■ The medial canthus is displaced inferiorly, and the lacrimal canaliculus on the
lower eyelid is usually disrupted.
■ Usually, it is possible to identify the punctum of the lower eyelid (FIG 3).
■ There is a coloboma of the lower eyelid (FIG 4).
■ The involved muscles of facial expression are distorted in their orientation.
■ The alar base and the lateral part of the nasalis muscle are displaced upward,
and the medial part of the cheek muscles is displaced downward.
■ The underlying maxillary bone is depressed or separated according to the
severity of the cleft.
■ Patients may have independent pathology in their cheek region and lip (eg, an
intact lip with the cleft only seen in the cheek region1–4) (FIG 5).
HISTORY AND PHYSICAL FINDINGS
■ The history taking and physical examination are similar to other patients with
craniofacial anomalies.
■ The history should include the family history, medications during the first
trimester, and prenatal diagnosis.
■ Physical examination should include the extent and location of soft tissue cleft,
eye condition, involvement of alveolus, palate, and also other associated
systemic problems.5
FIG 1 • A. Tessier no. 3 facial cleft. B. Tessier no. 4
facial cleft.
FIG 2 • A. The cleft lip of a Tessier no. 3 is similar to an
ordinary cleft lip. B. The cleft lip of a Tessier no. 4 is
lateral to the philtral column.
IMAGING
■ Radiographic images are usually not necessary in the first visit.
■ CT scanning is helpful to determine the extent of bony defect on orbital floor
if the patient has severe orbital dystopia (FIG 6).
FIG 3 • The punctum can be identified lateral to the
cleft.
FIG 4 • Coloboma of left lower eyelid.
■ Panoramic x-rays and CT scans are also helpful to evaluate the necessity of
bone grafting in the alveolar or cheek region when the patient is older (FIG 7).
■ In the author’s center, cephalometric and panoramic radiographs and CT scans
with 3D reconstruction are routinely recorded every 2 years from ages 5 to 17
years to monitor facial growth.
SURGICAL MANAGEMENT
■ Surgical correction of the soft tissue problems is undertaken between 3 to 6
months of age. This correction should aim at the restoration of normal facial
appearance and protection of the eye.
■ Bony reconstruction of the alveolar clefts is similar to the timing of alveolar
bone grafting in cleft patients, ie, in the mixed dentition period before the
eruption of the teeth in the cleft region.
■ The bony reconstruction for the skeletal defects on the face and orbit is
generally not undertaken till the patient is at least 3 years of age and more
commonly at the age of 5 years.
■ This allows the inner and outer tables of the calvarium to be split and
therefore obtain a large amount of bone graft with minimal donor morbidity.
■ However, when there is severe bony deficiency on the orbital floor causing a
significant dystopia of the globe, orbital floor reconstruction should be
considered at an earlier age.
FIG 5 • Tessier no. 3 cleft with cleft on cheek region and
intact lip.
FIG 6 • 3D CT scan of a right Tessier no. 4 facial cleft.
■ The repair of the soft tissue deficit in the Tessier no. 3 and no. 4 craniofacial
clefts is based on five basic components as outlined.6
■ A medial canthopexy with 2 mm overcorrection in an upward vertical
position
• The postoperative scar contracture will always pull the new medial canthus
inferiorly.
■ Midface rotation advancement is performed with rotation of the medially
based nasal flap, advancement of the laterally based cheek flap, and
repositioning of the facial muscles.
• This helps to lengthen the shortened oculo-oral and oculo-alar distances
while restoring normal facial expression.
• This is similar to the rotation-advancement repair in ordinary cleft lip.
• The lengthening achieved in the midface region should primarily be due to
muscle repositioning rather than skin Z-plasties.
■ Placement of the surgical scars should be along the junction of the facial
units, rather than placing multiple Z-plasties on the cheek, to avoid color
mismatching due to skin differences in the nasal and cheek regions.
