Operative Technique in Plastic Surgery – Kevin C.Chung

■ Further movement of the flap may be needed in the following situations:
■ Nevus involvement of the root of the nose or nasal sidewall
■ Nevus involvement of the upper lip
■ In these cases, the inferior and medial-most extent of the flap can reliably
reach these extremes. The additional backcuts into the flap should be
designed to allow this.
■ For all backcuts and incisions within the substance of the expanded flap,
care should be taken to include sufficient capsule with the smaller or more
tenuous of the created flaps. This helps to maximize vascular supply within
the flap, particularly the venous drainage.
■ This may require beveling of the incisions in some cases, capturing more
of the subcutaneous tissue and particularly the capsule.
■ Upon flap transposition, a dog-ear of excess tissue will be apparent in the
temporal region.
■ This is excised above the level of the lateral canthus, allowing secure flap
fixation high enough to prevent the development of ectropion due to flap
descent.
■ At this stage, the port for the expander is close to the level of dissection,
and removal is relatively easy.
■ Excision
■ Once the flap release is completed, the extent of nevus excision can be
decided.
■ The defect created by excision should allow reconstruction without
excessive tension on the flap.
■ In general, the superior extent of excision should be the lower eyelid-cheek
junction. When the nevus also involves the lower eyelid, the very thin
eyelid skin is best reconstructed with a full-thickness skin graft at a later
stage.
■ The excision of nevus in this area should be delayed until the cheek flap is
fully secured both medially and laterally.
■ If possible, the triangle of skin immediately lateral to the alar-cheek
junction should be preserved if not involved by nevus.
• This allows the scar in the nasolabial groove to terminate at the nose-
cheek junction rather than the alar base.

■ The nevus is excised at the level of the superficial musculoaponeurotic
system (SMAS), and hemostasis is achieved with bipolar cautery (TECH
FIG 2).
■ This can be performed in stages, securing the flap to the deeper tissues at
several points as the dissection progresses superiorly.
■ This allows the most accurate determination of the flap’s reach, ensuring
that the distal portion of the flap is not placed under excessive tension due
to overly aggressive nevus excision.
TECH FIG 2 • The nevus is excised at the level of the
superficial musculoaponeurotic system (SMAS).
■ Suturing
■ Deep sutures can be used to serve dual purposes.
■ Suturing the deeper tissue of the flap to the underlying periosteum with
clear nylon can help prevent later downward migration of the flap.
■ Suturing the more superficial tissue of the flap to deeper tissues can help
recreate anatomical grooves or folds (eg, at the alar base–cheek junction).
At this site, through-and-through sutures into the nasal cavity are often
used. Based on the requirements of the specific case, the flap can be
anchored to the periosteum along the pyriform. On the flap side, the depth
of the suture is dependent on how much of a groove is desired. Placing the

suture higher (eg, grabbing part of the dermis) will create a deeper groove.
The flap may also be anchored through and through into the nasal cavity at
the level of the alar-cheek junction, using an absorbable suture.
■ Key sites for deep suture fixation of the transposed flap:
■ Lateral orbital rim
■ Medial orbital rim
■ Nose-cheek junction
■ Alar base–cheek junction
■ The remainder of the flap is inset with deep dermal sutures.
■ 5-0 clear nylon is used in areas with sufficient flap thickness.
■ 5-0 Monocryl (Ethicon, Inc., Somerville, NJ) is used in areas of thinner
overlying tissue.
■ It is of utmost importance to include the expander capsule along the entire
border of the flap in the closure of the deep layer and in the placement of
dermal sutures.
■ This ensures that the capsule is “unfolded,” maximizing the venous return.
Folds or wrinkles in the capsule due to incomplete or incorrect inset can
significantly affect the viability of the flap.
■ The flap may often appear congested until the capsule is fully unfolded.
■ The final excision and inset along the nasolabial groove and melomental fold
are not carried out until the above flap inset is near complete in order to avoid
traction on the upper lip and commissure where final nevus excision will be
required, leaving the lip intentionally “long” and the commissure pushed
medially. This allows final excision to achieve symmetry.
■ With scar contraction, there is a tendency for the ipsilateral nostril to be
pulled downward and the lip to be pulled upward.
■ The inset of the flap is designed to compensate for this, with the nostril
slightly higher and the lip lower compared with the contralateral side
(TECH FIG 3).

TECH FIG 3 • Flap inset is designed to compensate
for subsequent scar contraction, with the ipsilateral
nostril slightly higher and the lip lower than the
contralateral side.
■ Closure
■ A single butterfly drain is placed through a separate stab incision and
secured as before.
■ Skin closure is with running 6-0 chromic and 7-0 Vicryl (Ethicon, Inc.,
Somerville, NJ) sutures (TECH FIG 4).
TECH FIG 4 • The completed reconstruction. A,B. On-

table. C. Mature postoperative result, prior to excision
of the small areas of residual nevus.
PEARLS AND PITFALLS
Technique
■ Transposition of the flap should be completed before completely
excising the lesion, ensuring adequate flap coverage.
■ The lower cheek/neck expanded flap can reach as far as the root of
the nose or upper lip when needed.
■ Deep suture fixation at several points is key to preventing
downward migration of the flap and resultant ectropion.
■ Through-and-through sutures at the alar base–cheek junction can
help recreate the natural groove in this area.
■ The thin skin of the lower eyelid cannot be satisfactorily
reconstructed with a cheek flap and is best addressed with full-
thickness skin graft at a subsequent procedure.
Postoperative
■ Postoperative care is essentially the same as for the first stage.
POSTOPERATIVE CARE
■ The face is cleaned and dressed with bacitracin, Vaseline gauze, gauze fluffs,
and a loose SurgiNet dressing.
■ Ophthalmic bacitracin should be used around the eye.
■ Patients may go home on the day of surgery, depending on parent comfort.
■ Postoperative regimen is unchanged from the first stage.
OUTCOMES
■ In the authors’ hands, expanded flap reconstruction with a laterally based
lower face/neck flap provides the most pleasing results for this type of lesion.
■ The quality and thickness of the expanded flap match that of the uninvolved
skin.
■ The contour of the lower cheek and neck is superior to that achieved with a
medially based flap.
■ Deep suture fixation is effective in preventing later ectropion with vertical
descent of the flap tissue.

■ For nevi involving the lower eyelid, a further stage is generally required.
■ This allows time for the flap to settle into its final position and any small
adjustments to be made.
• Medial or lateral canthopexy can be utilized to address any scar contraction
or minor flap descent.
• If further deep fixation is required at the lateral orbital rim, a permanent
suture can be placed through a drill hole in the bone of the orbital rim.
■ The eyelid nevus is then excised and reconstructed with a full-thickness skin
graft.
■ When excision of nevus involves expanded flap reconstruction of the forehead
and scalp as well as neck, the reconstruction should be sequenced to address
the scalp, forehead, and cheek; the cheek should be reconstructed last.
■ The cheek excision and reconstruction can commence once the forehead flap
is inset in final position. Skin closure may be performed at the same time.
COMPLICATIONS
■ Infection during expansion can occur and is more common in the neck
compared with other sites in the head and neck.
■ Infection can usually be managed with antibiotics, occasionally for the
remainder of the expansion process.
■ Severe infection requiring removal of the expander is rare.10
■ Extrusion of the expander is rare.
■ Maintaining a flap of sufficient thickness minimizes this risk.
■ In older children where a 250-cc expander may be used, the most important
part of expander placement is ensuring that the dissected pocket is large
enough for the expander to be placed with minimal folds or irregular points.
■ The main complication of the second stage is vascular compromise of the
expanded flap.
■ The increased vascularity afforded by expansion lowers this risk.
■ Flap compromise is always partial; total flap compromise does not occur.
■ Partial flap necrosis is generally limited to the distal aspect of the flap.
■ Sloughing is most often partial thickness and manageable with local wound
care.
■ Infection is very rare following removal of the expander.
■ With placement of scars along the junctions of aesthetic subunits, the mature
scar is very good in most cases. However, problems with hypertrophic or

