Pediatric Plastic and Reconstructive Surgery

females (4:1). The median age of appearance is 2 weeks; 50% are
noted at birth as a telangiectatic stain, pale spot, or ecchymotic
area. IH grows faster than the child during the first 9 months of
age (proliferating phase); 80% of its size is achieved by 3.2 (±1.7)
months. IH is red when it involves the superficial dermis. A le-
sion beneath the skin may not be appreciated until 3 to 4
months of age when it has grown large enough to cause a visible
mass; the overlying skin may appear bluish. After 12 months,
the tumor begins to regress (involuting phase); the color fades
and the lesion flattens. Involution ceases in most of children by
age 4 years (involuted phase). After involution, one-half of chil-
dren will have residual telangiectasias, scarring, fibrofatty resid-
uum, redundant skin, or destroyed anatomical structures.
Infants with five or more small (<5 mm) IHs have a 16% risk
of having hepatic hemangiomas, which are typically asympto-
matic. PHACE association consists of a plaquelike IH in a
regional distribution of the face with at least one of the follow-
ing anomalies: Posterior fossa brain malformation, Heman-
gioma, Arterial cerebrovascular anomalies, Coarctation of the
aorta and cardiac defects, Eye/Endocrine abnormalities. Because
8% of children have a stroke in infancy, these patients should
have an MRI to evaluate the brain and cerebrovasculature.
Infants are referred for ophthalmologic, endocrine, and cardiac
evaluation to rule out associated anomalies. LUMBAR associa-
tion (Lower body infantile hemangioma, Urogenital anomalies,
Myelopathy, Bony deformities, Anorectal malformations, Renal
anomalies) typically a ects the sacral area or lumbar region.
Ultrasonography is obtained to rule out associated anomalies in
infants less than 4 months of age. MRI is indicated in older
infants or when ultrasound is equivocal.
40.2.2 Congenital Hemangioma
CHs are fully grown at birth and do not have postnatal growth.
They are red-purple with coarse telangiectasias, central pallor,
and a peripheral pale halo. These lesions are more common in
Fig. 40.3 Differentiating infantile hemangioma from a vascular malformation. (a-d) Infantile hemangioma grows rapidly during the first few months of
life and then involutes. (e-h) Venous malformation of the lip. Vascular malformations are present at birth, slowly worsen, and do not regress.
Table 40.3 Mutations associated with vascular anomalies
Condition

Mutated
gene
Inheritance
Capillary malformation

GNAQ

Somatic
Sporadic venous malformation

TIE2

Somatic
Verrucous venous malformation

MAP3K3

Somatic
Glomuvenous malformation

Glomulin

Dominant
Cutaneomucosal venous malformation

TIE2

Dominant
Cerebral cavernous malformation

KRIT1

Dominant
Sporadic lymphatic malformation

PIK3CA

Somatic
Familial congenital primary lymphedema

VEGFR3

Dominant
Lymphedema-distichiasis

FOXC2

Dominant
Lymphedema-hypotrichosis-telangiectasia

SOX18

Recessive
Hennekam syndrome

CCBE1

Recessive
Capillary malformation–arteriovenous mal-
formation
RASA1

Dominant
Hereditary hemorrhagic telangiectasia type
1 (HHT1)
ENG

Dominant
Hereditary hemorrhagic telangiectasia type
2 (HHT2)
ACVRLK1

Dominant
PTEN-associated vascular anomaly

PTEN

Dominant
Abbreviation: PTEN, phosphatase and tensin homolog.
Integument
384

the extremities, have an equal sex distribution, and are solitary
with an average diameter of 5 cm. There are two forms: rapidly
involuting congenital hemangioma (RICH) and noninvoluting
congenital hemangioma (NICH). RICH involutes rapidly after
birth and 50% complete regression by 7 months of age; the
remaining tumors are fully involuted by 14 months. NICH, in
contrast, does not regress and remains unchanged.
40.2.3 Kaposiform
Hemangioendothelioma
KHE is a rare neoplasm (1/100,000 children) that does not
metastasize. KHE is present at birth in 50% of patients, has an
equal sex distribution, and a ects the head/neck (40%), trunk
(30%), or an extremity (30%). The tumor is often greater than
5 cm and is reddish-purple. Seventy percent of patients have
Kasabach–Merritt phenomenon (KMP; thrombocytopenia <
25,000/mm 3, petechiae, bleeding). KHE partially regresses after
2 years of age, although it usually causes chronic pain and sti -
ness. MRI is indicated for diagnostic confirmation and to assess
the extent of the tumor.
40.2.4 Pyogenic Granuloma
PG is a solitary, red papule that grows rapidly on a stalk. It is
small, with an average diameter of 6 mm; the mean age of onset
is 6 years. PG commonly is complicated by bleeding (64%) and
ulceration (36%). PG involves the skin (88%) or mucous mem-
branes (11%). It is distributed on the head or neck (62%), trunk
(19%), upper extremity (13%), or lower extremity (5%). In the
head and neck region, a ected sites include cheek (29%), oral
cavity (14%), scalp (11%), forehead (10%), eyelid (9%), or lips (9%).
40.2.5 Capillary Malformation
CM (previously called “port-wine stain”) is the most common
type of vascular malformation. The lesion is noticed at birth
and can involve any area of the integument. Over time, the le-
sion progresses: (1) it darkens, (2) fibrovascular cobblestoning
can occur, (3) PGs may develop, and (4) soft-tissue and bony
may enlarge underneath the stain. The birthmark referred to as
an “angel kiss” or “stork bite” is a fading capillary stain. It is
present in one-half of Caucasian newborns and is located on
the forehead, eyelids, nose, upper lip, or posterior neck. No
treatment is necessary because it lightens over the first 2 years
of life.
40.2.6 Venous Malformation
Although VMs are present at birth, they may not become evi-
dent until childhood or adolescence when they have grown
large enough to cause a visible deformity or symptoms. Lesions
are blue, soft, and compressible. Hard, calcified phleboliths may
be palpable. The primary morbidity is psychosocial because
most lesions a ect the skin and cause a deformity. The second
most common complication is pain secondary to thrombosis
and phlebolith formation. Patients are not at risk for throm-
boembolism, unless a large phlebectatic vein is connected to
the deep venous system. VMs are diagnosed by history and
physical examination. Dependent positioning will cause a le-
sion to enlarge. Small, superficial VMs do not require further
diagnostic workup. Large or deep lesions are evaluated by MRI.
Approximately 10% of patients with VM have multifocal, fami-
lial lesions that are autosomal dominant: glomuvenous malfor-
mation (GVM), cutaneomucosal-venous malformation (CMVM),
or cerebral cavernous malformation (CCM).
Several phenotypical subtypes of VM exist. Blue rubber bleb
nevus syndrome (BRBNS) is characterized by multiple, small
VMs involving the skin, soft tissue, and gastrointestinal tract.
Morbidity is associated with gastrointestinal bleeding. Di use
phlebectasia of Bockenheimer specifies an extensive extremity
VM involving skin, subcutaneous tissue, muscle, and bone.
Sinus pericranii refers to a venous anomaly of the scalp or face
and transcalvarial communication with the dural sinus. Verru-
cous VM is clinically similar to a hyperkeratotic VM. Fibroadi-
pose vascular anomaly is di erentiated from intramuscular VM
by significant pain, contractures, and a solid component.
40.2.7 Lymphatic Malformation
LM is defined by the size of its channels: macrocystic, microcys-
tic, or combined. Lesions are usually noted at birth, although
small or deep LMs may not become evident until childhood or
adolescence after they have enlarged and/or become sympto-
matic. The most commonly a ected sites are the neck and
axilla. LM causes three major problems: (1) psychosocial
morbidity, (2) infection, and (3) bleeding. Macrocystic lesions
contain cysts large enough to be accessed by a needle (typi-
cally ≥5 mm) and are amenable to sclerotherapy. The most
commonly a ected sites are the neck or axilla. Microcystic LMs
have cysts that are too small to be cannulated by a needle and
thus cannot be treated by sclerotherapy. These lesions com-
monly a ect the face and extremities and are often associated
with cutaneous vesicles that can bleed and leak lymph fluid.
Approximately one-half of LMs are not purely macrocystic or
microcystic; they contain both macrocysts and microcysts. Pri-
mary lymphedema (a ecting 1/10,000 persons) is a type of LM.
Ninety percent of LMs are diagnosed by history and physical
examination. Small, superficial LMs do not require further diag-
nostic workup. Large or deep lesions are evaluated by MRI.
40.2.8 Arteriovenous Malformation
AVM has an absent capillary bed, which causes shunting of
blood directly from the arterial to venous circulation through a
fistula (direct connection of an artery to a vein) or nidus (abnor-
mal channels bridging the feeding artery to the draining veins).
Although present at birth, an AVM may not become evident
until childhood, after it has enlarged or become symptomatic.
Lesions have a pink-red cutaneous stain, are warm, and can have
palpable pulsations. Patients are at risk for pain, ulceration,
bleeding, and congestive heart failure. AVMs also can cause dis-
figurement, destruction of tissues, and obstruction of vital struc-
tures. Ninety percent of AVMs are diagnosed by history and
physical examination. Hand-held Doppler examination shows
fast flow. If the diagnosis is equivocal after history and physical
examination, ultrasound is the first-line study to confirm the
diagnosis. MRI usually is obtained to determine the extent of the
lesion.
Vascular Anomalies
385

Capillary malformation-arteriovenous malformation (CM-
AVM) is an autosomal-dominant condition. Patients have atypi-
cal CMs that are small, multifocal, round, pinkish-red, and often
surrounded by a pale halo (50%). One-third of patients also have
an AVM: Parkes Weber syndrome (PWS; 12%), extracerebral
AVM (11%), or intracerebral AVM (7%). A patient presenting
with multiple CMs, especially with a family history of similar
lesions, should be evaluated for a possible AVM. Because 7% of
patients with CM-AVM will have an intracranial fast-flow le-
sion, brain MRI should be considered. Exploratory imaging of
other anatomical areas is not necessary because extracranial
AVMs have not been found to involve the viscera.
40.3 Nonoperative Management
40.3.1 Infantile Hemangioma
Most IHs are observed because they are small, localized, and do
not involve anatomically important areas. During the prolifera-
tive phase, 16% ulcerate at a median age of 4 months; the lips,
neck, and anogenital region are the most common locations. To
protect against ulceration, IH in these areas should be kept
moist with hydrated petroleum during the proliferative phase
to minimize desiccation and shearing of the skin. If an ulcera-
tion develops, the wound is washed gently with soap and water
at least twice daily. Small, superficial areas are managed by the
application of topical antibiotic ointment and occasionally
with a petroleum gauze barrier. Large, deep ulcers require
damp-to-dry dressing changes. To minimize discomfort, a small
amount of topical lidocaine may be applied no more than four
times daily to avoid toxicity. Bleeding from an ulcerated IH is
usually minor, and is treated by applying direct pressure. Ulcer-
ations will heal with local wound care within 2 to 3 weeks.
40.3.2 Topical Pharmacotherapy
Topical corticosteroid is relatively ine ective, especially if IH
involves the deep dermis and subcutis. Although lightening
may occur, if there is deep component it will not be a ected.
Adverse e ects include hypopigmentation and cutaneous atro-
phy. Topical timolol may be e ective for superficial lesions, but
will not a ect IHs with a subcutaneous component.
40.3.3 Intralesional Corticosteroid
Problematic IHs that are well localized and less than 3 cm are
best managed by intralesional corticosteroid (Fig. 40.4). Tri-
amcinolone (not to exceed 3 mg/kg) will stop the grow th of the
lesion; two-thirds will decrease in size. The corticosteroid lasts
2 to 3 weeks, and thus infants may require two to three injec-
tions during the proliferative phase.
40.3.4 Systemic Pharmacotherapy
Problematic IHs greater than 3 cm are managed by oral predni-
solone or propranolol (Fig. 40.5). Prednisolone has been used
Fig. 40.4 Management of a localized proliferating infantile hemangioma with intralesional corticosteroid. (a) Three months of age at time of kenalog
injection. (b) Accelerated regression of the tumor at 4 months of age. (c) Age 8 months. (d) Age 12 months.
Fig. 40.5 Treatment of a large proliferating infantile hemangioma with oral prednisolone. Note accelerated regression of the tumor.
Integument
386

to treat IH for over 40 years and has proven to be very safe and
e ective. Patients are given 3 mg/kg/d for 1 month; the drug is
then tapered by 0.5 mL every 2 to 4 weeks until it is discontin-
ued between 10 and 12 months of age when the tumor is no
longer proliferating. The drug is given once a day in the morn-
ing, and infants have monthly outpatient follow-up. Using this
protocol, all tumors will stabilize in growth and 88% will
become smaller (accelerated regression). Twenty percent of
infants will develop a cushingoid appearance that resolves dur-
ing tapering of therapy. Approximately 12% exhibit decreased
gain in height, but return to their pretreatment growth curve
by 24 months of age.
Propranolol dosing typically is 2 mg/kg/d. Approximately 90%
of tumors will stop growing or regress. Risks (<3%) include
bronchospasm, bradycardia, hypotension, hypoglycemia, seiz-
ures, and hyperkalemia. Preterm infants and those less than 3
months of age are more likely to have adverse events. Patients
usually have cardiology consultation, electrocardiogram, echo-
cardiogram, glucose/electrolyte measurements, and frequent
blood pressure, heart rate, and respiratory examinations. Inpa-
tient initiation of treatment is used for premature or infants
less than 3 months of age. Potential contraindications include
asthma, glucose abnormalities, heart disease, hypotension, bra-
dycardia, PHACES association. The drug should be discontinued
if the infant is ill because reduced oral intake can increase the
risk of hypoglycemia and seizures. Patients treated with propra-
nolol experience later rebound growth compared to predniso-
lone, and thus may necessitate longer treatment. Recently,
concern has been raised about potentially negative long-term
neurocognitive e ects of propranolol when given to infants.
40.3.5 Laser Therapy
Pulsed dye laser treatment generally is not indicated for prolif-
erating IH. The laser penetrates only approximately 1 mm
into the dermis, and thus only a ects the superficial portion of
the tumor. Although lightening may occur, the mass is not
a ected. Patients have an increased risk of skin atrophy, ulcer-
ation, pain, bleeding, scarring, and hypopigmentation. Pulsed
dye laser is indicated during the involuted phase to fade resid-
ual telangiectasias.
40.3.6 Congenital Hemangioma
RICH usually does not require intervention during infancy
because it regresses quickly. Rarely, it can cause congestive
heart failure, which is treated with corticosteroid or emboliza-
tion. NICH is rarely problematic in infancy and is observed.
40.3.7 Kaposiform
Hemangioendothelioma
Most lesions are extensive and are unable to be resected. Vin-
cristine is first-line therapy; the response rate is 90%. Recently,
patients have been treated with sirolimus with good e cacy.
Thrombocytopenia is not significantly improved with platelet
transfusion, which should be avoided unless there is active
bleeding or a surgical procedure is planned. By 2 years of age,
the tumor has undergone partial involution and the platelet
count normalizes.
40.3.8 Pyogenic Granuloma
Because PG frequently bleeds, lesions are covered to avoid inci-
dental trauma. Bleeding is controlled by application of pressure.
Rarely, a PG can “fall o ” and not recur. However, almost all
lesions require resection.
40.3.9 Capillary Malformation
Patients with CMs in nonvisible locations may not elect for in-
tervention. The mainstay of treatment is pulsed dye laser,
which lightens its color. Intervention during infancy or early
childhood is recommended because (1) superior lightening of
the lesion is achieved, (2) the risk of darkening and hypertrophy
is reduced, and (3) psychosocial morbidity is minimized. Pulsed
dye laser is less e ective for CMs that have progressed to a dark
color with cutaneous thickening.
40.3.10 Venous Malformation
VM can be observed; intervention is reserved for symptomatic
lesions or asymptomatic areas at risk for thromboembolism.
Patients with an extensive extremity VM are prescribed cus-
tom-fitted compression garments. Individuals with recurrent
pain secondary to phlebothrombosis are given aspirin to pre-
vent thrombosis. Disseminated intravascular coagulopathy
(DIC) can occur following traum a or interventions. Chronic
consumptive coagulopathy can cause thrombosis (phlebo-
liths) or bleeding (hemarthrosis, hem atoma, intraoperative
blood loss). Low molecular weight heparin is considered for
patients at risk for DIC. Individuals who develop a serious
thrombotic event require long-term anticoagulation or a vena
caval filter.
40.3.11 Lymphatic Malformation
LM is benign and thus intervention is not mandatory. Intrale-
sional bleeding is treated with pain medication. Patients with
more than three infections a year are given prophylactic antibi-
otics. First-line management for problematic macrocystic/com-
bined LM is sclerotherapy. This technique involves aspiration of
the cysts followed by the injection of an inflammatory sub-
stance, which causes scarring of the cyst walls to each other.
Sclerotherapy shrinks the lesion and alleviates symptoms; it
gives superior results and has lower morbidity compared to
resection. Oral sirolimus is now being used to treat problematic
microcystic LMs that have failed or are not amenable to other
interventions.
40.3.12 Arteriovenous Malformation
AVM does not necessarily require intervention. Because the le-
sion is often di use and involves multiple tissue planes, cure is
rare. An asymptomatic AVM should be observed unless it can
be removed for possible cure with minimal morbidity.
Vascular Anomalies
387

