Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Treatment and Complications
Management protocols for the surgical treatment of craniofacial fibrous dysplasia differ. On the radical
end, complete excision of all diseased bone with reconstruction is advocated, whereas on the more
conservative end, skeletal reshaping with burring to restore normal skeletal morphology is performed. 2,9
Between these two surgical protocol extremes, limited resection (without sacrificing or injuring vital
structures) with osseous reconstruction is offered (Figure 35.2). Chen and Nordhoff based their treatment
approach to the occurrence in four designated zones in the craniofacial skeleton 2 :
Total excision for zone 1 (fronto-orbital, zygomatic, and upper maxillary regions)
Conservative excision (contouring) for
Zone 2 (hair-bearing cranium),
Zone 3 (the central cranial base), and
Zone 4 (the tooth-bearing regions of the maxillary alveolus and mandible).
FIGURE 35.2 Fibrous dysplasia involving the left orbit, temporal, and zygomatic, regions (polyostotic). A. Preoperative frontal
image demonstrating vertical dystopia and temporal fullness. B. Postoperative frontal image improvement after orbital box
repositioning and recontouring of temporal bone. (Courtesy of H.K. Kawamoto.)
When comparing limited resection with reconstruction to bone contouring, patients undergoing resection
had a lower recurrence rate and required fewer total operations but had a slightly increased
postoperative complication rate. 9 Medical adjuncts for fibrous dysplasia hold an adjunctive role only and
include bisphosphonates, steroids, and tamoxifen. 2 Radiotherapy has no role in treatment of fibrous
dysplasia and should be avoided.
Optic nerve decompression for optic canal involvement is controversial. Optic canal decompression
without any vision loss is generally deemed too risky even with radiographic evidence of involvement.
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2,10 However, when vision loss is first noted, optic canal decompression is advised. The outcome of optic
nerve decompression ranges from (1) halting vision loss, (2) improvement in vision, to (3) worsening in
vision. 2,9
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Parry-Romberg Syndrome
Definition and Clinical Findings
Parry-Romberg syndrome is characterized as a progressive hemifacial hypoplasia with regional fat
atrophy, medial canthal malpositioning, enophthalmos, dystopia, and skin hyperpigmentation. 11 It
manifests as unilateral atrophy of the subcutaneous facial fat in the paramedial area with equal frequency
on both sides of the face. Atrophy of the skin, muscle, fascia, cartilage, and bone may follow (Figure 35.3).
The most evident sign is loss of fullness in 3-D parameters (atrophy of orbital, palpebral, zygomatic, and
masseter muscles). The classic linear vertical deficiency is called coup de sabre sign (or mark of the sword). 12
FIGURE 35.3 Romberg syndrome with left side involvement. A. Preoperative image shows C-shaped linear coup de sabre
deficiency. B. Postoperative image shows correction after serial fat grafting, septorhinoplasty, and lateral canthoplasty.
(Courtesy of H.K. Kawamoto.)
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Etiology and Pathogenesis
Parry-Romberg syndrome results in progressive hemifacial atrophy and may be highly variable.
Although primarily characterized by soft tissue involvement including fat, skin, muscle, and connective
tissue, in more severe cases, bony, ophthalmological, and/or neurological involvement occurs (Figure
35.4). Onset can vary from infancy, to early or mid-childhood, to adolescence, or even adulthood. Rate of
progression of disease is also unpredictable and may vary. Some (but not all) cases are believed to
undergo remission or burnout after puberty. 13
FIGURE 35.4 Romberg syndrome with left side involvement. A. Preoperative image shows both hard and soft tissue disruption
with left vertical orbital dystopia, nasal deviation, and skin changes. B. Postoperative image shows correction after left orbital
box elevation, lateral canthoplasty, septorhinoplasty, fat grafting, and face-lift. (Courtesy of J.P. Bradley.)
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Treatment and Complications
Current treatment options to fill deficient tissues include fat or dermal grafts, dermal fillers (hyaluronic
acid or calcium hydroxyapatite), bone and/or cartilage grafts, and/or free tissue transplantation. 11 Many
authors advocate waiting for the disease to reach a stable phase after facial growth completion in late
adolescence before beginning surgical procedures. 14 Burnout of disease may not occur for 10 years or
more. By contrast, children living with the deformity, while waiting for surgical correction, may have
psychosocial difficulties at home and school.
Patients with mild to moderate deformity are typically treated with serial fat grafting procedures because
of the effectiveness and low risk. Fat grafting is preferred to injection of silicone and other synthetic
injectables because of higher rates of infection, seroma, capsule formation, exposure, migration, and long-
term scar formation. The take of fat grafting in the diseased Romberg region is less than fat graft take in
normal regions. 11 Interestingly, the fat graft take improves in subsequent fat grafting procedures for
Romberg patients. This improved take is thought to be due to improved vascular milieu of the grafted
bed. For skeletally mature patients with severe deformity, dermal fat grafts or adipofascial free flaps
(such as a parascapular or inframammary extended circumflex scapular [IMECS] flaps) are accepted
alternatives 14 (Figure 35.5). The back region provides better adipose-fascial fill for facial contouring than
that of other free flaps. Although excellent results may be obtained with an IMECS flap, this is a major
procedure compared with fat grafting, and subsequent revisions or debulking procedures are typically
necessary. In more severe manifestations of the disease, skeletal reconstruction and soft tissue
rearrangement are required to restore anatomic landmarks and facial symmetry.
FIGURE 35.5 Romberg syndrome with left side involvement. A. Preoperative image shows significant soft tissue atrophy,
nasal deviation, and skin changes. B. Postoperative image shows correction after IMECS flap, lateral canthoplasty, and
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composite graft to nose. (Courtesy of H.K. Kawamoto and S.W. Seibert.)
In summary, Romberg patients required multiple corrective surgeries but showed improvements even
when beginning before puberty. Fat graft take is poorer in diseased regions compared with normal
regions, but the procedure is still beneficial. For severe Romberg deformities, soft and hard tissue
reconstruction is necessary to improve symmetry.
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Treacher Collins Syndrome
Definition and Clinical Findings
Treacher Collins syndrome (TCS) is generally characterized by bilaterally symmetrical dysostosis of
craniofacial structures derived from the first and second branchial arches. 15 Although there is a wide
variation in phenotypic expression, common clinical features include hypoplasia of the zygomatic
complex and the mandible, antimongoloid slanting of palpebral fissures, coloboma of the lower eyelids,
complete or partial cleft palate, and atresia of external ear canals with abnormalities of the external ears
accompanied by conductive hearing loss 15-17 (Figure 35.6).
FIGURE 35.6 Treacher Collins syndrome. A. Preoperative image shows bilateral symmetric malar deficiency, down-slanting
lateral canthus. B. Postoperative image shows correction after cranial bone grafting to zygomatic arches, lateral orbital wall,
and upper to lower eyelid switch flaps. (Courtesy of J.P. Bradley.)
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Etiology and Pathogenesis
Genetic, physical, and transcript mapping techniques have demonstrated that approximately 93% of TCS
is caused by loss-of-function mutations in the TCOF1 gene (for Treacle) located on chromosome 5 (5q32-
33.1). 18,19 TCS resulting from mutations in the TCOF1 gene is inherited in an autosomal dominant fashion,
with only 40% of patients inheriting a mutated copy of the gene and 60% of cases arising from de novo
mutagenesis. 19,20 On a cellular basis, TCOF1 knockout mice have a diminished number of migrating
neural crest cells from extensive apoptosis and decreased proliferation.