• Placement of the scar lateral to the junction of the facial units will result in
visible scars on the cheek region.7–11
■ Repair the cleft lip using the standard technique (ie, discard the redundant
tissue lateral to the proposed philtrum to avoid an unnatural-looking lip).12,13
■ Downward and medially repositioning the involved alar base with 1 to 2 mm
of overcorrection in vertical dimension
• The postoperative scar contracture in cheek will always pull the alar base
upward.
Positioning
■ The soft tissue repair is performed under general anesthesia.
■ The endotracheal tube is usually fixed to the center of the lower lip with tape.
■ The patient is placed in supine position with the face disinfected and draped.
FIG 7 • Panoramic radiograph of a bilateral Tessier no.
4 facial cleft.
T E C H N I Q U E S
■ Markings
■ With the patient prepared under general anesthesia, the key elements of the
cleft lip are marked, excluding the skin lateral to the philtral column on the
cleft side.
■ On unilateral facial clefts, the markings for the anatomical landmarks are
relatively easy.
■ The positions of the displaced medial canthus, the alar base, and the peak
of the Cupid’s bow can be determined by comparison with equivalent
landmarks on the noncleft side (TECH FIG 1A).
■ It should be emphasized that the position of the medial canthus and the alar
base should be overcorrected, ie, 2 mm higher for medial canthus and 2
mm lower for alar base (TECH FIG 1B,C).
■ It is much more difficult to determine these landmarks in the presence of
bilateral facial clefts.
■ However, the position of the medial canthus can still be determined by the
position of the lateral canthus, together with the width of the eyes.
■ The position of the alar base is determined by the position of the columella
and the vertical height of the lateral lip, which is further determined by the
vertical height of the prolabial segment.
TECH FIG 1 • A. A case of bilateral asymmetric
incomplete no. 4 facial clefts. The cleft lip on the left
side is not at the typical location of an ordinary cleft
lip. The left medial canthus is also slightly downward
displaced. The reason that surgery is not performed
on the cheek on the left side is because the deformity
is so mild that it is not worthwhile to reposition the left
medial canthus, with the resultant long paranasal scar.
Hence, only the cleft lip is repaired in bilateral fashion,
whereas unilateral repair is performed on the cheek.
The ruler demonstrates the mirror-image position of
the medial canthus on the cleft side. B. The ruler
demonstrates the mirror-image position of the nasal
floor and alar base on the cleft side. C. The new
position of the medial canthus on the cleft side with 2
mm overcorrection.
■ Incisions
■ Incision lines are marked along the margin of the nasal unit medially in a
curvilinear fashion upward to the true medial canthus, then turning laterally,
incorporating a triangular-shaped medially based flap on the upper eyelid.
■ The incision then comes back medially to the proposed point of the new
medial canthus.
■ The lateral skin incision is made on the lateral cleft margin up to the medial
canthus.
■ The lip landmarks and incision lines are marked as regular unilateral or
bilateral cleft lip repairs (TECH FIG 2).
TECH FIG 2 • The incision lines: the prolabial width is
similar to the design of an ordinary bilateral cleft lip.
The tissue lateral to the proposed philtral column is
discarded. A medially based triangular flap is designed
on the upper eyelid.
■ Dissection and Facial Flap Elevation
■ The redundant skin is excised and discarded to avoid an unnatural-looking
repaired cleft lip.
■ The extent of the muscle dissection is similar to that in an ordinary cleft lip
repair (TECH FIG 3A–C).
■ On the midface, the laterally based cheek flap is then elevated
subperiosteally with a relaxing incision on the periosteum lateral to the
infraorbital neurovascular bundle (TECH FIG 3D,E).
■ This allows the cheek flap to be advanced medially up to the lateral edge of
the nasal flap and down to the philtral column.
■ The muscle layer of the cheek is then separated from the skin for a few
millimeters to facilitate the muscle approximation in later steps.
■ The medially based nasal flap (in unilateral lesions) and the alar base are
raised off the underlying nasal cartilages and the frontal process of the
maxilla to allow downward rotation of the shortened oculoalar tissue. In
patients with bilateral lesions, the nasal flap is based superiorly as there are
incisions along both sides of the nose.