widened scars can occur and may require scar revision. This can often be
combined with other planned procedures (eg, excision of satellite nevi).
■ When there is some extension of the nevus medial and inferior to the
nasolabial fold, leaving that final excision until the scars have settled allows
more precise final closure and may avoid the need for later scar revision.
REFERENCES
1. Cramer SF. The melanocytic differentiation pathway in congenital
melanocytic nevi: theoretical considerations. Pediatr Pathol. 1988;8:253-265.
2. Roh MR, Eliades P, Gupta S, Tsao H. Genetics of melanocytic nevi. Pigment
Cell Melanoma Res. 2015;28(6):661-672.
3. Krengel S, Hauschild A, Schaefer T. Melanoma risk in congenital melanocytic
nevi: a systematic review. Br J Dermatol. 2006;155:1-8.
4. Marghoob AA, Schoenbach SP, Kopf AW, et al. Large congenital melanocytic
nevi and the risk for the development of malignant melanoma. A prospective
study. Arch Dermatol. 1996;132:170-175.
5. Marghoob AA, Dusza S, Oliviera S, Halpern AC. Number of satellite nevi as
a correlate for neurocutaneous melanocytosis in patients with large congenital
melanocytic nevi. Arch Dermatol. 2004;140:171-175.
6. Krengel S, Scope A, Dusza SW, et al. New recommendations for the
categorization of cutaneous features of congenital melanocytic nevi. J Am
Acad Dermatol. 2013;68(3):441-451.
7. Magalon G, Casanova D, Bardot J, Andrac-Meyer L. Early curettage of giant
congenital naevi in children. Br J Dermatol. 1998;138:341-345.
8. Zutt M, Kretschmer L, Emmert S, et al. Multicentric malignant melanoma in a
giant melanocytic congenital nevus 20 years after dermabrasion in adulthood.
Dermatol Surg. 2003;29(1):99-101.
9. Bauer BS, Vicari FA. An approach to excision of congenital giant pigmented
nevi in infancy and early childhood. J Pediatr Surg. 1988;23(6):509-514.
10. Adler N, Dorafshar AH, Bauer BS, et al. Tissue expander infections in
pediatric patients: management and outcomes. Plast Reconstr Surg.
2009;124(2):484-489.

19
CHAPTER
Surgical Treatment of
Congenital Midline Nasal
Mass
Patrick A. Gerety and Scott P. Bartlett
DEFINITION
■ Congenital midline nasal masses are a rare occurrence but require a thorough
workup for an intracranial component, which then necessitates a transcranial
resection.
■ These masses typically represent one of three diagnoses: dermoid cyst,
encephalocele, or glioma.
■ A nasal dermoid sinus cyst (NDSC) is lined by stratified squamous
epithelium and contains dermal adnexal structures such as hair follicles and
sebaceous glands. The cystic contents are sebum. If the mass has a punctum
with drainage to the outside world, it is a dermoid sinus; otherwise, it is a
dermoid cyst.
■ A nasal encephalocele is an extracranial protrusion of a cerebrospinal fluid
(CSF)-containing dural mass. Twenty-five percent of all encephaloceles are in
the nasofrontal region (sincipital). If brain matter is within the mass, this is
referred to as meningoencephalocele.
■ A nasal glioma represents extracranial brain matter with no patent CSF
connection.
■ Intracranial extension—extension of midline nasal masses from extracranial
nasal location to the anterior cranial fossa
■ Transcranial approach—a combined extracranial and intracranial resection
with reconstruction
ANATOMY
■ Formation of a midline nasal mass results from disruption of early

embryologic processes. The exact theory of embryology responsible for the
formation of transcranial nasal masses remains debated.1 Several operating
theories lend rationale to the occurrence and anatomy of these masses. Some of
these theories include the prenasal space theory, the nonseparation theory, and
the ectodermal inclusion theory.
■ Transcranial nasal congenital masses are believed to exist for two important
reasons (FIG 1):
■ Failure of normal regression of a transcranial embryologic structure (dural
diverticulum)
■ Embryologic contact of surface and neural ectoderm
■ Per one theory, a dural diverticulum from the anterior cranial fossa normally
exits the cranium via the nasofrontal junction (the fonticulus (naso) frontalis)
and ends between the nasal bones and nasal soft tissues.
■ This diverticulum normally involutes and can be identified as the foramen
cecum. This foramen has no contents typically but may contain a fibrous
stalk.
■ If this process is disrupted, a persistent transcranial connection may be
present at birth.
■ Importantly, the neural ectoderm of this diverticulum makes contact with the
surface ectoderm of nasal skin. Normally, this connection also regresses. If it
does not, it may pull skin elements beneath the nasal skin resulting in an
NDSC.
■ The transcranial connection may occur at a number of anterior cranial fossa
midline structures: cribriform plate, crista galli, foramen cecum, nasofrontal
junction, or any combination of these.
■ Anterior cranial encephaloceles can also occur via the nasofrontal,
nasoethmoidal, or naso-orbital bony junctions.
PATHOGENESIS
■ The etiologic basis of this occurrence is likely multifactorial with a genetic
component. Family history and multiple siblings have been identified with
these masses. Midline nasal masses are not related to any known syndromes or
other known congenital anomalies.
■ Conceptually and for disease categorization, these masses particularly the
encephalocele are related to the embryologic failure of anterior neuropore
(normally embryologic day 24) during early embryologic development.

FIG 1 • Embryologic origin of congenital frontonasal
masses. Early embryologic connection between the
nasal space and the intracranial space may lead to the
presence of the congenital nasal mass. The existence
of NDSC may be related to neural and surface
ectoderm, which as the diverticulum regresses may
draw skin contents into a subcutaneous position and in
some case maintain a transcranial connection.
■ A cranial defect must be present for either of the three main masses to be seen
nasally and transmitted transcranially.
■ Dermoid cysts likely result from trapping of skin structures subcutaneously as
the dural diverticulum involutes normally.
■ Encephalocele represents a neural tube defect. Terminology varies depending

upon what the extracranial mass contains.
■ Meningocele—meninges only
■ Meningoencephalocele—meninges and brain matter
■ Encephalocele and glioma represent neuroectodermal malformations.
■ Dermoid cysts and sinuses represent a somatic (surface) ectodermal
malformation.
■ Intranasal gliomas and encephaloceles likely have a herniation point in the
anterior cranial fossa that is more posterior (ie, posterior to the cribriform
plate) as compared to masses that are seen on the nasal dorsum.
NATURAL HISTORY
■ Congenital midline nasal masses are rare with an approximate incidence of 1
in 30 000 live births.
■ These masses, particularly encephaloceles, occur in higher rates in Asia
(Western incidence, 1 in 35 000; Asian incidence, 1 in 5000).
■ NDSC—midline nasal cysts represent 3% to 12% of head and neck dermoid
cysts.2
■ Intracranial extension of midline nasal dermoids has been found to be
common among these masses on the order of 25% to 45% of cases.
■ Encephalocele—approximately 80% are occipital and 20% frontonasal
(sincipital).3
■ Glioma
■ Approximately 15% have a transcranial connection; thus, nasal resection
without intracranial exclusion can lead to CSF leak and meningitis.
■ 3:1 male predominance4
■ Sixty percent are extranasal, 30% are intranasal, and 10% are mixed.
■ There is a reported association with other nonspecific congenital abnormalities
(often quoted as approximately 40%). However, several series have not shown
the same association. A transcranial midline nasal mass may occur in isolation.
■ Risk of meningitis—with good soft tissue coverage and no sinus punctum,
patients are likely at low risk of developing a meningeal infection even in the
presence of intracranial CSF connection.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Visible and palpable midline nasal mass (FIG 2)
■ May occur from the columella to the radix but most commonly along the nasal

