Operative Technique in Plastic Surgery – Kevin C.Chung

FIG 3 • A. Ricketts S-line. B. Reidel plane. C. Facial
convexity angle.
SURGICAL MANAGEMENT
■ Operative interventions to address chin aesthetics include the implantation of
alloplastic materials (ie, porous polyethylene or silicone implants) and surgical
osseous genioplasty.
■ Indications for surgical intervention include displeasure with one’s
appearance, with the patient specifically identifying the chin as an area of
focus.
■ The balance between lip, nose, and midface position must be addressed with
the patient, as any deformity or asymmetry in these areas may either be
contributing to the appearance of chin disharmony or may become more
obvious after the chin has been addressed.
■ The risks of osseous genioplasty include bleeding, infection, injury to the
mental nerve, paresthesias, injury to the mentalis muscle, “witch chin”
deformity, mentalis muscle, pain, poor wound healing, scarring, persistent
asymmetry, hardware malfunction, and the need for future additional
operations.
■ Each skeletal configuration on the differential diagnosis list calls for a unique
surgical solution to address the problem or some combination of these.
■ The surgical steps for correction vary primarily in the planning and execution

of the osteotomies and positioning and fixation of the bony segment:
■ Microgenia, vertical—lengthen (vertically)
■ Macrogenia, sagittal—setback (usually wedge removed)
■ Macrogenia, vertical—shorten (wedge removed)
■ Asymmetry, frontal—rotate (mediolateral)
■ Asymmetry, vertical—wedge reduction
■ Combination—combination of the above
Preoperative Planning
■ Panorex and CBCT imaging should be reviewed prior to any surgical
procedure to assess for dental maturity and cessation of bone growth.
■ Based on clinical and skeletal analysis, the osteotomy is planned to address
each patient on a case-by-case basis.
■ The surgeon must explain to the patient that this is a primarily cosmetic
procedure and expectations should be set early that this may include out-of-
pocket expenses, even if combined with other insurance-covered surgery.
Positioning
■ The patient is placed supine on the operating table with the head positioned in
a semi-extending position on the head rest.
■ Nasal RAE endotracheal tube is used for intubation with exception of
concurrent rhinoplasty.
■ The entire face, mouth, ears, and neck should be prepped with Betadine or
chlorhexidine solution while protecting the corneas. Steri-Strips are placed to
secure the eyelids.
■ A head drape is placed securing the patient’s hair and clearing the operative
field.
■ The surgeon must visualize the entire face given the chin’s role in facial
harmony.
■ A throat pack is placed into the oral pharynx to minimize ingestion of
irrigation solution and blood throughout the procedure.
■ Local anesthetic with epinephrine is injected for regional anesthetic and
hemostatic effect prior to incision.
■ Decadron is administered preoperatively to control postoperative edema.
■ Perioperative antibiotics are administered to prevent surgical site infections
(ie, cefazolin or clindamycin).

T E C H N I Q U E S
■ Basic Surgical Steps
Exposure
■ The anterior labial vestibule is incised using a no. 15 blade scalpel
perpendicular to the mucosa with a dart designed at the lower lip frenulum3
(TECH FIG 1A).
■ This incision is placed at least 10 mm from the mucogingival line.
■ Using a fine tip Colorado blade electrocautery, a horizontal incision is made
through the mentalis muscle, preserving a cuff at the gingival border,
allowing precise reattachment at closure (TECH FIG 1B).
■ Blunt dissection is then performed with an elevator to expose and visualize
the mental nerves as well as the mental prominence and associated structures.
Osteotomy Design
■ Midline should be marked prior to osteotomy design.
■ The osteotomy is then traced with a sterile pencil above the pogonion,
avoiding the mental nerves but typically extending laterally beyond the
mental foramina (TECH FIG 2A).
TECH FIG 1 • A. The anterior labial vestibule is
incised using a no. 15 blade scalpel perpendicular to
the mucosa with a dart designed at the lower lip
frenulum. B. Cuff of mentalis muscle remains attached

to mandible (arrow) to facilitate closure. Mental nerve
(*).
TECH FIG 2 • A. The osteotomy extends lateral to the
mental foramen (arrow). Mental nerve (*). B.
Genioglossus muscle remains attached to the mobile
bony segment (arrow). C. Inferior osteotomy (arrow) is
performed first in shortening genioplasty with planned
wedge resection. In cases of prominent tubercles (D),
a pineapple bur or reciprocating rasp is used to
smooth the contour (E).
■ Angulation and wedge resection might be planned based on anticipated
course.
■ Using the reciprocating saw, the planned osteotomies are performed. Irrigate
the blade with saline to prevent thermal injury to the mandible and
surrounding structures.
■ Leave the genioglossus muscle attached to retain blood supply to the mobile
segment(s) (TECH FIG 2B).
■ In the case of a shortening genioplasty, where wedge resection is planned,
the inferior osteotomy is performed first (TECH FIG 2C).
■ In cases of asymmetry, wedge resection will be unilateral.
■ For cases with prominent tubercles, the pineapple bur or reciprocating rasp is

employed to smooth the contour (TECH FIG 2D,E).
Fixation
■ The mobile genioplasty segment is secured to the mandible using a variety
of surgical plates and screws depending on the goals of the procedure.
■ Options include the X-plate, step-off advancement plate, and straight H-plate
(TECH FIG 3A–E).
TECH FIG 3 • Fixation can be accomplished with a
variety of plates, including the X-plate (A), the step-off
advancement plate (B), and the straight H-plate (C).
Closure and Dressing
■ Following bony fixation, the oral cavity and surgical field are irrigated with
Betadine and normal saline solution.
■ Precise reattachment of the mentalis muscle over the plate/screws is critical
for good outcomes, both functionally (lower lip motion) and aesthetically
(soft tissue volume/contour).
■ Multiple interrupted 3-0 Vicryl sutures are used to reapproximate the muscle
(TECH FIG 4A).
■ The overlying mucosa is then reapproximated with 3-0 chromic gut sutures.
■ A chin dressing is applied using Mastisol, surgical tape (elastoplasty tape is
used by the authors), and Tegaderm designed to provide additional support
and limit edema to the chin postoperatively (TECH FIG 4B).
■ The patient is placed in a face bra once the dressing has been applied to
provide additional external support to the mandible and soft tissues of the
chin (TECH FIG 4C).
■ This dressing is removed at the time of follow-up, approximately 1 week

after surgery.
TECH FIG 4 • A. Using 3-0 Vicryl suture, the mentalis
muscle (arrow) is closed over the underlying hardware
and precisely repositioned to avoid chin deformity. B.
A chin dressing is applied using Mastisol, surgical tape
(elastoplasty tape is used by the authors), and
Tegaderm designed to provide additional support and
limit edema to the chin postoperatively. C. The patient
is placed in a face bra once the dressing has been
applied to provide additional external support to the
mandible and soft tissues of the chin.
■ Variations
Osseous Genioplasty for Sagittal or Vertical Microgenia
■ Advancement and vertical lengthening (TECH FIG 5)

Osseous Genioplasty for Sagittal Macrogenia
■ Setback, typically with wedge resection (TECH FIG 6A–D)
Osseous Genioplasty for Vertical Macrogenia
■ Shortening, typically with wedge resection (TECH FIG 7A,B)
Osseous Genioplasty for Frontal or Vertical Asymmetry
■ Rotation (medial or lateral), typically with wedge resection and reduction
(TECH FIG 8A–D)
TECH FIG 5 • A,B. In cases of both vertical and
sagittal microgenia, a combination of advancement
and vertical lengthening will be required to achieve the
desired aesthetic result.