■ The nasalis muscle is identified and dissected from the overlying skin in a
sheet for about 5 mm for later muscle reorientation (TECH FIG 3F).
■ In cases of complete no. 3 facial clefts, the nasal lining and the lining of the
eyelid are repaired by local turnover mucosal flaps between the nasal and
cheek incisions (TECH FIG 3G).
(Trang không có chữ)
TECH FIG 3 • A. The incision of the lip part. The
excessive tissue on the right lateral lip is preserved
initially as it might be needed later for lengthening of
the cheek region. B. The incision and dissection of the
lip part is similar to a bilateral cleft lip repair. C. The
extent of the muscle dissection on the lateral lip. D.
Subperiosteal dissection of the cheek flap. E. A
relaxing incision is made lateral to the infraorbital
neurovascular bundle (arrow) to achieve adequate
mobilization of the cheek tissue. F. The dissection of
the nasalis muscle (arrow) in the nasal flap. G.
Turnover flap along the cleft margin for reconstruction
of the nasal lining.
■ Eyelid Correction
■ Attention is then turned to the lower eyelid.
■ After dissection and freeing of the displaced facial musculature, the
subperiosteal mobilization of the laterally based cheek flap is extended
superiorly up to and including the inferior orbital rim (TECH FIG 4A,B).
■ Usually, it is not very difficult to identify the remnant of the medial canthal
tendon (TECH FIG 4C).
■ The overlying lower eyelid tissue incorporating the tarsus and the attached
conjunctiva are then transposed medially.
■ If necessary, a lateral canthotomy may be required to facilitate the medial
movement of the lower eyelid, correction of the coloboma, and
reconstruction of the lower eyelid (TECH FIG 4D).
TECH FIG 4 • A. Dissection along the infraorbital rim.
B. Dissection into the orbital floor. C. The remnant of
the medial canthal tendon (upper forceps). D. The
remnant of the medial canthal tendon and the lower
eyelid are advanced medially and superiorly to the
proposed position of the medial canthus.
■ Repositioning the Facial Musculature
■ The lateral cheek flap is then advanced medially.
■ A medial canthopexy is performed by transnasal wiring or directly suturing
to the local periosteum using the medial edge of the tarsal plate and the
remnant of the medial canthal tendon (TECH FIG 5A).
■ This will ensure adequate support for the lower eyelid.
■ The orbicularis oculi muscle of the lower eyelid is identified and apposed to
the superior limb of the newly created medial canthus (TECH FIG 5B).
■ It should be re-emphasized that this medial canthopexy always needs some
overcorrection in cephalic direction for about 2 mm; otherwise, the position
of the canthus will always be lower than its peer in the noncleft side after
skin closure.
■ The nasal complex of the medially or superiorly based nasal flap is then
rotated downward matching the alar base on the noncleft side to achieve
adequate oculoalar distance in unilateral lesions.
■ For bilateral clefts, the positions of the alar bases are determined by the
position of the columellar base (TECH FIG 5C,D).
■ Repositioning of the remaining facial musculature, in particular the nasalis
and the muscles of facial expression, is then performed.
■ The sutures are placed more superiorly on the nasal muscles and more
inferiorly on the cheek muscles to achieve a “rotation advancement” (TECH
FIG 5E).
■ With the redundant skin of the upper lip lateral to the philtral edge
previously excised, a rotation-advancement repair of the cleft lip is
performed in unilateral cases and bilateral cheiloplasty is performed for
patients with bilateral lesions (TECH FIG 5F).
■ The orbicularis oris is reconstituted and reapposed to the muscle bundle on
the noncleft side.
■ The medial edge of the cheek flap is then sutured to the lateral edge of the
nasal flap with the suture line placed along the junction of the facial units.
■ The medially based upper eyelid flap can be trimmed at this moment if the
oculo-oral or oculonasal distance can be restored without difficulty, or can be
placed as a small triangular flap on the lower eyelid under the medial canthus
to further lengthen the oculo-oral or oculonasal distance, or to break down
the straight line scar and to prevent possible postoperative scar contracture14
(TECH FIG 5G).