40.4 Operative Treatment
40.4.1 Infantile Hemangioma
Resection generally is not recommended during the proliferat-
ing phase. The tumor is highly vascular, and there is a risk for
blood loss, iatrogenic injury, and an inferior outcome, compared
to excising residual tissue after the tumor has regressed.
Approximately 50% of IH leave behind fibrofatty tissue or dam-
aged skin after the tumor regresses, causing a deformity. It is
preferable to intervene surgically between 3 and 4 years of age
(Fig. 40.6). During this period, the IH will no longer improve
significantly, and the procedure is performed before the child’s
long-term memory and self-esteem begin to form at about 4
years of age. Also, waiting until the IH is at its smallest will result
in the shortest possible scar and the most favorable outcome.
Circular lesions located in visible areas, particularly the face, are
best removed by circular excision and purse-string closure. This
technique minimizes the length of the scar as well as distortion
of surrounding structures. Lenticular excision of a circular IH
results in a scar as long as three times the diameter of the lesion.
In comparison, a two-stage circular resection followed by lentic-
ular excision/linear closure will leave a scar approximately the
same length as the diameter of the original lesion.
40.5 Congenital Hemangioma
After regression, RICH can leave behind atrophic skin and subcu-
taneous tissue. Reconstruction with autologous grafts (fat, der-
mis) or acellular dermis may be indicated. Resection of NICH
may be indicated to improve the appearance of the a ected area,
as long as the surgical scar will be less noticeable than the lesion.
40.5.1 Kaposiform
Hemangioendothelioma
KHE typically involves multiple tissue planes and is too di use
to resect. Rarely, if a lesion is localized and amenable to
operative intervention, excision should be considered to obvi-
ate the need for systemic chemotherapy.
40.5.2 Pyogenic Granuloma
PGs require intervention to control likely ulceration and bleed-
ing. Because PG extends into the reticular dermis, the lesion
usually is out of the reach of the pulsed dye laser, cautery, or
shave excision. Consequently, these modalities have a recur-
rence rate of approximately 50%. Full-thickness excision is
definitive treatment. Superficial, small lesions can be treated
with needle-tip cautery through the reticular dermis.
40.5.3 Capillary Malformation
CMs typically do not require operative intervention. Surgical
procedures are indicated to correct overgrowth caused by the
malformation (Fig. 40.7). Small fibrovascular nodules or PGs
can be excised. Trunk or extremity CM associated with
increased subcutaneous adipose tissue can be improved with
suction-assisted lipectomy. Severe cutaneous thickening and
cobblestoning may be resected and reconstructed by linear clo-
sure, skin grafts, or local flaps. Malocclusion can be corrected in
adolescence with orthodontics; an orthognathic procedure is
considered when the jaws are completely grown. Facial asym-
metry caused by overgrowth of the zygoma, maxilla, or mandi-
ble can be improved by contour burring.
40.5.4 Venous Malformation
First-line treatment is sclerotherapy, which is safer and more
e ective than resection ( Fig. 40.8). Di use lesions are man-
aged by targeting specific symptomatic areas. Sclerotherapy is
repeated until symptoms are alleviated or when injectable vas-
cular spaces are no longer present. Although sclerotherapy
e ectively reduces the size of the lesion and improves symp-
toms, the malformation remains. Consequently, patients may
have a mass or visible deformity after treatment that may be
Fig. 40.6 Resection of infantile hemangioma. It is preferable to wait until the tumor has regressed (>3 years of age) before operative intervention. (a)
Age 1 year. (b) Age 3 years at time of resection. (c) Following removal of the involuted lesion.
Integument
388

Fig. 40.7 Management of a capillary malformation with pulsed dye laser and transverse mucosal resection of the left lower lip. (a) Prior to
intervention. (b) Postoperative appearance.
Fig. 40.8 Intervention for venous malformation.
(a-c) Adolescent female with new onset swelling
of the left mandible managed with sclerotherapy.
(d,e) Resection of a localized venous malforma-
tion of the scalp.
Vascular Anomalies
389

improved by resection. VM usually re-expands after sclerother-
apy, and thus patients often require additional treatments. Our
preferred sclerosant is sodium tetradecyl sulfate. Most patients,
especially children, are managed under general anesthesia
using imaging.
In contrast to sclerotherapy, resection is rarely primary treat-
ment because (1) the entire lesion is di cult to remove; (2) the
risk of recurrence is high because hidden channels adjacent to
the visible lesion are not excised; (3) the risk of blood loss and
iatrogenic injury is greater. Resection should be considered for
(1) small, well-localized lesions that can be completely removed
or (2) persistent mass or deformity after completion of sclero-
therapy (patent channels are no longer accessible for further
injection). When considering resection, the postoperative scar/
deformity should be weighed against the preoperative appear-
ance of the lesion. Subtotal resection of a problematic area is
indicated rather than attempting “complete” excision of a
benign lesion that might result in a worse deformity than the
malformation itself. Almost all VMs should have sclerotherapy
prior to operative intervention. After adequate sclerotherapy,
the VM is replaced by scar and thus the risk of blood loss, iatro-
genic injury, and recurrence is reduced. In addition, fibrosis
facilitates resection and reconstruction.
40.5.5 Lymphatic Malformation
Sclerotherapy is first-line management for large or problematic
macrocystic/combined LM (we prefer doxycycline; Fig. 40.9).
Recently, bleomycin sclerotherapy has shown e cacy for
microcystic LMs. This modality can be considered for problem-
atic lesions where resection would be unfavorable; a 10 to 25%
reduction in the size of the lesion can be expected. Cutaneous
LMs that are bleeding or leaking lymph fluid can be treated
with carbon dioxide laser. Radiofrequency ablation can improve
intraoral lymphatic vesicles.
Resection is reserved for (1) symptomatic microcystic LM
causing bleeding, infection, distortion of vital structures, or sig-
nificant deformity; (2) symptomatic macrocystic/combined LM
that no longer can be managed with sclerotherapy because all
macrocysts have been treated; (3) small, well-localized LM
Fig. 40.9 Intervention for lymphatic malformation. (a,b) Child with a macrocystic lesion of the left upper eyelid treated with sclerotherapy. (c,d)
Resection of a microcystic lymphatic malformation of the lower extremity.
Integument
390

(microcystic or macrocystic) that may be completely excised.
When considering resection, the postoperative scar/deformity
should be weighed against the preoperative appearance of the
lesion. For di use LMs, staged resection of defined anatomic
areas is recommended. Subtotal excision of problematic areas,
such as bleeding vesicles or a hypertrophied lip, should be car-
ried out rather than attempting to “complete” resection that
might result in a worse deformity than the malformation itself.
Bleeding or leaking cutaneous vesicles can be controlled by
resection if they are localized. Large areas of vesicular bleeding
or drainage are best managed by sclerotherapy or carbon diox-
ide laser; alternatively, wide resection and skin graft coverage is
required. Microcystic vesicles involving the oral cavity respond
well to radiofrequency ablation.
40.5.6 Arteriovenous Malformation
Embolization is generally the first-line treatment. The proce-
dure involves the delivery of a substance through a catheter
into the AVM nidus to occlude blood flow and/or fill a vascular
space. Scarring reduces arteriovenous shunting, shrinks the
lesion, and diminishes symptoms. Embolization is used either
as a preoperative adjunct to resection or as monotherapy for
lesions not amenable to extirpation. Despite the high likelihood
of re-expansion, embolization can e ectively palliate an AVM
by reducing its size, slowing enlargement, and alleviating pain
and bleeding. The embolic material is delivered to the nidus,
not to the proximal arterial feeding vessels. Occlusion of inflow
will cause collateralization and expansion of the AVM; access to
the nidus also will be blocked, preventing future embolization.
Resection of AVM has a lower recurrence rate than emboliza-
tion alone; it is considered for a well-localized lesion or to
correct deformity (i.e., bleeding or ulcerated areas, labial hyper-
trophy; Fig. 40.10). Wide extirpation and reconstruction of
large, di use AVM should be exercised with caution because (1)
cure is rare and the recurrence rate is high; (2) the resulting
deformity is often worse than the appearance of the malforma-
tion; (3) resection is associated with significant blood loss,
iatrogenic injury, and morbidity. When excision is planned, pre-
operative embolization will facilitate the procedure by reducing
the size of the AVM, minimizing blood loss, and creating scar
tissue to aid the dissection. Excision should be done 24 to 72
hours after embolization, before recanalization restores blood
flow to the lesion.
40.6 Vascular Malformation
Overgrow th Syndromes
CLOVES Syndrome (Congenital Lipomatosis Overgrow th, Vascu-
lar malformations, Epidermal nevi, and Scoliosis)
All patients have a truncal lipomatous mass, a slow-flow
vascular malformation (most commonly a CM overlying the
lipomatous mass), and hand/foot anomalies (increased width,
macrodactyly, first webspace sandal gap). Patients also may
have AVM (28%), neurological impairment (50%), or scoliosis
(33%).
40.6.1 Klippel–Trénaunay Syndrome
This is a capillary-lymphatic-VM of an extremity causing over-
growth. The condition almost always a ects the lower limb. A
large, embryonic vein in the subcutaneous tissue isolated in the
lateral calf/thigh and communicates with the deep venous sys-
tem. Complications include thrombophlebitis and pulmonary
embolism. The lymphatic abnormalities are usually macrocystic
in the pelvis/thigh and microcystic in the abdominal wall, but-
tock, and distal limb. Because embryonal veins can connect to
Fig. 40.10 Resection of an arteriovenous malformation. Child with a bleeding, ulcerated lesion of the scalp managed with preoperative embolization
followed by excision.
Vascular Anomalies
391

the deep venous system causing thromboembolism, they are
removed in early childhood with sclerotherapy or endovascular
laser.
40.6.2 Parkes Weber Syndrome
PWS consists of a di use AVM of an extremity (usually the leg)
causing soft-tissue and/or bony overgrowth. A CM involves the
skin of the a ected limb. Patients have subcutaneous and intra-
muscular microshunting and can develop congestive heart fail-
ure. Most children are observed until symptoms necessitate
intervention. Embolization may reduce congestive heart failure,
pain, or ulceration. Occasionally, amputation is necessary.
40.6.3 Phosphatase and Tensin
Homolog Hamartoma-Tumor Syndrome
Patients with phosphatase and tensin homolog (PTEN) muta-
tions, a tumor suppressor gene, have PTEN hamartoma-tumor
syndrome (PHTS), an autosomal dominant condition. Approxi-
mately 50% of patients have a unique fast-flow vascular anom-
aly with arteriovenous shunting, referred to as a PTEN-
associated vascular anomaly. PHTS also is associated with
autism (19%), thyroid lesions (31%), or gastrointestinal polyps
(30%).
40.6.4 Sturge–Weber Syndrome
The Sturge–Weber syndrome (SWS) is defined by (1) a CM in
the V1 trigeminal nerve distribution with either (2) ocular
abnormalities (glaucoma, choroidal vascular anomalies) and/or
(3) a leptomeningeal vascular malformation. Pial vascular
lesions may cause refractory seizures, hemiplegia, and /or
delayed cognitive development. Any child with a CM in an
upper trigeminal nerve distribution should be screened for
SWS. MRI is obtained to rule out leptomeningeal vascular
lesions. Patients undergo ophthalmology evaluation to assess
for choroidal anomalies and glaucoma.
40.7 Conclusion
The field of vascular anomalies is confusing because lesions
look similar and terminology is di cult. More than 90% of
patients can be diagnosed by history and physical examination.
Treatment of a vascular anomaly is not considered until the
diagnosis is confirmed. An experienced physician can manage
most vascular anomalies independently. However, many lesions
require interdisciplinary care and should be referred to a vascu-
lar anomalies center. Although vascular tumors are at least 10
times more common than vascular malformations, vascular
malformations are generally more problematic and comprise
two-thirds of patients managed in a vascular anomalies center.
If the clinician initially focuses on the major types of vascular
anomalies, then he/she will be able to manage approximately
95% of patients correctly (Table 40.4).
40.8 Key Points
● Vascular anomalies are divided into tumors or malformations.
● IH is the most common tumor of infancy; it grows rapidly
after birth and involutes during childhood.
● Most IHs are observed; problematic lesions are treated phar-
macologically or by resection.
● Vascular malformations are present at birth, although not
always obvious; they may slowly enlarge during childhood
and adolescence.
● Vascular malformations are managed by observation, laser,
sclerotherapy, embolization, or resection; pharmacotherapy
is not available.
Suggested Readings
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants
and children: a classification based on endothelial characteristics. Plast
Reconstr Surg. 1982; 69(3):412–422
Chang LC, Haggstrom AN, Drolet BA, et al. Hemangioma Investigator Group.
Growth characteristics of infantile hemangiomas: implications for manage-
ment. Pediatrics. 2008; 122(2):360–367
Table 40.4 Management summary for the major types of vascular anomalies
Tumors

Malformations
Biological name

Treatment

Biological name

Treatment
Infantile hemangioma

Observe
Topical timolol
Kenalog injection
Oral prednisolone
Oral propranolol
Resection
Capillary malformation

Observe
Laser
Resection
Congenital hemangioma

Observe
Resection
Lymphatic malformation

Observe
Sclerotherapy
Resection
Kaposiform hemangioendothelioma Vincristine
Sirolimus
Venous malformation

Observe
Sclerotherapy
Resection
Pyogenic granuloma

Resection

Arteriovenous malformation

Observe
Embolization
Resection
Integument
392

Hassanein AH, Mulliken JB, Fishman SJ, Greene AK. Evaluation of terminology
for vascular anomalies in current literature. Plast Reconstr Surg. 2011; 127
(1):347–351
Greene AK, Liu AS, Mulliken JB, Chalache K, Fishman SJ. Vascular anomalies in
5,621 patients: guidelines for referral. J Pediatr Surg. 2011; 46(9):1784–1789
Greene AK, Couto RA. Oral prednisolone for infantile hemangioma: e cacy
and safety using a standardized treatment protocol. Plast Reconstr Surg.
2011; 128(3):743–752
Couto RA, Maclellan RA, Zurakowski D, Greene AK. Infantile hemangioma:
clinical assessment of the involuting phase and implications for management.
Plast Reconstr Surg. 2012; 130(3):619–624
Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol
for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;
131(1):128–140
Couto JA, Greene AK. Management of problematic infantile hemangioma
using intralesional triamcinolone: e cacy and safety in 100 infants. J Plast
Reconstr Aesthet Surg. 2014; 67(11):1469–1474
Vascular Anomalies
393

41 Burns
Matthias B. Donelan and Branko Bojovic
Sum m ary
Reconstruction of pediatric burns have improved recently. Ma-
nagement is based on the principles of wound healing and scar
maturation. Tissue can be manipulated with topical and intrale-
sional modalities, local tissue rearrangements, grafts, and
photomedicine.
Keywords: pediatric, burn, scar, reconstruction, skin graft, laser
41.1 Introduction
There has never been a more exciting and optimistic time in
the history of pediatric burn therapy than that exists today in
the developed world. Current therapeutic options to treat chil-
dren who sustain burn injuries of all varieties have now reached
a state of e ectiveness that was unimaginable only a few short
decades ago. Progress in the understanding of wound healing,
improved diagnosis through technological advances, vastly
superior critical care, as well as new and refined techniques of
reconstructive surgery make the care of pediatric burns
and restoration of the patients’ quality of life an exciting and
rewarding field of pediatric plastic surgery. Because burns can
involve all areas of the body and vary in size from the very small
to almost the entire body surface area, they require a com-
pletely competent and caring plastic surgeon to treat the whole
patient, the whole family, and the whole range of deformities
that can be encountered. A few salient strategic concepts, how-
ever, are worth emphasizing at the beginning of this chapter.
Acute burn injuries that are not life-threatening should be
cared for with the most conservative burn wound management
possible. Early excision and grafting commits a patient to a
donor site and a grafted outcome that is iatrogenic and perma-
nent ( Fig. 41.1). When necessary, to prevent serious compli-
cations, such early surgical intervention is essential. When not
necessary, it is harmful for the patient. Improved diagnostic
techniques such as laser Doppler imaging have been validated
in multiple large studies and should be incorporated in the care
of all pediatric burn injuries if at all possible. The overwhelming
consensus from recent literature reports originated in the
United Kingdom and Belgium has demonstrated that following
the adaptation of laser Doppler imaging, the incidence of early
excision and grafting in pediatric plastic surgery units has sig-
nificantly decreased. Early excision and grafting has been advo-
cated in smaller burns because of an alleged superior outcome
and prevention of hypertrophic scarring. Profound advances in
our ability to prevent and treat hypertrophic scarring as well as
combining local plastic surgery techniques and the use of lasers
and other photomedicine have allowed us to regenerate scars
in ways that were inconceivable as recently as the 1990s. Today,
a patient’s hypertrophic, contracted scars are often their most
valuable reconstructive donor sites. The reason for that is that
nothing beats “original equipment,” and their own scarred
tissue is potential reconstructive material in the right location
with the best possible color and texture match. It also does not
require the creation of an iatrogenic donor site deformity on an
uninjured part of the patient’s body ( Fig. 41.2). The ability to
help scars to regenerate themselves is an essential part of
pediatric burn care and reconstruction.
41.2 Diagnosis
Burn deformities present unique and complex reconstructive
challenges for the pediatric plastic and reconstructive surgeon.
A thorough understanding of the burn wound and its evolution
is critical to successful treatment outcomes and is part of sound
surgical judgment and expertise. Burn care can be considered
as one of the most challenging areas of medicine because it
involves multiple medical and surgical treatment modalities
Fig. 41.1 A 4-year-old female who sustained a small, mixed second-
degree scald burn at age 18 months. Early excision and meshed skin
grafts resulted in a permanent, severe cosmetic deformity.
Integument
394

