1Departments of Plastic and Reconstructive Surgery,
and 2Department of Dermatology, The University
of Tokushima School of Medicine, Tokushima, Japan;and 3Department
of Orthodontics, The University of Tokushima School of Dentistry,
Tokushima, Japan
Abstract: Distraction osteogenesis has been applied to the
craniofacial skeleton as well as the long bones of the extremities.
This technique does not require bone grafting and allows
correction of craniofacial deformities with less invasion.
Moreover, the distraction procedures can expand the overlying
soft tissues simultaneously. We determined the indications
of distraction osteogenesis, analyzed the types of devices
available, and examined patients treated with distraction
for the mandible, midface, and cranium. In all three sites,
the devices tended to be the buried type and made of absorbable
materials. Administration of some cytokines for shortening
the consolidation period may be considered. Among disorders
indicated for distraction osteogenesis, there are several
syndromic craniosynostoses, which involve mutations in the
fibroblast growth factor receptor (FGFR) 2 gene. The FGFR
2 mutation was suggested to clinically accelerate osteogenesis
at the distraction site. The usefulness and appropriateness
of the distraction protocol must be assessed for each individual
disorder. Although distraction osteogenesis in the craniofacial
skeleton has advanced technologically, all possible risks
must be discussed with the patient and family members when
obtaining preoperative informed consent, especially until
establishment of fully safe distraction procedures. J. Med.
Invest. 50:117-125, 2003
Keywords:distraction osteogenesis, craniofacial skeleton
INTRODUCTION
The technique of generating new bone by gradual distraction
of a callus was first described in 1905 by Codivilla (1).
The early procedures were complicated by edema, skin necrosis,
infection, deviation, and delayed ossification of the expanded
segment (2). This technique remained undeveloped until Ilizarov
rekindled interest in the process of bone lengthening (3-5),
and several studies reported significant advances in reducing
the incidence of complications associated with bone lengthening
(6-10). Bone lengthening by distraction osteogenesis has
been successfully applied to the enchondral bone of the
upper and lower extremities.
In the craniofacial region, the initial description of the
distraction osteogenesis technique should be credited to
Rosental, for bone lengthening of the mandible in a microgenia
patient in 1927, and Wassmund, for the clinical advancement
of a maxilla in a patient with hypoplasia of the upper jaw
in 1926 (11). However, distraction osteogenesis only really
came to be seen as a promising and popular method in the
reconstruction of the membranous bones of the human craniofacial
skeleton after McCarthy et al. reported in Western literature
the clinical application of mandibular lengthening by gradual
distraction in patients with hemifacial microsomia and Nagers'
syndrome in 1992 (12). Recently, distraction osteogenesis
procedures were also applied to the midface and cranium
in addition to the mandible (13-16).
The technique of the distraction osteogensis does not require
bone grafting and enables correction of craniofacial deformities
with less invasion. Furthermore, a unique feature of the
distraction technique is that bone regeneration by distraction
osteogenesis is accompanied by simultaneous expansion of
the functional soft tissue matrix, including blood vessels,
nerves, muscles, skin, mucosa, fascia, ligaments, cartilage
and periosteum (17). Consequently, the use of the distraction
technique is now wide-spread in the field of craniofacial
surgery. We present the indications of distraction, the
types of devices used, and the history of patients treated
with distraction for the mandible, midface, and cranium.
MANDIBLE
Clinical application of the distraction technique to the
craniofacial skeleton first began in the mandible (12).
Mandibular elongation and remodeling by corticotomies and
distraction are relatively simple procedures with minimal
morbidity and complications. This technique in the mandible
inevitably involves the condition of occlusion and has been
established in cooperation with orthodontists. The conditions
that indicate mandibular distraction include hemifacial
microsomia, segmental bone defects after trauma and tumor,
congenital micrognathia, Class II occlusion, Treacher-Collins
syndrome, and Pierre Robin sequence.
The age at which distraction is applied varies from 6 days
to 64 years of age, and depends on the aims of treatment,
which are mainly related to the airways, occlusion, and
appearance (17). Children with craniofacial anomalies are
predisposed to airway obstruction and frequently require
airway intervention. For example, the Pierre Robin sequence
was reported to require tracheotomy or endotracheal intubation
in 12.2% or 42%, respectively, of those affected, with an
average age of 3.1 years at decannulation (18, 19). Mandibular
distraction has been shown to alleviate airway obstruction
in the pediatric population and is an alternative method
with which to avoid long-term tracheotomy associated with
significant morbidity. This treatment was reported to be
successfully carried out in a neonate with acute airway
obstruction at birth (20). In micrognathia without airway
obstruction, the mandible is distracted frequently between
2 and 12 years of age to correct occlusion and appearance
(17).