TECH FIG 6 • A,B. Preoperative planning for setback
genioplasty in a patient with sagittal and vertical
macrogenia. C,D. Setback genioplasty with wedge
resection for a patient with sagittal macrogenia.
TECH FIG 7 • A,B. The osseous genioplasty for
vertical macrogenia typically involves a wedge

resection and impaction of the bony segment,
reducing the vertical height of the chin.
TECH FIG 8 • A–D. In cases of combined vertical and
frontal asymmetry, a combination of wedge resection
and unidirectional rotation may be required to achieve
the desired result.
PEARLS AND PITFALLS
Preparation
■ When draping, it is essential to visualize the entire face and upper
neck given the chin’s role in facial harmony.
Intraoperative
edema
management
■ The use of preoperative Decadron can reduce postoperative edema
and lead to faster patient recovery and satisfaction.
Incision
■ Place the incision so that a cuff of muscle at the gingival border

planning
remains attached to the mandible to facilitate ease and precision of
closure at the end of the case and minimize postoperative
dehiscence.
Nerve
preservation
■ Do not avulse the mental nerves when mobilizing the bony
segment.
Blood supply
■ The genioglossus muscle should remain attached to the bony
segment because this is the primary blood supply to the bone flap.
Closure
■ It is critical to precisely reattach the mentalis muscle at the time of
closure to preserve functionality (lower lip motion) and aesthetics
(soft tissue volume/contour) and prevent chin ptosis, or witch’s
chin deformity.
POSTOPERATIVE CARE
■ Head elevation and ice pack application are encouraged to minimize edema.
■ Patients are instructed to use warm salt water rinses and Peridex rinses
postoperatively to manage oral flora.
■ A short course of clindamycin or amoxicillin is prescribed postoperatively to
reduce infection risk.
■ Patients are instructed to avoid sports, physical exercise, or activities that
might induce facial trauma for at least 6 weeks.
■ Dental soft diet is recommended for the first 1 to 2 weeks after surgery.
■ Pain control can be achieved with a combination of over-the-counter anti-
inflammatory medications (ie, ibuprofen and acetaminophen) and narcotics (ie,
hydrocodone or codeine prescriptions).
■ Patients are instructed to follow up in the clinic 1 week after surgery for
dressing removal and 3D photographic imaging (ie, Vectra camera).
OUTCOMES
■ Functional and aesthetic outcomes are excellent in thoughtfully planned and
well-executed procedures using osseous genioplasty to correct mild to
moderate sagittal chin deficiencies.
■ Osseous genioplasty provides the advantage of 3D versatility, larger
advancements, and decreased foreign body-related complications when
compared to alloplastic implants (ie, silicone, porous polyethylene).
■ Only osseous genioplasty addresses both vertical excess and anterior
mandibular asymmetry.

COMPLICATIONS
■ Mental nerve sensory deficits following this dissection have been reported in
the literature ranging from 6% to 10%.4–7
FIG 4 • Chin ptosis, or witch’s chin deformity, may result
if the mentalis muscle is not precisely reapproximated
at the time of closure resulting in a senile appearance.
■ Chin ptosis, or “witch’s chin” deformity, can result with failure to precisely
reapproximate the mentalis muscle after dissection8 (FIG 4).
REFERENCES
1. Ward JL, Garri JI, Wolfe SA. The osseous genioplasty. Clin Plast Surg.
2007;34(3):485-500.
2. Guyuron B. Genioplasty. Plast Reconstr Surg. 2008;121(4 Suppl):1-7.
3. Ward JL, Garri JO, Wolfe SA. The osseous genioplasty. Clin Plast Surg.
2007;34:485-500.
4. Posnick JC, Al-Qattan MM, Stepner NM. Alteration in facial sensibility in
adolescents following sagittal split and chin osteotomies of the mandible. Plast
Reconstr Surg. 1996;97(5):920-927.
5. Spear SL, Kassan M. Genioplasty. Clin Plast Surg. 1989;16(4):695-706.
6. Lindquist CC, Obeid G. Complications of genioplasty done alone or in
combination with sagittal split-ramus osteotomy. Oral Surg Oral Med Oral

Pathol. 1988;66(1):13-16.
7. Troulis MJ, et al. Extended genioplasty: long-term cephalometric,
morphometric and sensory results. Int J Oral Maxillofac Surg. 2000;29(3):167-
175.
8. Bernardi C, Amata PL, Dura S. Witch’s chin: a progressive three-step
technique. Plast Reconstr Surg. 1999;104(2):552-556.

45
CHAPTER
Alloplastic Chin
Augmentation
Barry L. Eppley and Sunil Tholpady
DEFINITION
■ Alloplastic chin augmentation involves a three-dimensional shape change
using implants.
■ The most common alloplastic chin augmentation used is with a silicone
material.1
ANATOMY
■ The chin is the anterior extension of the mandible, which has a dominant
influence on the shape of the lower face and the neck below it.
■ The shape of the chin is a function of the thickness of the bone and how well
the ramus and body of the mandible have developed.
■ Class II and III malocclusions are always associated with obvious aesthetic
chin deformities.
■ The classic ideal chin position aligns with a vertical line dropped from the
base of the nose and the forward position of the lips.
■ A male chin should fall close to this line, whereas an aesthetic female chin
can be positioned behind this line (FIG 1).
PATHOGENESIS
■ Chin hypoplasia should be viewed as a three-dimensional problem that
involves
■ Horizontal chin projection
■ Vertical chin height
■ Transverse chin width
NATURAL HISTORY

■ Once complete mandibular growth has occurred by the late teens or early 20s,
no further change in the chin shape or forward projection will occur.
FIG 1 • Aesthetic chin projection assessment in a
woman (A) and a man (B).
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Occlusion (class I or II)
■ Amount of horizontal chin weakness
■ Vertical chin height
■ Depth of labiomental fold
■ Desired position of chin determined with the help of computer imaging
■ Presence of chin dimples or vertical chin cleft
IMAGING
■ Standard imaging includes patient photographs from multiple views (front,
side, and oblique).
■ Cephalometric x-rays and pantomogram can be used to evaluate hard tissue
position and mental nerve foramen location.
■ Three-dimensional (3D) computed tomography (CT) is useful if a custom chin
implant is needed or prior chin implant or bony genioplasty has been
performed.
DIFFERENTIAL DIAGNOSIS
■ Isolated chin deficiency vs total mandibular deficiency

NONOPERATIVE MANAGEMENT
■ Synthetic injectable fillers
■ Fat injections
SURGICAL MANAGEMENT
■ Aesthetic chin augmentation can be performed at any age beyond the early
teenage years. Absolute skeletal maturity does not have to exist in severe chin
deficiencies.
■ There are no absolute contraindications other than the patient’s willingness to
accept a synthetic facial implant.
■ Determine what style and size of chin implant are needed based on aesthetic
objectives (FIG 2).
Preoperative Planning
■ Computer imaging of facial photographs should be completed before surgery
to determine the exact goals and expectations of the chin augmentation surgery.
Positioning
■ The patient is placed in the supine position on the operative table.
■ The procedure can be performed with local anesthesia, IV sedation, or general
anesthesia, depending upon patient and surgeon preference.
■ Regardless of anesthetic choice, local anesthetic of 2% Xylocaine with
1:100,000 Epinephrine is infiltrated into the proposed incision and the region
of the chin.
■ Mental nerve blocks can be performed first if the procedure is done under
local anesthesia.

FIG 2 • Clinical examples of different chin implant
styles.
T E C H N I Q U E S
■ Exposure and Pocket Creation
■ A submental incision employs a 3-cm incision within or directly behind the
submental skin crease (TECH FIG 1).
■ When using larger or custom chin implants, the incision may be placed
further behind the submental skin crease to compensate for significant
forward positioning of the soft tissue chin pad.
■ Dissection proceeds through the mentalis muscle to reach the inferior
portion of the bony chin.
■ An intraoral incision uses a 3-cm mucosal incision above the depth of the
vestibule on the lower lip side (TECH FIG 2).