implemented over the course of time, frequently many years. It
is beyond the scope of this chapter to discuss acute burn care,
but su ce to say that well-described methods for recognizing
burn injury from a number of modalities are replete within the
literature. Thermal, electrical, and chemical injuries form the
basis of the majority of the causative factors for acute burn
injuries.
Burns, in general, constrict and deform and in doing so alter
contour, proportions, features, form, and function. Burn care
should strive to restore the injured patient as much as possible
to their preburn condition and as close to “norm al” as is possi-
ble with minimal morbidity (Fig. 41.3). A return to normal,
that is, the way in which the patient existed prior to the burn, is
not possible. This must be clearly communicated to the patient
and his or her loved ones so that they understand that the jour-
ney of burn care and reconstruction is one that sets realistic
expectations and goals in treatment and o ers the hope that
we can get such patients to “near-normal.” Such an approach is
Fig. 41.2 (a) A 4-year-old female from Mongolia who sustained second- and third-degree flame burns at age 2 in yurt (ger) fire. Wounds healed by
contraction and epithelialization, resulting in severe contractures and hypertrophic scarring. (b) Contractures were relieved by Z-plasties using the
contracted, hypertrophic scar tissue. (c) Three years after three Z-plasty procedures and six treatments with the fractionated CO2 laser and topical
triamcinolone (10 mg/mL). The contractures are completely released and normal contour is restored.
Fig. 41.3 (a) An 11-year-old female 18 months following a flash burn to the face resulting in diffuse hypertrophic scarring, contractures, and
ulceration, as well as severe pruritus. (b) Contractures were relieved and contours leveled with multiple Z-plasties during four separate procedures over
6 years. Scars were also treated simultaneously nine times with the pulsed dye laser and six times with the fractionated, ablative CO2 laser. No scars
were excised. (c) Six years after initial treatment, her face is normal except for slight textural irregularity and mild dyspigmentation.
Burns
395

realistic, responsible, and still o ers optimism and hope to the
patient, and family, that he or she can look forward to a return
as close to the preburn injury condition as is possible, save for
what the future may hold with regenerative medicine or tech-
nological advancements on the horizon.
The ultimate goal of burn reconstruction should be the resto-
ration of an aesthetically pleasing and absolutely tension-free
appearance to allow for appropriate form and function. The
keys to achieving that goal are to appropriately diagnose the
burn scar deformity, understand what issues are preventing
that goal, and then provide a sound plan to address the prob-
lems. In concept, this sounds simple, but in practice it requires
expertise and resources to make this goal a reality. Controversy
still exists on what the optimum treatment is for moderate to
severe burns. The vast majority of burns treated conservatively
with topical antibiosis should heal in approximately 3 weeks.
Burns that are obviously full thickness are best treated by early,
tangential excision and grafting within 7 to 10 days. Even for
controversial areas, the majority of burns can and are treated
expectantly in most pediatric burn centers with a combination
of modalities, which will be addressed later in this chapter.
Several useful generalizations regarding diagnosis and cor-
rect characterization of burns are important to mention and
keep in mind. Superficial second-degree burns typically heal
without scarring or pigmentary changes to the skin. Deeper or
medium-thickness second-degree burns that epithelialize in 10
to 14 days will often heal without scarring although long-term
changes in skin pigmentation and texture are frequent occur-
rences. Deep second-degree burns that epithelialize in 14 to 28
days or longer require careful observation; they are prone to
develop severe late hypertrophic scarring. These patients, in
particular, require close and continuous follow-up after the
initial healing phase and must be managed via all available
ancillary modalities, including appropriately timed surgical in-
tervention when indicated.
41.3 Nonoperative Management
Whether we are considering nonoperative or operative treat-
ment of burns, both modalities are based on an overall strategy
and understanding of the specific problem at hand. This was
briefly mentioned already in the “Diagnosis” section. Many
techniques, including a plethora of surgical ones, have been
described in the literature to address burns of many varieties
and depths. What is most important is that in all cases, funda-
mental principles are recognized and followed such that the
goals of management and treatment are arrived at in a predict-
able fashion. Therefore, developing a strategic concept of burn
treatment is paramount and will help avoid unfortunate results.
Deep second- and third-degree burns heal by contraction
and epithelialization and the deeper the injury, the more heal-
ing takes place by contraction. This category of burn depth will
be expanded upon in the operative treatment section to follow
as these typically do not do well without operative manage-
ment. More superficial burns, as mentioned previously, require
patience, observation, and providing the wounded area a milieu
to allow for rapid, infection-free re-epithelialization. This is typ-
ically accomplished by good hygiene of the area, judicious pain
control, and local wound care with a combination of modalities
that include antibiotic topical creams or gels to more sophisti-
cated wound dressings that are applied and changed daily or
every several days while awaiting wound re-epithelialization.
Typically, these burns wounds are those which heal within days
to less than 2 weeks. Once the wound is re-epithelialized and
risk of infection has passed, other well-described modalities
exist for adjunctive therapy. Pressure garments have long been
demonstrated to be e ective in suppressing and reversing
hypertrophic scarring. The addition of silicone to pressure
therapy has also been a significant advance. Judicious use of
sunscreen on immature and newly re-epithelialized burn scars
is mandatory and typically should last a year as the scar under-
goes maturation and is prone to hyperpigmentation from
ultraviolet (UV) radiation. Sunscreen, in general, should be
encouraged and recommended as part of a daily program of
skin protection and treatment, regardless of burn depth and as
part of normal pediatric skin care and skin maintenance.
41.4 Operative Treatment
As discussed earlier, a clear understanding of the pathophysiol-
ogy responsible for a chronic open burn wound or burn scar
contracture is essential to be able to devise an operative strat-
egy to treat it. Several fundamental concepts and techniques
are important to discuss prior to describing specific operative
interventions: contractures, contour and proportion, tension,
donor site availability, aesthetic units, Z-plasty, grafts, flaps,
and lasers. These nine basic treatment strategies must be
understood in order to have a comprehensive overview of oper-
ative pediatric burn wound management and treatment.
41.4.1 Contractures
Burn injury leads to tissue loss and the resultant wounds heal
with contraction, which results in contractures. Contractures
are either “intrinsic” or “extrinsic.” Intrinsic contractures are
those that result from loss of tissue in the area of the contrac-
ture with subsequent distortion of the part a ected. Extrinsic
contractures include those in which the tissue loss is at a dis-
tance from the a ected area, but the distorted structures are
themselves not injured. It is critical to recognize these di er-
ences given that they are often overlooked in burn scar and
burn injury treatm ent. Not only must corrective measures be
directed at the cause of the contracture to prevent iatrogenic
deformities, but it is also essential to minim ize the amount of
skin and scar that are excised when dealing with contrac-
tures. Minimizing excision decreases the amount of new skin
that must be provided. In fact, due to the beneficial e ects of
releasing tension, most scars will mature favorably once a
release has been carried out ( Fig. 41.2 and Fig. 41.3). It is
important to understand that scars are living things, not
static entities, and that they will respond to a change in their
environment.
Integument
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41.4.2 Contour and Proportion
Contractures are the most common result of burn injuries
and the tension generated by the contractures alters contour
and proportion. Contour is a ected by hypertrophic scarring
bowstringing across concave surfaces, and compression of
the subtle convex and concave surfaces that compose normal
human anatomy. Tension, in addition, shifts mobile structures,
particularly facial features, in ways that adversely a ect normal
proportions. The e ect of this can be devastating as demon-
strated in Fig. 41.4. Profound improvement can be accom-
plished by restoring normal facial proportion and the normal
shape of features despite the persistence of extensive facial
scarring. These facial proportions are well defined in the litera-
ture and can be further studied in the works listed in the “Sug-
gested Readings” section, given that it is beyond the scope of
this chapter. Focusing on treating burn scars can be counter-
productive. Emphasizing the restoration of normal anatomic
proportions and relationships is uniformly rewarding when
accomplished. If they are not restored, the patient’s appearance
will always be displeasing to the eye, even if the amount of
actual physical scarring has been decreased by replacing it by
either graft or flap material.
41.4.3 Tension
There is a well-recognized phenomenon of “invisible skin loss”
associated with medium second-degree depth or deeper burns
resulting in a tightening e ect, especially for burns of the face
or the dorsum of the hand. Such tension may be obvious when
examining deforming contractures of mobile soft tissues such
as the eyelids or lips but only subtle in its compression of soft-
tissue contours. Relaxed scars are indeed happy scars. This can
be easily observed in scars under tension that are erythema-
tous, hypertrophic, pruritic, painful, and tender. Once tension is
relieved, such scars will respond in kind and become flattened,
softer, paler, and less symptomatic ( Fig. 41.2 and Fig. 41.3).
Relief of tension is a practical, achievable, and essential compo-
nent of operative treatment of burn scar injury.
41.4.4 Donor Sites
Burn scar deformities rarely exist in isolation and are often
associated with extensive burns of the patient’s entire body.
This presents a major di culty in burn reconstruction as donor
site access may be limited. The principles already set forth
allow for intelligent decision-making when considering choice
of procedure and the judicious use of available donor sites. This
also applies to the choice of reconstructive modality as it relates
to either graft or flap variety as that will have an e ect on
successful outcome. It cannot be overstated that each burn
reconstructive case is unique and operative treatment must be
individualized.
41.4.5 Aesthetic Units
The concept of aesthetic units has had a profound e ect on
plastic surgical thinking in reconstructive surgery, especially as
it relates to burn reconstruction. It is critical to keep in mind
aesthetic units during reconstructive operative treatment for
burns; however, it should not overtake common sense. Excision
of normal skin is rarely indicated and scar revisions with Z-
plasties can allow for profound changes that camouflage scars.
The sites of treatment can then be returned to more propor-
tional, well-contoured, and tension-free units that allow for
return of form and function to near baseline levels. The elimina-
tion of visible scarring is not realistic and such a focused pursuit
of an elusive goal can and will lead to unfortunate outcomes
and significant iatrogenic trauma.
Fig. 41.4 (a) Severe, type II, pan-facial burn
deformity in this 5-year-old female with signifi-
cant loss of facial anatomy after a 70% total body
surface area flame burn in a house fire. (b) After
restoration of normal facial proportions, normal
contours, and a normal shape to the facial
features, her face is attractive despite her scars.
The fractional CO2 laser would greatly further
improve her appearance today.
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397

41.4.6 Z-Plasty
The Z-plasty is well described for its ability to lengthen linear
scars by recruiting relatively lax adjacent lateral tissue and is an
essential and powerful tool in operative burn scar treatment. As
mentioned previously, its use on a scar causes a profound, ben-
eficial e ect on the physiology of the scar. This is consistent
with the principle that scars are living things and not static
entities. A properly performed Z-plasty accomplishes two
important goals: (1) it decreases longitudinal tension on the
scar and (2) it decreases the width of the scarred area. When a
properly designed Z-plasty is used, especially a 60-degree limb
Z-plasty, this will theoretically lengthen a scar by 75% and nar-
row it by approximately 30% (Fig. 41.5). It will also help to
camouflage the scar by making its borders more irregular,
thereby making it less conspicuous to the human eye. In fact,
after a successful Z-plasty, hypertrophic scars resolve rapidly,
elasticity improves, and significant scar narrowing is accom-
plished (Fig. 41.6). Most importantly, even though grafts and
flaps can and do accomplish a similar goal of relieving tension,
the use of the Z-plasty does so in a simple, elegant, powerful,
and much less morbid and technically di cult fashion. When
incising Z-plasty flaps, general principles include that the tips
should be cut perpendicular to the central limb for a short dis-
tance to add additional tissue to the flap tips and improve blood
supply. Also, a Z-plasty should be designed so that once the
transposition of the flaps is completed the tight transverse limb
of the Z-plasty is located where a normal concavity would
occur. Please refer to the patient examples for demonstration of
the use of Z-plasty in representative selected cases.
41.4.7 Grafts
It is well established that skin grafts are critical as a tool in
burn reconstruction. We have already discussed about consid-
erations with regard to donor site selection, but a few
generalizations will be further helpful as it relates to some addi-
tional facets of graft choice and use. Grafts can be divided into
split thickness and full thickness, and an entire discussion
about the indications and merits of one versus the other is
beyond the scope of this chapter. Some generalizations, how-
ever, are appropriate to mention. Split-thickness skin grafts
contract more, have more propensity to wrinkle, and typically
will remain shiny with a “glossy finish” look to them. Split-
thickness skin grafts are usually the best alternative in many
di erent areas of the body to relieve broad areas of contracture
that are not suitable for reconstruction with Z-plasty or local
flap tissue rearrangement. Even on the face, when used in
inconspicuous locations, split-thickness grafts can decrease ten-
sion on hypertrophic scaring and enhance scar maturation
(Fig. 41.7). Caution must be used in split-thickness skin graft-
ing in dark-skinned patients because hyperpigmentation is a
problem that is encountered frequently. Full-thickness skin
grafts contract less, have a matte finish, often retain skin appen-
dages, and are therefore the ideal skin replacement if the recip-
ient bed is adequate and the price of the donor site is worth the
potential improvement. Full-thickness skin grafts are reliable
“workhorses” for facial burn reconstruction, particularly when
resurfacing broad, conspicuous areas of the face in aesthetic
units ( Fig. 41.8). Unlike flaps, over time these grafts allow for
excellent facial expression. Treatment with the fractional CO2
laser makes the grafts feel thinner and more elastic, further
improving their advantages over flaps on the face. When using
full-thickness grafts for facial reconstruction, all contractures
should be eliminated, the defect overcorrected, and larger grafts
than one might think necessary must be used. Normal skin
should never be excised in burn patients as the junctional
suture lines are easily revised with Z-plasties and laser therapy,
as demonstrated in this patient, and the fundamental problem
is a skin deficiency. Additionally, postoperative management
with conformers and pressure therapy is particularly essential
when using these grafts.
Fig. 41.5 The Z-plasty in burn scars relieves
tension by lengthening the scar. It also can used
to narrow the scar, level the scar, and make it less
conspicuous by creating irregular borders.
Integument
398

41.4.8 Flaps
Flaps must be judiciously and skillfully used, whether with or
without tissue expansion. There can be problems and iatrogenic
deformities created when flaps are performed contrary to the
principles laid forth previously. In particular, flaps used in the
facial region are particularly prone to misuse. This is because
the thickness of skin flaps from almost all donor sites is greater
than that of normal facial skin and will tend to mask normal
facial movement and expression. The same is true regarding tis-
sue expansion and flap design and use in the lower extremities.
Conversely, tissue expansion and flap usage for burn scar scalp
alopecia is an ideal treatment modality. It is also important to
realize that the size of flaps required to adequately relieve con-
tractures can be remarkably large and this can easily create
extrinsic contractures that can adversely a ect form and func-
tion. Inadequate contracture releases with flaps are common,
and iatrogenic deformities from flap usage are unfortunately
very common (Fig. 41.9). In addition, color and texture match
are very important considerations in flap design, and donor
sites must be carefully chosen with this in mind. However,
despite these cautionary points, well-chosen and expertly per-
formed flaps can be very helpful in selected cases and are
essential for complex injuries such as electrical burns.
41.4.9 Lasers
Advances in laser therapy have greatly facilitated scar treat-
ment and rehabilitation, further decreasing the indications for
scar and, in particular, burn scar excision. Laser scar therapy
represents a well-established, although newer, technology that
is not only promising, but also still remarkably underutilized in
the multidisciplinary treatment of burn scars and other trau-
matic scars. This has heralded changes to existing scar treat-
ment paradigms that allow for integration of lasers as well as
combination therapies into future scar treatment protocols.
Pulsed dye laser, and now fractional photothermolysis, thera-
pies have emerged over the past decade as successful alterna-
tives to excision in patients with hypertrophic burn scars.
Lasers have been proven to be safe, consistently e ective, and
demonstrate potential functional as well as cosmetic benefits in
burn scar treatment. The basic premise is that laser treatment
of scars allows for the production of microscopic patterns of
thermal injury in the dermis, which stimulates the complex
process of tissue remodeling. Laser treatment is particularly
e ective when combined with Z-plasty release or some of the
other modalities mentioned earlier (Fig. 41.3). Prospective,
randomized trials are in progress to evaluate how to optimize
already clearly e ective laser treatment protocols and will pro-
vide further insights on how to take maximum advantage of
this powerful tool in the plastic surgical armamentarium.
41.5 Complications
As has been mentioned thus far, each of the considerations dis-
cussed has also been tempered with caution with regard to
unfavorable results and complications, especially iatrogenic,
that may occur even with the best intentions and most pre-
cisely crafted nonoperative management or operative treat-
ment plans. Often, many of the complications seen and the
secondary “deformities” created by nonprincipled treatment
plans are thankfully avoidable or able to be minimized such
that forward progress is maintained. Frequently, Z-plasty repair
may lead to tip necrosis or wound separation that can both be
Fig. 41.6 (a) Preoperative: This hypertrophic, contracted scar is raised,
erythematous, shiny, and pruritic. (b) Postoperative: After the Z-plasty
has been incised and transposed, the scar is longer, completely level
with the adjacent skin in the center, and irregular in outline. (c) Three
weeks postoperation, the change in the scar is profound. Flattening is
continuing, erythema is resolving, and pruritus is significantly less.
Changing a scar’s environment changes its behavior.
Burns
399

