Chẩn Đoán Và Điều Trị Phẫu Thuật Tái Tạo Hiện Đại (CURRENT Reconstructive Surgery)

VASCULAR MALFORMATIONS • 83
been described as treatment options, but direct excision
is usually the treatment of choice. A minimal margin is
needed, as malignant glomangiosarcomas are extremely
rare. Microscopic analysis will confi rm the presence of
glomus cells, distinguishing them from small venous mal-
formations ( Figure 8-8 ).
Varicose veins of the extremities, whether congenital or
acquired, may be candidates for sclerotherapy, intravenous
laser coagulation, surgical ligation and division/excision, or
a combination of the above. Preoperative assessment should
confi rm the presence of a normal venous drainage system,
as removal of the abnormal system is contraindicated if it
is the only source of vascular outfl ow. Additionally, scle-
rotherapy may be less effective or contraindicated if the
abnormal veins connect directly to large normal veins, as it
will be diffi cult to keep the sclerosant from fl owing proxi-
mally. Combined venous ligation and division combined
with sclerotherapy in such situations may produce out-
standing results ( Figure 8-9 ).
Lymphatic and Venolymphatic
Malformations
Lymphatic malformations are among the most diffi cult
to treat due to their extensive distribution and tendency
to microscopically extend into normal appearing tissues,
often making complete removal impossible or undesirable.
Since veins and lymphatics travel together, it is common
to have malformations that involve both structures, and
many lymphatic cysts also contain blood from adjacent
veins. Lymphatic malformations are present at birth but
not always clinically apparent. Time and gravity may lead
to progressive lymphatic engorgement that was not obvi-
ous in early childhood.
A
B
FIGURE 8-5. (A–D) Massive lower lip hypertrophy associated with a capillary
vascular malformation of the lower face. Aggressive debulking may require wide wedge
resections. Temporary placement of vascular clamps will decrease intraoperative
bleeding.
C
D

84 • CHAPTER 8
Although the location and age of presentation have led
to various names such as cystic hygromas for congenital cer-
vicofacial lymphatic malformations, Milroy disease for con-
genital lymphedema of the extremities present at birth, 2
Meigs disease (lymphedema praecox) for lymphedema pre-
senting after birth and up to age 35 years, and lymphedema
tarda for lymphedema presenting after age 35 years; in fact,
one can think of each of these conditions as a form of lym-
phatic malformation with varying degrees of severity.
Lymphatic malformations become problematic because
of their tendency to undergo increasing soft tissue distor-
tion as individual lymphatic cysts enlarge, but also because
dermal lymphatic vesicles have a tendency to rupture and
bleed. Cellulitis is a common problem requiring periodic
antibiotic suppression. Hypertrophy of the involved area,
including osseous overgrowth, complicates attempts at
debulking surgery. Recurrent episodes of cellulitis and
infl ammation make dissection diffi cult as tissue planes are
obliterated and vital structures such as nerves are diffi cult
to identify and preserve. Cervicofacial lymphatic malfor-
mations can be life-threatening because of the risk of air-
way obstruction, and a tracheostomy may be necessary for
airway management.
MRI scans are useful to document the extent of a lym-
phatic malformation and also distinguish macro from
microcystic disease. Large lymphatic cysts may shrink sig-
nifi cantly with intralesional sclerotherapy, and the morbid-
ity of surgical debulking can often be avoided with the aid
FIGURE 8-6. (A, B) Disseminated venous malformations (blue rubber bleb nevus
syndrome) involving all four extremities. These can be directly excised if they become
symptomatic, as in the case of this long fi ngertip malformation. Large malformations may
be better candidates for sclerotherapy.
A
B
FIGURE 8-7. (A, B) Massive tongue hypertrophy associated with venous
malformations of the oral cavity can be safely debulked, resulting in signifi cant
improvement in tongue mobility and oral airway obstruction.
A
B

VASCULAR MALFORMATIONS • 85
FIGURE 8-8. Glomus tumors appear as clusters
of dark bluish nodules in the dermis and subcutaneous
tissues. Surgical removal is indicated for those that are
symptomatically tender.
of a skilled interventional radiologist. Alcohol and doxy-
cycline are commonly used in the United States; whereas
in Japan, a streptococcal protein derivative OK-432 is an
effective sclerosant that still awaits FDA approval.
Surgical debulking involves dissection and removal
of large discrete lymphatic cysts or empiric debulking of
excessive lymphatic tissue characterized by countless small
cysts ( Figure 8-10 ). It is important to remind patients that
reoccurrence is common and the degree of improvement is
often disappointingly small and often temporary. It is not
uncommon to spend hours of tedious dissection on a cer-
vicofacial lymphatic malformation, only to have postopera-
tive edema obscure the postoperative result, complicated
by a facial nerve injury. Compression garments and wraps,
where applicable, may prolong the benefi cial effect of surgi-
cal debulking.
Carbon dioxide laser vaporization of ulcerated cutane-
ous lymphatic vesicles may offer palliative relief from con-
stant lymphatic drainage, as the fi brosis caused by the laser
may help to cap the leaking lymphatic vessels. If the vesi-
cles contain heme, the KTP laser may offer similar pallia-
tion as the blood in the vesicles absorbs the laser energy.
Klippel-Trenaunay Syndrome
The combination of a patchy capillary vascular malfor-
mation (port wine stain), an underlying venolymphatic
malformation, and (usually) hypertrophy of the involved
area is characteristic of Klippel-Trenaunay syndrome
( Figure 8-11 ). In the lower extremity, a prominent lat-
eral varicose vein may be evident, a persistent lateral
vein that normally obliterates in utero. Dermal lymphatic
FIGURE 8-9. (A–C) Congenital venous malformation of the thigh and knee.
A diagnostic venogram shows massively dilated veins. Surgical ligation and division of
the draining vein adjacent to its junction with the femoral vein, followed by ultrasound-
guided sclerotherapy, produced outstanding results.
A
B
C

86 • CHAPTER 8
involvement often creates problematic crusting lesions
within the area of capillary malformation (angiokerato-
mas). Patients complain of ulcerated angiokeratomas that
cause troublesome bleeding, which is actually heme-col-
ored lymphatic drainage that clots poorly. The KTP laser
may be useful for palliative photocoagulation, directed
through a glass slide to control bleeding. Alternatively,
direct excision and suture closure will also control these
annoying cutaneous vascular lesions.
Compression garments, particularly when the lower
extremity is involved, are the treatment of choice. Surgical
debulking and sclerotherapy can usually be avoided if
compression stockings are used on a consistent basis. Most
patients with Klippel-Trenaunay syndrome are poor sur-
gical candidates, given the extensive distribution of the
malformation. Occasionally, limited involvement can be
surgically debulked, but microscopic peripheral involve-
ment makes cure unlikely.
Hypertrophy of the lower extremity may also manifest
itself as excessive limb length, and periodic plane fi lms
of the femur and tibia are used to document exact limb
length. A shoe lift for the shorter leg may be prescribed
by a pediatric orthopedic surgeon, and destruction of
the growth plate (epiphysiodesis) may be indicated when
the longer leg reaches predicted adult size.
Arteriovenous Malformations
Arteriovenous malformations are usually congenital, but
acquired arteriovenous shunting can occur as the result
of penetrating injuries and central line placement. These
high-pressure, high-fl ow malformations can occur any-
where, and they are potentially dangerous as they progres-
sively expand, particularly during puberty.
Clinical characteristics include a pulsatile mass with
increased warmth, sometimes with an audible bruit. An
arteriovenous malformation (AVM) may have an overlying
capillary vascular malformation (Parkes-Weber syndrome).
An MRI/MRA scan with contrast will provide the most
information to formulate a treatment plan.
Complete surgical excision represents the best chance
for cure, and preoperative embolization by an interven-
tional radiologist on the day prior to surgery can limit sur-
gical blood loss as well as provide a useful roadmap to aid
surgical dissection ( Figure 8-12 ). By staying just periph-
eral to the malformation, the surgeon can operate in nor-
mal tissues and avoid excessive bleeding. Feeding arterial
branches outlined on the MRA scan or arteriogram can
be sequentially ligated and divided. Extensive resections
may require fl ap reconstruction or skin grafting to achieve
closure.
Palliative, highly selective embolization by an inter-
ventional radiologist may be done periodically for facial
AVM that are not problematic enough to warrant exten-
sive extirpative and reconstructive surgery. Patients must

FIGURE 8-11. Klippel-Trenaunay syndrome is
characterized by a patchy port wine stain with an underlying
venolymphatic malformation and hypertrophy of the involved
region. When the leg is involved, a persistent lateral varicose
vein is often present, which may cause problematic bleeding.
B
FIGURE 8-10. (A, B) Congenital lymphatic malformation
of the left leg. Aggressive debulking results in a signifi cant
contour improvement, but the treatment is only palliative, and
a compression stocking is recommended to control recurrent
edema and tissue hypertrophy.
A

VASCULAR MALFORMATIONS • 87
FIGURE 8-12. (A–C) Arteriovenous malformations of
the buttocks supplied by the middle sacral and internal iliac
arteries. Preoperative embolization reduced intraoperative
bleeding and provided a vascular roadmap to assist with
surgical planning and complete excision.
understand that these procedures are not intended to be
curative, but may decrease much of the fl ow, bulk, and
discomfort associated with these malformations. The pro-
cedure avoids embolization of the external carotid artery
itself, as this would only encourage collateral fl ow from
the internal carotid or contralateral carotid system, mak-
ing future embolization technically impossible or prohibi-
tively dangerous.
A
C
B

88 • CHAPTER 8
Pyogenic Granulomas
Pyogenic granulomas are a true misnomer, being neither
pyogenic nor granulomatous. They are more accurately
termed lobular capillary hemangiomas, benign dermal
vascular tumors that may arise from minor skin trauma.
They can occur at any age, and are often seen in preg-
nant women, suggesting a hormonal component in their
pathophysiology.
They present as shiny red papules with a fragile epi-
dermal cover that ulcerates easily. Bleeding can be fright-
eningly excessive, and many patients present with silver
nitrate stains and bandages (souvenirs of the local emer-
gency room).
Surgical excision provides defi nitive treatment and
a biopsy specimen, but at the cost of a surgical scar.
Tangential excision followed by either KTP laser or elec-
trocautery coagulation of the residual dermal proliferative
vessels provides a biopsy specimen and often no percep-
tible scar ( Figure 8-13 ). Complete destruction by electro-
cautery or laser without a biopsy may be curative, but it is
medicolegally risky because it fails to provide a confi rma-
tory pathology report.
Angiofi bromas
Patients with tuberous sclerosis develop erythematous
papular lesions in a prominent butterfl y distribution on
the face, but also on the chin and alar regions. Historically
called “adenoma sebaceum,” they are neither adenomatous
nor sebaceous and are instead fi brovascular nodules that
are more accurately called angiofi bromas. Although the
pulsed yellow dye laser has been recommended for the
hypervascular erythema, most patients and their parents
are more concerned with the nodular skin irregularity.
The carbon dioxide laser is very effective in vaporizing
these lesions, using a slightly defocused 1 mm spot and
1 to 3 watts of power ( Figure 8-14 ). Patients should expect
about 2 weeks of crusting until each spot re-epithelializes.
The treatments are palliative and completely smooth skin
is an unrealistic expectation, but the degree of improve-
ment can be dramatic. Recurrent angiofi bromas can be
periodically retreated.
Telangiectasias, Spider Angiomas,
Cherry Angiomas, and Spider Veins
All of these vascular malformations are characterized by
dilated dermal vessels that are readily apparent to the

FIGURE 8-13. Pyogenic granuloma of the cheek.
Tangential biopsy followed by laser or electrocautery of the
base has a high success rate and a scar that may be superior
to excision and suture closure.
FIGURE 8-14. (A, B) Angiofi bromas associated with tuberous sclerosis can be easily
vaporized with minimal bleeding using a carbon dioxide laser. Laser safety precautions
include the use of protective scleral shields and a plume evacuator.
A
B

VASCULAR MALFORMATIONS • 89
COMPLICATIONS OF TREATMENT
OF VASCULAR MALFORMATIONS
Laser therapy is basically selective thermal delivery to the
abnormal vessels to cause intentional rupture or coagula-
tion. Nonselective photothermolysis can occur with exces-
sive energy delivered to the tissues. Blisters, ulceration,
crusting, and scarring can occur when the energy density,
the pulse duration, or the laser power setting is too high.
Sclerotherapy can cause cutaneous ulceration and tissue
necrosis if the sclerosant is erroneously injected extravas-
cularly, or if the sclerosant extravasates into the soft tis-
sues. In addition to skin necrosis, muscle and nerve injury
can occur, and depending on the type of sclerosant this
may be permanent. More importantly, excessive intravas-
cular injection can cause hemodynamic complications and
death. Airway compromise from postsclerotherapy edema
may require prolonged intubation when treating oropha-
ryngeal venous and lymphatic malformations.
Embolization of the feeding arteries to treat AVMs can
cause undesirable distal ischemia of unintended targets,
particular when treating the extremities. When treating
head and neck AVMs, the risk of potential stroke must be
discussed with the patient.
Excessive bleeding during debulking procedures, espe-
cially with high-fl ow arteriovenous malformations or large
venous or venolymphatic malformations, may warrant the
use of a cell saver, and blood and plasma transfusions may
be required. Patients should be advised of the potential
need to abort the debulking procedure if life-threatening
hemorrhage occurs.
Patients with large venous or venolymphatic malfor-
mations may have ongoing clotting issues (disseminated
intravascular coagulation) which put them at risk for deep
venous thrombosis and pulmonary embolism. Surgery and
sclerotherapy may result in life-threatening thrombosis
and embolism. Patients should be evaluated by a hematol-
ogist prior to their procedures, which may warrant lifetime
anticoagulation therapy. Placement of a vena cava fi lter is
not a guarantee against pulmonary embolism in the setting
of venous malformations which may present atypical vas-
cular anatomy that allows clots to bypass the fi lter.
naked eye. Telangiectasias are often called “broken blood
vessels” because they undulate at different depths within
the dermis. Although the vessels are continuous, only the
superfi cial segments are easily visualized. Spider angiomas
have a central feeding arteriole that arborizes into many
peripheral branches, similar to a tree trunk and its branches.
They blanch with digital pressure, then fi ll readily from
the central feeder out to the periphery when pressure is
released. Cherry angiomas are dilated superfi cial dermal
vessels that appear as papular red spots. Spider veins con-
sist of small dilated dermal vessels that can appear on the
nose, face, and legs.
Laser is a popular modality to treat all of these con-
ditions, and there are a wide variety of vascular lesion
lasers that are potentially effective. Pulsed yellow dye
lasers rapidly cover large areas, and circular handpieces
are generally preferred for discrete spots like cherry or spi-
der angiomas, while the elliptical 3 3 10 mm handpiece
is appropriate for tracing spider veins. Continuous wave
lasers require meticulous yet rapid tracing of tiny spider
veins, with care being taken to avoid prolonged energy
delivery to any given spot to minimize the risk of burn
injury. The patient should be aware of the need for mul-
tiple treatment sessions for best results, and realize that
it is not possible to remove every spider vein and telang-
iectasia. On the other hand, spider angiomas and small
cherry angiomas are commonly obliterated with a single
laser treatment. Continuous wave lasers such as the KTP
laser may be more effective than pulsed lasers in obliterat-
ing cherry angiomas as the heat penetrates throughout the
vascular cluster.
Large spider veins, particularly on the nose and legs
may be refractory to multiple laser treatments, and
greater success may be achieved with either sclero-
therapy, or the combination of sclerotherapy followed
immediately by laser photocoagulation for a synergistic
vascular insult.
Intense pulsed light may be effective for both small
telangiectasias as well as benign melanocytic pigmentation,
depending on the fi lters used to target oxyhemoglobin and
melanin.
1. Meyerle JH. Dermatological manifestations of glomus
tumors.
.
Up dated Sept 17, 2010.
2. Kiel RJ. Milroy disease.
topic1482.htm . Updated June 21, 2006. Accessed August 30,
2006.
REFERENCES
SUGGESTED READING
Arneja JS, Gosain AK. Vascular malformations. Plast Reconstr
Surg. 2008;121(4):195e–206e. Huang JT, Liang MG. Vas-
cular malformations. Pediatr Clin North Am. 2010;57(5):
1091–1110.
Marler JJ, Mulliken JB. Current management of hemangiomas
and vascular malformations. Clin Plast Surg. 2005;32(1):
99–116.

