Pediatric Plastic and Reconstructive Surgery

9.4.1 Odontoma
The most common odontogenic tumor of the jaws is the odon-
toma. There are two types of odontomas: compound and com-
plex. The compound odontoma consists of multiple toothlike
structures surrounded by a radiolucent zone, whereas the com-
plex odontoma is a radiodense mass made up of enamel and
dentin without toothlike structures and is surrounded by a
radiolucent zone. The mean age of people found with odontoma
is 15 years, and odontomas are often asymptomatic and
found on routine dental radiographs. They may cause delayed
eruption of teeth. Clinically, they are typically well-circum-
scribed dense masses that can be treated with simple surgical
excision that must also include complete removal of associated
soft tissue, follicular tissue, or cyst. Prognosis is excellent, with-
out recurrence ( Fig. 9.6).
9.4.2 Cementoblastoma
Similar to odontomas in appearance, cementoblastoma tends to
appear radiographically in patients younger than 20 years as a
round radiopaque mass fused to the roots of vital teeth
Fig. 9.5 (a) Nasopalatine duct cyst seen as an expansile lesion of the incisive canal of the maxilla. (b) Coronal view of lesion.
Fig. 9.6 (a) Odontoma of the maxilla adjacent to an erupting canine and lateral incisor. (b) Specimen: Odontoma of the maxilla.
Craniofacial Disorders
84

(Fig. 9.7). This is a rare benign tumor of odontogenic origin
and is treated with surgical excision of the mass and the
a ected tooth or teeth, followed by curettage or peripheral
ostectomy. Cementoblastomas tend to recur if not completely
removed, with reported recurrence rates near 40%.
9.4.3 Keratocystic Odontogenic Tumor
The keratocystic odontogenic tumor is a benign intraosseous
cystic tumor of odontogenic origin, and it can be locally
destructive. Majority of the lesions occur in the posterior man-
dible, with bony expansion and tooth displacement. Depending
on the size and location, small lesion can be treated with enu-
cleation and peripheral ostectomy, whereas larger KCOTs
should be first decompressed, enabling the placement of a
stent, followed by daily irrigation for 9 months, which reduces
the lesion’s size and causes dedi erentiation of the residual epi-
thelium, thus enabling cystectomy. It has lower recurrence rates
of around 10% ( Fig. 9.8). This is useful in children with multi-
ple lesions and or lesions that are in close proximity to vital
structures such as the inferior alveolar nerve. Finally, marginal
or en bloc resection can be utilized for large, recurrent lesions.
Recurrence rates range between 10 and 55%, with enucleation
alone having the highest rate.
9.4.4 Nevoid Basal Cell Carcinoma
Syndrome
When multiple KCOTs are present, the child must be further
worked up for nevoid basal cell carcinoma syndrome (NBCCS),
also known as Gorlin–Goltz syndrome. Keratocystic odonto-
genic tumors occur in 75% of patients with NBCCS. Keratocystic
odontogenic tumors that occur as a part of this syndrome tend
to have a higher recurrence rate.
9.4.5 Adenomatoid Odontogenic Tumor
The adenomatoid odontogenic tumor is a benign cystic hamar-
toma that is known as the “two-thirds” tumor, because about
two thirds occur in maxilla, two thirds occur in young women
(preteenagers and teenagers), two thirds are associated with
impacted tooth, and two thirds of those teeth are canines.
They can become very large and radiographically appear as a
well-demarcated, unilocular radiolucent lesion usually associ-
ated with an impacted tooth. Treatment involves direct explo-
ration and enucleation, ensuring that the capsule is intact
upon removal. Recurrence is rare.
9.4.6 Ameloblastic Fibro-odontoma
Ameloblastic fibro-odontoma (AFO) is usually seen in teenagers
as an asymptomatic jaw expansion that may resorb adjacent
teeth. It has a mixed radiolucent–radiopaque presentation and is
radiographically similar to a developing odontoma (Fig. 9.9a).
Majority of these tumors are found in the posterior mandible
and are often associated with an unerupted tooth. Treatment
includes simple enucleation and curettage (Fig. 9.9b, c). Prog-
nosis is excellent, and recurrence is rare.
9.4.7 Ameloblastic Fibroma
Ameloblastic fibroma (AF) is usually seen between 6 and 15
years of age and usually presents as an asymptomatic expan-
sion of the jaw in the molar region of the mandible. It can be of
unilocular or multilocular radiolucency, occurs most commonly
in the mandible, and is associated with an impacted tooth
(Fig. 9.10). Because it may have a similar appearance to other
large destructive lesions, an incisional biopsy should be per-
formed in children as an AF is treated with simple enucleation
and curettage versus a resection for other lesions of similar
appearance. Although rare, in some cases, an AF can convert
into an ameloblastic fibrosarcoma. There is a significant higher
risk of malignant transformation and recurrence in patients
older than 20 years. As a result, resection is the treatment of
choice in patients older than 20 years, patients with massive
tumors, and those who have recurrent ameloblastic fibroma.
Ameloblastic fibrosarcoma is the most common malignant
odontogenic tumor and is capable of metastasis. It tends to
appear later than ameloblastic fibroma, usually in the 20 s.
Treatment involves resection by using 1.0 to 1.5 cm bony mar-
gins and one uninvolved anatomic carrier.
Ameloblastoma is the most common odontogenic tumor,
with about 50% of them occurring between 20 and 40 years
of age. In the pediatric population, ameloblastoma must be
Fig. 9.7 Cementoblastoma associated with root
of left mandibular first molar.
Osseous Lesions of the Craniofacial Region
85

considered in the di erential diagnosis of any unilocular or
multilocular radiolucency. Unicystic ameloblastoma represents
about 5% of all ameloblastomas and tends to occur in younger
age range (10–24 years) ( Fig. 9.11a, b). For all ameloblasto-
mas, 80% occur in the mandible and 75% in molar–ramus re-
gion. They are benign, locally aggressive, expansile neoplasms
that commonly cause dental changes such as mobility, tooth
displacement, and root resorption. Untreated tumors can prog-
ress to a tremendous size. Treatment involves resection with
1.0 to 1.5 cm bony margins and one uninvolved anatomical bar-
rier (Fig. 9.11c, d). Consider frozen sections of soft tissue mar-
gins as well as taking a radiograph of the specimen to ensure
that you have 1-cm margins past radiographic margins
(Fig. 9.11e). Cure rate with resection is about 98%. Contro-
versy exists around immediate reconstruction at the time of
resection versus a staged approach. If enucleation and curettage
are performed, there is a recurrence rate of more than 80%
(Fig. 9.12).
9.5 Nonodontogenic Tumors
9.5.1 Giant Cell Lesions
Giant cell lesions in children and young adults usually present
clinically as painless expansion of the anterior jaw, favoring
women over men and the mandible over the maxilla. They
present more often in the anterior mandible, with the canine–
premolar area being the most common site ( Fig. 9.13). Giant
cell lesions are classified as either nonaggressive or aggressive,
based on criteria from clinical behavior and radiographic
characteristics. It is critical to make the correct diagnosis as
the treatment, and recurrence rate is substantially di erent
between aggressive and nonaggressive lesions. For example, a
nonaggressive giant cell lesion can be treated successfully with
simple enucleation and curettage, which have low recurrence
rates, whereas if the lesion were aggressive, the same treatment
would result in a recurrence rate as high as 75%.
Fig. 9.8 (a) Large left mandibular KCOT associ-
ated with impacted third molar. (b,c) Stenting of
left mandibular KCOT.
Craniofacial Disorders
86

Aggressive giant cell lesions tend to have a high recurrence
rate and tend to occur in younger patients (mean age, 6 years).
In order to be classified as aggressive, the lesion must be larger
than 5 cm, be recurrent, or show three of the following six clin-
ical characteristics: (1) rapid growth, (2) tooth loosening, (3)
tooth displacement, (4) root resorption, (5) cortical thinning,
and (6) cortical perforation. Treatment options vary from intra-
lesional steroid injections and calcitonin injections to treatment
with interferon-α. For a biopsy-confirmed giant cell lesion
meeting the aggressive classification, the author recommends
enucleation and curettage of the tumor and use of interferon-
α-2 (3,000,000 units/m 2), starting 48 to 72 hours postopera-
tively. Patients are then followed by clinical examination and
radiography every 3 months, until bony cavity is completely
healed, and then after every 6 months. It is important to note
that patients may develop infections, flulike symptoms, and
other side e ects of interferon therapy, requiring close moni-
toring by an experienced team. In patients in whom adjuvant
therapy is not advisable, en bloc resection with 1.0 to 1.5 cm
margins and one anatomic barrier is appropriate.
Nonaggressive giant cell lesions, when compared with
aggressive lesions, tend to occur in slightly older children (mean
age, 9 years) and are smaller in size (less than 5 cm). Lesions
meeting the nonaggressive classification are appropriately
treated with enucleation and curettage, with close monitoring
to ensure bony fill. Bone grafts into the cavities are not typically
recommended as the intact periosteum facilitates regeneration
in young patients.
Fig. 9.9 (a) Ameloblastic fibro-odontoma of the anterior mandible, radiographically similar to an odontoma. (b) Intraoperative view of ameloblastic
fibro-odontoma of the anterior mandible. (c) Ameloblastic fibro-odontoma after being removed.
Osseous Lesions of the Craniofacial Region
87

9.5.2 Brown Tumor of
Hyperparathyroidism
The brown tumor of hyperparathyroidism is microscopically
identical to the giant cell lesions described previously and
therefore needs to be considered and ruled out. Lesions of this
type are seen in patients with renal failure or secondary to
hyperparathyroidism. Laboratory tests that should be ordered
are serum calcium, parathyroid hormone (PTH), and alkaline
phosphate. These lesions resolve once the underlying pathology
is cherubism.
9.5.3 Cherubism
Patients with cherubism also have similar microscopically
identical lesion to giant cell lesions. Cherubism needs to be con-
sidered if multiple giant cell lesions are found in a child
(Fig. 9.14). Cherubism favors males population over female
population, presents early in life (2–5 years) and, in mild cases,
can present later (10 years), and typically presents as bilateral
painless expansion of the posterior mandible. The giant cell
lesions often only involve the angle and ascending ramus of the
mandible but not the condyle. Prognosis is variable, making
treatment di cult. In some patients, early intervention with
enucleation and curettage of the lesions results in excellent
results, whereas in other patients, it is followed by rapid
regrowth of the lesions and worsening deformity. Although
cherubism is self-limiting by the age of 30 years, observation
alone can occasionally result in severe facial deformity, with
functional limitation and associated psychologic problems.
9.5.4 Multiple Giant Cell Lesion
Syndrome
There is a subset of patients with Noonan’s syndrome who also
have multiple giant cell lesions, also referred as Noonan-like/
multiple giant cell lesion syndrome (MS/MGCLS). These
patients often have short stature, wide forehead, ocular hyper-
telorism, prominent posteriorly angulated ears, high arched
palate, webbed neck, and multiple giant cell lesions of the
bones. Lesions associated with Noonan’s syndrome may resolve
after puberty, and therefore, conservative therapy is typically
recommended.
9.5.5 Fibrous–Osseous Lesions
Fibrous–osseous lesions are a group of lesions that are known
to a ect the jaws and the craniofacial bones. This group of
lesions shares a common process, in which the normal architec-
ture of bone is replaced by fibrous tissue containing varying
amount of mineralized tissue. Some of these lesions are felt
to be neoplastic, whereas others are related to metabolic distur-
bances. They share common characteristic clinical, radio-
graphic, and microscopic features. They are typically found in
patients between 10 and 20 years of age and radiographically
present as ovoid expansions of the jaw with ground-glass or
orange-peel radiopaque appearance. Unfortunately, these
lesions have been under frequent renaming and reclassification
due to their varied features. In this chapter, we utilize the
Speight and Carlos’s classification from 2006 that is focused on
histopathologic features. The main groups in this classification
are the following: fibrous dysplasia (FD), osseous dysplasia
(OD), and ossifying fibroma (OF).
9.5.6 Monostotic Fibrous Dysplasia
Fibrous dysplasia can be further classified as monostotic FD,
craniofacial FD, and polyostotic FD. Monostotic FD accounts for
about 70% of all lesions and involves only one bone ( Fig. 9.15).
When lesions are in the maxilla and involve adjacent structures
such as the zygoma and sphenoid, the designation craniofacial
FD is utilized (Fig. 9.16). When craniofacial bones are
involved, cranial nerve compression can occur. Craniofacial FD
tends to be in younger patients when compared with mono-
stotic FD. Both typically present as a painless swelling that
Fig. 9.10 (a) Ameloblastic fibroma of left maxilla associated with impacted first molar. (b) Ameloblastic fibroma—radiolucent lesion associated with
impacted left maxillary first molar.
Craniofacial Disorders
88

demonstrates very slow growth. Teeth involved in the lesions
are usually stable but may be displaced by the mass. Radio-
graphically, FD is seen as a fine-ground-glass opacification that
is not well demarcated from the surrounding bone and often
involves the cortex ( Fig. 9.15). The final subtype of FD is poly-
ostotic FD, which is characterized by involvement of two or
more bones. Two thirds of patients with polyostotic FD are
younger than 10 years, and when seen with café au lait pigmen-
tation, the condition is termed Ja e–Lichtenstein syndrome. In
addition, about 3% of patients with polyostotic FD have multiple
endocrinopathies, a pattern known as McCune–Albright syn-
drome. All patients with polyostotic FD have symptoms that
Fig. 9.11 (a) Unicystic ameloblastoma of the right mandible. (b) Three-dimensional rendering of right mandibular ameloblastoma. (c) Resection of
the right mandibular ameloblastoma and placement of a reconstruction plate. (d) Specimen figure. (e) Radiograph of specimen ensuring 1 cm bony
margins.
Osseous Lesions of the Craniofacial Region
89

tend to be dominated by the involved long bones and not the
craniofacial region.
Overall, the treatment of FD varies depending on the clinical
presentation, degree of deformity, and age of the patient.
Smaller lesions, particularly in the mandible, may be surgically
resected, but when lesions involve the zygoma, maxilla, and/or
sphenoid, resection is not often possible and surgical reduction
is the only option. Regrowth of the lesion often occurs over time
and is more common in younger patients. In rare cases, malig-
nant transformation to osteosarcomas has been described, and
they are more commonly associated with lesions that have
received radiation therapy. Finally, bisphosphonates have been
used in the treatment of FD with varied success, most consis-
tently improving pain associated with FD.
9.5.7 Osseous Dysplasia
Osseous dysplasia is a group of fibrous–osseous lesions that
includes periapical OD, focal OD, florid OD, and familial giganti-
form cementoma. The first three, periapical, focal, and florid, do
not present in the pediatric population and occur only in the
tooth-bearing areas of the jaws. Familial gigantiform cemen-
toma presents during the first decade of life as multifocal radio-
lucencies that eventually evolve into a mixed radiolucent,
radiopaque pattern, ultimately resulting in massive sclerotic
masses involving both the maxilla and the mandible. The
masses tend to grow rapidly during adolescence, before enter-
ing the final slow sclerotic stage. Because of the rapid regrowth
of the dysplastic tissue when in the mixed stage, treatments
such as shave down and resection should not be done before
the final sclerotic stage, when the lesions are predominately
radiopaque.
9.5.8 Ossifying Fibroma
Ossifying fibroma includes conventional OF, juvenile trabecular
OF, and juvenile psammomatoid OF. Conventional OF is a true
neoplasm seen in adults, mainly in the premolar and molar
areas of the jaw. In the pediatric population, juvenile OFs typi-
cally present before the age of 15 years, and both patterns have
similar radiographic features and growth patterns. They tend to
occur more commonly in the maxilla and often appear as a
well-circumscribed, radiolucent lesion. Both types demonstrate
slow growth, and the psammomatoid variant is usually outside
Fig. 9.12 Postresection radiograph.
Fig. 9.13 Three-dimensional rendering of CT demonstrating giant cell
of anterior mandible.
Craniofacial Disorders
90