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Treatment and Complications
The treatment of TCS usually begins during infancy (when airway management at birth may be
required), continues during mid-childhood (with bilateral total ear reconstruction and malar/orbital bony
and soft tissue reconstruction), and is completed at facial maturity (with orthognathic procedures and a
septorhinoplasty) (Figure 35.7).
16,17 In the newborn, severe airway obstruction may require a
tracheostomy (the standard). If obstruction is limited to the tongue base and epiglottis, mandibular
distraction can be effective in the newborn age or at a later age if sleep apnea develops. Choanal atresia,
obstructing the posterior nasopharyngeal airway, may also need to be treated with dilatation or bony
removal. Before 2 years of age, other procedures may be required, including cleft lip repair, cleft palate
repair, macrostomia (Tessier no. 7 cleft), or removal of preauricular skin tags. In addition, optimizing
hearing with BAHA (bone-anchored hearing apparatus) is beneficial in early childhood.
FIGURE 35.7 Treacher Collins syndrome. A. Preoperative image shows bilateral anterior open-bite malocclusion and
symmetric malar deficiency. B. Postoperative image shows correction after bilateral mandibular distraction (for tracheostomy
removal), cranial bone grafting to zygomatic arches, lateral orbital wall, and upper to lower eyelid switch flaps. (Courtesy of J.P.
Bradley.)
In mid-childhood (ages 6–9 years of age), corrective procedures may include 16 :
mandibular distraction lengthening
malar, zygomatic, and eyelid reconstruction
total external ear reconstruction
Both distraction and total ear reconstruction require staged procedures in which fat grafting may be
added at each stage. 21 This addition of soft tissue to the malar region helps while waiting on malar
reconstruction, especially when adverse psychosocial peer experiences are occurring.
For mandibular distraction, a vertical vector is recommended to correct the short ramus; strong guiding
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elastics are used to close the open bite and improve the obtuse gonial angle. Relapse of this abnormal
mandibular morphologic shape has been noted in long-term follow-up from the strong genioglossal
muscle pull. Relapse and malocclusion may be addressed later during an orthognathic procedure.
For total ear reconstruction, the two-stage Firmin technique is preferred unless there is paucity of skin as
in a very low lobule or anotia. 16 For these patients, the four-stage Brent technique may be used. Laser hair
removal is performed for low-set hairlines and preauricular sideburns before first-stage ear
reconstruction.
Malar/orbital reconstruction should not be attempted too early to avoid premature resorption of bone
grafts. At age 8 to 12 years, malar/orbital reconstruction may be undertaken with full-thickness parietal
skull bone grafts for the zygomatic arch and lateral orbit, with split thickness grafts for the orbital floor,
and with simultaneous upper to lower eyelid switch flaps with lateral canthopexies. 22
After facial skeletal maturity (16–18 years), correction of residual jaw abnormalities using orthognathic
techniques and a septorhinoplasty are performed. Although a common procedure to close an open bite is
Le Fort I posterior impaction, for the Treacher Collins patient, this will likely worsen the airway and
aesthetics because of the short cranial base and abnormal mandible. Tessier described l’integrale
procedure combining a Le Fort II osteotomy, bilateral mandibular advancement, orbital bone grafting,
and genioplasty for the severe, uncorrected form of TCS with airway compromise. With distraction
osteogenesis, similar osseous movements may be performed while reducing the relapse. 23 Genioplasty
distraction with hyoid advancement has also been used for airway improvement and reduces relapse
from genioglossal downward pull. 24 In summary, TCS remains a difficult entity to treat, often requiring
multiple-staged procedures throughout childhood for functional and aesthetic improvements.
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Neurofibromatosis
Definition and Clinical Findings
Neurofibromatoses are a common group of conditions separated into five types, of which type I and type
II are the most relevant for plastic surgery. Neurofibromas are benign peripheral nerve sheath tumors
and may appear as discreet masses or as diffuse, plexiform masses without distinct borders.
Clinical manifestations of neurofibromatosis are detected at birth in about 40% of patients, and over 60%
have manifestations by the second year of life starting with café au lait spots. Café au lait spots are
pigmented macules present in over 99% of patients with neurofibromatosis. They are frequently present
at birth and increase in size in childhood. Cutaneous neurofibromas appear around puberty and increase
throughout life. Approximately one-third of patients develop plexiform neurofibromas and about 6%
may develop malignancy. Neurologic problems occur in 10%, scoliosis in 5%, learning disabilities in 25%,
and intellectual developmental delay in 8% to 9%. In young children, macrocephaly without
hydrocephalus, short stature, hypertelorism, and thoracic abnormalities are frequently found.
Malignant peripheral nerve sheath tumors, also called neurosarcoma or neurofibrosarcoma, may develop
in patients, particularly those with plexiform neurofibromas. The lifetime risk of development is 5% to
10% with the mean age of diagnosis at 41 years of age. Pain is the most reliable sign of malignant
transformation.
Neurofibromatosis, type I (NF1): NF1 accounts for 90% of all cases. Clinical diagnosis is suspected
when six or more café au lait spots, cutaneous neurofibromas, and melanocytic hamartomas
projecting from the surface of the iris (Lisch nodules) are found. 25
Neurofibromatosis, type II (NF2): NF2 is characterized by bilateral acoustic neuromas with hearing
loss usually present as a teenager or young adult. Café au lait spots and cutaneous neurofibromas are
present but usually less common than NF1. Plexiform neurofibromas and central nervous system
tumors may also be present occasionally.
Craniofacial plexiform neurofibromas: Craniofacial involvement in neurofibromatosis is a
significant reconstructive challenge, as it is likely to be plexiform in nature with widespread
destruction of facial structures and potential intracranial extension. Two types of craniofacial
neurofibromas are generally considered separately:
Orbitotemporal neurofibromas
Plexiform neurofibromas resulting in hemifacial hypertrophy
The specific variety of neurofibromas localized to the orbitotemporal region involves the orbit, the
eyelids, and the temporal area (Figure 35.8). It may have an intracranial component associated with
headaches and seizures. 26 Manifested during childhood with lid swelling, it becomes worse over time
with severe involvement of the subcutaneous tissues of the eyelids and variable degrees of ptosis and
proptosis of the globe (see Figure 35.8). In the most severe of cases, involvement of the globe leads to
blindness. The skeletal changes in orbitotemporal neurofibromas consist of an absence of the greater wing
of the sphenoid and a defect of the posterolateral wall of the orbit or an enlargement of the superior
orbital fissure. The orbit is enlarged and hypoplasia of the supraorbital and infraorbital rims is present.
The zygoma is hypoplastic and the orbital floor is inferiorly positioned.
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FIGURE 35.8 Neurofibromatosis type 1: left orbital involvement. A. Preoperative image shows vertical dystopia; patient had
pulsatile globe. B. Postoperative image shows correction after left orbital box osteotomy with elevation and orbital roof
reconstruction. C. Preoperative coronal CT scan depicts absent orbital roof (arrow). D. Postoperative coronal CT scan depicts
titanium plate reconstruction of orbital roof (arrow). (Courtesy of J.P. Bradley and J.C. Lee.)
A second type of craniofacial involvement by plexiform neurofibromas results in a unilateral overgrowth
of face because of diffuse proliferation of Schwann cells and axons associated with bony hypertrophy.
This type typically forms from the trigeminal, facial, or glossopharyngeal nerves.
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Etiology and Pathogenesis
Neurofibromatosis occurs in 1 in 2500 to 3000 births. NF1 is caused by mutations in neurofibromin, a
tumor suppressor gene on chromosome 17q, and inherited in an autosomal dominant manner. NF2 is
caused by mutations in the neurofibromin-2, a tumor suppressor gene on chromosome 22q.