■ Repositioning the facial musculature and placing the suture line along the
junction of facial units results in a more natural facial expression and
therefore a better aesthetic result.
G B
TECH FIG 5 • A. The medial canthal tendon is sutured
to the periosteum of the new position of the medial
canthus using 4-0 Prolene suture. B. The orbicularis
oculi muscle of the lower eyelid is identified and
apposed to the superior limb of the newly created
medial canthus. C. The alar base position on the cleft
side is determined by the base of columella. D.
Excision of the excessive skin on the cheek flap helps
for the lengthening of the oculonasal distance. E.
Approximation of the muscle layer in cheek flap and
nasal flap. The sutures are placed higher on the nasal
flap and lower on the cheek flap to achieve the
“rotation-advancement” movement. F. The lateral lips
are approximated to the prolabium. The marking
under the right nasal floor shows the skin needs to be
excised for further downward movement of the nasal
floor. The medially based upper eyelid flap is still
preserved at this moment. G. The medially based
upper eyelid flap is excised finally as the length of the
oculonasal distance is adequate after reconstruction.
The suture lines are placed along the junction of the
facial units.
PEARLS AND PITFALLS
Markings
■ Precise marking of the anatomic position of the medial canthus
and alar base
Incision
■ Make an incision along the cleft to raise the laterally based
cheek flap and medially/superiorly based nasal flap.
■ The incision line of the lip is similar to ordinary cleft lip repair
with special care not to preserve the redundant tissue lateral to
the philtral column.
■ Design a medially based flap on the upper eyelid.
Dissection
■ Extensive subperiosteal dissection of the cheek tissue lateral to
the infraorbital foramen
■ Extensive perichondrial dissection of the nasalis muscle
Reconstruction
■ Local turnover flaps for the lining of eyelid and nose
■ Midface rotation advancement with approximation of the cheek
muscles and nasalis muscles
■ Medial canthopexy in an overcorrected position
■ Adjust the lower eyelid by the medial-based flap from the upper
eyelid.
Aesthetic
concerns
■ Placement of the suture lines along the junction of the facial
subunits
■ Design for overcorrection of the oculonasal or oculo-oral
distance (eg, move the proposed medial canthus upward and alar
base downward)
POSTOPERATIVE CARE
■ The postoperative care is similar to that used for primary cleft lip repair.
■ The airway maintenance and chest care are as important as that in any patient
who received general anesthesia.
■ The routine facial wound care in Chang Gung Craniofacial Center is to use
normal saline wet dressing changed with the frequency of every 4 hours.
■ This wound care can be replaced by local antibiotic ointment if the wounds are
clean without much discharge.
OUTCOMES
■ A retrospective review of the outcomes of surgical repair for rare facial clefts
in Chang Gung Craniofacial Center was done in the early 2000s. The study
reviewed the facial clefts over a period of 25 years.
■ Among the 41 different Tessier craniofacial clefts seen, most (17 patients)
were Tessier no. 3 and no. 4 clefts. Patients could be roughly divided into two
groups: a group of eight patients treated by the traditional Z-plasty principles
and second group of nine patients treated with the rotation-advancement
technique described. Of these nine patients, five had Tessier no. 3 clefts and
four had no. 4 clefts. Seven were primary cases, and the other two were
secondary or tertiary revisions.
■ None of the patients in the Z-plasty group had a satisfactory result in terms
of scar quality, color matching, or natural facial expression. The cases in the
rotation-advancement group, while their medial canthus and alar base might
not be well repositioned, still had much better results in their scar and facial
expression6 (FIGS 8 and 9).
FIG 8 • An example of the midface rotation
advancement on a Tessier no. 3 facial cleft. Before
operation (A) and at 13 years old (B and C).
FIG 9 • An example of the midface rotation
advancement on a Tessier no. 4 facial cleft. Before
operation (A) and at 17 years old (B–D).
COMPLICATIONS
■ Postoperative bleeding
■ Wound infection
■ Wound dehiscence
■ Hypertrophic scar formation
■ Undercorrection with malposition of the medial canthus or alar base and
persistent ectropion of lower eyelid.