Fig. 9.14 (a) Multiple
lesions—Noonan’s syn-
drome. (b–d) Three-di-
mensional rendering of
multiple lesions—
Noonan’s syndrome.
Osseous Lesions of the Craniofacial Region
91

of the jaws, with more than 70% occurring in the orbital, frontal,
and paranasal sinuses. Typically, complications from this lesion
are secondary to impingement of adjacent structures, resulting
in nasal obstruction, exophthalmos, or proptosis. Clinical
management of juvenile ossifying fibroma is unclear, with
some lesion demonstrating rapid aggressive growth, especially
in infants and young children. Small lesions may be locally
excised, whereas larger lesions may require resection. Unlike
the adult conventional form, juvenile OFs tend to have a higher
recurrence rate, and close follow-up is required.
9.5.9 Osteoblastoma
Osteoblastoma occurs rarely in the jaws. It is more common in
the vertebrae and long bones and tends to present in children
and young adults. Patients typically experience swelling and
have a history of chronic dull pain. The calvarium is the second
most common site, and patients who have the temporal bone
or zygoma involved may have secondary trismus. Clinically,
patients present with bony expansion, mild, dull pain that is
tender to palpation, and, if the mandible is involved, possible
Fig. 9.15 (a,b) Fibrous dysplasia.
Fig. 9.16 (a,b) Craniofacial fibrous dysplasia.
Craniofacial Disorders
92

paresthesia. Radiographically, they present as mixed radiolu-
cent–radiopaque lesions ( Fig. 9.17). Recommended treatment
is wide en bloc resection with 1.0 cm bony margins, including
one anatomic soft tissue layer, when possible. Lesions may
slowly recur up to 10 years after resection. With enucleation
and curettage alone, there is a high recurrence rate of about
25%.
9.5.10 Myxoma
Myxoma present as a rapidly growing, painful jaw mass, most
commonly in the mandibular angle and ramus, in children. It
has a clinical and radiologic presentation similar to ameloblas-
toma. Peak incidence is between 15 and 25 years of age, but
unlike ameloblastoma, this tumor frequently presents in chil-
dren younger than 10 years. The rapid growth and pain in chil-
dren often result in a misdiagnosis of an acute infection.
Radiographically, myxoma is present as a multiloculated radio-
lucency with soap-bubble appearance, typically within the pos-
terior jaw. Cortical perforation and soft tissue involvement are
often present. Treatment involves 1.0 to 1.5 cm margins and
one uninvolved anatomic barrier, but others have advocated
smaller margins in pediatric population due to the resulting
facial disfigurement and disruption in facial growth. Recurrence
is as high as 25% and typically within the first 2 years after
treatment. Myxomas can also occur in other areas of the face,
such as the paranasal sinus. Midfacial myxomas are rare and
should be di erentiated from other benign and malignant
tumors such as dermoid and rhabdomyosarcomas.
9.5.11 Aggressive Fibromatosis
Aggressive fibromatosis, also known as a desmoid tumor when
located within the abdomen, is a benign but locally aggressive
tumor found in children. Diagnosis is usually between 8 and 15
years of age, and it often presents in the head and neck region.
The mandible is most commonly a ected, and radiographically,
it presents as a radiolucent, destructive lesion causing erosion
of the adjacent cortexes. Clinically, it often presents as a rapidly
growing, painless mass in the head and neck region. Because of
its local infiltration, treatment requires en bloc resection.
Recurrence rate is up to 50%, and wide surgical excision is the
most important factor in decreasing the recurrence rate. Che-
motherapy and radiation therapy have been e ective only in
Fig. 9.17 Osteoblastoma.
Osseous Lesions of the Craniofacial Region
93

controlling inoperable lesions and have not been shown to be
curative.
9.5.12 Desmoplastic Fibromas
Desmoplastic fibromas are rare bone tumors that many con-
sider the bony counterpart to the desmoid tumor mentioned
previously. They are histologically identical. Desmoplastic fibro-
mas do not metastasize but are locally aggressive, requiring
wide local en bloc resection.
9.5.13 Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare disease involving
proliferation of antigen-presenting cells, with clinical presenta-
tions ranging from an isolated bone lesion to multisystem
disease. More than 50% of patients are younger than 10 years,
and most often first present with symptoms of the maxillofacial
region. Lesions tend to involve the posterior mandible and
clinically present as swelling of the jaw, ulcers, loose teeth, and
pain. Radiologic examination typically reveals punched-out
radiolucent lesions, with teeth floating in space (Fig. 9.18).
Jaw lesions are present in all three types. Langerhans cell histio-
cytosis is clinically divided into three groups, unifocal, multifo-
cal unisystem, and multifocal multisystem. Unifocal LCH
typically presents with solitary or multiple bone lesions and is
treated with curettage, radiation, and interlesional corticostero-
ids. Overall, it has a good prognosis. Multifocal unisystem LCH
is characterized by fever, bone lesions, and di use eruptions.
When diabetes insipidus is present with exophthalmos and
lytic bone lesions, this is called Hand–Schuller–Christian triad.
Peak onset is at 2 to 10 years of age, and treatment involves
immunosuppressive agents and corticosteroids, with an overall
good prognosis. The last type is known as Letterer–Siwe disease
and is found in children younger than 2 years, and the progno-
sis is poor, with a 5-year survival of 50%.
9.5.14 Burkitt’s Lymphoma
Burkitt’s lymphoma (BL) is a high-grade B-cell non-Hodgkin’s
lymphoma and exists in multiple subtypes. The African or
endemic variant usually involves the maxilla and other facial
bones, whereas head and neck manifestations of nonendemic
BL are rare. It primarily a ects African children aged 4 to 8
years and is twice as common in boys. Patients often experience
swelling, loss of teeth, and mild pain. Treatment involves inten-
sive intravenous chemotherapy and selective surgical debulk-
ing. Burkitt’s lymphoma is aggressive, with a doubling time of
24 hours, and if left untreated, death occurs in 6 months.
9.5.15 Neurofibroma
Neurogenic tumors are extremely rare within osseous struc-
tures in the head and neck region. Neurofibromas are the most
common jaw tumors of neurogenic origin. They often present
as multilocular radiolucencies and bony expansion. Treatment
consists of close observation and surgical excision of lesions
that produce pain, paresthesia, or rapid growth. Goal is to excise
soft tissue mass and recontour the underlying bone. The tumors
are extremely vascular and have a high recurrence rate.
9.5.16 Melanotic Neuroectodermal
Tumor of Infancy
The melanotic neuroectodermal tumor of infancy (MNTI) is
an uncommon osteolytic-pigmented neoplasm that presents
within the first year of life. It primary a ects the jaws, with 95%
Fig. 9.18 Langerhans cell histiocytosis.
Craniofacial Disorders
94

of lesions involving the anterior maxilla (Fig. 9.19a, b). Similar
to other tumors of neural crest origin, such as neuroblastoma,
and pheochromocytoma, infants often demonstrate a high
urinary excretion of vanillymandelic acid (VMA). Clinically,
the lesions rapidly grow and can often impair sucking and
feeding. Often times, there is a deciduous tooth displaced
within the lesion. The lesion has a dark melanotic pigmentation
(Fig. 9.19c). Treatment involves surgical excision with 2- to 5-
mm margins and is usually curative. Existing teeth and devel-
oping buds must be included in the specimen. Recurrence rates
have been reported up to 15%. There are some case reports of
neoadjuvant therapy reducing the lesion by 50% and should be
considered in lesions that will involve the orbit or are
inoperable.
9.5.17 Vascular Lesions
Vascular lesions are almost never of primary osseous origin.
Intraosseous mandibular arteriovenous malformations are less
than 5% of all vascular malformations (Fig. 9.20a–c). They are
more frequent in women and are more common in the head
and neck area than extremities. Malformations are usually
noticed at birth and grow proportionately but may become
clinically relevant when the adult teeth erupt. Teeth may be
mobile and pulsatile. Life-threatening hemorrhage may occur
with extractions. Management primarily involves interven-
tional radiology for sclerotherapy and embolization (Fig. 9.20e).
Primary osseous surgical intervention is rarely indicated.
Malignant tumors involving osseous structures include
osteosarcoma and Ewing’s sarcoma.
Osteosarcoma is the most common malignant tumor in chil-
dren, representing more than 50% of all malignant pediatric
bone lesions. Less than 5% of pediatric osteosarcomas present
in the head and neck area, most often within the jaws
(Fig. 9.21a). It is felt that there is a slight male predilection,
and peak incidence is during late adolescence.
The most significant risk factor for head and neck osteosar-
coma in children is hereditary retinoblastoma. Other risk fac-
tors include previous radiation therapy and certain syndromes
that predispose individuals to osteosarcomas such as Li–Frau-
meni and Rothmund–Thomson syndromes.
Definitive diagnosis and staging for osteosarcoma require a
tissue biopsy, head and neck computed tomography (CT), chest
CT, local magnetic resonance imaging (MRI), and bone scan
(Fig. 9.22b, c). The histologic and molecular profile deter-
mines if the lesion is low versus high grade. Management
involves local control with surgery and radiation for those
patients with positive surgical margins, even though it is felt
that osteosarcoma is not radiation-sensitive. For high-grade
lesions, adjunctive chemotherapy is often utilized, initially pre-
operatively as a neoadjunct, followed by a month for recovery
and biopsy to determine the percentage of tumor kill. This is
followed by surgical resection and 6 week of postoperative che-
motherapy. The most significant prognostic factor influencing
the overall survival is complete resection with negative mar-
gins. Patients with negative margins have a 75% 5-year survival
rate compared with 35% 5-year survival rate of those who have
positive margins. Unfortunately, bony margins of 3 cm and soft
tissue margins of 2 cm are nearly impossible in the head and
neck area due to the complex anatomy. The most common site
for metastasis is the lungs. However, the regional lymph nodes
are rarely involved in metastasis of the tumor, and as a result,
neck dissection is not recommended.
9.5.18 Ewing’s Sarcoma
Ewing’s sarcoma is a destructive and aggressive malignancy of
the bone that is rare in the head and neck area. It is mostly seen
in patients younger than 20 years and is extremely rare in peo-
ple of African or Asian descent. Most patients have a painless
mass, and up to 20% of patients have metastatic disease at the
time of diagnosis. CT imaging shows ill-defined, irregular
resorption of bone with focal areas of bone.
Definitive diagnosis and staging for Ewing’s sarcoma require
a tissue biopsy, head and neck CT, chest CT, local MRI, and bone
scan. In addition, Ewing’s sarcoma is positive on fluorodeoxy-
glucose positron emission tomography (FDG-PET), which
should also be performed. Finally, bilateral bone aspirates and
biopsies are routinely performed for staging in pediatric
patients. In addition to traditional formalin fixation, a fresh
sample should be secured for cytogenic testing.
The standard approach to treatment is a multimodality
approach with chemotherapy administration and local control
either via surgery or radiation therapy. Surgical control
involves 3 cm margins and 1 to 2 cm of soft tissue margins.
The surgeon should place a plate for reconstruction and delay
definitive reconstruction until completion of radiotherapy and
chemotherapy.
Fig. 9.19 (a) Melanotic neuroectodermal tumor of infancy (MNTI) of the maxilla in a 4-month-old infant. (b) Three-dimensional printing of an MNTI
lesion in a 4-month-old infant. (c) Incisional biopsy of MNTI demonstrating pigmentation.
Osseous Lesions of the Craniofacial Region
95

Fig. 9.20 (a–c) Arteriovenous malformation (AVM) of the right mandible. (d) Three-dimensional rendering of vasculature feeding AVM of the right
mandible. (e) Postembolization of AVM of the right mandible.
Craniofacial Disorders
96

9.5.19 Other Lesions of the Bones
Idiopathic Osteosclerosis
Idiopathic osteosclerosis is a common radiologic finding of
asymptomatic, radiodense lesion most commonly associated
with vital teeth, found in late first to early second decades of
life. If the tooth is nonvital, then the lesion is referred to as con-
densing osteitis and is thought to be a response to an inflam-
matory stimulus. The lesions tend to be well-defined, rounded,
radiodense masses. They can be managed with radiographic
imaging, as they tend to not progress or change into adulthood.
Infantile Osteopetrosis
Infantile osteopetrosis is a rare hereditary skeletal disorder
characterized by increased bone density. Bone is unable to be
absorbed because of defective osteoclast function, resulting in
thick bone and sclerosis of the cancellous bone. When discov-
ered at birth or early in infancy, children often have a di usely
sclerotic skeleton, and often times, there can be evidence of cra-
nial nerve compression. In addition, a common finding is
deformity of the face, with frontal bossing, and hypertelorism.
Common complications are blindness, facial nerve palsy, and
Fig. 9.21 (a) Intraoral view of an osteosarcoma of the right maxilla. (b,c) Osteosarcoma of the right maxilla.
Osseous Lesions of the Craniofacial Region
97

osteomyelitis secondary to tooth extraction because of tooth
disease or delayed tooth eruption. Treatment and management
are dependent on the clinical challenge being faced, but in gen-
eral, care should be taken to minimize potential osteomyelitis
and/or fractures of the maxillofacial region.
Gorham–Stout Disease
Gorham–Stout disease (GSD), also known as vanishing bone dis-
ease and massive osteolysis, is a rare, poorly understood skele-
tal condition characterized by spontaneous and progressive
disruption of one or more bones. The a ected area does not
regenerate or repair and fills instead with dense fibrous tissue
(Fig. 9.22a). The disease starts in one bone and may spread to
involve adjacent bony and soft tissue structures. Lesions have
been reported in the mandible and skull ( Fig. 9.22a–c). Most
a ected patients are children and young adults. There is often a
history of trauma, and the lesions can occur anywhere in the
body. The rate of destruction and extent are quite varied, and
there are no predictable factors. Typically, surgical intervention
is futile as the process is best managed with radiation therapy
and antiosteoclastic medications.
Suggested Readings
Marx RE, Stern D. Oral and Maxillofacial Pathology: A Rationale for Diagnosis
and Treatment; 2012
Rahbar R, Rodriguez-Galindo C, Meara JG, Smith ER, Perez-Atayde AR, eds.
Pediatric Head and Neck Tumors: A-Z Guide to Presentation and Multimodal-
ity Management; 2014
Kaban LB. Pediatric Oral and Maxillofacial Surgery
Thompson LDR, Wenig BM. Diagnostic Pathology: Head Neck; 2011
Richter GT, Suen JY. Head and Neck Vascular Anomalies: A Practical Case-
Based Approach; 2015
Kademani D, Tiwana P. Atlas of Oral and Maxillofacial Surgery; 2015
Fig. 9.22 (a) Gorham–Stout disease with subsequent thinning of the right skull base, condyle, and ramus. (b) Gorham–Stout disease involving the
zygoma and ramus in the same patient. (c) Three-dimensional rending of a child with Gorham–Stout disease involving the right skull base, zygoma,
TMJ, and ramus.
Craniofacial Disorders
98

