanelle
Lacrimal bone
Nasal bone
Temporal bone
Maxilla
Squamous part
Petrosquamous fissure
Zygomatic bone Sphenoid
bone Petrous part
Greater wing (mastoid process absent)
Palatine bone Lateral plate of Tympanic part (bony external
Pyramidal process pterygoid process acoustic meatus absent)
Hamulus of medial Oval (vestibular) window
plate of pterygoid
process Round (cochlear) window
Styloid process
Superior view
Mandibular fossa
Zygomatic process
Frontal bone
Anterior fontanelle
Coronal suture
Parietal bone
Sagittal suture
Posterior fontanelle
Occipital bone Lambdoid suture
Figure 4-1, contd

formed the styloid process, the stapes, the hyoid bone, and prominence at the midline results in the formation of the
the stylohyoid ligament. intermaxillary segment.
Face Palate
The face is formed mainly from the neural crest, which The palate is formed by the primary palate (intermaxillary
makes three swellings that surround the stomodeum segment) and the secondary palate (protrusions from the
(Fig. 4-2). The three swellings are the frontonasal promi- lateral prominences) (Fig. 4-3). The intermaxillary segment
nence, the maxillary prominence (from the first pharyngeal (primary palate) is the initial portion of the palate to
arch), and the mandibular prominence (from the first develop. It contains the central and lateral incisors. Swell-
pharyngeal arch). ings of the maxillary prominence form shelves that project
Lateral to the frontonasal prominence, there are two medially but that are separated by the tongue. When the
areas of ectoderm that form the nasal placodes, which invagi- tongue no longer occupies the space between the palatal
nate in the center to form the nasal pits. The placodes that shelves, these processes fuse together to form the secondary
invaginate create ridges of tissues on either side of the pits. palate. The primary and secondary palatal tissues all meet
The ridges are called the lateral nasal prominence and the at the incisal foramen. The primary and secondary palates
medial nasal prominence. The fusion of the medial nasal and the nasal septum fuse to form the definitive palate.
Lateral view at 5 to 6 weeks Ventral view at 5 to 6 weeks Frontonasal

4.0 mm process
Left eye Location Medial nasal
Maxillary of otic vesicle prominence
process of (future membranous
labyrinth of Right Lateral nasal
1st arch nasal
inner ear) prominence
Nasolacrimal pit
1st Maxillary
groove pharyngeal process of
Right
Nasal pit groove eye 1st arch
1st pharyngeal 1st pharyngeal
1st cervical
arch Nasolacrimal arch (mandibular
somite
(myotome groove part)
Cardiac
prominence portion) 2nd pharyngeal
Stomodeum arch
2nd pharyngeal Arm bud 3rd and 4th
1st
arch pharyngeal
pharyngeal
groove arches (sites
of future
Pharyngeal laryngeal
Site of future
arches 3 and 4 cartilages)
hyoid bone
Lateral view at 6 to 7 weeks Ventral view at 6 to 7 weeks

Opening of right nasal
7.0 mm sac (future naris) Intermaxillary segment
1st pharyngeal Medial nasal
Left eye groove Maxillary prominence
Nasolacrimal Nodules that will process
groove of 1st arch Lateral nasal
merge to form prominence
Maxillary auricle of ear
process Right eye Nasolacrimal groove
2nd pharyngeal
Opening of arch Oral opening
left nasal sac 1st pharyngeal
(future naris) 3rd and 4th arch Nodules that merge to
pharyngeal form auricle of ear
Cardiac 1st pharyngeal
arches in 3rd and 4th pharyngeal
prominence cleft (future
cervical sinus arches in cervical sinus
external acoustic
1st pharyngeal meatus) (sites of future laryngeal
Oral opening cartilages)
arch 2nd pharyngeal arch
Figure 4-2 Illustrations of the development of the face. From www.netterimages.com. Elsevier Inc. All rights reserved.
Ventral view at 7 to 8 weeks
Site of nasolacrimal groove (fusion of

lateral nasal and maxillary processes)
Site of fusion of medial nasal and maxillary
processes (site of cleft lip)
Auricle of ear
Philtrum of upper lip (fusion of
medial nasal processes)
Lateral view at 7 to 8 weeks Lateral view at 8 to 10 weeks

10.0 mm 24.0 mm
Auricle of ear
Auricle
of ear
Fused eyelids
Lateral nasal process
Philtrum of
Medial nasal process upper lip
Philtrum
Figure 4-2, contd
Syndromes and Anomalies that Include holoprosencephaly), although these patients sometimes
Dentofacial Deformities have mild mental deficiencies. Syntelencephaly (the absence
or hypoplasia of the corpus callosum or absent or hypoplas-
Many of the syndromes and anomalies that involve the tic olfactory tracts and bulbs) is compatible with life and
cranio-orbito-zygomatic (upper face) region also affect the should thus be treated. Associated facial anomalies include
maxillomandibular (lower face) location.1,20,124 These condi- retinal colobomas, cleft lip, cleft palate, single maxillary
tions may involve any of the tissue components, including central incisor, and midface deficiencies. Holoprosenceph-
the skin, the muscles, the nerves, the fat, the vessels, the aly has multiple causes and includes many chromosomal
bone, the teeth, the cartilage, and the associated viscera (i.e., types, many monogenetic disorders, and at least three
brain, ears, eyes, salivary glands, sinuses). Known syndromes teratogens.
and anomalies comprise only a small proportion of the
dentofacial deformity group (see Chapters 27 through
Deficiencies and Anomalies of Neural
31).19 These conditions can be further subdivided according
Crest Origin
to etiology or tissue of origin.
Most of the tissues of the face, including the muscular and
skeletal components, are derived from the mesoderm. Inter-
Deficiencies of Midline Tissue Structures
estingly, throughout the rest of the body, these components
Holoprosencephalies involve variable deficiencies of midline (i.e., muscle and skeleton) are derived from an ectodermal
tissues. These structural deficiencies have been documented origin.19 The facial mesodermal elements develop from the
to occur early during embryologic development.16,18,23,27 neural crest and then migrate downward beside the neural
They may range from severe brain anomalies to simple tube and laterally under the surface ectoderm. When the
absence or hypoplasia of the corpus callosum. Severe facial neural crest cells have completed migration to their specific
anomalies such as cyclopia, ethmocephaly, and cebocephaly location, then facial development is dominated by specific
are always associated with severe holoprosencephaly, and growth centers, the formation of visceral structures (e.g.,
they are incompatible with life. This can also be true of brain, eyes, sinuses), and the differentiation of the tissue
premaxillary agenesis; however, in some instances, patients layers. Facial anomalies of neural crest origin are thought to
may survive for several months or even years. There are arise when the neuroepithelium has apoptosis (i.e., the
reported cases of premaxillary agenesis with normal head cells die).25 This results in less neuroepithelium to migrate
circumference and no brain abnormalities (i.e., without through into the facial locations and to ultimately
Frontal (coronal) section at 7 to 8 weeks
Right olfactory bulb
Ethmoid cartilage
Frontal bone
Ocular muscles
Eyeball Septal cartilage
Eyelid
Maxilla Nasal septum
Right lateral palatine process Tongue

of secondary palate
Muscles of facial expression
Meckels cartilage
Mandible
Submandibular salivary gland
Hypoglossal (XII) nerve
Hyoid cartilage
Platysma
Thyroid cartilage
Lumen of larynx
Esophagus
Frontal (coronal) section at 8 to 10 weeks
Septal cartilage
Superior concha and meatus
Ethmoid cartilage
Eyeball
Frontal bone
Lens
Ocular muscles
Middle concha and meatus
Fused eyelids
Right nasal cavity Maxilla
Inferior concha and meatus Zygomatic bone
Muscles of facial expression

Enamel organs of deciduous
molar teeth Sites of fusion of lateral
Palatine processes and
Meckels cartilage nasal septum
Oral cavity
Submandibular salivary gland
Mandible
Hyoid cartilage
Tongue
Infrahyoid muscles Platysma
Figure 4-3 Illustrations of the development of the palate. From www.netterimages.com. Elsevier Inc. All rights reserved.
Roof of stomodeum (inferior view; 6 to 7 weeks)

Frontal area
Opening of right nasal sac
Medial nasal process
Right eye
Lateral nasal process
Primitive or primary palate Nasolacrimal groove

(median palatine process)
Definitive nasal septum
Oronasal membrane (primitive Maxillary process of 1st arch
posterior naris or choana)
Lateral palatine process
Roof of stomodeum (secondary palate)
(base of skull)
Opening of Rathkes pouch
Palate formation (inferior view; 7 to 8 weeks)
Left naris
Philtrum of upper lip (fusion

of medial nasal processes) Site of fusion of medial nasal and
maxillary processes (cleft lip site)
Primary palate (median Primitive posterior naris or choana

palatine process) (former site of oronasal membrane)
Left lateral palatine process

Definitive nasal septum
Site of evagination of
Mucosa covering base of skull Rathkes pouch
Roof of oral cavity (inferior view; 8 to 10 weeks)

Left naris
Broken lines border area
formed from medial nasal Upper lip
processes and primary palate
Medial palatine process Gingiva (gum)

