Geoffrey 3 C. Gurtner, Peter C. Neligan, Daniel Z. Liu – Plastic Surgery-Craniofacial, Head and Neck Surgery-Pediatric Plastic Surgery. 3-Elsevier (2018)

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CHAPTER 14 • Pharyngeal and esophageal reconstruction
The loss of the implantable Doppler signal should prompt
immediate exploration following clinical evaluation with the
potential for a high false positive rate. Nonetheless, a negative
exploration for malfunction of the implantable Doppler is
preferable to delaying exploration and having to manage a
dead flap.
Recipient vessel selection
The recipient vessels are critical for any microvascular free
tissue transfer and should be confirmed prior to committing
to a free flap reconstruction. When available, the authors
prefer to use branches off the external carotid such as the
lingual or facial arteries which are usually an adequate size
match for an ALT flap, jejunal flap, or forearm flap. The
superior thyroid artery, which is anatomically the first branch
off the external carotid, is often smaller at 1.5–2 mm but may
serve as an excellent size match for a lateral arm flap which
usually has an artery comparable in size. Recipient veins are
usually a branch off the internal jugular vein such as the facial
vein or its branch so that end-to-end anastomosis can be
completely using a coupler. However, if there are no branches
available, an end-to-side anastomosis can also be completed
in a hand-sewn fashion.
In the setting of a “frozen neck”, there may be a paucity of
recipient vessels. The great vessels can be encased in scar, and
dissection to expose these vessels can pose significant risk for
carotid artery rupture. We prefer using the transverse cervical
vessels as potential recipients.57 The right side is preferred
over the left side to avoid injury to the thoracic duct (Fig.
14.25A); however, both sides can be used safely with careful
dissection. In most cases, the transverse cervical artery arises
from the thyrocervical trunk which originates from the sub-
clavian artery. However, there are a number of anatomical
variations as the artery can arise directly from the subclavian
in 21% of cases and even the internal mammary artery in 2%
of cases. The transverse cervical artery crosses anterior to the
paddle from a fasciocutaneous flap, being either the ALT (Fig.
14.8D), radial forearm (Fig. 14.22), or UAP flap (Fig. 14.19E),
can usually be created based on a second perforator either for
neck resurfacing or solely for monitoring purposes.56 A
segment of the vastus lateralis muscle can also be externalized
for monitoring (Fig. 14.23). In the setting where a second
paddle was not necessary or if a monitoring segment was not
included with the flap harvest, alternate means of flap moni-
toring need to be employed. The use of the implantable
Cook–Swartz Doppler has been used for monitoring buried
flaps and can be placed on the artery, vein, or both. Recently,
the introduction of the Flow coupler (Synovis Inc., Birming-
ham, AL, USA) combines an implantable venous Doppler
with the coupler device used to complete the venous anasto-
mosis (Fig. 14.24). The utility of these new technologies
remains to be determined; however, in the setting when no
monitoring segment or skin paddle is available, these devices
represent a useful adjunct to detect microvascular thromboses.
Fig. 14.22  A second skin island of the radial forearm flap can be created based on
a separate proximal perforator.
Fig. 14.23  A vastus lateralis muscle can be externalized as a monitor during
anterolateral thigh flap reconstruction.
Fig. 14.24  The Synovis Flow Coupler combines the venous coupler and an
internal Doppler probe that is used for simultaneous venous anastomosis and flap
monitoring.

375
Reconstruction of post-laryngectomy pharyngocutaneous fistulas
recipient vein. The transverse cervical vessels are available in
92% of the cases in our experience even in the presence of a
frozen neck.57 The supraclavicular region is usually free from
radiation damage and surgical scars from previous neck dis-
section as modern neck dissections for laryngeal cancer often
spare level V.
In certain circumstances, the use of vein grafts may be
necessary to reach more distant recipient vessels. The internal
mammary vessels can serve as excellent recipient vessels in
the setting that no other vessels are readily available.58,59
However, vein grafts are typically necessary in order to reach
the internal mammary vessels. We recommend isolating the
vessels in the third intercostal space after removing the third
costal cartilage. Careful attention should be paid to avoid
puncturing the pleura during this dissection. Alternatively,
with the help of a thoracic surgeon, the clavicular head and
manubrium are resected and the internal mammary vessels
dissected out all the way to their origin and turned upward
to the neck to avoid vein grafting.
Reconstruction of post-laryngectomy
pharyngocutaneous fistulas
Since radiotherapy became the primary treatment for early
stages of laryngeal and hypopharyngeal cancers, physicians
have faced a new problem in these patients: higher rates of
pharyngocutaneous fistulas after salvage total laryngectomies
for recurrent cancers. Although the overall incidence of post-
laryngectomy pharyngocutaneous fistulas has decreased sig-
nificantly in the past decade, it remains very high in previously
irradiated patients.60–62 McCombe et al. reported that the
incidence increased from 4% to 39% when radiation was
included in multi-modal therapy.61
Once fistulas develop, the radiation effects are compounded
by salivary contamination of the neck, leading to chronic
inflammation and hypoxia of the neck skin and vasculature
and putting the patient at risk for loss of neck skin and the
remaining pharynx, tracheostoma compromise, and even
carotid artery rupture. Longstanding fistulas will inevitably
promote scar formation leading to pharyngeal stricture. These
more severe cases create a very difficult challenge for even the
most experienced reconstructive surgeon.
The goals of surgical reconstruction should be to minimize
surgical morbidity and mortality in addition to providing
reliable reconstruction that achieves primary healing and a
reasonable appearance and restores swallowing and speech
functions. The strategies for reconstruction are the same as
those for managing a frozen neck. During surgery, the fistula
track and scar tissues are excised, without exposing the great
vessels in the neck if they are cancer free. All patients have
various degrees of pharyngeal stricture, particularly those
with a longstanding fistula. The hypopharynx is opened
superiorly to the base of the tongue, where the pharyngeal
inlet is incised laterally and widened to approximately two
to three fingers wide. This is important because primary
closure of the pharynx following a total laryngectomy
inevitably narrows the pharyngeal inlet. In patients with
a relatively normal pharynx and cervical esophagus and
no complications, a small degree of narrowing may not
cause dysphagia. After pharyngoesophageal reconstruction,
brachial plexus and middle scalene muscle on its way to the
lateral border of the levator scapulae muscle. The artery typi-
cally lies deep to the omohyoid which serves as a good
landmark to identify the vessels (Fig. 14.25B). The supracla-
vicular artery which is the pedicle for the supraclavicular flap
usually arises from the transverse cervical artery. The main
transverse cervical artery continues laterally, and its descend-
ing branch enters to supply the trapezius muscle. The trans-
verse artery is dissected out proximal to the take-off of the
supraclavicular artery or even to the thyrocervical trunk to
gain adequate caliber and flow. There is usually a transverse
cervical vein that accompanies the artery. Alternatively, the
external jugular vein near the clavicle can be used as a
SCM
Clavicle
A
Fig. 14.25  To explore the transverse cervical vessels, an incision was made toward
the mid-clavicle, lateral to the sternocleidomastoid muscle. The right side is
preferred to avoid injury to the thoracic duct (A). The omohyoid muscle is a good
landmark to locate the transverse cervical vessels (B).
B

376
SECTION II
CHAPTER 14 • Pharyngeal and esophageal reconstruction
A
B
Fig. 14.26  Resection of tracheostoma recurrence often exposes the previously
radiated great vessels (A). An internal mammary artery perforator (IMAP) flap is a
reliable and simple flap for this type of reconstruction (B). The flap heals well
although it may distort the nipple (C).
C
however, food transit through the reconstructed conduit is
compromised no matter what type of flap is used. Any nar-
rowing in the pharyngeal inlet or weakness of the base of
the tongue thus further compounds the problem and causes
dysphagia. Once the pharyngoesophageal defect is recreated,
the transverse cervical vessels are explored and a multi-
island ALT flap is employed to reconstruct the defects as
described earlier.
We have seen that delayed repair of post-laryngectomy
fistulas often result in a severe frozen neck and a circumfer-
ential pharyngoesophageal defect. Therefore, early surgical
intervention to reconstruct the neck in these patients once
large fistulas develop is strongly recommended.63 Reconstruc-
tive surgery can be performed shortly after the development
of the fistula (within days) before severe fibrosis sets in. Surgi-
cal dissection in the neck is therefore much easier than in
patients with a longstanding fistula. The use of various
amounts of the well-vascularized vastus lateralis muscle to
repair neck defects may help to achieve primary healing.
Reconstruction of isolated cervical
esophageal defects with an
intact larynx
The vast majority of tumors arising in the cervical esophagus
are malignant, and laryngectomy is often required to achieve
tumor-free margins.58,64 For benign tumors such as schwanno-
mas and granular cell tumors, smaller margins are acceptable,
but resection of a portion of the cricopharyngeus muscle
increases the risk of reflux and aspiration. Therefore, the
reconstruction of isolated cervical esophageal defects can be
challenging. With an intact larynx and trachea, exposure of
the cervical esophagus is limited, making reconstruction
technically difficult. Given the small space around the esopha-
gus, a free tissue transfer offers the best flexibility for insetting
the flap; a thin flap is required for the same reason. The radial
forearm flap is, therefore, the author’s flap of choice for both
partial and circumferential defects. These defects are usually
only a few centimeters long, so the radial forearm flap can be
oriented 90° to the conventional flap design so that the width
of the flap becomes the length of the neoesophagus. The
longitudinal length of the flap is rolled along the vascular
pedicle to form a tube. The transverse cervical vessels are
preferred since they are closer to the defect.
Reconstruction of tracheostoma
recurrence
Trachesostoma recurrence following a previous total laryn-
gectomy often requires tracheal resection and sometimes
a total esophagectomy. For small recurrence, the anterior
tracheostoma, the manubrium, and both clavicular heads
are often resected, exposing great vessels which are often
radiated (Fig. 14.26A). A pectoralis major myocutaneous
flap or an IMAP flap (Fig. 14.26B,C) can be used for such
reconstruction.34 The IMAP flap is based on the perforator
in the second intercostal space, and the flap is designed
horizontally parallel to the ribs. The distal extent of the flap

377
Reconstruction of tracheostoma recurrence
A
B
Fig. 14.27  For combined total esophagectomy and tracheal defects, a supercharged jejunal flap is used for esophageal reconstruction (A). An anterolateral thigh (ALT) flap
is used for tracheal reconstruction (B). The ALT flap is sutured to the tracheal remnant using parachuting sutures before the jejunal flap is pulled through the mediastinum to
the neck. The jejunal flap is then supercharged to the internal mammary vessels, and the ALT flap is revascularized to the transverse cervical vessels. The parachuting
sutures are then tied down to the trachea (C). The ALT flap provides stable tracheal reconstruction (D) while the patient could tolerate a soft diet.
C
D
can reach the axilla. The flap is then rotated superiorly to the
defect.
When a total esophagectomy is also performed combined
with tracheal resection, our preferred approach is to use the
supercharged jejunal flap for total esophageal reconstruction65
and use the ALT flap for tracheal reconstruction (Fig. 14.27).66
Recipient vessels include the right transverse cervical vessels
for the ALT flap and the left internal mammary vessels for the
supercharged jejunal flap. The ALT flap is tubed and sutured
to the tracheal remnant with 3-0 polydioxanone (PDS) sutures
in a parachute fashion so as not to obstruct the passage of the
supercharged jejunal flap. The jejunal flap is then pulled up
to the neck through the substernal route and re-vascularized.
The parachuting sutures of the ALT flap for tracheal recon-
struction are then tied down. This sequence is important
because once the jejunal flap is pulled to the neck, it is nearly
impossible to suture the ALT flap to the trachea as the jejunal
flap and its mesentery tend to obstruct the tracheal remnant.
The superior end of the ALT flap is inset to the neck and chest
skin. A Shiley tracheostomy tube is placed in the tube ALT
flap to stent the lumen open. The patient should be spontane-
ously breathing at this time point to avoid positive ventilation

378
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CHAPTER 14 • Pharyngeal and esophageal reconstruction
drainage. Early washout may prevent pedicle thrombosis and
allow primary closure of the neck incision. Serious infections
should also raise the suspicion of a fistula or a potentially
even dead flap which will be described later in this chapter.
In such circumstances, a staged approach is recommended
with serial washouts and debridements before definitive
repair.
Fistula
Pharyngocutaneous fistulas occur in 10% and 6% of patients
with ALT flap reconstruction for circumferential and partial
defects, respectively.26 Proximal fistulas are rare with the ALT
flap unless there is a dehiscence due to technical errors or
poorly vascularized tissue. Fistulas usually develop between
1 and 4 weeks postoperatively and manifest as leakage of
saliva or liquids or, in some patients, as a neck infection.
Therefore, any neck infection or abscess that occurs after a
pharyngoesophageal reconstruction should raise suspicion
for anastomotic leakage. Risk factors for fistula formation
include improper suturing techniques, poor tissue quality at
the anastomosis site, previous radiotherapy, and turbulent
postoperative course. At the time of surgery, any questionable
tissue should be trimmed.
Once a fistula is identified, oral intake is withheld and local
wound care is initiated. Small fistulas, in the absence of tumor
recurrence or distal obstruction, usually heal spontaneously
within 2 weeks with conservative management. Therefore, an
modified barium swallow (MBS) is repeated 2 weeks later if
the leakage has stopped. Larger fistulas or those with infec-
tion should be evaluated with computed tomography to rule
out abscess and assess the proximity of the fistula/abscess to
the carotid artery. Any dead space or abscess around the
carotid artery, especially in patients who have undergone
previous radiotherapy or chemoradiation, should be thor-
oughly but carefully debrided without jeopardizing the
carotid artery. A pectoralis major muscle flap is commonly
used to fill the dead space. This should be done as soon as
possible to prevent rupture of the carotid artery. Attempting
to repair the leak or dehiscence at this stage will not be suc-
cessful and may cause more tissue damage.
Stricture
Circumferential defects and subsequent reconstruction were
significantly more prone to strictures than partial defects. For
these reasons, it is always critical to preserve the posterior
mucosa if possible, and converting a partial pharyngectomy
defect to a circumferential defect should be avoided. The
proximal anastomosis is not typically prone to stricture as the
base of tongue and oropharynx is usually of adequate diam-
eter that should allow easy passage of a food bolus. The distal
anastomosis is not surprisingly the source of most strictures
and subsequent dysphagia. For these reasons, we recommend
spatulating the distal anastomosis and insetting a dart into a
longitudinal opening in the cervical esophagus. This not only
widens the diameter of the distal anastomosis, but also inter-
rupts a circumferential scar, thereby reducing the likelihood
of a stricture.
If a patient develops a stricture, the assistance of a gastro-
enterologist is indicated to dilate the stricture. The use of a
bougie with a rigid laryngoscopy to dilate the stricture is no
which can cause air leakage or disruption at the ALT-trachea
anastomosis. The Shiley tube is replaced with a soft Lary tube
on postoperative day 3, which is managed as regular post-
laryngectomy patients.
Complications
As with any complex surgery, particularly involving micro-
vascular free tissue transfer, there are always the potential
risks of bleeding, infection, and the most dreaded complica-
tion of loss of the free flap requiring a secondary free flap in
order to reconstruct the defect. In our experience, our success
rate for free flap head and neck reconstruction is greater than
97%, but 99% for pharyngoesophageal reconstruction.67 In the
setting that a flap is lost, careful analysis is necessary to
identify the underlying etiology for the flap loss before
embarking on another free flap.
Medical complications occur frequently since alcohol and
tobacco use is prevalent in this patient population. Tradition-
ally, these patients are kept in the intensive care unit (ICU)
overnight on a ventilator after surgery. Because of the need
for sedation, hypotension is common and many patients
become fluid overloaded due to fluid resuscitation since
vasopressors are generally considered contraindicated after
free flap reconstruction. Patients with limited cardiopulmo-
nary reserve, such as those with pre-existing cardiopulmonary
conditions and elderly patients may easily develop congestive
heart failure and respiratory failure. The senior author’s
current practice is to extubate or discontinue mechanical
ventilation at the completion of surgery and allow patients to
go to regular recovery and regular flap floor instead of ICU.
We have shown that this practice significantly reduces the
incidence of medical complications and shortens length of
stay.68 If ICU monitoring is indicated, patients are allowed to
breathe spontaneously in the ICU to avoid mechanical venti-
lation and heavy sedation. In patients with a history of alcohol
abuse and narcotic dependence, postoperative confusion and
agitation are common and may cause hypertension, hema-
toma, anastomotic breakdowns, and avulsion of the vascular
pedicle. Therefore, prompt management of these issues with
the primary team, pain management, and neuropsychiatric
staff is important.
Infection
Given the prior radiated and compromised tissue and long
surgery time, along with the contamination from the com-
munication with the oral cavity, there is a high risk for infec-
tion. We routinely irrigate the neck with copious amounts of
antibiotic irrigation prior to closure and recommend place-
ment of closed suction drains to minimize the risk of a fluid
collection that can progress to an abscess. Patients are also
maintained on intravenous antibiotics, usually Unasyn,
during the entirety of their hospital stay. Hematoma can
also lead to infection. Meticulous hemostasis is therefore
important.
Early signs of infection, including increased swelling and
erythema, can sometimes be masked by the prior radiated
tissue. Occasionally, broadening the antibiotic coverage is
adequate in treating a superficial cellulitis; however, any signs
of a possible abscess warrant surgical exploration and