treated with local dressing care until re-epithelialization
ensues. Typically, in these cases, because tension has been
relieved by the Z-plasty, wound healing goes on to be quite
favorable and without hypertrophic sequelae. Grafts and flaps
have their own unique considerations, and the informed con-
sent process must focus on bleeding, infection, partial or com-
plete graft or flap loss, unfavorable scarring, tissue expander
infection or extrusion, donor site complications, and need for
further procedures due to secondary iatrogenic deformities cre-
ated by primary operative intervention.
As it relates to laser therapy, scars seem to tolerate this well
with a remarkably low rate of complication. Immediate onset of
transient erythema and localized swelling are well-known
issues, as well as pinpoint bleeding, depending on laser settings
and depth of penetration. Reports of infection and new or wor-
sening scarring do exist in the literature but are very infre-
quent, and overall the safety profile is exceptional. More
important, however, are the safety considerations around laser
therapy for not only the patient, but also the treatment pro-
vider and team. Eye protection, fire safety protection, smoke
evacuation, and other safety measures are critical and must be
closely adhered to whenever using any laser device.
41.6 Conclusion
Pediatric burn care and reconstruction have witnessed signifi-
cant and remarkable advances in the care of acute and secon-
dary burn patients. Owing to typical child’s robust health,
robust nutritional status, and lack of comorbidities at the time
of most burn injuries, even those unfortunate patients with
greater than 90% total body surface area burns are surviving
and benefiting from the expertise and multidisciplinary care
provided to them. Therefore, once the acute burn chaos has
subsided, successful secondary pediatric burn scar reconstruc-
tion can restore these patients su ciently that they may enjoy
a happy, healthy, and very productive life. This is evidenced by
well-reported, large series in the literature that demonstrate
excellent long-term outcomes when compared with normal
controls. What is most important is that the essential qualities
to ensure such outcomes are adhered to: patience, persistence,
determination, adherence to fundamental principles, and a
curiosity to keep an open mind as newer technologies become
available. Thoughtful and skillful application of these qualities
to the treatment of pediatric burn injuries will lead to optimiza-
tion of outcomes and provide gratification to the patients and
providers of care alike. The critical principle of pediatric burn
reconstruction is learning that has been espoused thus far in
this chapter, namely, to understand, appreciate, and most favor-
ably influence the process of wound healing and scar matura-
tion. This is a goal that is attainable and one that we must
steadfastly strive to achieve for each and every patient we as
pediatric plastic and reconstructive surgeons have the privilege
to care for.
41.7 Key Points
● Scars are your friends.
● Relaxed scars are happy scars.
● Scar excision is an oxymoron.
● Adhere to fundamental principles and do not rely on specific
techniques as techniques are transient, but principles stand
the test of time.
● Laser therapy should be included as early as possible in the
care of burn scar injuries and considered as another critical
tool in the armamentarium of burn care and treatment.
Fig. 41.7 (a) This 3-year-old male sustained
severe burn injury to the head and neck in a
house fire in China. The facial burns healed
without grafting. He was adopted and brought to
the United States. (b) Eight years later, after Z-
plasties, small releases/grafts, and treatment
with the pulse dye laser and fractional CO2 laser,
his facial scars are completely rehabilitated. No
scars were excised.
Integument
400

Fig. 41.8 (a) An 8-year-old female who sustained
65% total body surface area flame burns in a
house fire. Her facial split-thickness grafts were
hyperpigmented, contracted, irregular, and con-
spicuous. (b) At age 11, the split-thickness skin
grafts were replaced with a full-thickness graft
from her chest, the best available match for color
and texture. The donor site was closed with a
split-thickness graft. (c) Only the most noticeable
area of the split-thickness skin graft was excised.
The surrounding tissue was released to allow it to
retract. A much larger graft than the measured
defect was harvested. (d) Multiple scar revisions
with Z-plasties at the margins of the full-thickness
skin graft and treatment with the fractional CO2
laser resulted in significant improvement and a
happy patient.
Burns
401

Suggested Readings
Parrett BM, Donelan MB. Pulsed dye laser in burn scars: current concepts and
future directions. Burns. 2010; 36(4):443–449
Anderson RR, Donelan MB, Hivnor C, et al. Laser treatment of traumatic scars
with an emphasis on ablative fractional laser resurfacing: consensus report.
JAMA Dermatol. 2014; 150(2):187–193
Krakowski AC, Totri CR, Donelan MB, Shumaker PR. Scar management in the
pediatric and adolescent populations. Pediatrics. 2016; 137(2):e20142065
Pape SA, Skouras CA, Byrne PO. An audit of the use of laser Doppler imaging
(LDI) in the assessment of burns of intermediate depth. Burns. 2001; 27
(3):233–239
Monstrey SM, Hoeksema H, Baker RD, et al. Burn wound healing tim e
assessed by laser Doppler imaging. Part 2: validation of a dedicated colour
code for image interpretation. Burns. 2011; 37(2):249–256
Donelan MB, Liao EC. Principles of burn reconstruction. In: Thorne CH, ed.
Grabb and Smith’s Plastic Surgery. 7th ed. Philadelphia, PA: Lippincott Wil-
liams & Wilkins; 2014:142–154
Serghiou MA, Ott S, Whitehead C, et al. Comprehensive rehabilitation of the
burn patient. In: Herndon DN, ed. Total Burn Care. 4th ed. London, UK: Elsev-
ier; 2012:517–549
Gonzalez-Ulloa M. Restoration of the face covering by means of selected skin
in regional aesthetic units. Br J Plast Surg. 1956; 9(3):212–221
Fig. 41.9 (a) This 12-year-old male has a normal
facial appearance as well as hypertrophic burn
scars with minimal contractures. Today, the scars
could easily be rehabilitated with Z-plasties and
fractional CO2 laser therapy. (b) Scar excision was
performed and cheek resurfacing carried out
with a tissue expanded cervicopectoral flap.
(c) Flap tip ischemia and downward tension are
frequent complications. (d) Following excision of
scar and flap resurfacing, there is lower lid
displacement, malar flattening, loss of jawline
definition, and increased contracture of the oral
commissure and lips.
Integument
402

42 Melanocytic Nevi
Aladdin H. Hassanein and Arin K. Greene
Sum m ary
Melanocytic nevi are common in children. The primary indica-
tion for removal is to improve a deformity. Giant nevi are asso-
ciated with a low risk of malignant degeneration. Generally,
serial excision is preferred to skin grafting or tissue expansion
when feasible.
Keywords: melanocytic, nevus, melanoma, pediatric
42.1 Introduction
Melanocytic nevi are one of the most common pediatric cutane-
ous conditions; lesions can be present at birth or acquired.
Congenital nevi a ect 1% of the population and form when
nevomelanocytes become ectopically located during migration
from the neural crest to the epidermis. Congenital melanocytic
nevi can cause a deformity and psychosocial morbidity. Patients
and families frequently are anxious about malignant degenera-
tion, but the risk of transformation to melanoma often has been
overestimated. This chapter will focus on evidence-based ma-
nagement of congenital melanocytic nevi.
42.2 Diagnosis
Melanocytic nevi are diagnosed by history and physical exami-
nation. Age of onset (congenital or acquired), asymmetry, bor-
der irregularity, color, size, and symptoms should be elicited. In
contrast to acquired nevi, histopathology of congenital lesions
exhibits nevomelanocytes in the lower two-thirds of the dermis
and subcutis involving sebaceous glands, hair follicles, vessels,
and nerves. Congenital melanocytic nevi can be classified based
on their diameter or percent total body surface area (TBSA).
Although a consensus definition does not exist, lesions usually
are categorized as small (<1.5 cm), medium (1.5–10 cm), large
(11–20 cm), and giant (>20 cm or >2% TBSA). We favor the defi-
nition of giant nevi greater than 2% TBSA because it is easy to
assess clinically by determining if the lesion is twice the area of
the infant’s hand (1% TBSA). Giant congenital melanocytic nevi
are rare (1/20,000 births) but problematic.
Imaging is unnecessary to diagnose congenital melanocytic
nevi. However, patients with giant lesions are at risk for
melanocytes involving the leptomeninges. Neurocutaneous
melanosis can cause developmental delay, hydrocephalus, and
seizures. Children with lesions located in the midline head or
trunk and those with multiple satellite nevi are at greatest
risk for neurocutaneous melanosis. Magnetic resonance imag-
ing of the central nervous system is performed during infancy
to rule out neurocutaneous melanosis in patients with giant
pigmented nevi.
Biopsy of a congenital melanocytic nevus is indicated to
exclude melanoma if there is a change in appearance, ulcera-
tion, bleeding, or enlargement. Additional criteria include
the “ABCDs”: Asymmetric shape, Borders are irregular, multiple
Colors are present, and the Diameter is greater than 6 mm.
Malignant degeneration is very rare (0.03% lifetime risk) for
small nevi. The rate of melanoma for giant lesions historically
has been overestimated (up to 12%). However, recent studies
suggest that malignant degeneration is approximately 3%. The
incidence of melanoma is greatest within the first 3 years of life,
followed by another peak during adolescence. Nevi at highest
risk for malignancy are those located on the trunk and lesions
covering an extensive area. Melanoma has an approximately
equal chance of occurring in the main nevus, a satellite lesion,
or in the central nervous system.
42.3 Nonoperative Management
Congenital melanocytic nevi can be observed and do not man-
date intervention. Nonexcisional treatments (e.g., curettage,
dermabrasion, hydroquinone, laser) may be used to improve
the appearance of the nevi. These nonextirpative methods
are reserved for lesions in aesthetically sensitive areas where
resection would cause greater disfigurement than the nevus.
However, the cancer risk is not eliminated and fibrosis may
complicate later histopathology.
42.4 Operative Management
Indications for removing congenital melanocytic nevi are (1)
concern for malignancy, (2) lesions causing psychosocial mor-
bidity, or (3) to reduce the risk of malignant transformation for
giant congenital nevi.
42.4.1 Timing of Resection
Concern for melanoma requires immediate excision of the sus-
picious area. Removal of a lesion that is likely to cause psycho-
social morbidity can be delayed until 3 to 4 years of age when
self-esteem and long-term memory develop. Extirpation of nevi
on the scalp during infancy is advantageous because the tissue
is particularly lax during this period. Lower extremity lesions
should be removed in infancy while the patient is nonambula-
tory to reduce the risk of suture line dehiscence.
Because the peak incidence of malignant conversion for giant
lesions is greatest within the first 3 years of life, prophylactic
resection to decrease the occurrence of melanoma is most
e ective if completed before this time. We begin excision at 6
months of age when we are trying to completely remove a giant
nevus to reduce the likelihood of malignant conversion. We
avoid elective procedures prior to 6 months of age because the
risk of anesthesia is higher.
42.4.2 Small and Medium Congenital
Melanocytic Nevi
Small and medium-sized lesions are resected to aesthetically
improve a deformity or if the clinical appearance becomes wor-
risome for malignancy. These nevi usually can be removed len-
ticularly and closed linearly in one stage. Circular excision and
Melanocytic Nevi
403

purse-string closure may be performed for small, round nevi on
the face to minimize the length of the scar; lenticular excision
of a round lesion results in a scar two to three times its diame-
ter (Fig. 42.1). Approximately 50% of patients are satisfied
with the circular cicatrix, which can appear as an acne or
chicken pox scar. One-half of patients undergo a second stage to
convert the circular scar into a line that is approximately the
same length as the diameter of the original lesion. Lesions
located in di cult locations can result in more challenging
reconstructive problems (e.g., nose, eyebrow; Fig. 42.2,
Fig. 42.3, Fig. 42.4, Fig. 42.5). Raised dermal nevi that
are located in unfavorable locations (particularly the nose)
can be improved by shave excision to the papillary dermis
(Fig. 42.6). The patient’s appearance and contour will be
improved without a scar, but the individual likely will require
repeat shave excision in the future if the nevus re-enlarges.
Fig. 42.1 Small nevus of the chin causing lowered self-esteem treated with circular excision and purse-string closure to limit the length of the scar. (a)
Preoperative view. Note the marking for lenticular resection and increased size of scar if this method was chosen. (b) After circular excision with
approximately 1-mm margins. (c) One year postoperatively. The patient was pleased with the appearance of the scar and did not want a second
procedure to convert the circular scar into a line.
Fig. 42.2 A child with a small nevus treated with excision and cheek advancement to place the scar in the alar crease. (a) Preoperative view. (b)
Markings prior to resection. (c) After removal. (d) Postoperative image.
Fig. 42.3 Infant with a medium-sized nevus in an
unfavorable location. It was treated with der-
mabrasion, hydroquinone, and serial excision to
avoid skin grafting or a local/regional flap
reconstruction. (a) Preoperative appearance. (b)
Intraoperative view following dermabrasion. (c)
After dermabrasion, hydroquinone, and an inter-
mediate stage of serial excision. (d) Final
appearance after completing serial excision. Note
the patient ultimately had a single linear skin at
the junction of aesthetic subunits.
Integument
404

Fig. 42.4 A child with a medium-sized nevus on the nose reconstructed with skin grafting. (a) Preoperative view. (b,c) Following resection and
reconstruction with a full-thickness retroauricular skin graft. (d) One year postoperatively.
Fig. 42.5 An adolescent patient with a medium-sized nevus involving the eyebrow managed with lenticular excision. (a) Preoperative appearance. (b)
Postoperative view.
Fig. 42.6 Patient with a raised dermal nevus of the nasal tip managed with shave excision.
Melanocytic Nevi
405

Small/medium melanocytic nevi on the face being removed
for “cosmetic” reasons should have minimal gross margins (0–
1 mm) to ensure the most favorable scar. If no atypical cells are
present, then positive margins require no further treatment,
although there is a low risk (~1%) of minor re-pigmentation
along the scar, which can be resected secondarily. If histopa-
thology shows cellular atypia then re-excision to negative mar-
gins is required. Small/medium nevi located in nonaesthetically
important areas or those at risk for atypia can be extirpated
with 1- to 2-mm margins.
42.4.3 Large/Giant Congenital
Melanocytic Nevi
Although giant lesions are at greater risk for melanoma, gener-
ally large and giant nevi are approached similarly. These lesions
can be unsightly, but complete extirpation may create a greater
deformity. We agree with Zuker and colleagues that the risk of
malignancy should be weighed against the aesthetic outcome.
The frequency of malignant conversion (~3%) for giant nevi is
lower than historical estimations; no evidence proves that the
frequency of melanoma is decreased by prophylactic excision.
Therefore, we typically manage lesions nonoperatively if
removal would be more disfiguring or compromise function.
Giant nevi that are not removed are monitored by a dermatolo-
gist; biopsies are performed if an area becomes suspicious for
malignancy.
Techniques to remove giant congenital melanocytic nevi are
(1) serial excision, (2) skin grafting, and (3) tissue expansion.
We prefer serial excision because it has many advantages com-
pared to skin grafting or tissue expansion. First, the method is
simpler and only necessitates wide skin undermining to close
the wound. Second, the operations are short and performed on
an outpatient basis. Third, patients have a quicker recovery and
less risk of operative morbidity. Fourth, complications (e.g.,
infection, dehiscence) do not adversely a ect the final outcome.
A dehisced wound can be allowed to heal secondarily and
the wider scar excised during the next stage. If dehiscence or
infection complicates skin grafting or tissue expansion, the
consequences are more problematic. Finally, children are left
with a single linear scar, which is aesthetically more favorable
than skin grafting or tissue expansion. A limitation of serial
excision is that it relies on the ability to recruit adjacent tissue
using subcutaneous undermining; it is unable to be performed
in circumferential extremity lesions and areas that lack skin
laxity. Several stages may be required; we have performed as
many as five excisions for extensive lesions.
Skin grafting following resection of giant congenital melano-
cytic nevi allows for treatment in one procedure ( Fig. 42.7).
However, the recipient site appears as an indented, hairless
patch with a color distinct from the surrounding native skin.
Graft failure can occur and a painful donor site scar is created.
Children usually require hospital admission to ensure revascu-
larization of the graft. Patients are limited postoperatively to
ensure immobilization of the area.
Tissue expansion also is used to treat giant congenital nevi.
Similar to serial excision, tissue expansion replaces the lesion
with adjacent, local tissue, which is better aesthetically than a
skin graft (Fig. 42.8). However, the technique requires a pro-
cedure to place the expander, multiple outpatient visits for fill-
ing, and an additional operation to remove the implant, excise
the lesion, and inset the expanded skin. Rotation, transposition,
and/or advancement of expanded flaps create longer and more
numerous scars compared to a single line from serial excision.
As the volume in the expander increases and becomes more
visible to others, patients can experience pain and psychosocial
morbidity. There is a risk for expander infection, malposition,
deflation, malfunction, and extrusion. Tissue expansion is most
problematic in the pediatric population.
42.4.4 Head/Neck
Melanocytic nevi involving the head/neck are more likely to
undergo resection because they cause an obvious deformity.
Large lesions over the scalp, forehead, periorbital area, nose,
and ear are problematic to treat with serial excision because of
poor skin laxity and di culty recruiting local tissue with sub-
cutaneous undermining ( Fig. 42.9, Fig. 42.10, Fig. 42.11).
Fig. 42.7 An adolescent patient with a giant
congenital melanocytic nevus of the forehead
and scalp managed with skin grafting. (a) Pre-
operative photo. (b) Following resection. (c)
Reconstruction with a split-thickness skin graft.
(d) Three months postoperatively.
Integument
406

Fig. 42.8 Giant congenital melanocytic nevus of the scalp and forehead treated with tissue expansion. (a) Prior to resection. (b,c) Following tissue
expander placement and filling. (d) After removal of the expander and flap advancement. (e) Postoperative image.
Fig. 42.9 Medium-sized congenital melanocytic nevus of the cheek treated with serial excision. (a) Preoperative image. (b) After first stage removal.
(c) Following second resection.
Fig. 42.10 Serial excision of congenital melanocytic nevus of the cheek and eyelids. (a) Initial presentation. (b) Following first resection. (c) After
intermediate stage. (d) Postoperative appearance following serial excision. Note that the eyelid is not amendable to serial removal and will be
reconstructed with full-thickness skin grafts.
Melanocytic Nevi
407