■ Dissection proceeds through the superior attachment of the mentalis
muscle by leaving a cuff of muscle attached to the bone to aid closure.1,2
■ Subperiosteal dissection is performed over the entire chin bone, extending
laterally below the mental nerve foramen if the chin implant has extended
wings (such as an anatomic chin implant) (TECH FIG 2B).
■ The dissection should extend inferiorly to the mandibular border and
superiorly to accommodate the exact height of the implant.
TECH FIG 1 • A. Submental approach to chin
augmentation. B. Retraction of soft tissue gives good
exposure of the bony chin.
TECH FIG 2 • A,B. Intraoral approach to chin
augmentation.

■ Implant Placement and Fixation
■ The chin implant is placed into the pocket with the sides of the implant
extended into the lateral dissection along the inferior border of the mandible.
■ The midline marker on the implant helps achieve a centric position on the
bone.
■ Careful implant positioning includes checking for tilt, as this will lead to
exaggerated asymmetry at the lateral wings.
■ Fixation of a chin implant is not always necessary but is highly desired.
■ In a submental approach, fixation can be achieved with sutures or small
titanium screws (1.5 or 2.0 mm) along the inferior border.
■ In an intraoral approach, the only secure method to maintain the implant low
on the bone is screw fixation.
■ Closure over the implant should be performed in three layers: muscle,
dermis, and epidermal skin closure.
■ With intraoral approaches, resuspension of the mentalis muscle up and over
the implant is critical.
PEARLS AND PITFALLS
Presurgical
assessment with
computer imaging
■ Evaluate each patient for potential chin augmentation
changes from three picture views.
Chin implant selection
■ Chin implants change the projection of the lower face
in three dimensions, not just from a profile view.
Subperiosteal implant
placement
■ There is no biological advantage to place the implant
above the periosteum.
Implant positioning
■ The implant needs to slide back and forth freely in the
pocket to ensure that the lateral wings are not bent or
folded.
Intraoral approach
■ Screw fixation is essential to prevent immediate
upward migration of the implant.
Postoperative
management
■ Patients need to wait 6 to 12 wk after surgery for an

accurate assessment of the implant results.
POSTOPERATIVE CARE
■ A chin strap or tape may be used for a few days for postoperative compression.
■ Intraoperative IV steroids can be used to help decrease swelling.
■ Intraoperative IV and postoperative oral antibiotics with coverage of
staphylococcal and oral flora are preferred for 5 to 7 days after surgery.
OUTCOMES
■ Implants always increase chin projection.
■ The style and shape of the implant, as well as the patient’s expectations, will
determine whether the aesthetic objectives are achieved.
COMPLICATIONS
■ Infection usually presents as new swelling and pain 2 to 3 weeks after surgery.
■ Asymmetry can be assessed no sooner than 6 weeks after surgery when most
of the swelling has resolved in order to determine the midline positioning of
the implant and/or wing asymmetry.
■ Nerve impingement. Unremitting pain and numbness on one side that have
been present for up to a month after surgery may indicate mental nerve
compression by the wing of the implant.
■ Overcorrection/undercorrection. The surgeon should allow a full 3 months
after surgery for all swelling to subside and for the patient to adjust to his/her
new look. Earlier interpretation, particularly that of overcorrection, may be
premature.
REFERENCES
1. Romo T, Lanson BG. Chin augmentation. Facial Plast Surg Clin North Am.
2008;16:69-77.
2. Fanous N, Yoskovitch A. Estimating implant size in chin augmentation: a
simplified approach. Can J Plast Surg. 2003;11:161-165.

46
CHAPTER
Surgical Removal of Fibrous
Dysplasia From the
Craniofacial Skeleton
Alexander Govshievich and Gaby G. Doumit
DEFINITION
■ Fibrous dysplasia (FD) is a benign bone disorder characterized by progressive
replacement of normal bone by abnormal fibrous tissue.
■ FD accounts for 2.5% of all bony tumors and 5% to 7% of all benign bony
tumors.1
■ This disorder may be monostotic (MFD) or polyostotic (PFD), involving one
or more bones. The term craniofacial fibrous dysplasia (CFD) has been used to
describe FD confined to contiguous bones of the craniofacial skeleton.
■ PFD accompanied by café au lait spots and endocrinopathies including
hyperthyroidism, Cushing syndrome, and precocious puberty, is known as
McCune-Albright syndrome (MAS).
■ Craniomaxillofacial involvement in FD occurs in 27% of monostotic and up to
50% of polyostotic cases.2
■ FD can remain asymptomatic or present with pain, stress fractures, localized
swelling, or deformity.
■ In the craniofacial skeleton, neurologic impairment can result from nerve
compression by expansile FD lesions, potentially leading to blindness,
deafness, or spinal cord compression.
■ Diagnosis is based on an accurate clinical history, physical examination, and
imaging studies.
■ Bone biopsy is required to confirm diagnosis.
ANATOMY
■ Any bone of the craniofacial skeleton may be affected by FD.

■ Although the exact prevalence for each bone is unknown due to the relative
rarity of this condition, the maxillary bone is the most frequently involved in
several series.3–5
■ However, due to the compact and complex nature of the craniomaxillofacial
construct, solitary FD lesions of the skull are seldom confined to individual
bones, often affecting several contiguous structures.
■ As a result, surgical approach and treatment principles are focused on general
anatomic regions rather than individual bones.
■ Chen et al. classified the craniomaxillofacial region into four major zones
based on anatomic location, structures involved, surgical access, importance of
reconstruction, and recommended treatment methods (FIG 1).6
■ Zone 1: Includes the frontal, nasal, ethmoidal, and zygomatic bones as well
as the superior, non–tooth-bearing portion of the maxillary bone
■ Zone 2: Parietal, occipital, and squamous portion of the temporal bones
■ Zone 3: Petrous portion of the temporal bones including the mastoid process
and the sphenoid bone
■ Zone 4: Tooth-bearing portion of the maxillary bone and the mandible and is
separated into zones 4a and 4b
PATHOGENESIS
■ FD is a noninherited genetic disease stemming from a postzygotically
occurring missense mutation in the GNAS gene.
■ Two mutations have been described, resulting from replacement of the
arginine residue in codon 201 by either cysteine or histidine.
■ The mutation leads to somatic activation of the alpha subunit of signal-
transducer G protein (Gs), causing increased levels of cAMP and in turn
resulting in high rates of cellular proliferation.7
■ The abnormal proliferation and high cellular turnover produce poorly
differentiated osteoblasts and immature mesenchymal cells, which are
deposited within the bone and marrow of FD lesions.
■ Elevated cAMP levels have also been demonstrated to upregulate the growth
of thyroidal, adrenal, and gonadal cell lines resulting in the characteristic
endocrinopathies seen in MAS.