dorsum
■ Wide nasal bridge
■ Hypertelorism—this may be true hypertelorism with total orbital widening or
more commonly in this case interorbital hypertelorism with widening of only
the medial orbital wall.
■ May be present in 70% of encephalocele patients
■ Normal interorbital distance increases from approximately 18 mm in infancy
to 25 to 30 mm in adulthood.
FIG 2 • Physical appearance of congenital midline nasal
mass. A. Dermoid sinus with pathognomonic hairs from
punctum. NDSC may also present as subcutaneous
firm mass with no punctum. B. Nasal glioma with
pathognomonic telangiectasia. This may be
indistinguishable from a nasal dermoid without a dermal
sinus. C. Nasal encephalocele with bluish hue, large
size, hypertelorism, and positive fluid wave on exam.
■ NDSC are firm, round masses.
■ NDSC may present with a punctum. The punctum may contain hair, which is
a pathognomonic sign of the diagnosis.
■ If a punctum is present, patients are more likely to present with infection.
■ Gliomas are firm, round masses.
■ Surface telangiectasia is present and this is pathognomonic.
■ May be extranasal, intranasal, or both
■ Does not transilluminate or change in size
■ Intranasal glioma may cause nasal airway obstruction or nasolacrimal
dysfunction. Airway obstruction is particularly problematic in
neonates/infants who are obligate nasal breathers.
■ Encephalocele may occur through one of three sutures (junctions): nasofrontal,
nasoethmoidal, and naso-orbital.

■ Often appear blue in color
■ Transillumination is pathognomonic because of CSF content.
■ Size change with crying may occur (Valsalva).
■ Size change may also be elicited by compression of the internal jugular veins
(Furstenberg sign).
■ Patients may present with CSF leak or frank meningitis.
■ The cranial exit point has been found to be fairly variable and may occur at the
nasofrontal suture but also through other cranial bone junction points.
■ This can be difficult to differentiate on physical examination.
IMAGING
■ Because of the occurrence of transcranial extension, there should be a low
threshold for obtaining cross-sectional imaging of congenital midline nasal
masses.
■ In general, both a maxillofacial CT scan and brain MRI are obtained.
■ CT scan provides a better examination of bony distortion and bony
relationships.
■ MRI is thought to be more sensitive and specific for transcranial soft tissue
extension and for brain structural examination.5
■ Typical imaging findings (FIG 3):
■ Interorbital distance—widened
■ Frontonasal cranial defect
■ Nasal bone—displaced and angulated
■ Bifid crista galli
■ Tissue diagnosis via biopsy is contraindicated in these masses before a
noninvasive, cross-sectional imaging workup has been performed.

FIG 3 • A,B. MRIs reveal extra and intra components
(arrows) in a 1-year-old girl with glabellar mass. C,D.
Midline encephalocele is apparent in AP and lateral CT
scans in a 4-month-old girl.
DIFFERENTIAL DIAGNOSIS
■ The following are other diagnoses outside of NDSC, encephalocele, and
glioma, which may occur on the nasal dorsum congenitally.6
■ Hemangioma/vascular malformation
■ Teratoma is pathologically distinct and contains all three layers, including

endoderm, which is not present in NDSC.
■ Lipoma
■ Neurofibroma is often seen in concert with other neurofibromatosis
cutaneous findings.
SURGICAL MANAGEMENT
Preoperative Planning
■ Multidisciplinary collaboration
■ Plastic surgery
■ Neurosurgery
■ Ophthalmology—preoperative exam (interorbital, intercanthal, and
interpupillary distances as well as eye movements), postoperative
management of strabismus, and nasolacrimal management
■ CT and MRI
■ Hydrocephalus should be managed preoperatively by neurosurgery possibly
with ventriculoperitoneal (VP) shunt before definitive correction (only pertains
to encephalocele).
■ Timing of surgery should be for at least late infancy (8 to 12 months) to allow
better perioperative physiology and maturation of bone, which aids
osteotomies and fixation.
■ Transfusion and postoperative critical care are required.
■ Computer-based 3D surgical planning may play a role in hypertelorism
correction in the future.
Positioning
■ Resection and reconstruction are done in a supine position.
Approach
■ FIG 4 is a decision-making algorithm for resection choice.
■ Extracranial approach
■ Transnasal excision
■ Reduction of excess frontonasal skin
■ Open rhinoplasty—has been demonstrated when skin excess is minimal and
there is no punctum. This can avoid dorsal nasal scar.
■ Intranasal—excision may be done through nares or open rhinoplasty
approach if the mass is intranasal (more often with glioma).

■ Intracranial
■ Frontal craniotomy
■ Limited craniotomy
■ Endoscopic minimally invasive approach
■ Immediate interorbital hypertelorism correction
■ Dual approach
■ Extracranial
■ Intracranial
■ Aims
■ Divide nasal and meningeal tissue and seal them apart.
■ Correct nasal soft tissue excess as needed.
■ Repair cranial base dural and bony defects.
■ Move medial orbits/medial canthi into normoteloric position if necessary.
FIG 4 • Algorithm for choice of resection technique.
T E C H N I Q U E S
■ Resection of Transcranial NDSC (“The Keystone
Technique”)
■ Havlik et al. are responsible for describing and popularizing this technique.7

Nasal Approach/Frozen Biopsy
■ In the case of dermoids with uncertain transcranial component, the mass is
dissected down to its stalk. The stalk is biopsied for frozen section.8
■ If it does not contain dermal contents, then the procedure is terminated with
extracranial excision only.
■ If dermal elements are present, an intracranial approach is followed (TECH
FIG 1).
TECH FIG 1 • External approach to midline nasal
mass. This approach is combined with an intracranial
approach when imaging, or intraoperative frozen
biopsy confirms intracranial extension. A. Elliptical
design for excising this nasal NDSC. B. The cyst and
its stalk have been dissected down to the nasal
bones, and a biopsy can be done at the deepest point.
Incisions, Dissection, and Craniotomy
■ Bicoronal incision is designed at a symmetric point in the hairline just above
the helical root. This is designed as a zigzag pattern to decrease visibility.
■ Dorsonasal—a conservative elliptical skin excision is designed for nasal skin
reduction and as access for dissection of the mass.
■ An anterior scalp flap is raised in the areolar subgaleal plane (TECH FIG

2A).
■ The pericranium should be carefully preserved.
■ Upon approaching the orbits and nasofrontal junction, a plan for raising a
subpericranial flap and subperiosteal dissection should be formulated.
■ A large pericranial flap should be marked and raised to keep it in continuity
with the scalp. This also allows full subperiosteal dissection of the orbits.
■ The medial orbits and frontonasal defect edges are dissected subperiosteally
with an elevator.
■ A bifrontal craniotomy approximately 1cm above the supraorbital bandeau is
performed by neurosurgery (TECH FIG 2B).
■ The frontal lobes can be dissected and retracted to reveal the cranial base and
the intracranial/dural extension of the mass (TECH FIG 2C).
Keystone Osteotomy and Dermal Sinus Excision
■ To visualize the dermoid sinus tract, the frontonasal junction is osteotomized
and removed (TECH FIG 3).
■ This is done via vertical parasagittal osteotomies.
■ This can be done to take only midline frontal bone or to include the nasal
bones.
■ Both approaches require reconstruction of the frontonasal junction or of the
keystone area depending upon what is osteotomized.