Tissue expansion is preferred for scalp lesions that cannot be
removed with serial excision. Large forehead nevi with a pauc-
ity of normal skin to expand/recruit can be treated with skin
grafting. Periorbital lesions often require skin grafting. The
benefits of removing a congenital nevus of the head/neck must
justify creating an unaesthetic, iatrogenic deformity that may
result from resection (e.g., facial nerve injury during tissue
expansion).
42.4.5 Trunk
Serial excision is our preferred technique for giant nevi of the
trunk. Some authors favor tissue expansion to treat nevi on this
region, but we have found that most lesions are amendable to
removal with serial excision ( Fig. 42.12). Serial excision is
lower on the reconstructive ladder and results in a single, linear
scar. Although more stages may be required with serial excision
compared to skin grafting or tissue expansion, avoiding the
complications and potential morbidity warrants the additional
procedures. We prefer to observe patients with “bathing-suit”
nevi involving the entire trunk because the nevus is hidden by
clothing ( Fig. 42.13). We do not believe the risks and benefits
of extensive skin grafting and/or tissue expansion are favorable
for these patients.
42.4.6 Extremity
Congenital melanocytic nevi of the extremities have lower risk
of malignant transformation than other areas. In addition, these
lesions usually can be concealed by clothes. Most large/giant
nevi of the limbs are amendable to treatment with serial exci-
sion (Fig. 42.14). However, circumferential lesions are unable
to be removed with serial excision because adjacent, normal
tissue cannot be recruited. Therefore, skin grafting or tissue
expansion is required. Large hand and foot nevi also are di cult
to treat with serial excision. Skin grafting of hand lesions can
result in contracture and a ect function. Tissue expanders often
become infected in the lower extremities. Thus, the risk/benefit
profile of treating extremity lesions must be carefully consid-
ered. Large circumferential lesions, particularly involving joints,
can be resected and reconstructed with full-thickness skin
grafts that have been expanded from the lower trunk.
Fig. 42.11 Giant congenital melanocytic nevus of the scalp managed with serial excision. The procedure was initiated at 3 months of age to take
advantage of infant scalp laxity. (a) Preoperative image. (b) Prior to second-stage resection. (c) Postoperative view after the final fifth excision.
Fig. 42.12 A child with a giant congenital nevus of the trunk. (a) Preoperative view. (b) Prior to second-stage resection. (c) After the fourth and final
stage.
Integument
408

42.5 Complications
The greatest concern in managing congenital melanocytic nevi
is a delay in diagnosis of melanoma. Lesions that are treated
nonoperatively should be followed serially by a dermatologist
and biopsied if there is a suspicious change in appearance.
Patients with a giant congenital melanocytic nevus are assessed
with magnetic resonance imaging for neurocutaneous melano-
sis in order to anticipate possible developmental delay, hydro-
cephalus, and seizures. Complications of serial excision include
infection and wound dehiscence. Skin grafting can result in
partial or complete failure and cause a donor site scar. Tissue
expansion is associated with many di culties and is more chal-
lenging than serial excision or skin grafting. There is a risk for
expander infection, malposition, deflation, and extrusion. These
complications may limit the amount of available donor tissue
or cause abandonment of this reconstructive option.
42.6 Conclusion
Congenital melanocytic nevi are common pediatric skin lesions
that a ect 1% of the population. They cause a deformity and
psychosocial morbidity. Small and medium-sized lesions can be
removed for aesthetic improvement or if they are suspicious
for malignancy. Giant congenital melanocytic nevi are at risk
for neurocutaneous melanosis and malignant transformation.
These lesions can be resected using serial excision, skin grafts,
or tissue expansion. The benefits of removal should be weighed
against the morbidity of extirpation.
Fig. 42.13 Giant congenital melanocytic nevus
involving the trunk and lower extremities. The
lesion is hidden by clothing and was managed
with observation by a dermatologist.
Fig. 42.14 Large congenital melanocytic nevus of the leg treated with serial excision. (a) Preoperative image. (b) Prior to third and final resection. (c)
Postoperative appearance.
Melanocytic Nevi
409

42.7 Key Points
● Congenital melanocytic lesions are common and are catego-
rized as small, medium, large, or giant.
● Removal is performed for aesthetic improvement or if there is
a concern for malignancy.
● Giant congenital melanocytic nevi have a low risk for malig-
nant transformation (~3%).
● Resection is not mandatory; lesions can be observed and fol-
lowed for suspicious changes.
● Extirpation of giant lesions must be weighed against the mor-
bidity of the procedures and reconstruction.
Suggested Readings
Gosain AK, Santoro TD, Larson DL, Gingrass RP. Giant congenital nevi: a 20-
year experience and an algorithm for their management. Plast Reconstr Surg.
2001; 108(3):622–636
Watt AJ, Kotsis SV, Chung KC. Risk of melanoma arising in large congenital
melanocytic nevi: a systematic review. Plast Reconstr Surg. 2004; 113
(7):1968–1974
Bauer BS, Corcoran J. Treatment of large and giant nevi. Clin Plast Surg. 2005;
32(1):11–18, vii
Chung C, Forte AJ, Narayan D, Persing J. Giant nevi: a review. J Craniofac Surg.
2006; 17(6):1210–1215
Rogers GF, Maclellan RA, Liu AS, et al. Extremity fasciotomy wound closure:
comparison of skin grafting to staged linear closure. J Plast Reconstr Aesthet
Surg. 2013; 66(3):e90–e91
Arad E, Zuker RM. The shifting paradigm in the management of giant congen-
ital melanocytic nevi: review and clinical applications. Plast Reconstr Surg.
2014; 133(2):367–376
Hassanein AH, Rogers GF, Greene AK. Management of challenging congenital
melanocytic nevi: outcomes study of serial excision. J Pediatr Surg. 2015; 50
(4):613–616
Hassanein AH, Greene AK. Congenital melanocytic nevus of the nose removed
using derm abrasion, hydroquinone, and serial excision. J Craniofac Surg.
2015; 26(7):2216–2217
Hassanein AH, Couto JA, Greene AK. Circular excision and purse-string clo-
sure for pediatric facial skin lesions. J Craniofac Surg. 2015; 26(5):1611–1612
Integument
410

43 Pediatric Skin Lesions
Aladdin H. Hassanein and Arin K. Greene
Sum m ary
Cutaneous lesions are common in children. Although almost
always benign, they may cause psychosocial morbidity
and anxiety concerning malignant degeneration. This chapter
reviews the diagnosis and management of the most frequently
treated pediatric skin lesions: acrochordon, dermoid cyst, epi-
dermal cyst, epidermal nevus, granuloma annulare, hypertro-
phic scar/keloid, lipoma, juvenile xanthogranuloma, mucocele,
nevus sebaceous, neurofibroma, pilar cyst, pilomatrixoma, Spitz
nevus, and verruca vulgaris.
Keywords: dermoid, cyst, keloid, lipoma, mucocele, nevus, neu-
rofibroma, pilomatrixoma, skin lesion
43.1 Introduction
Cutaneous lesions are common in children and frequently are
managed by pediatric plastic surgeons. They are almost always
benign, although some can be premalignant. Skin lesions may
cause a deformity and psychosocial morbidity. Patients and
families often are anxious about malignant degeneration. Most
conditions are diagnosed by history and physical examination;
imaging or biopsy rarely is indicated. Lesions necessitating
removal usually require a general anesthetic in young children;
adolescents may tolerate excision using local anesthesia. This
chapter reviews the most common pediatric skin conditions
that are encountered by plastic surgeons: acrochordon,
dermoid cyst, epidermal cyst, epidermal nevus, granularum
annulare hypertrophic scar/keloid, juvenile xanthogranuloma,
lipoma, nevus sebaceous, neurofibroma, mucocele, pilar cyst,
pilomatrixoma, Spitz nevus, and verruca vulgaris (Table 43.1).
43.2 Diagnosis
Most cutaneous lesions can by diagnosed by history and physi-
cal examination. Age of onset (congenital or acquired), changes
in appearance, and symptoms (e.g., bleeding, infection, pain,
ulceration) are elicited. Examination should assess color, loca-
tion, mobility, size, symmetry, and tenderness. Pinching the le-
sion in relation to the surrounding soft tissue can determine
the depth of involvement. If the skin can be approximated over
the lesion, then the abnormality is located beneath the integu-
ment (e.g., dermoid cyst, lipoma, etc.).
Imaging rarely is indicated if (1) the diagnosis is uncertain
despite clinical evaluation and (2) the results of the study will
Table 43.1 Common pediatric skin lesions
Lesion

Clinical features

Histopathology
Acrochordon

Soft, flesh-colored fibroepithelial polyp

Epidermal hyperplasia; ±adipose central core
Dermoid cyst

Congenital; location in brow, nasoglabellar, or orbital
areas
Epithelial lining, pilosebaceous structures in the cyst wall
Epidermal cyst

Proliferation of epidermal cells within the dermis

Lined with epithelium and filled with keratin
Epidermal nevus

Hyperkeratotic lesion

Epidermal granular layer, increased basal layer pigment,
inflammatory dermal infiltrate
Granuloma annulare

Inflammatory condition; ringlike, erythematous lesion
Degenerative collagen surrounded by histiocytes, fibro-
blasts, and lymphocytes
Juvenile xanthogranuloma

Pinkish-yellow; can regress

Histiocytes and giant cells
Keloid

Grow beyond the wound borders; predilection for chest,
ear, shoulder
Fibrous tissue, and irregularly arranged dermal collagen
Lipoma

Subcutaneous, mobile lesion with normal overlying skin Benign adipose overgrowth
Mucocele

Oral pseudocyst from trauma or obstruction of a minor
salivary gland
Mucin-fluid, no epithelial lining
Nevus sebaceous

Yellow-orange hairless plaque; predilection for the scalp
and face
Cutaneous hyperplasia of adnexal elements
Neurofibroma

Benign peripheral nerve sheath tumor

Fibroblasts, mast cells, and Schwann cells
Pilar cyst

Subcutaneous hair follicle outer root sheath cyst that
occurs on the scalp
Keratin filled; wall of stratified squamous epithelium
Pilomatrixoma

Firm, fixed mass with a bluish hue; can ulcerate

Benign calcifying tumor of the hair follicle matrix cells
Spitz nevus

Acquired dome-shaped pink nodule, typically<1 cm

Benign tumor of spindled and epithelioid melanocytes
Verruca vulgaris

Flesh-colored, firm papules

Hyperkeratosis, acanthosis
Pediatric Skin Lesions
411

influence management. Generally, ultrasonography is the first-
line imaging modality. It can distinguish solid from cystic
lesions and assess vascularity. Magnetic resonance imaging
(MRI) or computed tomography (CT) is considered if ultraso-
nography is unclear. Biopsy to elucidate a histopathological
diagnosis prior to extirpation usually is unnecessary. If removal
is required, definitive diagnosis will be confirmed with an exci-
sional biopsy.
43.3 Nonoperative Management
Asymptomatic lesions with a low malignant potential can be
observed and followed serially. Nonexcisional treatments (e.g.,
dermabrasion, laser) may be used to improve the appearance of
some cutaneous conditions, but the cancer risk is not elimi-
nated and fibrosis may complicate later histopathology.
43.4 Operative Management
Skin lesions are excised because of (1) symptoms, (2) malignant
risk, or (3) aesthetic concerns. Removal of a lesion that is likely
to cause psychosocial morbidity can be considered between 3
and 4 years of age when self-esteem and long-term memory
develop. Most cutaneous disorders that necessitate extirpation
are lenticularly excised and closed linearly. Round facial lesions
may benefit from circular excision and purse-string closure to
give the shortest possible scar. A second stage can be performed
to convert the circular scar into a line. Subcutaneous lesions are
removed using an incision (or small skin excision) over the
abnormality. Disorders beneath the skin usually can be resected
with a scar that is shorter than the diameter of the lesion.
43.4.1 Acrochordon
Acrochordon (i.e., skin tag) is a common pedunculated fibro-
epithelial polyp. It exhibits epidermal hyperplasia; large lesions
may have an adipose component. Acrochordons are soft, flesh-
colored, and can be sessile or pedunculated. They have a predis-
position to develop in skin folds (e.g., neck, axilla, groin) and
occur more frequently in diabetic or pregnant patients. Large
lesions can cause discomfort and deformity. Symptomatic acro-
chordons are usually treated by excision; cryotherapy is a less
common option.
43.4.2 Dermoid Cyst
Dermoid cysts are congenital and usually located on the face or
cranium. Lesions contain ectodermal and endodermal tissue;
lining consists of keratinizing squamous epithelium and pilose-
baceous structures. Orbitofacial dermoids are caused by dermal
and epidermal cell displacement along embryonic lines of
fusion. They are asymptomatic, slow growing, and most fre-
quently located in the frontotemporal, nasoglabellar, or orbital
area (the most common site is the lateral brow; Fig. 43.1). If a
dermoid is suspected, imaging is unnecessary unless the lesion
is along the nasal midline. MRI is obtained to determine
whether a midline nasal dermoid extends intracranially along a
tract. Dermoids located over the nasal bones have a low risk of
intracranial extension, while lesions involving the nasal tip
have an approximately 50% risk of intracranial communication.
Brow and orbital dermoids usually can be removed through an
eyelid incision. Nasoglabellar dermoids without intracranial
involvement can be excised directly or using an open-rhino-
plasty approach. Dermoids with intracranial extension may
require a nasal and coronal incision with a craniotomy to
remove the entire tract.
43.4.3 Epidermal Cyst
Epidermal (“sebaceous”) cysts are benign proliferations of epi-
dermal cells within the dermis that can occur congenitally or by
implantation of epithelial cells into dermis (Fig. 43.2). Lesions
are filled with keratin and lined with stratified squamous epi-
thelium, which contains a granular layer. Epidermal cysts
appear as round nodules often with a central pore. They slowly
Fig. 43.1 (a) A child with a dermoid cyst of the
lateral brow. (b) An infant with a nasal dermoid. A
midline nasoglabellar dermoid requires magnetic
resonance imaging to assess for intracranial
extension.
Integument
412

enlarge over time, may become infected, and can cause a
deformity. Imaging is not required. On physical examination,
they are attached to the skin and cannot be separated from the
integument. Treatment is excision of the entire lesion. Drainage
of the lesion will temporarily make it smaller, but it will re-
enlarge because the cyst wall will continue to produce keratin.
43.4.4 Epidermal Nevus
Epidermal nevi are benign overgrow ths of epidermal elements
(including keratinocytes); they may be congenital or arise dur-
ing infancy (Fig. 43.3). A thick epidermal granular layer,
increased basal layer pigment, and inflammatory dermal infil-
trate are present. Lesions appear as hyperkeratotic plaques on
the extremities, neck, or trunk that may follow the lines of
Blaschko. Most are sporadic, but some are associated with epi-
dermal nevus syndromes. Epidermal nevi cause a deformity
and pruritus; they do not undergo malignant transformation.
Types of epidermal nevi include (1) Becker nevus (late-onset
melanosis), (2) comedone nevus (hair follicle nevus), and (3)
inflammatory linear verrucous (irritated, pruritic, scaly). Full-
thickness removal is performed for symptomatic lesions. Epi-
dermal nevi in unfavorable locations for resection can be
improved by shaving of the superficial portion of the lesion, but
recurrence is likely.
43.4.5 Granuloma Annulare
Granuloma annulare is a benign, inflammatory condition
characterized by ringlike, erythematous lesion ( Fig. 43.4). His-
tology demonstrates degenerative collagen surrounded by his-
tiocytes, fibroblasts, and lymphocytes. The most common form
(localized) is present on the dorsum of the distal extremities.
These self-limiting lesions are asymptomatic and typically
resolve within 2 years. Large areas can cause disfigurement and
reduce the patient’s self-esteem. Lesions may be observed or
treated with intralesional corticosteroid. Excision can be per-
formed if the diagnosis is unclear or if a problematic lesion does
not regress.
Fig. 43.2 A child with epidermal cyst of cheek. Note the round
subcutaneous appearance.