(primary palate contribution
to definitive palate) Palatine raph (cleft palate site)
Lateral palatine process
(secondary palate Palatoglossal arch
contribution to
definitive palate) Palatopharyngeal arch
Arrow emerging from choana
(posterior naris of right nasal cavity) Uvula
Figure 4-3, contd
differentiate. Current knowledge of Treacher Collins syn- (variations of hemifacial microsomia and Treacher
drome indicates that this is not a migration problem but Collins syndrome). Despite these known relationships,
simply a lack of cells to migrate (Figs. 4-4 and 4-5). teratogenic agents are not the most frequent causes of these
Teratogens that are known to result in head and neck syndromes.1
(neural crest) congenital anomalies include cytomegalovi- The branchial arch syndromes are neural crest anomalies
rus (microcephaly, hydrocephaly, microophthalmia); Dilan- that comprise an etiologically heterogeneous group of dis-
tin (cleft lip and palate); vitamin D excess (premature orders.14 They account for only a small percentage of the
suture closure); Valium (cleft lip and palate); Rubella virus patients who present to the surgeon or orthodontist in need
(microophthalmia, cataracts, deafness); and thalidomide of jaw reconstruction. The best known of these are
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Figure 4-4 An 11-year-old boy who

was born with Treacher Collins syndrome
has typical malformations of the soft Orth
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tissues and the skeletal structures within
the first and second branchial arches on
both sides. There is significant hypoplasia
of each zygomatic complex, the orbits,
and the maxillomandibular structures
(Kaban type II-A mandible). The soft
tissues of the adnexal region are deficient.
The external ears are malformed, but
all parts are present. The central part of
the face is fully formed. A, Frontal facial
and computed tomography scan views.
B, Profile facial and computed tomogra-
phy scan views. C, Oblique facial and
C
computed tomography scan views.
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Figure 4-5 This mother and daughter demonstrate the extent of variation of expression of Treacher
Collins syndrome within a family. The mother was not aware that she carried the Treacher Collins gene
until after the birth of her daughter. From Posnick JC: Treacher Collins syndrome: perspectives in
evaluation and treatment. J Oral Maxillofac Surg 55:11201133, 1997.
hemifacial microsomia, its variant Goldenhar syndrome, monozygotic twins (35%) as compared with dizygotic twins
and Treacher Collins syndrome.14 Hemifacial microsomia (7.8%).138 Nevertheless, orofacial clefting is heterogeneous
affects aural, zygomatic, and mandibular growth at a and variable, and it is likely determined by a number of
minimum (Fig. 4-6). The disorder may be mild or severe, major genes, minor genes, environmental factors, and a
and it is generally limited to one side of the face. However, developmental threshold.37 Some syndromes associated
bilateral involvement, with more severe expression on one with clefting in which specific gene mutations have been
side, is also known to occur. Goldenhar syndrome, which is identified include van der Woude syndrome (IRF6), popli-
a variant of hemifacial microsomia, also includes epibulbar teal pterygium syndrome (IRF6), autosomal-recessive cleft
dermoids and vertebral anomalies. Involvement is often palate/ectodermal dysplasia (PVRL1), hypodontia/clefting
but not always limited to the face. There may be cardiac, (MSX1), Hay-Wells syndrome (TP63), and cleft palate/
renal, skeletal, and central nervous system anomalies ankyloglossia (TBX22). These mutations explain only about
(see Chapter 28). At a minimum, patients with Treacher 5% of clefting cases, with an additional 10% to 15% of
Collins syndrome demonstrate the physical findings cases explained by variations involving the IRF6 gene.110
of bilateral zygomatic hypoplasia, down-slanting palpebral Although orofacial clefting is caused by a malformation, the
fissures, malformed ears, and micrognathia (see Chapter development of secondary deformities of the jaws after birth
27). Inheritance occurs in an autosomal-dominant fashion, is well known (see Chapters 32, 33, and 34). For the most
and expressivity varies (see Figs. 4-4 and 4-5). The syn- part, maxillary hypoplasia in patients with orofacial clefts is
drome maps to 5q32-q33.1, and mutations in the TCOF1 felt to be the result of palate surgical interventions carried
gene are of the nonsense, insertion, deletion, or splice- out during infancy and early childhood and not from the
site types.14,30,139 primary congenital anomaly. Although van der Woude and
Stickler are just 2 of more than 100 clefting syndromes, they
demonstrate the importance of having an awareness of asso-
Clefting of the Lip and Palate
ciated anomalies to assist with family counseling and to
The most prevalent congenital defect of dentofacial devel- achieve effective reconstruction.
opment is clefting of the lip, the palate, or both (Figures 4-7
through 4-19). This condition occurs in approximately 1 in Van der Woude Syndrome
1000 whites, 1 in 500 Asians, and 1 in 2000 blacks.27 The In 1845, Demarquay was the first to report the occurrence
etiology of clefts is often complex and multifactorial. Evi- of congenital sinuses of the lower lip in combination with
dence supports the view that genetic factors are associated cleft lip and palate; this condition became known as van der
with orofacial clefting.37 In twins with cleft lips and palates, Woude syndrome. This syndrome occurs in roughly 1% to
concordance is far greater among monozygotic twins (40%) 2% of patients with lip and palatal clefts. Van der Woude
as compared with dizygotic twins (4.2%). In twins with syndrome has an autosomal-dominant inheritance pattern;
isolated cleft palate, concordance is also higher among Text continued on p. 91
Orthognathic Surgery; J.C. Posnick
Figure 4-6 A 7-year-old boy born with hemifacial microsomia and unilateral cleft lip and palate (left side) has typical malformations of the soft
tissues and skeletal structures within the first and second branchial arches on the left side. There is an absence of the left zygomatic complex and
hypoplasia of the orbit, the anterior cranial vault, and the maxilla. The mandibular malformation is a Kaban type III. The soft tissues of the left
external ear, the ear canal adnexal structures, and the cheek region are markedly deficient. A, Frontal facial and computed tomography scan views.
B, Oblique facial and computed tomography scan views.
Figure 4-7 Cleft lip and palate represents a spectrum of morphologic findings that are initially dependent on the
individuals specific anomalies but that are then affected by surgical intervention during growth. A newborn with a
complete (left side) unilateral cleft lip and palate is shown.
Elsevier Inc. Elsevier Inc.

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Figure 4-8 A Hispanic child was born with complete clefting of the left primary lip and palate. He was
adopted when he was approximately 1 year old, before repair. He then underwent single-stage primary
lip, nasal, and palate repair. He is shown before repair and then at the age of 2 years after single-stage
repair.
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Figure 4-9 A child was born with an incomplete cleft of the left lip that also involved the alveolar ridge back
to the incisal foramen. She is shown before primary lip and nasal reconstruction and then at the age of 5 years
before alveolar cleft grafting.
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C D
Figure 4-10 A child was born with a complete (left side) unilateral cleft lip and palate. He is shown before and at intervals after primary lip and
palate repair. A, He is shown before primary lip and nasal reconstruction and B, at the age of 10 months, just before cleft palate repair. C, The
same child at the age of 3 years and D, at the age of 8 years just before mixed dentition bone grafting.

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Figure 4-11 Three newborns with bilateral cleft lip and palate (BCLP) are shown. A, A newborn with complete BCLP. B, A newborn
with BCLP but with partial attachment of the lip and the nasal floor on the left side. C, A 3-month-old infant with complete BCLP is
shown. The premaxilla is attached to the septum of the nose; it is forwardly projecting without attachment to the lateral lip segments.
The wide separation of the palatal shelves is also demonstrated by the intraoral view. D and E, A series of computed tomography
scan views of the maxillofacial complex of the infant shown in C demonstrate the skeletal anatomy associated with BCLP.

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Figure 4-12 A child born with complete bilateral cleft lip and palate. He is shown
before and then 7 years after primary lip and palate repair, just before mixed dentition
bone grafting.
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Figure 4-13 A child born with incomplete bilateral cleft lip and palate. He is shown before and then
8 years after primary lip and palate repair, just before mixed dentition bone grafting.
Elsevier Inc. Elsevier Inc. Elsevier Inc.

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Figure 4-14 A child born with a complete cleft of the right lip and palate and incomplete clefting of the left lip. He is shown
A, before and then B, at 2 and C, 8 years after primary lip and palate repair. The last photo was taken just before mixed dentition
bone grafting.
A B
Figure 4-15 A, A child born with complete clefting of the secondary palate (i.e., incisal
foramen through the uvula) is shown. B, An adult born with a complete unilateral cleft
lip and palate is also shown. He underwent lip repair during childhood, but the cleft
palate was neglected. Note the severe septal deviation that obstructs the right nasal
valve.
Figure 4-16 A teenage boy from Jamaica who was born with a bilateral cleft lip and a unilateral cleft of the
alveolar ridge and palate. He underwent rudimentary lip repair as a child. The unrepaired clefted alveolus and
palatal anatomy are shown. Note the normal growth parameters of the upper jaw when the palate is unrepaired.
With a lack of continuity of the alveolar ridge, there has been typical distorted growth of the maxilla.
B C
Figure 4-17 A woman who was born with bilateral cleft lip and palate and pitting of the lower (central) lip. At the time of her
pregnancy, an ultrasound confirmed twins, one of which was suspected of having bilateral cleft lip and palate and the other of
having unilateral cleft lip and palate. This was documented at the time of delivery. A, A family with van der Woude syndrome,
including a mother with a repaired bilateral cleft lip and palate and newborn twins. B, Twin A with bilateral cleft lip and palate. C,
Twin B with unilateral cleft lip and palate.
Figure 4-18 A 16-year-old boy who was born with Stickler syndrome (type II collagen mutation). At the time of birth, Pierre Robin sequence
was appreciated. The patient underwent repair of the cleft palate before he was 1 year old. He had positive eye findings, and he required the
treatment of a retinal detachment during his teenage years. A small cataract is being followed. He arrived for the evaluation of a jaw deformity and
malocclusion characterized by maxillomandibular deficiency with anterior open-bite malocclusion. This was combined with chronic obstructive nasal
breathing and a long face growth pattern. Attempted growth modification and camouflage orthodontics earlier during life were ineffective. There
was generalized root deficiency throughout the maxillary and mandibular dentition, likely as a result of the collagenopathy. A, Frontal views in repose
before and after treatment. B, Frontal views with smile before and after treatment. Continued
Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