TECH FIG 2 • Transcranial approach to nasal
dermoid. A. Design and reflection of coronal scalp
flap. B. Design of frontal craniotomy and limited
bandeau (so-called keystone approach—frontonasal
osteotomy). C. Keystone osteotomy of frontonasal
junction in order to access the transcranial component
of the mass.
■ The dermal sinus tract is resected from its dural connection following
visualization.
■ The NDSC, glioma, or redundant meningeal tissue is excised.
■ At the site of the mass that is resected, a careful dural repair is performed by
neurosurgery. Dural substitute is used as needed.
Bandeau/Frontal Bone Repair
■ The keystone portion of the frontal bone is fixated back in place with
resorbable hardware in infants and rigid hardware in older children.

TECH FIG 3 • View of the transcranial component of
the mass after frontal craniotomy.
■ Fixation of the frontal bone should not compress or strangle the pericranial
flap.
■ Sizeable full-thickness cranial defects such as large bur holes can be filled
with cranial bone shavings/particulate.
■ A subcutaneous, closed suction drain is placed and the scalp is closed.
Cranial Base Repair and Seal
■ If a bony defect is present in the cranial base, it should be treated with full or
split calvarial bone graft (TECH FIG 4A). This bone is fashioned to the size
and shape of the defect but not typically fixated.
■ The pericranial flap harvested during scalp dissection (TECH FIG 4B) is
then placed over the graft covering the cranial base.
■ If possible, sutures are used to secure the flap. Fibrin glue is used to seal the
edges of the flap (TECH FIG 4C).
■ The pericranial flap acts to provide vascularized barrier both for the dural
repair and the cranial base bone defect.
Completion
■ The frontal sinus is not an issue in infants as the sinuses have not yet

developed.
■ In patients with delayed presentation and treatment, the frontal sinuses
should be cranialized and the frontonasal duct should be obliterated with
cautery and bone graft.
■ The nasal skin redundancy is evaluated and additional skin is removed if
necessary. The nasal skin is closed.
TECH FIG 4 • Seal of the anterior cranial base after
transcranial resection. A. Bone graft (either full- or
split-thickness calvarium) is placed in the cranial base
defect. B. A pericranial flap is planned preoperatively
for coronal scalp flap dissection. C. The pericranial
flap is placed over the bone graft, and fibrin tissue
sealant is used along with sutures to ensure a
watertight seal.
■ Medial Orbit Composite Unit Translocation

(MOCUT) Procedure
■ This technique has been described and popularized by Boonvisut et al. for
immediate hypertelorism repair in nasal encephalocele.9
Approach and Dissection
■ The same coronal incision and bifrontal craniotomy as described for
Resection of Transcranial NDSC above is utilized.
■ The nose can be opened via either a vertical incision on the dorsum or
through a conservatively designed vertical ellipse.
■ Subperiosteal dissection is performed at the supraorbital bandeau into the
orbits.
■ Orbital dissection is limited to the medial aspects of each orbit between the
supraorbital notches. Care is taken to keep the medial canthal tendon
insertion intact.
■ Careful subperiosteal dissection is used to ensure that the orbital and soft
tissues are moved in concert in an uninterrupted fashion.
■ The subperiosteal dissection is carried medially to the bone edges at the
frontonasal defect.
Osteotomies, Resection, and Reconstruction
■ Medial orbital wall is cut vertically from the craniotomy at the level of the
supraorbital notch down to the inferior orbital rim (TECH FIG 5A).
■ Via the nasal incision, a transverse osteotomy is performed from the piriform
aperture to the inferior orbital rim to free the composite medial orbital unit.
■ In the midline, bone will need to be removed from the medial orbital unit to
establish the final interorbital distance.
■ This distance is planned preoperatively using a CT scan.
■ Osteotomies are performed with a combination of conventional osteotomy,
reciprocating saw, and ultrasonic scalpel.

TECH FIG 5 • Encephalocele resection with
transcranial approach and immediate hypertelorism
correction. A. Bifrontal craniotomy is performed.
Laterally vertical osteotomies are planned to descend
from just medial to the infraorbital notch. A variable
bony resection at the midline will be performed to
allow medialization of the orbit and canthal units. The
inferior horizontal osteotomy may be completed
through the nasal incision. B. The medial orbit
composite unit technique (MOCUT) can be seen here
to immediately produce hypertelorism correction.
■ Completion of the osteotomies mobilizes the orbital units allowing for
improved exposure.
■ The mass, typically an encephalocele with this approach, should be dissected
free from its surroundings.
■ This is done via the nasal incision and the frontal craniotomy.
■ Dural repair and cranial base/frontal sinus repair are as described above.
■ The medial orbital units are joined at the midline with fixation (TECH FIG
5B).
■ This is typically done with a 30-gauge wire but may also be done using
suture, permanent plates, or resorbable plates and screws.
■ Transverse bony gaps at the supraorbital bandeau that have been created
through orbital medialization should be treated with fixated interposition
calvarial bone graft.

■ Any disruption of the nasal cartilages to bone or of the nasofrontal junction
should be repaired with suture.
■ Redundant nasal skin should be conservatively resected. The nasal skin is
closed.
■ Some have argued for more complex nasal skin flaps. Our preference is for
nasal skin revision at a second stage if necessary.
■ Endoscopic Excision of Intranasal Glioma
■ The approach to intranasal gliomas also includes an external rhinoplasty
approach and a lateral rhinotomy approach.10 These specific approaches will
not be discussed here.
■ In general, this approach has been used by those who practice endoscopic
surgery and skull base approaches.
■ Many have argued that with evidence of transcranial extension, a
traditional craniotomy should be performed to separate the nasal-
intracranial connection. Others have demonstrated that intranasal glioma
with intracranial extension can be successfully excised endoscopically,
thereby avoiding craniotomy.
■ An angled endoscope is introduced intranasally to visualize the mass (TECH
FIG 6).
■ Electrocautery is used to incise the nasal mucosa along the mass.
■ Retraction and blunt dissection define the mass.
■ After defining the stalk, it is divided using electrocautery and the mass is
removed.
■ The base is examined for CSF leak. If necessary, it may be oversewn. Fibrin
tissue sealant may also be used to ensure a seal.
■ The nasal mucosa is closed with simple sutures.
■ The affected nasal passage is packed. Nasal packing is removed in the early
postoperative period.