FIG 1 • Subdivision of the craniomaxillofacial region into
4 major zones as described by Chen et al.
NATURAL HISTORY
■ The onset and natural evolution of FD can be variable and unpredictable,
largely depending on lesion type.
■ MFD lesions generally develop in childhood, progress through puberty, and
arrest at the end of adolescence once skeletal maturity is complete.
■ PFD and MAS variants have been associated with more aggressive growth
patterns, demonstrating younger age at presentation and continued tumor
growth into adulthood. Up to 90% of these lesions may present before 15 years
of age.8
■ Lesions seen in MAS can continue growing indefinitely, causing progressive
disfigurement and functional deficits, requiring prompt surgical

intervention.9,10
■ Pregnancy and other settings of estrogen excess such as estrogen therapy have
been associated with heightened lesion growth.9,11
■ Spontaneous malignant transformation in FD is possible but is rare. It can be
observed in MFD, PFD, and MAS forms with reported frequencies ranging
from 0.5% to 6.7%.12
■ Any rapidly developing symptoms such as swelling or pain should raise
suspicion of malignant change and prompt the appropriate investigations.
■ Exposure to radiotherapy has been demonstrated to be the main risk factor for
malignant transformation, being associated with a 44% rate of sarcomatous
change. For this reason, radiotherapy is contraindicated in patients with FD.
■ Osteosarcoma accounts for the majority of cases, with the remainder
consisting of fibrosarcoma, chondrosarcoma, and malignant
fibrohystiocytoma.13
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Patient history should include the age at presentation, evolution of the lesion
(including periods of rapid growth), and associated symptoms.
■ Physical examination should characterize the location of the lesion, its
dimensions and any associated contour deformities, facial asymmetry, or
functional deficits.
■ Although FD is often asymptomatic, presenting as a slowly growing, painless,
and nontender swelling, it may be associated with a variety of signs and
symptoms that are specific to the anatomic zone affected.
■ Zone 1
• Hypertelorism, vertical dystopia, and exophthalmos from fronto-orbital
involvement and displacement of intraorbital contents
• Changes in visual acuity and blindness can result from impingement of the
optic nerve by lesions invading the anterior cranial fossa
• Nasal obstruction and recurrent sinusitis can be caused by sinus
involvement (FIG 2)
• Facial asymmetry secondary to tumor enlargement
■ Zone 2
• Headaches and elevated intracranial pressure (ICP) may result from
calvarial involvement.
• Cranial vault asymmetry secondary to tumor growth

• Macrocephaly
■ Zone 3
• Otalgia, fullness, conductive hearing loss, and cholesteatoma formation
from external auditory canal (EAC) involvement
FIG 2 • Lateral (A) and anterior (B) views of a patient
with large lower zone 1 fibrous dysplasia. Important
nasal airway involvement is seen.
• Vertigo, tinnitus, sensorineural hearing loss, or facial nerve paralysis from
invasion of the inner ear and internal auditory canal
• Visual disturbances or blindness with a lesion affecting the sphenoid bone
■ Zones 4a and 4b
• Displacement of teeth, vertical maxillary excess, or malocclusion
• Patients with lesions neighboring the orbit, namely, those in zones I and III,
must be followed with serial visual acuity and visual field measurements to
rule out impingement of the optic nerve. Conduction of neuro-
ophthalmological studies is also recommended.
• Given that craniofacial FD can be part of PFD or MAS, the primary clinical
assessment should include a full body examination looking for any
additional bony lesions, café au lait spots, or findings suggestive of
hyperthyroidism, Cushing syndrome, or precocious puberty.
• When suspected, a multidisciplinary approach with an endocrinologist is

recommended for further investigations and follow-up.
IMAGING
■ Radiologic investigation should be conducted in all patients with FD.
■ Radiologic appearance of lesions demonstrates three radiologic patterns,
namely, pagetoid, sclerotic, and cystic.13 Mixed changes also can be seen.
■ Standard roentgenography is of limited diagnostic use in FD. Images can
demonstrate expanded bone with ground-glass appearance, widened diploic
space with displacement of the outer table and sparing of the inner table, cystic
skull vault lesions, obliteration of paranasal sinuses, or dental malocclusions.
■ High-resolution CT (1-mm cuts) is the preferred imaging modality, providing
useful information for diagnosis, follow-up, and surgical planning. Three-
dimensional (3D) reconstruction can further assist in surgical planning.
■ Images obtained with CT scan:
■ Provide more accurate information regarding lesion size, location,
surrounding structures, and potential compression sites (FIG 3).
■ Appearance is related to lesion age, with younger lesions appearing more
radiolucent and mature lesions appearing more opaque. Ground-glass
appearance is seen in established lesions and often represents the best
diagnostic clue.9
FIG 3 • CT scan of patient with large lower zone 1
fibrous dysplasia. A. Sagittal cut. B. Coronal cut. C. 3D
reconstruction.
■ Bones affected by FD are usually expanded with intact cortices. Normal
corticomedullary differentiation is lost. The margin between abnormal and
normal bone is often difficult to identify because the two regions blend into

each other.
■ MRI is of lesser diagnostic value than CT. Image appearance is variable,
depending on the degree of lucency vs sclerosis.
■ T1- and T2-weighted images generally demonstrate a heterogeneous
appearance or decreased signal.
■ Injection of contrast demonstrates variable enhancement, which depends on
lesion type.
■ Bone scan has a very limited diagnostic role in FD; however, images can
provide useful information regarding the presence of secondary lesions in the
setting of PFD. Radionuclide uptake is variable.
DIFFERENTIAL DIAGNOSIS
■ Paget disease of bone
■ Cherubism
■ Interosseous meningioma
■ Garré sclerosing osteomyelitis
■ Cemento-ossifying fibroma
■ Craniometaphyseal dysplasia
■ Sclerotic metastasis
NONOPERATIVE MANAGEMENT
■ Nonoperative treatment in FD can be twofold, including expectant
management or medical therapy.
■ Expectant management is appropriate in most cases, particularly when lesions
are small, slowly growing or static, asymptomatic, and causing minimal
functional or aesthetic deficits.
■ Regular follow-up and routine annual checkups with CT imaging are required
to assess for progression or development of complications.
■ Medical therapy is primarily aimed at providing symptomatic relief and can be
used as an adjunct to surgery.
■ Acetaminophen and NSAIDs
■ Used for mild bone pain
■ Narcotics
■ Used for more severe bone pain
■ Bisphosphonates
■ Inhibit osteoclast activity, thus improving bone density and potentially

delaying progression of lesions into adjacent structures.4
■ Reduce pain associated with FD, improve radiologic appearance of lesions,
and decrease biochemical markers of bone remodeling (ie, serum ALP).14
■ Typically used in more severe cases such as those seen in MAS.
■ The most commonly used agent is pamidronate and is administered
intravenously every 6 months (180 mg for adults and 3 mg/kg in children and
adolescents).
■ Alendronate and zoledronate have also been used.
■ Monoclonal antibodies
■ The most commonly used agent is tocilizumab.
■ Decreases IL6 levels and subsequent osteoclast activity by binding to IL6
receptors.
■ Used for pain reduction in patients that fail to respond to bisphosphonate
treatment.
■ Calcium, phosphate, and vitamin D supplementation can be useful in patients
with deficiencies or mineralization defects such as hyperparathyroidism,
osteomalacia, and renal phosphate wasting.
SURGICAL MANAGEMENT
■ Surgery is the mainstay of treatment for symptomatic FD and is the only
definitive treatment modality.
■ Because of the complexity of the craniofacial skeleton and the important
structures it contains, a multidisciplinary team including otolaryngology,
ophthalmology, neurosurgery, and plastic surgery is required.
■ The presence of FD alone is not an indication for surgery because many
lesions remain static and asymptomatic and are often found incidentally with
CT imaging.
■ Indications for surgery include the presence of pain not controlled by medical
therapy, significant aesthetic deformity, functional deficit (ie, optic nerve
compression), rapidly growing lesions, or malignant transformation.
■ Surgical approaches are generally divided into shaving/contouring procedures,
subtotal/partial resections, and total or wide excisions.
■ Total resection of dysplastic bone is the preferred treatment modality (for
rapidly growing, malignant, or recurrent lesions), minimizing the risk of
recurrence and subsequent need for revisional surgeries. However, total
resection may not be feasible, particularly in extensive CFD lesions where
wide resection can significantly increase morbidity and the risk of