Fig. 43.3 A 13-year-old male with a pigmented and hyperkeratotic
epidermal nevus of the neck.
Fig. 43.4 A 6-year-old male with granuloma annulare of the foot.
Pediatric Skin Lesions
413

43.4.6 Hypertrophic Scar/Keloid
Hypertrophic scars and keloids result from abnormal cicatricial
formation. Keloids grow beyond the wound borders and hyper-
trophic scar does not. Keloids tend to worsen over time, while
hypertrophic scars typically improve. Both have a predilection
to form on the chest, ear, and shoulder (Fig. 43.5). Histopa-
thology exhibits fibrous tissue and irregularly arranged dermal
collagen. Lesions occur more frequently in dark-skinned indi-
viduals. Hypertrophic scars and keloids can be erythematous,
painful, and pruritic. First-line nonoperative treatments include
pressure, topical silicone, and corticosteroid injection. If these
modalities fail, the scar can be excised and the nonoperative
therapies can be initiated immediately postoperatively to mini-
mize recurrence.
43.4.7 Juvenile Xanthogranuloma
Juvenile xanthogranuloma is composed of histiocytes and giant
cells. It can be congenital (20%) or acquired in early childhood.
Lesions initially are pink and transform to a yellow color
(Fig. 43.6). They may grow rapidly. Juvenile xanthogranuloma
can be observed because it has the ability to regress, usually
within 3 years. Lesions are di cult to diagnose clinically
and thus most undergo excisional biopsy. If the lesion fails
to resolve, patients may request removal to improve their
appearance.
43.4.8 Lipoma
Lipomas are benign adipose tissue growths of mesenchymal
origin. Lesions are subcutaneous with normal overlying skin.
They are mobile and may be lobulated ( Fig. 43.7). Lipomas
typically are asymptomatic and slow growing. They may be
removed for aesthetic reasons or if there is a concern for malig-
nancy. Lipomas are excised through a linear incision over the
lesion. Recurrence is minimized by complete extirpation.
43.4.9 Mucocele
Mucoceles are oral pseudocysts that result from trauma or
obstruction of a minor salivary gland. Lesions typically are fluc-
tuant, mobile, and round. They may have a bluish hue and are
most common in the lower lip. Rupture and re-accumulation
of mucin can cause size fluctuations. Although mucoceles
may spontaneously resolve, patients often request that they be
removed to improve their appearance.
43.4.10 Nevus Sebaceous
Nevus sebaceous is a congenital cutaneous hyperplasia of
adnexal elements. Lesions are yellow-orange hairless plaques
with a predilection for the scalp and face (Fig. 43.8). Benign
(e.g., trichilemmoma), premalignant (e.g., syringocystoade-
noma), and malignant (i.e., basal cell) tumors may develop
within nevus sebaceous. The risk of benign or malignant tumor
formation is <2%. Nevus sebaceous becomes progressively
hyperkeratotic over time (particularly during adolescence) and
Fig. 43.5 An adolescent male with a keloid of the chest.
Fig. 43.6 A child with a juvenile xanthogranuloma of the trunk.
Fig. 43.7 A 17-year-old male with a lipoma of flank. Note the lobulated
and encapsulated appearance.
Integument
414

thus creates a worsening deformity. Most lesions are removed
to eliminate the risk of tumor degeneration and to improve the
patient’s appearance.
43.4.11 Neurofibroma
Neurofibromas are benign peripheral nerve sheath tumors that
contain fibroblasts, mast cells, and Schwann cells. Neurofibro-
mas typically are found in patients with the autosomal-domi-
nant disorder neurofibromatosis type I. A mutation of the NF1
gene on chromosome 17 results in an abnormal tumor suppres-
sor protein called neurofibromin. Patients with neurofibroma-
tosis type I also may exhibit café au lait spots, axillary macules,
Lisch nodules of the iris, optic nerve gliomas, and sphenoid
dysplasia.
Neurofibromas are categorized as dermal or plexiform. Der-
mal neurofibromas are soft cutaneous masses or subcutaneous
nodules ( Fig. 43.9). They appear commonly during adoles-
cence and remain benign. Most dermal neurofibromas are
asymptomatic; resection is reserved for symptomatic lesions
causing pain or a deformity.
Plexiform neurofibromas are congenital and cause destruc-
tion of soft tissue and bone. They have a 10% risk of malignant
degeneration. MRI can define the extent of the lesion. Removal
of these lesions is di cult because they may involve multiple
tissue planes and functionally critical nerves.
43.4.12 Pilar Cyst
Pilar (trichilemmal) cysts are keratin-filled lesions surrounded
by a wall of stratified squamous epithelium ; they may exhibit
autosom al-dominant transmission. They originate from the
hair follicle outer root sheath and typically occur on the scalp
( Fig. 43.10). In contrast to epidermal cysts, the epithelial
lining of a pilar cyst wall lacks a granular layer and lesions
do not have a punctum. They typically are concealed by hair
but can become infected. Treatment is complete removal of
the cyst.
43.4.13 Pilomatrixoma
Pilomatrixoma is a benign calcifying lesion of hair follicle
matrix cells. Lesions predominantly a ect the head and neck
(Fig. 43.11). It is a slow-growing, firm, fixed mass with a blu-
ish hue. Lesions are usually asymptomatic but can enlarge over
time, ulcerate, and become infected. Large pilomatrixomas may
cause a visible deformity and psychosocial distress. Pilomatrix-
omas are excised for definitive diagnosis, to prevent complica-
tions associated with their enlargement, and to improve the
patient’s appearance.
43.4.14 Spitz Nevus
Spitz nevi are benign tumors of spindled and epithelioid mela-
nocytes. These acquired lesions typically exhibit an initial
growth period and then remain static. They are reddish brown
and usually smaller than 1 cm in diameter. Although Spitz nevi
are solitary, they can appear in clusters (agminated) or be di use
(Fig. 43.12). Spitz nevi may have histological features similar
to melanoma. The Spitz tumor histological spectrum includes
Spitz nevus (benign), atypical Spitz tumor (potentially malig-
nant), and Spitzoid melanoma (malignant). Lesions are excised;
recommended margins are 2 mm. Treatment of large agminated
Spitz nevi can be challenging in areas where complete removal
may leave a significant deformity. Localized areas within the
agminated lesion can be excised if they change clinically.
Fig. 43.8 A child with nevus sebaceous of the scalp.
Fig. 43.9 A 17-year-old female with neurofibro-
matosis and a plexiform neurofibroma of the
chest.
Pediatric Skin Lesions
415

43.4.15 Verruca Vulgaris
Common warts (verruca vulgaris) are flesh-colored, firm pap-
ules that occur from human papilloma virus infection. These
growths exhibit hyperkeratosis and acanthosis ( Fig. 43.13).
Lesions frequently occur on the hands but can develop on
any mucocutaneous surface. Warts are contagious and may
be spread by contact. Lesions often resolve spontaneously
over a few years, but families typically are eager to treat
them because they cause a deformity. Therapeutic modalities
include topical medications (e.g., salicylic acid, imiquimod),
cryotherapy, curettage, or cauterization. Resection is reserved
for warts that have failed other interventions, but should be
performed with caution because the viral infection can recur
through the scar.
43.5 Conclusion
Cutaneous lesions are common in children. Although skin
growths may appear similar, most can be distinguished by
history and physical examination. Excision is reserved for
symptomatic lesions, precancerous abnormalities, or to provide
definitive histological diagnosis.
Fig. 43.11 An 8-year-old female with a pilomatrixoma of the brow.
Fig. 43.12 (a) A child with a Spitz nevus of the cheek. (b) A 5-year-old female with an agminated atypical Spitz tumor of the nose.
Fig. 43.10 A child with pilar cysts of the scalp.
Integument
416

43.6 Key Points
● Cutaneous pediatric lesions are common.
● Most disorders can be diagnosed by history and physical
examination.
● Management is based on the type of lesion; some mandate
resection, while others can be observed.
● Excision is performed to obtain a histopathological diagnosis,
treat symptoms, or improve a deformity.
Suggested Readings
Bartlett SP, Lin KY, Grossman R, Katowitz J. The surgical management of orbi-
tofacial dermoids in the pediatric patient. Plast Reconstr Surg. 1993; 91
(7):1208–1215
Hassanein AH, Gellis SE, Schmidt BA, Greene AK. Agminated atypical Spitz
tumor: large nasal lesion in a child with Down syndrome. J Pediatr Surg.
2011; 46(7):1435–1437
Hassanein AH, Alomari AI, Schmidt BA, Greene AK. Pilomatrixoma imitating
infantile hemangioma. J Craniofac Surg. 2011; 22(2):734–736
Hassanein AH, Couto JA, Greene AK. Circular excision and purse-string clo-
sure for pediatric facial skin lesions. J Craniofac Surg. 2015; 26(5):1611–1612
Pirouzmanesh A, Reinisch JF, Gonzalez-Gomez I, Smith EM, Meara JG. Piloma-
trixoma: a review of 346 cases. Plast Reconstr Surg. 2003; 112(7):1784–1789
Rosen H, Schmidt B, Lam HP, Meara JG, Labow BI. Management of nevus seba-
ceous and the risk of basal cell carcinoma: an 18-year review. Pediatr Derma-
tol. 2009; 26(6):676–681
Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S. Should
nevus sebaceus of Jadassohn in children be excised? A study of 757 cases,
and literature review. J Craniofac Surg. 2003; 14(5):658–660
Fig. 43.13 An adolescent patient with verruca
vulgaris on the brow.
Pediatric Skin Lesions
417

418
Index
Note: Page numbers set bold or italic
indicate headings or figures,
respectively.
2
22q deletion 133, 158, 164
A
Acrochordon 411
Adenomatoid odontogenic tumor 85
Adhesion cheiloplasty 152
Aesthetic units 397
AF, see Ameloblastic fibroma (AF)
AFO, see Ameloblastic fibro-odontoma
(AFO)
Aggressive fibromatosis 93
Airway management
– in facial paralysis 236
– in Robin's sequence 165
Alloplastic implants, in microtia 211
Alopecia 246, 246, 247–248
ALTF, see Anterolateral thigh flap (ALTF)
Ambiguous genitalia 283–284, 285
Ameloblastic fibro-odontoma
(AFO) 85, 87
Ameloblastic fibroma (AF) 85, 88–90
Amniotic band disruption 164
Amputations, in hand trauma 377
Anesthesia, in unilateral cleft lip re-
pair 146
Aneurysmal bone cyst 82, 83
Anterior cranial distraction 109
Anterior trunk disorders
– complications with 274
– diagnosis of 272
– nonoperative treatment of 273
– operative treatment of 273
Anterolateral thigh flap (ALTF), in phal-
loplasty 286, 288
Antibiotics, in soft-tissue injury re-
pair 188
Apert syndrome
– cephalometry in 18
– classification of 20
– cleft palate in 158
– clinical features of 17–18
– craniosynostosis in 3
– diagnosis of 18, 18
– surgical management in 25
– syndactyly in 330, 330, 330
Aphallia 283–284
Arnold-Chiari malformation 4
Arteriovenous malformation (AVM) 96,
383, 385, 387, 391, 391
Articulation errors, in velopharyngeal
dysfunction 179
Auricular rectangle 212, 213
Auricular sinus tracts 200
Auricular template 213, 214
AVM, see Arteriovenous malformation
(AVM)
Axes of development 304
B
Babysitter procedure 233, 234
BBC, see Buccal bifurcation cyst (BBC)
Beckwith-Wiedemann 164
Bilateral coronal synostosis 13, 14
BL, see Burkitt's lymphoma (BL)
Bladder exstrophy 271, 273, 274, 283–
284, 286
– See also Anterior trunk disorders
Blue rubber bleb nevus syndrome
(BRBNS) 385
Botulinum toxin, in facial paralysis 235
Box osteotomy, in craniofacial cleft 59
Brachial plexus palsies
– active movement scale in 344
– assessment of 343
– in isolated neuroma of upper
trunk 352, 352, 353–354
– indications for surgical exploration
in 344, 346, 346
– initial assessment of 343
– intraoperative assessment in 350
– Mallet scale for 359
– musculoskeletal considerations and
complications in 369, 370
– neonatal 343, 344, 351, 355, 360
– nerve exploration and reconstruc-
tion in
–– clavicular suspension in 350, 350,
351
–– elbow in 367, 369
–– exposure in 346
–– in infraclavicular region 349
–– in retroclavicular region 349, 349
–– in supraclavicular region 346, 346,
347–348
–– investigations prior to 346
–– outcomes in 363
–– positioning in 346, 346
–– postoperative care in 363, 367
–– secondary 365
–– shoulder in 365, 367–369
–– traumatic 356
– nerve grafting in 354, 357
– nerve transfers in 358
–– fascicular transfer for elbow flex-
ion 362, 363
–– intercostal to musculocutane-
ous 362, 366
–– spinal accessory to suprascapu-
lar 358, 361
–– triceps to axillary 359, 362, 364–
365
– nonsurgical treatm ent of 344
– pan plexus 353–355, 357–359
– presentation of 343
– traumatic 343–344, 356
– upper 352, 352, 353, 353, 354–356
–– C5/C5 avulsion in 355
–– with delayed presentation 355
–– with isolated deficits 355, 360
– with failed reconstruction of elbow
flexion 355
Brachycephaly, as term 28
–– See also Deformational flattening
Branchio-oculo-facial syndrome 158
BRBNS, see Blue rubber bleb nevus syn-
drome (BRBNS)
Breast asymmetry
– complications in 269
– diagnosis of 264
– etiology of 264, 265
– nonsurgical management of 264,
266
– operative treatment of 267
– postoperative management in 269
– psychosocial aspect of 264, 267
Brown tumor of hyperparathyroid-
ism 88
Buccal bifurcation cyst (BBC) 81, 82
Burkitt's lymphoma (BL) 94
Burns
– aesthetic units and 397
– complications with 399
– contour in operative treatment
of 27, 397
– contractures in 395, 396
– diagnosis of 394
– donor sites in 397
– early excision and grafting in 394,
394
– flaps in 399, 402
– grafting in 398, 400
– laser therapy in 399
– nonoperative management of 396
– operative treatment of 396
– proportion in operative treatment
of 27, 397
– scars as donor sites in 394, 395
– tension in operative treatment
of 397
– Z-plasty in 398
C
Capillary malformation (CM) 383, 385,
387–388, 389
Capillary malformation-arteriovenous
malformation (CM-AVM) 386
Cartilage harvesting, in microtia re-
pair 215
Cementoblastoma 84, 85
Central polydactyly 236–237, 332
– complications in 338
– nonoperative management of 334
– operative treatment of 336, 337
Central ray deficiency 311, 312, 312–
313
CFM, see Craniofacial microsomia
(CFM)
CHARGE 164
Cheek deformities
– diagnosis of 243
– nonoperative treatment of 243
– operative treatment of 249
Cherubism 88, 91
Cleft hand, see Central ray deficiency
Cleft lip and palate
– bilateral cleft lip
–– adhesion cheiloplasty in 152
–– anatomy in 149
–– classification of 149, 149
–– gingivoperiosteoplasty and 154
–– labial closure in 154
–– labial dissection in 153
–– late presentation of 151
–– marking with 152
–– nasal correction in 154, 174
–– nasal vestibule and sill closure
in 154
–– nasolabial adhesion and 151, 152
–– orthopedics in 151
–– postoperative care with 155
–– premaxillary ostectomy and 152,
153
–– presentation in infancy 150
–– primary repair of 152, 154
–– secondary reconstruction of 170,
171
–– skin closure in 155
–– surgical planning for 150
–– surgical preparation for 151
–– terminology in 149, 149
–– treatment algorithm 150, 151
– cephalometry in 125, 125
– cleft palate
–– anatomy in 156, 156, 157–158
–– classification of 157, 157
–– complications with 161, 161, 162
–– diagnosis of 158, 158
–– embryology in 156
–– epidemiology of 157
–– feeding in 159
–– Furlow's palatoplasty in 160, 161
–– nonoperative management of 158
–– normal anatomy and 156, 156
–– operative treatment of 158, 159,
160–161
–– palatoplasty in 159, 160–161
–– submucous 157, 158
–– timing of surgery for 159
–– two-flap palatoplasty in 159, 160
– dental care in 120
– fixed orthopedic treatment in 121,
122, 122, 123
– isolated cleft palate in 130
– nasal reconstruction in, secon-
dary 172, 173–175
– nasoalveolar molding in 121, 121
– orthopedics in 121, 121, 122–124
– retention in orthodontic treatment
of 130
– secondary reconstruction in
–– bilateral 170, 171, 174
–– timing of 169
–– unilateral 170
–– vermilion deficiency and 170
–– vertically short lip in 170
–– white roll misalignment in 170
– taping in 121, 121
– unilateral cleft lip
–– adjunctive treatment with, and na-
sal deformity 138, 139–140
–– anatomy in 134, 135
–– anesthetic considerations with 146
–– anthropometry in 135, 136
–– classification of 133
–– cleft nasal deformity in 135, 136,
143
–– closure in 142, 142, 144
–– complications with 146
–– cupid's bow in 147
–– dissection in 141, 142
–– evolution of care for 133
–– gingivoperiosteoplasty in 137, 139
–– lip adhesion in 137, 139
–– lip scars in 147
–– markings in 140, 141–144
–– microform 144, 145
–– mucosal variance in 147
–– operative treatment of 134, 137,
138–144