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9
Hemangiomas
David W. Low, MD / Richard L. Agag, MA, MD
c h a p t e r
INTRODUCTION
Hemangiomas are the most common benign tumor of
infancy, occurring in 10% of full-term babies and up to 25%
of premature babies. Although hemangiomas can occur
anywhere, the majority occur in the head and neck region,
raising cosmetic as well as functional concerns. Whereas
historically pediatricians involved plastics surgeons in the
care of patients with persistent deformity in late child-
hood, nowadays with widespread access to the Internet
and hemangioma support groups, parents are seeking early
plastic surgical consultation and surgical solutions to this
common vascular anomaly.
Natural History
Typical hemangiomas are not present at birth, but appear
during the fi rst few weeks of life. Dermal hemangiomas
appear as bright red strawberry patches that gradually
protrude above the surface of the skin. Subcutaneous
hemangiomas are also composed of proliferating capillar-
ies but appear blue in pigmentation due to their deeper
location. Many hemangiomas have both a subcutaneous
and dermal component ( Figure 9-1 ). Hemangiomas may
also occur in solid organs such as the parotid gland and
liver. Large facial hemangiomas may be part of a constella-
tions of anomalies designated by the mnemonic PHACES
( p osterior cranial fossa abnormalities, h emangiomas,
a rterial abnormalities, c oarctation of the aorta and other
cardiac anomalies, e ye abnormalities, and s ternal cleft-
ing). Midline back hemangiomas may be associated with
a tethered spinal cord.
Proliferation typically occurs over the fi rst 6 to
12 months, followed by slow regression over many years.
Regression is characterized by a change in pigmenta-
tion as the bright strawberry color gives way to grayish
white, and the hemangioma becomes less engorged and
decreases in size. Pediatricians tend to advise parents that
most hemangiomas will disappear by age 5 years, but only
50% of patients will have completely regressed by that
time (70% of hemangiomas fully regress by age 7 years).
Although all hemangiomas will show regression, 30%
will leave signifi cant residual deformity in the form of
redundant skin and excess fatty bulk that may warrant
plastic surgical intervention. More importantly, cosmeti-
cally important areas, such as the eyelids, nose, lips, and
cheek, seem to have a much higher risk of leaving signifi -
cant deformity, creating not only great concern amongst
parents, but also the potential for litigation if parents feel
an alternative management strategy might have resulted
in less deformity.
Nonsurgical Therapy for Hemangiomas
Pediatric plastic surgeons are in a unique position to
become the primary treating physicians for young infants
with proliferating hemangiomas, as they can offer the
entire spectrum of treatment options. In many cases fi rst-
line therapy is medical rather than surgical, and consists of
systemic or intralesional steroid therapy. Steroid therapy
may signifi cantly decrease the degree of residual defor-
mity, and although it may not remove the need for later
reconstructive surgery, it may simplify the complexity of
the surgical procedure and increase the chance for a suc-
cessful outcome.
Oral prednisolone is commonly used for airway or
vision-threatening hemangiomas, but it is also widely used
for potentially disfi guring hemangiomas of the face. Nasal
and lip hemangiomas routinely leave permanent defor-
mity and most parents readily opt for steroid suppression
to limit the degree of deformity. Orapred is commonly
prescribed as it is reportedly the most palatable and more
concentrated than other oral preparations. The starting
dose is 2 to 4 mg/kg/d, usually mixed with the morning
bottle. Most patients will show obvious signs of regression

92 • CHAPTER 9
within a week. Although steroid tapering schedules vary
by physician, it is usually easiest for parents to taper the
dose every 1 to 2 weeks in easily measured increments.
Parents should be advised that infants may require ste-
roid therapy for many months, and at lower steroid dos-
ages rebound growth may necessitate a temporary increase
in dose and a more gradual taper. Side effects commonly
include a change in appetite, increased irritability, gastroin-
testinal upset, cushingoid facies, and a plateau in growth,
all of which are temporary and reversible. Hypertension
may necessitate a more rapid taper or the addition of anti-
hypertensive therapy. Live, attenuated virus vaccinations
(measles, mumps, rubella, and chicken pox) should be
withheld until steroid therapy has been discontinued for
a month.
To decrease the risk of gastrointestinal upset associated
with steroid therapy, Zantac liquid is administered orally
3 mg/kg/d until steroids are discontinued.
The ophthalmologic literature supports the potential
benefi t of triamcinolone (Kenalog-40) as an intralesional
injection for periorbital hemangiomas. The risk of potential
embolization of the steroid suspension to the retinal artery
with subsequent blindness suggests that steroid injection
in this area be limited to ophthalmologists and oculoplas-
tic surgeons. However, small discrete hemangiomas in
other parts of the face or body can be carefully injected
by plastic surgeons. Intralesional injection on a monthly
FIGURE 9-1. Typical compound hemangioma with both
a central dermal and a subcutaneous component.
as-needed basis is certainly easier than daily oral adminis-
tration, but there is no evidence that it avoids systemic side
effects. Injection is unlikely to distribute the medication
uniformly within the tumor, and it is not appropriate for
large hemangiomas.
Recent Medical Advances
Propranolol, used as a non-selective, beta-adrenergic
receptor blocker in young children with cardiologic indi-
cations, was observed serendipitously to have dramatic
effects on the growth of hemangiomas. Due to the variable
response rates of systemic corticosteroid therapy (range
30%–60%) and side effects, excitement has grown over
this new therapy. After the sentinel case report, multiple
case reports and then a larger follow-up report by the ini-
tial authors has shown much promise; however, much of
the details still need to be worked out. In this follow-up
study, because of the potential side effects, the children
were admitted into the hospital for a 24-hour period and
the drug was administered 2 mg/kg/d divided over two to
three doses. The responses to the drug were remarkable
( Figure 9-2 ). Included in these 32 patients were 13 infants
who had shown no response or at best stabilization of
the lesion after treatment with systemic corticosteroids.
Within 24 hours of treatment of propranolol, therapeutic
effects were seen, including change in color and softening
of the lesions.
Yet another case report showed treatment of a multi-
centric airway hemangioma with rapid regression and res-
olution of symptoms. This subset of children often suffer
from symptoms such as stridor, breathing diffi culties, feed-
ing diffi culties, respiratory distress, barking cough, hoarse-
ness, and acute airway obstruction. The patient in this
case suffered from biphasic stridor, which improved over
the course of 10 days after administration of propranolol.
Reduction in bulk and improved subglottic stenosis was
confi rmed by repeat laryngotracheobronchoscopy 1 week
after initiation of the drug.
However, use of propranolol does not come without
risk, although seemingly less than that of corticoster-
oids. FDA labeling indicates that safety and effectiveness
of propranolol have not been established in pediatric
patients. The most common hemodynamic side effects
are bradycardia and hypotension. Hypoglycemia is also
another reported side effect. There is also a subset of
patients that are at risk for high-output cardiac failure.
These include patients with posterior fossa brain malfor-
mations, cardiac abnormalities, and suspected PHACES
(segmental facial hemangioma) to name a few. These
patients require a careful premedication screening and
clearance.
Little is known about how propranolol directly affects
hemangiomas; however, there is data on the mechanism of
action of the drug to which these effects can be attributed.
Specifi cally, how propranolol interferes with endothelial
cells, vascular tone, angiogenesis, and apoptosis. Early effects
(brightening of the hemangioma surface within 1–3 days)

HEMANGIOMAS • 93
are attributed to vasoconstriction due to decrease nitrous
oxide release. Intermediate effects are due to blocking of
pre-angiogenic signals (vascular endothelial growth factor,
basic fi broblast growth factor, matrix metalloproteinases
2/9), which result in arrest of growth. Long-term effects
are the result of apoptosis in proliferating cells, which
results in tumor regression.
We have developed a protocol at the Children’s Hospital
of Philadelphia which included contributions from mul-
tiple specialties. Our relative contraindications to propra-
nolol include history of hypersensitivity to beta-blocker
therapy, ischemic intracranial anomalies, structural cardiac
abnormalities, decrease cardiac output, and active hypo-
glycemia or reactive airway disease. Patients with these
conditions require a more extensive workup including a
cardiology consult. We do not routinely consult cardiol-
ogy before initiation of propranolol unless contraindica-
tions to the therapy are elucidated after history, physical
examination, and electrocardiogram. Parents need to be
made aware of the off-label nature of treatment during
the consent process. The patient is admitted to the ICU
setting or inpatient hospital fl oor based on risk stratifi ca-
tion. Baseline vitals are taken and then propranolol treat-
ment is initiated at 0.5 mg/kg/d divided in three doses.
Blood glucose levels measurements are taken 1 hour after
each of the fi rst two doses. Day 2 and day 3 of hospital
admission include increasing the dosage to 1 mg/kg/d to
2 mg/kg/d with continued monitoring of blood glucose
levels, blood pressure, and heart rate. If suboptimal results
are obtained, doses may be escalated to 3 to 5 mg/kg/d in
0.5 to 1 mg/kg/d increments. After target dose has been
achieved, heart rate and blood pressure monitoring is rec-
ommended weekly or biweekly in an ambulatory setting.
Anticipation is that the propranolol therapy may be nec-
essary for approximately 3 to 6 months, or until the child
is 6 to 12 months of age.
SURGICAL TREATMENT
OF HEMANGIOMAS
Patient Evaluation and Selection
The vast majority of hemangiomas do not require urgent
surgical intervention during the proliferative phase and
never come to the attention of a plastic surgeon until
pediatricians and pediatric dermatologists refer those with
inadequate involution for debulking and scar revision.
However, if life-threatening subglottic hemangiomas with
airway obstruction and periorbital hemangiomas with visual
obstruction respond poorly to steroid suppression, they may
require urgent debulking surgery, usually by otolaryngolo-
gists and oculoplastic surgeons, respectively. Intrahepatic
hemangiomas associated with high-output cardiac failure
may require selective embolization by an interventional
radiologist if steroid therapy is ineffective.
Urgent plastic surgical intervention may be needed for
ulcerated hemangiomas that have eroded into an artery
large enough to cause signifi cant hemorrhage, perior-
bital hemangiomas that are causing visual obstruction
and decreased visual acuity, large lip hemangiomas that
are interfering with feeding, and occasionally, ulcerated
hemangiomas of the lips and perineum that are causing
excruciating pain.
Elective plastic surgical intervention is appropriate for
hemangiomas that are destined to leave signifi cant residual
deformity in the form of redundant skin, fi brofatty bulk,
signifi cant dermal fi brosis, scarring alopecia of the scalp,
distortion of involved facial features, and potential func-
tional problems anywhere on the body ( Figure 9-3 ).
Patient Preparation
Timing. While it is technically feasible to operate on any
patient with a signifi cant hemangioma as soon as they
FIGURE 9-2. Patient at 3 months of age with a left eyelid hemangioma.
Improvement after 1 months of propranolol treatment. (Photographs courtesy of Albert Yan,
MD, Department of Dermatology, Children’s Hospital of Philadelphia.)
A
B

94 • CHAPTER 9
present for consultation, one should take into consider-
ation how far along the course of its natural history the
hemangioma has progressed. Although it is a continuum,
one can semi-arbitrarily divide the clinical course into
multiple phases:
1. Proliferative phase—the hemangioma is still actively
growing without any signs of regression.
2. Early involutional phase—the hemangioma shows
signs of pigmentary lightening and reduced turgor,
suggested a diminution in blood fl ow, but still fi lls
readily after compression.
3. Mid involutional phase—the hemangioma still has
some fl ow, fi lls slowly after compression, and much
of its vascular pigmentation is gone.
4. Late involutional phase or completely involuted—the
fl ow has essentially stopped, leaving stable fi brofatty
tissue and redundant skin without any expectation of
further improvement.
5. RICH— r apidly i nvoluting c ongenital h emangiomas
6. NICH— n on i nvoluting c ongenital h emangiomas
Proliferative Phase . Hemangiomas in the very early
proliferative phase are candidates for conservative obser-
vation, laser, or steroids, but surgical excision is rarely
indicated as one cannot predict the growth of the heman-
gioma and excision will always leave a scar. Additionally,
many hemangiomas proliferate over an extensive area and
removal of a small nidus will not reliably prevent periph-
eral growth.
Biopsy of an atypical hemangioma is indicated in the
proliferative phase to rule out a more aggressive vascu-
lar tumor such as a kaposiform hemangioendothelioma
FIGURE 9-3. Involuted chest hemangioma that has
left residual fi brofatty tissue and redundant skin. Excision is
indicated since the anticipated contour benefi t outweighs the
anticipated surgical scar.
or vascular malignancy, or a soft tissue malignancy.
Kaposiform hemangioendotheliomas tend to feel harder
than hemangiomas and are associated with thrombocy-
topenia (Kasabach-Merritt syndrome).
Hemangiomas that are well into their proliferative
phase and are causing signifi cant disfi gurement and
parental anxiety must be carefully evaluated, and the
risks and benefi ts of early excision must be thoroughly
weighed.
Ulcerated hemangiomas with signifi cant hemorrhage
may be urgently controlled with a hemostatic suture.
Excruciatingly painful ulcers can be palliated with 2%
lidocaine jelly. Ulcerated hemangiomas always leave a
cutaneous scar, and therefore small problematic heman-
giomas can be excised, and parents will readily accept the
surgical scar in exchange for peace of mind.
Early Involutional Phase. Hemangiomas that have
stopped growing will take years to naturally regress, and
parents’ eagerness to resolve the problem as soon as pos-
sible must be weighed against the risks of general anes-
thesia, the increased risk of perioperative bleeding, and
the chance that the permanent surgical scar will be more
disfi guring than natural involution. A linear central cheek
scar may be far more noticeable than a fl at patch of atro-
phic skin, and the overly aggressive plastic surgeon may be
doing his young patient a lifelong disservice by operating
too early. Pedunculated hemangiomas will almost certainly
leave excessive skin and soft tissue, and can be considered
for early excision. Hemangiomas that are too large to be
completely excised in a single stage may be better left to a
later year when the risk of hemorrhage and wound dehis-
cence is lower.
Mid-Involutional Phase. The majority of hemangioma
excisions take place during this period, when it is obvious
that the hemangioma is not going to regress satisfactorily,
and the risk of intraoperative hemorrhage is lower as the
blood fl ow has decreased. If the procedure can be done
without the risk of needing a transfusion and with minimal
risk of injury to local vital structures, then there is no need
to make the parents wait for complete involution.
Late or Complete Involutional Phase. These patients
usually have excess skin and/or bulky fi brofatty tissue and
few residual vessels. Therefore, it is much easier to estimate
how much tissue to excise or leave behind, and ancillary
procedures such as liposuction or tissue expansion can be
planned without risk of excessive bleeding.
RICH—Rapidly Involuting Congenital Hemangiomas.
A small subset of hemangiomas appear to demonstrate in
utero growth and accelerated regression during the fi rst
year of life. Rapid involution may be associated with prob-
lematic ulceration and central tissue necrosis. Treatment
should be individualized and the timing of surgical inter-
vention can follow the same guidelines as for typical
hemangiomas.