complications.
■ It is of paramount importance to ensure that the defect caused by the procedure
does not cause a greater functional or aesthetic deficit than present before
surgery. Risks and benefits should be clearly described to patients and families.
■ No guidelines exist for surgical management of FD. Treatment should be
personalized and guided by the patient’s age, lesion characteristics (location,
extent, and evolution), and the presence of any functional or aesthetic deficits.
■ Bone biopsy must be obtained before surgical intervention when diagnosis is
uncertain or when there is concern for malignancy.
■ Timing of intervention:
■ Defer surgical procedures until lesion growth subsides and skeletal maturity
is attained.
■ Earlier intervention may become necessary if progressive expansion of the
lesion causes significant functional, aesthetic, and psychological disturbances
to the affected child.
■ Conservative shaving is recommended in these cases. However, patients
should be informed that recurrence is possible and further contouring
procedures may be required, particularly in cases of PFD and MAS, where
continued growth is anticipated.
■ Conversely, if severe deformity is present, which cannot be corrected by
contouring, radical excision and reconstruction is indicated. Patients should be
made aware that recurrence is possible despite a wide excision.
■ In the adult population, static lesions causing a contour deformity can be
appropriately managed by shaving of the lesion. The presence of a functional
deficit (such as airway obstruction, oral obstruction or optic nerve
compression, etc.) warrants a more radical resection or an en bloc resection,
when feasible, and immediate reconstruction.
■ Zone 1
■ Represents the most cosmetically sensitive part of the face and is of particular
relevance to the craniofacial surgeon
■ Wide excision followed by immediate reconstruction is recommended in
cases of significant aesthetic or functional deficit, rapidly growing lesions,
malignant transformation, recurrent lesions, and severe pain.
■ The goals of reconstruction include restoration of facial skeletal integrity
(reconstruction of horizontal and vertical buttresses), symmetry, globe
position, and orbital volume and separation of cranial, orbital, and nasal

cavities.
■ Separation between the intracranial and nasal cavities if the anterior cranial
base is involved is of paramount importance to prevent infectious
complications.
■ Reconstruction is preferably performed using split-thickness calvarial bone
grafts (SCBG). If the calvarium is unavailable as a donor site due to extensive
involvement by FD, split rib grafts or iliac crest bone grafts may be used.
■ In cases of incomplete resection of a growing lesion where recurrence is a
concern, reconstruction can be temporized with a titanium mesh to match the
contralateral normal anatomy.
■ Zone 2
■ Represents most of the calvarial region of the cranium, a hair-bearing region
of lesser aesthetic importance, where both conservative and radical treatments
are possible.
■ Asymptomatic lesions can be adequately treated by shaving in order to
restore a symmetric profile.
■ Symptomatic lesions often require wide resection with immediate
reconstruction. A variety of materials can be used:
■ Autologous SCBG are preferred when the defect size does not exceed the
amount of graft available. Rib grafts and iliac crest grafts are other potential
donor sites. Reimplantation of autoclaved resected bone post remodeling has
also been described but is not recommended by the senior author.3
■ Cadaveric calvarial allografts.
■ Alloplastic materials such as titanium mesh, methyl methacrylate, porous
polyethylene, and polyetheretherketone (PEEK), particularly in the setting of
larger defects where the quantity of autologous material is insufficient.
■ Zone 3
■ Represents an anatomically complex area containing large vascular structures
and multiple cranial nerves.
■ Surgical intervention is challenging, generally avoided, and reserved
uniquely for symptomatic cases such as optic nerve compression or EAC
stenosis.
■ Optic nerve decompression should be strictly performed in symptomatic
cases in an effort to prevent further damage to the optic nerve and
deterioration of vision. Improvement of symptoms is not guaranteed.
• Unroofing of the canal is performed by the neurosurgery team following
anterior craniotomy, using an intracranial approach. Simultaneous bilateral

anterior clinoidectomy is often performed.
• Prophylactic decompression based solely on radiographic measurement of
optic canal narrowing is contraindicated and has been associated with severe
complications such as blindness.15 Lee et al. have demonstrated that
encasement of the optic canal in itself does not correlate with visual loss or
results of neuro-ophthalmologic examinations. Hence, the risks of this
procedure far outweigh the benefits.
■ EAC stenosis: Decompression is performed by the ENT team. The primary
goal of the procedure is to restore conductive hearing caused by EAC
stenosis.
■ Zone 4
■ Represents a functionally important area housing the dentition.
■ The main objective of surgical intervention in this area is to preserve the
native dentition.
■ The lesion should be managed with serial debulking procedures during
growth to address facial asymmetry.
■ Orthodontic management of malocclusion and orthognathic surgery
following arrest of growth provide stable results.16 Orthognathic surgery
within dysplastic bone is challenging as osteotomies must be performed
through solid bone.
■ Radical excision should be reserved only for cases of massive bone
involvement, severe functional impairment, or malignant degeneration.
■ Free bone graft techniques or free osseous flaps followed by placement of
osteointegrated dental implants offer good anatomical and functional
recovery.
■ Free flaps are indicated for bony deficits larger than 6 cm.
Preoperative Planning
■ The surgeon should discuss the risks and benefits of the procedure with the
patient and family.
■ All major risks and complications specific to the zone being operated on
should be explained, including the possibility of recurrence and need for
additional surgery.
■ The patient and family should be aware that intraoperative transfusion of
blood products may be required in case of bleeding and appropriate consent
should be obtained.
■ It is recommended to perform a bone biopsy and obtain a final

histopathological diagnosis prior to proceeding with definitive surgery when
the diagnosis is uncertain.
■ A CT scan of the craniofacial skeleton is essential for preoperative planning.
In cases of complex CFD, a 3D model of the craniofacial skeleton is helpful
during resection. A mirror-image 3D model of the nonaffected side is helpful
during reconstruction by facilitating bone graft reshaping and accurate plate
bending.
■ In the case of bilateral FD involvement, a 3D model based on CT data
matched for age, sex, and race is used in the reconstruction process.
■ Magnification loops with headlights can improve visualization during surgery.
Positioning
■ The patient is positioned supine with the head centered on a Mayfield head
rest.
■ The entire head and neck area is prepped; drapes are positioned around the
neck, and the head is left exposed to allow optimal intraoperative assessment of
symmetry and access to the calvarium if bone grafts are required.
■ For extensive lesions involving the nasal or oral airway where wide resection
is anticipated, particularly those in lower zone 1, a tracheostomy should be
performed by the ENT surgeon before the procedure. This is essential to ensure
adequate operative exposure and secure the airway both during the surgery and
in the postoperative period, as significant swelling may occur.
■ If orotracheal intubation is performed, the endotracheal tube should be secured
to the patient’s teeth with a 26-Fr stainless steel wire as a precautionary safety
measure.
Approach
■ The surgical approach and choice of incision are determined by the location of
the tumor, the anatomic structures involved, and the goal of the surgery.
■ In general, wide resections require wide exposure and a more invasive
approach is necessary.
■ For shaving or contouring procedure, however, a minimally invasive approach
should be privileged to minimize scar burden and avoid compromising facial
aesthetics.
■ In zone 1, the bicoronal and Weber Ferguson approaches are commonly used
for wide resection of lesions involving upper (supraorbital bar and frontal bone