379
Postoperative function
our experience, a secondary TEP has higher success rates
compared to a primary TEP and, therefore, it is our preference
to perform the TEP secondarily several months following the
reconstruction. The TEP is performed by puncturing the
common wall between the esophagus and trachea 1.5 cm or
2 cm below the rim of the tracheostoma. In patients with a
very low resection of the cervical esophagus below the trache-
ostoma, the puncture needs to go through the flap and the
posterior tracheal wall, which is more difficult to do. Initially,
a 14 Fr. red rubber catheter is inserted through the TEP and
subsequently changed to voice prosthesis in 2–4 weeks once
the tract has matured. The patients are followed up regularly
by speech pathologists to adjust the prosthesis to obtain a
tight fit so the device cannot be dislodged. Complications
include widening of the puncture site with leakage around
the voice prosthesis, frequent mucus plugs inside the prosthe-
sis, and fungal infections. In our series of 349 patients, 147
patients received a TEP and 87% of them were able to achieve
fluent speech.69
Swallowing function
For patients who have received prior radiation, an MBS is
performed to assess for a leak approximately 6 weeks follow-
ing a fasciocutaneous flap reconstruction and 2 weeks in
patients that have not received radiation. In cases when small
leaks are noted, patients are maintained on tube feeds and the
MBS is repeated in 2 weeks, and most will heal spontaneously.
Large leaks are usually apparent clinically and managed as
previously described. If there is no evidence for a leak, patients
are started on a liquid diet and then advanced to a soft or
pureed diet in 3 days.
longer recommended since there is a risk for perforation of
the esophagus. At our institution, endoscopic balloon dilation
is the preferred method of dilating an anastomotic stricture.
Unfortunately, while dilation may provide some initial relief,
the majority of patients will develop progressive dysphagia
and will likely need to have the dilation repeated in the future
and will eventually require surgical correction or depend on
tube feeding. Surgical repair may be achieved with a radial
forearm flap or by turning the external skin paddle inward
for small strictures.
Postoperative function
The goal of reconstruction for pharyngoesophageal defects is
to restore gastrointestinal continuity so patients will be able
to tolerate an oral diet and also potentially be able to undergo
a TEP for speech rehabilitation. In our experience, swallowing
function is comparable between a fasciocutaneous flap and an
intestinal flap, although patients will likely need to drink
more fluid to help lubricate the food bolus down the recon-
structed conduit. However, speech function tends to be much
superior with fasciocutaneous flaps compared to intestinal
flaps.24,26
Speech rehabilitation
Tracheoesophageal puncture (TEP) is the preferred method of
voice rehabilitation and affords patients the possibility of
speech after a total laryngectomy. The TEP can be performed
at the time of surgery (primary TEP) or several months after
surgery with use of endoscopy (secondary TEP); however, in
1. Mikulicz J. Ein Fall von Resection des Carcinomatosen Esophagus
mit Plastischem Ersatz des Excidirten Stuckes. Prag Med Wchnschr.
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2. Wookey H. The surgical treatment of carcinoma of the hypopharynx
and the oesophagus. Br J Surg. 1948;35:249.
3. Wookey H. The surgical treatment of carcinoma of the pharynx and
upper esophagus. Surg Gynecol Obstet. 1942;75:499.
4. Bakamjiam VY. A two-stage method for pharyngoesophageal
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5. Shefts LM, Fischer A. Carcinoma of the cervical esophagus with
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9. Frederickson JM, Derrick JH, Wagenfeld MB, et al. Gastric pull-up
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internal mammary artery perforator flap in head and neck
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36. Shayan R, Syme DY, Grinsell D. The IMAP flap for
pharygoesophageal reconstruction following stricture release. J Plast
Reconstr Aesthet Surg. 2012;65(6):810–813.
37. Schellekens PP, Paes EC, Hage JJ, et al. Anatomy of the vascular
pedicle of the internal mammary artery perforator (IMAP) flap as
applied for head and neck reconstruction. J Plast Reconstr Aesthet
Surg. 2011;64(1):53–57.
38. Wong C, Saint–Cyr M, Rasko Y, et al. Three- and four-dimensional
arterial and venous perforasomes of the internal mammary artery
perforator flap. Plast Reconstr Surg. 2009;124(6):1759–1769.
39. Mirghani H, Leymarie N, Amen F, et al. Pharyngotracheal fistula
closure using the internal mammary artery perforator island flap.
Laryngoscope. 2014;124(5):1106–1111.
40. Hsu PW, Yu P. Complex salvage of a failed pharyngoesophageal
reconstruction with impending airway disaster. Plast Reconstr Surg.
2010;125:208e–210e.
41. Lin SJ, Rabie A, Yu P. Designing the anterolateral thigh flap without
preoperative Doppler or imaging. J Reconstr Microsurg. 2010;26:
67–72.
42. Yu P. Inverse relationship of the anterolateral and anteromedial
thigh flap perforator anatomy. J Reconstr Microsurg. 2014;30:
463–468.
43. Yu P, Selber JC. Perforator patterns of the anteromedial thigh flap.
Plast Reconstr Surg. 2011;128:151e–157e.
44. Yu P, Selber JC, Liu J. Reciprocal dominance of the anterolateral and
anteromedial thigh flap perforator anatomy. Ann Plast Surg.
2013;70:714–716.
45. Antony AK, Hootnick JL, Antony AK. Ulnar forearm free flaps in
head and neck reconstruction: systematic review of the literature
and a case report. Microsurgery. 2014;34:68–75.
46. Azizzadeh B, Yafai S, Rawnsley JD, et al. Radial forearm free flap
pharyngoesophageal reconstruction. Laryngoscope. 2001;111:807.

379.e2
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CHAPTER 14 • Pharyngeal and esophageal reconstruction
47. Scharpf J, Esclamado RM. Reconstruction with radial forearm flaps
after ablative surgery for hypopharyngeal cancer. Head Neck.
2002;25:261.
48. Yu P, Chang EI, Selber JC, Hanasono MM. Perforator patterns of the
ulnar artery perforator flap. Plast Reconstr Surg. 2012;129(1):213–220.
49. Huang JJ, Wu CW, Lam WL, et al. Anatomical basis and clinical
application of the ulnar forearm free flap for head and neck
reconstruction. Laryngoscope. 2012;122(12):2670–2676.
50. Marques Faria JC, Rodrigues ML, Scopel GP, Kowalski LP, Ferreira
MC. The versatility of the free lateral arm flap in head and neck
soft tissue reconstruction: clinical experience of 210 cases. J Plast
Reconstr Aesthet Surg. 2008;61(2):172–179.
51. Nahabedian MY, Deune EG, Manson PN. Utility of the lateral arm
flap in head and neck reconstruction. Ann Plast Surg. 2001;46(5):
501–505.
52. Wax MK, Briant TD, Mahoney JL. Lateral-arm free flap for
reconstruction in the head and neck. J Otolaryngol. 1996;25(3):
140–144.
53. Reece GP, Bengtson BP, Schusterman MA. Reconstruction of the
pharynx and cervical esophagus using free jejunal transfer. Clin
Plast Surg. 1994;21:125–147.
54. Perez–Smith D, Wagels M, Theile DR. Jejunal free flap
reconstruction of the pharyngolaryngectomy defect: 368 consecutive
cases. J Plast Reconstr Aesthet Surg. 2013;66:9–15.
55. Moradi P, Glass GE, Atherton DD, et al. Reconstruction of
pharyngolaryngectomy defects using the jejunal free flap: a 10-year
experience from a single reconstructive center. Plast Reconstr Surg.
2010;126:1960–1966.
56. Ferguson RE, Peirong Yu. Techniques of monitoring buried
fasciocutaneous free flaps. Plast Reconstr Surg. 2009;123:525–532.
57. Yu P. The transverse cervical vessels as recipient vessels for
previously treated head and neck cancer patients. Plast Reconstr
Surg. 2005;115(5):1253–1258.
58. Schneider DS, McClain L, Robb PK Jr, Rosenthal EL, Wax MK. Use
of internal mammary vessels in head and neck microvascular
reconstruction. Arch Otolaryngol Head Neck Surg. 2012;138(2):
172–176.
59. Roche NA, Houtmeyers P, Vermeersch HF, Stillaert FB, Blondeel
PN. The role of the internal mammary vessels as recipient vessels in
secondary and tertiary head and neck reconstruction. J Plast
Reconstr Aesthet Surg. 2012;65(7):885–892.
60. Genden EM, Rinaldo A, Shaha AR, et al. Pharyngocutaneous fistula
following laryngectomy. Acta Otolaryngol. 2004;124:117–120.
61. McCombe AW, Jones AS. Radiotherapy and complications of
laryngectomy. J Laryngol Otol. 1993;107:130–132.
62. Weber RS, Berkey BB, Forastiere AA, et al. Outcome of salvage total
laryngectomy following organ preservation therapy: the radiation
therapy oncology group trial 91-11. Arch Otolaryngol Head Neck
Surg. 2003;129:44–49.
63. Iteld L, Yu P. Pharyngocutaneous fistula repair after radiotherapy
and salvage total laryngectomy. J Reconstr Microsurg. 2007;23(6):
339–345.
64. Marin VP, Yu P, Weber RS. Isolated cervical esophageal
reconstruction for rare esophageal tumors. Head Neck. 2006;28:
856–860.
65. Poh M, Selber JC, Skoracki RJ, Walsh GL, Yu P. Technical challenges
of total esophageal reconstruction using a supercharged jejunal flap.
Ann Surg. 2011;253:1122–1129.
66. Ghali S, Chang EI, Rice DC, Walsh GL, Yu P. Microsurgical
reconstruction of combined tracheal and total esophageal defects. J
Thorac Cardiovasc Surg. 2015;50(5):1261–1266.
67. Chang EI, Zhang H, Liu J, et al. Analysis of risk factors for flap loss
and salvage in free flap head and neck reconstruction. Head Neck.
2016;38(suppl 1):E771–E775.
68. Clemens MW, Hanson SE, Rao S, et al. Rapid awakening protocol
in complex head and neck reconstruction. Head Neck. 2015;37:
464–470.
69. Selber JC, Xue A, Liu J, et al. Pharyngoesophageal reconstruction
outcomes following 349 cases. J Reconstr Microsurg.
2014;30(9):641–654.

SECTION II • Head and Neck Reconstruction
15 
Tumors of the facial skeleton:  
Fibrous dysplasia
Alberto Córdova-Aguilar and Yu-Ray Chen
SYNOPSIS
■ Tumors of the facial skeleton may arise from multiple cellular lineages.
However, tumor-like lesions are more common than true bone tumors.1
The most common craniofacial tumor-like lesion encountered by
plastic surgeons is fibrous dysplasia.2
■ Fibrous dysplasia is a benign fibro-osseous lesion in which normal
bone is replaced by fibro-osseous tissue. It can present as an isolated
disease or in association with other syndromes.
■ Craniofacial fibrous dysplasia commonly presents as a gradual,
painless, immobile mass enlargement resulting in facial asymmetry.
■ Diagnosis is based on a combination of clinical, radiographic, and
sometimes histological findings.
■ Indications for surgical intervention include aesthetic consideration,
functional impairments, relief of symptoms, and in situations where
malignancy cannot be ruled out.
■ The two main approaches to reach the facial skeleton are through the
coronal and intraoral incisions. Surgery can be divided into
conservative bone contouring or radical excision with immediate
reconstruction. Planning these reconstructions usually requires a
multidisciplinary team.
■ Many of the principles applied in the management of fibrous dysplasia
can be extended to other tumors of the facial skeleton.
Introduction
Since the skeletal framework basically determines the facial
appearance, many factors come into play when treating a
patient with a craniofacial tumor. Understanding the features
of these tumors, the interaction of surface deformities, and
their underlying anatomic counterpart is paramount to design
an individualized solution for each patient. Due to the
complexity of the craniofacial region and the consequences
of inappropriate treatment, planning these reconstructions
usually requires a multidisciplinary team.
The facial skeleton includes the frontal bone, the midface
bones, and the mandible. Tumors of these bones may arise
from multiple cellular lineages; however, primary malignant
tumors are rare. In contrast, tumor-like lesions are more
common than true bone tumors.1 The most common cranio-
facial tumor-like lesion encountered by plastic surgeons is
fibrous dysplasia;2 therefore, by using it as a model, many of
the principles applied in the management of this pathology
can be extended to other tumor types. As will be seen, this
chapter reviews the clinical considerations, including a
detailed differential diagnosis with other bone tumors and
surgical approaches in patients with fibrous dysplasia. More-
over, the surgical techniques presented here give the surgeon
full access to the facial skeleton, as well as various strategies
for the resection and reconstruction of other craniofacial
tumors.
Access the Historical Perspective section online at

Basic science/disease process
General considerations
Tumors of the facial skeleton can be classified as either primary
or secondary, but also as benign or malignant. Primary tumors
may arise from osteogenic, chondrogenic, fibrogenic, vascular,
hematopoietic, and other elements of the bone, as opposed to
secondary tumors which originate in other organs and metas-
tasize to the skeleton. By definition, benign osseous tumors
do not metastasize and may vary in etiology (developmental,
traumatic, infectious, or inflammatory). Many lesions of the
facial skeleton that may mimic primary bone tumors, such
as metastatic lesions and non-neoplastic processes, over-
whelmingly exceed the incidence of true bone tumors.1 Fre-
quent craniofacial conditions simulating primary bone tumors
or tumor-like lesions are giant cell reparative granuloma,
Langerhans cell histiocytosis, Paget’s disease, and fibrous
Access video and video lecture content for this chapter online at expertconsult.com

380.e1
Historical perspective
Historical perspective
Fibrous dysplasia has been a prevalent disease for a long time.
This is confirmed by two typical cases of the disease which
have been reported in the skeleton of a seventh century
Anglo-Saxon from around 650 AD, and the other in a prehis-
toric American-Indian around 1000 AD.3,4 Nevertheless, it was
not until 1891, on the occasion of the 71st birthday of Rudolf
Virchow, that his students published a Festschrift in his honor.
One of the contributors to this work was the German patholo-
gist Professor Friedrich Daniel von Recklinghausen, who
reported the postmortem findings in the skeletons of various
patients who had suffered from various bone diseases. From
these patients were three who showed changes characteristic
of “osteitis fibrosa cystica generalisata”; however, von Reck-
linghausen referred to an earlier description by Gerhard Engel
in 1864.5,6 Indeed, for many years, primary hyperparathyroid-
ism and fibrous dysplasia were described pathologically and
radiologically as “osteitis fibrosa cystica generalisata”. This
confusion existed due to fibrous replacement of the bone
marrow which is an important histological feature in both
diseases. Fortunately, in 1930, another pathologist Christian
Georg Schmorl described the specific biochemical changes in
hyperparathyroidism and the histology of bone sections in
Paget’s disease leading to the separation of these pathologies
which are common conditions in the differential diagnosis of
fibrous dysplasia.
A few years later, a pediatrician, Donovan James McCune,
in 1936, described a patient who showed the polyostotic
form of fibrous dysplasia, precocious puberty, multiple skin
pigmentation, and hyperthyroidism.7 An endocrinologist,
Fuller Albright, published a case with similar characteristics
in 1937.8 Although this condition was later referred to as
McCune–Albright syndrome, the first recorded case appears
to be that of Weil in 1922.9 The following year (1938) an
American pathologist named Louis Lichtenstein described
eight cases and suggested the term “polyostotic fibrous
dysplasia” to designate a skeletal developmental abnormal-
ity affecting one or several bones with a predominantly
unilateral distribution, and in 1942 he and his colleague
Henry Lewis Jaffe reported fifteen further cases and reviewed
the literature to include a total of 90 cases, introducing the
term “fibrous dysplasia” to define a group of bony lesions,
previously designated under a variety of titles. Even though
the term “fibrous dysplasia” was coined in 1942, it does
not appear in the Index Medicus (MEDLINE precursor)
until 1967.10,11