HEMANGIOMAS • 95
NICH—Noninvoluting
Congenital
Hemangiomas.
Another subset of hemangiomas that do not follow the
expected course of typical hemangiomas show no tendency
to involute. They often have the clinical appearance of
bluish subcutaneous masses with small cutaneous telangi-
ectasias and increased warmth to palpation ( Figure 9-4 ).
Elective excision is curative.
Laser Considerations
A variety of vascular lesion lasers that target oxyhemo-
globin may be useful in treating hemangiomas, predomi-
nantly in the early proliferative or the late involutional
phases. The laser energy is absorbed by dermal blood ves-
sels, resulting in either rupture or coagulation, depending
on whether the laser delivers pulses or a continuous beam
of light.
Pulsed Yellow Dye Lasers. The current fourth genera-
tion yellow dye laser (Perfecta Vbeam) has a wavelength
of 595 nm, handpieces that range from 5 to 12 mm,
adjustable power density and pulse duration, and a cryo-
gen spray that decreases the discomfort of the laser pulse
and provides a protective coolant effect that decreases
the risk of epidermal damage. Initially developed for the
treatment of port wine stains, this laser has applicability
for early proliferating hemangiomas, ulcerated heman-
giomas, and involuted hemangiomas with persistent
ectatic vessels. The laser light penetrates no more than
1 to 2 mm into the dermis; therefore, it is most effective
for fl at dermal hemangiomas. It may completely abort
small hemangiomas, signifi cantly shrink larger heman-
giomas, and decrease the pain of ulcerated hemangiomas.
FIGURE 9-4. NICH—noninvoluting congenital
hemangioma. These hemangiomas show no tendency to
regress and are characterized by bluish pigmentation and
small cutaneous ectatic vessels. Surgical excision is curative.
Parents should be advised that hemangiomas may show
rebound growth after 2 to 4 weeks, and a series of treat-
ments may be needed until permanent involution occurs.
The manufacturer supplies a cookbook recipe of sug-
gested treatment parameters. The practitioner should be
aware that overly aggressive laser treatment can actually
cause ulceration and scarring; therefore, conservative
power settings may be preferable during the initial laser
session.
Residual vascular pigmentation in the form of telangi-
ectasias and spider veins following complete involution
may respond to a series of pulsed yellow dye laser treat-
ments ( Figure 9-5 ). Sclerotherapy for larger residual ves-
sels can be combined with laser treatment for a synergistic
benefi t. Intravascular sclerosis followed immediately by
laser photocoagulation may signifi cantly obliterate persis-
tent vessels.
KTP and Nd:YAG Lasers. Continuous beam lasers such
as the Nd:YAG laser (1064 nm) and the KTP laser (532 nm)
(Laserscope) offer deeper penetration and vascular coagu-
lation, but risk cutaneous scarring from nonspecifi c heat
delivery to the tissues. Although fi beroptic insertion into
the hemangioma has been described for deep coagula-
tion, the amount of energy delivery is diffi cult to quantify
and the technique is heavily operator dependent.
General Techniques
The vast majority of hemangiomas are excised like nevi,
the circular or oval wound converted into an elliptical
(spindle) shape to avoid excessive dog ears at the ends of
the linear closure. Although the orientation of the ellipse
is readily apparent for most hemangiomas, areas like the
glabella are often unpredictable, and it is preferable to
excise these hemangioma as a circle and then manipu-
late the wound margins to determine the best direction
of closure.
Hemangiomas with both a central dermal and a larger
subcutaneous component can be debulked by incising
around the border of the dermal portion and then using
this opening to remove the deeper portion, analogous to a
skin-sparing mastectomy.
It is not always desirable to remove the entire heman-
gioma, particularly when the dermal part extends into cos-
metically important areas or complete excision will leave
a soft tissue defi ciency. Bipolar cautery may be extremely
useful when it is necessary to debulk a hemangioma with
active vessels.
Fully involuted hemangiomas with subcutaneous bulk
and minimal residual circulation are often amenable
to suction-assisted lipectomy when skin excision is not
necessary.
Although circular excision followed by purse-string
closure has been advocated to try to minimize the size of
the surgical scar, the prolonged pleating and potential need
for a second stage excision have made this approach less
attractive to most surgeons.

96 • CHAPTER 9
Region-specifi c Techniques
Periorbital. Similar to cosmetic blepharoplasty, redundant
upper or lower eyelid skin with subcutaneous bulk can be
excised using elliptical incisions, with care being taken to
avoid excessive skin excision. Extensive brow hemangiomas
may lead to scarring alopecia and loss of eyebrow hair, or
expanded skin with distorted brows and inadequate hair
density. Micrografting or scalp strip grafts can be used to
reconstruct missing eyebrows. Micropigmentation may
be preferable to hair transplantation to restore eyebrow
contour if one is unwilling to deal with the constant
trimming needed for transplanted scalp hair.
Signifi cant upper eyelid scarring may benefi t from a
large full-thickness skin graft applied to the entire aes-
thetic unit. A graft of suffi cient size and excellent color
match can be prefabricated by tissue expanding the post-
auricular donor site.
More complicated deformities of the lids such as true
ptosis or missing tissue are beyond the scope of this chap-
ter and may be better left to the expertise of ophthalmolo-
gists and oculoplastic surgeons.
Nasal. Nasal hemangiomas may occur anywhere along
the nose and can create signifi cant permanent deformities
of the nasal ala, alar base, nasal tip, nasal dorsum, radix,
and paranasal regions. Nasal tip hemangiomas commonly
act as tissue expanders that create redundant tip skin, splay
the alar cartilage, and leave behind bulky fat—the “Cyrano
deformity.” Steroid therapy may signifi cantly reduce the
severity of the deformity and is highly recommended to
facilitate later reconstruction. It may be exceedingly diffi -
cult to normalize the appearance of tip hemangiomas that
have created marked skin redundancy and dermal scarring.
Most parents are anxious to have the nasal tip debulked
prior to complete involution, so usually the procedure
requires cutting through active bleeding vessels.
The basic strategy in nasal tip reconstruction involves
removal of redundant skin, removal of excess heman-
gioma or redundant subcutaneous tissue, and centraliza-
tion of the alar domes. Although a wide variety of patterns
exist, a particularly useful technique is analogous to an
open rhinoplasty approach, except that the rim incisions
are externally placed to permit removal of redundant
skin ( Figure 9-6 ). The hemangioma is left attached to
FIGURE 9-5. The pulsed yellow dye laser can remove residual hemangioma
vessels in situations where complete excision is contraindicated. An ellipse of redundant
hemangioma is initially excised, orienting the scar along the natural wrinkle lines, followed
at a later date by Vbeam photocoagulation.
A
B

HEMANGIOMAS • 97
the overlying skin as the alar domes and lateral crura are
exposed. Clear monofi lament suture is used to centralize
the domes. Excess hemangioma tissue is excised but it
is essential to leave some attached to the overlying der-
mis for future subcutaneous padding. Aggressive removal
and combined with ongoing hemangioma regression can
leave thin atrophic skin with inadequate subcutaneous
tissue. Lastly redundant skin is excised, leaving a scar in
the naturally shaded underside of the nose.
If there is minimal excess skin, the hemangioma can
be debulked through internal nasal incisions, or via small
crescent-shaped nasal rim incisions ( Figure 9-7 ). Again, it
is important to avoid excessive debulking that may leave
long-term contour defi ciency.
Lip. Lip hemangiomas rarely regress satisfactorily, and
therefore the presence of a lip hemangioma is a relative
indication for steroid therapy. Many lip hemangiomas
are transmural, extending from the vermilion or der-
mis, through the orbicularis, to just beneath the mucosa.
Vermilion involvement frequently leads to ulceration
and subsequent scarring. Despite some improvement
with regression, the majority of patients are left with
excess bulk and signifi cant distortion. The vermilion bor-
der is often involved and obliterated by the expanding
hemangioma.
The approach to upper lip hemangiomas involves many
of the principles of cleft lip repair. Debulking procedures
create an iatrogenic cleft deformity, and accurate preop-
erative marking are essential to identify key anatomic land-
marks and determine the amount of tissue that needs to
be removed. Fully regressed hemangiomas that have left
excessive but stable soft tissue are easier to gauge than
hemangiomas in mid-involution, in which case the surgeon
must allow for some additional loss of volume through
natural regression. Furthermore suture lines are at risk for
partial dehiscence and delayed healing if one is approxi-
mating skin edges involved with hemangioma-infi ltrated
dermis.
Cupid’s bow marking are made as best as possible,
and if the hemangioma is unilateral, the uninvolved side
is used as a template. Unlike a cleft lip, the involved
side is too large, and therefore the philtral column and
lip height need to be shortened by removing triangu-
lar wedges of tissue. Commonly a full thickness verti-
cal wedge of upper lip is removed to decrease the width
of the involved side, followed by a multilayered closure
( Figure 9-8 ).
Hemangiomas involving only the vermilion may be
excised with vertically oriented ellipses, leaving scars that
mimic natural lip creases. A T-shaped closure, with the
crossbar hidden inside the transition to wet mucosa may
A
B
FIGURE 9-6. Modifi ed, open rhinoplasty approach to nasal tip hemangiomas. This
provides excellent exposure of the tip cartilage for centralization of the domes, facilitates
judicious tip debulking and excision of redundant skin, and leaves a favorable scar.

98 • CHAPTER 9
be useful for larger hemangiomas with excessive vermil-
ion. Hemangiomas along the free vermilion margin can be
excised with a horizontally oriented ellipse, to completely
hide the surgical scar.
Lower lip hemangiomas may also require wedge exci-
sions to shorten excessively wide lips. Alternatively, a
transverse ellipse is useful for hemangiomas that cross the
vermilion border, strategically placing the mucosa/skin
suture line along the obliterated vermilion border.
Ear. Signifi cant ear distortion and redundant skin can
result from periauricular hemangiomas, but it is impor-
tant to realize that hemangiomas in this area often regress
remarkably, leaving only atrophic skin. It may be advisable
to wait until mid to late involution to avoid overcorrection
and defi cient soft tissue bulk, particularly with the lobule.
Staged excision may be useful to palliate a severe defor-
mity, postponing the fi nal stage until after complete invo-
lution. A protruding or lop ear deformity from a bulky
postauricular hemangioma may warrant early excision and
conservative splinting to prevent a permanent deformity.
Cheek/Parotid. Cheek hemangiomas may occur in the
dermis and/or subcutaneous layer only, or they may com-
monly involve the parotid gland. Bilateral parotid involve-
ment is usually an indication for steroid suppression to
avoid external ear canal obstruction and hearing prob-
lems. Hemangiomas with both a dermal and a subcutane-
ous component will often leave a patch of atrophic scar
or redundant skin and subcutaneous fat, and therefore the
use of systemic or intralesional steroids may be justifi ed.
Purely subcutaneous or intraparotid hemangiomas of small
to moderate size, without a skin component, may regress
completely with excellent cosmetic results. Conservative
observation is recommended and a surgical scar can usu-
ally be avoided.
Large parotid or subcutaneous hemangiomas may act as
tissue expanders and create redundant skin. The potential
FIGURE 9-7. Similar to a bilateral cleft nasal repair, hemangiomas with limited
skin redundancy can be debulked using crescent-shaped rim incisions. The splayed tip
cartilage can then be sutured together to improve tip projection.
A
B

HEMANGIOMAS • 99
benefi t of steroid suppression should be weighed against
the risks of steroid therapy. Often a unilateral facelift
approach can address the problem of redundant cheek
skin, but only if the skin envelope exceeds the expected
volume of the growing face.
Medially and centrally located cheek hemangiomas can
be excised at the expense of obvious linear scars. Parents
and patients may be advised to postpone surgery until it
is certain that the hemangioma is going to leave a contour
deformity that is more noticeable than the surgical scar.
Trying to strategically place the scar along the nasolabial
fold may be useful for medially located hemangiomas.
Large, relatively fl at cheek hemangiomas with textural
irregularities may be better candidates for laser skin resur-
facing or dermabrasion than surgical excision. If excision is
felt to be the best option, staged excision may be prefer-
able to a single procedure that may result in excessive scar
widening due to a tight closure.
Tissue expansion may be useful to replace large areas
of dermal scar, particularly in children with extensive
cervico-facial hemangiomas ( Figure 9-9 ). The technique is
similar to burn scar reconstruction. Pre-expanded rotation
fl aps may offer more creative options than direct excision.
As with tissue expansion for burns or giant congenital nevi,
all the risks and benefi ts of tissue expanders must be dis-
cussed with the parents.
COMPLICATIONS: AVOIDANCE
AND MANAGEMENT
The greatest controversy in the surgical treatment of
hemangiomas relates to the timing of the procedure. Small
hemangiomas can be safely removed at any age with mini-
mal risk of bleeding, but judgment must be exercised to
decide which hemangiomas may spontaneously resolve
without needing excision. Larger hemangiomas may risk
perioperative hemorrhage, nerve injury, and wound heal-
ing complications, particularly in the case of large facial
hemangiomas that cannot be completely excised. Overly
anxious parents and overconfi dent surgeons may be
doing their young patients a disservice by operating too
early. Over-resection may result in soft tissue defi ciency.
Hemangiomas of the breast should generally be followed
conservatively in girls to avoid injury to the developing
breast bud.
From a medicolegal standpoint, parents must be advised
of all treatment options, whether they be medical or sur-
gical, and unrealistic expectations must be discouraged.
Facial hemangiomas commonly leave permanent defor-
mities despite the best efforts of treating pediatricians,
dermatologists, and surgeons, and parents should never be
admonished or led to believe they did not offer their chil-
dren the standard of care.
FIGURE 9-8. Redundant upper lip tissue often requires a full-thickness wedge
excision. The closure pattern is analogous to a cleft lip repair, with strategic cuts
designed to reestablish symmetry.
A
B

100 • CHAPTER 9
SUGGESTED READING
Frieden IJ, Haggstrom A, Drolet BA, et al. Infantile hemangiomas:
current knowledge, future directions: proceedings of a research
workshop on infantile hemangiomas. Pediatr Dermatol . 2005;
22(5):383–406.
Lawley LP, Siegfried E, Todd JL. Propranolol treatment for
hemangioma of infancy: risks and recommendations. Pediatr
Dermatol . 2009;26(5):610–614.
Marler JJ, Mulliken JB. Current management of hemangiomas
and vascular malformations. Clin Plast Surg . 2005;32(1):
99–116.
Sans V, Dumas de la Roque E, Berge J, et al. Propranolol for
severe infantile hemangiomas: follow-up report. Pediatrics.
2009;124:e423–e431.
FIGURE 9-9. Extensive cervico-facial scarring from
a large ulcerated hemangioma. Bilateral cheek expanders
provide good quality skin fl aps for lower facial skin
replacement.
C
A
B

10
Burn Reconstruction
Brian M. Parrett, MD / Julian J. Pribaz, MD
c h a p t e r
PATIENT EVALUATION AND SELECTION
The goal of burn reconstruction is restoration of function
and appearance. Reconstruction is aimed at the correction
of tissue defi ciency and deformity manifested as contrac-
tures, hypertrophic scarring, or exposed structures.
A single patient will often have multiple needs and their
chief complaints and deformities should be analyzed, not-
ing damaged tissue as well as unburned tissue. Functional
reconstruction is the priority followed by aesthetic recon-
struction. Procedures should be divided into urgent,
essential, or desired. Urgent procedures include eyelid con-
tracture release and coverage of exposed bone or tendons.
Essential procedures restore function. Desirable procedures
improve appearance. In general, nonurgent reconstruction
should be delayed for more than 1 year to allow scar matu-
ration and maximization of conservative management.
If the patient can tolerate a procedure medically and
emotionally, there are few, if any, contraindications to
burn reconstruction. Reconstruction should continue until
the patient is satisfi ed or the surgeon has no procedures
left to offer that can improve the patient’s appearance or
function.
PATIENT PREPARATION
Patient preparation begins with the prioritization of
reconstructive needs. A set of carefully planned proce-
dures, often occurring over many years, should be devel-
oped; proper sequence is important—don’t “burn bridges.”
Planning should involve all burn team members, patients,
and their families. Realistic goals and expectations must be
explained to the patient.
General anesthesia is appropriate for most procedures.
Donor sites should be identifi ed and rationed prior to the
procedure. Patients should be positioned to allow access to
the burnt areas and donor sites. Sterile technique is essen-
tial as the potential for infection is high.
Essential equipment will vary depending on the proce-
dure. Generally, a dermatome, electrocautery, bolster dress-
ing supplies, and splinting devices should be available.
SURGICAL TREATMENTS
Reconstructive Ladder
Surgical treatments are aimed at scar revision, contracture
release and coverage of exposed structures. The reconstruc-
tive ladder, considering the simplest procedure to achieve
the desired result before using more complex alternatives,
guides burn reconstruction. 1 It is not uncommon to utilize
every modality of the ladder in the reconstruction of mul-
tiple deformities in a single patient.
Direct Closure. Excision of a burn scar followed by pri-
mary closure is the fi rst option in scar revision. One should
orient scars parallel to relaxed skin tension lines and
approximate the dermis properly.
Z-plasty . Z-plasty lengthens the line of contracture
( Figure 10-1 ). In a simple z-plasty, three lines of equal
length are made with the central limb on the line of ten-
sion. The angles between the central and lateral lines should
be less than 90 degrees and equal to each other. The result-
ing two triangular fl aps are mobilized and transposed. The
fl ap bases will then lie along the line formerly occupied
by the central limb, resulting in a gain of length along the
scar. The greater the angle between the central and lateral
lines, the greater the length gain, but the more diffi cult the
transposition. Within these limits, a variety of z-plastys are
possible ( Figure 10-1 ).
Skin Grafts . Scar and contracture incisional or excisional
release with skin autograft coverage is the mainstay of burn
reconstruction. The release should penetrate through the
burn scar to the normal tissue underneath. An incision can