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Treatment and Complications
Treatment of craniofacial neurofibromatosis is complex due to the variability in presentations and
destructive nature of the tumors. Orbitotemporal neurofibromas associated with sphenoid wing
dysplasia and plexiform neurofibromas involving the soft tissue of the face are the two main
classifications of the types of neurofibromas addressed. Because surgery cannot cure the process, the goal
of surgical resection is to debulk and lessen the visual impact of the deformity.
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Orbitotemporal Plexiform Neurofibromas
An algorithm for the treatment of orbitotemporal neurofibromas has been described in detail by Jackson.
26 In the presence of orbital soft tissue involvement without significant bony involvement and intact
vision, resection of the neurofibroma through a supratarsal incision may proceed. If the neurofibroma is
found to be a discreet mass, dissection and complete resection should be undertaken. However, if there is
more widespread involvement of the intraconal tissues, partial resection must be undertaken with every
effort to preserve vision. A bony defect of the orbit may be found in the orbital roof which should be
reconstructed at the same time to prevent enlargement of the defect due to prolapse of the intracranial
contents. Although bone grafts are usually the best choice for reconstruction of most osseous defects, in
the orbital roof region, resorption of the graft is common because of the pulsatile intracranial contents.
Thus, in this particular location, alloplast (titanium or methylmethacrylate) should be used. Shortening of
the levator aponeurosis may be necessary at the time of closure to account for the reduction of proptosis
of the globe from neurofibroma resection. In addition to reconstruction of the orbital roof, vertical orbital
dystopia may be present with inferior displacement of the orbit due to a mass effect from the
neurofibroma. When this occurs, an orbital box repositioning may be necessary.
In the most severe of presentations, the neurofibroma is present in the temporal area and the orbit with
pulsating exophthalmos because of a massive bony defect. In such cases, vision is not present.
Neurosurgical involvement is necessary to reduce the temporal lobe back into the middle cranial fossa.
Resection is performed with globe exenteration and reconstruction of the bony defect in preparation for
prosthesis. A recent Australian study focused on visual outcomes demonstrated that vision loss and
amblyopia are common in this cohort. 27
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Facial Plexiform Neurofibromas With Hemifacial Hypertrophy
Similar to the orbitotemporal neurofibromas, consideration for significant bleeding should be taken into
account. In the treatment of hemifacial hypertrophy, it is necessary to sacrifice a certain amount of normal
soft tissue structures, as it is not possible to separate the neurofibroma from unaffected tissues. One
method of soft tissue excision was proposed based on transposition of aesthetic units from the
contralateral normal side to the affected side. 28 Following transposition, the remaining tissue is excised
en bloc with incisions hidden in the areas such as the nasolabial fold. Underlying bony abnormalities are
also found, thus frequently requiring bone grafting for reconstruction of the mandible, maxilla, or
zygoma as necessary. 29
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Pierre Robin Sequence
Definition and Clinical Findings
Pierre Robin sequence (PRS) describes a triad of manifestations in neonates that included micrognathia,
glossoptosis, and upper airway obstruction; cleft palate deformity is an associated abnormality but not a
requirement for the sequence. 30 Because PRS has been associated with other anomalies, it is often divided
into:
PRS: isolated (Figure 35.9)
PRS plus: with comorbidities, Stickler, 22q deletion
Syndromic PRS: Treacher Collins syndrome, Nager syndrome (Figure 35.10)
FIGURE 35.9 Pierre Robin sequence (isolated) in a 6-week-old boy. A. Preoperative image shows significant micrognathia
(workup revealed upper airway obstruction). B. Postoperative image shows mandibular position improvement after bilateral
mandibular distraction (and relief of airway obstruction). (Courtesy of J.P. Bradley.)
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Etiology and Pathogenesis
When genetic syndromes or deformational factors disrupt prenatal growth, micrognathia ensues and
causes the retropositioning of the tongue. If this process occurs before the eighth week of gestation,
closure 
 of the palatal shelves is prevented, thereby resulting in the formation of 
 a U-shaped cleft palate.
31 When PRS occurs in isolation, in utero deformational factors such as intrauterine growth restriction are
generally considered to be important. Although neonates born with PRS may have clinically significant
symptoms, a portion of children may escape surgical intervention because of postnatal mandibular catch-
up growth. This phenomenon is generally more common to patients with isolated PRS, likely because of
the release of interference by deformational intrauterine factors after delivery. However, some patients
treated conservatively continued to have respiratory disturbances throughout life. 32
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Treatment and Complications
The most critical consequence of PRS is upper airway obstruction in the neonatal period. The severity of
airway obstruction may not always correlate with the degree of anatomic deformity (micrognathia). An
algorithmic workup is often warranted to determine the degree of tongue-based obstruction and
indication for surgical treatment to maintain airway patency. 32 In the mildest of circumstances, side or
prone positioning may be sufficient to prevent respiratory compromise. These mild PRS cases must still
be monitored closely. When cleft palate exists, delay in the repair is recommended (at 18 months or more,
as opposed to 10–12 months). In patients who cannot maintain airway patency with positioning alone,
intubation using a nasopharyngeal tube or an endotracheal tube may be necessary. In neonates who
require endotracheal intubation for survival, a surgical procedure is virtually universal to extubate the
child. 33 The current surgical armamentarium for relief of airway obstruction includes glossopexy,
subperiosteal mandibular stripping, mandibular distraction, and tracheostomy.
In PRS, retrodisplacement of the mandibular attachment of the genioglossus diminishes the ability to
hold the tongue out of the pharyngeal airway. Although glossopexy has the ability to temporarily relieve
obstruction with anterior movement of the tongue, this procedure depends on sufficient mandibular
catch-up growth for long-term reversal of airway obstruction. In addition, glossopexy does not address
the core anatomical disturbance.
When considering neonatal mandibular distraction for PRS patients, a decision tree model or
management algorithm is recommended (Figure 35.11). 32 Patients who exhibit other abnormalities such
as central apnea, multilevel airway obstruction, neurologic compromise, or severe airway edema from
reflux are precluded from mandibular distraction. Outcome studies on neonatal mandibular distraction
have shown improvements in oxygen saturation, apneic events, reflux, and feeding in neonates who
received mandibular distraction. 34 In patients who are not eligible for distraction based on the
management algorithm, a standard tracheostomy is performed. These tracheostomy patients will need
decannulation at a later age after growth and resolve their airway abnormalities. Mandibular distraction
and laryngotracheal reconstruction may be needed before tracheostomy removal. Skeletally mature,
untreated PRS patients may require mandibular advancement with a sagittal split osteotomy for class II
malocclusion after orthodontic preparation.
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FIGURE 35.10 Syndromic Pierre Robin sequence (Nager Syndrome) in a 3-month-old boy. A. Preoperative image shows
significant micrognathia (workup revealed upper airway obstruction). B. Postoperative image shows correction of chin point
(and improvement in airway). (Courtesy of J.P. Bradley.)
The reported complications of mandibular distraction are respiratory failure, relapse, nerve injury, tooth
injury, infection, incorrect distraction vector, and device failure. 35 These complications can be minimized
with the use of proper operative indications and perioperative protocols including appropriate rate
(2 mm), length (15–30 mm), and extubation times (after 7 days). In syndromic PRS patients, there is a
higher rate of temporomandibular joint ankylosis after distraction. 36 Elastic condylar unloading reduces
the risk of these ankylosis events.