381
Basic science/disease process
craniofacial skeleton. Therefore, this pattern cannot be catego-
rized as monostotic or polyostotic owing to the involvement
of multiple adjacent bones of the craniofacial skeleton. Typi-
cally, bones outside of the craniofacial complex are usually not
involved. Approximately 50–100% of patients with polyostotic
disease will have craniofacial involvement, whereas only
10% with monostotic lesions will have involvement of
these bones.20 Skull involvement occurs in 27% of monostotic
patients and up to 50% of polyostotic patients. Maxillary
lesions may extend to include the zygoma, sphenoid bone,
maxillary sinus, and floor of the orbit. The mandibular body
is the most frequently affected region of the mandible.
The polyostotic form is a sine qua non for syndromic cases,
such as Mazabraud, Jaffe–Lichtenstein, or McCune–Albright
syndromes. Mazabraud syndrome is FD with soft tissue
myxoma, usually intramuscular. Jaffe–Lichtenstein syndrome
is FD with café-au-lait (coffee with milk) pigmentation of the
skin. McCune–Albright’s syndrome is FD lesions with café-
au-lait skin pigmentation and multiple endocrinopathies.
Endocrine dysfunction may produce gonadal hyperfunction
leading to sexual precocity (particularly in females), hyper-
thyroidism, hyperparathyroidism, Cushing’s disorder, acro-
megaly, and pituitary adenomas. In addition, the borders of
the skin lesions are typically serrated or irregular usually
described like the “coast of Maine”, as opposed to the regular
margins observed in the café-au-lait spots of patients affected
with neurofibromatosis. Specific not just to FD but also
observed in Paget’s disease, and secondary to chronic renal
failure is a clinical term “leontiasis ossea”.21 Leontiasis ossea
refers to a rare condition in which facial skeleton overgrowth
results in a lion’s face appearance. Recognizing the aforemen-
tioned syndromic constellations of symptoms allows early
diagnosis and appropriate treatment.
Natural progression and clinical behavior
FD lesions develop during skeletal growth and have a vari-
able natural evolution (Fig. 15.2). The phenotype depends on
the stage of development and timing of where the mutation
occurs; the earlier during embryogenesis the mutation, the
more widespread and severe the disease.14 Genetic studies
have shown that as FD lesions age, more of their mutated
stem cells fail to regenerate and their previous offspring
die by apoptosis, leading to normal bone cells increasing in
population and allowing for more normal skeleton to be pro-
duced. This explains why lesions typically stop progressing
after adolescence; however, pregnancy may stimulate their
growth.22 Even though reactivation in females may be due to
the elevation in sex hormone receptors in the mutated cells,
this event is also exhibited in males.23
dysplasia. The most common craniofacial tumor-like lesion
encountered by plastic surgeons is fibrous dysplasia.2
Fibrous dysplasia (FD) is a non-inherited genetic hamar-
tomatous disorder characterized by an intramedullary fibro-
osseous lesion of affected bones. Although FD can occur in
any bone, the most common locations are the craniofacial
bones, proximal femur, and rib. In the craniofacial region, it
frequently involves the maxillary bone, followed by mandible,
frontal, sphenoidal, ethmoidal, parietal, temporal, and occipi-
tal bones.12
Demographics
The incidence and prevalence of FD are difficult to estimate,
but it is reported to be approximately 1 : 4 000–1 : 10 000 and
1 : 30 000 individuals, respectively.13,14 FD represents 3% of all
bone tumors and around 7% of benign bone tumors.15 The
disease does not appear to have any gender predilection,
except in McCune–Albright syndrome, which is more com-
monly found in females. Lesions tend to appear unilaterally
without side predominance.16
Etiology and pathophysiology
The precise etiology of FD is currently unknown but has been
hypothesized to be associated with a genetic predisposition.
The pathogenesis involves a sporadic postzygotic mutation in
somatic cells located on the GNAS1 (guanine nucleotide
binding protein, α-stimulating activity polypeptide 1) gene
found on chromosome 20q13.2-13.3, which is responsible for
the formation of the alpha subunit of stimulating G-proteins.17
The mutation leads to sustained activation of adenylate
cyclase, thus producing increased cyclic adenosine mono-
phosphate (cAMP) activity. In skeletal tissues, overproduction
of cAMP impairs the ability of bone-forming mesenchyme to
transition from stem-like immature cells to mature osteogenic
cells. In addition, one of the downstream effectors of cAMP is
an increased secretion of interleukin-6 and expression of
receptor activator of nuclear factor κ-B ligand (RANK-L),
which increases the number of osteoclasts leading to
bone resorption.18 Incidentally, an overactive cAMP signaling
pathway also stimulates the growth of certain tissues such as
gonads, thyroid, adrenal cortex, and melanocytes, leading to
endocrinopathies and skin pigmentation.19
Classification and associations
The classic forms of FD (Fig. 15.1) can present in a single bone
or monostotic (80–85%), and multiple bones or polyostotic
(15–25%). A variant, termed craniofacial fibrous dysplasia,
involves lesions that are confined to contiguous bones of the
Fig. 15.1  Classification of fibrous dysplasia. MAS
(McCune–Abright syndrome).
Non-Syndromic
Syndromic
FIBROUS
DYSPLASIA
• Mazabraud
• Jaffe-Lichtenstein
• MAS
Polyostotic
Monostotic

382
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
A1
A2
A3
A4
B1
B2
Fig. 15.2  Natural progression in a patient with craniofacial fibrous dysplasia. (A1–A4) A 11-year-old patient diagnosed with left orbito-zygoma-maxillary FD lesion.
(B1–B4) Ten-year follow-up without surgical treatment.

383
Basic science/disease process
Continued
B3
B4
C1
C2
C3
C4
(C1–C4) Twelve-year follow-up without surgical treatment.
Fig. 15.2, cont’d

384
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
D1
D2
D3
D4
(D1–D4) Seventeen-year follow-up without surgical treatment.
Fig. 15.2, cont’d
Clinical presentation may occur at any age; however, the
polyostotic form usually manifests before the age of 10 and
the monostotic form between 5 and 20 years old. A longitudi-
nal study has shown that monostotic FD does not progress to
any polyostotic form.24 In McCune–Albright syndrome, while
growth of the bone lesions may also diminish after puberty,
the overall degree of bony enlargement and deformity is often
more severe and disfiguring than in patients with monostotic
or polyostotic non-syndromic FD.
Histopathology
While almost all the facial skeleton arises from intramembra-
nous ossification, the cranial base arises from an endochondral
process. This is important because FD is more common in
membranous rather than endochondral bones.
FD bone lesions are characterized by woven ossified
tissues, increased formation of bone matrix which does not
mineralize normally with extensive marrow fibrosis. Failure
of bony maturation leaves a mass of immature isolated
trabeculae enmeshed in dysplastic fibrous tissue that are
turning over constantly without completion of the remodel-
ing process. This is why the macroscopic appearance of FD
may appear as white and brown rigid mass located in the
medullary cavity.25
Microscopically, there are three main subtypes of the
disease. The Chinese writing type is commonly found in the
long bones and axial skeleton (rib, vertebrae). The bone
trabeculae are thin and disconnected, with ongoing osseous
resorption by osteoclasts. Frequently, resorption of the interior
of bone trabeculae, so-called “dissecting resorption”, similar
to findings in hyperparathyroidism. The osteogenic cells are
star-shaped, and numerous Sharpey fibers are present. Another
pattern, described as a sclerotic or pagetoid type is commonly
found in cases of fibrous dysplasia involving nongnathic
craniofacial bones. The appearance is similar to osseous tissue
found in Paget’s disease, with dense and sclerotic trabecular
tissue. The last one, the hypercellular type, is characterized
by the presence of discontinuous bone trabeculae distributed
in an ordered and sometimes parallel fashion. Typically, the
sides of trabeculae are associated with multiple osteoblasts
which are arranged in multiple layers. This type appears to
occur commonly in gnathic bones. Like the pagetoid variant,
a significant amount of bone is present in the hypercellular
type.26 It is notable that histology of FD does not predict the
biological behavior of these lesions.27

385
Diagnosis/patient presentation
needed if rapid swelling and pain occur in a pre-existing FD
lesion.31
Imaging
Since many patients are asymptomatic, the diagnosis is often
made as an incidental radiologic finding. However, as a
general principle, almost all patients in which FD is suspected,
as well as other tumors of the facial skeleton, should undergo
radiological imaging. Radiographic features of any craniofa-
cial tumor should evaluate location, margins and zone
of transition, periosteal reaction, mineralization, size and
number of lesions, and involvement of a soft-tissue. While
benign tumors usually grow slowly with cortical expansion,
have well-defined and often sclerotic borders, and may
produce a solid or smooth periosteal reaction; malignant
lesions usually show poor marginal definition, along with
cortical destruction, a soft tissue mass, and a spiculated or
interrupted periosteal reaction.32
Plain films
Radiography constitutes the initial imaging tool for describ-
ing primary bone tumors and tumor-like conditions. However,
owing to the overlapping of adjacent structures, plain radio-
graphs are not recommended for diagnostic purposes for
craniofacial FD lesions, although dental films, Panorex, or a
conebeam computed tomography (CT) are appropriate to
evaluate lesions around the dentition. A skeletal survey may
be indicated if there is a suspicion of polyostotic FD, particu-
larly in patients skeletally immature. Additional FD lesions
beyond the craniofacial region require further evaluation by
an orthopedic surgeon.27
Computed tomogram (CT)
Usually from the top of the head to the thyroid region, a CT
scan without contrast and with slice thickness 3.75 mm or less
is adequate to diagnose tumors of the facial skeleton.27 Similar
requirements should be used for monitoring lesions radio-
graphically or evaluating for recurrence of disease. CT pro-
vides more detailed information about the tumor size, location,
any local invasion or compression, the mineralization within
the lesion, and may demonstrate periosteal reaction earlier
than plain radiography.32
According to the bone–fibrous matrix ratio within the
lesion on CT scan, three distinct patterns have been described
in FD patients.33 The Pagetoid pattern lesions shown a mixture
of radiodense and radiolucent areas of fibrosis. Sclerotic
pattern is the most common of craniofacial FD lesions showing
a homogeneous, dense, or “ground-glass” appearance with a
thin cortex devoid of distinct borders. Radiolucent or cystic
pattern lesions contain one or more ovoid lucencies that are
surrounded by a dense periphery. The radiolucency is because
of cystic degeneration, simple bone cyst, and aneurismal bone
cyst.
Even though CT findings are characteristic, variability of
FD lesions may be due to the stage of development within
the lesion. Polyostotic FD lesions on CT and the natural
radiographic progression may vary. As these patients enter
the second decade of life, the FD lesions progress to a
mixed appearance, often stabilizing in adulthood but without
homogeneity on appearance (Fig. 15.4). This may explain the
Malignant potential
Malignancies can occur in any form of FD, but they are
rare, ranging from 0.5% to 4%. Malignant transformation is
higher in polyostotic forms and previously irradiated sites,
commonly occurring in the third or fourth decade of life.
The most common malignancies in descending order are
osteosarcoma, fibrosarcoma, chondrosarcoma, and malignant
fibrous histiocytoma.28
Diagnosis/patient presentation
Medical history and examination
The anamnesis and clinical evaluation guides the diagnosis of
FD, but radiographic studies are necessary to determine the
extent of disease and form (monostotic or polyostotic). Some-
times biopsy findings are required for a definitive diagnosis.
Because radiographs cannot distinguish FD from various
tumors of the facial skeleton, the information about tumor
location and patient’s age are sufficient to raise a suspicion of
the disease. The medical history should also include the onset
and types of symptoms as well as the presence of functional
impairments and duration. Although signs and symptoms
depend on the site, extent, and nature of the disease, FD
of the facial skeleton commonly presents as a gradual,
painless, immobile mass enlargement resulting in facial
asymmetry.27
In general, the signs and symptoms correlate well with the
primary bone involved and the extent of the involvement. As
a consequence of the enlarging lesion, adjacent structures may
be invaded or compressed, resulting in various symptoms
such as orbital dystopia, proptosis, diplopia, strabismus,
blindness, epiphora, anosmia, facial paralysis, tinnitus,
hearing impairment, sinusitis, pain, paresthesia, tooth loss,
and malocclusion. When the frontal bone is involved, the
globe is usually displaced inferiorly with or without proptosis.
The most feared ocular manifestation is the compressive optic
neuropathy secondary to sphenoid bone disease, which may
present as an acute or chronic loss of visual acuity and fields
(Fig. 15.3).29
Specific documentation in FD cases include onset of men-
arche in females to rule out precocious puberty, endocrine
disorders (hyperthyroidism, pituitary abnormalities, and
renal phosphate wasting), growth abnormalities, skin lesions
(café-au-lait spots), and history of fractures to rule out the
presence of other FD lesions in the extremities. Thereby, a
referral to an endocrinologist is strongly recommended to rule
out McCune–Albright syndrome.27 Moreover, if the symptoms
include rapid expansion, new onset of pain, visual change or
loss, hearing change or loss, evidence of airway obstruction,
new onset of paresthesia, or numbness, a referral to a surgical
specialist should be made imminently. In some FD patients,
rapid growth is associated with other pathological lesions
such as aneurysmal bone cysts or mucoceles, or rarely with
malignant transformation.30 The most common symptoms of
sarcomatous change are swelling and pain, usually develop-
ing rapidly. A coexisting aneurysmal bone cyst, or cystic
degeneration of FD, may clinically mimic a sarcomatous
change. In addition, malignancies arising from pre-existing
FD lesion are typically aggressive, thus, urgent evaluation is

386
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
A1
A2
A3
A4
B1
B2
Fig. 15.3  Fibrous dysplasia associated with optic nerve compression. (A1–A4) A 17-year-old man with fibrous dysplasia of the sphenoid and left fronto-orbital bones. At
the time of initial consultation he only has mild proptosis. (B1–B4) Five-year follow-up without surgical treatment.
non-pathognomonic radiographic findings such as absence
of “ground-glass”, “pagetoid”, “lytic”, and “cystic”; making
it difficult to differentiate FD from other conditions such as
ossifying osteoma and Paget’s disease. In addition, this period
of change in CT appearance coincides with case reports of
increased activity of the FD lesions either through rapid
growth, worsening facial asymmetry, malignant transforma-
tion, or association with other pathologic, radiolucent lesions
such as an aneurismal bone cyst and accelerated expan-
sion.34,35 Thus, an updated CT is crucial in the context of the

387
Diagnosis/patient presentation
B3
B4
C2
C1
C3
C4
(C1–C4) Eight-year follow-up without surgical treatment. In the interim, the patient developed insidious loss of vision in the left eye.
Fig. 15.3, cont’d
new onset of symptoms or rapid enlargement of the lesion at
any age.
Magnetic resonance imaging (MRI)
Although MRI does not use any ionizing radiation and has
been suggested by some authors as a diagnostic modality for
FD, it is a complementary exam to CT.36 MRI features of FD
do not share the characteristics seen on plain radiography and
CT. Moreover, MRI images of FD often resemble other tumors.
This heterogeneity results from a combination of calcification,
cysts, fatty areas, and septations, which shows evidence of
intermediate signal intensities on T1-weighted images, high
signal intensities on T2 images, and enhancement after injec-
tion of contrast material.37 The likelihood of correctly diag-
nosed FD by MRI is high only when the signal intensities on

388
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
A
B
Fig. 15.4  Bone involvement in McCune–Abright syndrome (MAS). (A,B,C) An 18-year-old patient diagnosed with McCune–Albright syndrome. CT scans shown the
severity and extension of the disease. These FD lesions showed a mixed appearance on CT such as “ground-glass”, “pagetoid”, “lytic”, and “cystic”. (D) Plain films also
showed the bone lesions outside the craniofacial skeleton.
C
D
both T1- and T2-weighted images are low despite the injection
of contrast material.38
Nuclear medicine
Radionuclide scan is highly sensitivity but has low speci-
ficity in diagnosing fibrous dysplasia. It may be used for
assessing of the extent of skeletal involvement in polyostotic
disease. In these cases, a full-body 99Tc-methylene diphos-
phonate bone scan is recommended to estimate the biologic
activity of the index lesion and to detect any additional lesions
that may exist throughout the skeleton. The appearance of
FD on a bone scintigraphy scan is the result of increased
tracer uptake in the diseased bone, especially if the lesion
is metabolically active, especially in the adolescent period.
The detectable bony activity decreases as the lesion
maturates.39
Another imaging modality, single-photon emission com-
puted tomography (SPECT) has been also reported to be
sensitive in the detection of lesions that are not seen on CT.40