TECH FIG 6 • Excision of intranasal mass. A.
Endoscopic appearance of purely intranasal glioma.
B. An endoscope may be used to visualize and aid
excision of intranasal glioma. Endoscopic approaches
to the cranial base are also well described.
PEARLS AND PITFALLS
Biopsy
■ Congenital nasal masses, particularly those occurring at or near the
midline, should not be biopsied for diagnosis. Biopsy can lead to CSF
leak and ascending/meningeal infection.
Radiologic
evaluation
■ Congenital nasal masses should be evaluated radiographically. This
often includes both a CT scan and MRI.
VP shunt
■ Decompression by neurosurgery in the setting of a large or growing
encephalocele can allow surgery to be delayed until the patient is a
better candidate for surgery.
Age at
surgery
■ Timing of treatment, particularly with large lesions and
encephaloceles, is a balance between the risk of

infection/meningitis/family stress and the safety of surgery/quality of
bone for reconstruction. We recommend waiting until late infancy if
there are no other time-sensitive factors.
Intracranial
seal
■ Transcranial approach requires careful reconstruction of the cranial
base defect including bone grafting, pericranial flap, and fibrin glue
seal.
POSTOPERATIVE CARE
■ Pediatric ICU is mandatory with expected hospital stay of 5 to 7 days.
■ Neurologic monitoring
■ Routine labs for bleeding and infection
■ Monitoring of nasal and drain output for CSF leak—the drain should be in
place for at least 5 days.
■ Close outpatient monitoring for the development of CSF leak, meningitis, and
hydrocephalus
■ Secondary nasal scar revision is often necessary.
OUTCOMES
■ Complications
■ In these rare conditions, most case series are small.
■ Minor complications in any transcranial approach with nasal-intracranial
communication should be expected to be moderate to high.
■ Catastrophic complications should be rare.
■ Aesthetic outcome
■ Disruption of nasal growth and form is a high risk.
■ Revision of nasal scar/form and nasal reconstruction with rib graft should be
expected.
■ Second hypertelorism correction may be necessary.
COMPLICATIONS
■ CSF leak
■ Meningitis
■ Death
■ Scar hypertrophy
■ Residual hypertelorism
■ Residual nasal deformity and skin excess
■ May require re-excision, local tissue rearrangement, onlay grafting of the

nasal dorsum, and formal rhinoplasty at maturity
REFERENCES
1. Charrier J-B, Rouillon I, Roger G, et al. Craniofacial dermoids: an
embryological theory unifying nasal dermoid sinus cysts. Cleft Palate
Craniofac J. 2005;42:51-57.
2. Blake WE, Chow CW, Holmes AD, Meara JG. Nasal dermoid sinus cysts: a
retrospective review and discussion of investigation and management. Ann
Plast Surg. 2006;57:535-540.
3. Tirumandas M, Sharma A, Gbenimacho I, et al. Nasal encephaloceles: a
review of etiology, pathophysiology, clinical presentations, diagnosis,
treatment, and complications. Child’s Nerv Syst. 2013;29(5): 739-744.
4. Rahbar R, Resto VA, Robson CD, et al. Nasal glioma and encephalocele:
diagnosis and management. Laryngoscope. 2003;113:2069-2077.
5. Hedlund G. Congenital frontonasal masses: developmental anatomy,
malformations, and MR imaging. Pediatr Radiol. 2006;36:647-662.
6. Bartlett SP, Lin KY, Grossman R, Katowitz J. The surgical management of
orbitofacial dermoids in the pediatric patient. Plast Reconstr Surg.
1993;91:1208-1215.
7. Van Aalst JA, Luerssen TG, Whitehead WE, Havlik RJ. ‘Keystone’ approach
for intracranial nasofrontal dermoid sinuses. Plast Reconstr Surg. 2005;116:13-
19.
8. Sessions RB. Nasal dermal sinuses—new concepts and explanations. The
Laryngoscope. 1982;92(8 Pt 2 Suppl 29):1-28.
9. Boonvisut S, Ladpli S, Sujatanond M, et al. A new technique for the repair
and reconstruction of frontoethmoidal encephalomeningoceles by medial
orbital composite-unit translocation. Br J Plast Surg. 2001;54:93-101.
10. Wu C-L, Tsao L-Y, Yang AD, Chen M-K. Endoscopic surgery for nasal
glioma mimicking encephalocele in infancy. Skull Base. 2008;18:401-404.

20
CHAPTER
Section VI: Soft Tissue
Masses
Surgery of
Neurofibromatosis in the
Pediatric Patient
McKay McKinnon
DEFINITION
■ Patients with cutaneous neurofibromatosis (NF), the most common of the
myriad of phenotypic manifestations of the disease (and Von Recklinghausen’s
original case reports), can usually expect permanent relief of individual lesions
by full-thickness excision. For those patients with so-called plexiform NF
and/or malignant peripheral nerve sheath tumors (MPNST), the puzzlement of
a permanent treatment (medical or surgical) has remained largely unsolved for
most patients.
■ For well over a century, the textbook recommendation for children with NF1
plexiform tumors has been to defer surgery until after puberty or until serious
morbidity is evident. When surgery has been deemed necessary, it has most
often been a superficial resection of the tumor, commonly referred to as
“debulking.” Subsequent recurrence of tumor has led to a default strategy of
withholding surgery for pediatric patients, based upon fear of iatrogenic injury
to normal structures, uncontrollable hemorrhage, and perceived inability to
achieve permanent tumor removal.
■ Over the past 30 years, the author has observed that radical resection of
plexiform tumors in adults has resulted in permanent “nonrecurrence” of nearly
all tumors, even those over 50 kg and some MPNST.1 Despite a higher

propensity for active tumor growth in children and adolescents, it seemed
logical to extend the concept of radical surgical resection to pediatric patients.
ANATOMY
■ Craniofacial
■ Most facial and frontotemporal scalp tumors derive from sensory branches of
cranial nerve V. The ophthalmic division of the trigeminal nerve (FIG 1A)
includes some sensory nerves that are within the musculofascial cone
(annulus of Zinn) of the orbit. This area presents special risks of injury to the
optic nerve and the extraocular muscles and should be avoided.
• Orbital NF is often accompanied by a defect of the sphenoid greater wing,
which may permit herniation of the temporal lobe into the orbit and
subsequent orbital dystopia, pulsatile exophthalmos or enophthalmos, and
pressure on the globe and optic nerve. Destruction of bone, ligaments,
fascia, muscle, and skin may be present.
• Tumor confined to the optic nerve (optic glioma) deserves neurosurgical
management.
• Motor nerves are not intrinsically involved with NF tumor.
■ NF tumors of the mandible, parotid, ear, and temporal scalp develop largely
from the mandibular division of the trigeminal nerve, including the
auriculotemporal nerve.
■ Tumors of the neck and posterior scalp derive from upper cervical sensory
nerves (C1–C4), including the occipital nerves. Lower cervical nerves (C4–
C8, sensory and motor) constitute most of the brachial plexus.
■ Trunk and extremity
■ Plexiform NF can exist anywhere from the spinal cord to the sensory nerve
terminus in the skin, bone, or viscera (FIG 1B).
■ Plexiform tumors most commonly appear between the deep fascia and the
skin but may involve and destroy skin, muscles, bones, joints, and visceral
organs. Careful dissection proximal to the tumor mass (toward their CNS
origin) often reveals the specific sensory nerve origin. Large truncal lesions
over time can develop paraspinal hypervascularity, which resembles an
arteriovenous malformation. Scoliosis is common with posterior trunk
lesions, and limb hyperplasia is common with extremity lesions.
PATHOGENESIS
■ NF is an autosomal dominant condition, which can be attributed to a defect on

chromosome 17, but mosaicism can also occur. The consequential lack of
neurofibromin (a tumor inhibitor) permits growth of myelinated axonal tumors
in any bodily location. Tumor types are cutaneous or plexiform. So-called
plexiform tumors are solid tumors, which have an obligatory angiogenic
influence, similar to other solid tumors. They express multiple angiogenic and
neurogenic factors. Puberty, pregnancy, and childhood growth spurts can
exacerbate their growth. Tumor vascularity increases proportionate to tumor
growth. Transformation of benign NF tumors into MPNST can occur in
plexiform tumors. This transformation may have a direct correlation to
numerical tumor cell replication events.
■ Tumor vessels in NF are pathologic arteries that reveal absent media and
abnormally thin adventitia. Established tumors can exhibit vascular lakes
and/or nests of abnormal vessels resembling a congenital vascular
malformation.
NATURAL HISTORY
■ Neurofibromas, once established as plexiform tumors, do not regress
spontaneously. Their growth is unpredictable and nonregular, including
possible onset throughout a patient’s life of new lesions. Malignant
transformation is more common in truncal/extremity lesions but is also
unpredictable. Café au lait cutaneous spots do not necessarily signal plexiform
tumors, although they themselves represent microscopic tumor. If present at
birth, plexiform tumors may have already inflicted defective tissue
development of the newborn.