102 • CHAPTER 10
often release a contracture, whereas a full excision of a scar
may leave a better aesthetic result.
Donor sites should be of similar texture, thickness, and
color to the recipient site. The nearer the donor site to the
recipient, the better the match. Recipient site hemostasis
is essential. Full-thickness skin grafts (FTSG) are preferred
as they are less likely to contract and resemble normal skin
better than split-thickness skin grafts (STSG). However,
STSG, preferably at least 0.016 inches in thickness in adult
patients, are less limited by donor site availability and work
well in most situations. STSG may be applied as sheet or
mesh grafts harvested with a dermatome, but mesh grafts
generally have poorer aesthetic results. The graft should be
secured with chromic gut sutures or staples and a bolster
placed to immobilize the graft. A vacuum-assisted closure
device (eg, V.A.C.) can also be used as a bolster at a pres-
sure of 100 mm Hg. The bolster is usually removed after
4 to 5 days for a STSG and longer after a FTSG, refl ecting
the longer time for graft revascularization.
Tissue Expansion and Flaps . Tissue expanders are
implanted into a nearby subcutaneous tissue pocket 1 cm
D
D
C
C
C
C
B
B
A
A
FIGURE 10-1. (Upper Row) A simple z-plasty is designed
with diagonal lines ( AC and BD ) drawn at 60-degree angles
to the central limb, which is along the scar band ( AB ). The
triangular fl aps and the surrounding skin are undermined. The
fl aps are transposed and the length of the wound ( red line )
is increased. (Lower Row) A 5-fl ap z-plasty places two
opposing z-plastys along the same band, such that they are
mirror images. This results in a central fl ap ( C ), which inserts
additional tissue into the band. The fl aps of the z-plastys are
transposed and the central fl ap advanced in a Y-V fashion,
providing a gain in length.
larger than expander, and infl ated with saline over several
weeks, thus expanding the skin peripheral to the scar. The
length of the expander should be at least the length of the
defect to be covered and should result in an expanded dome
2.5 to 3.0 times the width of the defect. The expanded
skin is then used as a FTSG, a regional advancement fl ap
or a free fl ap.
Local and free fl aps are useful in select diffi cult wounds,
including extensive neck contractures, fl exural contractures,
and areas of exposed bone, tendon, or joints. Generally,
local fl aps are the fi rst option followed by free fl aps. Thin
fasciocutaneous fl aps are preferable in the head, neck, and
hand while musculocutaneous fl aps are often needed in
the lower extremity. Most fl aps will require future debulk-
ing for thinning.
Prefabrication and prelamination expand the fl ap
options in burn reconstruction and are utilized when con-
ventional fl aps are not feasible. 2 Prefabrication involves
the implantation of a vascular pedicle into a new territory
followed by a period of maturation of 6 to 8 weeks and
then fl ap transfer based on the new pedicle. Flaps may be
prefabricated at a distant site by transferring a vascular
pedicle to a more superfi cial location with a goal to cre-
ate thin fl aps; the fl ap is then transferred as a free fl ap in a
second operation. Alternatively, fl aps can be prefabricated
near the recipient site to match color and texture by the
local or free transfer of a vascular pedicle. The fl ap is then
rotated into the defect. This is especially useful for facial
reconstruction using the neck, scalp, supraclavicular and
retroauricular skin . Pretransfer expansion of free and pre-
fabricated fl aps is useful as it expands and thins donor skin,
often allowing primary closure of the donor site.
Head and Neck Burn Reconstruction
Excision and grafting is required in acute deep partial
thickness or full thickness facial burns and post-burn
facial scar reconstruction. 3 Excisions should be performed
as aesthetic units ( Figure 10-2 ) and grafts placed with
seams at the unit boundaries. 4 FTSGs are preferable, but
unmeshed thick STSGs may be used and should be 0.018
to 0.021 inches thick in adults and 0.008 to 0.012 inches
thick in children. For best color match, grafts and fl aps are
preferably harvested from nearby donor sites, above the
nipple line, including the neck, post- and preauricular area,
and supraclavicular area. Bolster dressings are applied to
provide compression to enhance graft take and prevent
hematomas.
The following are common cervicofacial burn deformi-
ties and reconstruction approaches. 1 – 3 , 5
Eyelid Contractures. Perform ectropion release in a
timely fashion to prevent any exposure keratopathy. Make
releasing incisions 2 mm from the ciliary margin and extend
lateral to the lateral canthus and medial to the medial can-
thus. Avoid injury to the orbicularis oculi muscle and do
not violate the supratarsal fold in the upper eyelid. Apply
a FTSG for the upper lid, which may be harvested from

BURN RECONSTRUCTION • 103
the contralateral upper lid or retroauricular area. A FTSG
is used for the lower lid, harvested from the retroauricu-
lar area. Apply grafts with the eyelids pulled by traction
sutures and fi x with bolsters. Perform upper and lower lids
as separate procedures.
Perioral Contractures . Perioral contractures are best
treated once the scars have matured. However, micros-
tomia causing diminished oral opening should be treated
early. A release of the oral commissure contracture can be
performed with a transverse skin incision with a T or Y lat-
eral extension followed by an intraoral mucosal advance-
ment fl ap.
For upper lip ectropion, an incisional release is made
along both nasolabial folds and the base of the nose. The
lip should fall back into its normal position and a FTSG is
applied. Reconstruction of the philtrum is best performed
with a composite cartilage and skin graft from the trian-
gular fossa of the ear. An everted lower lip release should
proceed with an incision at the vermilion scar junction
and must be released fully so that all of the teeth are cov-
ered. The underlying scar, especially from the labiomen-
tal crease, can often be used as a local turnover fl ap to
augment the chin and help accentuate the labiomental
crease. A FTSG is then placed to cover the entire subunit
( Figure 10-3 ). Chin implants can help reestablish chin pro-
jection in select cases.
Mustache and beard reconstruction can be performed
with a pedicled or free scalp fl ap based on the superfi cial
temporal artery. Another option is a prefabricated fl ap
in the hair-bearing scalp region with second-stage pedicle
transfer to the mustache and beard area.
Cheek Contractures and Hypertrophic Scarring .
Cheek contractures and scarring are treated by excision of
the entire cheek aesthetic unit. The defect is then closed
with a thick STSG, FTSG, or fl ap. Flap reconstruction
involves tissue expansion of the neck skin, which is then
FIGURE 10-2. Face reconstruction proceeds according
to the aesthetic units of the face shown in the image.
advanced to the cheek area. Additionally, neck skin can be
prefabricated, tissue expanded, and then advanced to the
cheek defect. Care must be taken to avoid lower eyelid
traction that can cause ectropion.
Nasal Scarring and Deformities. The nose is very com-
monly involved in facial burn injuries, with subtotal or
total loss of the tip being the most common injury. For
complex defects, use the subunit principles detailed by
Burget and Menick 6 and replace each layer to provide nasal
lining, skeletal support, and skin coverage. Nasal lining can
be obtained from local turn-in fl aps or mucosal bipedicle
or septal fl aps. Nasal support is provided by cartilage grafts.
Skin coverage is best obtained with a midline forehead fl ap
using the subunit principles.
For smaller defects in the upper two thirds of the nose a
FTSG may be used. Alar deformities may be reconstructed
with nasolabial fl aps after scar release and attention to ade-
quate nasal lining and support. Full release of an alar con-
tracture is performed with a curved incision in the region
of the alar crease extending medially to the nasal tip.
Nostril stenosis is corrected with z-plasty in minor
stenosis and with excisional or incisional release in more
signifi cant stenosis. A FTSG is sewn into the defect and a
nostril stent placed. Otherwise, an inferiorly based nasola-
bial fl ap is elevated and transferred to the defect.
If most of the nose is destroyed, total nasal reconstruc-
tion is the procedure of choice. This can be performed
with a forehead fl ap but, if unavailable, a radial forearm
free fl ap or dorsalis pedis free fl ap may be used. A classical
Tagliacozzi fl ap is rarely indicated today.
Scalp Defects and Alopecia. Small scalp defects
(,3 cm) are corrected with scar excision and primary clo-
sure. Medium-sized defects can be covered with local fl aps
from the temporal, parietal, or occipital scalp based on the
superfi cial temporal artery. Use a Doppler to map the ves-
sel territory, perform wide undermining and scoring of the
galea, then fl ap transposition.
Alopecia reconstruction should be delayed until wounds
are healed. Tissue expanders can reconstruct burnt areas
of up to 50% of the scalp. Expanders are placed in the
subgaleal plane of the hair-bearing scalp and expanded
weekly for 2 to 4 months. The scar is then excised and the
expanded tissue is advanced or rotated to fi ll the defect.
For extensive areas of alopecia, multiple-staged expansion
procedures are used ( Figure 10-4 ).
Cranial bone exposure requires a local scalp fl ap or a free
fl ap. A classical method of covering a small area of exposed
bone is Millard’s crane principle in which a scalp fl ap is
rotated into the defect and allowed to mature for 3 weeks. 1
The fl ap is raised and returned to its donor site, leaving tis-
sue over the calvarium. The scalp wound is then grafted.
Larger areas of exposed bone often require free fl ap cover-
age with musculocutaneous fl aps, such as latissimus dorsi
fl ap, or fasciocutaneous fl aps, such as the parascapular or
radial forearm fl ap. Later tissue expansion of the remaining
hair-bearing skin can be used with gradual excision of the

104 • CHAPTER 10
non-hair bearing fl ap. Residual non-hair bearing areas can
be treated with punch hair grafts.
Forehead Scarring. Forehead scars should be excised
and small defects closed primarily. If less than half of the
forehead is involved, tissue expanders may be placed under
the frontalis muscle and then fl aps advanced for coverage.
If a majority of the forehead is involved, excise the entire
aesthetic unit from the eyebrows to hairline and the outer
canthi bilaterally ( Figure 10-2 ). Resurface with a thick
STSG or FTSG that is oriented transversely and placed
symmetrically. Exposed bone requires free tissue transfer,
most often with a parascapular or radial forearm fl ap.
Eyebrow Defi cit. Eyebrows can be reconstructed with a
composite hair-bearing temporal scalp graft. Using a pat-
tern mimicking the opposite eyebrow, extend the incision
through the galea, trim excess fat and galea and insert into
the recipient site. This gives a thin, less dense eyebrow,
ideal for women. Hair growth can be unpredictable and
may need to be supplemented with micro- and mini-
grafting.
FIGURE 10-3. (Upper Left) A 28-year-old woman has hypertrophic scarring of lower
lip complex causing lip eversion. (Upper Right) The scar is excised as an entire aesthetic
unit. (Lower Left) An FTSG is placed with seams at the unit boundaries. (Lower Right)
One year later, there is proper mouth opening and no lip eversion.

BURN RECONSTRUCTION • 105
A temporal scalp fl ap based on the superfi cial temporal
artery may be used for men to give a fuller, wider eyebrow,
but hair growth can be excessive requiring frequent trim-
ming. To perform this fl ap, Doppler the branches, outline
the pedicle, and raise a wide strip of superfi cial temporalis
fascia with overlying scalp of the ipsilateral scalp. Tunnel
the fl ap subcutaneously to the eyebrow.
For partial eyebrow loss, micro- and mini-grafting with
occipital scalp hair can be performed. In expert hands,
this may in fact be the preferred method of eyebrow
reconstruction.
Neck Contractures. Neck contractures should be released
early. Z-plasty or local fl aps work well in isolated contrac-
tures of one third or less of the anterior neck with unburned
skin on each side of the contracture. If one third to two thirds
of the neck is involved and the adjacent skin is unburnt, tis-
sue expanders can be expanded above the platysma, allow-
ing local fl aps to be advanced after scar excision. If more
than two thirds of the neck is involved, incisional release or
preferentially complete excision of the scar should be per-
formed, including the platysma muscle if involved in the
scar. The resulting defect can be covered with a thick STSG,
which should be long, wide, harvested as a sheet, and placed
in a hammock-like fashion, extending well beyond the scar
on each lateral aspect of the neck. Alternatively, fl ap cover-
age may be performed. Pre-expanded, pedicle fl aps from
the infraclavicular area, the deltoid area and the trapezius
area have been described. Free tissue transfer employing
a parascapular fl ap, prefabricated thigh fl ap, or groin fl ap
may be performed ( Figure 10-5 ). Flaps tend to be bulky
and need future debulking to decrease blunting of cervi-
comental angle. Grafts require postoperative splinting and
compression.
Upper Extremity and Hand
Burn Reconstruction
Extremity contractures are released from proximal to dis-
tal. Linear contractures are corrected with z-plastys, and
broader contractures with release and skin grafting or fl ap
closure. Joint contractures are released with an incision in
line with the axis of rotation. The extent should be judged
on improvement in joint mobility. Stabilize bone and
joints in position of function; postreconstructive splinting
is crucial.
Hand reconstruction is performed under tourniquet
control. Proceed with incisional release of contractures
at each joint level and excisional release of hypertrophic
scars. Resurface hands with thin and pliable tissue. FTSGs
are preferred but thick STSGs may be used. If STSGs
are used, sheet grafts or 1 to 1.5 meshed grafts should be
placed followed by a compressive dressing. Sheet grafts
have a better aesthetic outcome and are preferred if there
are adequate donor sites.
Pedicle and free fl aps are important in upper extrem-
ity reconstruction. Local or regional fasciocutaneous fl aps,
even using previously burnt or grafted arm skin, are reli-
able for burn coverage as the fascia and vessels are usually
spared. 7
The most common upper extremity deformities and
their reconstruction are reviewed. 1 , 8
Exposed Bone/Joints/Tendons. The proximal interpha-
langeal (PIP) joint is the most commonly exposed joint.
The joint may be stabilized with an axial Kirschner wire
(K-wire), multiple moist dressing changes performed and
skin grafting performed when suffi cient granulation tissue
develops. In small areas of the fi ngers, local fl aps may be
FIGURE 10-4. (Left) A 30-year-old man has extensive burn alopecia. (Center) He
had serial expansions and excisions and is shown with an occipital scalp expander after
3 months of expansion. The region of scar alopecia is marked for excision. (Right) The
expanded hair-bearing skin is then rotated into the defect.

106 • CHAPTER 10
performed with proximally or distally based fasciocutane-
ous fl aps based on the digital or metacarpal arteries. Cross-
fi nger fl aps can also be used for dorsal or volar coverage.
More extensive bone exposure in the hand and arm
requires acute coverage with fl aps. Groin or abdominal
fl aps are the “workhorse” fl aps and require 2 to 3 weeks
of immobilization ( Figure 10-6 ). Local fl aps for the wrist
and hand include the proximally based digital or fi rst
metacarpal fl ap or the distally based reverse radial, reverse
ulnar, or reverse posterior interosseous fl aps ( Figure 10-7 ).
Otherwise, a free tissue transfer is required using the radial
forearm (opposite arm and covers entire dorsum of hand
and digits), dorsalis pedis (sensate if includes superfi cial
peroneal cutaneous nerve), temporoparietal fascia (cov-
ered with a STSG), or scapular or lateral arm fl aps.
Axillary Adduction Contractures. Solitary scar bands in
the axilla are released with z-plasty. If only the axillary folds
are involved, an incisional release, followed by closure with
a thick STSG, can be performed. If the contractures involve
the axillary folds and the axillary dome, one can perform
an incisional release involving the entire axis of rotation of
the shoulder. A pedicled parascapular fl ap, island scapu-
lar fl ap or pedicled latissimus fl ap is then rotated into the
defect ( Figure 10-8 ). Postoperatively, abduction splints are
applied, especially if skin grafts are used.
Elbow Contractures. Elbow contractures are incision-
ally released and resurfaced with a thick STSG. When the
release results in exposed bone or when there is olecranon
ulceration, several fl aps are available for closure, the most
common being the reverse lateral arm fl ap ( Figure 10-9 ).
Other local fl aps that may be used include the proximally
based radial, ulnar, or posterior interosseous fl aps. The
elbow is splinted in extension with a volar splint.
Dorsal Hand Contractures and Hypertrophic Scarring.
Linear contractures with adequate lateral tissue laxity are
corrected with z-plastys. Otherwise, an incisional release is
made proximal to metacarpophalangeal (MCP) joints. If
FIGURE 10-5. (Upper Left) A 30-year-old male has extensive neck contractures.
(Upper Center) A prefabricated thigh fl ap is designed. The descending branches of the
lateral femoral circumfl ex vessels and a small amount of attached muscle and fascia
are transferred superfi cially over a tissue expander. (Upper Right) After 8 weeks of
maturation, a Doppler exam is performed and a thin fl ap is raised based on the implanted
pedicle. (Lower Left) After excision of the contracture, the prefabricated free fl ap is
transferred to the neck. (Lower Right) The patient is shown 3 years later after debulking.