C. Posnick
Figure 4-18, contd The patient agreed to an orthodontic and surgical approach. Further orthodontic (dental) decompensation was cautiously
carried out as a result of the compromised periodontal apparatus. The procedures included maxillary Le Fort I osteotomy (vertical intrusion, hori-
zontal advancement, counterclockwise rotation, and arch expansion); bilateral sagittal split ramus osteotomies (horizontal advancement and coun-
terclockwise rotation); osseous genioplasty (vertical shortening and horizontal advancement); and septoplasty, inferior turbinate reduction, and nasal
floor recontouring. C, Oblique facial views before and after treatment. D, Profile views before and after treatment.
Prior to retreatment
Pre surgery
E
After treatment
Figure 4-18, contd E, Occlusal views before retreatment, with orthodontics in progress, and after treatment.
Continued
Maxillary counter-
clockwise rotation
Arch expansion
Mandibular counter-
rotation
F
Figure 4-18, contd F, Articulated dental casts that indicate analytic model planning. G, Lateral cephalometric radiographs
before and after treatment.
H
After surgery
Figure 4-18, contd H, Panorex radiographs before and after treatment that demonstrate generalized
limited root formation. The right maxillary first molar was lost 5 years earlier as a result of limited roots.
The right maxillary first molar was lost 1 years after surgery as a result of limited root support; the right
mandibular first molar will likely be lost to similar pathology. Periodontal evaluation and treatment are
ongoing. Future plans are for the placement of dental implants in the right posterior maxilla and
mandible.
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Figure 4-19 A child born with Robin sequence, which consists of retrognathia with glossoptosis, clefting of the secondary
palate, and a degree of respiratory distress. A, Frontal facial and intraoral views demonstrating clefting of the secondary palate.
B, Profile facial view indicating retrognathia. Computed tomography scan demonstrating retrognathia but with all of the compo-
nents of the jaw present. For this child, the small mandible is the result of deforming forces during fetal development rather than
the result of a malformation. Catch-up growth of the mandible is anticipated during childhood. C, Profile and occlusal views when
the patient was 9 years old, with expected catch-up growth.
it has approximately 90% penetrance, and it is variably Treacher Collins syndrome) may also be missing parts or
expressed (see Fig. 4-17). Manifestations of the syndrome sections of the jaw (i.e., Kaban types IIB and III) and
outside of the oral and facial regions are unusual. Generally, cannot be expected to grow out and self-correct. This is
the pits are characterized as depressions that are observed different from a deformed mandible (i.e., retrognathic at
on the vermilion border of the lower lip; they are usually birth) caused by fetal malpositioning, which can be expected
bilateral and symmetrically placed, although an asymmetri- to grow out and become relatively normal. The newborns
cal pit or single central pit may occur. Roughly 33% of respiratory compromise that occurs with Robin sequence
patients with van der Woude syndrome have pits without will vary according to the childs physical findings, and
clefting, 33% have pits with cleft lip and palate, and 33% treatment should proceed accordingly. For example, with
have lip pits with isolated cleft palate or submucous cleft spondyloepiphyseal dysplasia congenita, the causes of respi-
palate. Approximately 10% of those individuals with van ratory compromise include a small and mechanically abnor-
der Woude syndrome do not exhibit lip pits. mal chest, tracheobronchial malacia, and central apnea as a
result of cervical or medullary compression caused by cervi-
Stickler Syndrome cal instability.47 This will require a different level of airway
In 1967, Stickler and Pugh were the first to describe a management as compared with an isolated retrognathic
series of patients who had a combination of eye findings, mandible, which is simply deformed as a result of intrauter-
hearing loss, isolated cleft palate, a Marfanoid habitus, and ine compression. If the deformed (retrognathic) mandible
long-bone changes.1 It is now known that Stickler syndrome is present at birth, it is expected to catch up during growth
is associated with basic defects in collagen. These colla- and basically to self-correct. For these infants, mandibular
genopathies include mutations on type II collagen and osteotomies with advancement carried out during child-
mutations in type XI collagen.25 The inheritance pattern hood should be avoided.
with this syndrome is autosomal dominant with variable
expressivity. Cohen and others have reviewed the relation-
Achondroplasia
ship between high myopia and clefting of the secondary
palate and the other features of Stickler syndrome.19 High Achondroplasia is the most common condition associated
myopia (i.e., 8 to 18 diopters) is found in 75% of patients with severe disproportionate short stature.22 The cause of
by the time they are 5 years old. The eye findings are this condition is the failure during development of the
progressive, with vitreous and chorioretinal degeneration primary growth cartilages of the limbs and the cranial base.
and retinal detachment observed in 70% of patients by This results in short arms and legs as well as a characteristic
the time they are 20 years old. Additional eye findings midface deficiency or deformity that is most visually notable
include astigmatism, cataracts, strabismus, and glaucoma. at the nasofrontal process.
Individuals with Stickler syndrome may manifest any or Most affected individuals do well but attain a final height
all of a range of craniofacial findings, including mild to of only approximately 4feet. The condition is known to be
moderate midface hypoplasia, shallow orbits with eye pro- caused by mutations on the FGFR3 (fibroblast growth
ptosis, epicanthal folds, a flat nasal bridge, and submucous factor receptor 3) gene.22 Eighty percent of reported cases
or full clefting of the secondary palate. Some affected indi- are sporadic, whereas 20% are familial. Although craniosyn-
viduals will have progressive degeneration of the dental ostosis is not a typical feature, three cases with craniosyn-
roots and the associated periodontium. This is primarily ostosis have been reported.
due to the inborn error in collagen production, but it may
be exacerbated by malocclusion (secondary trauma) and
Premature Suture Closure of the Cranial Vault
orthodontic manipulations (see Fig. 4-18). Progressive
and Skull Base
neurosensory high-tone hearing loss has been reported in
80% of patients. Some patients have a Marfanoid body The flat bones of the cranial vault develop from mesenchy-
habitus, whereas others have short stature. There may be mal condensations over the developing brain (see Fig. 4-1).
progressive early joint degeneration by the mid-adult years. These bones grow primarily via the apposition of bone at
Any newborn who is found to have Robin sequence should their edges. The regions in which the cranial bony edges
undergo an initial genetic and ophthalmologic assessment collide are called sutures. In addition to appositional growth
to rule out Stickler syndrome. at the edges (i.e., suture growth), cranial vault development
also occurs through the process of remodeling (i.e., resorp-
Robin Sequence tion and deposition).17 Continued separation of the sutures
Robin sequence is commonly defined as cleft palate, micro- during the process of the apposition of bone at the edges is
gnathia, and glossoptosis (see Fig. 4-19). This sequence of also important to the normal growth of the bones of the
events can occur as a result of a variety of etiologic and cranial base and the midface.
pathogenetic conditions, and it involves a wide spectrum of Synostosis is a condition of premature fusion (i.e., the
phenotypes. The distinction must be made between a mal- arrest of appositional growth at the bone edges) that occurs
formed mandible (micrognathia) and a deformed mandible before the associated visceral structures (e.g., brain, eyes)
that is simply retrognathic. The malformed mandible (i.e., complete growth.17 When this happens, there is distortion
or deformity of the shape of the affected bones and possible Figures 4-20, 4-21, and 4-22). However, prematurely fused
compression of the underlying visceral structures (i.e., the cranial vault sutures that extend into the cranial base will
brain). Premature closure of the cranial vault sutures (cra- also affect midfacial growth and development (e.g., Crouzon,
niosynostosis) that do not extend into the cranial base will Apert, and Pfeiffer syndrome; Figures 4-23 through 4-26;
have minimal effects on the maxillofacial form (e.g., see Chapter 30).
metopic, unilateral coronal, and sagittal suture synostosis; Text continued on p. 102
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Figure 4-20 A child born with metopic synostosis that resulted in trigonocephaly. He underwent anterior cranial vault and three-quarter orbital
osteotomies with reshaping when he was 10 months old. A, Facial and computed tomography scan views before surgery. B, Illustrations of the
craniofacial skeleton in a child with metopic synostosis that resulted in trigonocephaly before and after anterior cranial vault and three-quarter orbital
osteotomies. C, Birds-eye view of removed orbital osteotomy unit before and after reshaping. Part B modified from an original illustration by
Bill Winn.
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Figure 4-20, contd D, Frontal views before and 1 year after

reconstruction. E, Facial views of patient at 5 years old. F and
G G, Computed tomography scan views before and after
reconstruction.
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Figure 4-21 A child born with right unilateral coronal synostosis that resulted in
anterior plagiocephaly. He underwent anterior cranial vault and three-quarter orbital
osteotomies with reshaping when he was 9 months old. A, Facial and computed
tomography scan views before reconstruction. B, Illustrations of the craniofacial
skeleton in a child with unilateral coronal synostosis that resulted in anterior pla-
giocephaly before and after anterior cranial vault and three-quarter orbital osteoto-
mies. Part B modified from an original illustration by Bill Winn.
Orthognathic Surgery; J.C