REFERENCES
1. Tessier P. Anatomical classification of facial, craniofacial and latero-facial
clefts. J Maxillofac Surg. 1976;4:69.
2. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: order
out of chaos (Tessier classification). Clin Plast Surg. 1976;3:529-572.
3. van der Meulen JCH. Oblique facial clefts: pathology, etiology and
reconstruction. Plast Reconstr Surg. 1985;76(2):212-224.
4. Ortiz Monasterio F, Fuente del Campo A, Dimopulos A. Nasal clefts. Ann
Plast Surg. 1987;18(5):377-397.
5. Allam KA, Lim AA, Elsherbiny A, Kawamoto HK. The Tessier number 3
cleft: a report of 10 cases and review of literature. J Plast Reconstr Aesthet
Surg. 2014;67:1055-1062.
6. Chen PK, Chang FC, Chan FC, et al. Repair of Tessier no. 3 and no. 4
craniofacial clefts with facial units and muscle repositioning by midface
rotation advancement without Z-plasties. Plast Reconstr Surg. 2012;129:1337-
1344.
7. Maeda T, Oyama A, Okamoto T, et al. Combination of Tessier no. 3 and 4:
case report of a rare anomaly with 12 years’ follow-up. J Craniomaxillofac
Surg. 2014;42:1985-1989.
8. Alonso N, Freitas RS, Oliveira e Cruz GA, et al. Tessier no. 4 facial clefts:
evolution of surgical treatment in a large series of patients. Plast Reconstr
Surg. 2008;122(5):1505-1513.
9. Laure B, Picard A, Bonin-Goga B, et al. Tessier number 4 bilateral orbito-
facial cleft: a 26-year follow-up. J Craniomaxillofac Surg. 2010;38:245-247.
10. Cizmeci O, Kuvat SV. Tessier no. 3 incomplete cleft reconstruction with alar
transposition and irregular Z-plasty. Plast Surg Int. 2011;2011:Article ID
596569.
11. Versnel SL, van der Elzen MEP, Wolvius EB, et al. Long term results after 40
years experience with treatment of rare facial clefts. Part 1: oblique and
paramedian clefts. J Plast Reconstr Aesthet Surg. 2011;64:1334-1343.
12. Noordhoff MS, Chen YR, Chen KT, et al. The surgical technique for the
complete unilateral cleft lip-nasal deformity. Operat Tech Plast Reconstr Surg.
1995;2(3):167.
13. Chen PK, Noordhoff MS. Treatment of complete bilateral cleft lip-nasal
deformity. Semin Plast Surg. 2005;19(4):329-341.
14. Longaker MT, Lipshutz GS, Kawamoto HK. Reconstruction of Tessier no. 4
clefts revisited. Plast Reconstr Surg. 1999;99(6):1501-1506.
17
CHAPTER
Tessier 7—Macrostomia
Repair
Patrick A. Gerety and Scott P. Bartlett
DEFINITION
■ Macrostomia—enlarged oral aperture–related clefting of the oral commissure
■ Tessier 7 cleft—lateral (transverse) facial cleft from the oral commissure to the
zygomaticotemporal suture
■ Cheilion—anatomic name for commissure point
■ Commissuroplasty—recreation of oral commissure
ANATOMY1
■ Oral commissure (FIG 1)
■ Position
• Normal position: at a vertical line dropped from the medial limbus (some
variability)
■ Shape
• Normal shape: The upper and lower lip vermilion comes to sharp corner.
• Macrostomic commissure: Oblique angle, gaping at rest, round with smile
■ Tessier 7 cleft resulting in macrostomia
■ Variable in presentation—may be slightly wider oral commissure or severe
cleft of skin, subcutaneous tissue, muscle, and bone
■ Trajectory—the cleft occurs along a line from the oral commissure to the
zygomaticotemporal suture.
FIG 1 • Lateral facial cleft creates discontinuity of the
orbicularis oris at the oral commissure. This in turn
affects the location and insertion of the zygomaticus,
risorius, and depressor anguli oris muscles.