389
Diagnosis/patient presentation
grows centrifugally and is clearly demarcated from the sur-
rounding normal bone. On CT, it presents as a well-delineated
sclerosing mass with osseous trabeculae. Histopathology
reveals irregular bony spicules surrounded by extensive
osteoblastic activity. This feature differs from FD, in which
bone deposits lack of osteoblastic activity.29 The aim of treat-
ment is early surgical removal.
Giant cell granuloma (GCG)
As FD, giant cell reparative granuloma, also called GCG, is a
reactive, benign process within the first two decades of life
that is more common in females. Radiographically, the central
part of the lesion forms a well-demarcated, radiolucent,
multiloculated lesion. These lesions have a hemorrhagic
appearance on gross inspection and microscopically consist of
groups of spindle-shaped fibroblasts that are mixed with col-
lagen and numerous, multinucleated osteoclast-like giant
cells. This means that the lesion is identical to brown tumor
and only the absence of biochemical findings of hyperpara-
thyroidism would differentiate these two pathologies.29 The
best management is aggressive surgical curettage.
Another craniofacial entity is cherubism or multilocular
cystic disease of the jaws; although sometimes classified as
a variant of fibrous dysplasia, cherubism is likely to be a
form of giant cell reparative granuloma. Cherubism is a very
rare autosomal-dominant benign fibro-osseous disorder that
is characterized by symmetrical bone involvement limited to
the mandible and maxilla, which typically first appear at the
age of 2 to 7 years. Bone lesions are filled with soft, fibrous,
giant cell-rich tissue that can expand and infiltrate the orbital
floor and cause the characteristic upward tilting of the eyes
causing scleral show, and hence the “cherubic” phenotype
reflects jaw hypertrophy. The radiographic hallmark of
cherubism is the development of symmetrical, multilocular,
radiolucent, expansile lesions in the mandible and/or the
maxilla. Bilateral mandibular involvement and preservation
of the mandibular condyles is considered pathognomonic
of cherubism.42 In most cases, the dysplastic expansile
masses begin to regress with the onset of puberty. Because
cherubism is usually self-limiting, operative treatment may not
be necessary.
Langerhans cell histiocytosis (LCH)
LCH or eosinophilic granuloma of bone is another rare disease
with a variable clinical involvement that presents usually
during the first decade with a female predominance. Despite
unknown etiology, the pathogenesis involves an abnormal
proliferation of antigen-presenting histiocytes (Langerhans
cells) causing medullary bone resorption. Although the lesions
may arise in any hematopoietically active bone, in the cranio-
facial skeleton they are most common in the skull and jaw.
Patients may present with painful or painless bone lesions,
and solitary lesions are more frequent than multiple bone
involvement. Radiographic findings on plain films and CT
vary widely. The early stage shows a poorly defined, lamel-
lated periosteal reaction, and the later stage is characterized
by ovoid, well-defined, and homogeneously lytic, with vari-
able marginal sclerosis. Treatment depends on the clinical
presentation and may include chemotherapy, radiotherapy,
and/or surgery.32
The scenario and select timing for use of SPECT remains
unclear.
Optical coherence tomography (OCT)
Many tumors of the facial skeleton including craniofacial FD
may affect the orbit, causing partial or total loss of vision. OCT
is a useful test to help diagnose optic neuropathy. It uses high
resolution cross-sections of the optic nerve to determine the
thickness of the retinal nerve fiber layer (RNFL). This modal-
ity may be useful for examining patients that cannot undergo
a visual field exam, such as children, or may predict visual
recovery after optic nerve decompression surgery. In the case
where the RNFL may be thin prior to surgery, it is unlikely
that surgery will improve vision while a patient with a normal
RNFL may have some improvement after surgical treatment.27
Biopsy
Patient history and physical examination, in combination
with the classic radiographic presentation, are often adequate
to establish the diagnosis of various craniofacial tumors,
including FD. However, in some cases, an isolated incisional
bone biopsy by the appropriate surgical specialist is indicated
for histologic confirmation whenever the site is accessible. As
the histopathologic differential diagnosis may be difficult, the
size of the biopsy should be as large as possible. Biopsy of FD
does not induce growth of the lesion.27,29 Tissue samples can
be obtained at the time of surgical resection of the lesion itself.
The caveat of the latter is that decision for a more radical
resection cannot hinge on the result of the tissue biopsy. To
avoid this scenario, it is recommended to do the biopsy as a
separate procedure and wait for the result before the resec-
tion. When the histologic sample is to be obtained during the
operation, and if there are any doubts intraoperatively regard-
ing the nature of the lesion, the resection part of the operation
is deferred until final histological results are available. The
surgeon must biopsy with caution, as FD lesions may be quite
vascular and bleed profusely. Meticulous hemostasis is
imperative even with tissue biopsy. Cytological studies such
as fine-needle aspiration are limited and not recommended.
Differential diagnosis
In general, the differential diagnosis of monostotic FD
includes solitary unicameral cyst, aneurismal bone cyst, giant
cell bone tumor, Langerhans cell histiocytosis, and plasma cell
myeloma; whereas the polyostotic form includes hyperpara-
thyroidism, Paget’s disease, unilateral enchondromatosis,
neurofibromatosis, and cherubism.41 Nevertheless, the basic
purpose of this chapter is to differentiate craniofacial FD from
other tumors of the facial skeleton. Thus, we will focus on the
most common related pathologies: ossifying fibroma, giant
cell granuloma, Langerhans cell histiocytosis, and aneurysmal
bone cyst.
Ossifying fibroma (OF)
OF, also known as juvenile ossifying fibroma or psammoma-
toid ossifying fibroma, like FD, is a hamartomatous fibro-
osseous bone lesion that affects adolescents and young adults.
Though less frequent than FD, it is more common in females
with maxillary and mandibular predominance. Unlike FD, it

390
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CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
Aneurysmal bone cyst (ABC)
ABC is neither a cyst nor an aneurysm, but an expansile
osteolytic lesion, usually multilocular, with blood-filled spaces
separated by fibrous septa.43,44 It occurs more commonly in the
second decade of life within a pre-existing or de novo bone
lesion. Although these lesions are most often seen in long
bones, they may occur at any site in any bone. Microscopy
shows blood-filled cystic spaces lined not by endothelial cells
but with plump, fibroblast-like cells.29,32 The etiology still
remains unclear. Radiographs and CT demonstrate an expans-
ile, multilocular “soap bubble” (honeycomb) radiolucency,
resulting in cortical thinning. Rapidly expanding lesions may
have poorly defined margins and an aggressive periosteal
reaction. MRI commonly shows cystic spaces with internal
septa and septal contrast enhancement. On skeletal scintigra-
phy a “donut sign” is commonly seen due to the intense
uptake at the periphery and little activity in the center of the
lesion.45 Treatment of ABC is surgical excision.
Patient selection
Surgical indications and contraindications
Since many small solitary bone lesions remain asymptomatic
and static, craniofacial FD, like many other tumors of the
facial skeleton, is not in itself an indication for medical or
surgical intervention. Nonetheless, the four main surgical
indications in any tumor include aesthetic concern, functional
impairment, symptom relief, and situations in which malig-
nancy cannot be ruled out. From these, the most common
surgical indication in craniofacial FD is a progressively
enlarging bony lesion that causes visible and disfiguring
deformity. Common functional impairments include maloc-
clusion. Surgical intervention is also warranted with persistent
pain. As a general principle for non-malignant lesions, any
surgical procedure performed must not result in aesthetic and
functional impairment worse than the original deformity. The
contraindications for surgical intervention of any tumor of the
facial skeleton include the patient not medically fit for general
anesthesia, the patient not keen for operation intervention,
and if the surgical procedure is unlikely to meet the patient’s
expectations.
Preoperative considerations
Tumors of the facial skeleton are optimally treated through a
multidisciplinary approach. Depending on the location of the
lesion, referrals are made to appropriate specialists for discus-
sion and collaboration. In general, specialists involved in a
craniofacial team include craniofacial surgeons, neurosur-
geons, oral and maxillofacial surgeons, otolaryngologists,
neuroophthalmologists, audiologists and dentists.27 After
consultation and discussion with the patient, a treatment plan
is organized. While the overall treatment is multidisciplinary
in nature, usually the craniofacial surgeon must coordinate all
the physicians involved and act as the principal caregiver to
the patient, making sure that all goals and expectations are
understood by all parties involved. To enhance the long-term
partnership between the surgeon and the patient, it is impor-
tant to remain somewhat flexible and acknowledge the
patient’s role and control in the management process.
Understanding and managing patient’s expectations is likely
to improve patient satisfaction.
Timing of operation
A patient may present to the craniofacial surgeon at any point
in time during the course of the disease, frequently during
early childhood. Because FD lesion growth stops after child-
hood, surgery is delayed until after adolescence. This is to
minimize recurrence and to intervene only after facial growth
is complete. In cases of cystic degeneration, in which signifi-
cant lesion expansion is associated with functional and aes-
thetic problems, surgery, even in childhood, is appropriate. In
addition, lesions suspicious for malignant change should be
investigated and treated promptly.
Variability in the growth rate of lesions combined with
variation in patient age affects timing of surgery quite differ-
ently. The decision for surgical intervention must be balanced
based on patient maturity, ability to cope with the disease
process, and ability to balance daily responsibility (which
affects school and job schedule). The degree of patient concern
about the condition provides the surgeon with some leeway
in the timing of the operation. This largely depends on when
the patient and family feel is the right time, with the surgeon
offering guidance based on their knowledge and experience
of the clinical behavior of the lesions. Moreover, the degree of
deformity resulting from FD is variable, as is a patient’s per-
ception of this. At some time points during the disease process,
the surgeon may feel that the deformity, whether it is initial
or residual, is of a minor degree such that they are not capable
of achieving a level of improvement sufficient to meet the
patient’s expectations. In such cases, it is better to defer opera-
tion. Through serial examinations, photographic comparisons,
and revisiting both surgeon’s and patient’s expectations, both
will decide together when the optimal timing for operation or
reoperation is.
Treatment/surgical technique
Although medical therapy can be used in FD associated pain
and minimizing bone resorption, the mainstay of treatment is
surgery. Future therapies may encompass stem cells with
regenerative capacity to differentiate into bone, bringing a
novel treatment strategy to genetic diseases of the skeleton. A
hypothetical approach using lentivirally expressed hairpin
RNA (shRNA) to silence target gene expression via RNA
interference (RNAi) serves to alter an FD-causing mutation.46
However, this has not been implemented clinically.
Nonsurgical treatment
Medical treatment has not proven to be effective in preventing
the progress of the disease but plays an adjunctive role. It is
focused on optimizing function and minimizing morbidity
related to skeletal as well as the extraskeletal involvement.
Medical therapy may vary according to the extraskeletal
disease location including aromatase inhibitors, estrogen
receptor modulators, growth hormone receptor antagonist,
and bisphosphonates. Because the most severe deformities
and symptoms occur in patients who have poorly controlled
growth hormone excess, it is recommended for management
of aggressively expanding lesions.47–49 On the other hand, for

391
Treatment/surgical technique
Fig. 15.6  A treatment algorithm in craniofacial fibrous dysplasia (CFD). Although
this algorithm is made for CFD, it also can be extended to other benign tumors of
the facial skeleton.
Observation or trimming & bone
recontouring
Trimming & bone
recontouring
Radical bone
excision &
immediate
reconstruction
ZONE 3
ZONE 4
CFD
ZONE 1
ZONE 2
skeletal involvement, bisphosphonates, human monoclonal
antibodies, vitamin D and calcium supplement may be used.
Radiotherapy is not advisable in the management of FD
because of the possibility to induce sarcomatous change.28,50
Pain is a common skeletal symptom in FD yet is more
frequent in the lower extremities than the craniofacial
region.51–53 Non-steroidal anti-inflammatory drugs (NSAIDS),
bisphosphonates, and opiates are the most common analgesics
used in FD.52,53 Despite the possibility of bisphosphonate-
related osteonecrosis of the jaws (BRONJ), bisphosphonates
are still being used to reduce pain and bone turnover in FD.52
A bisphosphonate, pamidronate (60 mg/day administered
intravenously for 3 successive days given every 6 months for
18 months), has been shown to inhibit osteoclastic bone
resorption by inducing apoptosis in actively resorbing osteo-
clasts. This mechanism increases bone density and leads to
improvement in radiologic appearance and reduces bone pain
associated with FD.54 A newer drug on the market, deno-
sumab, is a human monoclonal antibody, which binds to
receptor activator of nuclear factor κ-B ligand (RANK-L)
inhibiting osteoclast recruitment from precursor cells. Like-
wise, it may represent a potential treatment for FD bone
pain.55 Some authors recommend the use of vitamin D and
calcium supplementation, as serum calcium is noted to be low
in a subset of patients.52
Surgical treatment
The surgical approach is determined by tumor location,
though many approaches to a specific skeletal region are pos-
sible. The objective of the surgery is to restore a more aestheti-
cally pleasing and symmetrical facial appearance. Although
there are some basic principles specific to craniofacial recon-
struction, an artistic talent and imagination helps the surgeon
reconstruct challenging deformities (Video 15.1
).
Being mindful of facial cosmesis, selection of incisions is
critical in order to hide scars. The two main approaches to
Fig. 15.5  Four zones of craniofacial fibrous dysplasia. (Redrawn from Chen YR,
Morris DE. Craniofacial tumours (fibrous dysplasia). In: Guyuron B, Eriksson E,
Persing JA (eds). Plastic Surgery: Indications and Practice. Philadelphia: Saunders
Elsevier; 2009:437–454.)
1
2
3
4
reach the facial skeleton are through the coronal and intraoral
incisions. Craniofacial defects resulting from resection of
many tumors of the facial skeleton are reconstructed immedi-
ately using autogenous bone grafts. Traditionally, calvarium,
rib, and iliac crest are the common donor sites. Calvarial bone
graft is very useful, especially when the scalp has been elevated
for exposure during the procedure. In addition, the resorption
rate of calvarial bone graft is also noted to be low. Moreover,
segments of the resected specimen have also been used as
graft material to reconstruct defects.
According to Yu-Ray Chen and Samuel Noordhoff,56 for the
appropriate surgical management of FD the craniofacial
skeleton can be divided into four zones (Figs. 15.5 & 15.6):
■ Zone 1 comprises the fronto-orbital, zygomatic, and upper
maxillary regions. This is the most aesthetically apparent
area in the whole craniofacial region; consequently,
complete resection of the lesion is advisable here to
minimize the possibility of recurrence. Reconstruction is
performed immediately, usually with bone grafts.
■ Zone 2 represents the hair-bearing cranium. Since
cosmesis is of less consequence in this area, zone 2
lesions are generally treated with a more conservative
surgical approach, for example, by shaving, which tends
to restore a more normal bony contour.
■ Zone 3 encompasses the central cranial base, petrous
mastoid, and pterygoid bones. Given the important
vascular structures and various cranial nerves, surgical
intervention in this area is avoided when possible.
Lesions here are observed unless they develop symptoms
such as visual disturbance secondary to optic nerve
compression. In such a scenario, optic canal
decompression is recommended.
■ Zone 4 refers to the teeth-bearing bones, which are the
maxillary alveolus and the mandible. For lesions in this
zone, a more conservative approach is also preferred, at
least at the initial stage. This is because resection of
teeth-bearing bones will result in the patient having to
require dentures, which are less functional than the
original dentition.
Zone 1: Surgical technique
The eyes are protected by temporary tarsorrhaphy sutures.
The head is positioned on a Mayfield head rest. The whole
head is prepped to allow movement of the head during the

392
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
bony plate to expose the frontal lobe. With gentle retraction
of the frontal lobe and the placement of a malleable plate to
protect the orbital contents, osteotomies are made through the
supraorbital rim and orbital roof to remove the segment of
bone containing the lesion. The osteotomies are made using a
combination of burrs and saws. The orbitomaxillary osteotomy
is used to resect lesions affecting the lateral orbital wall,
maxilla, zygomatic body, and zygomatic arch. A frontotempo-
ral craniotomy combined with osteotomies through the
superolateral orbit and zygomatic arch are made to facilitate
the removal of corresponding lesions (Fig. 15.7B).
For lesions involving the inferior aspect of zone 1, the
coronal flap alone may be insufficient for adequate exposure.
In this case, a combination of coronal flap and Weber–Ferguson
incision or degloving face incision along the buccal sulcus is
employed. Osteotomies are performed through the glabella,
orbit, nasal bones, and maxilla above the level of the dental
roots (Fig. 15.7C). In this situation it is particularly important
to seal the extradural space from the nasopharynx, usually by
using the galeal-frontalis flaps.
After the bone segment containing the lesion is removed,
the defect is assessed for reconstruction. For defects involving
the orbit, the goals of reconstruction are to restore symmetric
skeletal form, symmetric orbital position and volume, normal
anatomic compartmentalization, and to protect vital struc-
tures (i.e., globe and brain). Split calvarial grafts can be
assembled into a three-dimensional construct. Alternatively, a
operation for bone graft harvest and assessment for symmetry.
A bicoronal or bi-temporal approach is used to gain adequate
exposure to different areas of the upper and midface, includ-
ing the zygomatic arch. The planned incision is roughly 8 cm
posterior to the mid-anterior hairline; from the midline, the
incision is extended laterally and inferiorly to the root of the
helix.
Dissection and elevation of the flap are easily performed in
the subgaleal plane, however, at about 1.5 cm above the
supraorbital rim, the dissection changes from subgaleal to
subperiosteal plane. Dissection and soft-tissue elevation
continue over the orbital roof, nasal dorsum, zygomatic arch,
and anterior maxilla to obtain adequate exposure. Care should
be taken to identify and preserve the lacrimal apparatus.
The planned osteotomy lines are marked. The osteotomies
are designed to excise all abnormal-appearing bony lesions
completely. For superficial lesions, these osteotomies are used
to excise the actual bony lesions. For lesions situated deeper
in the skull base, these same osteotomies may be used in the
osteotomy and replacement approach. In this scenario, the
osteotomized bone segment is removed to expose the deeper
underlying lesion (for example, in the midline cranial base or
nasal septum). After the lesion has been resected and removed,
the osteotomized segment is placed back into position.
The supraorbital osteotomy is used in the resection of
lesions of the superior half of the orbit (Figs. 15.7A). The
neurosurgeon performs a frontal craniotomy and removes the
Fig. 15.7  Osteotomies used in resection of craniofacial fibrous dysplasia. (A) Supraorbital osteotomy. (B) Orbitomaxillary osteotomy. (C) Osteotomies through the glabella,
orbit, nasal bones, and maxilla above the level of the dental roots. (Redrawn from Chen YR, Morris DE. Craniofacial tumors (fibrous dysplasia). In: Guyuron B, Eriksson E,
Persing JA (eds). Plastic Surgery: Indications and Practice. Philadelphia: Saunders Elsevier; 2009:437–454.)
A
B
C