C. Posnick Orthognathic Surgery; J.C. Posnick
Orrthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

C. Posnick
Figure 4-21, contd C, Birds-eye view of removed orbital osteotomy units before and after reshaping. D, Frontal views
before and 5 years after reconstruction. E, Oblique views 5 years after reconstruction. Continued
Figure 4-21, contd F and G, Computed tomography scan views of the cranial vault before and just after reconstruction.
Figure 4-22 A child born with sagittal synostosis that resulted in a scaphocephalic shape of the cranial vault that was characterized by
increased anteroposterior length and decreased bitemporal and biparietal width. It was not until the child was 2 years old that he was diagnosed
with sagittal synostosis. After a comprehensive evaluation, he underwent total cranial vault reshaping through a coronal (scalp) incision. He is
shown before and after a single-stage reconstruction. A, Frontal facial views before and after reconstruction. B, Profile facial views before and
after reconstruction. Continued
C1
C2
Figure 4-22, contd C, Illustrations of the craniofacial skeleton in a child with sagittal synostosis that resulted in scapho-
cephaly before and after total cranial vault and upper orbital osteotomies with reconstruction. Part C modified from an original
illustration by Bill Winn.
Orthognathic Surrggery; J.C

C. Posnick
Figure 4-23 A 6-month-old girl who was born with Apert syndrome and bilateral coronal synostosis. A, Facial and computed tomography
scan views without intervention. B, Profile and computed tomography scan views without intervention.
Figure 4-24 A 28-year-old woman with uncorrected Apert syndrome. She was unable to undergo craniofacial
or extremity reconstruction in her home country. Facial and extremity views are shown.
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Figure 4-25 A 50-year-old man who was born with Crouzon syn-
drome. His dysmorphic features include oxycephaly, orbital dystopia
with eye proptosis, and midface deficiency with an Angle Class III
malocclusion. Despite these difficulties, he is married and employed
as a taxicab driver; he also has a son with Crouzon syndrome.
Figure 4-26 A 2-month-old child who was born with Pfeiffer syndrome (type I). She has bilateral coronal
synostosis that resulted in brachycephaly but without the suggestion of midface deficiency. A, Profile facial and
computed tomography (CT) scan views before intervention. B, Oblique facial and CT scan views before interven-
tion. C, Frontal facial and CT scan views before intervention. Continued
Figure 4-26, contd D, Additional CT scan views before intervention.
Overgrowth Syndromes and Anomalies

mandibular prognathism that resembles the human
Acromegaly is caused by an anterior pituitary tumor that variant.82,117,119 Harris and Johnson examined the longitu-
secretes excessive growth hormone after the normal com- dinal records from the Bolton Brush Growth Study and
pletion of growth.21 The response of specific growth centers concluded that the heritability of maxillomandibular char-
to abnormal serum levels of growth hormone is the overde- acteristics is relatively high48 (Figures 4-28, 4-29, and 4-30).
velopment of the hands, the feet, the supraorbital ridges, It is assumed that approximately one third of children who
and the mandible. When there is excessive mandibular present with a severe Class III malocclusion will have a
growth, the position of the teeth within the bone and the parent with the same problem and that one sixth of these
occlusion are also effected. An enlarged sella turcica (i.e., children will have an affected sibling.73 Interestingly, a short
the resorption of bone in response to pituitary tumor face growth pattern with a deep-bite malocclusion is found
expansion) can be seen on a lateral cephalometric radio- more frequently in whites (see Chapter 23). Bimaxillary
graph or a computed tomography scan (Fig. 4-27). Excess dento-alveolar protrusion with anterior open-bite malocclu-
growth hormone causes the proliferation of the condylar sion is more prevalent among blacks (see Chapter 24).112
cartilage of the mandible, thereby resulting in the bilateral The Class III skeletal pattern of maxillary deficiency
symmetric forward projection of the jaw. This overgrowth with relative mandibular excess is known to be more fre-
continues until the serum growth hormone levels return to quent in certain races.2,73,84,123,139 Incidence rates of Class III
normal. Unfortunately, the resultant skeletal deformities malocclusion of as high as 13% have been reported in spe-
persist. Other examples of maxillomandibular overgrowth cific regions of Asia.136 Lew and colleagues examined occlu-
include hemifacial hypertrophy (see Chapter 31), hemi- sal parameters in 1050 Chinese school children between the
mandibular hyperplasia, and hemimandibular elongation ages of 12 and 14 years. They found an incidence of 12.6%
(see Chapter 22). for Class III malocclusion, 58.8% for Class I malocclusion,
and 21.5% for Class II malocclusion.67 Samman and col-
leagues investigated 300 Chinese individuals residing in
Hereditary Tendencies in the Hong Kong who were known to have a dentofacial defor-
Development of Dentofacial mity and who had been referred to an orthognathic surgery
Deformities clinic. After excluding those individuals with cleft lip and
palate, 222 study patients remained. Forty-seven percent of
Jaw deformities are thought to be more common these individuals (n = 104) were found to have a Class III
among individuals with genetic backgrounds that are skeletal growth pattern.106
crosses between different racial and ethnic groups as Several of the common maxillomandibular disharmonies
compared with those from isolated human popula- are known to result from environmental influences (see
tions.10,32,40,57,59,60,62,83,85,86,91,93,108 It has been documented Chapters 10 and 21). The dental position in each arch and
that crossing different breeds of dogs and other mammals the resulting occlusal variations are felt to have an even
can result in striking dentofacial deformities, particularly greater environmental component.3,11,29,41,64,92 For example,
Orrtth
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Figure 4-27 A middle-aged woman who presented for the orthodontic evaluation of a progressive malocclusion. She had a
normal occlusion, average facial features, and no distortions of the extremities earlier during life. Gradually, over the prior 5 years,
malocclusion and changes in facial and extremity morphology had occurred. At presentation to the orthodontist, standard records
were taken (lateral cephalometric and Panorex radiographs and dental models). The patient was then seen for surgical evaluation.
A review of the lateral cephalometric radiograph showed an enlarged sella turcica. An endocrinology consult confirmed the
diagnosis of acromegaly. Appropriate treatment was undertaken without further worsening of the facial or extremity deformities.
A, Facial and occlusal views at presentation indicate growth hormone changes. B, Profile and lateral cephalometric views at
presentation. Note the enlarged sella turcica. C, A view of the dorsal aspects of hands indicates growth hormone changes.
Orrtthhoggnnaatthic
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Figure 4-28 Biologic identical twin sisters are shown during their teenage years to confirm a hereditary tendency toward the
occurrence of developmental jaw deformities with malocclusion. A, Facial and occlusal views of Twin A demonstrating bimaxillary
dental protrusion with jaw disharmony and anterior open-bite malocclusion. B, Facial and occlusal views of Twin B demonstrating
bimaxillary dental protrusion with jaw disharmony and anterior open-bite malocclusion.
dento-alveolar morphology is commonly affected by thumb such as the sternocleidomastoid (i.e., torticollis) may result
sucking and other para-oral habits (i.e., environmental in the deformation of the growing bones (i.e., cranial vault,
influences; Figures 4-31 and 4-32). The more severe the orbit, and zygoma; Fig. 4-34).71
dentofacial deformity, the more likely that inheritance plays
at least a partial role. Maxillary deficiency with relative
Muscle and Soft-Tissue Effects on
mandibular excess (i.e., a Class III skeletal pattern) and
Skeletal Growth
maxillomandibular deficiency (i.e., the short face growth
pattern) are both thought to have a significant hereditary Experimental animal research and clinical observational
component.31,36,58,79,116,120,137 studies confirm that the influences of the mandibular rest
posture, the neck rest posture, and the tongue and lip rest posi-
tion of the affected individuals eventual facial morphology
Environmental and Neuromotor Effects are real and more important than the effects of intermittent
on Skeletal Growth muscle contracture (see Chapters 8 and 10).128-130 It is also
known that functional stresses that are placed on the skel-
The mandible can be viewed as a somewhat malleable block eton will increase bone density.113,114,118 Acorollary is that,
of hard tissue that is adjacent to the skull base. It is separated during periods of inactivity (e.g., mandibular and neck rest
from the skull by working temporomandibular joints. It posture), skeletal demineralization occurs.
houses the inferior alveolar neurovascular bundle, and it Erupting teeth carry alveolar bone with them. Therefore,
provides a matrix for the formation, eruption, and eventual forces that counteract the normal eruption of teeth can
alignment of the teeth. Muscular activity in the head and shape the dental arches and the alveolar processes of both
neck can directly affect skeletal growth through one of the the maxilla and the mandible.5,101-105,107,109,111,126,140 Experi-
following mechanisms: 1) hyperactivity of muscles at the mental studies confirm that pressure against the active erup-
point of attachment may cause excessive skeletal remodeling tion of the teeth that is maintained for 4 to 6 hours can
or contour deformities (as discussed later in this chapter) affect tooth movement and initiate bone remodeling (see
2) hypoactivity of the masticatory muscles may cause distor- Chapter 5). Habits such as finger or thumb sucking, which
tions of the normal skeletal growth (i.e., long face growth apply consistent pressure against the teeth, can have the
pattern; Fig. 4-33) and 3) hyperactivity of specific muscles Text continued on p. 113
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Figure 4-29 A father and his biologic daughters are shown during the daughters teenage years to confirm a hereditary ten-
dency toward the occurrence of developmental jaw deformities with malocclusion. A, Facial and occlusal views of the father that
demonstrate a Class III skeletal pattern. Continued
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Figure 4-29, contd B, Daughter A with a similar class III skeletal pattern. C, Daughter B with a similar Class III skeletal pattern.
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Figure 4-30 Biologic twin sisters are shown during their teenage years to confirm a hereditary tendency toward the occurrence
of developmental jaw deformities with malocclusion. A, Facial and occlusal views of Twin A demonstrating a long face Class III
growth pattern. B, Facial and occlusal views of Twin B demonstrating a similar long face Class III growth pattern.
Orth
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Figure 4-31 A 10-year-old girl with a severe anterior open bite. She demonstrates how a para-oral
habit (thumb sucking) can exert the long duration of light force at the periodontal ligament that is
required to move the teeth. This patient was a consistent and constant thumb sucker from soon
after her birth and throughout the mixed dentition.
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Figure 4-32 A 12-year-old girl was referred by an orthodontist for surgical evaluation. She had persistently sucked her
thumb since infancy. This parafunctional habit resulted in the severe deformity of her maxilla and mandible. The upper jaw
has a constricted arch width and an excessive curve of Spee, whereas the mandible has a Class III reversed curve of Spee.
A severe anterior open-bite malocclusion is also present.
Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