Neonatal morbidity and mortality associated with PRS have significantly decreased with improved
nonsurgical and surgical methods for airway protection. With the understanding that improved
outcomes occur with mandibular distraction in a management algorithm, the majority of severely
micrognathic patients are no longer destined to tracheostomies. The challenge to institutions is to
implement and optimize systematic methods for evaluation and treatment for PRS neonates to achieve
timely, consistent, and cost-effective outcomes.
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Moebius Syndrome
Definition and Clinical Findings
In 1888 and 1892, Moebius classically described combined palsies of the abducens (CN VI) and facial (CN
VII) nerves as a composite entity. 37 This description was later expanded by others to include other CNs,
limb abnormalities, chest wall defects, and developmental delay.
The typical craniofacial manifestations of Moebius syndrome include mask-like facies, bilateral facial
paralysis, and the inability to abduct the globes (palsy of the abducens or sixth CN) (Figure 35.12).
Despite the classic description, there are variable presentations for Moebius syndrome including
incomplete or unilateral facial palsy. Several other CNs have also been associated with Moebius
syndrome, most frequently the third, fifth, ninth, and twelfth. Clinically, paralysis of the orbicularis oculi
is the first sign of Moebius syndrome detected in the neonate, prompting consultation by pediatricians
and neonatologists.
38 Ptosis, nystagmus, strabismus, epicanthal folds, hypertelorbitism, and
lagophthalmos are all frequently manifested. The nasal root is frequently widened. The mouth opening is
usually small and the commissures are downturned. Intraoral manifestations include tongue hypoplasia,
oligodontia, mandibular hypoplasia, cleft palate, and poor palatal mobility associated with difficulties in
feeding and speech. Feeding difficulties may resolve over time.
FIGURE 35.11 Pierre Robin sequence decision-tree model (algorithm) for all infants admitted to the pediatric intensive care
unit (PICU) with upper airway obstruction. Mild obstruction is treated as an outpatient with monitoring. Moderate/severe
obstruction undergoes multidisciplinary consultations and tests (sleep study, direct laryngoscopy, reflux study). Infants with
central apnea, airway lesions (other than tongue-based obstruction), or severe reflux undergo a tracheostomy. All other infants
have internal mandibular distraction.
In addition to craniofacial manifestations, both trunk and limb differences may be found. Absence or
hypoplasia of the sternal head of the pectoralis major is a common finding. 39 Moebius combined with
Poland syndrome has been reported in combination in 15% to 20% of cases. 40 Scoliosis is also found in
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approximately 14% of cases. 40 Limb abnormalities occur in over half of all patients diagnosed with
Moebius syndrome. The most common abnormality is clubfoot in 40% to 50% of patients followed by
limb reduction abnormalities in approximately 20% of patients. Intellectual developmental delay occurs
in 10% to 30% of patients with Moebius syndrome and ranges in severity. Autism has been associated
with Moebius syndrome. Motor delay, independent of the limb abnormalities, and speech delay are
frequently seen.
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Etiology and Pathogenesis
The prevalence of Moebius syndrome is estimated at 1 in 50,000 with equal distribution between male
and female. Although the pathogenesis is not well understood, both environmental and genetic
relationships have been described. The correlation between Moebius syndrome and misoprostol, a
synthetic prostaglandin E1 analogue, was described in Brazil in the early 1990s. 41 Within 96 infants with
Moebius syndrome identified in the Brazilian multicenter study, 49% of the mothers had taken
misoprostol during the first trimester of pregnancy. Moebius syndrome may also be related to genetic
causes inherited in an autosomal dominant, autosomal recessive, or X-linked pattern. Four genetic loci
have been identified (MBS1-4) located on chromosomes 13q, 1p, 3p, and 10q, respectively.
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Treatment and Complications
Reconstructive surgery in Moebius syndrome is a multidisciplinary endeavor requiring plastic surgery,
ophthalmology, and orthopedic surgery. The main procedures performed by the latter two specialties are
strabismus correction and clubfoot correction. For plastic surgery, the primary reconstructive procedure
for the treatment of Moebius syndrome is facial reanimation for smile restoration. The ability to
reconstruct facial motion using functional muscle transfers has undergone a renaissance age over the past
40 years. In the treatment of Moebius patients, the majority of patients suffer from bilateral complete
paralysis. Thus, functional muscle transfers frequently require innervation by nonfacial nerves such as
the masseteric nerve.
In the absence of suitable facial nerves as the donor innervator, functional muscle transfers for
reanimation are typically performed in one stage. 42,43 The most commonly used one-stage procedure is
the innervated, partial gracilis free muscle flap. Preoperatively, the new nasolabial fold is marked on the
face and a vector connecting the modiolus and the cephaloauricular junction at the root of the helix is
marked, mimicking the pull of the zygomaticus major muscle. Intraoperatively, a preauricular incision is
made and a dissection is performed in a sub-SMAS plane to the new nasolabial fold and the modiolus.
The masseteric nerve is then identified and dissected within an area bordered by the zygomatic arch,
coronoid process of the mandible, sigmoid notch, and condyle of the mandible. Dissection is carried out
through the parotid and between the superficial and middle heads of the masseter muscle, upon where
the nerve is visualized and coapted with the anterior branch of the obturator nerve from the gracilis
muscle flap. The harvested partial gracilis muscle is then inset to the insertion on the new nasolabial fold
and modiolus and to the origin at the deep temporal fascia following the premarked vector.
Microsurgical anastomoses of the vascular supply of the gracilis muscle are performed last, typically to
the facial vessels.
Alternatively, if CN V is functional, lengthening temporalis myoplasty or Labbe procedure may be
performed. 44 In this procedure, the nasolabial fold is marked out preoperatively. Intraoperatively, a
coronal incision is made, a scalp flap is elevated, and the temporalis muscle is dissected immediately on
the deep temporal fascia down to the zygomatic arch. Subperiosteal dissection is then carried over the
zygomatic arch and osteotomized for access. Next, the temporalis aponeurosis is incised 1 cm below the
temporal crest and the muscle is dissected away from the bone preserving the temporal nerve and
vessels. To inset the muscle, the new nasolabial fold is incised and dissected down to the coronoid
process. The temporalis tendon is detached from the coronoid process and sutured to the new nasolabial
fold. The temporalis muscle is then stretched and resuspended to the remaining strip of aponeurosis.
Although static procedures for smile restoration are not generally considered standard of care for
Moebius syndrome, auxiliary static procedures for the treatment of lagophthalmos are common and
beneficial for globe protection. The mechanism of lagophthalmos in Moebius syndrome is due to the lack
of opposition of the levator palpebrae muscle by the paralyzed orbicularis oculi muscle. Although a
number of modalities have been described to treat lagophthalmos, the most common and widely used is
the placement of a gold or platinum weight in the upper eyelid. Preoperatively, an appropriate weight is
estimated by taping the weight on the patient’s eyelid. Intraoperatively, an incision is made within the
supratarsal fold and dissection is carried out through the orbicularis oculi muscle. Two positions have
been described for the weight placement: (1) pretarsal and (2) postseptal. 45 In the pretarsal position, the
weight is secured to the tarsus underneath the orbicularis oculi muscle. Although this is the classic
placement position, there are complications such as entropion and visibility associated with pretarsal
weight placement. In the postseptal position, the septum is incised and the weight is placed immediately
superior to the tarsus. In this position, complications are diminished; however, the weight chosen should
be 0.2 g heavier than the preoperatively estimated weight.