BURN RECONSTRUCTION • 107
FIGURE 10-6. (Upper Left) A 53-year-old man with bilateral hand burns with
exposed bone and tendons shown in the right hand. (Upper Right) Groin fl aps are
centered over the superfi cial circumfl ex iliac arteries, which are traced laterally by Doppler
past the anterior superior iliac spine. (Lower Left) The fl aps are inset into the hand defects
with direct donor site closure and tubing of the attached pedicle. The pedicle is divided
2 to 3 weeks later. (Lower Right) The right hand is shown 1 year later after debulking to
create web spaces.
the entire dorsal surface is involved, release extends from
the medial to lateral joint axis. The release should result
in a resistance-free complete range of motion of the MCP
joints. The defect is closed with an FTSG, and the hand
is splinted in the intrinsic-plus safe position. Alternatively,
postgrafting stabilization in a full-fl ex hand position with
fl exion at the distal interphalangeal and PIP and MCP
joints may be useful. 1 Extensive dorsal scarring should be
excised completely, grafted, and splinted.
Digital Flexion Contractures. Digital fl exion contrac-
tures are treated with z-plasty if linear ( Figure 10-10 ) or, if
broader, with a transverse incision and a FTSG or local fl ap.
Fixed or immobile joints may need release of periarticular
structures, including the volar plate, collateral ligaments,
or joint capsule. The fl ap option of choice for reconstruc-
tion is the local side-fi nger fasciocutaneous fl ap, based on
the digital and metacarpal vessels, and can be proximally
or distally based. Cross-fi nger fl aps can also be used. When
FIGURE 10-7. (Left) There is extensor tendon exposure of the thumb after a burn
injury. (Center) A distally based reverse radial artery fl ap is marked for reconstruction.
(Right) The result is shown 6 months later.

108 • CHAPTER 10
FIGURE 10-8. (Left) A 52-year-old woman has dense axillary contractures and is
marked for a pedicled parascapular fl ap. (Center) The contracture is incisionally released
and the fl ap is rotated into the defect. (Right) One year later, she has improved shoulder
abduction.
using arterialized side-fi nger fl aps, a Doppler exam is per-
formed to ensure patency of both digital arteries, and the
fl ap is raised with the artery in the fl ap. Fingers are splinted
in the extended position.
Web Contractures and Syndactyly. Web contractures
manifest as dorsal hooding over the web space or syndac-
tyly. Dorsal hooding is corrected with z-plastys or inci-
sional release with a FTSG. Syndactyly is corrected with
web space release and web reconstruction with a dorsal
pedicle fl ap. The classic dorsal fl ap is proximally based
and extends from the MCP joint up to two thirds the
length of the proximal phalanx. The subcutaneous fat,
fascia, and dorsal metacarpal vessels are included in the
fl ap. The fl ap is inset with a 45-degree incline in the dor-
sal-to-volar direction, with the distal edge ending at the
midshaft of the proximal phalanx. The fl ap should pro-
vide a wide web space, and residual defects on the sepa-
rated fi ngers are covered with an FTSG. Postoperatively,
compression gloves with web space conformers are
worn.
First web space contractures cause thumb adduction and
are released with a fi ve or four fl ap z-plasty ( Figure 10-10 ).
Alternatively, after release, the defect may be covered with
a thick STSG, FTSG, or a local fl ap. Postoperative splinting
keeps the thumb in extension.
Boutonniere Deformity. Boutonniere deformity at the PIP
joint is common and reconstruction of the central extensor
tendon slip and cutaneous coverage with a local fl ap can be
pursued. However, if the deformity is complicated and the
joint is stiff, the repair may be performed with fusion of
the PIP joint at a 25- to 50-degree angle, increasing toward
the more ulnar digits.
Breast Burn Reconstruction
Chest contractures can restrict breast enlargement during
puberty, causing asymmetry and displacing the nipple are-
olar complex. Reconstruction is performed when normal
breast growth is affected. Nipple-areola reconstruction
is delayed until breast mound reconstruction is com-
plete. Use the opposite unburned breast as a model for
reconstruction. 9
Breast Contractures. Contracture release is obtained by
incision or excision of the restricting burn scar. Care must
be taken to not disturb the breast bud in young girls, as
this can lead to severe breast hypoplasia. A wide release of
the inframammary fold is performed down to the muscle
fascia. Occasionally, release will require incisions superior
to the breast mound, around the areola, or between the
breasts. STSG are applied to the defect and bolsters placed.
Occasionally, the entire breast bud is destroyed and needs
to be reconstructed with a regional musculocutaneous fl ap
such as a latissimus dorsi fl ap with a tissue expander or a
transverse rectus abdominus myocutaneous fl ap. Balancing
procedures are often required on the opposite breast.
Nipple-Areola Deformities. For a malpositioned nipple-
areola complex, the scar is released and the nipple-areola
complex advanced as a fl ap into a better, more symmetrical

BURN RECONSTRUCTION • 109
FIGURE 10-9. (Upper Left) A 45-year-old man has a
burn contracture of his elbow limiting fl exion. He is marked
for a distally based reverse lateral arm fasciocutaneous fl ap
using previously grafted skin. (Upper Right) The contracture
is excised resulting in exposed joint and bone. (Lower Left)
A lateral arm fl ap is rotated into the defect. (Lower Right)
He has appropriate elbow fl exion and wound coverage
1 year later.
position. An STSG may be needed in the resulting defect. If
the nipple is lost, it will require reconstruction as after mas-
tectomy, using a C-V fl ap or a skate fl ap. For nipple recon-
struction on a grafted breast, tent up a circle of skin and
place a tight FTSG around the base. Composite grafts, such
as postauricular cartilage or toe pulp, can also be used to
construct a nipple. It is best to make the nipple larger than
the opposite side, allowing for long-term fl attening. The are-
ola is reconstructed with an FTSG taken from the opposite
breast areola with an outer “donut” technique or taken from
hairless groin skin. Alternatively, the areola may simply be
tattooed once the nipple reconstruction has healed.
Perineum Burn Reconstruction
Scar contracture is common after perineal burns. Surgical
reconstruction of the external genitalia is complex, and
often requires multiple specialized procedures.
Perineal Contractures. Isolated perineal scar bands are
released with multiple z-plastys with the patient placed
in the lithotomy position. More diffuse scars require inci-
sional or excisional releases followed by the placement
of meshed STSG, which are more pliable and better fi t
the perineal contour. Local fl aps from the perineal area or
medial thigh can also be used.
Lower Extremity Burn Reconstruction
Meshed STSGs are the mainstay of treatment in the lower
extremity. However, narrow scar bands can be divided and
lengthened with z-plastys. Immobilization is needed post-
reconstruction, with the long-term use of postoperative
splinting.
Fourth-degree
Burns. Exposed
tendon
or
bone
requires fl ap closure. The gastrocnemius and soleus mus-
cle fl aps can be used for knee or proximal tibial defects.
Common free fl aps used for exposed tibia include the
rectus abdominus, latissimus dorsi, or parascapular fl ap.
Small ankle and foot defects can be closed with a broad
array of local fl aps. The abductor hallucis, abductor digiti
minimi, and extensor digitorum brevis muscle fl aps can
cover small proximal dorsal defects. The abductor hal-
lucis, abductor digiti minimi, and fl exor digitorum brevis
muscle fl aps can cover malleolar and weight-bearing heel
defects. The lateral calcaneal artery fl ap is useful for heel
and Achilles tendon defects. The dorsalis pedis artery fas-
ciocutaneous or myofascial fl ap with an STSG can recon-
struct ankle defects and, when used in a reverse fashion,
distal foot defects.
Extensive exposed foot structures require free fl ap cov-
erage. This can be accomplished with a radial forearm or
temporoparietal fascia fl ap to the dorsal foot. For plantar
defects, a muscle fl ap, such as the latissimus dorsi, gracilis,
or rectus abdominus fl ap, or a fasciocutaneous fl ap, such as
the tensor fascia lata fl ap, can be used.
The V.A.C. device and tibial burr holes are options in
patients who cannot undergo fl ap closure. The V.A.C. is
placed at 125 mm Hg and changed every 2 to 3 days. If the
wound contracts and granulates enough, it can be closed
with delayed primary closure or grafting. Tibial burr holes
may help expedite granulation tissue formation by burr-
ing through the outer table of bone. Dressing changes or
the V.A.C. should follow this. If these interventions fail
or result in a poorly functional leg, amputation may be
the best option. Attempts should be made to maintain as
much limb length as possible.
Foot Contractures. Dorsal foot contractures or hyper-
trophic scarring are released with excision of the scar tis-
sue and coverage with a STSG. Plantar contractures can be
incisionally released and an STSG placed under a bolster.
Postoperative immobilization is necessary and may require
K-wire fi xation. With extensive and severe contractures,
free fl ap transfer may be required.

110 • CHAPTER 10
Popliteal Flexion Contractures. Popliteal contractures
require incisional release, avoiding injury to the underlying
neurovascular structures of the popliteal fossa. The defect
is closed with an STSG or a fl ap if there are exposed vessels
or tendons. If adjacent tissue is viable, a transposition fl ap
can be created adjacent to the defect with the length of the
fl ap equal to the length of the scar release and rotated into
the defect. Postoperative immobilization in knee extension
is required.
COMPLICATIONS
The most common adverse outcomes of burn reconstruc-
tion are infection, failed grafting and recurrent scarring.
Strict sterile technique is necessary. Tissue expanders
are prone to infection and should only be used in healed
wounds and removed at any sign of infection. Expansion
works best in the head, neck, and torso; but overall have
high complication rates due to infection and expander
extrusion or exposure.
The fi nal functional results of grafts are largely deter-
mined by postoperative care, including compression gar-
ments, facemasks, and extremity splints. Grafts should be
immobilized and compressed with bolster dressings. After
bolster removal, the grafts should be protected for an
extended period of time.
Customized pressure garments should be applied as
soon as possible after wound healing and continued for at
least 6 months to control scar maturation and minimize
hypertrophic scarring. Compression and splinting for the
face is important for several months after grafting and
is achieved with custom fi t pressure garments or masks.
Splints should be applied to extremities as well as the
neck. Occupational or physical therapy programs should
be initiated to maintain mobilization and prevent con-
tractures after reconstruction. Postoperative contractures
will reoccur if physical therapy and joint splinting are not
pursued.
OUTCOMES ASSESSMENT
Successful outcomes are dependent on forming a realistic
long-term reconstructive plan that involves the patient,
surgeon, therapists, and other health care providers.
Psychological response to burn scars or contractures is
a main factor in infl uencing the functional rehabilita-
tion and psychological state of burn patients. Thus,
reconstruction of these deformities can have a profound
impact on the psychological well-being of patients,
allowing future social reintegration and improvement in
function.
FIGURE 10-10. (Upper Left) A long linear contracture of the fi fth digit is designed
with multiple z-plastys in series along the contracture. (Upper Center and Right) The
fl aps are incised and transposed with successful lengthening of the contracture. (Lower
Left) A 5-fl ap z-plasty is designed over a fi rst web space contracture. (Lower Right) Flap
transposition lengthens the web space.

BURN RECONSTRUCTION • 111
REFERENCES
1. Herndon DN. Total Burn Care. London: Saunders; 2002.
2. Pribaz JJ, Fine N, Orgill DP. Flap prefabrication in the head
and neck: a 10-year experience. Plast Reconstr Surg. 1999;
103:808.
3. Klein MB, Moore ML, Costa B, et al. Primer on the manage-
ment of face burns at the University of Washington. J Burn
Care Rehabil . 2005;26:6.
4. Gonzalez-Ulloa M. Restoration of the face covering by
means of selected skin in regional aesthetic units. Br J Plast
Surg. 1956;9:212.
5. Achauer BM. Reconstructing the burned face. Clin Plast
Surg 1992;19:623.
6. Burget GC, Menick F. The subunit principle in nasal recon-
struction. Plast Reconstr Surg. 1985;76:239.
7. Pribaz JJ, Pelham FR. Use of previously burned skin in local
fasciocutaneous fl aps for upper extremity reconstruction.
Ann Plast Surg. 1994;33:272.
8. Smith MA, Munster AM, Spence RJ. Burns of the hand and
upper limb–a review. Burns. 1998;24:493.
9. MacLennan SE, Wells MD, Neale HW. Reconstruction of
the burned breast. Clin Plast Surg. 2000;27:113.

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c h a p t e r 11
Head and Neck Embryology
Geoffrey H. Sperber, BDS, MSc, PhD, FICD
INTRODUCTION
The signifi cance of head and neck embryology to surgi-
cal practice is based on a required understanding of the
underlying developmental phenomena leading to normal
craniofacial morphology. This, in turn, provides insights
into how maldevelopment leads to dysmorphology that
the clinician is called upon to analyze, treat, and prognos-
ticate its occurrence. The essence of treating any malady is
to fully understand its etiology and eliminate the causative
factors. The emergence of molecular genetics and advances
in gene identifi cation aided by the imaging capabilities of
electron, scanning, transmission, fl uorescence, and confo-
cal laser microscopy, together with immunohistochem-
istry, polymerase chain reaction (PCR) technology, and
stem cell and experimental cloning have revolutionized
our capabilities for investigating developmental events.
Molecular diagnostics have begun to revolutionize medi-
cine. Identifi cation of biomarkers allows the characteriza-
tion of the molecular basis of dysmorphology and possible
therapeutic intervention. Understanding these technolo-
gies is changing embryology from a descriptive science
into a predictive science, with the potential for control of
its mechanisms. 1
The craniofacial complex, subserving the elements of
the sensory organs of sight, hearing, olfaction, and taste,
while providing a muscularized masticatory apparatus and
a bony protective braincase is a composite of numerous
disparate evolutionary components melded into an essen-
tial functioning appendage to the body. This complexity of
diverse purposes contained in the head is refl ected in its
complicated embryology.
The emerging fi elds of regenerative medicine and
potential stem cell therapy are rooted in an understand-
ing of the mechanisms of embryogenesis and developmen-
tal differentiation of the initial unicellular totipotential
zygote resulting from the union of the male and female
gametes. Furthermore, the prenatal imaging capabilities of
embryoscopy, fetoscopy, and ultrasonography have accel-
erated gestational development into the fi eld of concern
to the clinician. Chorionic villus sampling and amniocen-
tesis provide advance information on the chromosomal
and genetic status of the developing fetus and any pos-
sible abnormalities, thus allowing for possible therapeutic
intervention.
EARLY EMBRYOLOGY
Embryogenesis is a series of cascading events transform-
ing the coded instructions contained in the genome of the
conceptus into proteins, tissues, and organs in an ordered
manner ( Figure 11-1 ). 2 The instructions of regulatory
genes and signaling factors shape an indeterminate mass
of cells into complex morphologies. The combination of
molecular biology with classical embryology results in the
production of an unfolding embryo turning into a fetus
at 8 weeks postconception and ultimately into an infant
( Figure 11-2 ).
The embryonic disk that develops from cellular dif-
ferentiation consists of the primary germ layers, ecto-
derm, endoderm, and mesoderm, all of which contribute
to the craniofacial structures. The intermediate meso-
dermal germ layer is defi cient in two locations in the
trilaminar disk—at the prechordal plate cranially and at
the cloacal plate caudally—demarcating the sites of the
future entrance and exit of the gut ( Figure 11-3 ). The
persistent contact of ectoderm and endoderm at these
two locations determines the dissolution of these two
plates, which are denoted as the oropharyngeal and cloa-
cal membranes, respectively, and perforate at the mouth
and anus. The oropharyngeal membrane sinks into a
central depression, the stomodeum, around which the
future face is built.