C. Posnick
Figure 4-33 A teenage girl with a lifelong history of obstructed nasal breathing demonstrates the resulting long face growth pattern. The nasal
obstruction resulted in forced mouth breathing with an open-mouth posture. This in turn is associated with maxillary dental hypereruption (i.e.,
hypereruption of the molars greater than the incisors), a steep mandibular plane, anterior open-bite Class II malocclusion, and increased lower
anterior facial height. Clinically, there is a gummy smile, lip incompetence, and a flat, long face. A, Frontal views with smile before and after
reconstruction. B, Profile views before and after reconstruction. Continued
Pre surgery
C
After treatment
Figure 4-33, contd C, Occlusal views before retreatment, with orthodontic decompensation in progress, and after the completion
of treatment.
Maxillary counter-
clockwise rotation
Arch expansion
Mandibular counter-
clockwise rotation
Figure 4-33, contd D, Articulated dental casts that indicate analytic model planning. E, Cephalometric radiographs before and after surgery.
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Figure 4-34 A child with right-sided congenital muscular torticollis and C1-C2 rotary subluxation. Ipsilateral right fronto-orbital flat-
tening and contralateral left occipitoparietal flattening were noted. The right sternocleidomastoid muscle (SCM) remained tight when the
patient was 2 years old. He underwent surgical release of the SCM, and this was followed by neck range-of-motion exercises with
good maintenance of function. A degree of right fronto-orbital flattening (skull molding) remains. A, Frontal view with the neck in a relaxed
position before treatment indicated a head tilt. Computed tomography scan views indicate fronto-orbitozygomatic asymmetry as a result
of skull molding. B, Intraoperative view of the tight SCM and the proposed incision site, and a close-up view of the exposed SCM
before release. C, Frontal views before and 2 years after neck muscle release. Residual fronto-orbital (skull molding) flattening remains.
From Slate RK, Posnick JC, Armstrong DC, etal: Cervical spine subluxation associated with congenital muscular torticollis and cranio-
facial asymmetry. Plast Reconstr Surg 91:11871195, 1993.
same effect on the shaping of the dental arches (see Figs. tongue is juxtaposed with intrinsic weaker bilabial (i.e.,
4-31 and 4-32). It seems as though the intensity (i.e., the upper and lower lip) muscle tone forces.135 This results in
amount of force) against the teeth is less important than the the predictable dental compensations (i.e., proclined maxil-
duration (i.e., the number of hours per day) with regard to lary and mandibular incisors) observed in patients with
causing this effect. bimaxillary dento-alveolar protrusion (see Chapter 24). This
It has been confirmed that the postural position of the also explains the consistent dental compensations that are
tongue at rest (e.g., the mandible open with the constant typically observed in patients with specific developmental
protrusion of the tongue) rather than where the tongue is dentofacial deformities such as long face growth pattern (see
placed during function (e.g., a tongue-thrust swallowing Chapter 21 and Fig. 4-33) or isolated mandibular defi-
habit) is most important for determining the eventual posi- ciency (see Chapter 19).122
tion of the teeth and the morphology of the dento-alveolar Severe scar contracture of the anterior neck and lower lip
complex.61,121 The intermittent forces that are generated soft tissues from a burn that occurred during childhood will
against the teeth during chewing, swallowing, and speaking also affect mandibular morphology. The soft-tissue contrac-
are documented to be more than heavy enough to produce ture will distort ongoing skeletal growth and cause the
tooth movement. However, during these normal functions, mandible to be retrusive, with an obtuse angle. The chin
the force is not maintained long enough at any point in time region of the mandible will grow with increased vertical
to have an appreciable effect on the position of the teeth or length and without sagittal prominence, and there will be
the morphology of the bone.35 Even repetitive intermittent an anterior open-bite malocclusion. This deformity and its
abnormal tongue and lip contraction with resulting pres- surgical correction were first described by Simon Hullihen
sure on the teeth during swallowing and speaking (i.e., in 1849 (see Chapter 2 and Fig. 2-19).
tongue thrusting)despite their frequencydo not sum up When an electric burn to an oral commissure occurs during
to the number of hours of force required to produce a patho- childhood and results in soft-tissue contracture with micro-
logic effect. By contrast, even very light forces sustained for somia, the constant soft-tissue forces against the teeth will
a long duration (i.e., the tonic contracture of masticatory distort the shape of the dental arch and the alveolar process.
muscles to maintain mandibular posture) can affect both the There will be anterior dental crowding, tipping of the inci-
path of tooth eruption and jaw development.8,94 sors, and malocclusion.
It is known that muscles of different size and force that With the congenital absence of part or most of the tongue,
attach to the mandible at specific locations will result in there will be a characteristic V-shaped deficiency or dysmor-
characteristic ridges and contours (e.g., gonial angles, coro- phology of the mandibular dental arch (e.g., hypoglossia
noid process, genial tubercle).12,13 For patients with the hypodactylia syndrome; Fig. 4-35).15 In the presence of a
condition known as masseteric muscle hypertrophy, there is volumetrically enlarged tongue (e.g., vascular malforma-
excess bone proliferation at the masseter muscles point tion, hemangioma, neurofibromatosis, Proteus syndrome,
of attachment along the inferior border of the mandible (i.e., Beckwith-Wiedemann syndrome), there will be dento-
the gonial angle ridge) in response to greater than average alveolar effects that result in spaces between the teeth (Fig.
muscle forces.43 By contrast, in patients with muscular dys- 4-36). The lower anterior teeth will be flared, and the overall
trophies (i.e., hypotonic cerebral palsy, Moebius syndrome mandibular arch form will be enlarged. The important
with cranial nerve palsy), the ramus may be short, and the factor that causes these dental compensations and arch form
gonial angles appear to be underdeveloped as a result of the deformities is the sustained and constant rest forces of the
limited muscle forces applied to these same regions.100 oversized tongue on the teeth and bone and not what the
The lips, cheeks, and tongue contain muscles in their tongue does when it is in motion.
deep layers that exert varied pressures against the teeth and In the individual with a normal tongue volume and
the alveolar process. They normally do so with a constant neuromotor capability, it is known that the rest position of
level of tone while at rest as well as during dynamic func- the tongue in the mouth is primarily influenced by respira-
tion. These light (tone) forces applied for a long duration tory factors.73 It is the rest position of the tongue that influ-
are known to cause tooth movement and remodeling of the ences the pattern of dentofacial development rather than
alveolar process. It is the resting postural pressures of these the position of the tongue during function.
structures (i.e., lips, cheeks, tongue, and muscles), the pres- In the presence of chronic nasal obstruction, a typical long
ence of para-oral habits (i.e., thumb sucking, pencil biting; face growth pattern with anterior open-bite malocclusion is
see Figs. 4-31 and 4-32), and the man-made and controlled likely to occur. In these circumstances, a speech therapist
orthodontic pressure that can exert the long duration of may have success with teaching the individual altered move-
light force at the periodontal ligament that is required to ment of the tongue for the formation of more accurate
move teeth.55 When the difference between applied ortho voluntary independent speech sounds.90 However, unless
dontic forces and pressures of the tongue, cheek, and lips the anterior open bite and the nasal obstruction are cor-
that counteract those forces applied to the periodontal liga- rected, the myofunctional therapy will not allow the indi-
ment becomes great enough for sustained periods of time, vidual to reliably position his or her tongue, lips, teeth, and
tooth movement will occur. Another example of dental mandible for correct articulation during rapid conversa-
adaptation is seen when the constant strong force of the tional speech (see Chapter 8).
Orrth
O thogn
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ath Surgery
ry; J.C.
C Poosssnnicck
Palatal view Facial-occlusal view Lingual view
Figure 4-35 A 10-year-old girl who was eventually diagnosed with hypoglossiahypodactylia syndrome. In this photo, she dem-
onstrates how the congenital absence of part or most of the tongue will result in a characteristic V-shaped deficiency and dysmor-
phology of the mandibular dental arch and how this will negatively affect the maxillary arch form.
Elsevier Inc.
Orth
Orthoggnnaatthicc Su
Surgery
ry;; J.C
C.. Poosssnniicck
Figure 4-36 A 3-year-old child with a congenitally enlarged tongue is shown. The secondary
effects on the oral cavity include a Class III anterior open-bite malocclusion and a lateral cross-
bite malocclusion.
In the skeletal Class III adult patient (i.e., a retrusive Clinical observation confirms that, when an individual
maxilla with relative mandibular excess) who undergoes with a longstanding dentofacial deformity undergoes suc-
successful orthognathic correction, a normal oral cavity cessful reconstruction, it is generally not necessary to retrain
volume and a normal occlusion are established. Interest- the tongue with active myofunctional therapy. This only
ingly, in most cases, the tongue position and lip posture will needs to occur under the following conditions: 1) when
then naturally adapt to the new morphology.80,81 the tongue volume is truly excessive (e.g., vascular
malformation, neurofibromatosis) 2) when there is the pres- apposition on the condyles in association with the forced
ence of intrinsic neuromotor dysfunction (e.g., cerebral oral respiratory pattern. The apposition of new bone on the
palsy) or 3) when the nasal airway remains obstructed and condyles continued (in the usual way) independently of
a failure of tongue adaptation is seen.63 aberrant mandibular positioning or open-mouth posture.107
In review, the growing experimental animals studied by
Harvold coped with nasal obstruction by changing neck,
Respiration Patterns and Their Effects on
tongue, facial, and masticatory muscle tone, thereby result-
Skeletal Growth
ing in alterations in mandibular posture and tongue posi-
Harvold conducted primate experiments that were designed tion.44,45,55 The animals typically did this in one of two
to test hypotheses regarding the relationship between nasal different ways but always with the goal of maintaining
obstruction, mouth breathing, mandibular rest posture, tongue adequate respiration. This resulted in varied but consistent
rest position, jaw growth, and dental malocclusion.49-54 He secondary changes in the maxillomandibular morphology
created an experimental model in growing rhesus monkeys (i.e., developmental jaw deformity) and dental compensa-
(Macaca mulatta) to study these relationships. To do this, he tions (i.e., malocclusion). The measured lower facial height
completely obstructed the nasal passages in the study sub- increase that occurred in the presence of a constant open-