The apparatus used for mandible distraction have advanced
technologically. In the first study, a standard external
fixator with two double pin holders was used (12). With
the accumulation of clinical experience, two main problems
became apparent. One was the limitations of uniplanar distraction
for the three-dimensional reconstruction of the deficient
mandible (e.g., the precise correction of malocclusion or
the increase in bigonial distance). To solve this problem,
a multiplanar distraction device was developed, and its
effectiveness demonstrated (21). The other problem was residual
scars along the cheek which resulted from half-pins cutting
through the skin during active lengthening. This led to
the development of intraoral distractors. Diner et al. reported
that insertion of the submerged intraoral distraction apparatus
in juvenile cases of mandibular hypoplasia yielded excellent
results (22).
Complications of mandibular distraction include pin loosening,
accidental trauma, transient hypesthsia of the inferior
alveolar nerve, minor local infection, premature consolidation,
and transient weakness of the facial nerve (17).
We present a 7-year-old girl with Treacher-Collins syndrome
who underwent distraction of the mandible. The patient had
marked micrognathia (Figure 1). We simulated the mandible
distraction using a three-dimensional model of the patient's
head build from the preoperative computed tomography data
(Figure 2). She was eventually able to take foods that were
too hard to eat prior to treatment, and her profile was
markedly improved (Figure 3).
MIDFACE
A standard midface advancement consists of simultaneous
fixation following Le Fort osteotomy, but gradual distraction
of the midface has been used increasingly for midfacial
advancement (23). Soft tissues resistance to advancement
of the osteotomized fragment is an issue in conventional
osteotomy and rigid fixation for advancements greater than
10 mm, but distraction procedures can solve the problem
of resistance, which leads to postoperative relapse. Among
the types of Le Fort osteotomy preceding distraction, type
I osteotomy has been the most frequent and has been applied
to adult patients with severe midfacial hypoplasia secondary
to cleft lip and palate (17). Type III osteotomy has been
performed mainly on patients with syndromic craniosynostosis
disorders such as Apert, Crouzon, and Pfeiffer syndrome.
The Le Fort III osteotomy is justifiably indicated during
early childhood for psychological and physiologic reasons
(24). The distraction procedure can make the procedures
less invasive and allows midface advancement at almost any
age, including early childhood (13). However, several craniofacial
surgeons have recommended waiting until after 4 years of
age due to the fragility of the bone (25).
Polley and Figueroa used an external, adjustable, rigid
distraction device for midface advancement of patients with
cleft lip and palate (14). The device, which was mounted
as a halo onto the temporal and frontal portion of the skull,
served to distract the midface after osteotomy by anchoring
to the maxillary dental arch. Subsequently, an internal
device was developed, which had better patient acceptance
and ease of integration into an overall treatment plan (26).
Moreover, a new system of miniature distraction devices
that can be implanted internally and accessed through a
small percutaneous screw was also reported (27). Internal
distraction devices can achieve only unidirectional distraction
after the placement, whereas the advantage of the external
devices is the ability to direct the direction of distraction
during the advancement process.
We treated midface hypoplasia in a 20-year-old female with
Crouzon syndrome using a rigid external distraction device.
The patient showed severe exophthalmos and maxillary retrusion,
although relatively good occlusion had been achieved by
long-term orthodontic procedures (Figure 4). It was suggested
that this patient's particular condition could not be resolved
by the usual Le Fort III osteotomy/midface distraction procedure,
so we devised a segmental approach. The midface, mobilized
by Le Fort III osteotomy, was divided into two segments
by Le Fort I osteotomy;each fragment was connected to the
rigid external distraction device to be distracted separately.
Distraction at a rate of 1 mm per day was begun 1 day after
the osteotomy. The upper and lower segments were distracted
over 17 and 12 days, respectively. The patient's occlusion
was fully corrected, and her facial contours were significantly
improved. After 3 weeks of consolidation, we removed the
distraction device (Figure 5). The clinical course was without
complication, and no relapse was observed on the cephalogram
or computed tomographic scan obtained 1 year after the procedure
(Figure 6). Our modified technique was helpful in increasing
the usefulness of the external distraction system and in
refining the midface distraction procedure(28).