region) and lower zone 1 (naso-orbito-ethmoidal [NOE] and
zygomaticomaxillary [ZM] regions).
■ A superior gingivobuccal sulcus incision is used for shaving of lesions located
in lower zone 1.
■ The bicoronal approach allows adequate exposure of the anterior and posterior
cranial vault for excision or shaving of lesions involving zone 2.
■ Access to lesions in zones 4a and 4b is obtained using superior and inferior
gingivobuccal sulcus incisions. In cases of wide resection, submandibular
incisions can be used.
T E C H N I Q U E S
■ Surgical Removal of Fibrous Dysplasia in Upper
Zone 1
Exposure
■ The bicoronal incision is marked in a zigzag fashion at least 5 cm behind the
hairline, extending from one auricular helix to the other.
■ The hair is combed away from the marked incision line and grouped in
bundles, which are twisted and secured with elastic bands.
■ A narrow corridor of hair (1–2 cm) can be shaved directly over the planned
incision to facilitate wound closure; however, this is not necessary.
■ The incision is infiltrated with a 1% Xylocaine solution containing 1:100
000 epinephrine. Incision is carried out with a no. 10 blade through skin,
subcutaneous tissue, and galea, parallel to the orientation of the hair follicles.
■ Monopolar cautery use is minimized to diminish the risk of alopecia.
■ Bipolar cautery is used to perform hemostasis.
■ Incision margins are lifted and Raney clips are applied along the anterior and
posterior flaps for hemostasis.
■ Anteriorly, dissection proceeds in the loose areolar plane until 2 cm above
the superior orbital rim.
■ If no pericranial flap is anticipated, the periosteum is incised horizontally
from one temporal fusion line to the other, and dissection proceeds
subperiosteally until adequate exposure of the nasofrontal and supraorbital

regions is achieved.
■ If a pericranial flap may be required, that is, with lesions involving the
anterior cranial base, the periosteum is incised at the level of the skin
incision and laterally along the temporal fusion lines. It is then dissected off
the skull bone using sharp periosteal elevators and surgery proceeds as
above (TECH FIG 1).
■ Laterally, the temporal fusion lines are released. Dissection is carried
immediately superficial to the deep temporal fascia until the superficial
temporal fat pad is reached.
■ At this point, the superficial layer of the deep temporal fascia is incised, and
sharp dissection is carried just superficial to the temporal fat pad until the
superior aspect of the zygomatic arch and posterior aspect of the lateral
orbital rim are reached. Here, the periosteum is incised, and dissection is
merged with that described for the anterior segment.
■ If access to the orbital roof and medial orbital wall is required, the
supraorbital neurovascular bundles must be identified and carefully released
from within the confines of the superior orbital notch.
■ If a foramen is present, it is converted into a notch using a 2-mm osteotome
by dividing a small bony wedge.
■ Dissection then proceeds subperiosteally until the orbital roof and medial
wall are exposed.
■ The trochlea of the superior oblique muscle can be released with minimal
consequences if required.

TECH FIG 1 • After the anterior scalp has been raised
in the subgaleal plane, a pericranial flap is designed.
The periosteum is incised posteriorly at the level of the
skin incision and laterally along the temporal fusion
lines. It is then dissected off the calvarium using sharp
periosteal elevators.
■ Preserve the medial canthal ligament and lacrimal apparatus unless resection
of the lacrimal bone is planned.
■ Lateral orbital wall dissection is performed with a Freer while retracting the
globe with a malleable. The lateral canthal ligament can be released from the
Whitnall tubercle to increase exposure and facilitate supraorbital bar
resection.
Resection
■ Once adequate exposure has been achieved, the extent of the FD lesion is
evaluated. Correlation to preoperative CT imaging and the use of anatomical

landmarks such as the distance to the zygomaticofrontal or nasofrontal
sutures can further aid in accurate identification of lesion margins.
■ Moderate to severe frontal bone deformities are marked for resection. Mild
deformities are burred down to achieve symmetry.
■ Resection begins with the frontal craniotomy segment, which is carried out
by the neurosurgery team. Bur holes are made at the extremities of the
planned resection and on either side of the sagittal sinus. A side-cutting saw
with a footplate is used to connect the bur holes.
■ The frontal bone is dissected off the dura. Dissection then proceeds
inferiorly to expose the anterior cranial base if required.
■ Optic nerve decompression is performed at this point by the neurosurgery
team when indicated.
■ Lesions involving the supraorbital bar are excised using a reciprocating saw.
The orbital contents and brain must be protected with malleable retractors at
all times to avoid injury.
■ Constant irrigation is used to minimize thermal damage to surrounding
tissues.
■ Warm bone wax, bipolar cautery, and hemostatic agents (ie, Floseal) are used
for hemostasis.
Reconstruction
■ Once the CFD lesion is completely removed, the anatomical deficit is
assessed.
■ Preferred reconstruction is carried using SCBG.
■ SCBG can be used to reconstruct all necessary segments, including the
frontal bone, anterior skull base, and superior orbital rim.
■ If all the above structures are involved, the superior orbital rim should be
addressed first, followed by the anterior cranial base and the frontal bone.
■ Ideally, grafts are outlined on the parietal cranium in the hair-bearing region
to optimize symmetry and diminish donor-site morbidity. The graft is usually
harvested from the parietal bone of the nondominant hemisphere, at least 1
cm lateral to the sagittal suture. The other hemisphere can be used if
additional material is required.
■ In cases of unilateral FD involvement, a malleable bent to the shape of the
unaffected fronto-orbital bar can be used to help find a harvest site with a
curvature most suitable to ensure a symmetric result.

■ Strips of bone to be used for reconstruction are outlined and their outer
cortex is burred with a 2-mm bur until diploe is reached. An osteotome is
used to divide the diploic layer and elevate the outer cortex graft. Utmost care
should be taken to avoid breaching the inner cortex. Hemostatic material may
be used for the donor site if needed.
■ A second option is full-thickness harvest of the outlined cranial bone by the
neurosurgeon, followed by ex vivo division of the two cortices on a side
table.
■ Grafts should span the defect and be secured edge to edge to healthy bone on
either end using plates and at least three screws from the 1-mm cranial set (4–
5 mm long) on each side.
■ Following restoration of the fronto-orbital bar, the anterior cranial base is
addressed.
■ It is advised to reconstruct the orbital roof with a titanium mesh as bony
resorption is known to occur in this region. An overlay of bone graft can be
placed if desired by the surgeon, but it is not necessary.
■ In the event where the nasal cavity or ethmoid bone has been violated, the
pericranial flap is secured over the titanium mesh to ensure a seal between
the nasal and intracranial cavities.
■ The pericranial flap is secured to the surrounding tissue and bone with a few
simple interrupted 4-0 Vicryl sutures.
■ It is of extreme importance to repair any dural tear and obtain a watertight
seal prior to closure. Tisseel is often used for this purpose.
■ Finally, the frontal bone deficit is addressed using the SCBGs, completing
reconstruction of the FD defect.
■ If the lateral canthal ligament was released, a lateral canthoplasty is
performed with a 4-0 Vicryl or a lateral canthoplasty stainless steel suture.
■ While protecting the globe, two 1.5-mm holes are drilled through and
through, above and below the zygomaticofrontal suture (3 mm posterior to
the rim).
■ The lateral canthal ligament is grasped with a single hook. Lateral tension on
the ligament with the hook should cause lateral movement of the corner of
the palpebral fissure. Once properly identified, the lateral canthal ligament is
sewn with a figure-of-8 stitch. One limb of the suture is passed through the
hole superiorly, while the second is passed inferiorly and then tied.
■ A Jackson-Pratt drain is placed on the cranium and the scalp is unfolded.