114 • CHAPTER 11
NEURAL CREST TISSUE
(ECTOMESENCHYME)
The neural crest is a pluripotent cell population derived from
the crests of the neural folds that form the neural tube and
brain. During neural tube closure (failure of which leads to
neural tube defects), induction by differential tissue inter-
actions produces an epithelial-mesenchymal transformation
resulting in ectomesenchyme that gives rise to a diverse
variety of tissues ( Table 11-1 ). Migration, proliferation, and
differentiation of neural crest cells provide the prominences
of the embryonic face and neck .3 The segmental pattern of
migrating neural crest is foreshadowed by hindbrain neu-
romeres, termed rhombomeres, that are determined by the
HOX genes ( Figure 11-4 ). It is the brain underlying the
future face that is a key component of cephalogenesis. 4
Defi ciencies in neural crest migration, proliferation, or
differentiation account for a wide range of craniofacial
malformations, the neurocristopathies, manifested in a
variety of syndromes. Abnormalities in form or function of
neural crest cells range from von Recklinghausen neurofi -
bromatosis through acoustic neuroma (neurofi broma) to
DiGeorge and Klein-Waardenburg syndromes.
CRANIOFACIAL DEVELOPMENT
Most of the skeletal and connective tissues of the face
and pharyngeal arch apparatus are dependent on neural
crest tissue migrating as ectomesenchyme into the ventral
regions of the future skull, face, and neck ( Figure 11-5 ). 5 , 6
Any defect in the quantity and quality of migrating ecto-
mesenchyme manifests itself in nearly every congenital
anomaly observed clinically, ranging from major holopros-
encephaly to the most minor clefts of the lips and dimples
in the cheeks. 7 – 11
During the critical days 21 to 31 of development, the
central stomodeum is surrounded by fi ve facial promi-
nences formed by underlying neural crest migration and
proliferation displaying selective regional characteristics.
GENETIC FACTORS
(MALFORMATIONS)
Transcription
Genome Imprinting
Cell Adhesion, Repulsion;
Migration;
Proliferation, Apoptosis
Signal
Transductions
Introduction
Organization
Patterning
Positioning
INDUCTORS
Growth Factors; Morphogens
Receptors
(DISRUPTIONS)
(Italicized parenthesized type indicates defective development)
(DYSPLASIAS)
CYTOPLASMIC FACTORS
ENVIRONMENTAL FACTORS
RNA organelle activity
Spatial Position of Cells
Matabolism. Hormones
(Transcription defects)
(Teratogens)
Genotype
Diploid Chromosomes
Genes
DNA activation &
inactivation
(aneuploidies, inversions,
translocations, deletions)
(Variable penetrance,
expressivity, pleiotropy,
heterogeneity, mutations)
(Mistiming of reactions)
(Failed receptor interactions)
CYTODIFFERENTIATION
Development of cell types:
ecto-, meso-, endoderm
(Immaturity; Necrosis)
(DEFORMATIONS)
MORPHODIFFERENTIATION
Development of “fields”,
organs
and system - Phenotype
(Misplaced organizational
fields; Dysmorphology)
HISTODIFFERENTIATION
Development of tissue types;
epithelia,: muscle, nerve,
connective tissue
(Hyper-and Hypoplasias)
FIGURE 11-1. Schema of embryogenesis. (Reprinted from Sperber GH. Embryology of the
head and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)

HEAD AND NECK EMBRYOLOGY • 115
A
A Spermatozoon penetrating ovum
to form zygote
E Primary germ layers forming in
germ disc
F Somite stage embryo
G Post-somite stage embryo
H Full term fetus
B Morula stage of blastula
C Blastocyst with inner cell mass
D Fetal membranes in chorion
B
3 days
18 days
Cut edge of
amnion (ectoderm)
Prechordal plate
Prechordal plate
Yolk sac (endoderm)
Head
Cloacal plate
Body stalk
(umbilicus)
Otic placode
Lens placode
Branchial arches
Heart
Heart
24 days
28 days
9 months
Inner cell mass
Trophoblast
Amnion
Germ disc
Yolk sac
Chorion
6 days
14 days
C
D
E
F
G
H
FIGURE 11-2. Diagrammatic synopsis of embryogenesis. (Reprinted from Sperber GH.
Embryology of the head and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott;
1990:1–36.)
Cloacal plate
Primitive gut
Body stalk
(Umbilicus)
Cut edge of
amnion
Occipital
somites
Neural folds
Prechordal
plate
Yolk sac
FIGURE 11-3. Diagram of 21-day-old embryonic disk
elevating neural folds, encompassed by fetal membranes.
(Reprinted from Sperber GH. Embryology of the head and neck.
In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott;
1990:1–36.)
Ectomesenchyme from the mesencephalic region contrib-
utes to the leptomeninges and the frontal and nasal bones,
whereas the parietal bones are derived from mesoderm.
The interaction at the boundaries between these dissimi-
lar cell populations may be infl uential in growth control,
potentially accounting for craniosynostotic syndromes.
Neural crest cells around the midbrain form part of
the trigeminal, facial, glossopharyngeal, vestibulcochlear,
and vagal nerve ganglia, joined by neuroblasts from the
maxillomandibular placodes. Lateral evaginations of
the forebrain, the optic vesicles, induce lens placodes in
the surface ectoderm, around which neural crest cells
migrate to form the scleral and choroid optic coats and
the facial frontonasal prominence innervated by the oph-
thalmic division of the trigeminal nerve. More caudally
located migrating neural crest cells encounter pharyngeal
endoderm, with which they interact to form the fi ve pha-
ryngeal arches. Springing from the fi rst pharyngeal arches

116 • CHAPTER 11
bilaterally are the maxillary and mandibular prominences
(innervated by the respective branches of the trigeminal
nerve); these prominences form the superolateral and
inferior boundaries to the wide stomodeal chamber. The
median superior boundary is the frontonasal prominence
( Figure 11-6 ). On the inferolateral corners of the fronto-
nasal prominence there develop bilateral nasal placodes,
precursors of olfactory epithelium. The nasal placodes
sink into the face to form the nasal pits that become the
anterior nares consequent to the elevation of the horse-
shoe-shaped medial and lateral nasal prominences. The
posterior aspect of each nasal pit is initially separated
from the oral cavity by the oronasal (bucconasal) mem-
brane, which disintegrates by the end of the fi fth week to
form the posterior choanae.
Elevation of the lateral nasal prominences creates the
alae of the nose. Defects of the medial nasal prominences
may produce arhinia, or a bifi d nose, or contribute to
clefts of the upper lip. The medial tip of the maxillary
prominences that contribute to the upper lip makes con-
tact with the medial nasal prominence separated by an
intervening epithelial “nasal fi n” that normally degener-
ates, allowing mesenchymal merging. Persistence of the
nasal fi n contributes to clefting of the upper lip and ante-
rior palate. 12
Clefting of the upper lip is one of the most frequent
of all congenital anomalies; its unilateral occurrence (usu-
ally on the left) varies among different racial groupings,
indicating its inherited character: it is highest in inci-
dence in Mongoloid peoples, intermediate in frequency
among whites and least in blacks, varying from 1:500 to
1:2000 births. The degree of clefting varies enormously;
the anomaly is rarely median, indicative of a major holo-
prosencephaly syndrome. Lip clefts may be coincidentally
associated with cleft palate, which is inherited separately.
The lower lip is rarely defective but, if so, it is clefted in
the midline ( Figure 11-7 ).
THE MOUTH
The primitive wide stomodeal aperture is reduced by
migrating mesenchyme fusing the maxillary and mandibu-
lar prominences to form the commissures of the defi nitive
mouth. Inadequate fusion results in macrostomia (uni- or
bilateral), a form of facial clefting, while excessive fusion
produces microstomia or astomia ( Figure 11-7 ).
The initial shallow depression of the stomodeum
becomes the familiarly deep oral cavity by forward growth
of the surrounding facial prominences. The stomodeum
becomes the common oronasopharyngeal chamber and
entrance to the gut by disintegration of the dividing
oropharyngeal membrane, providing continuity between
the mouth and pharynx. Rare persistence of the mem-
brane results in oropharyngeal atresia.
The foregut, extending from the oropharyngeal mem-
brane gives rise to the esophagus and the laryngotracheal
groove, generating the larynx and trachea ( Figure 11-8 ). 13
TABLE 11-1. Derivatives of Neural
Crest Cells
Connective Tissues
Ectomesenchyme of facial prominences and pharyngeal
arches
Bones and cartilages of facial and visceral skeleton
(basicranial and pharyngeal arch cartilages); portions
of ear ossicles
Dermis of face and ventral aspect of neck
Stroma of salivary, thymus, thyroid, parathyroid and
pituitary glands
Corneal mesenchyme
Sclera and choroid optic coats
Blood vessel walls (excepting endothelium); aortic arch
arteries
Dental papilla (dentin); portion of periodontal ligament;
cementum
Muscle Tissues
Ciliary muscles
Connective tissues, sheaths of pharyngeal
arch muscles (masticatory, facial, ossicular,
faucial, laryngeal) combined with mesodermal
components
Nervous Tissues
Supporting tissues
Leptomeninges of prosencephalon and part of
mesencephalon
Glia
Schwann sheath cells
Sensory ganglia
Bipolar neurons of vestibular and cochlear ganglia of
vestibulocochlear nerve
Autonomic ganglia
Spinal dorsal root ganglia
Sensory ganglia (in part) of trigeminal, facial
(geniculate), glossopharyngeal (otic and superior)
and vagal (jugular) nerves
Autonomic nervous system
Sympathetic ganglia and plexuses
Parasympathetic ganglia (ciliary, ethmoid,
sphenopalatine, submandibular, enteric
system)
Endocrine Tissues
Enteric ganglia
Adenohypophysis mesenchyme
Adrenomedullary cells and adrenergic
paraganglia: chromaffi n cells
(phaeochromocytes)
Calcitonin (C) cells—thyroid gland (ultimobranchial
body)
Carotid body
Part of pineal body
Pigment Cells
Melanocytes in all tissues; melanophores of iris

HEAD AND NECK EMBRYOLOGY • 117
FIGURE 11-4. A stage 15, 33-day-old human embryo upon which are depicted the
neural crest streams emanating from the rhombomeres (r1-r8), infl uenced by HOX gene
expression patterns. FNP, frontonasal prominence; FB, forebrain; E, eye; MB, midbrain;
HB, hindbrain; OV, otic vesicle; HRT, heart; pa 1, pa 2, pharyngeal arches. (Modifi ed from
Hinrichsen KV. Human-Embryologie. Berlin: Springer-Verlag; 1990.)
FIGURE 11-5. Schematic depiction of the fate map of neural crest tissue
segments (left) and their facial and pharyngeal arch destinations. R1-R8, rhombomeres;
1-4, pharyngeal arches; v, vii, ix, x, cranial nerves. (Reprinted from Sperber GH. Embryology of
the head and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)

118 • CHAPTER 11
THE PHARYNGEAL ARCHES
Ventrally migrating neural crest cells surround mesoder-
mal cores containing the six aortic arches to form the
pharyngeal arches ( Figure 11-9 ). Only fi ve pairs of arches
are discernible, however, since the fi fth is extremely tran-
sitory. The arches are separated by ectodermal grooves
externally and endodermal pharyngeal pouches internally.
Common to the initial arches are a central cartilage rod,
surrounded by mesoderm accompanied by a nerve along
the aortic arch artery. The fates of the archetypal ele-
ments in each of the pharyngeal arches are outlined in
Table 11-2 . The consequences of maldevelopment of
the derivatives of the pharyngeal system are listed in
Table 11-3 . 14 , 15
A
LS
FNP
DEL
CO
LS
NP
GP
BG
NOF
MXP
MNP
HA
DEL − Developing eyelids
CO − Cornea
GP − Globular prominence
MDP − Mandibular prominence
HA − Hyoid arch
NOF
LNP
MXP
MDP
HA
NP
MNP
ST
LS − Lens
NP − Nasal pit
MNP − Median nasal prominece
ST − Stomodeum
BG − 1st branchial groove
BG
CS
FNP − Frontonasal prominence
NOF − Naso-optic furrow
LNP − Lateral nasal prominence
MXP − Maxillary prominence
CS − Cardiac swelling
B
5 weeks
6 weeks
7 weeks
A
B
C
C
FIGURE 11-6. Schematic depiction of facial formation at 4, 5, 6, and 7 weeks
postconception. (Reprinted from Sperber GH. Embryology of the head and neck. In: English GM,
ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
A
D
B
E
C
F
FIGURE 11-7. Orofacial clefts: (A) unilateral cleft lip; (B) bilateral cleft lip; (C) oblique
facial cleft; (D) median cleft lip and nasal defect; (E) median mandibular cleft; (F) unilateral
macrostomia.

HEAD AND NECK EMBRYOLOGY • 119
THE PALATE
Three elements make up the secondary defi nitive palate:
the two lateral palatal shelves projecting into the sto-
modeum from the maxillary prominences and the ante-
rior midline primary palate derived from the frontonasal
prominence. These elements are initially widely separated
owing to the advancing edges of the lateral shelves being
defl ected vertically on either side of the tongue that occu-
pies most of the stomodeal chamber. Concomitantly, the
cartilaginous nasal septum descends from the roof of the
stomodeum ( Figure 11-10 ). 16
Consequent to expansion of the stomodeum and mouth
opening, the tongue withdraws from between the palatal
shelves, allowing their elevation into the horizontal plane,
enabling them to contact each other in the midline, with
the primary palate anteriorly and with the vertical nasal
septum superiorly. Thereby is the single chamber stomod-
eum converted into a three-chambered oronasal orifi ce—
the two nasal fossae and the mouth.
Fifth pharyngeal pouch
(ultimo-branchial body)
Fourth pharyngeal pouch
(thyroid and parathyroid)
Retrohyoid depression
(cervical sinus)
Third pharyngeal pouch
(thymus and parathyroid)
Second pharyngeal
pouch (tonsillar fossa)
First branchial groove
(external acoustic meatus)
Tympanum
First pharyngeal
pouch (auditory tube)
Tongue
Foramen
cecum
Thyroglossal duct
Thymus and parathyroid
migration path
Larynx (trachea)
Esophagus
FIGURE 11-8. Schematic depiction of pharyngeal pouch derivatives and their
migration paths. (Reprinted from Sperber GH. Embryology of the head and neck. In: English GM,
ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
Eye
Nasal pit
Stomodeum
Meckel’s cartilage
Interbranchial
bridge
Malleus blastema
Incus blastema
{
Otic placode
Stapes
blastema
Reichert’s
cartilage
Pharynx
Neural crest
Migrating around
mesoderm core
I
II
III
IV
V
FIGURE 11-9. Diagramatic sectioned view of facial and pharyngeal arch
development. (Reprinted from Sperber GH. Embryology of the head and neck. In: English GM, ed.
Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)

120 • CHAPTER 11
TABLE 11-2. Derivatives of the Pharyngeal System
Pharyngeal
Arch
Ectodermal
Groove
Endodermal
Pouch
Skeleton
Viscera
Artery
Muscles
Motor Nerve
Sensory
Nerve
1st Mandibular Ext. acoustic
meatus;
Ear hillocks;
Pinna
Auditory tube;
Middle ear
tympanum
Meckel’s
cartilage;
malleus; incus
(mandible
template)
Body of tongue
Ext. carotid
artery
Maxillary
artery
Masticatory Muscles
Tensor tympani
Mylohyoid, tensor palati
Ant. digastric
Mand. Div. V.
Trigeminal
V
Lingual nerve
2nd Hyoid
Disappears
Tonsillar fossa
Stapes, styloid
process; sup
hyoid body
Midtongue;
Thyroid gland
anlage; Tonsil
Stapedial
artery
(disappears)
Facial, stapedius,
stylohyoid, post.
digastric
VII
Facial
VII
Chorda tympani
3rd
Disappears
Inf. parathyroid 3;
Thymus
Inf. hyoid body,
grt. cornu
hyoid
Root of tongue;
Fauces;
Epiglottis
Thymus; Carotid
body
Inf. Parathyroid 3
Int. carotid
artery
Stylopharyngeus
IX
Glossopharyngeal
IX
Glossopharyngeal
4th
Disappears
Sup. parathyroid 4 Thyroid &
laryngeal
cartilages
Pharynx;
epiglottis
Sup. Parathyroid
4
Aorta (L)
Subclavian
(R)
Pharyngeal constrictors
Levator palatini
Palatoglossus
Palatopharyngeus
Cricothyroid
X Superior
laryngeal nerve
Vagus
X Auricular N.
to ext. acoustic
meatus
6th
Disappears
Telopharyngeal
(Ultimobranchial)
body (cyst))
Calcitonin “C”
cells
Cricoid,
Arytenoid,
Corniculate
cartilages
Larynx
Pulmonary
arteries
Ductus
arteriosus
Laryngeal muscles
Pharyngeal constrictors
X Inferior
laryngeal nerve
Vagus
X
Vagus
Postbranchial
region
Somites 4
Occipital
somites
Tracheal
cartilages
SCLEROTOMES
Basioccipital
bone
Trapezius;
Sternomastoid
MYOTOMIC MUSCLE
Intrinsic tongue muscles
Styloglossus Extrinsic
Hyoglossus tongue
Genioglossus muscles
Extrinsic ocular muscles
XI
Spinal accessory
XII
Hypoglossal
III. Oculomotor
IV. Trochlear
VI. Abducens
Spinal nerves,
C1, C2
Prechordal
somites
Upper cervical
somites
Nasal capsule
Nasal septum
Cervical
vertebrae
Geniohyoid; Infrahyoid
muscles