FIG 1 • Nerves of the orbit.
■ Eventual destruction by tumor of adjacent skin, bone, and other tissue should
be expected without intervention. Hyperplasia and/or fragility of bone occurs
in extremity lesions. Craniofacial tumors can also produce osteoclastic or
osteoblastic pathology, likely as a consequence of tumor growth rate. Tumors
involving the sphenoid and orbit can cause progressive orbital dystopia,
blepharoptosis, and blindness. Paraspinal tumors inflict progressive scoliosis
on the growing patient.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ The myriad presentations of NF tumors should not deter accurate diagnosis,
even in infancy, by the observant neurologist or experienced surgeon. If doubt
of the diagnosis exists, a biopsy should be performed with exception at least of
the optic nerve.
■ Physical findings are too numerous to justify an inclusive list, but palpable
tumor, pain, and confirmatory imaging should precede surgical intervention in
virtually all cases.

IMAGING
■ Pediatric patients with suspicion of plexiform NF tumor should have an MRI
study, preferably with and without contrast. MRI of the CNS is appropriate as
an early screening measure for all NF patients, even those without known
tumors. T2- and STIR-weighted images usually give the clearest depiction of
NF tumor.
■ MRI, especially with higher Tesla power, can frequently reveal the specific
cranial or spinal nerve involved with tumor. This information (nerve mapping)
should be sought out with the neuroradiologist.
■ High-resolution CT scans should only be ordered when and if the patient with
skeletal destruction or high risk of skeletal pathology is being evaluated
preoperatively.
DIFFERENTIAL DIAGNOSIS
■ NF tumors have been mistaken for vascular malformations, particularly by
their hypervascular imaging on CT or MRI.
■ Hyperpigmentation of NF lesions has been confused by the inexperienced
physician with congenital nevus. A biopsy can readily produce the correct
diagnosis.
■ Most patients, even infants, with NF will present with at least three or more
cutaneous lesions and/or café au lait lesions, axillary freckling, ocular Lisch
nodules, or a subcutaneous plexiform mass.
■ Congenital ptosis can usually be differentiated from NF-derived ptosis by
detection of an orbital mass, presence of enophthalmos or exophthalmos, and
by MRI findings. Rapid increase of ptosis may be associated with orbital
malignancy such as rhabdomyosarcoma, for which immediate incisional or
excisional biopsy is required.
■ Diagnosis of MPNST or other orbital malignancies cannot be reliably made by
symptoms, by imaging, or by clinical examination.
NONOPERATIVE MANAGEMENT
■ This default management decision is historically common but deserves
renewed challenge because it is controversial. Observation of growing NF
tumors is patently nontherapeutic yet may be justified if surgery is high risk,
the patient has significant comorbidity, or the tumor has not reached a level of
irreversible morbidity to vital structures. The surgeon and his or her medical
colleagues should be prepared to intervene to prevent irreversible morbidity,

even in young patients.
SURGICAL MANAGEMENT
■ An accurate diagnosis is the sine qua non of surgical management. With
plexiform NF this should include determination of the tumor nerve origin(s),
the degree of vascularity, bony deformity, proximity to other vital anatomy, and
degree of skin destruction. In the craniofacial tumors, accurate diagnosis also
should determine presence of brain herniation into the orbit (or elsewhere),
orbital dystopia, ptosis vs pseudoptosis, ophthalmic pathology, and airway and
carotid/jugular risks.
■ Because all NF1 tumors are caused by pathologic sensory nerves, either of
brain or spinal cord origin,1 if a total interruption of the responsible sensory
nerve(s) can be achieved (eg, a Sunderland V injury), permanent nonregrowth
of tumor may also be achieved.1 Thus, surgical strategy should aim to:
■ Radically resect the plexiform tumor and identify and amputate the
responsible sensory nerve(s) proximal to the tumor
■ Dissect and preserve any motor nerves in the tumor proximity
■ Undertake tumor resection even in the pediatric patient because progressive
tumor growth over time equates to more complex surgery, higher risk of
morbidity to normal adjacent structures and function, and possibly more risk
of malignant transformation of previously benign tumors
■ All techniques are subservient to the principles of reconstructive surgery. A
knowledge of and adherence to principles permit the surgeon to methodically
approach virtually any surgical problem to arrive at an organized plan. The
reconstructive surgeon’s “creed” has been summarized by Millard: “Know the
ideal beautiful normal. Diagnose what is present, what is diseased, displaced,
or distorted, and what is in excess. Then, guided by the normal in your mind’s
eye, utilize what you have to make what you want—and when possible go for
even better than what would have been!”
■ No clinical condition presents to the surgeon a more variable array of
pathology than neurofibromatosis. The NF surgeon needs, therefore, to first
make a strategy from application of principles. When the principles (such as
making an accurate diagnosis, establishing priorities, making a plan and a
backup plan, replacing lost tissue in kind, etc.) are applied, the surgical
techniques will devolve naturally. The surgeon, much like his or her military
counterpart, elucidates the goals and strategy of a military-type campaign
before determining the specific technique(s) required.

Preoperative Planning
■ Pertinent consultations with other specialists should be completed well prior to
surgery, if feasible.
■ Adequate warning to blood bank and ICU is made.
Positioning
■ Most craniofacial procedures are done in the supine or lateral position with a
standard OR table with a gel headrest.
■ Trunk and extremity procedures are done in standard positions according to
tumor site(s).
Approach
■ Orbital tumors are approached via coronal, superior lid crease, and
transconjunctival incisions as needed. Inferior orbital nerve (maxillary) tumors
are accessed via a lower eyelid incision as in orbital floor exploration or via a
nasojugal incision.
■ The surgeon should anticipate repeat procedures (ie, a subciliary incision is
always a higher risk for ectropion).
■ Posterior trunk tumors should be accessed via incisions that can resect not
only the palpable tumor but also any feeding paraspinal sensory nerves (FIG
2).

FIG 2 • Spinal sensory nerves of the trunk.
T E C H N I Q U E S
■ General Principles
■ The first priority of surgery in the child with NF is resection of tumor and
reconstruction of bone. Large tumor resections can be aided by cautery
devices such as LigaSure, but the surgeon should be facile in vascular
surgical techniques.