jects with silicone nose plugs. The experiments showed that mouth posture was primarily the result of maxillary molar
the monkeys adapt to total nasal obstruction in two different and, to a lesser extent, incisor dentalhypereruption (e.g.,
ways. Predominantly, the experimental animals maintained long face growth pattern).
an open-mouth posture with tongue protrusion. Interest- Harvold also reviewed the human observation studies of
ingly, some adapted to a forced oral airway by holding the Linder-Aronson and colleagues and found that they paral-
upper and lower teeth close together but with wide lip sepa- leled his experimental animal research.68-70,75-78 Their clinical
ration. Nevertheless, both adaptive mandibular, tongue, and research established that nasal obstruction with forced mouth
lip positioning patterns gradually resulted in maxilloman- breathing (i.e., open-mouth posture) in humans is fre-
dibular dysmorphology and dental malocclusion that was quently associated with maxillary dental extrusion, a steeper
notably different from that seen in the control animals. than normal mandibular plane, anterior open-bite maloc-
In the experimental animals, morphologic changes that clusion (i.e., the hypereruption of the molars is greater than
occurred in the lips, tongue, dentition, jaws, and mandibu- that of the incisors), and increased lower anterior facial
lar posture were documented by the research team through height. Similar to Harvolds experimental monkeys, growing
cephalometric measurements as well as through electromyo- humans may develop different neuromotor adaptations
graphic and behavioral findings. Harvolds cephalometric to cope with nasal obstruction and the need to maintain
findings documented changes in facial height (e.g., of the adequate respiration. The secondary skeletal and dental
naso-palatal plane and the symphysis-palatal plane) and morphologic deviations will therefore vary, but they tend
alterations in mandibular morphology. to occur in a predictable long face growth pattern (see
As stated, a minority of animals adapted to the nasal Chapter 21).39,125,133,134
airway blockage by holding their teeth together with wide Humans with severe hypoplasia of the maxilla, a retropo-
lip separation (rather than maintaining an open-mouth sitioned palate and a restricted nasal airway, as are seen in
posture with tongue protrusion) while still breathing patients with Crouzon or Apert syndrome, also become
through their mouths. This subgroup showed the least dependent on oral respiration (see Chapter 30).4 With
change in jaw morphology and dental alignment. Crouzon or Apert syndrome, the individuals neck is often
The observed morphologic and occlusal changes were maintained in hyperextension with an open-mouth posture,
most extreme when the mandible constantly remained the protrusion of the tongue, and the clockwise rotation of
open, with tongue protrusion (i.e., an open-mouth posture). the mandible; this head and neck posture is compensatory
The adaptive lowering of the mandible (with clockwise rota- to support respiration. During the day, this compensatory
tion) to achieve effective oral respiration was followed by posture may be sufficient, but it becomes inadequate at night
secondary changes in the maxilla, including a downward and results in obstructive sleep apnea (see Chapter 26).4
displacement (i.e., vertical lengthening) of the alveolar When these compensatory adaptive changes occur during
process in conjunction with excess extrusion of the teeth. childhood, they contribute to additional secondary maxil-
When molar (posterior) hypereruption was significantly lomandibular skeletal dysmorphology with growth that
greater than incisor hypereruption, an anterior open-bite further affects the occlusion (see Chapter 30).7,33,115 This is
malocclusion developed (Fig. 4-37).127 an example of a congenital midface deformity that is further
It became apparent to Harvold that the changes in man- influenced by the environment.
dibular shape and the direction of mandibular growth (i.e.,
clockwise rotation) observed in a majority of the experimen-
Congenital Myopathies and Effects on
tal monkeys were dependent on the adaptive activity of the
Skeletal Growth
facial, tongue, jaw, and neck muscles in response to respira-
tory need. Interestingly, in Harvolds experimental animal Individuals with congenital myopathies constitute
model, there was no indication of abnormal bony a heterogeneous subgroup of patients with congenital
Orthognathic Surgery; J.C. Posnickk Orrthognathic Surgery; J.C. Posnick
Figure 4-37 A 16-year-old with a nonprogressive congenital myopathy that resulted in a constant open-mouth posture. He developed a long
face growth pattern that was characterized by severe maxillary vertical excess and protrusion, a retrusive mandible, and an Angle Class II maloc-
clusion. He underwent a combined orthodontic and surgical approach. Orthodontic manipulation was limited as a result of hygiene control. The
surgery included a maxillary Le Fort I osteotomy (vertical intrusion, first bicuspid extractions, and alveolar bone removal with premaxillary setback
and cant correction) and sagittal split osteotomies of the mandible (clockwise rotation and asymmetric correction). A, Frontal views with smile
before and after reconstruction. B, Oblique facial views before and after reconstruction. C, Occlusal views before and after reconstruction.
Figure 4-37, contd D and E, Articulated dental casts that indicates analytic model planning.
F, Lateral cephalometric radiographs before and after reconstruction.
neuromuscular disorders in which the pathology is attribut- 4-20). Unless upper airway obstruction with obstructive
able to defects in the muscle fibers rather than in the sleep apnea is confirmed, the degree of maxillomandibular
neurons. Congenital fiber-type disproportions are rare con- deficiency is such that a surgical consult is generally not
genital myopathies that are considered slow or nonprogres- requested.
sive. Patients with these conditions, like those with other An infant, child, or young adolescent who sustains injury
types of primary myopathies, will generally exhibit facial to the craniofacial skeleton has the potential for growth dis-
features that often result in what is described as a myo- turbance of the injured bones. This is in addition to any
pathic face. The myopathic face has many similarities to skeletal deformity that remains if the fracture was not fully
the adenoid face that is seen in children with a blocked reduced with satisfactory initial healing (see Chapter 35).
nasal airway due to enlarged adenoids and other intranasal During some difficult deliveries through the vaginal canal,
obstructions, which result in an open-mouth posture. These especially those that require forceps, damage to one or both
abnormal human maxillomandibular growth patterns are of the temporomandibular joints or fracture of the man-
also consistent with the findings reported by Harvold when dible can occur. In modern times, with good prenatal care
rhesus monkeys were forced to maintain an open-mouth and skilled obstetrics support being available at the time
posture during jaw growth, as described previously in this of delivery, birth trauma to the mandible has become a
chapter. The myopathic face findings described by rare event.
Lehman and colleagues include characteristics of an A childhood injury to the cartilage of the nasal septum
increased lower anterior facial height, incompetent lips, a may result in nasal deformity. It may also lead to deficient
high-arched palate, and often anterior open-bite malocclu- midface growth (see Fig. 35-2).38 This has been confirmed
sion (Figs. 4-37 and 4-38).66 Clinical human and animal in experimental animals and documented through clinical
model studies confirm that these features are to be expected observation in humans.28 In experimental animals, when
in the presence of an open-mouth posture during jaw the cartilage of the nasal septum is removed before growth
growth, no matter what the cause. Although blocked nasal is complete, there will be a dramatic decrease in the hori-
breathing is the most common cause of an open-mouth zontal and vertical growth of the midface. Whether this is
posture during growth, clearly congenital myopathies that the result of a mechanical collapse with a secondary loss of
also result in an open-mouth posture will produce the same growth velocity of the maxilla or an intrinsic growth restric-
facial dysmorphology. tion of the upper jaw remains open to question.
Trauma to the growing mandible that is severe enough to
Effects of Trauma on Skeletal Growth cause fracture and the displacement of the fragments is also
known to result in secondary progressive growth deformi-
Intrauterine compressive force against the developing face is ties, at least in some cases. There are thought to be three
a form of trauma that can lead to deformations of the cra- major sites of mandibular growth that may be affected:
niofacial skeleton that are visually present at the time of
birth. For example, when there is a decrease in the volume 1. The mandibular condyle: Proliferating cartilage at the
of amniotic fluid, the fetus head is typically flexed tightly base of the fibrocartilage layer that covers the articular
against his or her chest in utero. The compressive forces to surface results in mandibular growth. At the completion
the chin will prevent the mandible from achieving its full of mandibular growth, the fibrocartilage layer is replaced
prenatal growth potential. The normally developing tongue by bone.
is then forced to rest high in the mouth, thereby preventing 2. The outer surfaces of the ramus: Extensive remodeling
approximation and fusion of the lateral maxillary processes. of the mandible through the apposition of bone on the
At the time of birth, clefting of the secondary palate is posterior and outer surfaces along with resorption on the
recognized. A pathologic process continues after birth, and anterior and inner surfaces is known to occur and is
this includes retrognathia, a cleft of the secondary palate, influenced by muscle forces and the compression of the
and varying degrees of respiratory distress as a result of the associated soft tissues (e.g., tongue volume) within the
otherwise normal tongue resting superior and posterior in mouth.
the mouth. This intrauterine deformation is responsible for 3. The alveolar process: New alveolar bone is added only as
approximately two thirds of those individuals with observed individual teeth develop and erupt into the arch.
Robin sequence at the time of birth. Significant but incom-
plete catch-up mandibular growth is expected. In general, In individuals who sustain a displaced condylar neck frac-
there will be sufficient mandibular growth in combination ture, a sequence of events generally occurs that includes the
with an expected degree of maxillary growth inhibition contraction of the lateral pterygoid muscle with the dis-
from cleft palate repair during childhood that results in placement of the condylar fragment anteriorly and medially
maxillomandibular harmony but at least mild horizontal (Fig. 4-39). The displaced fragment (i.e., the medial pole of
deficiency and the clockwise rotation of both jaws. Indi- the condyle or the total condyle) has a tendency toward a
viduals with these features are typically managed with degree of resorption and malunion. This may then result in
orthodontics to achieve an acceptable occlusion, but this a loss of posterior facial height, with a shift of the ipsilateral
does result in a degree of facial dysmorphology (see Fig. Text continued on p. 124