10 Distraction Osteogenesis
Elizabeth Zellner and Derek M. Steinbacher
Sum m ary
Distraction osteogenesis, the gradual repositioning of osteo-
tomized bone in order to generate new bony matrix, is a power-
ful tool for transforming the craniofacial skeleton. Compared
with conventional surgery, distraction may result in shorter
operating times, lesser blood loss, and lesser morbidity, along
with simultaneous expansion of the associated skin and soft tis-
sue. This gradual approach may overall engender fewer compli-
cations and relapse. However, distraction does require longer
overall treatment times and significant familial participation. At
present, clinical indications for distraction are expanding, and it
represents a major tool in the craniofacial surgery arsenal. A
thorough understanding of the current science and the applica-
tions of distraction osteogenesis is necessary for any pediatric
plastic surgery. Three-dimensional modeling and simulation
can provide valuable technological adjuncts in planning and
executing distraction treatment plans.
Keywords: distraction osteogenesis, mandibular distraction,
midface distraction, monobloc, craniosynostosis
10.1 Introduction
Distraction osteogenesis is a powerful tool in craniomaxillofa-
cial surgery, and its indications continue to broaden. Gradually
repositioning the bones of the craniofacial skeleton entails a
smaller initial operation and may mitigate the need for perma-
nent rigid fixation. During osseous expansion, not only new
bone is generated but also the soft tissue is influenced and adja-
cent spaces can be enlarged. Smaller devices with improved
mechanics and the advent of three-dimensional (3D) analysis
and planning have enabled improved patient experiences and
outcomes.
10.2 History of Distraction
Osteogenesis
Distraction osteogenesis was first described by Codvilla in
1905, where he described using axial distraction forces to
lengthen a femur. This was not adopted into mainstream medi-
cine until Ilizarov, a Russian physician, popularized the techni-
que in the lower extremity starting in 1949. The technique was
vetted in a series of physiologic experiments, where bone gene-
ration was confirmed following gradual traction to the callus.
The functional load on bones and their blood supply also
showed impact on morphology and articulation points. In par-
allel, steady traction also encouraged the accommodating
expansion of the adjacent soft tissue, including periosteum,
blood vessels, ligaments, cartilage, muscles, and overlying skin,
known as distraction histogenesis.
In the 1970s, Snyder described lengthening of the canine
mandible by using a similar method of bony distraction. This
technique was then applied to humans in the early 1990s, to
treat patients with Nager’s syndrom e and hemifacial microso-
mia. Since that time, additional craniofacial applications have
taken advantage of this powerful tool for gradually lengthening
the skull and facial bones, along with the overlying soft tissue.
Throughout the intervening decades, distraction has been
used, with significant success in the mandible, and also
for alveolar distraction, Le Fort I maxillary advancement, Le
Fort II and III midface advancement, monobloc/fronto-orbital
advancement (FOA), and cranial vault expansion. As each new
technique has evolved, a more thorough understanding of the
uses and limitations of distraction has also become more
apparent.
10.3 Principles of Distraction
The principle of distraction osteogenesis is straightforward.
The procedure begins in the operating room with a carefully
planned osteotomy across the region where bony expansion
is desired. This osteotomy may be full or partial thickness,
depending on the adjacent structures present, and the orienta-
tion of these cuts is critical to achieving the desired result. Once
the osteotomy is made, a distraction device (internal or exter-
nal) is applied with grounding footplates or pins on either side
of the osteotomy. The vector of this device placement deter-
mines the 3D direction of the distraction and bony regenerate
formation. Depending on the age of patient, time may be given
for a latency period, where the device is held in its original posi-
tion, with no forces applied across the devices. Once distraction
begins, the device is activated and it stretches the span across
the osteotomy site. This is done in a slow, controlled fashion,
which enables the regenerated bone matrix to form across the
osteotomy as it is stretched. Once the desired end position of
distraction is reached, the devices are left in place for consolida-
tion. This time period permits the bony regenerate to ossify and
strengthen into true new bone. After this, the devices can be
removed and the newly generated bone will maintain the dis-
tracted length (Fig. 10.1).
The advantages of distraction osteogenesis include the grad-
ual distraction histogenesis, first described by Ilizarov. Since the
two sides of the osteotomy are not extensively manipulated in
the operating room, there is less immediate trauma to the tis-
sue, which means less blood loss, less swelling, and shorter
operative and hospital admission times. Unlike osteotomies
with repositioning and bony fixation immediately in a new
location, there is no need for bone grafting, as the bone incre-
mentally forms itself. Distraction may also mitigate future
growth restrictions by avoiding need for wide subperiosteal
exposure and fixation. With external devices, there is also the
opportunity for midcourse vector correction if the tissues do
not respond exactly as predicted to the distraction plan. In
addition, distraction may be appropriate for smaller children
who may not tolerate single-stage bony movement.
Disadvantages of distraction pertain to the hardware require-
ment and total treatment time. Patients and families are edu-
cated that this is not a one-and-done surgery but instead
Distraction Osteogenesis
99

occurs over a period of weeks to months and requires activation
and cooperation by the caretakers. The distraction devices,
which may remain on for several months, may be distressing to
parents if not adequately prepared. Often, much of the distrac-
tion is carried out at home, which requires less hospitalization
time, but it does require participation of parents to turn the dis-
traction devices and accurately record the protocol. Distraction
procedures require a strong treatment relationship and com-
munication between the craniofacial teams and the patients/
families under their care, in order to reach a successful outcome.
10.3.1 Scientific Principles of
Distraction Osteogenesis
During distraction osteogenesis, a number of critical events are
carefully orchestrated at the molecular level. During the distrac-
tion phase, bone forms in response to the forces of tension and
stress on the regenerate, which heals by membranous ossifica-
tion. The collagen fibers are oriented parallel to the direction of
distraction, and osteoid is deposited in the center of the regen-
erate. Ossification then advances from either end of the central
fibrous zone, which forms an immature bone bridge across the
distraction defect. This immature bone then remodels, begin-
ning in the consolidation phase and continuing for 1 to 2 years.
Although the regenerate is never as strong as the native bone, it
eventually reaches approximately 75% of native bone strength.
Limitations of distraction osteogenesis may occur in patients
who have poor local and systemic substrates (Table 10.1). In
addition, devices may become dislodged by trauma or infection,
which can result in malunion or nonunion. Longer periods of
consolidation may also be cumbersome to some patients. As a
result, much recent research has been aimed at increasing bone
deposition to possibly shorten the overall treatment time. Rat
studies have used adenovirus-driven transcription of the gene
for BMP-2 to enhance bony deposition. It is also known that
ischemia can be a limiting factor in distraction. Studies have
shown that endothelial progenitor stem cells are drawn to this
ischemic site during activation and they remain through the
consolidation period. These stem cells may represent another
possible source to overcome ischemic limitations of distraction.
Cyclic mechanical strength has been related to expression of
IGF-1, TGF-B, and bFGF along with interleukins (IL)-1 and IL-6,
possibly the e ectors of further bone grow th in distraction
osteogenesis. Numerous interventional cytokines have been
utilized to optimize bony deposition and possibly decrease
overall treatment time. A list of these substances can be found
in Box 10.1.
Box 10.1 An Overview of Some Molecular
Cytokines’ Interventions and Their Effect on
Distraction Osteogenesis
● Bone morphogenic protein
● Endothelial progenitor cells
● Alkaline phosphatase
● Transforming growth factor-β1
● Vascular endothelial growth factor
● Basic fibroblast growth factor (bFGF)
● Interleukin 1
● Interleukin 6
Fig. 10.1 Three-dimensional plan showing (a) hypoplastic mandible before distraction osteogenesis, (b) mandibular osteotomy perpendicular to plane
of planned distraction, with internal device oriented along planned distraction vector. Please note the inferior fixation plates would be removed during
actual surgery. (c) Simulated position of mandible following planned distraction. Demineralized bony regenerate will form along the gap during the
activation period and will mature into calcified bone; however, the device stays in place during the consolidation period. Once this is complete, the
device will be removed, with newly formed bone spanning the distraction length.
Table 10.1 Local, systemic, and distraction factors that may a ect the
outcomes in distraction osteogenesis
Local factors

Systemic factors

Distraction factors
Osteoprogenitor supply
Blood supply
Infection
Soft tissue scarring
Bone stock
Radiation history
Age
Metabolic disorders
Vitamin D deficiency
Connective tissue
disease
Steroid therapy
Calcium deficiency
Latency period
Rate of distraction
Rigidity of fixation
Consolidation period
Regenerate length
Craniofacial Disorders
100

10.3.2 Distraction Protocols
Distraction osteogenesis consists of three general phases:
latency, distraction, and consolidation ( Table 10.2). The first
stage, latency, occurs after bony osteotomy but before activating
the distraction device. During this time, the surgeon allows pre-
liminary callus to form but does not want the regenerate (the
intermediate stage of bony deposition) to begin to reossify
through calcification. Depending on the bone being distracted,
those skeletal components with a richer blood supply or mem-
branous origin (such as the mandible and the skull plates)
require shorter latency periods. In addition, younger patients,
such as neonates and young infants, may not require any signif-
icant latency period before distraction. For mandibular distrac-
tion in these patients, the surgeon can either start distracting
immediately or wait only 24 hours to begin distraction. In
contrast, older patients, who are skeletally more mature, may
require a slightly longer latency period, usually 3 to 5 days
when distracting the mandible.
Distraction is the phase in which the physical lengthening of
the bone is instigated through active manipulation of the dis-
traction device. Using the distraction device, the callus that is
formed on either side of the osteotomy site is stretched gradu-
ally. During this time, there is exuberant angiogenesis. This vas-
cularized osteoid stimulates new bone formation across the
osteotomy defect, which forms parallel to the distraction vector.
Much has been written on the ideal frequency and rate of dis-
traction, and there is significant variation among di erent
authors and practitioners. There must be a balance between
distracting too fast, which results in a fibrous nonunion, and
distracting too slow, which can cause premature bony union
that prevents further lengthening. Perhaps, the most frequently
cited rate of distraction is 1 mm/day, generally separated
into two daily increments of 0.5 mm. However, distraction rates
of 2 mm/day and even 3 mm/day have also been reported.
Younger patients, such as neonates, can generally tolerate more
rapid distraction, as their bony healing patterns are more
robust. The indications of airway creation to avoid a tracheos-
tomy or of cranial expansion to allow brain growth in these
patients also generally provoke a more urgent, more rapid
course of distraction.
The final phase of distraction osteogenesis is consolidation.
During this period, the device is used to maintain the physical
lengthening achieved by the distraction period. While the bone
is being stabilized in this manner, the bony regenerate can reos-
sify through calcification into a mature bone. The consolidation
phase is generally twice as long as the active distraction phase
to allow su cient bone maturation, so that the device is no lon-
ger needed for structural stabilization. Consolidation periods of
4 to 12 weeks have been cited, but a period of at least 8 weeks
is preferred and has been shown to generally prove su cient
in most cases. Again, younger patients generally require shorter
consolidation phases because of their faster healing. Load-
bearing bones, such as the mandible, may require longer
consolidation periods. When in doubt about an appropriate
consolidation period, ultrasound or radiographs can be used to
check on the progress of bony remineralization.
10.4 Devices
To achieve these goals, a number of di erent devices have been
used throughout the years (Table 10.3). Initially, external dis-
tractor devices utilized transcutaneous pins to fixate either side
of the osteotomy site with external distraction arms to exact
the lengthening vector (Fig. 10.2). These devices, with their
active parts exposed and available for manipulation, allow for
alteration of the distraction vector during the active distraction
period. The obvious downsides of these devices include signifi-
cant bulk and unsightly pin tracks in the skin. Since then, inter-
nal devices have been developed. These are placed directly on
the bone under the skin and soft tissue; however, periosteum is
ideally left intact on at least one side of the osteotomy site to
guide the formation of the bony callus. Internal devices also
include a distraction arm, which comes through the skin, usu-
ally at some length away from the device to discourage
Table 10.2 Phases of distraction osteogenesis and considerations during each phase
Phase

Osteotomy

Device
placement
Latency

Activation

Consolidation

Device
removal
Growth
modification
Time

0

0

0–7 d

Days–weeks
Months (at least 6
weeks or 2 × acti-
vation)
End of treat-
ment
After device re-
moval
Considerations

Full thickness
Adjacent struc-
tures
Orientation
Orientation
Number
Age of patient
Anatomic area
Membranous vs
long bone?
Age of patient
Anatomic
area
Length de-
sired
Device length
Age of patient
Device complica-
tions
Infection risk
Reossified
bone
Timing
Retaining
hardware
Orthodontic ap-
pliances, elastics
Table 10.3 Types of devices used for distraction osteogenesis
Device type Pros

Cons
Internal

Comfort for patient and
family
Less obtrusive
Infectious risk
Unable to adjust vector
Second removal operation
External

Vector adjustable
Single or stage
Cumbersome to patient and
family
Linear

Straightforward
Device fixation
Limited dimensions
Curvilinear

More anatomic

Complex planning
Challenging engineering
Multivector Precise control
Multiple dimensions
Complex planning
Require multiple devices
Device fixation
Distraction Osteogenesis
101

infection ( Fig. 10.3). This arm is manipulated to activate dis-
traction of the device. Internal devices can be concealed in a
more aesthetic fashion and can be more tolerable to patients
and their families, with fewer external components to dislodge.
This may permit for longer consolidation periods. However,
these can be di cult to securely fixate in very young bone, they
must be removed at a second operation, and the vector of inter-
nal devices cannot be manipulated during the distraction
period.
Additional relevant factors include device placement to
determine the vector of distraction. In addition to the orienta-
tion of placement, devices can be uni- or multidirectional,
which will influence the course of distraction based on clinical
objectives. Curvilinear devices are also available and imple-
mented in certain contexts (Fig. 10.4). Many variations of
footplates, device arms, and morphologies exist, customized for
a wide range of applications.
Resorbable distraction devices have been designed and
implemented in patients for many types of distraction osteo-
genesis. These devices hold the promise of single-stage (or
much smaller second stage) distraction protocols that hold the
bones in consolidation for a longer period of time but never
need to be removed. In addition, programmable, automated
devices have been investigated and successfully used in human
mandibular distraction osteogenesis in an adult patient.
Although these devices have not yet gained widespread use,
expandable metal alloys, springs, and pneumatic controls may
make distraction protocols simpler for patients and their fami-
lies in the future.
Three-dimensional computed tomographies (CTs) and soft-
ware planning tools o er improvement to distraction planning
and application. An initial CT scan allows a quantitative meas-
urement of the morphology and the existing bone–soft tissue
relationships. Computer-assisted design and manufacturing
(CAD/CAM) permits virtual surgical osteotomies, device place-
ment, and proposed movements to optimize the clinical result.
Three-dimensional prints based on the simulation can then
translate the digital plan intraoperatively with cutting guides
and templates ( Fig. 10.5). Some have even advocated image-
guided and robotically performed osteotomies to further
enhance precision in delivering a 3D computer-aided surgical
plan.
The distraction plan must incorporate a number of factors.
The dysmorphology and deformity must be accurately ana-
lyzed, and the goals of correction (size, length, direction, and
m agnitude) m ust be accurately defined. Future growth and
overcorrection m ust also be considered. The device type,
direction, and placem ent must be carefully selected and
individualized to the patient. The distraction protocol is then
discussed with the patient or family at length before
Fig. 10.2 Initial studies utilized external distrac-
tors that were inserted percutaneously into bone.
Fig. 10.3 Internal distractors were subsequently developed with vector arms through which to transcutaneously submit distraction vectors.
Craniofacial Disorders
102

beginning distraction, as success is incumbent upon their
participation. Consolidation and other retention m easures
are implem ented to m aintain the distracted outcome goals
w ith time.
10.5 Specific Applications for
Distraction Osteogenesis
10.5.1 Mandible
Mandibular distraction is the most common application, typi-
cally for expanding the pharyngeal airway and alleviating glos-
soptosis. This is indicated for airway compromise that may
otherwise require a tracheostomy. Both syndromic (i.e., Nager’s,
Treacher Collins, or Stickler’s) and nonsyndromic (Pierre Robin’s
sequence) patients may benefit from distracting the mandible.
Mandibular distraction is considered if other sites of airway
obstruction are minimal or exonerated, for patients requiring
persistent intubation/tracheostomy (or the desire to decannu-
late these patients), for an airway that cannot be managed by
conservative measures such as prone positioning, or if sleep
studies indicate moderate to severe obstructive sleep apnea
(Table 10.4; Fig. 10.6).
Internal and external distractors can be used with a variety of
vectors. New-generation internal devices are small enough to
accommodate a neonatal mandible and are preferred. Distrac-
tion vector depends on surgeon preference, without broad
consensus. Multivector distraction is touted to mimic normal
morphology and growth, especially in older children. However,
a single-vector orientation is often used in small, infant mandi-
ble. Bilateral bicortical osteotomies are usually performed pos-
terior to the developing tooth roots, in an oblique or vertical
fashion, taking care to preserve the facial nerve and inferior
alveolar nerve branches. The devices should be placed parallel
and collinear, taking into account the inferior border, adjacent
anatomic structures, and planned trajectory. For micrognathia
or retrognathia causing airway obstruction, the primary
Fig. 10.4 An X-ray showing a curvilinear in place to recreate a more
complex anatomical vector in an older patient.
Fig. 10.5 Three-dimensional plans from CT scans utilized in posterior vault distraction. (a–c) Computed tomographic images can be used to assess
bony, soft tissue, and intracranial anatomy and print custom cutting guides to precisely and efficiently affect the surgical plan in the operating room.
In mandibular distraction, the course of the inferior alveolar nerve and tooth buds can be analyzed with imaging to assist surgical planning (d–f).
Distraction Osteogenesis
103