393
Treatment/surgical technique
segment of titanium mesh can be plated to the construct to
reconstruct one wall of the orbit. Bone tumors may reduce
orbital volume and push the orbital floor inferiorly, thus the
new orbital roof should be positioned at a height symmetrical
with the opposite roof (Fig. 15.8).58 Canthoplasty is performed
using clear nylon sutures. The new canthal position is deter-
mined by comparison to the disease-free side.
Edges of the bone graft are burred to ensure a flush fit with
no areas of prominence. Dural tenting sutures are placed to
minimize dead space between the dura and the graft (Fig.
15.9). To avoid palpable instrumentation or even a visible
prominence, we prefer to use low-profile plates and screws in
an area anterior to the hairline. In some cases, the hardware
is inset into a small groove burred on the graft and the adjacent
native bone so that the plate and screws do not rise above the
level of the graft and adjacent bone (Fig. 15.10). In placing
bone grafts, a step is burred along the native bone margin,
A
Fig. 15.8  Fronto-orbito-maxillary reconstruction after tumor resection.
(A) Anatomic configuration of the defect. (B) Configuration of the bone graft
segments. Note the placement of bone grafts on the orbital floor to achieve a
symmetrical height with the opposite roof.
B
Fig. 15.9  Dural tenting sutures. Sutures are placed through the dura and tied; they
are then passed through the holes drilled through the bone graft and retied.
Fig. 15.10  Burying the plates. Plates are inset into the bone to create a smooth
contour. A groove is burred into the graft and the adjacent native bone just larger
than the plate to allow inset.
into which the bone graft is inset (Fig. 15.11). This is to provide
additional support and increase the contact area between the
graft and the adjacent native bone.
Hemostasis is secured and the wound is irrigated with
saline, taking care to remove all bony debris. Any dural tears
should be repaired with sutures. Before the scalp is closed,
fibrin glue is sparingly sprayed into the wound, and pressure
is applied for a few minutes over the redraped forehead to
promote adhesion and minimize potential dead space.57 The
galeal layer is closed with interrupted 3-0 Vicryl® sutures,

394
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CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
decompression is advocated in patients with progressive
deterioration of vision. For cases with sudden visual loss, it
is recommended that decompression should be performed
within 1 week of the onset of the symptom to maximize the
chance of visual improvement.59 When indicated, optic nerve
decompression is done with neurosurgical collaboration
and may be done through a frontal craniotomy approach
to the orbital roof. The neurosurgeon proceeds with de-
compression of the optic canal under loupe or microscopic
magnification. Microburrs are used to free the optic nerve
completely from any bony impingement along its entire course
within the optic cone. Alternatively, a frontotemporal crani-
otomy or endoscopic nasal/transsphenoidal approach can
be performed.
Zone 4: Surgical technique
For lesions in zone 4, the goals of surgery are to re-establish
a normal symmetrical three-dimensional skeletal form and to
preserve a stable dental arch support. Radical resection might
cause greater deformity or functional deficit than the lesion
itself; therefore, a more conservative approach such as shaving
is preferred whenever possible. In some cases, shaving may
not be adequate. In this situation, the lesion is excised;
however, as mentioned, it is important to preserve the tooth-
bearing portion of the maxilla. Successful facial bone contour-
ing surgery in FD patients relies on first establishing the
patient’s goals and expectations and then identifying the
anatomic basis responsible for the patient’s complaints
(Fig. 15.12).
For maxillary lesions, the preferred approach is through
an upper gingivobuccal incision. The soft tissues are elevated
from the skeleton in the subperiosteal plane to expose the
lesion widely. The extent of the lesion, or the portion that
is causing deformity, is marked. Care should be maintained
to avoid injuries to the infraorbital nerve and dental roots.
With the assistant protecting the overlying soft tissues, the
lesion is shaved with burrs. The wound is irrigated with
a copious amount of saline to remove bone particles and
closed with absorbable sutures. If the medial and/or lateral
buttresses of the maxilla are sacrificed, these structures need
to be reconstructed. For immediate reconstruction, rib bone
grafts are preferred. This avoids exposing and potentially
contaminating the dura while harvesting the calvarial graft
in the same surgery involving the oral cavity. The graft
segments are placed to reconstruct the medial and lateral
maxillary buttresses, vertically supporting the facial height.
A step is burred along the periphery of the defect to facilitate
inset of the grafts. This increases the area of contact between
the graft and the adjacent native bone and enhances stabil-
ity of the inset graft. Additional graft segments are placed
to reconstruct the remainder of bony defects. Reference is
made to the contralateral side and to disease-free landmarks
(e.g., infraorbital foramina, sides of the pyriform aperture)
to achieve symmetry. Bone grafts are fixed with plates
and screws.
For lesions of the mandible, exposure is through a lower
gingivobuccal incision. The lesions are marked and shaved.
An attempt is made to preserve a symmetrical mandibular
contour. With this approach, the mandibular arch remains in
continuity. The level of the dental roots and mental foramina
are marked to avoid injuries to these structures.
and the skin is closed with staples. Two suction drains are
placed (one anteriorly and one posteriorly) beneath the scalp
flap, each exiting through a separate stab wound in the post-
auricular hair-bearing scalp. The scalp flap is closed in layers,
and the tarsorrhaphy sutures are removed at the conclusion
of the operation.
Zone 2: Surgical technique
Zone 2 lesions are approached through a coronal incision as
described above. For lesions posterior to the hairline, bone
shaving is completed using burrs. The goal here is to restore
anatomic form, contour, and symmetry. In these cases, ade-
quate bilateral exposure allows visual comparison and palpa-
tion for symmetry.
For more anterior lesions bordering on zone 1, a more
aggressive approach is often taken, as this is a more visible
location. A better cosmetic result can be achieved through
excision, and the defect is immediately reconstructed with
autogenous bone graft. Reconstruction with bone graft is
much simpler in zone 2 compared to zone 1, as a single
segment of bone graft is often sufficient.
Zone 3: Surgical technique
This region contains major vessels and cranial nerves; thus,
tumor resection in this region is to be avoided whenever pos-
sible. The indication for surgery in this region is for those
lesions that cause functional deficits, for example, visual
impairment.
Optic nerve decompression
Prophylactic decompression is not advised to be performed
as a primary procedure owing to the risks of failure of vi-
sual improvement after decompression or even blindness.
In patients with optic canal encasement but without visual
symptoms, regular observation with clinical examination
and diagnostic imaging is more appropriate. Therapeutic
Fig. 15.11  Beveling of the bone edges. A bony step is burred along the margin of
the adjacent native bone to facilitate inset and support of the graft segment. Note
the inner cortex ledge medially (arrows) that allows inset of the construct, and
avoidance of a step-type defect.

395
Postoperative care
A1
A2
A3
A4
A5
B1
Fig. 15.12  McCune–Abright syndrome. An 18-year-old woman diagnosed with McCune–Albright syndrome who shown a FD maxillary lesion. (A1–A5) Preoperative
photographs. (B1–B5) One year after shaving of FD lesion and bone contouring of the maxilla.
Despite a seemingly ideal reconstruction, some patients
will require a subsequent orthognathic operation to correct a
vertical height asymmetry or malocclusion (Fig. 15.13). This
may be secondary to bone graft resorption or to progressive
FD. In these cases, the operation is planned carefully with the
orthodontist. The goals of such an operation are to correct
malocclusion and to level a canted occlusal plane. This usually
involves an asymmetric Le Fort 1 osteotomy in which the
right and left sides are impacted or distracted to different
degrees. Intraoperatively, we find that the facebow combined
with direct measurements is helpful in assessing symmetry.60
For direct measurement, we expose both eyes and measure
from the lower lid margin to the oral commissure, comparing
both sides.
Postoperative care
Generally, the patient is extubated in the operating room. If
the operation involves craniotomy and exposure of the dura
then the patient is admitted to the neurosurgical intensive
care unit for neurological monitoring, and a postoperative CT
Continued

396
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
Outcomes, prognosis, and
complications
Prognosis and follow-up
Prognosis for many tumors of the facial skeleton including
craniofacial FD depends on the location and severity of the
pathology. Despite the lack of reliable prognostic biochemical
marker for FD, some biomarkers such as serum alkaline
phosphatase and urinary hydroxyproline are used occasion-
ally to monitor disease progression and the response to the
nonsurgical treatment.34,61
scan is done in the same evening or the next morning. Visual
acuity is checked immediately postoperatively and serially
while the patient is in the hospital. To reduce edema, the head
of the bed is kept elevated to at least 30°. Cold packs are
placed over the upper face beginning immediately postopera-
tively and continuing every hour for the next 24 h. For all
patients intravenous antibiotics are continued for 2 days and
changed to oral antibiotics to complete a 1-week course. If
inserted, suction drains are left in place for 2–3 days, and scalp
staples are removed after approximately 10 days. Patients are
advised to avoid engaging in strenuous activity for 2 weeks.
When transoral approaches are used, postoperative instruc-
tions include eating a soft diet and to maintain good oral
hygiene.
B2
B3
B4
B5
Fig. 15.12, cont’d

397
Outcomes, prognosis, and complications
A1
A2
A3
A4
B1
B2
B3
Fig. 15.13  Long-term surgical outcomes in a patient with craniofacial fibrous
dysplasia. (A1–A4) A 6-year-old patient with fibrous dysplasia of the left fronto-
orbital and maxillary bones. (B1–B3) Preoperative photographs. Important to notice
the deciduous dentition on the Panorex, thus a more conservative technique is
preferred for the maxilla.
Continued

398
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
Fig. 15.13, cont’d
C1
C2
C3
C4
D1
D2
(C1–C4) One year after subradical tumor excision (zone 1) and immediate calvarial bone graft reconstruction, and bone recontouring of the maxilla
(zone 4). (D1–D4) Twenty-four-year follow-up after surgery.

399
Outcomes, prognosis, and complications
5. Globe injury is best avoided by gentle anteroinferior
traction of the scalp flap during exposure at and below
the supraorbital rim.
6. Care should be taken to level the canthi at the end of
the procedure. Visual comparison and measurements
should be made to place the medial and lateral canthi at
the same level compared to the contralateral side. In
performing the canthopexy, a non-absorbable suture is
used to fix the canthal ligament to the periosteum.
7. If extensive dissection has been done to facilitate
exposure of the bone, the patient may develop
postoperative descent or “sagging” of the soft tissues.
During reconstruction, sutures should be placed at sites
of periosteal incision, to reattach and thereby resuspend
the soft tissues.
8. Scalp alopecia is a complication to be avoided. Prepping
and draping the entire head allow periodically lifting
and slightly rotating the head during the procedure,
reducing prolonged pressure at any one location. The
skin closure should be tension-free, and any tension
should be taken up by galeal sutures. After placing key
sutures to mark the vertex, wound closure is performed
in a lateral to medial direction bilaterally. This allows
slight advancement of the coronal flap from its bilateral
inferior aspects towards the midline, thus reducing
tension in the vertex region. Should alopecia occur, this
is treated as a delayed secondary procedure, at least 6
months later. A small area of alopecia can be treated
through excision and a local flap; a larger area may
require tissue expansion.
9. Long-term neurosensory disturbances after coronal or
transoral approaches can occur and are a common source
of complaints from patients. Although the majority of
neurosensory changes resolve by 6 months, occasionally
they may persist for up to 12 months. This possibility must
be clearly communicated to the patient before surgery.
10. For resection involving the maxillary alveolus and
mandible, injuries to the dental roots and inferior
alveolar nerve/mental nerve can be avoided by proper
surgical planning and meticulous surgical technique.
Minor injury to the nerve causes temporary
D3
D4
Fig. 15.13, cont’d
As part of follow-up, patients are seen at the outpatient
clinic weekly for the first month after surgery and then at 3
months, 6 months, and 1 year postoperatively for potential
late complications and to monitor the long-term surgical
results as well as tumor recurrence. Patients with craniofacial
FD should have yearly ophthalmologic and audiologist evalu-
ations. In addition, the patient who undergoes optic nerve
decompression requires repeated formal ophthalmologic
assessment.
Prevention and management of
potential complications
1. The superior sagittal sinus can be injured if an
osteotomy is performed too close to the midline. If this
occurs, it is treated in collaboration with the
neurosurgeon, by using Gelfoam® packing, local
pressure, and sutures.
2. Communication between the nasopharynx and the
intracranial cavity can lead to epidural infection and
meningitis. During reconstruction, the nasopharynx and
intracranial cavity must be separated by well-
vascularized tissues. The galea-frontalis flap serves well
for this purpose.
3. Errors in orbital reconstruction may result in improper
globe positioning. This may lead to enophthalmos,
telecanthus, or dystopia. There is a tendency for the
globe to assume a more inferior position. To avoid this,
additional strips of bone graft may be placed along the
orbital floor for reinforcement.
4. Patients may complain of a palpable or even visible
prominence of the plating beneath the skin. If there is
no sign of infection, the patient is reassured and the
hardware removed after bony union has occurred. This
problem can be avoided by several means. When
possible, place instrumentation posterior to the hairline,
use low-profile plating, and use the minimal quantity
required to achieve stability. In addition, the groove is
burred into the graft and the adjacent native bone and
the plate is inset into the groove to create a smooth
contour.