Ortthhogn
Or naatthic Su
S rgery;
ryy; J.C.
C. Possniicck Orrth
O thoggn
natthic Su
Surgeryy; J.C
C.. Pos
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ick

Orrtth
O hoggnnat
athhic Su
urgery
ry;; J.C. Poossnic
ick
ck Orrtth
O hogn
gnat
athicc Su
ath S rgeryy; J.C.
C. Pos
osnick
iicck
Figure 4-38 A 17-year-old boy who was born with a

Elsevier Inc. Elsevier Inc. nonprogressive congenital myopathy was referred by an
orthodontist for surgical evaluation. The lack of mastica-
Orrtthhogn
O gnaatthic
ic Su
Sur
urgery
ry;; J.C. Posnick Orth
Orthoggn
nath
athicc Su
at Surgeryy; J.C. Poossnic
Sur ick
tory muscle strength resulted in a constant open-mouth
posture. The long face growth pattern is characterized
by vertical maxillary excess, a clockwise rotated retrusive
mandible, a vertically long and retrusive chin, and an
anterior open-bite malocclusion. The patient underwent
a combined orthodontic and surgical approach. Four
bicuspid extractions relieved dental crowding. This
was followed by surgery that included a maxillary Le Fort
I osteotomy (vertical intrusion and horizontal advance-
ment); bilateral sagittal split osteotomies of the mandible
(horizontal advancement); and osseous genioplasty (ver-
tical reduction and horizontal advancement). A, Frontal
views in repose before and after reconstruction.
B, Frontal views with smile before and after reconstruc-
C
tion. C, Profile views before and after reconstruction.
Continued
Prior to treatment
D
After treatment including 4 bicuspid extractions
Figure 4-38, contd D, Occlusal views before and after reconstruction. E, Articulated dental casts that indicate analytic
model planning. F, Lateral cephalometric radiographs before and after surgery.
Displaced condyle fracture
Orrthhogn
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Surgery
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C. Pos
ossnnic
ick
Ortthhoggn
Or nat
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ic S
Suuurrgery
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C.. Possnicck
Figure 4-39 A 5-year-old girl sustained a right condyle fracture of the mandible and then presented as a teenager
with resulting facial asymmetry involving the maxilla and the mandible. She had full painless mandibular mobility.
She then underwent a combined orthodontic and orthognathic approach that included a Le Fort I osteotomy (cant
correction), bilateral sagittal split ramus osteotomies (asymmetry correction), an osseous genioplasty (horizontal
advancement), and intranasal procedures (septoplasty, inferior turbinate reduction, and nasal recontouring) during
her teenage years (see Chapter 35). A, Frontal view when the patient was 6 years old, which was 1 year after the
right condyle fracture. The anteroposterior facial radiograph indicates a medially displaced right condyle fracture.
B, Frontal view when the patient was 15 years old. An anteroposterior cephalometric radiograph confirms the result-
ing facial asymmetry that primarily involved the mandible but that now also involves the secondary deformity of the
maxilla and the chin. Continued
Roll Orientation Correction
Orth
Orthogn
gnat
athic
athic S
Suurgerryy; J.C.
C Poosssnnick
ick
ic Orrtthhogn
O gnat
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ic Su
Sur
urgery
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C.. Poossnic
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Figure 4-39, contd C, Articulated dental casts that indicate analytic model planning. D, Frontal views before and after
reconstruction.
Ortthhogn
Orth
Or g at
a hic
i Suurgerry
y; J.C
C.. Pos
osnic
ick Orrrth
O thogn
th gnaatthic
ic S
Su
urgerryy; J.C.
C Posnick
Figure 4-39, contd E, Profile views before and after reconstruction. F, Occlusal views before and after
reconstruction. G, Posteroanterior cephalometric radiographs before and after reconstruction.
mandible toward the affected side. In these cases, an asym- posterior facial height, ipsilateral mandibular hypoplasia,
metric retrusive lower jaw and predictable malocclusion can and secondary deformity of the contralateral side of the
be documented when the jaw is placed in centric relation. mandible, the maxilla, and the chin region. This sequence
Experimental and clinical studies confirm that the ability of of events results in significant maxillomandibular asymme-
the individuals mandible and masticatory muscles to adapt try and disproportion (see Fig. 35-8). There are also numer-
will determine the degree of eventual jaw deformity and ous reported examples of bony ankylosis of the condylar
residual malocclusion as well as what treatment is needed stump to the glenoid fossa after the fracture of the condyle
(see Chapter 35). (see Fig. 35-1).
When significant condylar trauma occurs during child-
hood, with or without fracture, there will have been injury
to the fibrocartilage, with a potential effect on growth. If in Effects of Tumors and Growths on
fact the condylar cartilagepush theory of mandibular Skeletal Growth
growth is all important, a devastating growth distortion
should occur over time in all affected individuals. Walker The presence of a sustained expansile mass (e.g., cyst, cellular
tested this theory by deliberately producing condylar frac- tumor, skeletal mass) will distort the normal growth and
tures in young monkeys and then observing the effect of morphology of the bones that are being compressed or exp
these fracture on subsequent growth.131,132 He was unable anded.1,6,9,20,24,26,34,46,87-89,95-99 Frequent examples of this
to document a consistent progressive mandibular deficiency occurrence in the maxillofacial region include the effects of
or deformity with malocclusion. Mandibular growth that neurofibromatosis, vascular malformations, Proteus syn-
approximated normal occurred in the majority of experi- drome, fibrous dysplasia, and odontogenic tumors and cysts
mental animals. Late postmortem gross and histologic (Fig. 4-40). In the adult, the compressive forces of a tumor
evaluation confirmed that there was regeneration of the mass may cause resorption and even result in pathologic
condylar process with a new layer of cartilage over the fracture. The tumor mass may also compress visceral struc-
articular surface that was identical to the contralateral (non- tures and cavities, thereby causing an impairment of func-
injured) side. Clinical studies on a series of human children tion. Depending on the tumors location, volume, and
who sustained similar condylar neck fractures confirmed the velocity of expansion, there may be alterations in cognitive
potential to regenerate bone that approximated the lost function, vision, speech, breathing, swallowing, chewing,
(injured) condyle adverse effects on growth in many of the lip control, hearing, neck range of motion, or mandibular
cases.42,74 It also became evident from these studies that a range of motion. When radiation therapy is required for the
notable minority (approximately 25%) of affected individu- management of a malignancy, damage to the adjacent soft
als do sustain significant growth disturbances (i.e., man- tissues and skeletal structures frequently occurs. The arrest
dibular hypoplasia or asymmetry) after a condylar injury, of growth in irradiated immature bones may result in orbital
often with secondary deformity in the growing maxilla (see dysplasia, zygomatic hypoplasia jaw deformity with maloc-
Fig. 4-39). The explanation for this variation in human clusion, and damage to the developing teeth and periodon-
response (i.e., repair versus deformity) is likely dependent tium (Fig. 4-41).
on the following: 1) the extent of initial injury; 2) the extent
of resulting scarring or the lack of regenerative potential; 3)
the variable importance of the condyle as a growth center Conclusions
in each individual child; and 4) the variable adaptive ability
of the muscles of mastication (see Chapter 35). The term dentofacial deformity is generally used to describe
Over the decades, clinicians have confirmed the work of a significant disproportion of the jaws that is associated with
Gilhuus-Moe 42 and Lund74 by documenting a spectrum of malocclusion. Knowing the etiologic factors of a jaw defor-
possible outcomes after condylar fracture. Reports confirm mity can be important to the fundamental understanding
instances in which the fracture dislocation of a condylar of how and when to treat the problem. According to current
process has occurred and is followed by a continued normal knowledge, factors that are known to cause or result in
downward and forward translation of the mandibular dentofacial deformities can be subdivided to include known
body with regeneration of a neocondyle back to baseline. syndromes and anomalies, hereditary tendencies, environ-
Other clinical reports show a complete loss of the condylar mental and neuromotor effects, effects of trauma, and
morphology without regeneration followed by decreased effects of tumors and growths.