Index
419
–– orbicularis oris in 147
–– orthopedics in 136, 137–138
–– philtrum in 147
–– postoperative care with 144
–– preoperative management in 136
–– secondary deformities with 146
–– secondary nasal deformity
with 147
–– secondary reconstruction with 170
–– technical di erences with 144, 146
–– vermilion in 147
– velopharyngeal dysfunction in 177
– with alveolar involvement 126, 126,
127–130
Cleft-associated syndrome 21
Clefts, craniofacial, see Craniofacial cleft
(s)
Cloacal exstrophy 283–284, 286
CLOVES syndrome 391
CM, see Capillary malformation (CM)
CM-AVM, see Capillary malformation-
arteriovenous malformation (CM-
AVM)
Commissuroplasty 39
Complications
– in anterior trunk disorders 274
– in breast asymmetry 269
– in cleft palate 161, 161, 162
– in cranial remodeling 26
– in craniofacial clefts 62
– in craniofacial microsomia 42
– in cranioplasty 78
– in craniosynostosis 15
– in ear reconstruction 206
– in hypertelorism 49
– in jaw deformities 117
– in lower extremity procedures 299
– in macrodactyly 341
– in melanocytic nevi 409
– in microtia 219
– in nasal reconstruction 225
– in neck deformities 251
– in phalloplasty 291
– in polydactyly 337
– in posterior trunk disorders 280
– in radial longitudinal deficiency 311
– in reduction mammaplasty 257
– in Robin's sequence 168
– in scalp deformities 251
– in soft-tissue injury 192
– in syndactyly 331
– in thumb hypoplasia 322
– in transverse arrest 309
– in unilateral cleft lip 146
– in velopharyngeal dysfunction 183
Congenital hemangioma 383, 384,
387–388
Congenital limb defect (CLD), see Cen-
tral ray deficiency
Constriction ring syndrome (CRS) 292,
293
Cornelia de Lange syndrome 158, 164
Cranial distraction, in craniosynosto-
sis 7, 9, 11
Cranial remodeling
– complications in 26
– in craniosynostosis 7, 8, 9, 9–10
Cranial sutures 2, 3
– See also Craniosynostosis
Craniofacial cleft(s)
– bone reconstruction in 58
– box osteotomy in 59, 59
– complications in 62
– dentistry in 58
– embryology in 51
– encephalocele in 59, 60, 61
– facial bipartition in 59, 60
– feeding in 57
– hypertelorism in 58
– nasal reconstruction in 58
– neurologic assessment in 58
– nonoperative management of 57
– number 0 53
– number 1 51, 53
– number 2 53
– number 3 54, 54
– number 4 54, 54
– number 5 54
– number 6 54, 55, 61
– number 7 55, 55, 61
– number 8 55, 62
– number 9 55, 56
– number 10 52, 54
– number 11 56, 57
– number 12 56
– number 13 57, 57
– number 14 57
– number 30 57
– nutrition with 57
– operative management of 58
– orthodontia in 58
– perioral reconstruction in 58
– periorbital reconstruction in 58
– soft issue reconstruction in 58
– speech in 58
Craniofacial growth patterns 63
Craniofacial microsomia (CFM)
– classification of 34
– commissuroplasty in 39
– complications in 42
– diagnosis of 34, 35–37
– di erential diagnosis of 36
– ear in 34, 40, 43
– evaluation of 38
– facial reanimation in 41, 43
– mandible in 34, 37, 39, 42
– natural history in 38
– nerves in 36
– noncraniofacial manifestations of 36
– operative treatment of 38, 38, 39,
41–42
– orbit in 34, 36
– orbital reconstruction in 39
– soft tissue in 36, 41, 43
– Treacher Collins vs. 36
– workup for 38
Craniofrontonasal syndrome 3
Cranioplasty
– complications with 78
– diagnosis and 73
– inlay 74, 75–80
– materials in 80
– onlay 73, 74, 80
Craniosynostosis
– anatomy and pathoanatomy in 2, 3
– bilateral coronal 13, 14
– classification of 3
– complications in 15
– cranial development and 2, 3
– cranial remodeling in 7, 8, 9, 9–10
– deforming forces in 7
– diagnosis of 4
– distraction of cranium in 7, 9, 11
– dural tears in 15
– frontoorbital advancement in 9
– functional aspects in 4, 5
– genetics in 2
– hydrocephalus in 4
– imaging in 4
– in Treacher Collins syndrome 17
– intracranial pressure in 3, 4, 5
– lambdoid 12, 13
– mental impairment in 5
– metopic 9, 10
– minimally invasive techniques in 6,
6, 23
– perioperative considerations in 7
– sagittal 8
– sagittal synostosis 7
– syndromic 16–17
– team approach in 6
– tim ing of surgical intervention in 6
– treatment of 6, 6, 7, 22
– unilateral coronal 10, 12
– unusual variants in 13, 14–15
– visual abnormalities in 5
Crouzon syndrome 17–18, 20
– distraction osteogenesis in 111
– surgical management of 26
CRS, see Constriction ring syndrome
(CRS)
Cryptotia 195, 203, 206
Cutis aplasia 243, 243
Cytokines, in distraction osteogene-
sis 100
D
Debridement, of soft tissue inju-
ries 190, 191
Deformational flattening
– assessment of 30, 31–32
– developmental delay and 32
– diagnosis of 28
– early identification of 30
– etiology of 30, 30
– helmet therapy in 32, 33
– imaging in 29
– in older infants 31
– risk factors for 30
– terminology with 28
– treatment of 30
Dental care, with cleft lip and pal-
ate 120
Dentistry, in craniofacial clefts 58
Dentoalveolar fracture 70
Dermoid cyst 411, 412, 412
Desmoplastic fibroma 94
Distraction of cranium, in craniosynos-
tosis 7, 9, 11
Distraction osteogenesis (DO)
– advantages of 99
– anterior cranial 109
– bone graft and flap 109, 110
– consolidation in 101
– cytokines in 100
– devices 101, 101, 102–103
– disadvantages of 99
– frequency of 101
– history of 99
– imaging in 102, 103
– in craniofacial microsomia 38, 39–
40, 40
– in Crouzon syndrome 111
– in Le Fort i osteotomy 104, 106
– in mandible 34, 103, 104, 105–106
– in midface 107
– in posterior vault 108, 110
– in Robin's sequence 165, 167
– Le Fort II 107
– Le Fort III 107, 107
– neonatal midface 108
– outcomes in 100
– phases of 101
– principles of 99, 100
– protocols 101
– rate of 101
– resorbable devices in 102
– scientific principles of 100, 100
– specific applications of 103
DO, see Distraction osteogenesis (DO)
Dressings, in soft-tissue injury re-
pair 192
E
Ear
– abnormal helical antihelix in 195
– abnormal helical root in 195
– accessory cartilage in 195, 198, 199
– anatomy 196, 212, 212, 213
– anomaly types 195
– constricted 196, 198, 202, 203–205
– in craniofacial microsomia 34, 40, 43
– in cryptotia 195, 203, 206
– in Stahl deformity 197, 198, 205
– lobule abnormality in 195, 199,
200–202
– measurements of 195
– prominent 195, 197, 198, 200, 202
– sinus tracts in 200
Ear abnormalities, see Ear reconstruc-
tion, Microtia
– diagnosis in 195, 195, 196
– molding of 197, 198
– nonoperative treatment of 197, 198
– operative treatment of 197, 199–208
– residual 198
Ear positioning template (EPT) 213,
213, 215
Ear reconstruction, see Ear abnormal-
ities
– antibiotics in 198
– complications in 206
– congenital lobule deficiency in 201–
202
– constricted ear in 202, 203–205
– cryptotia in 203, 206
– expanded lobule in 200, 201
– for keloid in lobule 199
– history of autogenous 210
– in hemifacial microsomia 218
– in microtia 211, 211, 212–219
– indications for 197
– lobule defects in 199, 200
– middle third defects in 205
– prominent ear in 200, 202
– sinus tracts in 200
– split earlobe in 199, 200
– Stahl deformity in 205
– timing of 198
– upper third defects in 205
– with accessory cartilage 198, 199
ECPR, see Elastic chain premaxillary re-
traction (ECPR)
EEC, see Exstrophy-epispadias complex
(EEC)
Elastic chain premaxillary retraction
(ECPR) 151
Emanuel syndrome 158
Embryology
– in cleft palate 156

Index
420
– in craniofacial clefts 51
– in hand di erences 304
– in nasal reconstruction 221, 222
Encephalocele, in craniofacial clefts 59,
60, 61
Epidermal cyst 411, 412, 413
Epidermal nevus 411, 413, 413
Epispadias 283–284
EPT, see Ear positioning template (EPT)
Ewing's sarcoma 95
Exstrophy-epispadias complex
(EEC) 283
Extravasation, intravenous 186, 189
F
Facial asymmetry, in jaw deform-
ity 113
– botulinum toxin in 235
Facial bipartition
– in craniofacial clefts 59, 60
– in hypertelorism 6
Facial expression 228, 228, 229–230
Facial fractures
– cranial/frontal sinus 65, 66–67
– dentoalveolar 70
– diagnosis of 64
– growth patterns and 63
– imaging in 65, 67–68
– in children vs. adults 63, 64
– initial assessment in 64
– intraoral examination in 65
– mandible 70
– maxillary 70
– nasal 69
– naso-orbito-ethmoid 67
– orbital 67
– outcomes in 71, 72
– panface 71
– physical examination in 64
– zygomaticomaxillary 67
Facial growth patterns 63
Facial muscles 228, 228, 229–230
Facial nerve anatomy 228, 228, 229–
230
Facial paralysis
– anatomy in 228, 228, 229–230
– appearance problems in 233
– assessment of 229, 231–232
– babysitter procedure in 233, 234
– brow procedures in 233
– co-morbidities in 227
– diagnosis of 229, 231–232
– etiology of 227, 231
– expectations in 227
– functional impairment in 232
– in adults vs. children 227
– in craniofacial microsomia 36
– lower eyelid in 236, 236
– nasal airway in 236
– neural plasticity and 227
– nonoperative management of 233
– ocular protection and 232
– operative management of 233, 234,
234–239
– oral commissure in 237, 237, 238–
241
– psychosocial factors in 227
– reconstructive aims with 232
– smile types and 230
– synkinesis in 233
– upper eyelid in 233, 235
– upper lip in 237, 237, 238–241
Feeding
– in Robin's sequence 165
– with cleft palate 159
– with craniofacial clefts 57
Fetal alcohol syndrome 164
Fibrous dysplasia 92
– monostotic 88, 92
Fibrous-osseous lesions 88
Fibula osteocutaneous free flap
(FOCFF), in phalloplasty 288
Fingertip injury, in hand trauma 376,
379
Five-fingered hand 317, 319
Flaps, in burns 399, 402
Flattening, see Deformational flattening
Flexor tendon injury, in hand trau-
ma 374, 377
Floating thumb 317, 318
– See also Thumb hypoplasia
Fluoroscopy, in velopharyngeal dys-
function 180
fMRI, see Functional magnetic reso-
nance imaging (fMRI)
FOCFF, see Fibula osteocutaneous free
flap (FOCFF)
Fractures
– facial
–– cranial/frontal sinus 65, 66–67
–– dentoalveolar 70
–– diagnosis of 64
–– growth patterns and 63
–– imaging in 65, 67–68
–– in children vs. adults 63, 64
–– initial assessment in 64
–– intraoral examination in 65
–– mandible 70
–– maxillary 70
–– nasal 69
–– naso-orbito-ethmoid 67
–– orbital 67
–– outcomes in 71, 72
–– panface 71
–– physical examination in 64
–– zygomaticomaxillary 67
– hand 372, 373
–– phalangeal condylar neck 373
–– Salter-Harris type II 372, 373
–– Seymour fracture 373, 376
–– ulnar collateral ligament injury var-
iant in 372, 375
– remodeling 372, 374
Frontoorbital advancement (FOA), in
craniosynostosis 9, 13, 22, 24, 25
Functional magnetic resonance imaging
(fMRI), in velopharyngeal dysfunc-
tion 180
Furlow's palatoplasty
– in cleft palate 160, 161
– in velopharyngeal dysfunction 181
G
Gastroschisis 271–272, 273
– See also Anterior trunk disorders
Gastrulation 304
Genetics
– in Apert syndrome 18
– in craniosynostosis 2
– in microtia 210
– in Robin's sequence 163
– in Treacher Collins syndrome 22
– in vascular malformations 384
Genital anomalies
– common 284
– diagnosis of 283
– nonoperative management of 283
– phalloplasty in 285
– preoperative assessment in 283
GHD, see Glenohumeral dysplasia
(GHD)
Giant cell lesions 86
Gingivoperiosteoplasty 154
Glenohumeral dysplasia (GHD) 346,
363, 365, 367, 369
Goldenhar's syndrome 36
Gorham-Stout disease 98, 98
Grafting
– nerve, in brachial plexus pal-
sies 354, 357
– skin
–– in burns 398, 400
–– in melanocytic nevi 406, 406
Granuloma annulare 411, 413, 413
Growth patterns 63
Gynecomastia 261–262
– complications with 262
– diagnosis of 258
– endocrine-related 261
– grading of 259, 260
– nonoperative treatment of 260
– operative treatment of 261
– physical exam in 260
– presentation of 258
– secondary to drug use 261
– self-resolving 258
– workup for 260
H
Hairline, in microtia 211
Hand, cleft, see Central ray deficiency
Hand di erences, see Upper limb failure
of formation disorders
– and axes of development 304
– classification of 305–306
– embryology in 304
– limb bud development and 304
– Oberg-Manske Tonkin classification
of 306
– skeletal di erentiation and 305
– soft-tissue deficiencies in 308
– soft-tissue di erentiation and 305
– Swanson classification of 305
Hand trauma
– amputations in 377
– diagnosis of 372
– fingertip injury in 376, 379
– fractures as 372, 373
–– phalangeal condylar neck 373
–– Seymour fracture 373, 376
–– ulnar collateral ligament injury var-
iant of 372, 375
– joint dislocation in 373
– joint injuries in 373
– nerve injury in 375, 378
– replantation in 379, 380
– tendon injury in 374, 377
Harvesting
– of costal cartilage for microtia re-
pair 215
– of skin for microtia repair 218
Helmet therapy, in deformational flat-
tening 32, 33
Hemifacial atrophy 249, 250–251
Hemifacial microsomia, auricular re-
construction in 218, 219
Holt-Oram syndrome 310, 315
Horizontal dystopia, see Hypertelorism
Humerus osteotomy, in glenohumeral
dysplasia 367, 369
Hydrocephalus, in craniosynostosis 4
Hyperparathyroidism, brown tum or
of 88
Hypertelorism
– complications in 49
– diagnosis of 44, 45
– facial bipartition in 6
– in craniofacial cleft 58
– in craniosynostosis 5
– nonoperative treatment of 45
– operative treatment of 45, 46, 48–49
– orbital box osteotomy in 46
– osteotomies in 48
– postoperative management in 50
Hypospadias 284, 287
– See also Phalloplasty
I
ICP, see Intracranial pressure (ICP), Iso-
lated cleft palate (ICP)
Idiopathic osteosclerosis 97
IH, see Infantile hemangioma (IH)
Imaging
– in craniosynostosis 4
– in deformational flattening 29
– in distraction osteogenesis 102, 103
– in ear deformity 195
– in facial fracture 65, 67–68
– in jaw deformities 112
– in velopharyngeal dysfunction 180
– radiation exposure and 65
Implants, in microtia 211
Index finger pollicization 321, 323
Infantile hemangioma (IH) 382, 383,
386, 386, 388, 388
Infantile osteoporosis 97
Inflammatory cysts 81
Inguinal hernia 273
Inlay cranioplasty 74, 75–80
Intracranial pressure (ICP), in craniosy-
nostosis 3, 4, 5
Intravelar veloplasty, in cleft pal-
ate 159
Intravenous extravasation 186, 189
Irrigation, of soft tissue injuries 190
Isolated cleft palate (ICP) 130
J
Jaw deformities
– classification of 112, 113
– complications with 117
– diagnosis of 112
– facial asymmetry in 113
– facial examination in 112
– fixation failure with 118
– hemorrhage with 117
– imaging in 112
– infection with 118
– Le Fort I osteotomy in 113, 114
– malocclusion with 117
– neurosensory impairment with 117
– nonunion in 118
– operative management of 113, 114–
116
– sagittal split osteotomy in 115, 116
– vertical maxillary excess in 113