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Quantifiable complications such as hematoma and flap failure in facial reanimation are rare. However,
qualitative and aesthetic issues such as incomplete correction, lack of strength in smile, asymmetry, and
lack of spontaneity are frequently present. The recent advent of more objective methods of analysis such
as the FACE-gram, an automated, objective assessment of facial movement, will likely assist in assessing
surgical outcomes. 46
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Dermoid Cyst
Definition and Clinical Findings
Congenital facial dermoid cysts are benign, developmental malformations formed by inclusion of
ectodermal elements. 47,48 Dermoid cysts are differentiated from the typical epidermoid cysts by the
presence of mature adnexal structures such as hair and glands. Because of the process of formation,
dermoid cysts are frequently, if not always, associated with some type of skeletal abnormality. Dermoid
cysts are more common in females than in males.
Craniofacial dermoid cysts can be generally divided into lateral and midline. Lateral cysts may be further
subdivided into the frontotemporal region and the orbit. By far, lateral dermoid cysts of the
frontotemporal region are the most common type of craniofacial dermoids with the majority near the
lateral eyebrow (Figure 35.13). The lesion is usually deep to the skin such that there is usually no evidence
of sinus ostia. The lesions are typically firm and present as a discreet nodule. Dermoids within this region
are rarely associated with intracranial extension and thus do not warrant imaging before surgical
resection without additional clinical suspicion.
FIGURE 35.12 Moebius syndrome in a 10-year-old patient. A. Preoperative image (when asked to smile) shows mask faces
with no expression. B. Postoperative image (2 years) shows correction after bilateral lengthening temporalis myoplasty.
(Courtesy of P.D. Nguyen.)
The orbit is the second anatomical subdivision of lateral cysts and accounts for the second most common
area for dermoid cysts of the face. Extraocular mobility is typically not affected by orbital cysts; however,
depending on the size, visual obstruction may occur (see Figure 35.13.A). Unlike epidermoid cysts,
dermoid cysts are frequently found with skeletal abnormalities. Although the skeletal anomaly in the
lateral brow dermoids is simple depressions within the frontal bone, orbital dermoids frequently traverse
sutures, such as the frontozygomatic suture (see Figure 35.13.B). In such cases, the dermoid has an
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intraorbital component and an extraorbital component.
Unlike lateral orbital cysts, the midline dermoid cysts may have more extensive and potentially
intracranial involvement. The estimated incidence of intracranial involvement ranges around 19% of all
nasal dermoids. 49 Midline dermoids may be found in the upper, middle, or lower face. In all cases,
because of the potential for intracranial extension, a CT scan and/or MRI is necessary to define the limits
of the dermoid. Within the nasoethmoid region, the external clinical manifestations may be particularly
mild. A small punctum, bulge of the glabella, or widening of the nasal dorsum may be seen. However,
mild clinical presentations may portend extensive involvement of internal structures and intracranial
involvement. Additionally, unlike the lateral dermoids, midline dermoids have a higher propensity to
display puncta and sinus ostia. Owing to the presence of adnexal structures, dermoid cysts have the
capability of enlarging over time. Although rare, complications of dermoid cysts include infection, with
several instances of calvarial osteomyelitis reported in the literature. 47
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Etiology and Pathogenesis
Dermoid cysts are most often found as isolated entities and very rarely found in families. Formation of
dermoid cysts is found at the embryologic fusion planes. For example, the lateral brow dermoid is found
at the frontotemporal suture; the lateral orbital dermoid is found at the frontozygomatic suture; and the
nasoglabellar suture is found at the frontonasal junction. In the circumstances of intracranial extension of
dermoid sinuses and cysts, the development can be traced back to 8 weeks of gestation. During this time,
the developing nasal and frontal bones are separated by dural extensions through the fonticulus
nasofrontalis anteriorly and through the frontal bones into the prenasal space inferiorly. As the frontal
bones grow, the dura migrates into the cranium and the fonticulus nasofrontalis fuses with the foramen
cecum to become the cribriform plate. When this process is incomplete, the dura remains extracranial and
becomes a cyst or sinus.
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Treatment and Complications
Surgery is the only treatment available for dermoids. In lateral dermoids of the frontotemporal or orbital
region, excision can be approached with an incision directly over the dermoid or hidden in the
supratarsal fold. Because the embryologic origins of the cyst formation, dissection of the cyst should be
through the soft tissues to the periosteum; this dissection is distinguished from that of the dissection of a
simple epidermal cyst. Additionally, a skeletal depression will be frequently found underneath the cyst.
In nasal dermoids, diagnosis requires imaging with an initial fine-cut CT scan. The two signs on CT scan
suggestive of intracranial extension are a patent foramen cecum and a bifid crista galli. If either finding is
present or suspicious for intracranial extension, an MRI should be performed next. If the MRI is negative,
simple excision may be performed as above for glabellar dermoids. In nasal dermoids positioned more
inferiorly, an open rhinoplasty approach should be considered.
If the MRI is positive for intracranial extension, excision of the dermoid requires neurosurgical
involvement. Coronal incision access is used for the frontal craniotomy. Dissection of the cyst requires an
osteotomy of the frontal bone which may be performed in the keystone technique with paramedian
osteotomies of the frontal and nasal bones. 50 By removing this V-shaped piece of bone, the dermoid cyst
can be better visualized for full excision.
Complications for extracranial dermoid excisions are rare but may include incomplete excision,
recurrence, or infection. Resection of dermoids with intracranial extension may be complicated with
osteomyelitis, incomplete resection, or cerebrospinal fluid leak.
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Questions
1. A 40-year-old woman diagnosed with fibrous dysplasia is noted on CT scan to have extension of
the abnormal osseous growth into the greater wing of the sphenoid bone close to the optic nerve.
Which statement about treatment decision is true?
a. Optic nerve decompression should be performed with definitive radiographic evidence of
fibrous dysplasia around or near the optic nerve.
b. Optic nerve decompression may result in worsening of vision because of heat injury and
therefore should not be performed.
c. Optic nerve decompression should be performed when there is the beginning of visual
changes.
d. Bisphosphonates, steroids, and tamoxifen are the treatment of choice when visual changes
occur because surgery is too risky.
2. For patients with Pierre Robin sequence (PRS), both mandibular distraction and glossopexy
address upper airway obstruction with improvement in which of the following?
a. Laryngomalacia
b. Tracheomalacia
c. Vascular webs
d. Retroposition of the tongue base
e. Chin position
3. Which of the following about the treatment of Parry-Romberg syndrome is true?
a. The fat grafting for the Romberg patients is not advised because the take in the diseased
Romberg region is less than the take in normal regions for cosmetic procedures.
b. The classic coup de sabre sign means there is deeper, bony involvement and osseous correction
will also be needed.
c. Because Romberg disease burns out in puberty, observation is recommended, as most cases
improve over time without treatment.
d. For adults with severe soft tissue deficiencies, the parascapular free flap is recommended over
the anterolateral thigh free flap because of the better adipose-fascial fill and sculpting.
e. Dermal fat grafts have fallen out of favor because of the high infection rate.
4. For Treacher Collins syndrome, orbitomalar reconstruction should not be attempted too early
because:
a. premature absorption of orbital zygomatic bone grafts may occur.
b. zygomatic body growth should be completed before custom implant placement.
c. correction of malocclusion should be the paramount concern.
d. all stages of total ear reconstruction should be completed first.
e. bone graft take is more reliable at an older age.
5. Which is true in facial plexiform neurofibromas with hemifacial hypertrophy?
a. The buccal fat is typically the most involved and should be radically resected.
b. Multiple small resections are often recommended for large tumors to avoid bleeding and
injury to vital structures.
c. It is sometimes necessary to resect all the soft tissue between the skin and bone including the
facial nerve and perform a reanimation procedure.
d. All aesthetic units are distorted by large tumor masses so do not need to be considered.
e. When considering risk/benefit, face transplantation is ultimately recommended for the best
overall result.