distraction goal is to enlarge the retroglossal airway. Ideally,
this is done to preserve normal mandibular morphology and to
avoid occlusal malrelationships, but the airway is the prime
outcome variable. Pulling the tongue anteriorly, and also inferi-
orly, has the greatest influence on the posterior airway space.
Both sagittal and oblique vectors can accomplish airway
enlargement with excellent clinical outcomes. Mandibular
distraction is e ective to avoid tracheostomy and allow decan-
nulation in both infants and older children with airway obstruc-
tion secondary to retrognathia.
Combined vertical ramus and horizontal mandibular hypo-
plasia in hemifacial microsomia and other causes of asymmetry
can also be addressed using distraction (Fig. 10.7). External
devices were used in early generations to vertically and coro-
nally improve the mandibular morphology. The Pruzansky/
Kaban’s classification of mandibular hypoplasia di erentiates
the degree of mandibular hypoplasia based on the degree of
mandibular ramus and temporomandibular joint involvement.
Type I and II mandibles are generally amenable to ramus dis-
traction in a largely vertical vector to correct the mandibular
asymmetry. In type III mandibular hypoplasia, the proximal
ramus–condylar unit is entirely absent, with no articulating
temporomandibular joint. An intact proximal stop in the form
of a functional temporomandibular joint is a prerequisite for
mandibular distraction. In these severe cases, replacement of
the ramus will be first necessary, often with either free rib graft
or free fibula flap, which may then require further lengthening
with distraction osteogenesis.
Controversy about the progression of asymmetry in hemifa-
cial microsomia (HFM) exists, and one reason to intervene with
distraction during the mixed dentition is to avoid worsening of
the deformity. Other evidence suggests that the asymmetry
remains proportional with time and grow th and that early dis-
traction may be subject to relapse. However, orthodontic bone
anchorage, bite wafers, and growth modulation may help
maintain postdistraction form. Early intervention may also be
indicated for psychosocial issues, and the type IIB or III mandi-
ble may benefit from ramus reconstruction with subsequent
distraction. For type I and IIA cases, if distraction is deemed
appropriate, then age 6 to 8 years is chosen both for the fre-
quency of peer interactions and to take advantage of permanent
tooth eruption to help level the frontal occlusal cant. A posterior
open bite should not be left to close on its own, but rather,
encouraged passive or active eruption, while maintaining the
mandibular position, is employed. Distraction is typically not
purely vertical in these patients but rather a combination, as
the deformity is in three dimensions.
10.5.2 Le Fort I
Distraction has also been used in the maxilla for midface defi-
ciency in cleft and syndromic patients ( Fig. 10.8). It has been
estimated that between 25 and 60% of patients born with uni-
lateral cleft lip and palate will require maxillary advancement
to correct functional deficits and unsatisfactory facial aesthetics
due to maxillary hypoplasia. Maxillary hypoplasia has been
associated with speech and feeding di culties as well as with
issues relating to nasopharyngeal airway patency. Traditionally,
very large Le Fort I advancements, such as those greater
than 10 mm of anteroposterior advancement, and significant
advancement in cleft patients have been associated with
increased relapse.
After observing success in mandibular distraction, facemasks,
interdental splints, and then internal devices were used to
advance the maxilla gradually following Le Fort I osteotomy.
Although the initial external distractors were sometimes
decried for being cumbersome, significant progress has since
been made with internal devices, even when maxillary
advancements greater than 10 mm are indicated. Distraction
can achieve greater advancement than conventional, one-stage
Table 10.4 An overview of distraction osteogenesis in the craniofacial skeleton
Anatomic Area

Indications

Benefits

Downsides
Mandible

Retrognathia (airway)
Vertical length (asymmetry)
Proximal ramus/TMJ
Bone generation
Alleviate glossoptosis
Vertical mandibular length
2 procedures
Maxilla

Le Fort I (cleft maxilla)
Large magnitude maxillary
movements
Achieve large sagittal advance-
ments
Less relapse
Speech
2 procedures
Occlusion
Alveolar

Vertical length
Medial/lateral (cleft)
Avoid or less bone graft for dental
implants, or close alveolar fistula
2 procedures
Midface/monobloc/
bipartition
Syndromic midface and frontal
hypoplasia
Achieve large advancements
Less relapse
Less dead space or infection
2 procedures
Cumbersome External device
Occlusion
Cranial

Syndromic synostosis
Chiari
Gradual vascularized expansion
Less relapse
2 procedures
Transport

Bone defects

Close critical-sized defect with
autologous tissue
2 procedures
Abbreviation: TMJ, temporomandibular joint.
Craniofacial Disorders
104

Fig. 10.6 Mandibular distraction done for mandibular hypoplasia in order to facilitate tracheostomy decannulation in an older patient (a,b) and to
avoid tracheostomy in an infant with isolated Pierre–Robin sequence (c–e). Pre- and postoperative 3D CTs of a patient undergoing mandibular
distraction (f,g).
Distraction Osteogenesis
105

Fig. 10.7 Mandibular distraction osteogenesis CT
planning (a,b) and pre- and postoperative pho-
tographs (c,d) in a patient with hemifacial
microsomia.
Fig. 10.8 Maxillary distraction in cleft patients
has been effective, with less relapse than
conventional surgery. Pre- and postoperative
cephalograms (a–c) and 3D photographs of a
patient with a history of cleft palate who under-
went maxillary distraction (d,e).
Craniofacial Disorders
106

Le Fort I movements and may minimize the need for mandibu-
lar setback, which can compromise posterior airway space.
Though not routinely used, maxillary distraction may be con-
sidered in young patients with severe maxillary deficiency.
Studies have also shown decreased relapse and improved
speech outcomes in cleft patients who undergo distraction
osteogenesis versus traditional maxillary osteotomy and plat-
ing. A hybrid approach may be employed, where the maxilla is
distracted to length, and rather than implementing a consolida-
tion phase, the devices can be removed and the maxilla plated.
Distraction devices have also been indicated in the orthodontic
literature to close large alveolar clefts, with some success.
Midface
Frontofacial and midface distraction osteogenesis has been
gaining more support in recent years, usually in patients with
syndromic craniosynostosis, such as Crouzon’s and Apert’s syn-
dromes. Computed tomographic morphometry has elucidated
that although these patients have similar midface bony volume
compared with normal controls, they have altered sphenoid
morphology, with diminished growth inferiorly and anteriorly.
Large anterior and inferior movements can be required to
address significant midface issues of exorbitism and nasopha-
ryngeal airway and could cause considerable morbidity, with
likely relapse in a single-stage operation. In addition, with a sin-
gle-stage procedure, a complete frontofacial procedure, such as
a monobloc, leaves a large dead space that is in communication
with both the cranial vault and the nasal passages, presenting a
concerning source for infection. In contrast, gradual distraction
obviates the formation of this dead space as the soft tissue
stretches in parallel with the bony movements. Frontofacial
advancement with distraction osteogenesis has been shown to
produce stable, satisfactory results in multiple studies.
Most distraction of the midface follows the conventional
Le Fort osteotomies shown by Tessier. Later, the monobloc
was described by Ortiz-Monasterio. It moves the midface and
frontal bone as one piece and was then distracted with success
by Polley et al. Although there are many options for midface
distraction, the individual osteotomies and movements must be
modified and the facial skeleton individually partitioned to
address the specific needs of the patient.
10.5.3 Le Fort II and III
The Le Fort III segment is distracted for syndromic midface
deficiency, where the orbits, nose, zygoma, and maxilla are all
deficient. The orbits and upper airway are enlarged, improving
globe position and protection as well as airway obstruction
(Fig. 10.9). In addition, the malar region is m ade more
prominent, the nasal morphology is improved, and occlusal
conditions are optim ized. Either internal or external devices
can be used in this area, both with success and proponents.
Longitudinal follow-up of these patients shows a trend
toward greater advancem ent, with less relapse with Le Fort
III distraction versus conventional Le Fort III. This m ay assist
in lowering the num ber of operations in patients who other-
wise would have a significant num ber of surgeries through-
out their lifetim e.
Hopper et al have proposed a di erential midface reposition-
ing using a Le Fort II distraction with zygomatic repositioning
for midface advancement in patients with Apert’s syndrome.
Through this technique, they hope to address the syndromic
central facial concavity, in addition to its malposition. Although
Fig. 10.9 Le Fort III 3D CT advancement plan for a patient with Crouzon’s syndrome (a,b).
Distraction Osteogenesis
107

there is less literature on advancement using a Le Fort II osteot-
omy, this distraction pattern combined with simultaneous
zygomatic repositioning showed improved normalization of
facial ratios on CT morphometry. Customizing the osteotomy
plan to an individual patient’s morphology, with guidance from
3D CT morphometry, may represent the future for improving
outcomes in these complex patients (Fig. 10.10).
Monobloc
In cases where both the midface and the fronto-orbital region
are deficient, monobloc distraction may be considered
(Fig. 10.11). In this circumstance, the midface and frontal
bones are moved in conjunction to address both the forehead
and the midface. This approach allows for the m ost complete
resolution of exorbitism but imparts com m unication of the
intracranial and nasal/oral cavities. A single-stage m onobloc
w ith significant advancem ent results in an unfavorable com -
plication profile. Patients with ventriculoperitoneal shunts
had the highest complications due to a lack of brain expan-
sion, to fill the extradural space, following surgery. For con-
ventional surgery, a two-staged (FOA and Le Fort III) m ethod
exhibited less m orbidity compared with m onobloc. Kawa-
m oto compared conventional with distracted m onobloc,
indicating a decrease in complications from 61% to 8%,
including m eningitis, cerebrospinal fluid (CSF) leak, bone
flap loss, and wound infections. The distraction group also
had less blood loss, a shorter inpatient hospital stay, and
larger advancem ents.
Therefore, when indicated, a m onobloc should be per-
form ed using distraction osteogenesis exclusively. Polley et
al first described using distraction osteogenesis in conjunc-
tion w ith a m onobloc osteotomy, using an external distrac-
tion device. Internal devices can also be used for monobloc
distraction. Distraction has reintroduced a powerful tool in
the m onobloc, with fewer adverse e ects. Facial bipartition
m ay also be used in conjunction with monobloc advance-
m ent to help with m idface concavity in syndrom ic patients.
Outcom es analysis of midface m onobloc distraction has been
satisfactory in num erous studies, w ith greatly improved
safety profiles.
10.6 Neonatal Midface Distraction
Neonatal midface distraction has been used in extreme cases of
exorbitism, especially in Pfei er’s syndrome. In 1983, Muhlba-
uer et al described conventional monobloc advancement in a
newborn but cautioned that this should be used only in cases of
severe functional impairment. Polley et al first described mono-
bloc distraction in a newborn with Pfei er’s syndrome and
severe exorbitism. Initially, there was enthusiasm for early in-
tervention in this patient, but this has dimmed after significant
relapse was observed, which required further surgery. If possi-
ble, supportive therapies such as tarsorrhaphies and even tra-
cheostomies may present better alternatives to support a young
infant for many months/years until the child’s bone stock and
ability to tolerate major surgery may be improved. However,
Dunaway’s recent review of personal experience in very young
children (average, 18 months) treated with monobloc for
functional issues showed successful resolution of symptoms.
The proper lower cuto for utilizing monobloc advancement
remains a highly contentious issue in craniofacial surgery.
10.7 Posterior Vault
Posterior vault expansion through distraction osteogenesis has
changed the treatment sequence paradigm for craniosynostosis
syndromes (Fig. 10.12). Although traditionally, the anterior
vault was the first target of surgical intervention, relapse and
lack of growth necessitate a high rate of redo FOAs later in
childhood in this population. In contrast, first expanding the
posterior vault enlarges the intracranial volume largely and
allows for delay of the FOA to a later date, in the hope of requir-
ing the FOA only once. In addition, compared with conventional
posterior vault remodeling, the modification of distraction ena-
bles a larger expansion, with soft tissue accommodation, and
less relapse associated with supine head placement. Distraction
also imparts the benefit of leaving the inner table attached to
dura, maintaining vascularization of the posterior cranial seg-
ment and enabling concurrent “brain” expansion with distrac-
tion. The decreased dissection also means decreased risk to the
torcula and posterior venous system, and the reduced dead
space may limit the risk of collection and infection.
Fig. 10.10 A number of different surgical plans can be utilized to improve skeletal outcomes in patients. (a) Le Fort III osteotomy. (b) Le Fort III
osteotomy with zygomatic repositioning. (c) Le Fort I osteotomy with Le Fort osteotomy.
Craniofacial Disorders
108

The human skull reaches approxim ately 77% of its adult
volume by 2 years of age and 90% by 5 years of age. In nor-
m al children, the m ajority of this initial grow th occurs in
the posterior fossa, w hereas in syndrom ic patients with
bicoronal craniosynostosis, expansion occurs in the m iddle
cranial fossa, with an underdeveloped posterior fossa. Poste-
rior distraction addresses this discrepancy anatom ically,
helps norm alize morphology, and limits turribrachycephaly.
The frontal region m ay experience added projection as an
indirect e ect of distraction, based on New ton’s third law. In
addition, Arnold-Chiari malform ations m ay be sim ultane-
ously addressed.
10.8 Anterior Cranial Distraction
Traditional fronto-orbital advancement with fixation remains
the gold standard for treating anterior cranial constriction.
However, several groups have reported on experimental success
with anterior cranial vault distraction. The data are currently
limited and without longitudinal follow-up, but theoretic bene-
fits may exist, including maintenance of vascularity. Critics of
anterior cranial distraction cite the e ectiveness of conventional
techniques, without technical di culty, and, perhaps, subopti-
mal correction of turricephaly with fronto-orbital distraction.
There are also concerns about postoperative stability and the
risk of reoperation in these patients with more scar tissue
present. A more recent direct comparison between conventional
FOA and distraction for cranial remodeling failed to show any
di erence in anthropometric outcomes between groups. How-
ever, frontal distraction may gain traction as an option for fore-
head deformities in the future.
10.9 Bone Graft and Flap
Distraction
In addition to the native bones of the craniofacial skeleton,
various reports have shown that distraction can be applied to
heterotopic bone from both avascular bone grafts and micro-
vascular free bone flaps to increase the utility of this trans-
planted tissue ( Fig. 10.13). The grafts and flaps are allowed to
incorporate and fully heal before distraction is applied.
Fig. 10.11 Monobloc advancement of the face,
including the frontal bone and the midface in one
concerted movement, was first described by
Ortiz-Monasterio. Monobloc distraction moves
the frontal bone in conjunction with the midface
but cannot increase nasal length. (a) Lateral
cephalogram of a child undergoing monobloc
advancement. (b) Preoperative photograph of a
patient with Apert’s syndrome. (c) The same
patient following monobloc distraction osteo-
genesis. Orbital protection and airway obstruc-
tion were greatly improved following the
procedure.
Distraction Osteogenesis
109

Distraction can provide even more needed bone in previously
deficient areas treated by grafts or flaps if undertaken in appro-
priate patients. Transport distraction osteogenesis uses the
principles of distraction to fill bony defects such as in the calva-
rial skull or mandible. In addition, techniques such as fat graft-
ing may improve transport distraction osteogenesis by
increasing the bone density of the regenerate.
10.10 Conclusion
Distraction osteogenesis is a powerful tool for transforming
the craniofacial skeleton through a carefully planned, gradual
approach. Advantages include shorter operating times and
concurrent expansion of the overlying soft tissue, with fewer
complications and less relapse. Distraction does require partici-
pation from and communication with families, with acceptance
of the longer course of treatment. Given the benefits, the clini-
cal uses are expanding. Technologies such as CAD/CAM and
other advances have improved the planning and reproducibility
of distraction. Grow th modification and overcorrection should
be employed when possible. Clinical results and outcomes will
continue to be improved with continued study and longitudinal
follow-up ( Fig. 10.14).
Fig. 10.12 (a) Pre- and (b) postoperative photo-
graphs of a patient with Pfeiffer’s syndrome,
bicoronal craniosynostosis, and resulting brachy-
cephaly, who underwent posterior vault distrac-
tion osteogenesis with (c) pre- and (d)
postoperative 3D CT scans.
Fig. 10.13 Reconstruction of a Pruzansky III mandible required rib
graft and eventual distraction to produce adequate mandibular stock.
Craniofacial Disorders
110