HEAD AND NECK EMBRYOLOGY • 121
TABLE 11-3. Possible Anomalies of the Pharyngeal System
Arch
Ectodermal
Groove
Endodermal
Pouch
Skeleton
Artery
Muscles
Nerve
1st
Mandibular
Aplasia, atresia,
stenosis,
duplication of
ext. acoustic
meatus
Diverticulum of audi-
tory tube; aplasia,
atresia, stenosis
of tube
Aplasia/
dysplasia of
malleus, incus,
mandible
Hypoplastic/absent
ext. carotid &
maxillary arteries
Defi cient
mastica-
tory/ facial
muscles
Absent mand.
nerve
2nd Hyoid
Cervical
(Branchial)
cleft, sinus,
cyst, fi stula
Tonsillar sinus,
pharyngeal
fi stula
Aplasia/
dysplasia of
stapes, styloid
process
Persistent
stapedial artery
Defi cient
facial,
stapedial
muscles
Defi cient
facial,
chorda
tympani N.
3rd
Cervical cleft,
sinus, cyst,
fi stula
Cervical thymus;
thymic cyst;
aplasia parathyroid
3; aplasia thymus
(DiGeorge
anomaly)
Defective hyoid
bone
Hypoplastic/absent
int. carotid artery
Defi cient
glossopha-
ryngeal
nerve
4th
Cervical cleft,
sinus, cyst,
fi stula
Fistula/sinus
from pyriform
sinus; aplasia
parathyroid 4
Congenital
laryngeal
stenosis, cleft,
atresia (Fraser
syndrome)
Double aortae;
aortic
interruption; right
aorta
Defi cient
faucial
muscles
Defi cient
vagus
nerve
6th
Aplasia of
calcitonin “C”
cells (DiGeorge
anomaly)
Aortico-pulmonary
septation anom-
alies (DiGeorge
anomaly)
Nasal septum
Crista galli and
perpendicular plate
of ethmoid
Labyrinth of ethmoid
Superior and middle
nasal conchae
Nasal
fossa
Nasal
capsule
Olfactory nerve fibres
Sphenoid (lesser wing)
Sphenoid
(lesser wing)
Eye
Fused
eyelids
Sphenoid (greater
wing and body)
Sphenoid (greater wing)
Inferior nasal concha
Fused palatal shelves
Ossifyng maxilla
Vomer (paired ossification centers)
Mandible
Hyoid cartilage
Oronasal chamber
Lateral palatal shelf
1st barnchial groove
Cardiac swelling
Meckel’s
cartilage
Tongue
FIGURE 11-10. Coronal sections of head at 7 weeks (A); 12 weeks (B) revealing
nasal capsule and palate development. (Reprinted from Sperber GH. Embryology of the head
and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
Palatoschisis
Clefting of the palate is a consequence of failure of any
one of the complex embryological interactions neces-
sary for intact palate formation. Palatal shelf develop-
mental defects can be categorized as (1) failure of shelf
formatio n; (2) fusion of palatal shelf(ves) with the
tongue or mandible; (3) failure of shelf(ves) elevation;
(4) failure of shelves to contact each other; and (5) per-
sistence of the midline medial edge epithelium that
normally transforms into mesenchyme or disintegrates
(apoptosis). 17
All degrees of clefting may occur, ranging from
nondysfunctinal submucous clefts to the major incapacitat-
ing cleft lip and palate (cheilouranoschisis) ( Figure 11-11 ).
The consequences of palatal clefting are multifarious, rang-
ing from oronasal food regurgitation, speech impediments,
dental malocclusion, facial growth retardation, and social
isolation.

122 • CHAPTER 11
THE SKULL
The neurocranium encapsulating the brain is divided
developmentally into the vault or calvaria, of membranous
bone origin, and the cranial base of endochondral bone for-
mation. Herein lies the discrepancy of embryopathologi-
cal distinctions of calvarial and basicranial developmental
lesions ( Figure 11-12 ).
The viscerocranium, composed of the face and orog-
nathic apparatus, is derived from the facial promi-
nences, previously described, and the pharyngeal arches.
Ossifi cation of these components, either intramembra-
nously or endochondrally follows a genetically determined
timetable, commencing at 8 weeks postconception at
various ossifi cation centers that coalesce to constitute the
osseous components of the skull. The intervening sutures
are of enormous signifi cance in determining skull size and
shape, and are the source of clinical syndromes of skeletal
dysplasias.
The calvaria is surgically signifi cant for its brain protective
function and its refl ection of the morphology of the under-
lying brain. Microcephaly, hydrocephaly, and macrocephaly
Upper lip
Vestibule
Alveolar
process
A
B
C
FIGURE 11-11. Cleft palate variations: (A) bifi d uvula; (B) unilateral cleft palate
and lip; (C) bilateral cleft palate and lip. (Reprinted from Sperber GH. Embryology of the
head and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
Frontal Bone
Parietal Bone
Squamous occipital bone
Squamous temporal bone
Basioccipital bone
Tympanic ring
Styloid process
Ear ossicles
Ethmoid Bone
Vomer
Nasal bone
Zygomatic bone
Palatine bone
Hyoid bone
Thyroid cartilage
Cricoid cartilage
Branchial arch cartilages
Endochondral bone
Membranous bone
Mandible
Nasal septum
Maxilla
Lacrimal bone
Sphenoid (body and lesser wings)
Sphenoid (greater wings and

pterygoid plates)
FIGURE 11-12. Skull bone origins. (Reprinted from Sperber GH. Embryology of the head
and neck. In: English GM, ed. Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
are revealed by the calvarial contour created by the fron-
tal, parietal-occipital, and temporal bones. Patency of the
sutures between these bones is critical for brain expansion
during fetal development and subsequent infancy. Skull
growth results from a combination of (1) bone remodeling
(deposition and resorption), (2) bone apposition at sutures
and synchondroses, and (3) transposition and displacement
of enlarged and remodeled bones. Premature synchond-
rosal fusion in the basicranium, prior to adolescence, will
lead to a foreshortened chondrocranium (brachycephaly)
that usually causes calvarial expansion and bulging frontal
bossing. Premature fusion of the calvarial sutures, convert-
ing syndesmoses into synostoses results in distorted skull
shapes characterizing craniosynostotic syndromes (Apert,
Crouzon) or isolated sutural fusions. 18 – 21
THE MASTICATORY APPARATUS
Development of the masticatory apparatus comprises
the jaws, temporomandibular joint, teeth, and masti-
catory musculature derives from the fi rst pharyngeal

HEAD AND NECK EMBRYOLOGY • 123
arch. 22 Maxillary and palate development are previ-
ously described. The primary template of the mandible,
Meckel’s cartilage, is the skeleton of the fi rst pharyn-
geal arch, to which are added secondary accessory car-
tilages, for the head of the condyle and coronoid process
( Figure 11-13 ).
Aplasia of the mandible (agnathia) and the hyoid bone
(fi rst and second arch syndrome) is a rare lethal condition
with multiple defects of the orbit and maxilla, refl ecting
defi ciencies of neural crest tissue migration to these areas
of the face and neck. Mandibular hypoplasia leads to
micrognathia, characteristic of Pierre Robin sequence, the
cri du chat (cat cry) syndrome, mandibulofacial dysostosis
(Treacher Collins syndrome), progeria, Down syndrome
(trisomy 21), oculomandibulodyscephaly (Hallermann-
Streiff syndrome), and Turner syndrome (XO).
Condylar cartilage
Coronoid cartilage
Meckel’s cartilage
Angular cartilage
Inferior alveolar nerve
Mandibular ossification center
Mandibular nerve
Mental nerve
Incisive nerve
Lingual nerve
Mental ossicle
FIGURE 11-13. Cartilages and membranous ossifi cation
center of fetal mandible (lateral view, left half). (Reprinted from
Sperber GH. Embryology of the head and neck. In: English GM, ed.
Otolaryngology. Philadelphia: JB Lippincott; 1990:1–36.)
FIGURE 11-14. Development of the mandible and temporomandibular joint.
(Reprinted from Sperber GH. Embryology of the head and neck. In: English GM, ed. Otolaryngology.
Philadelphia: JB Lippincott; 1990:1–36.)
THE TEMPOROMANDIBULAR JOINT
This complex double joint is secondary to the primary jaw
joint in utero between the malleus and incus formed in
Meckel’s cartilage between weeks 8 and 23. The defi ni-
tive temporomandibular joint develops as upper and lower
cavities arise in the mesenchyme between the condylar
cartilage and the temporal fossa, leaving a meniscus between
the bones, creating a double joint ( Figure 11-14 ). 23,24 Since
the condylar cartilage acts uniquely as both an articular
and growth cartilage, damage to the cartilage by trauma,
infection, or any of the arthritides impacts mandibular
growth, leading to unilateral or bilateral micrognathia.
Conversely, continued growth past adolescence accounts
for prognathism in acromegalics.
DENTITION
Dental development is enormously complex, incurring
interactions between ectoderm (forming enamel) neural
crest ectomesenchyme (forming dentin, pulp and cemen-
tum), and mesoderm (forming portion of the periodontal
ligament). The genetics of odontogenesis has expanded
prodigiously into a specialty invoking the whole of the
embryonic, fetal, infant, adolescent, and adult stages of
life. The prolonged chronology of odontogenesis makes
teeth the most protracted developing set of organs in the
body, exposing them to the greatest extent of possible
maldevelopment. This life-long duration of dental dys-
morphology, ranging from anodontia, and supernumer-
aries to malocclusion requires the specialty of dentistry
to devote to its stomato-odontognathological require-
ments. 25 Full details of odontogenesis are available in
Sperber et al.7

124 • CHAPTER 11
CONCLUSION
These synoptic insights into embryogenesis and develop-
ment provide the clinician with better comprehension of
the origin of the maladies they are called upon to rem-
edy and allow for insightful treatment planning to achieve
normality of function and performance. The multiferous
syndromes of the head and neck are rooted in deviations
of development that require etiological diagnosis before
surgical remediation. 26 Evaluation of congenital facial
anomalies is enhanced by computer tomography (CT)
scanning and magnetic resonance imaging (MRI) in con-
junction with maldevelopmental insights to appropriately
manage rehabilitational surgical interventions.27
ACKNOWLEDGMENTS
Grateful thanks are accorded to Joanne Lafrance for her
meticulous word processing skills.
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lsuhsc.edu/

c h a p t e r 12
Cleft Lip Repair
William Y. Hoffman, MD
PATIENT EVALUATION
Cleft lips occur in a wide range of phenotypes, from a min-
imal notch to complete cleft lip and palate ( Figure 12-1 ).
The lip is formed in the sixth week of gestation by the
fusion of the frontonasal process and the maxillary pro-
cesses. The frontonasal process gives rise to the nose and
the central lip (the prolabium) and the premaxilla back
to the incisive foramen (the primary palate). The fusion
of the maxillary processes with the central lip is stabilized
by migration of mesenchymal tissue, which is derived
from the neural crest. The stabilization of neural crest by
folate is well documented and contributes to the decrease
in cleft occurrence with administration of perinatal folate.
Although the remainder of the palate unites at a later
point in fetal development, cleft lip and palate frequently
occur together. Approximately one fourth of clefts are of
the lip only, one fourth of the palate only, and one half of
the cleft lip and palate.
Classifi cation may be done with simple description:
the most minimal cleft is described as microform or
forme fruste, incomplete clefts with intact nasal sill
may be described in terms of the percentage of the lip
involved, while complete clefts involve the nasal sill and
fl oor. The extent of involvement of the alveolus should be
documented as well as the palate. Kernahan formulated
a diagrammatic system to standardize these descriptions
( Figure 12-2 ).
Approximately 30% of clefts occur in association with a
known genetic syndrome. We are only beginning to under-
stand the expression of specifi c genes in relation to the
formation of clefts. Almost all syndromes present with a
right-sided cleft, although left-sided clefts represent about
two thirds of unilateral clefts. The presence of a right-sided
cleft should prompt heightened surveillance for possible
syndromic involvement.
Some syndromes are more common and should be rec-
ognizable by the practitioner. Relatively common is Van
Der Woude syndrome, which is manifested by clefts in
association with lip “pits,” which in fact are sinus tracts of
the minor salivary glands. This syndrome, as with many, is
autosomal dominant but has variable phenotypic expres-
sion, from submucous cleft palate through complete bilat-
eral cleft lip and palate. It is associated with the interferon
regulatory factor 6 ( IRF6 ) gene, also found in popliteal
pterygium syndrome.
Inheritance of nonsyndromic clefts is not typically
Mendelian; if an otherwise unaffected family has a child
with a cleft, there is a 2% to 5% chance that another child
will be affected. If two children are affected, the chances
for a third child is 9%; if a parent and a child are affected,
the chances for another child rise to 15%.
In general, there are few contraindications to cleft lip
repair. While the presence of a syndrome, particularly one
with signifi cant developmental delay, may mitigate against
cleft palate repair because of airway concerns, these con-
cerns are not as signifi cant regarding the lip. In bilateral lip
repairs the very short columella may result in a relatively
restricted nasal airway, but the use of nasal stents postop-
eratively will help both in lengthening of the columella
and in maintaining patency of the nasal airway.
PATIENT PREPARATION
The classic mnemonic for cleft lip surgery is the “rule of
10s”—the child should be 10 weeks old, weigh 10 pounds,
and have a hemoglobin level above 10 g/mL. These are
still useful criteria but it is important to remember that
they are based on an evaluation of the general well-being
of the infant. Ten to 12 weeks is the average age where the
other goals can be met. The hemoglobin is perhaps the

126 • CHAPTER 12
least important of the three, as fetal hemoglobin begins
to disappear at birth to be replaced by adult hemoglobin,
resulting in a nadir at about 6 to 8 weeks of age. However,
the expected blood loss for a lip repair should be negligible
FIGURE 12-1. (A) Microform of cleft lip (forme fruste).
(B) Incomplete unilateral cleft lip. (C) Complete unilateral
cleft lip.
A
B
C
FIGURE 12-2. Kernahan system for classifi cation of clefts.
1
2
9
10
11
3
4
8
Nose
1, 5 Nasal floor
2, 6 Lip
3, 7 Alveolus
4, 8 Hard palate
9, 10 Hard palate
11 Soft palate
Incisive foramen
7
6
5
and a hemoglobin level of 8 or 9 may be acceptable if the
child is otherwise fi ne.
The child’s weight is a general refl ection of adequate
nutritional intake. If there is a cleft palate in association
with the cleft lip, the child will not be able to suck ade-
quately for breastfeeding and requires specialized feeding
appliances. Inadequate intake, gastroesophageal refl ux, and
increased effort of breathing all may contribute to poor
weight gain, and these must be investigated thoroughly
prior to surgery.
Lastly, if there is a complete cleft through the lip and
alveolus (and generally the palate as well) the child must
be prepared for surgery by controlling the position of the
maxillary segments. Reduction of the gap in a unilateral
cleft and, even more importantly, controlling the position
of the premaxilla in a bilateral cleft are important factors
in preoperative preparation which make the surgery both
more facile as well as producing better results.
There are multiple means of manipulation of the
alveolar segments and the premaxilla. Latham originally
described alveolar molding plates as pin-fi xed appliances;
these have largely been supplanted by denture-like plates
that are maintained with denture adhesive. These can be
fashioned by orthodontists or pediatric dentists; creating