Index
421
Joint dislocation 373
Joint injury, in hand trauma 373
Juvenile xanthogranuloma 411, 414
K
Kaposiform hemangioendothelioma
(KHE) 383, 385, 387–388
Keloid 199, 411, 414, 414
Keratocystic odontogenic tumor 85, 86
KHE, see Kaposiform hemangioendo-
thelioma (KHE)
Klippel–Trénaunay syndrome 391
L
Lacerations 173, 186, 188
– See also Soft-tissue injury(ies)
Lambdoid craniosynostosis 12, 13
Langerhans cell histiocytosis 94, 94
Latissimus dorsi myocutaneous flap
(LDMF), in phalloplasty 286
LDMF, see Latissimus dorsi myocutane-
ous flap (LDMF)
Le Fort fractures 64
Le Fort I osteotomy
– distraction osteogenesis in 104, 106
– in jaw deformities 113, 114
Le Fort II distraction osteogenesis 107
Le Fort III advancement 23, 24
Le Fort III distraction osteogene-
sis 106–107, 107, 108
Limb bud development 304
Lipoma 411, 414, 414
Liposuction, in lymphedema 293, 293
LM, see Lymphatic malformation (LM)
Longitudinal vaginal septum 284
Lower extremity disorders
– constriction ring in 292
– diagnosis of 292
– nonoperative treatment of 292
– operative treatment of 292
– reconstruction following removal of
skin lesions in 294, 295–297
– traumatic 298, 298, 299–301
Lymphatic malformation (LM) 169,
383, 385, 387, 390
Lymphedema
– constriction ring syndrome in 292,
293
– diagnosis of 292
– nonoperative treatment of 292
– operative treatment of 293, 293
M
Macrodactyly
– complications in 341
– diagnosis of 340, 340
– nonoperative treatment of 341, 341
– operative treatment of 341, 341
Macromastia 254
– diagnosis of 254
– estrogen antagonists for 260
– indications for reduction mamma-
plasty in 255
– nonoperative treatment of 255
– operative treatment of 255
– psychosocial component of 255
Magnetic resonance imaging (MRI), see
Functional magnetic resonance
imaging (fMRI), Imaging
Mallet scale 359
Mandible
– development of 63
– distraction osteogenesis in 34, 103,
104, 105–106
– fractures 70
– in craniofacial microsomia 34, 37,
39, 42
Mandibular distraction, in Robin's se-
quence 165, 167
Maxilla, development of 63
Maxillary excess, vertical 113
Maxillary fracture 70
Melanocytic nevi
– complications with 409
– diagnosis of 403
– giant 406
– in extremities 408, 409
– in head and neck 406, 407–408
– in trunk 408, 408, 409
– large 406
– medium 403, 404–405
– nonoperative management of 403
– operative management of 403
– skin grafting in 406
– small 403, 404
– tim ing of resection of 403
– tissue expansion in 406, 407
Melanotic neuroectodermal tumor of
infancy (MNTI) 94, 96
Mental impairment, in craniosynosto-
sis 5
Metopic craniosynostosis 9, 10
Micropenis 283–284, 285, 287
– See also Phalloplasty
Microtia, see Ear abnormalities
– alloplastic implants in 211
– auricular curve analysis in 213, 214,
218
– auricular elevation in 215, 218–219
– auricular framework in 215
– auricular rectangle and 212, 213
– auricular template in 213, 214
– cartilage block in 218, 219
– clinical characteristics of 210
– complications with 219
– concha-type 215, 217
– ear positioning template in 213,
213, 215
– epidemiology of 210
– facial symmetry and 211
– genetics in 210
– hairline and 211
– history of autogenous reconstruction
in 210
– in hemifacial microsomia 218, 219
– in Treacher Collins syndrome 219
– middle ear problem in 210
– normal auricle anatomy and 212,
212, 213
– patient assessment in 211
– prosthetics in 211
– secondary construction in 220, 220
– sideburns in 212, 212
– skin envelope in 211
– tim ing of surgery for 210
– total auricular reconstruction
in 211, 211, 212–219
– trapezium space in 212, 212
– unsatisfactory outcomes with 219
– vestige skin in 211
Microvascular reconstruction, in radial
longitudinal deficiency 311
Midface
– development of 63
– distraction osteogenesis in 107
Midface distraction, neonatal 108
MNTI, see Melanotic neuroectodermal
tum or of infancy (MNTI)
Monostotic fibrous dysplasia 88
Mucocele 411, 414
Muenke's syndrome 3, 17, 20, 26
Multiple giant cell lesion syndrome 88,
91
Myelomeningocele 277, 278–279
Myxoma 93
N
NAM, see Nasoalveolar molding (NAM)
Nasal defects, see Nasal reconstruction
– development and 221
– diagnosis in 222, 222, 223
– embryology and 221, 222
– nonoperative repair of 223
Nasal fracture 69
Nasal reconstruction, see Nasal defects
– complications in 225
– in bilateral cleft lip 154, 174
– in craniofacial clefts 58
– in secondary cleft lip repair 172,
173–175
– nasal lining in 225
– nasal subunit theory of 224, 224
– skin in 225
– structure in 225
– techniques 224
– tim ing of 223
Nasoalveolar molding (NAM) 121, 121,
151
Nasolabial adhesion 151, 152
Nasopalatine duct cyst (NPDC) 82, 84
Nasopharyngoscopy, in velopharyngeal
dysfunction 178, 179
Neck deformities
– complications with 251
– diagnosis of 243
– nonoperative treatment of 243
– operative treatment of 250
Nerve grafting, in brachial plexus pal-
sies 354, 357
Nerve transfers, in brachial plexus
palsy 358
– fascicular transfer for elbow flex-
ion 362, 363
– intercostal to musculocutane-
ous 362, 366
– spinal accessory to suprascapu-
lar 358, 361
– triceps to axillary 359, 362, 364–365
Nerves, in craniofacial microsomia 36
Neurofibroma 94, 411, 415, 415
Nevoid basal cell carcinoma 85
Nevus sebaceous 411, 414, 415
NICH, see Noninvoluting congenital he-
mangioma (NICH)
Noninvoluting congenital hemangioma
(NICH) 385, 387–388
Noonan's syndrome 88, 91
NPDC, see Nasopalatine duct cyst
(NPDC)
Nutrition, with craniofacial clefts 57
O
Oberg-Manske Tonkin (OMT) classifica-
tion 306
Odontogenic cysts 81, 82
Odontogenic tumors 82, 84–91
Odontoma 84, 84
Omphalocele 271, 273, 275
OMT, see Oberg-Manske Tonkin (OMT)
classification
Onlay cranioplasty 73, 80
Opitz G/BBB syndrome 158
Oral-facial-digital syndrome 158
Orbicularis oris, in unilateral cleft lip
repair 147
Orbit
– in craniofacial clefts 58
– in craniofacial microsomia 34, 36
Orbital box osteotomy, in hypertelor-
ism 46
Orbital hypertelorism, see Hypertelor-
ism
Orbital reconstruction, in craniofacial
microsomia 39
Orthodontia, in craniofacial clefts 58
Osseous dysplasia 90
Ossifying fibroma 90
Osteoblastoma 92, 93
Osteoporosis, infantile 97
Osteosarcoma 95, 97
Osteosclerosis, idiopathic 97
Osteotomy
– box, in craniofacial cleft 59
– in hypertelorism 48
– Le Fort I
–– distraction osteogenesis in 104,
106
–– in jaw deformities 113, 114
– orbital box, in hypertelorism 46
– sagittal split, in jaw deformity 115,
116
P
Palatal split fractures 64
Palate anatomy 156, 156
– See also Cleft lip and palate
Palatoplasty
– Furlow's 160, 161, 181
– in cleft palate 159, 160–161
– in velopharyngeal dysfunction 181
– postoperative care in 160
– two-flap 159, 160
Panface fractures 71
Parkes Weber syndrome (PWS) 386,
392
Pectus carinatum 272, 273, 274
– See also Anterior trunk disorders
Pectus excavatum 271, 273, 274
– See also Anterior trunk disorders
Pedicled abdominal flap, in phallo-
plasty 285
Pedicled groin flap, in phalloplasty 285
Penis anomalies 284
Pfei er syndrome 17, 21
– classification of 20–21
– diagnosis of 20
– distraction osteogenesis in 110
– surgical management in 25
PG, see Pyogenic granuloma (PG)
Phalloplasty 285, 287
Pharyngeal flap, in velopharyngeal dys-
function 181, 183

Index
422
Pharyngeal wall augmentation, in velo-
pharyngeal dysfunction 182
Pharyngoplasty, sphincter, in velophar-
yngeal dysfunction 181
Phosphatase and tensin homolog ha-
martoma-tumor syndrome
(PHTS) 392
PHTS, see Phosphatase and tensin ho-
molog hamartoma-tumor syndrome
(PHTS)
Physical examination, in facial frac-
tures 64
Pilar cyst 411, 415, 416
Pilomatrixoma 411, 415, 416
Plagiocephaly, as term 28
–– See also Deformational flattening
Poland sequence 331
Poland syndrome 265, 266, 268, 268
Polydactyly
– central 236–237, 332
–– complications in 338
–– nonoperative management of 334
–– operative treatment of 336, 337
– complications in 337
– diagnosis of 334, 335
– nonoperative management of 334
– operative treatment of 335
– radial 332, 333
–– complications in 337, 338
–– nonoperative management of 334
–– operative treatment of 335, 336
– ulnar 332, 334–335
–– complications in 338
–– ligation of 334
–– nonoperative management of 334
–– operative treatment of 336
Posterior pharyngeal flap, in velophar-
yngeal dysfunction 181, 183
Posterior pharyngeal wall augmenta-
tion, in velopharyngeal dysfunc-
tion 182
Posterior trunk disorders
– complications with 280
– diagnosis of 277
– nonoperative treatment of 277
– operative treatment of 277
Posterior vault distraction osteogene-
sis 108, 110
Postoperative care
– in bilateral cleft lip repair 155
– in brachial plexus palsy nerve recon-
struction 363, 367
– in breast asymmetry 269
– in palatoplasty 160
– in reduction mammaplasty 256
– in soft-tissue injury repair 192
Postoperative considerations, in hyper-
telorism 50
PPP, see Progressive postnatal pansy-
nostosis (PPP)
Premaxillary ostectomy 152, 153
Pressure ulcer 279
Progressive postnatal pansynostosis
(PPP) 14, 15
Propranolol 387
Prosthetics
– in microtia 211
– in velopharyngeal dysfunction 180
Prune belly syndrome 272, 273, 274
– See also Anterior trunk disorders
PWS, see Parkes Weber syndrome
(PWS)
Pyogenic granuloma (PG) 383, 385,
387–388
R
Radial forearm free flap (RPFF), in phal-
loplasty 285, 288, 289–290
Radial longitudinal deficiency
(RLD) 310, 310, 310
Radial polydactyly 317
– complications in 337, 338
– nonoperative management of 334
– operative treatment of 335, 336
Radiation safety 65
Rapidly involuting congenital heman-
gioma (RICH) 385, 387–388
Reduction mammaplasty 255
– complications in 257
– indications for, in adolescents 255
– postoperative management in 256
Replantation, in hand trauma 379, 380
Resonance, in velopharyngeal dysfunc-
tion 179
RICH, see Rapidly involuting congenital
hemangioma (RICH)
RLD, see Radial longitudinal deficiency
(RLD)
Robin's sequence (RS)
– adjunct studies in 164
– airway management in 165
– cleft palate in 130
– complications in 168
– diagnosis of 163
– epidemiology of 163
– etiology of 163
– feeding in 165
– genetics in 163
– mandibular distraction in 165, 167
– nonoperative management of 164
– operative management of 165, 166–
167
– presentation of 164
– syndromic 164, 164
– tongue-lip adhesion in 165, 166
– tracheostomy in 167
RPFF, see Radial forearm free flap
(RPFF)
RS, see Robin's sequence (RS)
S
Saethre-Chotzen syndrome 3, 5, 17, 20
– surgical management of 26
Sagittal craniosynostosis 8
Sagittal split osteotomy, in jaw deform-
ity 115, 116
Sagittal synostosis 7
Salter-Harris type II fracture 372, 373
Scalp deformities
– alopecia 246, 246, 247–248
– complications with 251
– cutis aplasia 243, 243
– diagnosis of 243
– nonoperative treatment of 243
– operative treatment of 243
– skin lesions 244, 244, 245
– traumatic 246, 249
Scaphocephaly, as term 28
–– See also Deformational flattening
Secondary construction, in micro-
tia 220, 220
Secondary reconstruction, in cleft lip
– bilateral 170, 171
– nasal deformities in 172, 173–175
– tim ing of 169
– unilateral 170
– vertically short lip in 170
– white roll misalignment in 170
Sedation, in operative soft-tissue injury
treatment 187
Seymour fracture 373, 376
Short lip, cleft lip reconstruction
and 170
Sideburns, in microtia 212, 212
SIDS, see Sudden infant death syndro-
me (SIDS)
Sinus tracts, auricular 200
Skeletal di erentiation 305
Skin graft
– in melanocytic nevi 406, 406
– in microtia 218
Skin grafting, in burns 398, 400
Skin lesions
– common 411
– diagnosis of 411
– nonoperative management of 412
– operative management of 412
Smile types 230
Soft-tissue di erentiation 305
Soft-tissue distraction, in radial longi-
tudinal deficiency 311, 311
Soft-tissue injury(ies)
– complications in 192
– debridement of 190, 191
– diagnosis of 186, 188–189
– intravenous extravasation 186, 189
– irrigation of 190, 191
– lacerations 173, 186, 188
– nonoperative treatment of 186,
188–189
– operative treatment of 187, 189–193
–– absorbable sutures in 172
–– antibiotics in 188
–– dressings for 192
–– indications for 187, 190
–– nylon sutures in 172
–– postoperative management for 192
–– sedation in 187
–– wound inspection in 190, 190, 191
–– wound repair in 191, 192
Speech
– in craniofacial clefts 58
– in velopharyngeal dysfunction 179
Sphincter pharyngoplasty, in velophar-
yngeal dysfunction 181
Spinal wound coverage 280, 280, 281
Spitz nevus 411, 415, 416
Stahl deformity 197, 198, 205
Stickler's syndrome 158
Strabismus, in craniosynostosis 5
Sturge-Weber syndrome (SWS) 392
Sudden infant death syndrome
(SIDS) 28
Sutures, nylon, in children 172
Swanson classification 305
SWS, see Sturge-Weber syndrome
(SWS)
Symbrachydactyly 309
Syndactyly
– anatomy in 326, 326
– as lower extremity disorder 294,
294
– classification of 325, 325
– complications in 331
– defined 325
– diagnosis of 325
– etiology of 325
– in Apert syndrome 330, 330, 330
– in Poland sequence 331
– nail fold reconstruction in 330
– nonoperative management of 325
– operative treatment of 326, 327–
329, 330, 330
– release of complete simple 328, 328,
329
– release of first webspace in 327, 327
– release of incomplete 327
– tim ing of surgery for 326, 327
Syndactyly release 25
Synkinesis, facial paralysis and 233
T
Telorbitism, see Hypertelorism
Tendon injury, in hand trauma 374,
377
Tessier cleft 51, 53, 224
– See also Craniofacial cleft(s), Hyper-
telorism
Thumb hypoplasia
– aplasia 317, 319
– associated conditions in 315
– classification of 315, 315
– complications in 322
– defined 315
– diagnosis of 315, 315
– first webspace deficiency in 320,
320
– five-fingered hand 317, 319
– floating thumb 317, 318
– in radial polydactyly 317
– index finger pollicization in 321,
323
– metacarpophalangeal joint instabil-
ity in 320, 321
– mild 315
– moderate 316, 316
– nonoperative treatment of 317
– operative treatment of 319, 320
– outcomes in 322
– palmar abduction and opposition
in 321, 322
– severe 316, 317
Thumb metacarpophalangeal joint dis-
location 373
Tissue expansion, in melanocytic ne-
vi 406, 407
TLA, see Tongue-lip adhesion (TLA)
Tongue-lip adhesion (TLA), in Robin's
sequence 165, 166
Tracheostomy, in Robin's sequence 167
Transverse arrest 309, 309
Transverse vaginal septum 284
Trapezium space, in microtia 212, 212
Trauma, see Soft-tissue injury(ies)
– hand
–– amputations in 377
–– diagnosis of 372
–– fingertip injury in 376, 379
–– fractures as 372, 373
––– phalangeal condylar neck 373
––– Seymour fracture 373, 376
––– ulnar collateral ligament injury
variant of 372, 375
–– joint dislocation in 373
–– joint injuries in 373
–– nerve injury in 375, 378
–– replantation in 379, 380

Index
423
–– tendon injury in 374, 377
– lower extremity 298, 298, 299–301
– posterior trunk 280, 281
– to scalp 246
Treacher Collins syndrome 17, 21, 22
– auricular reconstruction in 219
– cleft palate in 158
– craniofacial clefts in 54
– craniofacial microsomia vs. 36
– microtia in 219
– surgical management of 26
Trisomy 13 158
Tuberous breast deformity 266, 267,
267
Tumors, odontogenic 82, 84–91
Turner syndrome 250
U
Ulcer, pressure 279
ULD, see Ulnar longitudinal deficiency
(ULD)
Ulnar collateral ligament injury, in
hand fracture 372, 375
Ulnar longitudinal deficiency
(ULD) 313, 314, 314
Ulnar polydactyly 332, 334–335
– complications in 338
– ligation of 334
– nonoperative management of 334
– observation of 334
– operative treatment of 336
Umbilical hernia 273, 273, 274
Unilateral coronal synostosis 10, 12
Upper limb failure of formation disor-
ders, see Hand di erences
– central ray deficiency in 311, 312,
312–313
– longitudinal deficiencies in 310, 310,
310
– transverse deficiencies in 309, 309
– ulnar longitudinal deficiency in 313,
314, 314
V
VACTERL 310, 315
Vaginal agenesis 284
Vaginal anomalies 284
Van der Woude syndrome 158
Vascular anomalies
– classification of 382, 383
– craniofacial 95, 96–98
– diagnosis of 382, 384
– genetics of 384
– incorrect terminology with 382
– intralesional corticosteroid for 386
– laser therapy for 387
– nonoperative management of 386
– operative treatment of 388, 388,
389–391
– systemic pharm acotherapy for 386,
386
– terminology with 382
– topical pharm acotherapy for 386
Vascular malformation overgrowth
syndromes 391
Velopharyngeal dysfunction (VFD)
– articulation errors in 179
– audible nasal emissions in 179
– causes of 177
– complications in 183
– defined 177
– diagnosis of 179
– fluoroscopy in 180
– functional MRI in 180
– Furlow palatoplasty in 181
– in cleft palate 177
– instrumental evaluation in 179
– nasopharyngoscopy in 178, 179
– nonoperative management of 180
– operative management of 180, 181,
182–183
– palatoplasty in 181
– perceptual evaluation in 179
– posterior pharyngeal flap in 181,
183
– posterior pharyngeal wall augmenta-
tion in 182
– prosthetics in 180
– resonance in 179
– sphincter pharyngoplasty 181
– treatment algorithm 183
– types of 178
– velopharyngeal incompetence
in 177
– velopharyngeal insu ciency in 178,
178
– velopharyngeal mislearning in 179
– veloplasty in 181
– visualization in 179
Velopharyngeal incompetence 177
Velopharyngeal insu ciency 178, 178
Velopharyngeal mislearning 179
Velopharynx anatomy 177
Veloplasty
– in cleft palate 159
– in velopharyngeal dysfunction 181
Venous malformation (VM) 383, 385,
387–388, 389
Vermilion deficiency 170
Verruca vulgaris 411, 417
Vertical maxillary excess (VME) 113
VFD, see Velopharyngeal dysfunction
(VFD)
Virchow's law 2, 3
Visual abnormalities, in craniosynosto-
sis 5
VM, see Venous malformation (VM)
W
White roll misalignment 170
Wrist correction, in radial longitudinal
deficiency 311
X
Xanthogranuloma, juvenile 411

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