Suggested Readings
McCarthy JG, Schreiber J, Karp N, Thorne CH, Grayson BH. Lengthening the
human mandible by gradual distraction. Plast Reconstr Surg. 1992; 89(1):1–
8, discussion 9–10
Tavakoli K, Stewart KJ, Poole MD. Distraction osteogenesis in craniofacial sur-
gery: a review. Ann Plast Surg. 1998; 40(1):88–99
Pfa MJ, Steinbacher DM. Plastic surgery applications using three-dimen-
sional planning and computer-assisted design and manufacturing. Plast
Reconstr Surg. 2016; 137(3):603e–616e
Schaefer RB, Stadler JA , III, Gosain AK. To distract or not to distract: an algo-
rithm for airway management in isolated Pierre Robin sequence. Plast
Reconstr Surg. 2004; 113(4):1113–1125
Werler MM, Starr JR, Cloonan YK, Speltz ML. Hemifacial microsomia: from
gestation to childhood. J Craniofac Surg. 2009; 20 Suppl 1:664–669
Kumar A, Gabbay JS, Nikjoo R, et al. Improved outcomes in cleft patients with
severe maxillary deficiency after Le Fort I internal distraction. Plast Reconstr
Surg. 2006; 117(5):1499–1509
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
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
Steinbacher DM, Skirpan J, Puchała J, Bartlett SP. Expansion of the posterior
cranial vault using distraction osteogenesis. Plast Reconstr Surg. 2011; 127
(2):792–801
Cetrulo CL , Jr, Knox KR, Brown DJ, Ashino RL, Dobryansky M, Ceradini DJ,
Capla JM, Chang EI, Bhatt KA, McCarthy JG, Gurtner GC. Plast Reconstr Surg.
2005 Sep 15; 116(4):1053–64; discussion 1065-7
Ashino RL, Cetrulo CL , Jr, Galiano RD, Dobryansky M, Bhatt KA, Ceradini DJ,
Michaels J , 5th, McCarthy JG, Gurtner GC. Ann Plast Surg. 2004 Jun; 52
(6):585–90; discussion 591
Eckardt H, Bundgaard KG, Christensen KS, Lind M, Hansen ES, Hvid I.. J Orthop
Res. 2003 Mar; 21(2):335–40
Farhadieh RD, Dickinson R, Yu Y, Gianoutsos MP, Walsh WR.. J Craniofac Surg.
1999 Jan; 10(1):80–6
Rowe NM, Mehrara BJ, Luchs JS, Dudziak ME, Steinbrech DS, Illei PB, Fernan-
dez GJ, Gittes GK, Longaker MT. Ann Plast Surg. 1999 May; 42(5):470–5
Fig. 10.14 Distraction osteogenesis represents a powerful tool in craniofacial surgery and can be employed in conjunction with other techniques in a
longitudinal fashion to optimize patient outcomes. A patient with Crouzon’s syndrome (a) preoperatively, (b) after Le Fort III distraction, and (c) and
following a Le Fort I, bilateral sagittal split osteotomies (BSSO), genioplasty, and subsequent rhinoplasty at skeletal maturity.
Distraction Osteogenesis
111

11 Orthognathic Correction of Jaw Deformities
Rushil Dang and Cory M. Resnick
Sum m ary
Orthognathic correction of jaw deformities in the pediatric
population aims to correct the dental occlusion, along with
addressing an equally important goal to improve facial aes-
thetics. A comprehensive presurgical workup and evaluation
must include intra- and extraoral examinations, facial photo-
graphs, diagnostic radiographs, mounted dental models, occlu-
sion records, and a facebow transfer. In skeletally immature
patients, the nature of the deformity, the operations necessary
for correction, and the psychosocial impact of surgery must all
be considered when determining the appropriate timing for an
operation. Recognition of common facial patterns can be helpful
in determining appropriate corrective operations. The most
common operations used in orthognathic surgery are the Le
Fort I (LFI) osteotomy for repositioning of the maxilla and the
bilateral sagittal split ramus osteotomies for movement of the
mandible.
Keywords: orthognathic surgery, Le Fort I, bilateral sagittal split
osteotomy, ramus osteotomy, facial growth, complications
11.1 Introduction
As many as 5% of adolescents in the United States have a dento-
facial deformity that cannot be corrected with orthodontic
treatment alone. Advances in operative and anesthetic techni-
ques since von Langenbeck’s original description of a maxillary
osteotomy in 1895 have allowed maxillary and mandibular
osteotomies to become safe and predictable. In the last decade,
the combination of three-dimensional treatment planning with
low-profile rigid fixation has ushered in a new era of precision
in orthognathic surgery.
11.2 Diagnosis and Treatment
Planning
Evaluation begins with comprehensive facial and oral examina-
tions, photographs, radiographs, mounted dental models, regis-
tration of the occlusion, and a facebow transfer.
The facial examination is performed in natural head position,
which is achieved by asking the patient to focus on a distant
point at eye level. The shape of the face is characterized, such as
square or round, and the facial index (ratio of height to width)
is calculated. Normal facial indices are 1.35:1 for men and 1.3:1
for women. The face is evaluated for symmetry at all levels. Ver-
tical facial proportions are assessed by measuring the distances
from trichion to glabella, glabella to subnasale, and subnasale to
menton; each section should comprise approximately one-third
of the total facial height. The lower facial third can be further
divided from subnasale to stomion and from stomion to men-
ton, which should account for the one-third and two-thirds,
respectively, of the entire lower facial one-third. Transverse
proportions are evaluated by partitioning the face vertically
into five equal sections that are each the width of the eye from
the medial canthus to the lateral canthus. Shape and symmetry
of the nose are determined. Lip morphology and posture are
evaluated both in repose and in function. During repose, 2 to
4 mm of the maxillary incisor teeth should be visible. In full
smile, the canine tips should be seen without excessive gingival
show or dark buccal corridors. Presence and depth of the labio-
mental sulcus are evaluated.
On intraoral examination, the presence and health of the
complete adult dentition, the dental midlines in relation to the
midsagittal plane, the angle classification of occlusion, overjet,
and overbite are determined. The occlusal plane is evaluated
from both the frontal and the lateral positions. Study casts are
used to analyze the intra-arch and interarch relationships.
The radiographic evaluation has historically consisted of a
series of plain X-rays, including panoramic, lateral cephalomet-
ric, and posterior–anterior cephalometric images. This two-
dimensional series has largely been replaced by computed
tomography (CT) or cone-beam CT, from which two- and three-
dimensional images can be extracted. Three-dimensional imag-
ing has improved accuracy in the planning and simulation of
surgical movements ( Fig. 11.1) and in evaluation of relevant
anatomic structures, including the airway space, temporoman-
dibular joints, and inferior alveolar nerve canals.
The preoperative assessment also includes an analysis of the
patient’s level of skeletal maturity. The chronological, skeletal,
and dental ages; the nature of the deformity and required cor-
rective operations; and the e ect of the deformity on psychoso-
cial development are all considered when determining the
appropriate time for an operation. Growth of the maxilla is
completed before that of the mandible, with the mandible
closely following the skeletal growth pattern. Girls typically
reach skeletal maturity between 15 and 16 years of age; boys
continue to grow until 16 to 18 years of age or longer. Menarche
is a useful marker for the growth cycle for girls, as skeletal
growth is usually completed by 2.5 to 3 years thereafter. Evalua-
tion of vertical height, use of hand-wrist radiographs, and over-
lays of tracings of serial lateral cephalograms can be used to
assess the timing of facial growth. An operation involving only
advancement of the mandible in the direction of grow th can be
pursued before skeletal maturity, with low risk for postoperati-
ve mandibular growth leading to a new malocclusion. Move-
ments that are not in the direction of growth and bimaxillary
surgery should be delayed until after the cessation of growth,
unless psychosocial indications dictate earlier operation.
Recognition of common facial patterns can be helpful in
determining appropriate corrective operations. Examples
include the following:
● Class III skeletal pattern: This is most commonly caused by
maxillary hypoplasia but can be due to mandibular hyperpla-
sia or a combination of these. It is corrected by maxillary
advancement mandibular setback or both ( Fig. 11.2).
● Class II skeletal pattern: This pattern is typically caused by
mandibular hypoplasia and is corrected by mandibular
advancement (Fig. 11.3).
Craniofacial Disorders
112

● Vertical maxillary excess (VME): It is characterized by excess
gingival show, a steep mandibular plane angle, increased total
anterior facial height, and decreased contribution of the
upper facial height to the total facial height. An anterior open
bite is often present. It is corrected by superior repositioning
of the maxilla, often with flattening of the occlusal plane.
Bimaxillary surgery is often required.
● Facial asymmetry: It presents with various etiologies, includ-
ing asymmetric mandibular grow th, condylar hyperplasia or
resorption, trauma, and malformations such as hemifacial
microsomia. It is corrected by bimaxillary surgery. The ideal
vertical position of the maxilla must be determined in order
to identify the proper fulcrum of rotation for the correction.
11.3 Operative Management
The most common operations used in orthognathic surgery are
the LFI osteotomy for repositioning of the maxilla and the
bilateral sagittal split ramus osteotomies (BSSO) for movement
of the mandible.
11.3.1 Le Fort I Osteotomy
The LFI osteotomy is a versatile procedure that allows the max-
illa to be repositioned in three planes of space.
Technique
1. Arrange for the use of controlled hypotensive anesthesia,
which reduces bleeding from the rich network of small ves-
sels at mucosal and bone edges that cannot be easily con-
trolled with surgical techniques. The need for transfusion in
orthognathic surgery has been nearly eliminated by using
this technique.
2. Local anesthesia with vasoconstrictor is administered into
the maxillary buccal vestibule.
Fig. 11.1 Three-dimensional virtual surgical planning images from a patient with maxillary hypoplasia. (a) Preoperative images. (b) Planned skeletal
movements.
Orthognathic Correction of Jaw Deformities
113

3. An external reference marker is placed at the nasion by
using a Kirschner’s wire or a bone-fixation screw. Measure-
ments are made from this reference to reproducible midline
and lateral maxillary landmarks to facilitate precise reposi-
tioning in the vertical plane. Internal reference markers are
inadequate.
4. A horizontal incision is made in the gingivobuccal sulcus 5
to 10 mm superior to the mucogingival junction, from first
molar to first molar. The submucosal incision is brought
inferiorly near the midline to avoid perforation of the nasal
mucosa.
5. A subperiosteal dissection is performed along the anterior
maxillary walls superiorly to the infraorbital foramina,
medially to expose the piriform rims, and laterally to the
zygomaticomaxillary sutures. Dissection is extended poster-
olaterally to the pterygomaxillary fissures.
6. A Freer’s or Cottle’s elevator is used to dissect the nasal
mucosa from the piriform rims and maxillary crest.
7. A reciprocating or piezoelectric saw is used to create a hori-
zontal osteotomy 5 mm superior to the root apices of the
canine and first molar teeth, from the zygomaticomaxillary
buttresses to the piriform rims on each side.
8. A long curved osteotome is inserted into the pterygomaxil-
lary fissure at a level at and inferior to the horizontal
osteotomy on each side. The osteotome is driven in anterior,
medial, and inferior directions to separate the posterior
maxilla from the pterygoid plates.
9. The osteotomies at the posterior maxillary walls are com-
pleted with a small osteotome.
10. A guarded nasal septal osteotome is driven posteriorly and
inferiorly along the maxillary crest to separate the caudal
nasal septum and vomer from the maxilla.
11. An osteotome is used to fracture the lateral nasal walls to
the perpendicular plates of the palatine bone.
12. Downward pressure is applied to the m axilla to create a
down fracture. If significant resistance is encountered,
the surgeon should stop and reevaluate each of the
osteotom ies.
13. The descending palatine arteries are identified on each side
and evaluated for tears. If necessary, the vessels can be
clipped or cauterized, without increasing the risk for vascu-
lar compromise of the maxilla.
14. The maxilla is mobilized by stretching the soft tissue enve-
lope by using Rowe’s disimpaction forceps and/or pterygoid
lifts. Mobilization of the maxilla until it fits passively into
the surgical splint in the planned position is imperative.
15. Segmentation of the maxilla can be performed as dictated
by the treatment plan.
Fig. 11.2 A 20-year-old woman with a class III skeletal pattern and a lower facial asymmetry. (a,b) Preoperative photographs demonstrating a thin and
poorly supported upper lip. (c–e) Preoperative dental occlusion. (f–h) Preoperative radiographs showing maxillary sagittal hypoplasia and mandibular
asymmetry.
Craniofacial Disorders
114

16. The maxilla is then positioned into the prefabricated surgi-
cal splint, and maxillomandibular fixation is applied with
wires.
17. The maxillomandibular complex is rotated superiorly while
posterior and superior pressure is applied to the mandibular
angles to fully seat the condyles. Bony interferences are
identified and reduced.
18. Vertical repositioning of the maxilla is determined by
observing the relationship between the maxillary central
incisors and the upper lip and by measurements from the
external reference marker. Measurements to lateral maxil-
lary landmarks are repeated to ensure that no undesired
cant of the maxilla has been introduced or that a preexisting
cant has been corrected.
19. The nasal septum is evaluated to ensure that it is not
buckled. A caudal septoplasty can be performed if
necessary.
20. With the maxillomandibular complex rotated to its planned
position and the condyles fully seated, fixation is applied
using miniplates at the piriform rims and zygomaticomaxil-
lary buttresses.
21. The maxillomandibular fixation is released, and the occlu-
sion is checked. Unplanned occlusal discrepancies must be
corrected; failure to recognize and correct a malocclusion at
this point will compromise the operative result.
22. The wounds are copiously irrigated.
23. Widening of the alar base is reduced, as desired, with an
alar cinch suture.
24. The mucosal wounds are closed with resorbable sutures.
The external reference marker is removed.
11.3.2 Sagittal Split Ramus
Osteotomies
Sagittal split ramus osteotomies have surpassed vertical ramus
osteotomies as the most common mandibular operations in
orthognathic surgery because of their versatility in correcting a
Fig. 11.2 (Continued) (i–l) Postoperative facial and intraoral photographs after Le Fort I osteotomy, with advancement and anterior disimpaction, and
bilateral sagittal split osteotomies, with mandibular rotation. (m–o) Postoperative radiographs demonstrating the planned osteotomies, skeletal
movements, and internal fixation.
Orthognathic Correction of Jaw Deformities
115