400
SECTION II
CHAPTER 15 • Tumors of the facial skeleton: Fibrous dysplasia
with the patient preoperatively. For instance, some patients
with craniofacial FD lesions may present months to years
following the initial resection with an occlusal cant. This may
be due to recurrent disease or graft resorption, thus CT scan
is required for evaluation. Depending on the cause of the
recurrent deformity, the treatment could be resection, addi-
tional bone grafting, or an orthognathic surgery. Special
mention should be made of the patients with a recurrent FD
of the mandible who can be treated with hemimandibulec-
tomy and immediate reconstruction using a microvascular
bone flap. The surgeon must carefully decide whether reop-
eration is likely to improve the degree of deformity. It is
advisable to wait at least 6 months before any decision is
made. This gives adequate time for edema to resolve so that
a more accurate evaluation is made. In cases where reopera-
tion is not warranted, the patient is counseled accordingly.
Conclusion
Tumors of the facial skeleton can cause significant functional
and aesthetic deformities. Since these bone lesions are often
located adjacent to important structures, management is best
done through a multidisciplinary approach. The craniofacial
surgeon coordinates input from various members in the team
and decides with the patient and family the ideal timing for
operation. When surgical treatment is undertaken, the goal is
to resect the lesion and reconstruct an adequate skeletal
framework within the same setting. It is important to be sure
of the diagnosis prior to planning a treatment approach.
Despite multiple surgical options, the ideal surgical technique
is chosen based on the histopathology, extent of tumor growth,
patient’s anatomy, and surgeon’s experience. While this
chapter focuses on fibrous dysplasia, many of the aforemen-
tioned operative principles are also useful for treating other
tumors of the facial skeleton.
hypoesthesia in the lower lip which usually will recover
after few months. Severe nerve damage might lead to
permanent sensory loss.
11. For resection involving the intraoral route, early
complications include hematoma and intraoral wound
dehiscence. These are largely preventable through
mindful surgical technique. We prefer to close intraoral
wounds in two layers. For the maxillary wound, several
interrupted sutures are placed in the periosteal-
muscular layers prior to mucosal closure. For the
mandibular closure, the dissected periosteal edge is
resuspended before the mucosa is closed. For cases
involving an orthognathic procedure, and for cases
where there has been significant exposure of the
mandibular angle, bilateral closed suction drains are
placed. The tip is placed at the angle, and the drain exits
from the anterior aspect of the mucosal wound and is
sutured around a tooth or to the orthodontics. These
drains reduce edema and fluid accumulation within the
dissected cavity. They are usually removed on
postoperative day 1. Patients with a wound dehiscence
are taken back to the operating room for irrigation and
reclosure. Those with a palpable hematoma are also
taken back to the operating room for evacuation and
reclosure.
12. Infection of the intraoral wounds, if it occurs, usually
presents subacutely from weeks to a few months after
surgery. There may be a nidus such as a segment of free
bone. Small, well-localized pus collections, or those that
have spontaneously begun to drain intraorally, are
treated in the clinic. Under local anesthetic, a portion of
the wound overlying the fluctuance is opened. The
wound is packed with single gauze. A more extensive
collection warrants formal exploration, irrigation, and
drainage in the operating room. For the patient with a
recurring infection, formal exploration is recommended
to remove any necrotic bone segment.
Secondary procedures
Patients may present after surgical treatment with residual or
recurrent asymmetry; this possibility should be discussed
1. Kindblom LG. Bone tumors: epidemiology, classification, pathology.
In: Davies MR, Sundaram M, eds. Imaging of Bone Tumors
and Tumor-Like Lesions. Berlin Heidelberg: Springer Press;
2009.
2. Gabbay JS, Yuan JT, Andrews BT, et al. Fibrous dysplasia of the
zygomaticomaxillary region: outcomes of surgical intervention.
Plast Reconstr Surg. 2013;131:1329–1338.
16. Wu H, Yang L, Li S, et al. Clinical characteristics of
craniomaxillofacial fibrous dysplasia. J Craniomaxillofac Surg.
2014;42:1450–1455.
26. Riminucci M, Liu B, Corsi A, et al. The histopathology of fibrous
dysplasia of bone in patients with activating mutations of the Gsα
gene: site-specific patterns and recurrent histological hallmarks. J
Pathol. 1999;187:249–258.
27. Lee JS, FitzGibbon EJ, Chen YR, et al. Clinical guidelines for the
management of craniofacial fibrous dysplasia. Orphanet J Rare Dis.
2012;7(suppl 1–2).
31. Chen YR, Wong FH, Hsueh C, et al. Computed tomography
characteristics of non-syndromic craniofacial fibrous dysplasia.
Chang Gung Med J. 2002;25:1–8.
32. Wootton–Gorges SL. Tumors and Tumor-Like Conditions of Bone.
In: Stein-Wexler R, Wootton-Gorges SL, eds. Pediatric Orthopedic
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34. Chen YR, Chang CN, Tan YC. Craniofacial fibrous dysplasia: an
update. Chang Gung Med J. 2006;29:543–548.
56. Chen YR, Noordhoff MS. Treatment of craniomaxillofacial fibrous
dysplasia: how early and how extensive? Plast Reconstr Surg.
1990;86:835–842.
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400.e1
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SECTION II • Head and Neck Reconstruction
16 
Overview of head and neck tumors
Andrew Foreman and Patrick J. Gullane
SYNOPSIS
■ Cancer of the oral cavity and pharynx is thought to be caused by
accumulated genetic alteration initiated by toxins from tobacco and
promoted in the United States by alcohol intake.
■ Tumors are staged according to the TNM system that considers
anatomic subsite in the upper aerodigestive tract, size, and adjacent
extension for the primary tumor (T), number, and laterality of cervical
lymph nodes (N), and presence or absence of distant metastases (M).
■ Surgery or radiotherapy are both effective in early stage tumors, but
surgery followed by postoperative adjuvant radiotherapy is preferable
in Stage III or above tumors.
■ The primary site and the cervical metastases, or in some cases
micrometastases, are treated together as a composite resection or
resection of the primary and neck dissection.
■ Because of the anatomical complexity of the area, surgery and
radiotherapy often interfere with the critical functions of respiration and
alimentation. Resection and reconstruction should be designed to
completely remove the cancer and preserve or restore as much
function as possible.
■ Extensive surgery with or without previous radiotherapy may result in
difficult and dangerous complications such as osteoradionecrosis,
fistula, carotid exposure, and hemorrhage.
Introduction
Despite being rare, cancers of the upper aerodigestive tract
(UADT) and salivary glands can have devastating conse-
quences for patients because of the impact the disease and
its treatment have on the critical functions of respiration and
alimentation, in addition to the cosmetic implications of
major surgery in the head and neck region. The most signifi-
cant risk factors for head and neck cancers are tobacco
smoking and alcohol ingestion. Consequently, this is a
patient population with significant comorbid disease. Thus,
tailoring treatment plans to the individual patient is essential
to maximize oncologic control and minimize the associated
morbidity. Unfortunately, most patients with head and neck
cancers present with late stage disease necessitating multi-
modality treatment, which increases the risk of subsequent
complications, deformity, and dysfunction. This dysfunction
has, in part, been ameliorated by the widespread application
of primary reconstructive procedures, including free tissue
transfer. The reconstructive surgeon needs to have a clear
understanding of the underlying disease, the therapeutic
approach to these cancers, and the unique challenges of
reconstructing complex defects of the head and neck in order
to restore form and function to the UADT.
Epidemiology
Cancers of the head and neck are relatively rare. Within the
United States, they comprise 3.2% of new cancers (40 000
cases/year) but 22% of cancer deaths (12 460/year).1 The
majority (90%) of mucosal head and neck cancers are squa-
mous cell carcinomas (SCC). Adenocarcinomas arising from
minor salivary gland tissue are less common, as are sarcomas,
which may be of many types. In the United States, the
male:female incidence ratio is 3 : 1, and a racial predilection
for the black population has also been reported. Patients are
typically in the fifth or sixth decade, although the advent of
human papilloma virus (HPV)-associated cancers has seen a
reduction in age at presentation.2
The epidemiology of head and neck cancers is changing.
Public health measures have seen a decline in tobacco use in
the western world and a commensurate reduction in the
incidence of smoking-related head and neck cancer.3 However,
there has been a rapid increase in the incidence of HPV-
positive cancers, which predominantly affect the oropharynx.
In the 20 years up to 2004, the number of oropharyngeal SCCs
that were HPV-positive rose from 16.9% to 71.9% (Fig. 16.1),
amounting to a 225% increase in the population incidence
of this disease.4 In 2010, the incidence of HPV-positive
oropharyngeal cancers in men surpassed the incidence of
HPV-positive cervical cancers (Fig. 16.2). By 2020, it is expected

402
SECTION II
CHAPTER 16 • Overview of head and neck tumors
is the most frequently cited carcinogenic effect of cigarette
smoking, although multiple other mechanisms are known to
exist.9 While they directly contact the lining of the respiratory
tract, for example the larynx, these carcinogens will also enter
the blood stream from where they are secreted via saliva and
can then bathe the other UADT sites such as the oral cavity
and oropharynx. In the Western world, alcohol is the most
common promoter in UADT cancer and is strongly associated
with cancers of the hypopharynx, as this is the highest expo-
sure site of the head and neck. In Southern Asia, betel nut
chewing is common and it is also a potent cancer promoter.
Betel nut chewers have a younger age of cancer onset with a
reduced 5-year survival on top of the 28-fold increase in oral
cavity SCC incidence.10,11 Other pathogenic associations
include Plummer–Vinson syndrome, occupational exposures
(nickel, radium, mustard gas and wood dust), and viruses, in
particular Epstein–Barr virus (EBV) and human papilloma
virus (HPV).
Relatively recently, the role of HPV has come to the fore in
SCC of the oropharynx.12 The appreciation of HPV as a car-
cinogen has caused a significant paradigm shift in the man-
agement of oropharyngeal malignancies as the underlying
genetic alterations as well as the clinical presentation and
prognosis differ markedly from the traditional smoking and
that the overall incidence of HPV-positive oropharyngeal
cancer, that is both men and women, will have exceeded the
incidence of cervical cancer. With the incidence of cervical
cancer declining due to early cancer detection screening
programs and the HPV vaccine, HPV-related oropharyngeal
cancer is becoming a hugely significant public health problem.
It is hoped that the introduction of vaccination against
HPV infection will reverse this rapid increase, although the
uptake of population-wide vaccination has varied amongst
population groups and remains a significant public health
challenge.5
Pathophysiology
Head and neck cancer represents a prototypical model of
chemical carcinogenesis.6 Multiple genetic hits enable cells to
escape the normal cell cycle regulatory mechanisms.7 Alone,
smoking is the most common initiating carcinogen and
increases a patient’s cancer risk by eight times, whereas
alcohol increases risk four fold, and these show clear dose-
response associations.8 Tobacco smoking and alcohol con-
sumption are synergistic in their effect and using both results
in a 32-fold increase in the risk of developing a head and neck
cancer. The production of polycyclic aromatic hydrocarbons
Fig. 16.1  The rising incidence of HPV positive oropharyngeal cancers saw a cross
over between HPV-positive and HPV-negative tumors in the late 1990s such that
HPV-positive tumors now account for >70% of all oropharyngeal malignancies in
the United States. (From Chaturvedi AK, Engels EA, Pfeiffer RM, et al. Human
papillomavirus and rising oropharyngeal cancer incidence in the United States.
J Clin Oncol. 2011;29(32):4294–4301.)
Oropharynx (overall)
10
1
0
Calendar years
Rates per 100,000
HPV-positive oropharynx
HPV-negative oropharynx
1988-1990
1991-1992
1995-1996
1999-2000
2003-2004
Fig. 16.2  In 2010, the incidence of HPV-associated oropharynx cancer in men
exceeded that of cervical cancer in women. By 2020, the overall number of
HPV-related oropharyngeal cancers will exceed the number of HPV-related cervical
cancers. (From Chaturvedi AK, Engels EA, Pfeiffer RM, et al. Human papillomavirus
and rising oropharyngeal cancer incidence in the United States. J Clin Oncol.
2011;29(32):4294–4301.)
Calendar years
Rates per 100,000
Oropharynx (overall)
100
10
2010
1
0.1
Oropharynx (men)
Cervix
Oropharynx (women)
1980
1995
2010
2025

403
Pathophysiology
Work-up of the head and neck cancer patient
Unfortunately, more than 60% of patients with head and neck
cancer present with advanced stage disease. This is due to
both the non-specific nature of the early symptoms of these
cancers and the fact these cancers may access lymphatic chan-
nels early in their course. 25% of oral and oropharyngeal
cancers and 50% of nasopharyngeal cancers will present with
neck nodal disease, thus rendering them at least Stage III
disease. Dysphonia, dysphagia, odynophagia, sore throat,
hemoptysis, referred otalgia, and weight loss are worrisome
presenting symptoms for a UADT malignancy.
After a complete history focused on these symptoms, as
well as risk factors for head and neck cancer, the physician
should undertake a complete head and neck examination.
Inspection and palpation of the oral cavity, oropharynx, ear,
nasal cavity, and neck are mandatory. Endoscopic examination
of the entire UADT with in-office flexible nasopharyngoscopy
has become a routine part of the head and neck examination.
Mucosal abnormalities such as red or white patches, ulcer-
ations, or masses should be noted as suspicious. Other signs
such as loose dentition, the presence of trismus, unilateral
middle ear effusion, a nasal mass, or proptosis are all concern-
ing signs the surgeon should note during the examination.
Imaging plays a crucial role in the work-up of these
patients.14 Contrast-enhanced computed tomography (CT)
scanning of the head, neck, and chest has become the standard
for assessing the primary tumor along with any regional and
distant metastatic disease. Magnetic resonance imaging (MRI)
may be helpful, in addition, to provide increased soft tissue
detail. This is particularly useful in sites such as the orophar-
ynx, base of skull and parotid gland. Positron emission
tonography (PET-CT) imaging is also developing a place in
the work-up of patients with head and neck cancer (Fig. 16.4).
alcohol induced cancers. HPV is a DNA virus from the papo-
virus family that infects basal keratinocytes and has a predi-
lection for sites with single epithelial cell layers, such as the
tonsillar crypts. There are greater than 120 subtypes that have
been broadly categorized into high, intermediate, and low
risk groups. HPV subtype 16 accounts for more than 90% of
HPV-associated oropharyngeal SCCs. HPV exerts its onco-
genic potential through the E6 and E7 proteins it produces
during its life cycle. These proteins interact with p53 and the
retinoblastoma (Rb) gene, respectively. E6 promotes degrada-
tion of p53, rather than mutating the protein, such that wild-
type p53 is maintained albeit in greatly reduced quantities.13
This has important implications for treatment response in
HPV-associated malignancies as the normal cell cycle repair
mechanisms are preserved. E7 binds Rb, maintaining it in a
hyperphosphorylated state (Fig. 16.3). This releases the protein
E2F from its binding site on Rb, enabling E2F to drive unregu-
lated cell cycle progression at the G2-S transition point. As a
result of the interruption of the negative feedback loop at the
level of the cyclin dependent kinases (CDK), there is overex-
pression of the CDK inhibitor p16, an important surrogate
marker for HPV infection.
Fig. 16.3  Cell cycle implications of the interaction between the HPV E7 protein
and the retinoblastoma protein. In the normal cell cycle control mechanisms, the
interaction of Rb and E2F causes a transcription block and controls the cell cycle at
the S-G2 transition. The phosphorylation of Rb releases E2F to drive the cell cycle
forward. This is normally tightly regulated by cyclin dependent kinases. E7 prevents
interaction of Rb-E2F and maintains Rb in the hyperphosphorylated state. This
releases E2F to bind to DNA and drive uninhibited progression at the G2-S cell
cycle checkpoint. This in turn interrupts the negative feedback loop to the cyclin
dependent kinase inhibitors (including p16), thereby leading to uninhibited p16
production, which manifests as p16 overexpression on immunohistochemistry. CDK
= cyclin dependent kinase
Growth factors
Cell cycle
CDK inhibitors pl6
Rb-E2F
Splits Rb-E2F
E2F refused
G2
G1
S
M
Rb-PO4
+
+
-
-
-
CDK
Fig. 16.4  Positron emission tomography (PET) image of a metastatic cervical
node. Hypermetabolism within the cancerous node results in increased uptake of
the labeled-FDG enabling functional staging. Combining PET with CT improves the
anatomical localization of involved structures.

404
SECTION II
CHAPTER 16 • Overview of head and neck tumors
reserved for salvage treatment of persistent or recurrent
disease. The concurrent advances in head and neck recon-
structive techniques have certainly aided the success of
surgery in this setting, which is often associated with an
increased risk of complications.
The traditional approach to surgical management of head
and neck cancer has been to resect the primary tumor with an
adequate margin of normal tissue (>5 mm in the pathological
specimen) and treat the regional nodal basins of the neck.
While surgery remains the mainstay of treatment for many
head and neck cancers, it is well established that advanced
stage disease requires multi-modality therapy. Selecting
patients at high-risk of locoregional recurrence after surgery
forms the basis for recommending postoperative adjuvant
therapy. Historically, this was largely based on anecdotal
evidence and experience of the treating physicians until the
results of two randomized controlled trials were published in
2004.18,19 A comparative analysis of these two trials (RTOG
#9501 and EORTC #22931) has subsequently enabled physi-
cians to make informed choices about their patients by
defining risk levels for locally advanced head and neck cancer,
and this has been incorporated into the National Comprehen-
sive Cancer Network (NCCN) guidelines.20 Microscopically
involved surgical margins and the presence of extracapsular
extension are the most significant prognostic factors for poor
outcome – both locoregional recurrence and survival. Patients
with one or both of these risk factors have improved outcome
if they receive adjuvant chemotherapy in addition to radio-
therapy. A number of minor risk factors were also identified,
Assessment of the patient presenting with carcinoma of
unknown primary, those at high risk of having distant meta-
static disease, and evaluating treatment response in patients
undergoing non-surgical therapy are all evolving indications
for the use of PET-CT.15
Along with appropriate imaging that enables accurate
staging of the patient a tissue diagnosis is essential. Fine
needle aspirate (FNA) biopsy of enlarged neck nodes or
in-office biopsy of an obvious primary site is recommended
at the earliest available opportunity. Traditional work-up of
the head and neck cancer patient included panendoscopy
(nasopharyngoscopy,
laryngoscopy,
bronchoscopy,
and
esophagoscopy) under general anesthesia; however, this has
largely been superseded by advances in imaging techniques
and is reserved for patients in whom home in-office biopsy is
not possible or when tumor extent is unclear from the imaging,
and critical decisions with respect to management cannot be
made without direct visualization under anesthesia.
Finally, assessment of nutritional status, dental hygiene,
employment status, and social situation are all-important
considerations when preparing a patient for potentially multi-
modality, intensive treatment that is often required for these
patients.
Staging
Head and neck cancers are staged using the tumor, nodal
metastasis, metastasis systemic (TNM) models developed by
the American Joint Committee for Cancer (AJCC) and the
Union Internationale Contre le Cancer (UICC).16 Through its
iterations, it has increasingly been able to predict prognosis,
provide guidance in determining treatment plans, and enable
comparisons of treatment efficacy.17 The relevant T staging
systems are included throughout this chapter with the relevant
primary site. Nodal metastasis staging encompasses size,
number, and laterality of nodes for all head and neck sites
except for nasopharynx, whereas distant metastases are
recorded as present or absent (Table 16.1). The TNM classifica-
tions can then be combined to produce an overall stage des-
ignation (Table 16.1), which is often applied for determining
therapeutic plans and comparing therapeutic interventions in
the research setting.
Decision-making in head and neck
cancer treatment
Following appropriate work-up and staging, decisions
regarding head and neck cancer treatment should be made in
the context of a multidisciplinary tumor board setting. In
general terms, early stage disease (Stages I and II) requires
single modality treatment, whereas advanced stage disease
(Stages II and IV) necessitates multimodality therapy. The
traditional approach to head and neck tumors was to perform
surgery initially and supplement this with adjuvant radiation
therapy, with or without chemotherapy, depending on stage
and pathological analysis of the surgical specimens. However,
in the last 30 years, there has been increasing acceptance of
the significant morbidity and quality of life implications
of this ablative surgery such that in some settings the use of
non-surgical therapy as primary treatment has gained increas-
ing acceptance in order to preserve critical anatomical struc-
tures, in particular the larynx. In these cases, surgery is
Table 16.1  N staging, M staging, and overall UICC staging for
cancers of the head and neck mucosa
Stage
Description
N stage
Nx
Nodal status cannot be assessed
N0
No evidence of nodal metastasis
N1
Single ipsilateral node <3 cm
N2a
Single ipsilateral node 3–6 cm
N2b
Multiple ipsilateral nodes <6 cm
N2c
Bilateral or contralateral nodes <6 cm
N3
Any node >6 cm
M Stage
Mx
Metastatic status cannot be
assessed
M0
No distant metastasis
M1
Distant metastasis present
Overall stage
I
T1N0M0
II
T2N0M0
III
T3N0M0, T1-3N1M0
IVa
T4aN0-1M0, T1-4aN2M0
IVb
T4bN1-2M0, T1-4bN3M0
IVc
Any M1