■ Autogenous bone reconstruction is superior in virtually all cases to
alloplastic reconstruction in NF, especially in the growing child.
■ Because resection of cranial nerve V tumors into the cavernous sinus is
dangerous (and some tumor will necessarily remain there), reconstruction of
the sphenoid defect should be undertaken with a combination of bone and
plate/mesh to ensure permanence of brain-orbit separation (even in the
enucleated patient). Bone should face the orbit to allow possible attachment
of the levator muscle origin.
■ Early ptosis correction is important to avoid amblyopia, but definitive ptosis
correction should be performed when the child can tolerate a nongeneral
anesthetic for refinement of the correction by patient participation and tumor
is not significantly present.
■ When approaching a large plexiform tumor, the surgeon should begin
dissection in normal tissue and proceed to identify the plane at the tumor
margin. Motor nerves that are within the tumor field should be identified by
direct nerve stimulation.
■ Illustrative Cases
Case 1: Inferior Orbital Nerve Tumor
■ A 4-year-old girl demonstrated a rapid regrowth of a right inferior orbital
nerve NF tumor 6 months following partial resection at an outside institution.
The tumor was creating a vertical orbital dystopia (TECH FIG 1A,B).
■ A resection of the inferior orbital nerve tumor from the cheek to the posterior
orbit was performed via the old nasojugal vertical scar (TECH FIG 1C,D).
■ Alternative approaches in an unoperated patient would be a transverse
lower lid incision and a separate nasolabial fold incision, or even an
intraoral incision, depending on extent of tumor.
■ Following resection, the marginotomy was repaired with a stainless steel
wire in a corrected (inferior) reposition.
■ Over the subsequent 20 years, the patient presented multiple other tumors of
alternate branches of the trigeminal nerve, but not of the inferior orbital
(maxillary) nerve (TECH FIG 1E,F).
Case 2: Hypogastric Plexiform Tumor
■ A teenage girl presented a painful left hypogastric plexiform tumor

recurrence 2 years after an attempted resection at an outside hospital (TECH
FIG 2A).
■ MRI revealed the tumor extending from the skin to the deep abdominal
fascia (TECH FIG 2B).
■ Resection via an extension of the old scar included dissection and resection
of the pathologic hypogastric nerve lying deep to the external oblique muscle
(TECH FIG 2C–E).
■ There had been no recurrence in 10 years of follow-up.
TECH FIG 1 • A,B. A 4-year-old girl with right inferior
orbital nerve NF tumor 6 months after partial
resection. C,D. A marginal osteotomy (a la Tessier) of
the orbital rim allowed visualization and tumor
resection in the posterior orbit to the cranial base. E–
H. Postoperative photos.

TECH FIG 2 • A. Teenaged girl with left hypogastric
plexiform tumor recurrence 2 years after attempted
resection. B. The tumor extended from the skin to the
deep abdominal fascia, as shown on MRI. C,D.
Dissection and resection of the pathologic hypogastric
nerve lying deep to the external oblique muscle. E,F.
The result 1 year after direct wound closure.
Case 3: Breast and Chest Wall Plexiform Neurofibromatosis
■ A 15-year-old girl presented with right breast and lateral chest wall
plexiform NF (TECH FIG 3A).
■ At surgery, a simultaneous resection of a right intercostal tumor and a right
breast reduction/tumor resection were performed (TECH FIG 3B,C).
■ The intercostal nerve proximal to the tumor mass was located and amputated
at the rib.
■ No recurrence had been observed in over 8 years of follow-up (TECH FIG
3D).
Case 4: Left Orbital and Mental Nerve Neurofibromatosis 1

■ A 12-year-old boy presented with left orbital and mental nerve plexiform
NF1 (TECH FIG 4A,B), severe ptosis, and pulsatile exophthalmos.
■ Through a coronal, left upper eyelid, and left nasolabial incisions (TECH
FIG 4C), the tumor was radically resected.
■ Via the coronal approach (without osteotomy), the temporal lobe dura was
located inside the posterior orbit and dissected from the periorbitum (TECH
FIG 4D) and then the brain/dura was repositioned into the cranial cavity and
separated from the orbit by a cranial bone graft/titanium mesh reconstruction.
■ The graft/implant was placed from the orbit into the cranial cavity and held
in position by autoretention.
■ In the same procedure, a cranial bone graft to the orbital floor and a
moderate reduction of eyelid skin excess was performed.
■ Vision was preserved (TECH FIG 4E). The patient will need future
additional floor elevation and definitive ptosis correction.
TECH FIG 3 • A. A 15-year-old girl with right breast
and lateral chest wall plexiform NF. Simultaneous with
resection of a right intercostal tumor (B), a right breast
reduction/tumor resection was performed (C). D.
Appearance at 8 years postoperatively.

TECH FIG 4 • A. A 12-year-old boy with left orbital and
mental nerve plexiform NF1, severe ptosis, and
pulsatile exophthalmos. B. MRI demonstrated a
plexiform tumor of the superior orbit and mental nerve,
a large defect of the sphenoid greater wing, and
prolapse of the temporal lobe into the orbit. C.
Markings for coronal, left upper eyelid, and left
nasolabial incisions. D. Dissection of the temporal
lobe dura inside the posterior orbit from the
periorbitum via the coronal approach. E. Postoperative
appearance.
PEARLS AND PITFALLS
Diagnosis
■ The most common pitfall in NF surgery is inaccurate diagnosis.
■ When recommending a tissue biopsy for definitive diagnosis, avoid

biopsy within a highly vascular region of NF tumor or adjacent to the
optic nerve.
■ Tumor heterogeneity on MRI may indicate a benign tumor adjacent to
a malignant tumor.
Surgical
strategy
■ The NF surgeon should avoid underestimation of tumor extent and
potential hemorrhage, underdissection with nerve stimulation of motor
nerves along the path of the tumor, and underestimation of a surgical
strategy that anticipates tumor recurrence in young patients.
■ Lifetime surveillance by the surgeon should be assumed because the
medical specialists are ill prepared to have a surgical perspective of
NF.
■ The occasional NF surgeon will inevitably experience high morbidity
and low patient satisfaction.
Technique
■ The surgical term “debulking” in NF1 represents a common pitfall of
partial, superficial resection. Rapid recurrence of tumor normally
ensues. Debulking of intraorbital tumors may, however, be justified to
save vision.
■ It should be clear to the NF surgeon that vision is also at risk due to
possible glaucoma, hemorrhage, tumor/brain pressure on the optic
nerve, glioma of the optic nerve, severe orbital dystopia, severe ptosis
(amblyopia), and palpebral incompetence.
Other
specialists
■ Orthopedic surgeons, neurosurgeons, and ophthalmologists frequently
treat pediatric patients with NF.
■ If limb hypertrophy or spinal scoliosis is present in conjunction with a
tumor that is resectable, the plastic NF surgeon should champion
primary tumor resection prior to orthopedic attempts to reduce
epiphyseal growth, reconstruct joints, to correct scoliosis, or even to
amputate parts. This should be intuitive as correct general surgical
principle, not a pearl of wisdom. Likewise, strabismus and/or ptosis
surgery should not precede tumor resection and orbital bone
reconstruction.
POSTOPERATIVE CARE
■ The NF surgeon should use drains freely due to the vascularity and vessel
pathology of NF tumors, following extensive resections, which predispose to
seroma formation.
■ Suction drainage may be hazardous near the dura and may exacerbate
hemorrhage near fragile-walled, residual tumor vessels.
■ Since many NF surgeries overlap into other surgical specialties, the plastic NF

surgeon should include those specialists in the postoperative care but remain
the captain of care and assume ultimate responsibility.
REFERENCE
1. McKinnon M. Why patients with neurofibroma don’t need allotransplantation.
Plast Reconstr Surg. 2012;130:12.