Fig. 11.3 An 18-year-old man with a class II skeletal pattern. (a) Preoperative photograph demonstrating a retrognathic mandible with a convex facial
profile. (b) Preoperative lateral cephalometric radiograph showing mandibular sagittal hypoplasia. (c,d) Postoperative three-dimensional photograph
and radiograph after bilateral sagittal split osteotomy, with advancement of the mandible and advancing and lengthening genioplasty.
Craniofacial Disorders
116

range of mandibular deformities and the ability to routinely
apply rigid internal fixation.
Technique
1. Local anesthesia with vasoconstrictor is administered.
2. An incision is made in the buccal vestibule, extending from
the external oblique ridge to the area adjacent to the second
premolar tooth.
3. The buccal aspect of the mandible is exposed in a subperios-
teal plane.
4. Dissection is continued to the inferior border of the mandi-
ble. A J-stripper is used to release all muscular attachments
from the inferior border in the area of the first molar tooth.
5. Dissection is continued along the anterior ramus to the
coronoid process, with stripping of the attachments of the
temporalis muscle. A Kocher forceps is applied to the tip of
the coronoid process to retract the soft tissues.
6. Dissection is continued on the medial aspect of the mandi-
ble, with attention to remain subperiosteal along the natu-
ral lingual concavity. The lingula and the mandibular
foramen are identified.
7. A Freer’s elevator is used to release the attachments of the
inferior alveolar neurovascular bundle from the lingula,
allowing the bundle to be retracted away from the planned
osteotomy site.
8. With the neurovascular bundle protected, a reciprocating or
piezoelectric saw is used to create a corticotomy through
the lingual mandibular cortex extending from the post–lin-
gula depression to the superior border of the mandible and
oriented parallel to the occlusal plane.
9. The cut is continued monocortically along the superior bor-
der of the mandible buccal to the dentition to the area of
the first molar tooth.
10. At the first molar, the cut is redirected to extend through
the buccal cortex toward the inferior border of the
mandible.
11. An inferior border osteotomy is then created, extending
from the buccal corticotomy and including both buccal and
lingual cortices. Special attention is paid to this osteotomy,
given that an inadequate cut at the inferior border is a com-
mon reason for an undesirable fracture pattern.
12. Osteotomies are used to refine each corticotomy.
13. The mandible is then split using a Smith’s spreader and an
inferior border spreader. The split is completed slowly while
observing for even separation across all bone cuts. Unequal
separation is addressed by further refining the cuts in the
a ected areas before completing the split.
14. After completion of the split, the inferior alveolar neurovas-
cular bundle is identified. The bundle must be completely
free from the proximal segment. If a portion of the bundle
remains within the proximal segment, a Freer, nerve hook,
small osteotome, or piezoelectric saw can be used to free
the remaining attachments.
15. The wound is packed, and attention is turned to the con-
tralateral side, where the steps described earlier are
repeated.
16. The distal segment of the mandible is positioned into the
surgical splint against the maxillary dentition, and maxillo-
mandibular fixation is applied.
17. The proximal segments are repositioned such that the con-
dyles are fully seated in their fossae and the inferior borders
of both the proximal and distal segments are aligned.
18. Bony interferences between the proximal and distal seg-
ments are reduced as necessary.
19. Fixation is applied on each side by using a minimum of
three interposition screws, a plate at the distal osteotomy
site, or a combination of these.
20. Maxillomandibular fixation is released, and the occlusion is
checked. Unplanned occlusal discrepancies must be cor-
rected; failure to recognize and correct an incorrect occlusal
relationship at this point will compromise the operative
result.
21. The wounds are copiously irrigated.
22. The mucosal wounds are closed with resorbable sutures.
11.4 Complications of
Orthognathic Surgery
11.4.1 Hemorrhage
Since the introduction of hypotensive anesthesia, hemodynami-
cally significant intraoperative bleeding is rare during orthog-
nathic surgery. Major bleeding is most likely to occur during
the LFI osteotomy from the descending palatine or sphenopala-
tine arteries. During the pterygomaxillary disjunction, bleeding
can be encountered from the pterygoid plexus of veins or, less
likely, from the internal maxillary artery and its branches.
Delayed hemorrhage may also occur several weeks after an LFI,
most often presenting as severe epistaxis. This is due to bleed-
ing from a pseudoaneurysm that formed as a result of trauma
to an intraoperative vessel wall. During sagittal split osteoto-
mies, bleeding most often occurs from the inferior alveolar
artery. The facial artery or vein can also be damaged during
inferior border osteotomy.
11.4.2 Neurosensory Impairment
Sensory deficits involving branches of the fifth cranial nerve
commonly occur after mandibular operations. The inferior
alveolar nerve is most often a ected, followed by the lingual
nerve. Alolayan and Lueng reported neurosensory deficits in
35.4% of patients 6 months after sagittal split osteotomies.
Ow and Cheung found a 27.8% rate of persistent neurosensory
deficit 1 year after sagittal splits (Ow and Cheung 2009). A tran-
sient sensory change of the infraorbital nerve is common after
LFI osteotomies, but it typically completely recovers within
6 months.
11.4.3 Malocclusion
Incorrect positioning of the mandibular condyles during fixa-
tion or unrecognized bony or occlusal interferences can lead to
an early postoperative malocclusion. The most common etiol-
ogy of an immediate postoperative malocclusion is incomplete
seating of the mandibular condyles during repositioning of the
proximal segments after sagittal split osteotomies. When max-
illomandibular fixation is released, the mandibular condyles
move posteriorly to their fully seated positions, resulting in
Orthognathic Correction of Jaw Deformities
117

increased overjet and anterior open bite. Management of an
early postoperative malocclusion may require reoperation. Late
malocclusion may occur due to orthodontic or skeletal relapse,
postoperative growth, unresolved parafunctional habits, or
resorptive processes.
11.4.4 Infection
Early postoperative infections occur in 3.1 to 7.4% of patients
after orthognathic surgery. Age, medical comorbidities, type
and length of the operation, and the use of perioperative anti-
biotics may a ect the risk for infection. Infection rates are
increased by concomitant insertion of alloplastic materials such
as polyethylene implants.
11.4.5 Fixation Failure
Inadequate fixation and/or early function may lead to hardware
failure. Fixation failure most commonly occurs after sagittal
split osteotomies and leads to clockwise rotation of the proxi-
mal segments and development of an anterior open bite. This is
corrected by reoperation with repositioning of the segments
and application of additional fixation.
11.4.6 Nonunion
Nonunion is rare and most commonly occurs after an LFI
osteotomy. This is typically due to the use of inadequate fixa-
tion, early function, or uncontrolled bruxism. Nonunion may
present as prolonged pain and fremitus of the maxillary denti-
tion during mastication several months after the operation.
Nonunion is managed by reoperation with debridement of
fibrous tissue from the previous osteotomy site, bone grafting,
application of additional fixation, and strict postoperative non-
chewing diet.
11.5 Key Points
● Le Fort I osteotomies and sagittal split ramus osteotomies are
versatile procedures for correcting skeletal malocclusions and
facial asymmetries.
● Orthognathic surgery requires thorough preoperative evalua-
tion, including facial and oral examinations, photographs,
radiographs, mounted dental models, registration of the
occlusion, and a facebow transfer.
● Preoperative treatment planning and physical or virtual
model surgery are used to create surgical splints that allow
accurate reproduction of the plan in the operating room.
● In skeletally immature patients, the nature of the deformity,
the operations necessary for correction, and the psychosocial
impact of surgery must all be considered when determining
the appropriate timing for an operation.
● Accurate and honest assessment of the occlusion in the oper-
ating room after release of the intermaxillary fixation is crit-
ical to avoid an early postoperative malocclusion.
Suggested Readings
Reyneke JP, ed. Essentials of Orthognathic surgery. Chicago, IL: Quintessence;
2003
Posnick JC. Orthognathic Surgery: Principles and Practice. St. Louis, MO:
Saunders; 2014
Pro t WR, Turvey TA, Phillips C. Orthognathic surgery: a hierarchy of stabil-
ity. Int J Adult Orthodon Orthognath Surg. 1996; 11(3):191–204
Pro t WR, Turvey TA, Phillips C. The hierarchy of stability and predictability
in orthognathic surgery with rigid fixation: an update and extension. Head
Face Med. 2007; 3(1):21
Carlos E, Monnazzi MS, Castiglia YM, Gabrielli MF, Passeri LA, Guimarães NC.
Orthognathic surgery with or without induced hypotension. Int J Oral Maxil-
lofac Surg. 2014; 43(5):577–580
Wolford LM, Karras SC, Mehra P. Considerations for orthognathic surgery dur-
ing growth, part 1: mandibular deformities. Am J Orthod Dentofacial Orthop.
2001; 119(2):95–101
Lanigan DT, Hey JH, West RA. Aseptic necrosis following maxillary osteoto-
mies: report of 36 cases. J Oral Maxillofac Surg. 1990; 48(2):142–156
Will LA, Joondeph DR, Hohl TH, West RA. Condylar position following man-
dibular advancement: its relationship to relapse. J Oral Maxillofac Surg. 1984;
42(9):578–588
Ow A, Cheung LK. Skeletal stability and complications of bilateral sagittal split
osteotomies and mandibular distraction osteogenesis: an evidence-based
review. J Oral Maxillofac Surg. 2009; 67(11):2344–2353
Chow LK, Singh B, Chiu WK, Samman N. Prevalence of postoperative compli-
cations after orthognathic surgery: a 15-year review. J Oral Maxillofac Surg.
2007; 65(5):984–992
Craniofacial Disorders
118

II
Part II
Cleft Lip and Palate
12 Dental and Orthodontic Management
of Cleft Lip and Palate

120
13 Unilateral Cleft Lip

133
14 Bilateral Cleft Lip

149
15 Cleft Palate

156
16 Robin’s Sequence

163
17 Secondary Cleft Lip and Nasal
Reconstruction

169
18 Velopharyngeal Dysfunction

177

12 Dental and Orthodontic Management of Cleft Lip and
Palate
Richard Bruun and Stephen Shusterman
Sum m ary
Dental and orthodontic management of the patient born with a
cleft of the lip and/or palate requires care providers interested,
motivated, and dedicated to a lengthy and sometimes compli-
cated process. They should be willing to work in concert with a
Cleft Palate Team and well versed in the principles of infant
orthopedics (allows a proficient surgeon the best opportunity
for primary repair of the cleft lip and nasal deformity) cleft den-
tal anatomy, dental arch coordination, secondary alveolar bone
grafting, facial esthetics, and comprehensive orthodontics and
dentofacial orthopedics. Patients with clefts require an empha-
sis on the prevention of dental caries because their teeth are
often more susceptible and their burden of care already great.
Following the guidelines outlined in this chapter will result in
successful treatment for the patient as well as an enjoyable and
rewarding experience for the pediatric dentist, orthodontist,
surgeon and other team members providing care.
Keywords: teeth, malocclusion, cleft lip and palate, alveolar
bone graft, presurgical infant orthopedics, nasoalveolar mold-
ing (NAM), latham, pediatric dentist, orthodontics
12.1 Introduction
Infants born with cleft lip and palate should be evaluated by the
cleft team, including the pediatric dentist, within days of birth.
Pediatric dentists are ideally trained to be a vital part of the
cleft team. Their training programs include growth and devel-
opment, behavioral guidance, guidance of the developing occlu-
sion, preventive dentistry, an introduction to anesthesiology,
and operating room protocols. The pediatric dentist has a
responsibility to instruct parents in a preventive dental pro-
gram, to prepare parents for the long dental road ahead, and to
actively manage any program in presurgical orthopedic prepa-
ration that the cleft team chooses to utilize.
12.2 Dental Care Prior to Bone
Graft
Parents of newborns or any young child are responsible for pre-
ventive dental care, especially in the case of a child born with
cleft lip and palate. Before teeth erupt, the gum pads (alveolar
ridges) should be wiped clean after feeding or nursing. By the
sixth month of life, the first teeth usually erupt—mandibular
teeth erupt before maxillary teeth. It is important to note that
there is considerable variability in the timing and pattern and
that precocious or delayed eruption should not be cause for
great concern or the exposure of radiographs. In children with
clefts, the teeth may erupt more slowly; they may be more
delayed in the cleft quadrant(s) and slower in males than
females. When teeth do erupt, the method of cleaning can be
changed to cleaning with a moist gauze sponge or a clean moist
washcloth. As the number of teeth increase and the child begins
to walk, the method of tooth cleaning should be changed to
brushing, first using nonfluoridated toothpaste and then mov-
ing on to fluoridated toothpaste at about 3 years of age, when
the child can be instructed not to swallow the often-good-tast-
ing toothpaste. Systemic fluoride should be available through
the water supply or via supplementation. The child’s pediatri-
cian or dentist should be familiar with the dosing and prescribe
appropriately.
During the first year of life, a well-baby visit to the dentist is
also recommended by the American Board of Pediatric Den-
tistry as well as by the American Board of Pediatrics. This initial
visit establishes a dental home for the child. It also a ords the
parents the opportunity to ask questions and to the dentist to
educate the parents in preventative methods as well as in den-
tal issues that pertain particularly to children with clefts. It is
important to note that even the most minimal instance of cleft-
ing may not be without its dental consequences, such as mal-
formed teeth, missing teeth, extra teeth, dysplastic enamel, and
even small boney defects.
The early years of childhood should be marked by e orts to
preserve the existing teeth and their bony support. Supernum-
erary palatal teeth, teeth in the cleft, or dysplastic teeth should
not be removed, and every e ort should be made to keep them
caries-free. These teeth are di cult for the child or the parents
to brush, thus putting the child at an increased risk of develop-
ing dental decay; however, the literature is mixed with regard
to the caries rate in children with clefts. In case of carious
lesions on malpositioned or malformed teeth, restoration is rec-
ommended before the onset of infection. Referral to the pedia-
tric dentist may be indicated, as his or her training in the
management of young children will make any necessary treat-
ment as atraumatic as possible.
The issue of the appropriateness of radiographs in the devel-
oping child is always a concern for the parents and the cleft
team. Cavity-detecting radiographs, exposed no more fre-
quently than every 18 months, are indicated in growing chil-
dren when posterior teeth contact, when hygiene is less than
ideal, and when the risk of caries is high enough to raise the
issue of interproximal caries. A child should not be exposed to
panoramic or periapical radiographs to determine dental devel-
opment or to image teeth in the area of the cleft until the child
has reached the early mixed-dentition stage (some permanent
teeth erupted) or when timing for bone graft is being deter-
mined. Just prior to bone graft and to provide the cleft team
with more complete imaging, cone beam computed tomogra-
phy (CBCT) is the radiographic image of choice. With this three-
dimensional (3D) reconstruction, proximity to the cleft and the
position of teeth can be more accurately visualized. This type of
image can also provide panoramic and cephalometric views, by
reconstruction. Radiation exposure, given the need for informa-
tion, is acceptable, and less than that from a medical CT.
Cleft Lip and Palate
120

12.2.1 Presurgical Infant Orthopedics—
Complete Cleft of the Lip and Palate
If the team chooses to go directly to lip repair, it is assumed that
the pressure created by the repaired lip will close the alveolar
cleft and that pressure on the lip will not a ect the esthetic out-
come of the surgical scar. However, the authors feel that, in
most cases, the repaired lip should not be the sole mediator of
pressure on the alveolus. Unavoidable tension on the repaired
lip will result in a more prominent surgical scar and the cleft
segments will collapse, with the lesser segment falling behind
the greater segment in unilateral clefts and both posterior seg-
ments remaining behind the premaxilla in bilateral clefts. These
failures result in increased complexity of future orthodontic
treatment, given later correction of this bony collapse is more
di cult. Although the resulting dental crossbites may be cor-
rected, the dentition will lie on a malpositioned bony base.
Eventual relapse of the malocclusion is therefore more likely,
and lip support, as a prime mediator of facial esthetics, may be
compromised.
If the cleft team wishes to incorporate presurgical orthope-
dics in their treatment protocol, there are several alternatives,
which are as follows:
● Taping of the lip +/– the use of a hat or bonnet to provide
retraction forces.
● Nasoalveolar molding (NAM) (removable).
● Fixed presurgical orthopedic treatment (FPOT).
Taping of the Lip
Taping of the lip provides another opportunity for pressure on
the alveolar segments to retract the more anteriorly placed
alveolar segment and close the alveolar cleft before surgical
repair. However, placement and retention of the tape is di cult,
and replacement of the tape, which must be done frequently, is
even more di cult for the parents. This method of closure may
be enhanced by the use of the Dynacleft taping system or a
bonnet with Velcro attachments for an elastic strap, which will
cross the upper lip for the anticipated and similar e ect
(Fig. 12.1). This method is not only di cult for parents but
also uncomfortable for the infant. Again, this method encour-
ages collapse of the segments in a manner similar to the e ect
of no presurgical treatment described earlier.
Nasoalveolar Molding
Nasoalveolar molding is the most widely adopted of the treat-
ment alternatives before lip surgery. In the early 1990s, Grayson
et al described a presurgical removable device to move the pal-
atal segments and mold the nasal cartilages. He and his group
perceived that the major drawback of previous presurgical devi-
ces was their inability to shape the nose. Nasoalveolar molding,
therefore, combines an intraoral removable molding appliance
with extraoral taping and adds nasal prongs for the purpose of
raising the nasal tip and shaping the alar cartilages. The intra-
oral appliance is adjusted to mold the alveolar segments in both
unilateral and bilateral clefts via the addition of self-cure acrylic
in the pressure areas and the alternate removal of acrylic on the
side toward which movement is desired. When the segments
have moved to reduce the alveolar cleft, nasal prongs are added
to raise and shape the alar cartilages. Proponents of NAM state
that the more di cult surgical goal is the elevation of the ala
and the elongation of the columella and that this appliance
supports these goals. Technical problems with NAM include
hard-to-obtain/unpredictable posterior expansion and poorly
directed retraction of the premaxilla in bilateral clefts as well as
di cult-to-adjust nasal prongs, resulting in pressure on the ala
that may be either inadequate or overzealous in both bilateral
and unilateral clefts. Practical problems include the need for a
dedicated in-house laboratory and a technician (to achieve the
best results) as well as the need for frequent adjustment visits,
placing a significant burden of treatment on the family
(Fig. 12.2).
Fixed Presurgical Orthopedic Treatment
The original sketches by Georgiaide and Latham emphasized on
pressure to rotate and align the palatal shelves while closing
the cleft alveolus in unilateral clefts. In bilateral clefts, it was
noted that retraction of the premaxilla was not possible with-
out expansion of the palatal shelves, so space had to be devel-
oped between the palatal shelves to accept the retracted and
aligned premaxilla. The appliances were designed with hinges
and activation screws. In case of bilateral clefts, symmetrical
expansion was the goal. Latham later added an “auxiliary
Fig. 12.1 Bonnet with elastic traction—infant with bilateral complete
cleft lip and palate. This method is not only difficult for parents but also
uncomfortable for the infant. Again, this method encourages collapse
of the segments in a manner similar to the effect of no presurgical
treatment.