Hemifacial microsomia is the second most common craniofacial
anomaly after cleft lip and palate. In this group, mandibular
hypoplasia may be associated with limitation of the vertical
growth of the maxilla. In children with hemifacial microsomia,
the occlusal changes after distraction of the mandible alone
can be corrected orthodontically because there is rapid
vertical growth of the maxilla when it is released from
the constricting effect of the mandible and soft tissues
(29). In the older patients, who have slanted occlusal planes
but for whom dental occlusion is efficient and stable before
the treatment, mandibular distraction alone alters dental
occlusion severely and requires unacceptably prolonged orthodontic
treatment. Therefore, simultaneous distraction of the mandible
and maxilla is indicated to correct the facial asymmetry
without disturbing the pre-existing dental occlusion (30,
31).
CRANIUM
Craniosynostosis is the term that designates premature fusion
of one or more sutures in either the cranial vault or cranial
base. The goals of surgery for the newborn with a craniosynostosis
are twofold. One is decompression of the intracranial space
to reduce intracranial pressure, prevent visual problems,
and permit normal mental development. The other is achievement
of satisfactory craniofacial form. Recently, surgical treatment
has been performed within the first year of life, and the
early effort has resulted in the children looking as good
as possible at as early an age as possible to spare the
psychological and social trauma associated with craniofacial
disfigurement. The surgical treatment includes strip craniectomies,
frontal bone advancement, cranial vault remodeling, monobloc
or craniofacial advancement, and shunt surgery for hydrocephalus
(32).
Gradual distraction was used in fronto-orbital advancement
and cranial vault remodeling (33-35). The major advantages
of gradual distraction were reported to be the elimination
of postoperative extradural dead space, preservation of
the blood supply of the bone flap by not detaching the underlying
bone from the dura, and the concomitant expansion of the
scalp enabling easy closure of the wound (33). However,
the benefits of application of distraction for purely cranial
reshaping, which includes fronto-orbital advancement and
correction of the plagiocephaly, are ambiguous at present
(34, 36). However, distraction for scaphocephaly is indicated
(34, 37).
We reported a case of a 1-year-old boy who underwent correction
of a scaphocephalic deformity using distraction devices
(Figure 7). Double pi-shaped osteotomies were cut, bilateral
temporal bone flaps were gradually expanded, and the frontal
bone was pulled back simultaneously (Figure 8). The patient
showed a good skull contour 2 years postoperatively (Figure
9). Although previous distractions were aimed only at expansion
of the cranium, we used the distraction technique to successfully
shorten the cranial vault in the anteroposterior direction.
This case demonstrates that the osteotomized bone flaps
can be moved in various directions. This feature of the
distraction technique may contribute greatly toward meticulous
reconstruction of the cranial vault (37).
RECENT ADVANCES
Distraction procedures in craniofacial surgery have some
disadvantages, including that the devices may produce scars,
a second operation to remove the devices is necessary, the
treatment period is prolonged, infection may occur near
the devices, and minute correction is impossible.
Biodegradable devices made of polylactic acid have been
developed to abridge the second operation for removal and
stabilization (38, 39). The relatively rapid resorption
of the implanted components should not interfere with facial
growth or provide a nidus for chronic infection. Use of
the resorbable devices is a step forward in the evolution
of distraction osteogenesis (39).
Various agents have been theoretically and experimentally
implicated as mediators of distraction osteogenesis. To
lessen the time the distraction device is in place on the
patient and to accelerate the entire distraction process,
a cannulated pin that can provide a route for administering
the agents to the precise anatomic point at the optimal
time has been developed (40). In addition, the effects of
hyaluronic acid, calcium sulfate, and chitosan on early
bony consolidation in distraction osteogenesis have been
studied in a canine model (41). Calcium sulfate and its
combined materials were found to be relatively effective
in early bony consolidation. The other materials such as
several kinds of growth factors may apply to the distraction
site in the future.
Treatment protocols for craniofacial distraction osteogenesis
have not yet been established, although there is some degree
of consensus (17). Among disorders indicated for distraction,
there are several syndromic craniosynostoses, which have
mutations in the fibroblast growth factor receptor (FGFR)
2 gene (42-45). Although the relation between the FGFR 2
gene and osteogenesis has not been clarified, some studies
have suggested that the FGFR 2 mutation accelerates the
growth or differentiation capability of osteoblasts (46-48).
Clinical studies of the distracted thumb in patients with
or without the mutation in the FGFR 2 gene has suggested
that osteogenesis at the distraction site may be quicker
in Apert syndrome patients with the mutation than in polydactyly
patients without the mutation (49). Thus, the efficacy and
appropriateness of the distraction protocol must be assessed
for each individual disorder.
Distraction osteogenesis in craniofacial surgery has been
developed, however, its procedures have not been fully established.
The indications for distraction must be carefully considered,
and the risks of the procedures thoroughly discussed when
obtaining preoperative informed consent from prospective
patients
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