405
Oral cavity cancer
more frequently on the lower lip. Pipe smoking may also
predispose to lower lip cancers. The surgical management of
lip cancer is of interest to the reconstructive surgeon and
poses unique challenges (Fig. 16.6). By dividing the lip into
thirds and determining how many thirds are removed in the
resection, the head and neck surgeon can tailor the defect
reconstruction using a number of well-established local
techniques (Table 16.3). Only when greater than two-thirds of
the lip has been resected does free tissue transfer need to be
considered.
The oral tongue is the most common intraoral site for cancer
to occur, and most often they present along the lateral border
of the middle third of the tongue. Transoral excision is possible
for most of these lesions with excellent visualization usually
possible with this approach. The greatest controversy in man-
aging these lesions is whether or not to treat the clinically node
negative neck electively. It is now recognized that for early
stage (T1 and T2) oral tongue SCC, depth of invasion is the
most important predictor of risk of nodal metastasis.23 Using
the 20% cut-off proposed by Weiss et al.,24 most studies have
determined that 4 mm is the cut-off for recommending elective
neck dissection in these patients. The buccal mucosa is a rare
site for cancer in the western world, accounting for <10% of all
oral cavity cancers. However, in South Asia, it is one of the
most common sites for cancer because the habit of chewing
betel nut exposes the mucosa of the buccogingival sulcus to
including greater than two positive nodes, perineural inva-
sion, vascular tumor embolisms, Stages III–IV disease or levels
IV–V nodes in oral cavity, and oropharyngeal primary tumors.
The presence of one of these factors is typically an indication
for postoperative radiotherapy. When a number of these are
present, chemoradiotherapy is again the recommended treat-
ment in patients deemed fit enough to tolerate this.21,22
Oral cavity cancer
The oral cavity extends from the vermillion border of the lip
to the junction of the hard and soft palate superiorly and the
circumvallate papillae inferiorly and is anatomically subdi-
vided into seven subsites: lip, buccal mucosa, dentoalveolar
ridge, retromolar trigone, hard palate, floor of mouth and oral
tongue (Fig. 16.5). Oral cavity cancer makes up 14% of all head
and neck cancers with 55% of these patients presenting with
early stage disease due to the ease of detection and surveil-
lance of the oral cavity (Table 16.2). Treatment of oral cavity
cancer can affect critical physiologic functions including
speech, mastication, and swallowing. For these reasons, as
well as the widespread application of free flap reconstruction,
primary surgery is preferable for almost all oral cavity cancer
patients barring those who are not medically fit or those who
refuse surgery.
While oral cavity cancer is often grouped as a single entity,
it is important to recognize that each subsite has unique
characteristics that impact the management of the cancers that
arise within them. The lip is the most common site of head
and neck cancer, excluding cutaneous malignancies. Lip
cancers are most often related to sun exposure, and they occur
Fig. 16.5  The anatomy of the oral cavity. The oral cavity extends from the
vermillion border to the hard palate and soft palate junction. Note the location of
the seven subsites of the oral cavity: lip, buccal mucosa, dentoalveolar ridge,
retromolar trigone, hard palate, floor of mouth, and oral tongue.
Soft palate
Hard palate
Buccal mucosa
Vermilion border
Lower gingiva
Retromolar trigone
Circumvallate papilla
Table 16.2  Oral cavity T staging as per AJCC, 7th Edition
T stage
Oral cavity
Lip
Tx
Primary tumor cannot be assessed
T0
No evidence of primary tumor
Tis
Carcinoma in situ
T1
Tumor 2 cm or less in greatest dimension
T2
Tumor larger than 2 cm but 4 cm or less in greatest
dimension
T3
Tumor larger than 4 cm in greatest dimension
T4a
Tumor invades adjacent
structures (e.g., through
cortical bone, extrinsic
tongue muscles, maxillary
sinus, skin of face)
Tumor invades through
cortical bone, inferior
alveolar nerve, floor of
mouth, skin of face
(i.e., chin or nose)
T4b
Tumor invades masticator space, pterygoid plates or
skull base or encases internal carotid artery
Table 16.3  Lip reconstruction is based on the extent of
resection by dividing the lip into thirds
Lip
< 13
13 – 2 3
> 2 3
Lower
Wedge
W-flap
Abbe–Estlander
(lateral)
Karapandzic
(middle)
Gilles fan flap
Bernard–Burrows
Webster advancement
Free flap
Upper
Wedge
Abbe +/−
Estlander
Burrow–Diffenbach
Abbe = lip switch, based on sup/inf labial artery. Divide pedicle 2–3 weeks later
Estlander modification if commissure involved

406
SECTION II
CHAPTER 16 • Overview of head and neck tumors
carcinogens for extended periods of time. The reconstruction
of such defects, typically with a fasciocutaneous free flap, is
aimed to reduce the debilitating effect of the severe trismus
that can result if large areas of the buccal mucosa are either
closed primarily or allowed to heal by secondary intention.
The floor of mouth can be a challenging area to both assess
clinically and to resect with clear margins. The proximity to
the mandible often necessitates at least a marginal mandibu-
lectomy to obtain adequate clearance of the tumor. Again,
reconstruction of this site needs to maintain the mobility of
this anatomic region to preserve near normal swallowing and,
in particular, speech. When performed in conjunction with
neck dissection, free flap reconstruction also recreates the
watertight seal that separates the oral cavity from the neck,
an important consideration in preventing the spread of infec-
tion to the neck. The dentoalveolar ridges, hard palate, and
the retromolar trigone account for only a small percentage of
oral cavity cancers (Fig. 16.7). Uniquely, however, the thin
mucosal layer that covers bone in these subsites predisposes
these tumors to early invasion of the mandible or maxilla.
Therefore, maxillectomy and/or mandible resection, be it
marginal or segmental, needs to be considered in many of
these tumors.
Fig. 16.6  Local flap reconstruction of a central lower lip defect using the Johansen step ladder. (A) Lower lip
melanoma occupying 13 to 23 of the lip. (B) Post-tumor excision. (C) The step-ladder around the mental
prominence is created by excision of skin wedges. (D) Advancement of the lip up the step ladder. (E) Bilateral
step ladders in preparation for defect closure. (F) End of procedure result. (G) Three months postoperative image
demonstrating well-healed incision and no microstomia.
A
B
C
D
E
F
G
Fig. 16.7  Upper alveolus cancer. Commonly associated with betel nut chewing in
Southern Asia. Although these tumors are rare, it is important to recognize tumors
in this location are prone to early bone invasion.

407
Oral cavity cancer
commonly achieved through a lip-split approach or may be
performed by raising a visor flap superiorly over the mandible.
This latter approach places the mental nerves at risk, which is
not desirable unless the mental foramina on both sides need
to be resected for oncologic purposes. Upper alveolus cancers
can often be accessed transorally as well. On occasion, the
upper lip may need to be split and connected to a lateral rhi-
notomy incision (i.e., the Weber–Ferguson incision) to expose
a greater portion of the anterior and lateral aspects of the
maxilla and enable wider resection of the maxilla (Fig. 16.9).
Finally, the transoral and facial approaches may be combined
in the midface degloving where an extended sublabial incision
is made and, as the skin flap is elevated superiorly, rhinoplasty
incisions are also made to deroof the nose and enable more
superior elevation of the flap (Fig. 16.10).
Management of the mandible
In managing oral cavity cancer, consideration of the adjacent
bony structures, mandible and maxilla, is important from
both oncologic and reconstructive perspectives. Tumors of the
dentoalveolar ridges, floor of mouth, and retromolar trigone
are the most likely primary sites to invade bone. Additionally,
metastatic neck disease in level 1 can become fixed to the
mandible, necessitating surgical management of the mandible.
The optimal imaging modality for assessing the mandible is
debated in the literature. CT is generally the standard inves-
tigation for staging patients with head and neck cancer.22 It is
also excellent at assessing the mandible with sensitivity for
cortical bone involvement reported to be 97–100% accompa-
nied by a specificity of 88% (Fig. 16.11).25 MRI, on the other
hand, may be better at assessing marrow space invasion;
however, its specificity is only 54% reflecting a high false
positive rate. SPECT scan has also been proposed as a second-
ary investigation to definitively exclude mandible involve-
ment after a negative CT scan because its reported specificity
is 100%.26
Through appropriate clinical and radiological evaluation,
involvement of the mandible can be reasonably predicted
prior to surgery.27 The mandibular periosteum is thought
to be a strong barrier to cancer spread, and in select cases
stripping of the periosteum may be adequate therapy,
Surgical approaches to the oral cavity
Most oral cavity cancers not involving the mandible can be
accessed transorally (Fig. 16.8). This approach results in
minimal morbidity for the patient and is the preferred approach
where possible. Management of the mandible is discussed
below, but the approach to accessing the mandible is most
Fig. 16.8  (A) Transoral resection of a lateral tongue border squamous cell
carcinoma. Note access to the posterior limit of the oral cavity. (B) The surgical
specimen demonstrating that adequate resection margins are obtainable with this
technique, which avoids the morbidity of mandibulotomy.
A
B
A
B
Fig. 16.9  Total maxillectomy performed via a Weber–Ferguson incision. (A) Preoperative marking of the Weber–Ferguson incision. (B) Exposure of the anterior face of the
maxilla.
Continued

408
SECTION II
CHAPTER 16 • Overview of head and neck tumors
C
D
E
(C) Osteotomies performed through the floor of the nose (i.e.,
the hard palate), medial, and lateral. (D) Reflecting the maxilla inferiorly to perform
posterior orbital floor release. (E) Post-ablation defect with orbital fat on view and
pterygoid plexus visible at posterior limit of dissection.
Fig. 16.9, cont’d

Fig. 16.10  Open surgical approaches to tumors of the upper alveolus and maxilla.
(A) Sublabial incision suitable for inferior maxillectomy and has the advantage of avoiding
facial incisions. (B) Weber–Ferguson incision allows cheek skin to be reflected off the maxilla
giving wide access to the bony maxilla. (C) Midface degloving. Sublabial incisions are
connected to external rhinoplasty incisions to deroof the nose and elevate skin flap superiorly
off the maxilla bilaterally.
A
B
C
A
Fig. 16.11  (A) Large left mandibular alveolus and retromolar trigone squamous cell carcinoma. (B) Frank bony erosion on CT scan.
B

410
SECTION II
CHAPTER 16 • Overview of head and neck tumors
osteotomy sites such that a sufficient margin can be obtained
around the tumor.
Oropharynx cancer
The oropharynx extends from the level of the hard palate
superiorly down to the tip of the epiglottis inferiorly (Fig.
16.15). It is continuous with the nasopharynx superiorly and
hypopharynx inferiorly as well as the oral cavity anteriorly.
The anterior tonsillar pillars and circumvallate papillae mark
the transition from oral cavity to oropharynx. The subsites of
the oropharynx are the palatine tonsils, base of tongue, soft
palate, and posterior pharyngeal wall (Fig. 16.16). The tonsil
is the most common site of tumor origin (60%), followed by
base of tongue (25%), soft palate (10%), and pharyngeal wall
(5%). A paradigm shift has occurred in oropharyngeal cancer
with the recognition of HPV as a strong and independent risk
factor for oropharyngeal SCC. HPV-associated oropharynx
cancer is different to the traditional smoking and alcohol
related cancers.2 HPV-associated cancers are seen in younger
patients who often do not smoke or drink alcohol, and an
even greater proportion of these are males. The gene expres-
sion profile is different (see pathophysiology of head and neck
particularly if the tumor is close but not abutting the bone.
Tumors that abut the mandible but do not obviously invade
it, as well as tumors that only superficially invade the bony
cortex, can be managed with a marginal mandibulectomy.28
This involves resection of part of the mandibular diameter
either as a rim resection or a lingual resection (Fig. 16.12). In
these cases, the resected mandible provides an appropriate
deep margin for the resection (Fig. 16.13). Partial resection of
the mandible reduces the bone stock available to counteract
the significant loads applied to the mandible. As a general
rule, at least 1 cm of mandible height should be preserved
in these cases to prevent pathologic fracture of the mandible.
This is particularly difficult in the edentulous patient. In
patients in whom this cannot be achieved, segmental resection
and appropriate reconstruction provide a more predictable
and durable functional result for the patient.
Significant invasion of the mandibular cortex, involve-
ment of the marrow space, or paresthesia of the mental
nerve suggesting inferior alveolar nerve involvement are
all indications to perform a segmental mandibulectomy
(Fig. 16.14). Preoperative imaging is useful in planning
Fig. 16.12  Marginal mandibulectomy. (A) Incorrect technique with right angle
osteotomies. This increases risk of subsequent fracture during to loading these
segments. (B) Curved marginal mandibulectomy reduces the chance of creating
weak points in the bone and more evenly distributes load across the mandible.
Incorrect
Correct
A
B
Fig. 16.13  (A) Floor of mouth tumor abutting the mandible. (B) Marginal
mandibulectomy in this site provides a bony margin, as well as providing excellent
access to the deep margin of these tumors, allowing adequate visualization to
appropriately resect the deep muscular margin.
A
B

A
B
Fig. 16.14  Segmental mandibulectomy performed in a patient with osteoradionecrosis after primary chemoradiotherapy for a left tonsil squamous cell carcinoma. (A) A
pathological fracture of the left mandible is demonstrated. (B) Following segmental mandibulectomy. (C) Fibula free flap reconstruction inset with mandibular plating
system. (D) End of procedure photo demonstrating the circumental lip split incision.
C
D
Fig. 16.15  Anatomy of the oropharynx. The oropharynx is
continuous with nasopharynx above and the hypopharynx below.
The subsites are visualized in this image also: tonsil, base of
tongue, soft palate, and posterior pharyngeal wall.
Nasal septum
Torus of tube
Torus tubarius
Inferior concha
Hard palate
Soft palate
Oral cavity
Palatoglossal arch
Palatine tonsil
Pharyngeal fossa
(Rosenmüller’s fossa)
Pharyngeal tonsil
Salpingopharyngeal fold
Hypopharynx
Oropharynx
Nasopharynx
Thyroid gland
Cricoid cartilage
Thyroid cartilage
Palatopharyngeal arch
Epiglottis
Hyoid bone
Median hyoepiglottic ligament
Laryngeal aditus
Transverse arytenoid muscle
True vocal cord

412
SECTION II
CHAPTER 16 • Overview of head and neck tumors
Surgical approaches to the oropharynx
The traditional approach to the oropharynx (Fig. 16.18) has
been through a mandibulotomy or alternatively via a trans-
cervical approach (pharyngotomy or lingual release). All of
these open surgical procedures are associated with signifi-
cant morbidity due to the interruption to the suprahyoid
musculature that is critical for swallowing function (Fig.
16.19). In addition, mandibulotomy is associated with poten-
tial complications such as non-union, malocclusion, and
chronic infection of mandibular hardware. With advances in
head and neck irradiation techniques, in particular intensity
modulated radiation therapy (IMRT), and extrapolation of
organ-preservation strategies from the larynx, non-surgical
treatment of oropharyngeal malignancies became increas-
ingly common. However, it is also recognized that this treat-
ment is not benign either, and the early and late effects of
radiation can significantly impair a patient’s quality of life.
Thus, there has been renewed interest in transoral approaches
to the oropharynx.30 Initially, this was with transoral laser
microsurgery (TLM), but the advent of the surgical robot
systems has sparked interest in transoral robotic surgery
(TORS), which is now FDA approved for early stage oropha-
ryngeal cancers.31–34 The advantage of a transoral surgical
approach is primarily the avoidance of a mandibulotomy
and its inherent morbidity. Patients return to a normal diet
more quickly, are less likely to require free flap reconstruc-
tion, and have a shorter hospital stay.35 TORS also offers the
benefit of distal chip telescopes that can be angled to visual-
ize around corners, the use of multiple instruments in the
mouth at one time to facilitate dissection and articulated
arms for increased maneuverability. Evaluation of the
oncologic and functional outcomes of transoral surgery is
currently underway in earnest. It appears that TORS is asso-
ciated with similar oncologic outcomes to primary radio-
therapy for p16 positive oropharyngeal SCC, however, a
difference in adverse event profile does exist between the
two modalities.36 Furthermore, this can be achieved in a cost-
effective manner.37 TORS is currently undergoing evaluation
through multi-center randomized controlled trials in the
management of oropharynx SCC.
cancer above) and the histology more commonly poorly dif-
ferentiated and basaloid in morphology due to the predilec-
tion for HPV to infect basal keratinocytes. Finally, the clinical
behavior is significantly different. The 3-year overall survival
for HPV positive patients who do not smoke is 93%, compared
with 46.8% for the typical non-HPV, tobacco-related orophar-
ynx cancer (Fig. 16.17).29 Thus, HPV-associated oropharyngeal
cancer is recognized as a unique clinical entity, a fact that may
be reflected in future iterations of the AJCC staging of this
primary site and already is driving change in treatment selec-
tion for such patients (Table 16.4).
Fig. 16.16  A left tonsil squamous cell carcinoma. Not the ulceroproliferative lesion
arising in the left tonsil fossa, extending onto the soft palate.
Fig. 16.17  Overall survival from oropharyngeal SCC stratified based on HPV status
and smoking history. HPV positive cancers in non-smoking patients has a 3-year
overall survival in excess of 90% compared with less than 50% for non-HPV
positive tumors in patients with a smoking history. (From Ang KK, Harris J, Wheeler
R, et al. Human papillomavirus and survival of patients with oropharyngeal cancer. N
Engl J Med. 2010;363(1):24–35.)
0
1
2
3
4
5
25
0
50
75
100
Hazard ratio for death, 0.38 (0.26–0.55); P<0.001
HPV-negative
HPV-positive
Table 16.4  Oropharynx T staging as per AJCC, 7th Edition
T stage
Oropharynx
Tx
Primary tumor cannot be assessed
T0
No evidence of primary tumor
Tis
Carcinoma in situ
T1
Tumor 2 cm or less in greatest dimension
T2
Tumor larger than 2 cm but 4 cm or less in greatest
dimension
T3
Tumor larger than 4 cm in greatest dimension or
involves lingual surface of epiglottis
T4a
Tumor invades larynx, deep/extrinsic muscle of
tongue, medial pterygoid, hard palate, mandible
T4b
Tumor invades lateral pterygoid, pterygoid plates,
lateral nasopharynx, skull base, or carotid artery