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1. Answer: c. Optic nerve decompression for optic canal involvement is controversial but should be
reserved for patients with both definitive radiographic evidence of fibrous dysplasia around or near
the optic nerve and the beginning of visual changes. Optic nerve decompression is deemed too risky
with normal vision even with radiographic evidence of involvement.
2. Answer: d. PRS is a manifestation of triad of micrognathia, glossoptosis, and upper airway
obstruction. Both mandibular distraction and glossopexy (tongue-lip adhesion) improve the
retroposition of the tongue base. Laryngomalacia, tracheomalacia, and vascular webs may all
obstruct the airway but neither mandibular distraction nor glossopexy will correct these lesions. The
retroposition of the chin, seen in PRS, will be improved by mandibular distraction lengthening, not
tongue-lip adhesion.
3. Answer: d. Although fat graft take in Romberg regions is diminished, it is still a worthwhile
treatment. The coup de sabre sign may just involve skin and fat hypoplasia but not necessarily bone.
Unlike hemangioma cases, Romberg hypoplasia does not get better with time. The back region
provides better adipose-fascial fill for facial contouring than that of other free flaps.
4. Answer: a. If orbitomalar bone graft reconstruction is performed before the age of 8 to 10 years,
premature bone absorption is likely. Alloplastic implants are not recommended for Treacher Collins
reconstruction in children. Malocclusion and ear reconstruction issues are independent of the timing
of orbitomalar reconstruction. Even with good initial bone healing and bone graft take, absorption
can still occur if performed early.
5. Answer: c. In the treatment of hemifacial hypertrophy, it is often necessary to sacrifice a certain
amount of normal soft tissue structures and even the facial nerve, as it is usually poorly functioning
and not possible to separate from the neurofibroma. Thus, smaller procedures with a goal of saving
the facial nerve may be unrewarding. For severe cases, face transplantation has been performed but
carries long-term risks associated with immunosuppression.
FIGURE 35.13 Dermoid cyst right lateral orbit. A. Preoperative image shows 2 cm mass. B. 3D CT scan shows erosion of
lateral orbit from dermoid mass (arrow). C. CT scan coronal view shows dermoid mass distorting bone from lateral orbital rim
(arrow). (Courtesy of J.C. Lee.)
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References
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the variation of fibrous dysplasia involvement as mild to severe in the craniofacial skeleton. This center has a broad experience and documents their care
protocols in treating this disorder and dealing with monostotic and polyostotic cases.
Weinstein, LS, Shenker, A, Gejman, PV, et al. Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J
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Posnick, JC. Fibrous dysplasia of the craniomaxillofacial region: current clinical perspectives. Br J Oral Maxillofac Surg. 1998;36:264-273.
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Slack, GC, Tabit, CJ, Allam, KA, et al. Parry-Romberg reconstruction: optimal timing for hard and soft tissue procedures. J Craniofac Surg.
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bone grafting is sometimes needed.
Stone, J. Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology. 2003;61:674-676.
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Saadeh, P, Reavey, PL, Siebert, JW. A soft-tissue approach to midfacial hypoplasia associated with Treacher Collins syndrome. Ann Plast
Surg. 2006;56:522-525.This article documents a microsurgical solution to soft tissue fill with a parascapular (or IMECS) flap after hard tissue
reconstruction.
Kumar, A. Treacher-Collins syndrome. In: Thaller, SR , Garri, JI , eds. Craniofacial Surgery. New York, NY: CRC Press; 2008:273-281.
Trainor, PA, Dixon, J, Dixon, MJ. Treacher Collins syndrome: etiology, pathogenesis and prevention. Eur J Hum Genet. 2009;17:275-283.
Posnick, JC, Tiwana, PS, Costello, BJ. Treacher Collins syndrome: comprehensive evaluation and treatment. Oral Maxillofac Surg Clin North
Am. 2004;16:503-523.Treacher Collins reconstruction is multistaged and challenging, and this paper offers acceptable evaluation and treatment that is
time-tested and accepted by multidisciplinary teams.
Trainor, PA. Craniofacial birth defects: the role of neural crest cells in the etiology and pathogenesis of Treacher Collins syndrome and the
potential for prevention. Am J Med Genet A. 2010;152a:2984-2994.
Dai, J, Si, J, Wang, M, et al. Tcof1-related molecular networks in Treacher Collins syndrome. J Craniofac Surg. 2016;27:1420-1426.
Splendore, A, Jabs, EW, Felix, TM, Passos-Bueno, MR. Parental origin of mutations in sporadic cases of Treacher Collins syndrome. Eur J
Hum Genet. 2003;11:718-722.
Konofaos, P, Arnaud, E. Early fat grafting for augmentation of orbitozygomatic region in Treacher Collins syndrome. J Craniofac Surg.
2015;26:1258-1260.
Fan, KL, Federico, C, Kawamoto, HK, Bradley, JP. Optimizing the timing and technique of Treacher Collins orbital malar reconstruction. J
Craniofac Surg. 2012;23:2033-2037.This comparative outcomes study is one of the few that critically looks at the timing of bone graft
reconstruction for the malar region. It discusses the psychosocial balance with timing of surgery to avoid reoperations. If in-lay
cranial bone grafting is performed less than 5 years of age, it will resorb.
Hopper, RA, Kapadia, H, Susarla, S, et al. Counterclockwise craniofacial distraction osteogenesis (C3DO) for tracheostomy-dependent
children with Treacher Collins syndrome. Plast Reconstr Surg. 2018;142(2):447-457.Mandibular distraction and orthognathic reconstruction is
challenging with Treacher Collins patients because of the abnormal mandibular shape and short cranial base. This paper illustrates a unique way of
using distraction osteogenesis to lengthen the posterior midface and mandible with a counterclockwise rotation .
Heller, JB, Gabbay, JS, Kwan, D, et al. Genioplasty distraction osteogenesis and hyoid advancement for correction of upper airway
obstruction in patients with Treacher Collins and Nager syndromes. Plast Reconstr Surg. 2006;117:2389-2398.
Hennekam, RCM, Gorlin, RJ, Allanson, JE, Krantz, ID. Gorlin’s Syndromes of the Head and Neck. Neurofibromatosis. 5th ed. New York, NY:
Oxford University Press; 2010.
Jackson, IT, Carbonnel, A, Potparic, Z, Shaw, K. Orbitotemporal neurofibromatosis: classification and treatment. Plast Reconstr Surg.
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1993;92:1-11.
Greenwell, TH, Anderson, PJ, Madge, SK, et al. Long-term visual outcomes in patients with orbitotemporal neurofibromatosis. Clin Exp
Ophthalmol. 2014;42:266-270.
Hivelin, M, Wolkenstein, P, Lepage, C, et al. Facial aesthetic unit remodeling procedure for neurofibromatosis type 1 hemifacial hypertrophy:
report on 33 consecutive adult patients. Plast Reconstr Surg. 2010;125:1197-1207.
Munro, IR, Martin, RD. The management of gigantic benign craniofacial tumors: the reverse facial osteotomy. Plast Reconstr Surg.
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Robin, P. Glossoptosis due to atresia and hypotrophy of the mandible. Am J Dis Child. 1934;48:541-547.
Hanson, JW, Smith, DW. U-shaped palatal defect in the Robin anomalad: developmental and clinical relevance. J Pediatr. 1975;87:30-33.