■ Raney clips are removed and hemostasis is ensured.
■ A two-layer closure is performed using interrupted 2-0 Vicryl sutures for the
galea and a running 4-0 nonresorbable suture or staples for the skin.
■ Antibiotic ointment is applied along the incision and a lightly compressive
head dressing is placed.
■ Surgical Removal of Fibrous Dysplasia in Lower
Zone 1
Exposure
■ A subcranial approach using a Weber Ferguson incision is preferred and
should be used for NOE and ZM lesions found below the nasofrontal suture
and sparing the cribriform plate.
■ Involvement of the cribriform plate will require the addition of a bicoronal
incision, elevation of a pericranial flap as described above, and frontal
craniotomy to ensure an adequate protection to the brain and a full seal
between the brain and nasal cavities.
■ A tracheotomy should be performed by the ENT surgeon for patients with
extensive NOE lesions where wide resection is anticipated, particularly if the
nasal airways are involved.
■ This is essential to ensure adequate operative exposure and secure the
airway both during the surgery and in the postoperative period, as
significant swelling may occur.
■ The incision is marked along the ipsilateral philtral column, around the nasal
ala, and superiorly along the nasofacial groove (TECH FIG 2).
■ Markings can extend laterally along a subciliary incision line, which can be
used if additional exposure is required.
■ Cupid’s bow is marked and tattooed to ensure exact reapproximation during
closure.
■ The incision line is infiltrated along its length with 1% Xylocaine with
epinephrine 1:100 000.
■ Using a no. 10 blade, the skin is incised along the nasofacial groove and
around the nasal ala until the periosteum is reached.
■ The superior lip is cut through and through.
■ Intraorally, a superior gingivobuccal incision is made 5 mm buccal to the

sulcus in order to preserve a mucosal cuff for closure.
■ Mucosa, submucosa, and muscles are divided directly down to bone.
TECH FIG 2 • Weber Ferguson incision markings on
the patient in FIG 2.
■ The flap is then elevated using a no. 9 periosteal elevator in the subperiosteal
plane, and the hemiface is retracted superolaterally. Identify the infraorbital
nerve (ION), which is usually found 4 to 7 mm inferior to inferior orbital rim
in line with the midpupil.
■ The ION should be preserved if the lesion’s location is medial to it. For more
extensive lesions, the ION is scarified and dissection proceeds laterally until
the FD is fully exposed.
■ A subciliary incision can be added for further access. The skin is incised
longitudinally 2 mm below the lash line. The pretarsal and some of preseptal
orbicularis oculi muscle should be preserved to prevent postoperative
ectropion.
■ Sharp subcutaneous dissection is performed to raise the inferior skin flap
over the muscle along the length of the incision. The muscle is transected at
the level of the inferior orbital rim, and dissection proceeds until the
periosteum to join the plane of the flap raised in the steps above.
■ The arcus marginalis is released.
■ The orbit is dissected with a Freer in a subperiosteal plane while protecting
the globe with a malleable.
Resection

■ With the entire lesion exposed, two approaches are available for complete
excision (TECH FIG 3A).
■ First, an en bloc resection can be performed with osteotomies carried
peripheral to the FD lesion. The extent of resection varies depending on the
size and location of the FD.
■ For large and extensive inferior zone 1 lesions as demonstrated in this
patient, resection begins superiorly by performing an osteotomy at the level
of the nasofrontal suture using a reciprocating saw (TECH FIG 3B).
■ The position of the cribriform plate should be determined to avoid
violating the intracranial cavity.
■ The osteotomy extends medially across the lacrimal and ethmoid bones
and then extended laterally along the orbital floor using a 3-mm osteotome
until the inferior orbital fissure is reached.
■ The globe is retracted and protected at all times with a malleable retractor.
■ Frequent communication between the anesthesiologist and surgeon is
maintained to monitor for the oculocardiac reflex.
■ The zygoma is cut at the level of the body or arch with a reciprocating saw.
■ The osteotomy proceeds laterally along the lateral wall of the maxilla.
■ Inferiorly, the osteotomy should be at least 3 mm above the dental roots (as
measured from preoperative CT or 30 mm from the crown of the canine) to
preserve the native dentition, as reconstruction of this region is
cumbersome, particularly in a growing child.
■ Osteotomy of the vomer is performed with a 5-mm osteotome from the
nasofrontal suture, in a 45-degree posterior angle aiming toward the
junction of the soft-hard palate.
■ The surgeon’s nondominant hand is placed on the palate to prevent a full-
thickness laceration.
■ Constant irrigation is performed during osteotomies to avoid thermal injury
to the bone.
■ Once all osteotomies have been completed, the FD lesion is removed en
bloc and hemostasis ensured using bipolar cautery, warm bone wax, and
hemostatic agents such as Floseal.
■ A second approach used by the senior author for very large lesions consists
of centrally coring out the lesion and separating the FD form healthy bone
along a well-defined plane. This technique is preferred with lesions abutting
the cribriform plate as it may prevent the addition of an intracranial approach
by limiting the extent of resection.

TECH FIG 3 • A. Raising of the flap is complete and
the fibrous dysplasia lesion is completely exposed. B.
Osteotomy lines for en bloc resection of a large
bilateral lower zone 1 FD lesion as seen in our patient.
The osteotomy begins superiorly at the level of the
nasofrontal suture and extends medially across the
lacrimal and ethmoid bones and then laterally along
the orbital floor until the inferior orbital fissure. The
zygoma is cut at the level of the body or arch, and the
osteotomy proceeds laterally along the lateral wall of
the maxilla. Inferiorly, it is important to keep the
osteotomy at least 3 mm above the dental roots. C.
After central coring of the lesion, a surgical plane is
sought between the FD and healthy bone. D. Once

revealed, dissection proceeds along this distinct layer
between the FD and healthy bone until the exophytic
part of the lesion is removed (arrow) and the origin of
the lesion identified. E. Complete removal of FD
lesion.
■ The exophytic core of the lesion is debulked using a reciprocating saw
starting centrally and proceeding concentrically outward until the outer
margin of the lesion is approached.
■ At this stage, a surgical plane is sought between the FD and healthy bone.
When present, the shell of the FD can collapse inward, revealing a distinct
layer between the FD and healthy bone (TECH FIG 3C,D).
■ Excision proceeds along this plane until the exophytic portion of the lesion
is removed and the origin of the lesion identified. The latter is then burred
until no dysplastic bone remains (TECH FIG 3E).
■ In this case, the approach allowed removal of the FD lesion while sparing
the cribriform plate and avoiding an intracranial approach.
Reconstruction
■ Reconstruction of the midface must re-establish the normal skeletal anatomy.
The horizontal buttress, vertical buttress, and nasal bone are essential to
obtain normal projection and width of the midface.
■ Reconstruction of the midface skeleton is performed preferentially with
cranial bone graft. The bone grafts are harvested from the parietal cranium
through a coronal or a sagittal incision, as described above.
■ A perfected computer-aided design (CAD) 3D model of the patient’s
craniofacial skeleton can be ordered and printed with the midface segment
virtually reconstructed (corrected to normal anatomy for patient age, race,
and sex or a mirror image of the normal side when possible; TECH FIG 4A).
■ The 3D model is used to shape the bone graft, bend the titanium plate (1.5-
mm midface system), and facilitate positioning of the construct (TECH FIG
4B). This method of reconstruction increases symmetry and improves
accuracy.
■ Because of the expansile nature of this lesion and this patient’s history of 10
previous debulking procedures, a 15 × 15 cm titanium mesh (1.0 mm thick)
was bent on the corrected 3D model preoperatively.