Elsevier Inc.
Ortthhoggnnaatthic
Or ic Sur
Surgery
Su ry; J.C.
C. Pos
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Orrtthhogn
O gnat
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Surgeryy; J.C.
C. Pos
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ick Orrtthhoggnnaatthic
O ic S
Suurgerryy; J.C
C.. Posn
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os ck
Elsevier Inc. Elsevier Inc. Figure 4-40 A 5-year-old girl with poly-
ostotic fibrous dysplasia with massive
Orrth
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C.. Posn
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Surgery
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C. Poossn
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involvement of the maxilla and mandible
bilaterally. An asymmetric swelling of the
mandible (left greater than right) was
first recognized when the child was 18
months old; it rapidly enlarged, and maxil-
lary involvement became evident with
the deterioration of breathing, chewing,
speech, and swallowing. Pathologic frac-
tures were sustained in all four extremities
as a result of the involvement of fibrous
dysplasia. A, Frontal and computed
tomography scan views before surgery.
B, Oblique facial views before and after
reconstruction. C, Profile facial views
C
before and after reconstruction.
Continued
Figure 4-40, contd D and E, Computed tomography views before and after a debulking procedure. From
Posnick JC, Hughes CA, Milmoe G, etal: Polyostotic fibrous dysplasia: an unusual presentation in childhood. J Oral
Maxillofac Surg 54:14581464, 1996.
Orthhoggnnat
Or athhiicc Su
Surrgery
y; J.C
C.. Poossniicck
Orrtthhogn
gnat
athicc Su
Surgery
ry; J.C
C.. Poossnic
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Figure 4-41 A teenage girl who at 9 years of age was found to have a malignancy of the parameningeal region. Biopsy confirmed the
diagnosis of rhabdomyosarcoma. She was treated with surgical debulking, chemotherapy, and radiation according to intergroup protocols.
There was resulting radiation growth disturbance of the skeletal and soft-tissue structures. The patient underwent staged reconstruction,
including stage I serratus muscle microvascular transfer to the left temporal region in combination with full-thickness autogenous cranial
graft reconstruction of the left zygomatic orbital complex when she was 13 years old. This was followed when she was 16 years old by
further staged orthognathic reconstruction to manage the jaw deformity and the malocclusion. The orthodontic portion of her treatment was
carried out cautiously, because there was radiation injury to her left posterior maxillary and mandibular dentition. The procedures included
maxillary Le Fort I osteotomy in two segments with interpositional autogenous iliac (hip) grafting, bilateral sagittal split ramus osteotomies
of the mandible, and an osseous genioplasty. Septoplasty, inferior turbinate reduction, and recontouring of the floor of the nose were also
carried out to open the airway. A, Facial and occlusal views are shown when the patient was 12 years old, before reconstruction. B, Oblique
facial and computed tomography scan views when the patient was 12 years old, before reconstruction. Continued
Orbito-zygoma hypoplasia
Orthogn
Or gnat
nat
a hiicc Su
Surgery
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C.. Posn
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Maxillary
hypoplasia
Mandibular
C hypoplasia
Orrtthhoggn
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Surgery
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C.. Pos
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ic Orrth
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gnaatthic
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S rgery
y; J.C
C.. Poossniicck
Orrrth
O thoggnnat
th athiicc Su
ath S rgery
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C.. Poossniicck
Figure 4-41, contd C, Profile facial and computed tomography scan views when the patient was 12 years old, before
reconstruction. D, Before and 1 year after left zygomatic and orbital cranial graft reconstruction and serratus muscle
free-tissue transfer reconstruction. E, Frontal and occlusal views when the patient was 13 years old, with orthodontics
in progress in preparation for jaw reconstruction.
Maxillary counter-
clockwise rotation
Arch expansion
Mandibular counter-
clockwise rotation
G
Roll Orientation Correction
Orrthhoggnnathicc Suurgery
O ry
y; J.C
C. Pos
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Orthoggnnat
athhicc Sur
Suurgery
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C Pos
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icckk
Figure 4-41, contd F, Panorex radiograph when the patient was 13 years old, with preoperative orthodontics complete
in preparation for jaw reconstruction. Note the short dental roots on the left side. G, Articulated dental casts that indicate
analytic model planning. H, Frontal facial views in repose before and after reconstruction. Continued
Orrth
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ry;; J.C.
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C. Poossnic
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Orrtthhogn
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C.. Poossnick
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ic Orrth
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Surgery
ry; J.C
C.. Poossnic
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Orth
Or
rthhogn
gnatthiicc Su
Surgery
ry; J.C
C.. Poossnic
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Or
rthhogn
gnaatthic
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Suurgery
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C.. Poossnick
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ic
Figure 4-41, contd I, Frontal views

with smile before and after reconstruction.
J, Oblique facial views before and after
reconstruction. K, Profile facial views
K
before and after reconstruction.
L
After treatment
Figure 4-41, contd L, Occlusal views before and after orthodontics and orthognathic surgery. M, Lateral cephalometric
radiographs before and after reconstruction.
References
1. Adamides IP, Spyropoulos MN: The effects obstructive sleep apnea syndrome. Cleft 8. Brudevold F: A basic study of the chewing
of lymphadenoid hypertrophy on the Palate J 25:374378, 1988. forces of a denture wearer. J Am Dent Assoc
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4 Hereditary Developmental and Environmental Influences On The Formation of Dentofacial Deformities 2014 Orthognathic Surgery

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Hereditary, Developmental, and

Development of the Head and Neck

Membrane bones at 12 weeks Site of future anterior

Lacrimal bone Greater wing of sphenoid bone

Lateral view Anterior fontanelle Parietal bone

Occipital bone Lambdoid suture

Figure 4-1, contd

Lateral view at 5 to 6 weeks Ventral view at 5 to 6 weeks Frontonasal

Lateral view at 6 to 7 weeks Ventral view at 6 to 7 weeks

Ventral view at 7 to 8 weeks

Site of nasolacrimal groove (fusion of

Lateral view at 7 to 8 weeks Lateral view at 8 to 10 weeks

Figure 4-2, contd

Frontal (coronal) section at 7 to 8 weeks

Right olfactory bulb

Maxilla Nasal septum

Right lateral palatine process Tongue

Frontal (coronal) section at 8 to 10 weeks

Superior concha and meatus

Inferior concha and meatus Zygomatic bone

Muscles of facial expression

Roof of stomodeum (inferior view; 6 to 7 weeks)

Primitive or primary palate Nasolacrimal groove

Palate formation (inferior view; 7 to 8 weeks)

Philtrum of upper lip (fusion

Primary palate (median Primitive posterior naris or choana

Left lateral palatine process

Roof of oral cavity (inferior view; 8 to 10 weeks)

Medial palatine process Gingiva (gum)

Figure 4-3, contd

Figure 4-4 An 11-year-old boy who

Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnick

Elsevier Inc. Elsevier Inc.

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Elsevier Inc. Elsevier Inc.

Elsevier Inc. Elsevier Inc.

Elsevier Inc. Elsevier Inc.

Elsevier Inc. Elsevier Inc. Elsevier Inc.

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Elsevier Inc. Elsevier Inc.

Figure 4-20, contd D, Frontal views before and 1 year after

Orthognathic Surgery; J.C

Orrthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surrggery; J.C

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Orthognathic Surgery; J.C. Posnick

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Orthognathic Surgery; J.C. Posnick

Figure 4-26, contd D, Additional CT scan views before intervention.

Overgrowth Syndromes and Anomalies

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C

Palatal view Facial-occlusal view Lingual view

Orthognathic Surgery; J.C. Posnick Orthognathic Surgery; J.C. Posnick

Orthognathic Surgery; J.C. Posnickk Orrthognathic Surgery; J.C. Posnick