CLEFT LIP REPAIR • 127
space on one side and adding material on the other can
gradually move the alveolus. The addition of prongs to
stretch out the abnormal nostril(s) has given rise to the
popular acronym NAM (nasal alveolar molding). These
devices generally require weekly visits to the orthodontist
and may require 3 to 4 months to accomplish. Treatment
must be started early as the segments are much more
mobile in the neonate.
In unilateral clefts, taping with gentle compression will
signifi cantly narrow the gap between the alveolar seg-
ments ( Figure 12-3 ). While this is a reasonable and inex-
pensive alternative to NAM in the unilateral cleft, in the
bilateral cleft it is diffi cult to move the premaxilla in a
predictable fashion and NAM is preferable, if available
( Figure 12-4 ).
Performing an operation on a 3-month-old child is a
signifi cant undertaking, even when the surgery is essen-
tially superfi cial. Pediatric anesthesiologists should be
administering anesthesia. Standard monitoring is suf-
fi cient (blood pressure, pulse oximetry), and the use of
an oral Ring–Adair–Elwin (RAE) endotracheal tube is
recommended, as this can be secured to the chin with-
out distortion of the upper lip or commissures. Occlusive
plastic sheets are placed over the eyes for protection and
to allow antiseptic preparation of the entire face. Betadine
is used for prepping as chlorhexidine is contraindicated
for facial preparation.
The oral RAE tube is useful for positioning; by turning
the patient 90 or even 180 degrees, the surgeon has access
to the entire face and the endotracheal tube is still directed
toward the ventilator and the anesthetist.
The lip repair should be marked with a fi ne-tipped
marking pen. The author’s preference is for brilliant green
dye, which is alcohol based and produces a fi ne line with a
cut, cotton-tipped applicator, even on mucosa. The muco-
cutaneous junction is tattooed with the dye as well. This
is critical for repairing the white roll during the closure,
as the mucosal blanching that occurs from epinephrine
may make this line obscure during the surgery. Once this
is completed, the lip is injected with lidocaine 0.5% and
FIGURE 12-3. Taping the cleft segments can be an
effective and readily available means of narrowing the cleft
prior to surgical closure.
FIGURE 12-4. (A) Bilateral cleft lip and palate with
marked anterior displacement of premaxilla. (B) Nasoalveolar
molding device shown in place. (C) Premaxilla shown in
good position in relation to the lateral segments. Note the
improvement in nasal contour as well.
B
A
C
epinephrine 1:200,000; 1 cc/kg can be safely administered,
although generally no more than 3 to 4 cc is required.
TECHNIQUES
Unilateral Cleft Lip Repair
In all unilateral cleft lip repairs, the goal is to lengthen
the cleft side to achieve a symmetrical appearance of the

128 • CHAPTER 12
Cupid’s bow. In the fi rst half of the twentieth century, rec-
ognition that a straight-line repair would inevitably con-
tract and shorten led to breaking up of the scar, initially
with quadrilateral repairs and later with the triangular
lip repair (also known as the Tennison repair, later modi-
fi ed by Randall), which is still popular today. In essence,
the triangular lip repair is a z-plasty placed just above the
vermilion border; as with any z-plasty, length is obtained
at the expense of width. The triangular lip repair creates
excellent length but because the area just above the white
roll is the tightest, the lip appears somewhat fl at on lateral
view. In addition, lip revision may be more diffi cult due to
the geometric closure.
The rotation advancement repair, also known as the
Millard repair after the initial description, addresses many
of these issues, by moving the z-plasty to just below the
nasal sill ( Figure 12-5 ). This places the tightest area of clo-
sure at the top of the lip rather than the bottom, and cre-
ates a more natural appearing lip with normal pout. The
scar follows the natural line of the philtral column on the
side of the cleft, contributing to the more normal appear-
ance. The introduction of a small z-plasty above the white
roll can reduce scar contraction but does not break up the
philtrum signifi cantly.
The rotation fl ap is designed by fi rst establishing the
high point of Cupid’s bow on the medial segment. This
is established by fi rst marking the high point on the intact
side, then the midline. Note that the midline is not usu-
ally obvious and that the gradually curving prolabium
can often be marked correctly in a number of locations.
A narrow prolabium is much more normal in appearance
FIGURE 12-5. Typical markings for a rotation
advancement repair. Note the small triangular fl ap of dry
mucosa on the lateral segment which will be used to augment
dry mucosa on the medial side.
and moving the midpoint and high point further medi-
ally reduces the amount that the lip needs to be rotated
for symmetry. The most common error is to have the high
point too far lateral, resulting in a scar that is too far lateral,
a prolabium that is too wide and a lip that is too short on
the repaired side.
Once the high point is established, the rotation fl ap
is designed from that point upwards, curving up to the
junction of the lip with the columella. The cutaneous
portion that is lateral to the rotation fl ap becomes the “c”
fl ap, which will be used to lengthen the columella. The
same line is continued down into the mucosa, creating the
medial mucosal fl ap that will be used for lining the vesti-
bule. Note that in an incomplete cleft, the medial and lat-
eral mucosal fl aps are basically trimmed near the vestibule
to permit suturing of the lateral lip fl ap.
The key to designing the advancement fl ap is again the
determination of the point that will be closed at the white
roll. The height of the lip is again the critical factor; the
point on the advancement fl ap is established by measuring
the normal side with calipers from the high point to the
nasal sill, then measuring the same length on the advance-
ment fl ap ( Figure 12-6 ). The advancement fl ap is drawn
with a line along the mucocutaneous junction, taking care
not to include any mucosa in the skin portion of the fl ap,
FIGURE 12-6. (A) Measuring the height of the lip on
the non-cleft side. (B) Transfer of the measurement from the
normal side to the cleft side. This determines the location of
the advancement fl ap on the cleft lateral segment.
A
B

CLEFT LIP REPAIR • 129
as this will result in a permanently pink scar (this is true
for the c fl ap as well). The upper part of the advancement
fl ap is cut just below the nasal sill but does not continue
around the alar base, where the scar is more obvious. The
mucosal fl ap on this side is a little more complex, leaving
a small triangular fl ap of dry mucosa on the lateral lip ele-
ment to insert into the central portion of the lip, where the
dry mucosa is almost always defi cient. The lateral mucosal
fl ap is taken from the remaining medial mucosa.
The incisions on both sides are made just through the
dermis, as the orbicularis muscle is quite superfi cial. On
both sides, the orbicularis must be dissected circumfer-
entially from skin and mucosa. Medially, the dissection
should be limited to the midpoint of the philtrum to pre-
serve any central depression that exists. On both sides the
muscle must be released from the abnormal attachments
to the nose; medially from the columella, laterally from the
nasal sill and alar base. The lateral dissection should not
be carried any farther than is necessary to complete the
circumferential release of the muscle.
Once the fl aps are all completely dissected, the nose
is addressed by separating the nasal skin from the alar
cartilage using small tenotomy scissors. This dissection
is carried out over the opposite alar dome and onto the
nasal dorsum, creating a space for rotation of the cleft side
alar cartilage up and medial, partially correcting the nasal
deformity. Sutures are placed at the end of the procedure,
either through an internal method or over external bol-
sters to hold the alar cartilage in the new position.
Adequate release of the lateral lip is crucial to obtain-
ing a tension-free repair. The lateral vestibular incision per-
mits release of the lip and muscle as a unit, with dissection
above the periosteum of the maxilla (some surgeons have
proposed a subperiosteal dissection but in general it is felt
that this may interfere with subsequent maxillary growth).
The nose is released along the piriform aperture laterally to
allow the nasal ala to be brought into a symmetrical posi-
tion with the opposite side. This creates a lateral mucosal
defect in the nasal sidewall, which is closed with the lateral
mucosal fl ap. The medial mucosal fl ap is turned over to
line the vestibule and the fl oor of the nose. The alar base is
anchored to the columella with an absorbable suture, which
brings the columella into the midline and should attempt
to position the ala symmetrically with the oppo site side.
This is generally an “air knot,” where the surgeon ties it
while watching the symmetry from above the patient.
Closure of the lip repair follows that of the nose and
starts along the lateral maxillary vestibule, as this is dif-
fi cult to see later. The orbicularis oris muscle is repaired
with two or three absorbable mattress sutures. In general,
the medial muscle must be rotated somewhat inferiorly to
achieve proper alignment of the two lip segments. A good
muscle repair is critical to avoid tension on the skin closure,
and is associated with more normal lip growth in the future.
The majority of the muscle repair is near the lower part of
the lip to achieve appropriate fullness and contour. There
have been techniques described to separate specifi c muscle
FIGURE 12-7. Noordhoff triangular fl ap is a z-plasty just
above the mucocutaneous junction which can increase length
of the lip repair slightly as well as decreasing scar contracture.
components to place in different sites, but the author has
found this technically diffi cult and unnecessary.
The lip skin is closed with 6-0 fast-absorbing catgut
sutures; these are dissolved in a matter of days, obviating
the need for suture removal and avoiding cross-hatching.
If the lip repair appears at all short at the time of repair,
Noordhoff triangular lip fl ap (really a small, 1–1.5 mm
z-plasty above the white roll) is an excellent means of
obtaining a little extra length and reducing scar contrac-
ture ( Figure 12-7 ). The mucosa is closed with particular
attention to the “red line,” the junction between the wet
and dry mucosa. In most unilateral clefts the dry mucosa is
defi cient on the medial side, and an incision at the red line
will allow insertion of the dry mucosa that was preserved
with the lateral lip element at the outset, improving the
amount of dry mucosa and ensuring alignment of the two
types; retention of wet mucosa in the external lip repair
can be quite obvious and will mar an otherwise good
effort. If there is any tension on the wet mucosa, a z-plasty
is placed near the vestibule to avoid notching.
The nasal correction is completed at the conclusion of
the lip repair. With the McComb technique, two bolster
sutures are placed. The fi rst goes through the lateral por-
tion of the dome and is brought out through the dorsum
of the nose on the contralateral side. This should bring the
alar cartilage up to a slightly overcorrected position; the sec-
ond suture anchors the lateral crus at the point where it is
most prominent in the nasal sidewall. The sutures used are
4-0 monofi lament, and are tied over red rubber bolsters.
The internal bolsters have silk sutures attached as well,
which makes removal in clinic (time: less than 1 week)
very straightforward.
Bilateral Cleft Lip Repair
The bilateral cleft lip presents a number of additional
challenges compared with the unilateral situation. First, as

130 • CHAPTER 12
noted above, the premaxilla can be almost at 90 degrees
to the plane of the lip in a complete bilateral cleft, and
considerable effort should be devoted to repositioning the
premaxilla prior to surgery. It is critical to the fi nal out-
come that there be minimal tension on the lip repair and
also that the orbicularis muscle from the two lateral seg-
ments be brought together across the premaxilla in the
midline, and both of these require that the premaxilla
be brought back more in line with the lateral segments.
Although taping or elastics have been used with reason-
able success, alveolar molding plates are an excellent
choice, as these can also control the position of the lateral
segments, which tend to collapse behind the premaxilla.
If these efforts are unsuccessful, either due to absence of
resources or family compliance, a last alternative is use of
the vomer osteotomy at the time of surgery. This requires
a small longitudinal incision along the vomer behind the
premaxilla, and removal of about 4 to 5 mm of bone from
the vomer with a small rongeur. The premaxilla then rotates
back, usually into contact with the lateral segments. This
maneuver has been associated anecdotally with decreased
maxillary growth but there are no good long-term studies
documenting growth in this situation.
The second challenge is the short columella. This is due
to multiple factors: there is separation of the alar cartilages
with interposed fi brofatty tissue, but there is also a true
lack of length of the columella as well. Various propos-
als for correction of the columella fall into three primary
categories: preoperative lengthening with the addition of
prongs to the NAM device; intraoperative suturing and
internal rotation of the alar cartilages; and later procedures
such as forked fl aps or V-Y advancements to lengthen
the columella, usually at about age 4 to 5 years. All of these
have some shortcomings: NAM may provide inadequate
length despite some defi nite improvement; suturing of
the alar cartilages may rotate the tip upward as in any rhi-
noplasty, adding to the already foreshortened nose; later
procedure produce scars and may not produce adequate
length as well. Virtually all patients with unilateral or bilat-
eral clefts require rhinoplasty in their teens.
Lastly, while subtle, there is minimal or no dry mucosa
in the prolabium. The design of the lateral lip incisions,
which is otherwise similar to the Millar repair above, must
have a step-cut with preservation of the dry mucosa to
be repaired in the midline beneath the prolabium. This
author preserves 1 to 2 mm of existing dry mucosa with
the prolabium; others remove all mucosa and suture the
lateral mucosa to the prolabial skin.
With an appreciation of these issues, one can then
design the bilateral cleft lip repair. The central tenet is
that the prolabium must be narrowed to about 4 to 5 mm;
the central lip always becomes wider and a prolabium of
15 mm or more in an adolescent is an obvious stigma of
the bilateral cleft even with good scars ( Figure 12-8 ). The
length of the prolabium determines the length of the cuta-
neous lip repair; there is not really any method of increas-
ing this length at the time of the initial repair. The central
lip is hinged on the columella, and this is the source of
blood supply. The additional skin on either side of the pro-
labium can be preserved as “forked fl aps” to be inset under
the nasal sill on each side and used later for columellar
lengthening. The lateral lip element is tailored to fi t the
length of the prolabium. The two alar bases are sutured to
each other, which may exaggerate the short columella in
more severe cases.
The nose may be addressed at the time of lip repair.
Mulliken was an early proponent of suturing the alar domes
together to obtain additional columellar length. Initially
this was done through a midline incision in the tip of the
nose, but this has now evolved to a procedure done through
bilateral rim incisions with excision of excess overhanging
skin at the alar margin. Cutting proposes doing a modifi ed
open rhinoplasty by elevating the medial crura of the alar
cartilages together with the prolabium and the columella.
This is done to preserve blood supply to the prolabium,
but requires a retrograde dissection of the nasal tip in order
FIGURE 12-8. (A) Markings for a bilateral lip repair.
Note the narrow prolabium and the step-cut on the lateral
segment to preserve additional dry mucosa. (B) Bilateral cleft
lip repair completed. Note the alignment of the dry mucosa
in the midline below the prolabium, and absence of whistle
deformity.
A
B

CLEFT LIP REPAIR • 131
to suture the tip cartilages together. Both of these tech-
niques show very nice improvement in the columella but
in lateral views the tip is often rotated cephalad.
Gingivoperiosteoplasty
Gingivoperiosteoplasty (GPP) refers to the creation of
subperiosteal fl aps at the cleft margin to close the alveolar
cleft. Skoog described this many years ago, and Delaire later
proposed more extensive subperiosteal dissection of the
lateral lip element. It is well documented that these repairs
will result in bone bridging the alveolar gap in a majority of
cases, but it is also widely held that extensive undermining
of the periosteum may create maxillary growth defi ciency.
Cutting and Grayson have popularized GPP after NAM
in both unilateral and bilateral clefts but have emphasized
that this should only be done if there is perfect alignment
of the alveolar segments with a minimal gap (1–2 mm at
most). Orthodontists are divided about the benefi t of this
procedure, as it may be more diffi cult to manipulate the
alveolar segments if there is bony continuity.
COMPLICATIONS
Complications of lip repair are quite rare, as there is excel-
lent blood supply; infections and dehiscence occur in well
under 1% of patients. While not specifi cally a complica-
tion, hypertrophic scarring can occur commonly, particu-
larly in darker complexions. This is often seen at about
3 to 6 weeks following surgery and usually resolves with-
out specifi c treatment. Taping and massage may be benefi -
cial. With hypertrophic scarring the lip usually becomes
somewhat shorter along the line of repair, but in general
the length returns as the scar diminishes and softens (this
may take as long as a year).
OUTCOMES
It is rare that a critical plastic surgeon would not see some-
thing to “touch up” in any cleft lip repair. The goal should
be to minimize such interventions, as repeated revisions
will remove some normal tissue with each procedure and
will inevitably result in a lip that is tight. Unless there is a
severe problem, the earliest revision should be considered
is in preschool, around age 5 years. Better still, if possible
revision should be delayed until orthodontic treatment is
completed, so that the fullness of the lip can be evaluated
appropriately. As children mature they should be involved
in the discussion of any revision; there is a surprising range
of opinions among the patients.
Nasal deformity is almost always addressed in early to
mid teens. Most unilateral cleft patients have septal devia-
tion into the side of the cleft and usually have deviation of
the bony pyramid to the opposite side. A septorhinoplasty
allows correction of the breathing issues and harvesting
of septal cartilage to use as graft material for any residual
tip asymmetry. An inverted U osteotomy allows the bony
pyramid to be shifted as a unit back into the midline.
CONCLUSION
Cleft lip is best treated by a team approach, and consis-
tent good results are now the expectation rather than the
exception. It is often the small details that make a large
difference in the fi nal outcome.
SUGGESTED READING
Losee JF, Kirschner RE, eds. Comprehensive Cleft Care. New York:
McGraw-Hill; 2008. An excellent text that is encyclopedic
in its coverage of cleft lip and palate with multiple tech-
niques reviewed.
Millard R. Cleft Craft. New York: Little Brown; 1976–1980.
Out of print but well worth the effort as a review of many
techniques and the evolution of the rotation advancement
repair.