Vyas, RM, Dipple, KM, Head, C, et al. Management of neonatal upper airway obstruction: decreased morbidity by utilizing a decision tree
model with mandibular distraction osteogenesis. J Neonat Perinat Med. 2008;1:21-29.Neonatal mandibular distraction for airway obstruction is
an effective treatment, but determining the indications for this procedure may be challenging. This algorithm study proves that a multidisciplinary team
may treat neonatal upper airway obstruction with the correct modality when the evaluations are performed immediately and carefully.
Izadi, K, Yellon, R, Mandell, DL, et al. Correction of upper airway obstruction in the newborn with internal mandibular distraction
osteogenesis. J Craniofac Surg. 2003;14:493-499.
Kohan, E, Hazany, S, Roostaeian, J, et al. Economic advantages to a distraction decision tree model for management of neonatal upper
airway obstruction. Plast Reconstr Surg. 2010;126:1652-1664.
Mofid, MM, Manson, PN, Robertson, BC, et al. Craniofacial distraction osteogenesis: a review of 3278 cases. Plast Reconstr Surg.
2001;108:1103-1114.
Fan, K, Andrews, BT, Liao, E, et al. Protection of the temporomandibular joint during syndromic neonatal mandibular distraction using
condylar unloading. Plast Reconstr Surg. 2012;129:1151-1161.
Hennekam, RCM, Gorlin, RJ, Allanson, JE, Krantz, ID. Gorlin’s Syndromes of the Head and Neck. Moebius Syndrome. New York, NY: Oxford
University Press; 2010.
Terzis, JK, Noah, EM. Möbius and Möbius-like patients: etiology, diagnosis, and treatment options. Clin Plast Surg. 2002;29:497-514.This
article documents the important diagnosis and technical details in dealing with and treating Möebius patients.
McGillivray, BC, Lowry, RB. Poland syndrome in British Columbia: incidence and reproductive experience of affected persons. Am J Med
Genet. 1977;1:65-74.
McClure, P, Kilinc, E, Oishi, S, et al. Mobius syndrome: a 35-year single institution experience. J Pediatr Orthop. 2017;37:e446-e449.
Pastuszak, AL, Schüler, L, Speck-Martins, CE, et al. Use of misoprostol during pregnancy and Möbius’ syndrome in infants. N Engl J Med.
1998;338:1881-1885.
Rozen, SM. Facial reanimation: basic surgical tools and creation of an effective toolbox for treating patients with facial paralysis. Part A
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Rozen, S. Facial palsy in children and young adults. In: Lee, JC , ed. Craniosynostosis and Rare Craniofacial Clefts. New York, NY: Nova Science
Publishers; 2016:223-247.
Labbé, D, Huault, M. Lengthening temporalis myoplasty and lip reanimation. Plast Reconstr Surg. 2000;105:1289-1297.This article documents a
better option for facial reanimation for many patients. It describes the details of the lengthening temporalis myoplasty procedure and outcomes.
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Bhama, PK, Weinberg, JS, Lindsay, RW, et al. Objective outcomes analysis following microvascular gracilis transfer for facial reanimation: a
review of 10 years’ experience. JAMA Facial Plast Surg. 2014;16:85-92.
New, GB, Erich, JB. Dermoid cysts of the head and neck. Surg Gynecol Obstet. 1937;65:48-55.
Bartlett, SP, Lin, KY, Grossman, R, Katowitz, J. The surgical management of orbitofacial dermoids in the pediatric patient. Plast Reconstr Surg.
1993;91:1208-1215.
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Pensler, JM, Bauer, BS, Naidich, TP. Craniofacial dermoids. Plast Reconstr Surg. 1988;82:953-958.
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Surg. 2005;116:13-19.
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PA R T I V
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Head and Neck
36. Facial Fractures and Soft Tissue Injuries
37. Head and Neck Cancer and Salivary Gland Tumors
38. Reconstruction of the Scalp, Forehead, Calvarium, Skull Base, and Midface
39. Reconstruction of the Eyelids, Correction of Ptosis, and Canthoplasty
40. Nasal Reconstruction
41. Reconstruction of Acquired Lip and Cheek Deformities
42. Facial Paralysis
43. Mandible Reconstruction
44. Reconstruction of the Oral Cavity, Pharynx, and Esophagus
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C H A P T E R 3 6
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Facial Fractures and Soft Tissue Injuries
Christine M. Jones and John A. van Aalst
Key Points
The patient with facial fractures is a trauma patient and requires a comprehensive evaluation for
calvarial, intracranial, and cervical spine injuries.
Impending optic nerve injury can be recognized early by examining for red desaturation.
Entrapment of the extraocular muscles and septal hematoma are two surgical emergencies in facial
trauma.
Restoration of facial height, width, and projection is accomplished through rebuilding the facial
buttresses in the vertical, horizontal, and sagittal dimensions.
Achieving normal occlusion is of primary importance in the reduction of maxillary and mandibular
fractures.
Sequencing should build from known to unknown in panfacial fractures in a top-down, bottom-up, or
inside-out fashion.
Facial trauma is one of the most common reasons for consultation with a plastic surgeon; an estimated
407,000 emergency department visits occur annually for facial fractures alone. 1 Appropriate evaluation
and treatment is a fundamental skill for trainees. The principles learned in approaching and stabilizing
fractures serve as a foundation for building more advanced concepts in soft tissue handling and
orthognathic surgery.
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Concepts in Facial Trauma
Mechanisms of injury vary and patients must first be considered from a comprehensive trauma
standpoint. Each patient must undergo a thorough trauma evaluation for concomitant injuries, beginning
with airway assessment, breathing, and circulation. Over 45% of patients with facial fractures have
concomitant intracranial injury and nearly 10% have cervical spine injuries. 2 With areas of thin bone and
sinuses, the face is designed to act as a shock absorber to help prevent trauma to these critical neurologic
structures.
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Stability of the Craniofacial Skeleton
The height, width, and projection of the facial skeleton is stabilized by facial buttresses, which are areas of
thick bone that act as scaffolds in the vertical and horizontal dimensions (Table 36.1). Horizontal facial
buttresses are divided into coronal and sagittal types (Figure 36.1). Fractures extending through these
buttresses are more likely to be clinically significant; proper reduction restores alignment and stability in
these areas. Whereas the frontal bone and mandible display stability in each plane, the midface lacks
sagittal support, lending it particularly susceptible to flattening after attempts at reconstruction,
especially centrally. 3
TABLE 36.1
Buttresses of the Craniofacial Skeleton 3,4
Buttress
Purpose
Vertical
Maintain facial height
Orbital
Nasofrontal
Zygomatic
Pterygomaxillary
Mandible
Horizontal
Coronal
Maintain facial width
Supraorbital
Infraorbital rim
Transverse maxillary
Mandible
Sagittal
Maintain facial projection
Frontal bone
Zygomatic arch
LeFort I segment
Mandibular body
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FIGURE 36.1 Buttresses of the craniofacial skeleton are divided into vertical (A,B) and horizontal (C,D) supports. Horizontal
buttresses are further subdivided into coronal (C) and sagittal (D) types.
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Facial Examination
To avoid missing injuries, the face should be systematically examined through inspection, palpation, and
evaluation of range of motion. Beginning in the upper third, the scalp should be inspected for lacerations
and foreign bodies. Scalp lacerations are obscured by hair, positioning, or cervical collars and can bleed
significantly. Moving onto the face, the soft tissue, including the auricle, external auditory canal, and
nose, including the septum, are inspected for lacerations and foreign bodies. Prompt recognition of both
auricular and septal hematomas is critical. Examination progresses to systematic palpation of bony
prominences to determine the presence of step-offs, instability, or crepitus; sites of examination include
the supraorbital ridge, lateral orbital walls, infraorbital rims, nasal bones, zygomatic arches, maxilla, and
mandible. Maxillary stability is determined by stabilizing the head at the nasofrontal junction with one
hand and using the examining hand to rock the maxillary alveolus in a forward-backward and medial-
lateral motion (Figure 36.2).