Fig. 12.2 Nasoalveolar molding device.
Dental and Orthodontic Management of Cleft Lip and Palate
121

elastic” to the unilateral design to assist in rotation and closure
of the wider clefts. In bilateral clefts, where retraction of the
premaxilla was required, a transvomer pin was designed to pass
through the bony portion of the anterior vomer just distal to
the premaxilla. This pin was attached to the palatal appliance
by parallel elastic chains. Both appliances were fixed in place to
the palatal shelves with four transpalatal pins.
Our use and studies have supported the routine incorpora-
tion of the auxiliary elastic in all unilateral cases. The cleft can
be closed by an average of 8.6 mm, thus reducing tension on the
repair and enabling the choice of gingivoperiosteoplasty for the
surgeon ( Fig. 12.3).
12.2.2 Fixed Presurgical Orthopedic
Treatment Protocol of Boston Children’s
Hospital
Unilateral Fixed Presurgical Orthopedic
Treatment
Infants are examined and parents interviewed between 2 and 8
weeks of age with the goal of lip adhesion or repair at 3.5
months. Diagnostic records are taken, and an impression of the
maxillary dental arch is made. All impressions are taken with
the baby lying on the parent’s lap, with feet toward the parent
and head on the operator’s knees (knee-to-knee position). Our
material of choice is a high-quality alginate with color-changing
characteristics. The alginate is placed in a “custom” acrylic tray
(made from previous infant impressions and kept in a sterile
pouch) (Fig. 12.4).
Excess alginate is removed posteriorly, before insertion into
the mouth. To limit the risk, the tray is not inserted in the
mouth until the alginate progresses through several setting
stages (purple to pink to white). In our experience, with the
baby in this position, the airway has never been an issue, and a
detailed impression may be obtained.
Infants with clefts who are to undergo fixed presurgical
orthopedics are admitted to the hospital for short stays and
brought directly to the operating room suite.
On the day of operation, the infant must have nothing by
mouth for 6 hours in advance. An oral endotracheal tube is
inserted; the patient is prepped and draped for a sterile proce-
dure, and the oropharynx is packed. The appliance is tried in
the mouth to ensure that it fits well; the pins are inserted in
slots through the acrylic using a needle holder and set in place
by using an orthodontic band seater and a surgical mallet
(Fig. 12.5 and Fig. 12.6).
Each pin is approximately 15 mm in length, with a spring
loop bend at the head, which will be compressed into the slot
in the acrylic to ensure that the pin cannot be lost. When the
pins are fully seated and the appliance is in position, the pin
slots are covered with a small amount of restorative compo-
site to ensure that the pin will not come out of the appliance,
and the composite is set with a curing light. The appliance is
activated by rotation of a screw within the cleft site, which
extends on a diagonal from the anterior of the lesser segment
posteriorly to a receiving cup at the posterior aspect of the
greater segment. Initial activation requires 4 to 5 full (360-
degree) turns, until the screw is firmly engaged in the cup.
The elastic chain extending from the posterior aspect of the
greater segment to the anterior aspect of the lesser segment
and across to a hook on the greater segment is activated
( Fig. 12.7).
Fig. 12.3 Unilateral fixed presurgical orthopedic treatment device with
elastic auxiliary.
Fig. 12.4 (a) Cleft impression tray. (b) Cleft
alginate impression.
Cleft Lip and Palate
122

The throat pack may then be removed. The child is extubated
in the operating room and awakened before being brought to
the postoperative care unit. Infants are kept overnight to ensure
that they begin to feed orally, that vital signs are stable, and that
they are voiding appropriately. Although infants are restless,
fussy, and have di culty feeding immediately postoperatively,
a recent study (Bronkhorst et al 2015) has assured us that the
classic signs of pain are not present postinsertion.
The infants are then seen at 1 week, 3 weeks, and 5 weeks
(more frequently if necessary) to ensure that the appliance is
being properly activated at home (the screw is turned) and to
permit reactivation of the elastic chain. The appliances are
removed at the time of lip repair or lip adhesion.
Bilateral Fixed Presurgical Orthopedic
Treatment
Infants with bilateral clefts are treated at a slightly older age
than those with unilateral clefts (4–8 weeks of age). In the bilat-
eral complete cleft, the anterior margins of the palatal shelves
are frequently narrow and the premaxilla on the vomer stalk is
markedly anterior and, in some cases, twisted in a lateral or
superior/inferior direction. The goal of this treatment is to
move the anterior aspect of the palatal shelves apart (trans-
versely), while at the same time retracting the premaxilla to a
position between the palatal shelves (Fig. 12.8).
In the operating room, the junction of the premaxilla
and vomer is visualized, and two points are marked along a
horizontal line bisecting the cephalad–caudal width of the
vomer, approximately 1 and 3 mm posterior to that junction
(Fig. 12.9). Using a handheld chuck and a twist drill (Peeso
Fig. 12.7 Unilateral fixed presurgical orthopedic treatment after
insertion—chain is activated.
Fig. 12.5 (a) Unilateral fixed presurgical orthopedic treatment during insertion. (b) Mallet and band seater during insertion.
Fig. 12.6 Retention pin.
Fig. 12.8 Bilateral fixed presurgical orthopedic treatment.
Dental and Orthodontic Management of Cleft Lip and Palate
123

endodontic), two parallel channels are drilled through the
vomer at these marks. A U-shaped 0.020 stainless steel wire (8–
10 mm in length) is then prepared for insertion through these
channels. Elastic chain is then attached to the more distal arm
of the “U,” and it is passed through the vomer. Elastic chain is
applied to the distal aspect, and the wire bent to prevent its loss
(Fig. 12.10 and Fig. 12.11).
The elastic chains are then pulled through the appliance, as it
is seated on the palate.
The appliance is secured with four pins in the same manner
as the dental maintenance alternative (DMA). The elastic chains
are then moved anteriorly and are separately attached with
moderate force to the cleats at the anterior edge of each palatal
shelf. The activation screw, which is in the posterior midline, is
activated (Fig. 12.12).
The expansion screw in the bilateral appliance (elastomeric
chain premaxillary retraction [ECPR]) moves the palatal shelves
in a “V” direction and provides enough width to accept the pre-
maxilla. At the same time, the premaxilla is retracted by the
elastic chain. The amount of expansion must be closely moni-
tored to prevent a flattening of the nasal tip, excessive width at
the alar base, and a prominence of the nasal eminences of the
maxillary bone. The patient is monitored at 1 week, 3 weeks,
and 5 weeks to ensure that the screw is activated appropriately
and to allow the elastic chains to be reactivated. Feeding is
again the main postoperative concern. In addition, mucus and
milk curd accumulate between the palatal shelves and are
cleaned with suction at each visit.
The impact of presurgical treatment on the family should not
be minimized.
Parents of infants born with clefts of the lip and palate have
already been significantly challenged in the postpartum stage.
Although most parents now know through prenatal ultrasound
that their child will be born with a cleft, the immediate mourn-
ful reaction after birth (when the reality is confronted) may be
overwhelming. Relatives and friends who may not be fully pre-
pared may become part of the problem. One of the advantages
of presurgical care is the introduction and support of the cleft
team and the knowledge that they are available to help at any
time. From the initial impression on, this is a di cult period for
parents. Our recent review of the postoperative course of infants
who have had Latham-type appliances has shown that pain, as
measured by vital signs and Face, Legs, Activity, Cry, and Consol-
ability (FLACC) scores, is not a significant problem. However, it is
not our intention to minimize the disruptions of these applian-
ces, which include absence from work, loss of sleep, di culties
Fig. 12.9 Visualizing junction of the premaxilla and the vomer.
Fig. 12.10 Insertion of U-shaped wire into the vomer.
Fig. 12.11 Elastic chains pulled through bilateral fixed presurgical
orthopedic treatment.

Fig. 12.12 Fully seated bilateral fixed presurgical orthopedic treatment
—activation screw visualized.
Cleft Lip and Palate
124

in feeding, the impact on other children in the family, and many
more. These are significant disruptions and require that mem-
bers of the cleft team, including the dentist and the feeding spe-
cialist, are available to the parents. However, the result of well-
planned and executed presurgical infant orthopedics will leave
the child with a dentition that is easily maintained, that has
acceptable occlusion and appearance, and that is ready for the
next phase of treatment in the mixed dentition.
12.3 Orthodontic Management—
Mixed Dentition to the Completion
of Treatment
12.3.1 Diagnosis
The diagnostic materials required for the evaluation of the mal-
occlusion associated with cleft of the lip and or palate vary con-
siderably according to the patients’ stage of development and
the exact diagnosis. Models of the teeth; intraoral and extraoral
photographs; and panoramic, lateral cephalometric, and frontal
cephalometric radiographs comprise the diagnostic materials
required, usually referred to as orthodontic records.
Lateral cephalometric analysis of the patient in natural head
position (NHP) will provide the orthodontist and surgeons with
excellent insight into the nature and magnitude of the skeletal
and dental malocclusion (in the sagittal plane) as well as help
them understand the perceived appearance of the dentition
and the profile. The use of “true” vertical and horizontal refer-
ence planes, based on NHP, will help you make the best
decisions regarding not only orthodontic therapy but also the
need for, and desired results of, orthognathic surgery
(Fig. 12.13).
In patients with any obvious or suspected bony alveolar
defect, it is important to fully evaluate the cleft area or areas
before (and following) secondary alveolar bone grafting. This
will help determine if there is a bony defect that requires graft-
ing as well as the nature and extent of any such defect. It is also
important to determine the usefulness of the teeth in the cleft
region and to identify any supernumerary, ectopic, and/or mal-
formed teeth. After the graft, one needs to understand the via-
bility of the new bone in terms of orthodontic tooth movement
and as a possible site for one or more osseointegrated dental
implants. These tasks are best accomplished using 3D imaging,
preferably low-dose CBCT, which provides more information
than 2D radiographs and can be used to construct the 2D images
that the orthodontist requires. The orthodontist and/or surgeon
must become proficient with one or more 3D viewing software
programs, such as Dolphin 3-D, in order to fully utilize the infor-
mation that can be derived from such a study (Fig. 12.14).
12.3.2 Management
Restoration of the dentition and treatment of malocclusions for
patients with cleft lip and palate require particular attention to
a number of issues specific to this group as well as an under-
standing of the overall nature of the treatments necessary,
whether they are dental, surgical, or other. It is imperative that
the orthodontist acts as a part of the cleft lip and palate team. A
coordinated and thoughtfully worked out, and executed, treat-
ment plan will ensure excellent care.
Fig. 12.13 (a) A patient with unilateral complete cleft lip and palate in “natural head position.” (b) Same patient oriented to Frankfort “horizontal”
(skeletal).
Dental and Orthodontic Management of Cleft Lip and Palate
125

In case of any cleft involving the alveolus where there is a
bony defect requiring surgical repair (most commonly complete
cleft of the lip/palate but also including cleft lip/alveolus with
intact secondary palate), cleft orthodontic treatment may be
divided into three phases. Phase I is the one that precedes the
secondary alveolar bone graft. Phase II is the phase that imme-
diately, or shortly thereafter, follows the graft. Phase III provides
definitive or end-stage orthodontia and is coordinated with
final restoration (prosthetic reconstruction) of the dentition
and orthognathic surgery, when indicated.
12.4 Cleft(s) Involving the
Alveolus
12.4.1 Phase I Orthodontics
Most patients with complete cleft lip and palate at this stage
present with a significant bony alveolar defect and a constric-
tion of the maxillary dentoalveolar arch, in which we see the
segment(s) collapsed more severely anteriorly than posteriorly
(Fig. 12.15). In unilateral clefts, there is often more collapse
on the cleft side than on the noncleft side. There may be tooth
anomalies such as agenesis and dysplasia of a tooth or teeth
(most commonly a missing lateral incisor and a dysplastic
central incisor adjacent to the cleft), supernumerary teeth,
ectopic teeth, and/or malformed teeth. There is often an ante-
rior crossbite reflecting a developing maxillary retrognathism
and palatal tipping and/or rotation of the maxillary incisors
(Fig. 12.16 and Fig. 12.17). In bilateral complete clefts, the
entire premaxilla may be malposed. It is not rare to find a pat-
ent or blocked oral–nasal fistula still present at this time.
The goals of phase I orthodontic treatment are to position
the dentoalveolar segments so as to approach ideal maxillary
arch form and width, to provide the surgeon with a better
access to the graft site, and to begin preparation of the occlu-
sion for definitive treatment. It most often takes place in the
mid to late mixed dentition, usually between 9 and 11 years of
age. Most surgeons perform the alveolar bone graft when the
cleft-side canine(s) has formed between half and two-thirds of
its root. Orthodontic treatment in the mixed dentition should
be started early enough to ensure that maxillary expansion,
alignment, and retention are completed by this time. Eruption of
the canine into the grafted bone will help ensure viability of the
bone graft, which will, in turn, provide adequate bony support
for the adjacent teeth and allow for later phases of orthodontic
treatment to be successfully performed. It should be noted that,
occasionally, a tooth other than the canine might dictate graft
timing, following the same principle as the canine would.
Recognizing that the maxillary constriction found in a patient
with cleft is usually asymmetric implies that maxillary expan-
sion should likewise be asymmetric. The ideal device to accom-
plish this is the one that expands the maxilla di erentially
(anterior greater than posterior) in a concise and predictable
manner. In case of unilateral complete clefts, expansion before
grafting allows for greater expansion of the lesser segment,
resulting in a more symmetric maxillary arch, more closely
approximating the form of the mandibular arch. At Boston
Children’s Hospital, we employ the use of a “fan”-type expan-
sion screw in a fixed maxillary expansion appliance, the di er-
ential MEA (Fig. 12.18 and Fig. 12.19).
Fig. 12.14 Cone beam computed tomography of right unilateral
alveolar defect.
Fig. 12.15 Maxillary occlusal radiograph of left alveolar bone defect.
Fig. 12.16 Typical clinical presentation—left unilateral complete cleft
lip and palate, early mixed dentition.
Cleft Lip and Palate
126