■ Independent mesh panels were used for both hemiface regions. The panels
were sterilized for reconstruction.
■ Bony construction was performed using large premolded titanium mesh,
which was prepared prior to surgery and sterilized. This was done in order to
preserve autologous reconstruction options in case of future recurrence.
■ The prebent midface mesh panels were fixed to the zygoma, nasofrontal
suture, and maxillary bone with multiple 4- to 6-mm screws (TECH FIG
4C).
■ The orbital mesh was attached to the midface mesh with 28-gauge wires.
■ An opening was made in the mesh to replicate the piriform aperture.
■ For reconstruction of the orbital floor, titanium mesh was bent in a lazy S
shape with extension to reconstruct the medial orbital wall. The mesh was
fixed medially and superiorly to the frontal bone with 4-mm screws and
laterally to the zygomatic bone.
■ The medial canthal ligament is grasped with a 4-0 PDS (or canthal wire
suture) in a figure-of-8 pattern and passed in a superior oblique vector across
the mesh. A similar step is performed on the contralateral side. Both ends are
tied together.

TECH FIG 4 • A. 3D CT perfected model with a
virtually reconstructed midface segment corrected to
normal anatomy for patient age, race, and sex. B.
Once printed, the perfected 3D model is used to bend
the titanium plates to the desired shape. C. Titanium
meshes with medial extensions bent in a lazy S shape
are used to reconstruct the orbital floors and medial
walls bilaterally. These are fixed medially and
superiorly to the frontal bone and laterally to the
zygomatic bone. Two prebent midface mesh panels
are then fixed to the zygoma, nasofrontal suture, and
maxillary bone. D. Placement of SCBGs for
reconstruction of large bilateral lower zone 1 defects.
E. Closure of Weber Ferguson incision. Image of

patient in early postoperative period. F. Lateral view of
patient in immediate postoperative period
demonstrating adequate midface projection.
■ The midface periosteum was suspended to the orbital rim with two 3-0
Vicryl sutures positioned at the level of the pupil and lateral canthus to
prevent midface ptosis.
■ A lateral canthoplasty was performed.
■ Lacrimal duct system reconstruction was not performed but was delayed
until a later stage.
■ Strips of SCBG were used to reconstruct the orbital rim. The bone grafts
were fixed with the 1.0- or 1.5-mm plating system using 4-mm screws.
■ The orbital floor and medial wall are then reconstructed as described above
with a titanium mesh or bone graft.
■ The zygomatico-maxillary and nasomaxillary buttress are then reconstructed
similarly with bone graft. These grafts are fixed with the 2.0-mm plating
system.
■ A cantilever bone graft was used to reproduce nasal contour and projection,
secured to the frontal bone using miniscrew fixation (TECH FIG 4D).
■ After ensuring hemostasis, closure begins with resuspension of the midface
to avoid facial ptosis by reattaching the periosteum of the midface. At least
two suspension sutures should be utilized, one at the level of the pupil and
the second at the level of the lateral canthus. The sutures grasp the
periosteum 3 cm inferior to the pupil and lateral canthus and suspend them to
the orbital rim using 3-0 Vicryl sutures.
■ The WF flap is then closed using standard two-layer closure using 3-0 Vicryl
and 4-0 nylon (TECH FIG 4E,F).
■ A lateral tarsorrhaphy suture is considered if the patient is likely to develop
chemosis.
■ Antibiotic ointment is placed over the incision line.
PEARLS AND PITFALLS

History and
physical
findings
■ Complete history and physical examination should be performed
in order to differentiate between the various forms of FD (MFD,
PFD, MAS).
■ Visual examination, including neuro-ophthalmological studies,
should be obtained for lesions in zones 1 and 3.
Preoperative
■ High-resolution CT scan is the preferred imaging modality to plan
for FD resection. 3D reconstruction can further assist in planning.
■ Bone biopsy and definitive pathological diagnosis are
recommended prior to definitive surgical treatment when
malignant fibrous dysplasia is suspected.
■ Medical therapy with bisphosphonates (ie, pamidronate) can be
used as an adjunct to surgery, providing symptomatic relief for
painful lesions.
Surgical
management
■ Surgical management is the only definitive treatment modality and
should be reserved for lesions causing pain unrelieved by medical
therapy, significant aesthetic deformity, functional deficit (ie, optic
nerve compression), rapidly growing lesions, recurrent lesions, or
malignant transformation.
■ Surgical treatment should be deferred until growth of the lesion
subsides and skeletal maturity. Shaving is recommended for
growing lesions.
■ Optic nerve decompression should only be performed for
symptomatic cases. Prophylactic decompression can be associated
with severe complications such as blindness.
Technique
■ Goals of reconstruction include restoration of facial skeletal
integrity, projection and symmetry (reconstruction of horizontal,
vertical buttresses and nasal bone), globe position, and orbital
volume.
■ A pericranial flap should be planned when anterior cranial fossa
resection is planned in order to separate the nasal and cranial
cavities.
■ Titanium mesh reconstruction should be used in orbital roof and
anterior cranial fossa reconstruction as late bony absorption occurs
with split cranial bone graft.
■ For large lesions, particularly in lower zone 1, central coring of the
lesion should be attempted prior to en bloc resection, as a distinct
plane may exist between FD and healthy bone, allowing to
minimize the resection and potentially sparing an intracranial
approach.
■ Inferior osteotomy for lesions in lower zone 1 should remain 3
mm above the dental routes when possible to preserve the native

dentition.
■ The orbital contents and brain must be protected at all times using
a malleable. Continuous irrigation should be used when using the
reciprocating saw around these structures to avoid thermal injury
to bone.
■ Immediate reconstruction with SCBG is preferred; however, in
cases of incomplete resection of growing lesions where recurrence
is a concern, reconstruction can be temporized with a titanium
mesh.
Postoperative
■ Careful long-term postoperative follow-up is essential to survey
recurrence. Annual CT scanning is recommended.
POSTOPERATIVE CARE
■ ICU care is essential for intracranial resection or when airway swelling is
expected.
■ Visual assessment in the first 48 hours is performed every 4 hours while
awake.
■ Postoperative antibiotics for at least 24 hours are essential in the setting of
intracranial resection.
■ Lateral tarsorrhaphy sutures are removed when chemosis has resolved.
■ Peridex mouth wash (TID and with oral intake) and teeth brushing are
important in the 1st week following surgery.
OUTCOMES
■ Careful long-term postoperative follow-up is essential to survey recurrence.
FIG 4 shows the patient 5 months following surgery.
■ Annual clinical assessments and CT scanning are recommended.
■ In a large series of pediatric CFD, the mean time to recurrence has been
demonstrated to be 2.25 years.
■ Recurrence rates are generally low and depend on several patient-specific and
treatment-specific factors:15,16
■ Time of intervention: Surgery before skeletal maturity is associated with a
higher rate of recurrence.
■ Type of intervention: Debulking is associated with a higher rate of recurrence
when compared to wide excision and reconstruction.
■ Type of lesion: Highest rates of recurrence are seen in patients with MAS,
followed by PFD and MFD.

■ Serum ALP has been demonstrated to be a useful marker to monitor for
disease recurrence.
■ Rising levels can be useful when malignant transformation is suspected.
FIG 4 • Anterior (A) and lateral (B) view of patient 1
year following surgery.
COMPLICATIONS
■ General complications associated with operative management of FD in the
craniofacial skeleton include bleeding/hematoma, surgical site infection, facial
asymmetry, and recurrence.
■ Zone 1
■ Superior sagittal sinus laceration
■ Dural tear/brain injury
■ Globe injury
■ Anterior cranial base fracture and communication between intracranial and
nasal cavities
■ Meningitis
■ Optic nerve injury
■ Infraorbital or supraorbital nerve injury
■ Midface ptosis
■ Ectropion
■ Alopecia
■ Zone 2
■ Superior sagittal sinus laceration
■ Dural tear/brain injury

■ Zone 3
■ Optic nerve injury
■ Temporal hollowing
■ Zone 4
■ Dental root injury
■ Inferior alveolar nerve injury
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