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FIGURE 36.2 Testing maxillary stability helps diagnose LeFort I, II, or III fractures. The face is stabilized at the forehead while
the dominant hand grasps the anterior maxilla and attempts to displace it in an anteroposterior (A) and lateral direction (B).
Ocular examination begins with inspection of the conjunctiva for hemorrhage, assessment of the lids and
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canaliculi for lacerations, inspection for foreign bodies, and visualization of penetrating injuries. Pupillary
examination is performed with a flashlight to compare pupillary size at rest and direct and consensual
responses to light. The swinging flashlight test distinguishes afferent pupillary defects from an
oculomotor nerve injury: an eye that has lost vision will lack the ability to constrict with direct light but
will still display a consensual response when light is shone in the opposite pupil. Extraocular muscle
excursion should be examined by asking the patient to look in each of the cardinal directions. In an
unresponsive patient, forced duction testing rules out entrapment (Figure 36.3). Monocular and binocular
vision is examined; the presence of diplopia is recorded. Because red desaturation is one of the earliest
signs of visual loss, each patient is asked to compare a red object with both the affected and unaffected
eye. A brief visual field examination reveals the presence of gross field cuts. Globe position is examined
for early enophthalmos, which reliably indicates the need for orbital floor fracture correction because
swelling usually masks enophthalmos in the acute setting.
FIGURE 36.3 To test for entrapment in an unresponsive patient, several drops of ophthalmic tetracaine are instilled into each
eye. The bulbar conjunctiva is grasped with toothed forceps near the inferior fornix and the globe is rotated upward, downward,
medially, and laterally. Excursion is compared with the contralateral side.
Moving to the middle third, the nasal septum is examined for boggy mucosal swelling, indicative of a
septal hematoma. An otoscope is useful for this step if a nasal speculum is not available. Hematoma can
be distinguished from septal deviation by probing with forceps and assessing for ballotability.
The temporomandibular joint (TMJ), positioned directly anterior to the tragus and under the zygomatic
arch, is evaluated for tenderness or deformity. The articular disc can be assessed by palpating anteriorly
from within the external auditory canal while the patient gently opens her mouth. Maximal interincisal
opening should be recorded.
Intraoral lacerations, open fractures, and loose or avulsed teeth should be noted. If a tooth is missing,
ensure that it is not retained within the oral cavity where it presents an aspiration risk. Dental hygiene
should be noted, as this affects the success of maxillomandibular fixation. The patient’s occlusion is
recorded, including the presence of cross-bite or open-bite deformities.
Cranial nerve (CN) function is examined by testing sensation to light touch in the V1, V2, and V3
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distributions, comparing sensitivity with the contralateral side and with baseline. Each branch of the
facial nerve is tested by asking the patient to raise the eyebrows (temporal branch), close the eyes tightly
(zygomatic), puff the cheeks (buccal), smile (marginal mandibular), and purse the lips (cervical). Loss of
lip depression while smiling can result from injury to the ipsilateral marginal mandibular nerve or
cervical branch, as the platysma (cervical branch innervation) can cofunction with the depressor anguli
oris (marginal mandibular innervation) to display a full denture smile. These can be distinguished by
asking the patient to evert her lower lip: with a cervical branch injury, eversion is preserved because of
intact mentalis function. The tongue should protrude at midline (CN XII), the palate should elevate
symmetrically (CN IX, X), hearing should be grossly symmetric (CN VIII), and the shoulders should be
able to shrug (CN XI).
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Radiographic Evaluation
Thin-slice CT scan should be obtained for patients whose mechanism of injury or examination suggests
facial fractures. Cuts should be 1 mm or finer. X-rays are rarely useful. CT scans should be examined in
each plane of section to rule out skeletal injuries.
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Regional Blocks
Small lacerations are managed by injecting local anesthetic at the site of injury. A 25-gauge needle is used
to inject through existing laceration; inject slowly using buffered anesthetic to minimize patient
discomfort. Larger areas of the face can be treated with regional blocks (Figure 36.4). 5
FIGURE 36.4 Multiple blocks are available to provide regional anesthesia for the face.
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Access Incisions
Numerous incisions are available to access various regions of the facial skeleton (Figure 36.5). 6
Lacerations from the injury can also be used to further improve access. For optimal access to correct bony
injuries, edema should be allowed to subside for 7 to 10 days. Scars should be placed in concealed
locations whenever possible; the transconjunctival and upper and lower buccal sulcus incisions are good
examples. Whenever a skin incision is made, it should be placed in the relaxed skin tension lines (Table
36.2). For access to the midface in particular, multiple simultaneous incisions are often necessary.
FIGURE 36.5 Selected incisions for periorbital and midface skeletal access: coronal (a), Wright (b), upper lid crease (c),
transconjunctival (d), lateral canthotomy (e), subciliary (f), subtarsal/midlid (g), infraorbital (h), Lynch (i), and upper buccal
sulcus (j).
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TABLE 36.2
Selected Facial Access Incisions
Skeletal
Access
Incision
Benefits
Drawbacks
General
Lacerations
No new facial scars
Limited control over which parts of skeleton are
accessible
Frontal
bone,
midface
Bicoronal
Wide access to frontal bone and midface
Conspicuous scar
Orbital
floor
Transconjunctival,
preseptal
Hidden; avoids protrusion of orbital fat
Tedious dissection through lower lid
Transconjunctival,
post-septal
Hidden; ease
Orbital fat must be retracted during surgery
Subciliary
Scar blends with resting tension lines
High risk of ectropion
Subtarsal
Scar blends with resting tension lines
High risk of ectropion
Infraorbital
Ease
Conspicuous scar, risk of ectropion
Zygomatic
arch
Gillies
Hidden in scalp
Utility limited to arch alone; blind
Midface
Upper buccal
sulcus
Ease, affords wide access, hidden
Risk of gingival retrusion
Mandible
Lower buccal
sulcus
Wide applications
Risk of mental nerve injury; higher risk of
infection for highly comminuted fractures or
poor oral hygiene
Risdon
Lower risk of infection, easier reduction for highly
comminuted fractures, edentulous patients, poor
oral hygiene
Risk of marginal mandibular nerve injury;
conspicuous scar
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Antibiotic Prophylaxis
Evidence supporting the use of perioperative antibiotics for the treatment of facial fractures is of low
quality. However, routine antibiotic prescription is a common practice, with penicillin, cephalosporins,
and clindamycin being the most commonly prescribed antimicrobials. 7,8 In a seminal article, two
perioperative doses of cefazolin reduced infection risk from 42% to 8.9%. 9 Current literature supports the
use of perioperative antibiotics (beginning < 60 minutes prior to incision and ending within 24 hours) for
operative facial fractures, especially those that involve transmucosal access incisions with clean-
contaminated surgical fields. 7,10 However, data describing use of antibiotics beyond this time period is
inconclusive.
Although infection is rare, it can have serious consequences. Transmigration of sinus bacteria into the
periorbital soft tissue can result in orbital cellulitis, leading to optic neuritis, optic nerve atrophy
blindness, cavernous sinus thrombosis, or meningitis. 11 An unclear risk-benefit ratio for the use of
antibiotics in facial trauma continues to make this a hotly debated topic.
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