The abutment teeth for this appliance are usually the first
permanent molars and the first primary molars; however, other
teeth in similar relative positions may be used if desired or nec-
essary. By varying the position of the fulcrum (hinge) of the
MEA, the ratio of anterior versus posterior expansion can be
reliably programmed, so that maxillary expansion results in the
proper maxillary arch form and width. In this manner, overex-
pansion of the permanent molars that might result from the
use of a symmetrical expansion appliance is unlikely to occur.
The multiple adjustments necessary to manage other often-
used expansion devices, such as the “quad-helix” or the “Porter
arch,” are also not an issue. Following expansion, the appliance
is removed and replaced with a fixed transpalatal arch, with
palatal arms extending anteriorly, which should remain in place
through the time of the bone graft (Fig. 12.20).
The magnitude of the expansion desired requires careful
analysis of the diagnostic records and an understanding of the
likely growth pattern of the patient. A significant number of
patients with complete cleft lip and palate become candidates
for surgical maxillary advancement at the end of their growth.
This is often indicated to provide the best facial aesthetics pos-
sible in light of their maxillary deficiency as well as to minimize
dental compensations that would otherwise be necessary to
achieve a satisfactory occlusion. A common error made by inex-
perienced orthodontists is overexpansion in phase I, done to
widen the maxillary arch to correct apparent constriction,
while failing to account for the future advancement of the max-
illary dental arch.
Orthodontic movement of anterior teeth before bone grafting
may be considered when strong indications exist, such as the
relief of traumatic occlusion and providing the surgeon with
Fig. 12.17 Panoramic radiograph—right unilat-
eral cleft lip and alveolus.
Fig. 12.18 Differential maxillary expansion appliance—preactivation.
Fig. 12.19 Differential maxillary expansion appliance—postactivation.
Fig. 12.20 Transpalatal arch—postexpansion retention device.
Dental and Orthodontic Management of Cleft Lip and Palate
127

access to the graft site, and only when adequate bone between
root and defect is confirmed by using 3D imaging. Great care
should be taken as the root of any incisor adjacent to a bony
defect is often separated, on some portion of its root from the
defect by very little or no bone. The distopalatal aspect of cleft-
side central incisor should be examined carefully on CBCT by
reorienting the study along its long axis and viewing it in all
three planes of space.
Orthodontic tooth movement of the incisor or incisors in
closest approximation to the cleft may lead to perforation of a
significant portion of the root into the defect. If this occurs, it
will likely lead to compromise of the graft and/or loss of the
tooth.
The application of an orthopedic force by means of a reverse-
pull headgear (facemask) may be considered as part of this
phase of treatment. Several protocols for its use have been
advocated and may be considered in cases of mild to moderate
maxillary deficiency. This treatment can often be combined
with maxillary expansion and should always be done with care
being exercised not to overly compensate the dentition (exces-
sively tip the maxillary incisors forward). Increased burden of
care, lack of cooperation, and uncertainty of the outcome are
the factors that discourage the use of these appliances in some
patients (Fig. 12.21).
For patients with bilateral complete cleft lip and palate, a
premaxillary osteotomy may be required in order to better
position a particularly malposed premaxilla that cannot be cor-
rected orthodontically (as is sometimes possible by using
orthodontic forces on a mobile premaxilla). The premaxilla may
be severely vertically malposed (usually inferiorly) and/or
rotated in the sagittal plane (anterior nasal spine forward and
the incisal edges of the incisor teeth backward).
Less frequently, it may be severely deviated to one side or
canted in the coronal plane. The premaxillary osteotomy can be
done at the same time as the bone grafts, and care should be
taken by the surgeon so as not to position the premaxilla as
superiorly as may seem indicated given that it will usually fail
to grow vertically to the same degree as the remainder of the
maxilla. The use of both acrylic and orthodontic wire splints
will help ensure the stability of the premaxillary segment and
the success of the bone graft.
12.4.2 Phase II Orthodontics
The purpose of phase II is to utilize, maintain, and support
development of the grafted bone by means of introducing the
mechanical stimulation provided by erupting and/or function-
ing permanent teeth into the graft. This is done by guiding
eruption of the canine(s) into the region and/or orthodontically
moving teeth adjacent to the site into the newly placed bone,
thus encouraging the lengthening and thickening of the alveo-
lar bone and preventing the thinning and shortening (atrophy)
that naturally occur over time in any edentulous area. Ortho-
dontic treatment in this phase should also strive to further
develop good maxillary arch form and width, align teeth,
improve esthetics, and prepare for and simplify definitive treat-
ment at a later stage.
The orthodontist must understand that the natural course of
jaw growth in patients with complete (involving the primary
and secondary palate) cleft is such that maxillary grow th has
likely been and will continue to be deficient, resulting in an
increasing relative horizontal retrusion of the maxilla to the
mandible and to the upper face. As the patient matures, any
underbite or open-bite discrepancies between the maxilla and
the mandible are likely to worsen, as the mandible grows but
the maxilla does not (Fig. 12.22).
For patients with cleft of the lip and alveolus not involving
the secondary palate, maxillary growth has likely been and will
continue to be more favorable than in patients with complete
cleft. This understanding should be factored into decisions
Fig. 12.21 (a) Reverse-pull headgear. (b) Lateral cephalometric radiograph—bilateral complete cleft lip and palate before premaxillary osteotomy.
Cleft Lip and Palate
128

about which patients might be amenable to nonsurgical treat-
ment and about the use of orthopedic forces during phase II.
For patients with a substantially unfavorable relationship
between the maxilla and the mandible (skeletal class III maloc-
clusion) at this stage, the knowledge that remaining grow th will
only worsen the problem signals that surgical orthodontic ther-
apy will be indicated in order to provide a satisfactory occlusion
and an esthetic profile. With that in mind, the orthodontist
should try to minim ize the amount and length of phase II, often
limiting the treatment to the maxillary arch, whenever
possible.
For patients with a favorable or marginally unfavorable rela-
tionship between the maxilla and the mandible, the orthodont-
ist may proceed with more substantial treatment during phase
II. However, before the completion of growth, it is quite di cult
to predict with certainty that a class III malocclusion would not
develop. Even a small change (as little as 1–2 mm) will likely
require orthodontic retreatment. More significant change could
require transition to surgical orthodontic treatment. Caution
should be taken to prevent acting on the natural desire that the
orthodontist has to use his or her skills to treat a malocclusion
that can be better treated in coordination with orthognathic
surgery with braces alone. Of particular concern is the margin-
ally class III patients who start phase II orthodontic treatment
with the hope that orthognathic surgery would not be required
and then begin to show an unfavorable growth pattern (puber-
tal growth spurt). At this point, the orthodontist should refrain
from further compensation of the teeth, given that continuing
to treat in this way, combined with continued unfavorable
growth, will leave the patient with a poor occlusion, severely
compensated teeth, and a skeletal discrepancy between the
jaws greatly exceeds the dental discrepancy. Teeth will then
need to be decompensated before orthognathic surgery. This
“round tripping” of teeth may lead to blunting of roots or even
loss of teeth.
A benefit of phase II treatment is the fact that it often takes
place during the time at which many other children are
undergoing orthodontics. This allows the patient with a cleft to
“fit in” with their contemporaries and reduces the feeling of
being di erent. In addition, the emotional value of improved
appearance, even if only a modest change during this phase,
should not be underestimated.
Phase II treatment is another good time to consider the possi-
ble benefits of treatment involving dentofacial orthopedics for
the patient with a cleft who has a developing class III malocclu-
sion that is not excessive in nature. If the patient and family
are willing to accept a compromise in the facial esthetics (pro-
file), dentofacial orthopedic treatment may prove useful in mit-
igating the developing class III malocclusion by encouraging
forward maxillary growth and/or discouraging forward man-
dibular growth. The e cacy of such treatment remains an area
of significant controversy within the orthodontic community,
as many believe the majority of the changes to be dentoalveolar
in nature and therefore the risk of excessive dental compensa-
tion (tipping of teeth to camouflage the skeletal malocclusion)
to be too high. If the patient’s family wants their child to have a
profile that approaches or reaches normal for their ethnic
group, dentofacial orthopedics is not indicated except in the
mildest manifestations of class III skeletal malocclusions. An
alternative to traditional dentofacial orthopedics is the use of
so-called “Bollard” plates, which are attached to the maxilla
and mandible directly, in the mandibular canine and maxillary
malar process regions, respectively, allowing the patient to
wear elastics without directly a ecting tooth positions but, pos-
sibly, a ecting maxillary position as far superiorly as the zygo-
matic arch (Fig. 12.23).
12.4.3 Phase III Orthodontics
The goals of definitive orthodontic treatment(s) are to provide
a stable, healthy, functional, and aesthetic dentition and occlu-
sion as well as to ensure good facial aesthetics. This phase of
treatment requires careful coordination with the team and
involves orthodontics, with or without orthognathic surgery,
Fig. 12.22 Unfavorable facial growth in a patient with unilateral complete cleft lip and palate.
Dental and Orthodontic Management of Cleft Lip and Palate
129

prosthodontics, and periodontics. It must also take into account
the facial changes that are likely to result from final lip or nasal
revision.
It is best to begin phase III with a decision having been made
as to whether or not orthognathic surgery is to be a part of
treatment. To proceed, the patient must be skeletally mature
enough, so that the team can be confident that there will be lit-
tle enough further change in the maxillomandibular relation-
ship to make such a choice.
Nonsurgical Orthodontics
The decision to provide definitive orthodontia without orthog-
nathic surgery does not mean that the orthodontist no longer
needs to communicate with and coordinate treatment with the
cleft team. Understanding the nature and expected outcome of
final lip revision and how that procedure will a ect tooth show,
at rest, during speech, and when smiling should be accounted
for by the orthodontist. Furthermore, any dental implants into
the grafted bone should be prepared for, as required by the cleft
team.
Surgical Orthodontics
An understanding of the planned operation is essential for the
orthodontist to prepare the patient properly for orthognathic
surgery. Vertical changes, often involving anterior maxillary
disimpaction and alteration in the occlusal plane, need to be
accounted for during orthodontic preparation, in order to
achieve the best possible outcome. Of particular concern is
achieving adequate forward incisor angulation and adequate
anterior arch width (intercanine, except really interpremolar in
this case) when the plan calls for canine substitution of missing
or extracted lateral incisor(s).
Surgical orthodontic treatment should begin no sooner than
the estimated time that the orthodontist will take to prepare
the patient for the operation. In addition, medical history,
hand–wrist bone age, and/or serial cephalometric radiographs
must ensure completion of skeletal growth by the time of sur-
gery (Fig. 12.24).
An ideal surgical orthodontic treatment plan will result in an
occlusal discrepancy equal to the amount of skeletal movement
required to achieve the desired soft tissue profile changes
(Fig. 12.25).
Frequent updates from the orthodontist and study models,
photos, and, less frequently, lateral cephalometric radiographs,
will assure the surgeon that the treatment is progressing as
planned and will produce a more accurate result. Following the
operation, the orthodontist continues treatment, using the
orthodontic appliances, to help provide stability in the immedi-
ate postsurgical period and then to detail the occlusion before
removing the braces.
12.4.4 Retention
Retention is important in every orthodontic case, but it is a sub-
ject of great concern in the treatment of patients with cleft lip
and palate, not only after definitive treatment but also after
each phase along the way. Maxillary expansion may be espe-
cially subject to relapse in patients with complete clefts of the
lip and palate, where transverse dimensional stability must be
supported for life due to the lack of a true secondary palate. The
use of a fixed transpalatal arch with anterior extensions cer-
tainly helps maintain the expansion achieved during phases I
and II, but after final treatment, the diligent use of a removable
retainer (part-time) is more practical.
12.5 Isolated Cleft of the Palate
Patients with the diagnoses of an isolated cleft palate (ICP) or
its associated subgroup, nonsyndromic Robin’s sequence (RS),
have a markedly di erent malocclusion than patients with cleft
of the lip and palate. This malocclusion is characterized by
bimaxillary skeletal retrusion, which is more pronounced in the
mandible in case of patients with RS.
12.5.1 Early Treatment (Mixed Dentition)
Early management of the malocclusion present in patients with
ICP and RS focuses on the dental crowding, most often found to
Fig. 12.23 Bollard plates with elastics in place. (This image is provided
courtesy of Heymann et al 2010.)
Fig. 12.24 H-W film.
Cleft Lip and Palate
130

be present, and the relative mandibular retrognathia seen in RS.
Crowding may be severe enough to warrant a “serial extraction”
protocol, whereby a series of primary and then secondary (per-
manent) teeth are extracted at selected intervals in order to
minimize the amount of orthodontic treatment. The use of a
“functional” appliance to encourage mandibular growth may be
considered when a class II skeletal and dental malocclusion
exists. The lack of predictable results and the risk of excessive
dental compensation are potential complications that should be
considered.
12.5.2 Definitive Treatment
When the retrusion of the maxilla and mandible are similar
enough in magnitude to bring the relative anteroposterior (AP)
malocclusion into a range treatable by orthodontics alone, the
treatment may follow a typical course of orthodontia. However,
if both jaws are significantly retrognathic, the child may be
predisposed to sleep apnea and/or may be dissatisfied with his
or her appearance. If one or both of these issues exist, bimaxil-
lary orthognathic surgery to advance both jaws may be indi-
cated. If one jaw is more retrusive than the other, it may be
reasonable to advance only that jaw, given that the other is only
mildly retrusive and no sleep apnea exists. If both jaws are only
mildly retrognathic, traditional orthodontic therapy should be
all that is required.
Suggested Readings
Allareddy V, Ross E, Bruun R, Lee MK, Shusterman S. Operative and immedi-
ate postoperative outcomes of using a Latham-type dentomaxillary appliance
in patients with unilateral complete cleft lip and palate. Cleft Palate Craniofac
J. 2015; 52(4):405–410
Bronkhorst A, Allareddy V, Allred E, Ross E, Shusterman S. Assessment of
morbidity following insertion of fixed preoperative orthopedic appliance in
infants with complete cleft lip and palate. Oral Surg Oral Med Oral Pathol Oral
Radiol. 2015; 119(3):278–284
Fig. 12.25 (a) A patient with left unilateral complete cleft lip and palate prepared for Le Fort I maxillary osteotomy. (b) Same patient with left
unilateral complete cleft lip and palate post Le Fort I maxillary osteotomy.
Dental and Orthodontic Management of Cleft Lip and Palate
131

Chan KT, Hayes C, Shusterman S, Mulliken JB, Will LA. The e ects of active
infant orthopedics on occlusal relationships in unilateral complete cleft lip
and palate. Cleft Palate Craniofac J. 2003; 40(5):511–517
Georgiade NG, Latham RA. Maxillary arch alignment in the bilateral cleft lip
and palate infant, using pinned coaxial screw appliance. Plast Reconstr Surg.
1975; 56(1):52–60
Latham RA. Orthopedic advancement of the cleft maxillary segment: a pre-
liminary report. Cleft Palate J. 1980; 17(3):227–233
Grayson BH, Santiago PE, Brecht LE, Cutting CB. Presurgical nasoalveolar
molding in infants with cleft lip and palate. Cleft Palate Craniofac J. 1999; 36
(6):486–498
Matic DB, Power SM. The e ects of gingivoperiosteoplasty following alveolar
molding with a pin-retained Latham appliance versus secondary bone graft-
ing on midfacial growth in patients with unilateral clefts. Plast Reconstr Surg.
2008; 122(3):863–870, discussion 871–873
Mulliken JB, Martínez-Pérez D. The principle of rotation advancement for
repair of unilateral complete cleft lip and nasal deformity: technical varia-
tions and analysis of results. Plast Reconstr Surg. 1999; 104(5):1247–1260
Spolyar J, Jackson IT, Phillips RJL, Sullivan WG, Clayman L, Vyas ST. The
Latham technique: contemporary presurgical orthopedics for the complete
oral cleft technique and preliminary evaluation—a bone marker study. Semi-
nars in Plastic Surgery . 1992; 6(1):179–210
Long RE, Semb G, Shaw WC. Orthodontic treatment of the patient with com-
plete clefts of lip, alveolus, and palate: lessons of the past 60 years. Cleft Pal-
ate Craniofac J. 2000; 37(6):533–533
Nishihara K, Nozoe E, Maeda A, et al. Outcome following secondary autoge-
nous bone grafting before and after canine eruption in patients with unilat-
eral cleft lip and palate. Cleft Palate Craniofac J. 2014; 51(2):165–171
Heymann GC, Cevidanes L, Cornelis M, De Clerck HJ, Camilla Tulloch JF.
Three-dimensional analysis of maxillary protraction with intermaxillary elas-
tics to miniplates. Am J Orthod Dentofacial Orthop. 2010; 137(2):274–284
Cleft Lip and Palate
132