413
Oropharynx cancer
A
Lingual
nerve
B
C
Fig. 16.18  Open surgical approaches to the oropharynx. (A) Preparation for
paramedian mandibulotomy with lip split incision and tooth removal. (B) Paramedian
mandibulotomy allows access to the oropharynx when the floor of mouth mucosa is
incised and mandible is swung away from the midline. (C) The midline split of the
tongue provides excellent access to the posterior pharyngeal wall and cervical spine.
(D) Visoring the skin flap over the mandible and then making mucosal incisions in
the floor of mouth that connect to the neck dissection perform comprises a lingual
release. The tongue is then delivered into the neck, allowing visualization of the
oropharynx.
D

414
SECTION II
CHAPTER 16 • Overview of head and neck tumors
Fig. 16.19  Surgical approaches to the oropharynx. (A) Lingual release enables visualization of the tongue base, soft palate, and posterior pharyngeal wall. (B) Paramedian
mandibulotomy and mandibular swing also allows excellent visualization of the oropharynx.
A
B
Fig. 16.20  Anatomy of the larynx. The subsites of the
larynx, supraglottis, glottis, and subglottis can be
appreciated. The glottis is a watershed area for lymphatic
drainage; hence cancers at the glottis level metastasize
relatively late. The supraglottis has abundant lymphatic
drainage to the neck bilaterally and typically presents
with early regional metastatic disease.
Supraglottis
Subglottis
Glottis
10mm
Larynx and hypopharynx cancer
The larynx occupies a unique place in the anatomical and
functional make-up of the head and neck. The primary func-
tions of the larynx are respiration, airway protection, and voice
production; thus disease or surgery of the larynx can inhibit
critical functions that contribute to both the patient’s medical
health and psychosocial well-being. The hypopharynx wraps
around the posterior larynx and provides a portal of entry
for food to the esophagus, meaning that cancers that arise
here can significantly alter swallowing and thus nutritional
intake. The larynx extends from the tip of the epiglottis to
the inferior border of the cricoid cartilage and is subdivided
into the supraglottis, glottis, and subglottis (Fig. 16.20). Of
particular relevance to cancer treatment, the glottis is an area
of lymphatic watershed, meaning that only advanced glottis
cancers will attain lymphatic access for cancer dissemination

415
Larynx and hypopharynx cancer
to the regional lymph nodes. The supraglottis, on the other
hand, has rich lymphatic supply, and cancers arising here
present with clinically evident neck metastasis early in their
disease course (Table 16.5).
The hypopharynx extends from the tip of the epiglottis
(level of the hyoid) to the cricopharyngeus muscle at the entry
to the upper esophagus (lower border of the cricoid) and
contains three subsites – piriform fossa, posterior pharyngeal
wall, and post-cricoid region (Fig. 16.21). Cancer of the hypo-
pharynx is the least common of the upper aerodigestive tract
malignancies accounting for less than 5% of all head and neck
cancers. In addition, they have a dismal prognosis that is in
part due to their advanced stage at presentation due to their
concealed primary location, their unique propensity to spread
submucosally, making resection with clear margins difficult
as well as their ready access to abundant lymphatic channels.
80% of hypopharynx cancers will have neck nodal disease and
25% have distant metastatic disease at the time of presentation
(Table 16.6).
Evidence for treatment of larynx and
hypopharynx cancer
The management of laryngeal and hypopharyngeal cancer
has changed significantly over the last 30 years with the
emergence of new surgical tools and data from large clinical
Fig. 16.21  Anatomy of the hypopharynx. The hypopharynx wraps around the
posterior aspect of the larynx and can be subdivided into the piriform fossa,
post-cricoid region, and posterior pharyngeal wall.
Posterior surface over
arytenoid cartilage
Esophagus
Trachea
Prominence of
cricoid cartilage
Piriform sinus
Laryngopharynx
(hypopharynx)
Medial wall
Lateral wall of
piriform sinus
Corniculate tubercle
Cuneiform tubercle
Table 16.5  Larynx T staging as per AJCC, 7th Edition
Note the different staging systems for the different subsites of the larynx: supraglottis, glottis, subglottis
T stage
Supraglottis
Glottis
Subglottis
Tx
Primary tumor cannot be assessed
T0
No evidence of primary tumor
Tis
Carcinoma in situ
T1
Tumor limited to one subsite* of
supraglottis with normal vocal cord
mobility
Tumor limited to vocal cord(s) (may
involve anterior or posterior
commissure) with normal mobility.
T1a: tumor limited to one vocal cord,
T1b: tumor involves both vocal cords
Tumor limited to the subglottis
T2
Tumor invades mocusa of more than
one adjacent subsite of supraglottis or
glottis or region outside supraglottis
(e.g., mucosa of base of tongue,
vallecula, medial wall of piriform sinus
(without fixation of the larynx)
Tumor extends to supraglottis and/or
subglottis, and/or with impaired vocal
cord mobility
Tumor extends to vocal
cord(s) with normal or
impaired mobility
T3
Tumor limited to larynx with vocal cord
fixation and/or invades any of the
following: postcricoid area, pre-
epiglottic space, or paraglottic space
Tumor limited to the larynx with vocal
cord fixation and/or invasion of
paraglottic space and/or inner cortex
of thyroid cartilage
Tumor limited to larynx with
vocal cord fixation
T4a
Tumor invades through the thyroid
cartilage, and/or extends into soft
tissues of the neck, thyroid, and/or
esophagus
Tumor invades through outer cortex of
thyroid cartilage and/or to other
tissues beyond the larynx (e.g.,
trachea, soft tissues of neck including
deep extrinsic tongue muscles, strap
muscles, thyroid, esophagus)
Tumor invades through cricoid
or thyroid cartilage and/or
extends to other tissues
beyond the larynx (e.g.,
trachea, soft tissues of
neck, thyroid, esophagus)
T4b
Tumor invades prevertebral space, encases carotid artery or invades mediastinal structures
*subsites include the following: ventricular bands (false cords), arytenoids, suprahyoid epiglottis, infrahyoid epiglottis, aryepiglottic folds (laryngeal aspect)

416
SECTION II
CHAPTER 16 • Overview of head and neck tumors
study of 547 patients (RTOG #91-11) attempted to further
define the contribution of radiotherapy and chemotherapy
by comparing three groups – radiotherapy alone, concurrent
chemoradiotherapy, and induction chemotherapy followed by
radiotherapy.47 While overall survival did not differ between
groups, the concurrent chemoradiotherapy group had the
highest rate of larynx preservation and locoregional control.
Thus, it has become the standard non-surgical therapy for
advanced laryngeal cancer. Involvement of the laryngeal
cartilages and pre-treatment laryngeal dysfunction remain
indications for primary surgery; however, in the absence of
these features concurrent chemoradiation has become the
favored treatment approach with the hope of preserving laryn-
geal function. It is important to acknowledge the long-term
morbidity associated with non-surgical treatment of larynx
cancer. In a review of 230 patients from three RTOG trials
(91-11, 97-03 and 99-14), 43% had a severe late toxicity (severe
laryngopharyngeal dysfunction, feeding tube dependence
beyond two years or death related to effects of treatment).48
Organ preservation does not always equate with function-
preservation, and the effects of radiation therapy progress
well beyond the follow-up time points referenced in many
clinical trials.
The VA study design has also been applied in a study of
194 patients with hypopharynx SCC.49 The 10-year survival
was not different between the induction chemotherapy and
radiation group (13.1%) and the surgical group (13.8%);
however, more than half (8.2%) of the survivors in the non-
surgical group kept their larynx at the 10-year follow-up
point. With the same caveats of advanced laryngeal cancer,
primary non-surgical therapy is typically recommended to
these patients with surgery being reserved for salvage of
persistent or recurrent disease.
Surgical approaches to the larynx
and hypopharynx
The surgical approaches to early larynx cancer have already
been discussed. The gold standard surgical management of
advanced laryngeal cancer is total laryngectomy. This involves
removal of the airway from above the hyoid to the upper
tracheal rings and creation of a permanent tracheostome to
the anterior neck skin. The pharynx is closed primarily to
allow near-normal swallowing. Voice rehabilitation following
total laryngectomy is preferentially achieved through use of
a trachea-esophageal valve speaking prosthesis. Esophageal
speech and electrolarynx devices are less desirable methods
of restoring the voice. The anatomical location of these tumors
dictates that primary surgery in these cases is complicated by
the almost universal need for laryngectomy in addition to
pharyngectomy (partial or total) in all but the earliest of
hypopharyngeal cancers.
Management of the neck
The neck is the most common site of metastasis for head and
neck malignancy. In addition, it is the most important deter-
minant of prognosis with it being well established that the
presence of neck disease reduces survival by approximately
50%. Thus, rational decisions about treating the neck are para-
mount in head and neck cancer decision-making. Treatment
trials presenting patients and their physicians multiple treat-
ment options.38 As with other head and neck sites, early larynx
and hypopharynx cancer is treated with single modality
therapy. The options for treatment of early laryngeal cancers
are open partial laryngeal surgery (e.g., vertical partial laryn-
gectomy, horizontal supraglottic laryngectomy), TLM, or
radiation therapy. All of these approaches have similar onco-
logic control rates, which exceed 85% 5-year locoregional
control and overall survival.39–41 The morbidity profile of each
is different, and decision-making involves a detailed discus-
sion between the treating physician and the patient. TLM
offers the advantage of a single treatment episode and avoids
the side effects of radiation treatment. In general, TLM and
radiation therapy are the primary treatment options with
open partial laryngeal surgery reserved for patients who
refuse radiotherapy and have poor endoscopic access pre-
cluding TLM. Early voice outcomes after treatment are better
for radiation when compared to laser; however, the long-term
results do not demonstrate significant difference.42–44 Transoral
approaches, such as TLM or TORS for early hypopharyngeal
lesions, located away from the post-cricoid region and medial
wall of the piriform fossa may be appropriate; however, these
cases represent a small subset of patients with most patients
presenting with more advanced disease.45 The neck should
always be treated electively in supraglottic tumors and hypo-
pharynx cancers, by way of the modality used to treat the
primary site.
While the historical standard treatment for advanced
laryngeal cancer has been surgery with total laryngectomy,
followed by postoperative radiation therapy, this paradigm
changed significantly in the late 1980s and became more
broadly accepted following the publication of the VA study
in 1991.46 This study of 332 patients compared induction
chemotherapy followed by radiotherapy in patients who had
a partial or complete response (non-responders proceeded
to surgery) with a control arm that underwent the standard
therapy of surgery (total laryngectomy plus neck dissections)
and postoperative radiation. At 2 years, the survival was
68% for both groups with 64% of the study group having
the added benefit of keeping their larynx. A subsequent
Table 16.6  Hypopharynx T staging as per AJCC, 7th Edition
T stage
Hypopharynx
Tx
Primary tumor cannot be assessed
T0
No evidence of primary tumor
Tis
Carcinoma in situ
T1
Tumor limited to one subsite of hypopharynx <2 cm
T2
Tumor invades more than one subsite of hypopharynx
or an adjacent subsite or 2–4 cm in greatest
diameter without fixation of hemilarynx
T3
>4 cm in greatest dimension or with fixation of
hemilarynx
T4a
Tumor invades thyroid/cricoid cartilage, hyoid bone,
thyroid gland, esophagus, or central compartment
soft tissue
T4b
Tumor invades prevertebral space, encases carotid
artery, or invades mediastinal structures

417
Management of the neck
Fig. 16.22  The non-lymphatic structures that can be preserved in a modified
radical neck dissection: internal jugular vein, accessory nerve, and sternomastoid
muscle.
Fig. 16.23  The levels of the neck that enable description of the neck dissection. IA
is the submental triangle between the anterior bellies of digastric and the mandible.
IB is the submandibular triangle. Levels II, III, and IV are the jugular chain of lymph
nodes divided by the hyoid bone and the cricoid cartilage. Level II is further
subdivided to IIA and IIB by the accessory nerve. Level V is the posterior triangle,
divided into VA and VB by the omohyoid muscle. (From Robbins KT, Clayman G,
Levine PA, et al. Neck dissection classification update: revisions proposed by the
American Head and Neck Society and the American Academy of Otolaryngology–
Head and Neck Surgery. Arch Otolaryngol Head Neck Surg. 2002:751–758.)
IIB
III
IIA
IA
IB
VA
VI
IV
nerve leads to the debilitating shoulder syndrome that was
encountered almost universally amongst patients undergoing
radical neck dissection. With improved recognition of the
drainage patterns of the UADT mucosal sites, more conserva-
tive procedures are now advocated.51,53 Preservation of at least
the accessory nerve can improve function considerably for
these patients (Fig. 16.24). The move towards conservatism in
neck surgery is particularly relevant for the clinically negative
neck whereby only the “most likely” nodal levels are dis-
sected in order to pathologically stage the neck and provide
guidance for adjuvant therapy and prognosis. For the clini-
cally positive neck, a comprehensive neck dissection remains
the recommended procedure, although even in the setting of
early stage nodal disease, a selective neck dissection may still
be adequate therapy.
Just as the neck dissection has evolved as a surgical tech-
nique, so too the classification of this procedure has also
changed. Currently, neck dissections are classified as per the
system agreed on by the American Head and Neck Society
and the American Academy of Otolaryngology-Head and
Neck Surgery (Box 16.1).54 This classification fundamentally
reports the number of levels dissected and the preservation
or resection of non-lymphatic structures, including the ster-
nomastoid muscle, accessory nerve, internal jugular vein,
overlying skin, hypoglossal nerve, carotid artery, and vagus
nerve, amongst others. The most commonly performed neck
dissection is a selective I–IV neck dissection, and the steps of
this procedure are listed in Box 16.2. It is important to
of the neck may be either therapeutic or elective depending
on whether or not there is clinically detectable nodal disease
at the time of presentation. In the setting of clinically evident
neck disease, a therapeutic neck dissection is warranted with
adjuvant therapy decided based on the pathologic features. In
the patient without clinical evidence of neck disease, the deci-
sion to treat or not to treat the neck is based on the probability
of occult neck disease being present, as treating this disease
while it is microscopic confers an improved prognosis for
the patient compared with attempting salvage surgery after
recurrence becomes clinically evident.50 In a landmark study,
Weiss et al. determined that above an occult metastasis risk of
20%, the benefits of elective neck treatment outweighed the
risks.24 In the head and neck, this encompasses all subsites
except for T1 lip and glottis cancers as well as thin early stage
tumors of the oral cavity.51,52 The choice of treatment modality
for the neck is dictated by the treatment of the primary site
– surgery or radiation therapy.
Surgical approaches to the neck:
neck dissection
First described by George W. Crile in 1906, the neck dissection
is an en bloc resection of the nodal drainage stations of the
UADT. In its original form, the radical neck dissection was a
systematic removal of the lymph nodes from the mandible to
the clavicle and from the midline to the trapezius in addition
to the sternomastoid muscle, internal jugular vein, and acces-
sory nerve (Fig. 16.22). This package of nodal tissue is now
divided into a number of levels, which allow accurate descrip-
tion of the procedure and indicate most common sites of
metastatic spread from different subsites of the head and neck
(Fig. 16.23). The radical neck dissection is undoubtedly a
morbid procedure, in particular the sacrifice of the accessory