21
CHAPTERVascular Anomalies
Arin K. Greene and Jeremy A. Goss
DEFINITION
■ Vascular anomalies are a group of lesions that can affect any part of the
vasculature.
■ Lesions are divided into tumors (with dividing endothelium) and
malformations (structural anomalies with minimal endothelial turnover).1
■ Eight types of vascular anomalies (four tumors and four malformations)
constitute approximately 95% of lesions.
ANATOMY
■ Vascular anomalies most commonly involve the integument (although they can
affect any anatomical structure).
■ The most common morbidity is lowered self-esteem because of a deformity.
■ Lesions involving the head and neck are more likely to be problematic.
NATURAL HISTORY
■ The somatic mutations responsible for most vascular anomalies recently have
been identified.
■ Some vascular tumors improve over time (infantile hemangioma, rapidly
involuting congenital hemangioma, kaposiform hemangioendothelioma).2
■ Vascular malformations slowly worsen, particularly during adolescence.
■ Following treatment of vascular malformations, recurrence is common and
patients often require repeated procedures.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Ninety percent of vascular anomalies can be diagnosed by history and physical

examination.
■ Before considering management, the type of vascular anomaly must be
identified because lesions have different natural histories and treatments.3
■ The most common vascular anomalies encountered in clinical practice are (in
order of frequency) pyogenic granuloma, infantile hemangioma, and capillary
malformation.
■ A handheld Doppler can determine whether the lesion has fast flow, which
aids the diagnosis.
■ Lesions with fast flow include infantile hemangioma, congenital hemangioma,
kaposiform hemangioendothelioma, and arteriovenous malformation. Slow-
flow anomalies are capillary malformation, lymphatic malformation, and
venous malformation.
IMAGING
■ Less than 10% of patients require imaging to diagnose their vascular anomaly.
■ If the diagnosis is unclear by history and physical examination, ultrasound is
the first-line imaging study because it is easy to perform and does not require
sedation in children.
■ If the diagnosis remains uncertain after ultrasonography, then MRI with
contrast is obtained.
■ Imaging is not required prior to resection of hemangiomas, pyogenic
granulomas, or capillary malformations.
■ Before operative intervention for a venous malformation, lymphatic
malformation, or arteriovenous malformation, MRI typically is obtained to
determine the extent of disease.
DIFFERENTIAL DIAGNOSIS
■ Fibrosarcoma
■ Neurofibroma
■ Pilomatrixoma
■ Teratoma
NONOPERATIVE MANAGEMENT
■ Nonoperative intervention is the mainstay of treatment for vascular anomalies;
lesions are benign and often can be observed.
■ Less than 10% of infantile hemangiomas require treatment. Problematic
lesions during the proliferative phase can be managed with topical timolol,

corticosteroid injection, prednisone, propranolol, or rarely, resection.
■ Kaposiform hemangioendothelioma is treated with vincristine or sirolimus.
■ First-line intervention for problematic vascular malformations typically is
nonsurgical: capillary malformation (pulsed-dye laser), venous malformation
(sclerotherapy), lymphatic malformation (sclerotherapy), arteriovenous
malformation (embolization).4–7
SURGICAL MANAGEMENT
■ Operative treatment of vascular anomalies generally is performed for
symptomatic patients who are not candidates or have failed nonoperative
interventions.
■ Residual infantile or congenital hemangiomas causing a deformity can only be
improved with resection (or pulsed-dye laser for telangiectasias).
■ The primary treatment of pyogenic granuloma is full-thickness skin excision
or cautery (curettage, laser, and cryotherapy have a recurrence rate as high as
50%).
■ Capillary malformation can cause overgrowth of tissues beneath the stain,
which can be improved by resection.
■ Operative intervention for venous malformation is reserved for small lesions
that may be removed for cure or for a residual deformity following
sclerotherapy.
■ Macrocystic lymphatic malformation may have redundant skin following
sclerotherapy that can be resected. Microcystic lesions are not amenable to
sclerotherapy, and thus, excision often is first-line treatment.
■ Arteriovenous malformations generally are removed if they remain
symptomatic following embolization.
Preoperative Planning
■ A critical principle is that vascular anomalies are benign and thus do not
require radical resection; the scar and reconstruction should not cause a worse
deformity than the appearance of the lesion.
■ Vascular anomalies typically are diffuse and involve multiple tissue planes;
consequently, most resections are subtotal.
■ The goal of operative management is to improve the patient’s symptoms by
removing enough of the lesion to reduce pain, bleeding, and/or ulceration
and/or improve appearance.

■ Symptomatic infantile hemangiomas are best excised between 3 and 4 years of
age after they have completed involution.
■ If possible, resection should be postponed until the child is at least 6 months of
age to reduce the anesthetic risk.
■ Consideration should be given to improving deformities before 4 years of age
to minimize impact on self-esteem.
■ Venous, lymphatic, and arteriovenous malformations usually are removed after
they have been treated with sclerotherapy or embolization; these interventions
reduce the size of the lesion, decrease bleeding, create fibrosis, and facilitate
the operation.
Positioning
■ The table is turned 90 degrees during resection of head/neck lesions.
■ To limit blood loss, the head of the bed is elevated for facial vascular
anomalies.
Approach
■ Intraoperative antibiotics are not administered except when resecting large
lymphatic malformations because they are prone to infection.
■ After marking the extent of the resection, bupivacaine with epinephrine is
infused throughout the operative site to reduce blood loss and pain.
■ Markings are tattooed for lesions located on the vermillion or mucosa.
■ To reduce blood loss, cold epinephrine-soaked sponges can be applied to the
operative field; clips may be applied to the skin edges to decrease bleeding.
■ After making the incision, hemostasis should be obtained prior to proceeding
with further excision to avoid significant blood loss.
■ Margins are not required; the least amount of tissue as possible should be
removed to achieve the operative aims.
■ A pressure dressing usually is applied to minimize the risk of postoperative
hemorrhage.
T E C H N I Q U E S
■ Lenticular Excision and Linear Closure

■ Most vascular anomalies act as a tissue expander, and thus, adequate skin is
available to close the wound (TECH FIG 1).
■ The majority of resections are subtotal, and thus, missing integument is rare.
■ Skin grafts or local/regional flaps rarely are necessary to reconstruct an area
following extirpation.
■ Incisions are designed to be as inconspicuous as possible (eg, relaxed skin
tension lines, hairline, etc.).
■ Vascular anomalies often cause local ischemia because of insufficient
oxygen delivery; consequently, interrupted rather than continues sutures are
preferred for closure.
■ After resecting a facial lesion in children who are less able to tolerate suture
removal, 7-0 chromic followed by cyanoacrylate glue and tape can be used.
TECH FIG 1 • A 3-year-old with an involuted infantile
hemangioma managed with lenticular excision and
linear closure. A. Preoperative appearance. B.
Following resection. C. Early postoperative view.
■ Circular Excision and Purse-String Closure
■ This technique is useful for round lesions to limit the length of the scar,
which would be 2 to 3 times the diameter of the lesion if it was excised
lenticularly (TECH FIG 2).8,9
■ Circular excision is most commonly performed for infantile hemangiomas
after they have involuted, but can be used for any round skin lesion.
■ Almost all procedures are conducted on the face where it is important to

limit the length of the scar; round lesions involving the trunk or extremity
usually undergo lenticular excision because the linear scar is concealed and
appears favorable in a relaxed skin tension line.
■ A contraindication to circular excision is the scalp because there is minimal
tissue laxity, a circular scar can cause visible alopecia, and it is not critical to
limit the length of a scar in the scalp because it is camouflaged by hair.
■ When closing the circular excision, it is important to undermine the skin
edges to reduce tension; I usually prefer a Vicryl purse-string suture. After
the purse-string closure, I approximate the epidermis/dermis with interrupted
chromic or nylon suture (depending on the age of the patient) followed by
cyanoacrylate glue and tape.
■ Disadvantages of this technique are that there is an increased risk of wound
dehiscence compared to linear closure and a second stage may be required to
convert the circular skin into a line.

D