■ Affected structures
• Commissure—laterally positioned, anatomically abnormal (ie, made of cleft
tissue [lighter in color than normal vermilion])
• Orbicularis oris—interrupted and malinserted. The superior and inferior
limbs contribute to the appearance of the lips on the macrostomic side (see
FIG 1).
PATHOGENESIS
■ Development of normal facial anatomy is dependent upon early first and
second branchial arch embryology.
■ The maxillary and mandibular prominences normally fuse in early
embryologic development (weeks 4–6) forming the normal cheeks and lips.
■ Stapedial artery is the blood supply before the external carotid system has
developed.
■ Stapedial disruption starves all tissue types—skin, fat, muscle, and bone.
■ Macrostomia results as a presumed failure of this process.
■ Interruption of embryologic development along the “orotragal” line can affect
the oral commissure, muscles of mastication, muscles of facial expression,
mandible, maxilla, and auricle.
■ Isolated macrostomia is a disorder of the first branchial arch only.
NATURAL HISTORY2
■ Incidence: rare, 1 in 225 000 live births
■ Unilateral 80%, bilateral 20%
■ As with cleft lip, the left side appears to be more commonly affected.
■ May be associated with syndromes including the following:
■ Hemifacial microsomia
• Including Goldenhar syndrome and variants of oculoauriculovertebral
spectrum
• May account for as many as 25% of macrostomia patients
■ Treacher Collins syndrome
■ Auriculocondylar syndrome
■ Tessier 7: the most common of the rare craniofacial clefts (5%–14%)
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Laterally displaced oral commissure
■ Clefted/interrupted orbicularis oris
■ Oral incompetence may be encountered.
■ Gaping oral commissure in repose related to cleft orbicularis oris muscle
■ The cleft margin is demarcated. It does not contain normal lip elements (eg,
white roll, dry vermilion), but it is mucosalized. The normal dark red vermilion
can be seen to transition into a lighter colored cleft vermilion.
■ Spectrum of the cleft3
■ Minor form—1 to 2 cm in length ending medial to the anterior border of the
masseter muscle
■ Major form—cleft ends lateral to the medial border of the masseter. It may
terminate at the tragus or at the tonsillar pillar with disruption of superficial
and deep structures.
■ The commissure is blunted/effaced, and when a baby with macrostomia cries,
the commissural angle of an open mouth is more oblique than the normally
formed commissure—this is thought to be related to disruption of the normally
intact orbicularis ring.
■ Associated anomalies
■ Preauricular tags
■ Microtia/ear anomaly
■ Mandibular hypoplasia
■ Condylar abnormality
■ Zygomatic arch abnormality
■ Hearing loss
IMAGING
■ 3D craniofacial CT—if bony involvement is suspected, this may be obtained
to evaluate anatomy.
SURGICAL MANAGEMENT
■ Trilaminar repair—repair of oral mucosal, orbicularis muscle, and facial skin
■ Repair consists of
■ Excision of abnormal cleft margin tissue (mucosa)
■ Repair/repositioning of orbicularis muscle
■ Recreation of the oral commissure
■ Rearrangement of the cutaneous scar to avoid deforming contracture
■ Repair types (external cutaneous scar):
■ Straight line
■ Z-plasty
■ W-plasty
Preoperative Planning
■ Standard facial photos should be obtained preoperatively including repose and
animated (smiling) if possible.
■ Appropriate expectations should be created with the parents in terms of
realistic commissure appearance and the risk of scar hypertrophy/contracture.
■ Evaluation for other anomalies and comorbidities with specialist consultation
or multidisciplinary team as necessary
■ 3D CT scan if cleft is thought to involve skeletal structures
■ Timing—as with cleft lip surgery, macrostomia repair may be done early in
life. It is also reasonable to delay until late infancy if there is minimal
functional or feeding issue. Late treatment reported in the literature is a
function of later age at presentation and is usually not due to planned delay in
treatment.
Positioning (FIG 2)
■ Nasal endotracheal tube allows unobstructed/distorted view of the mouth
during surgery.
■ Many groups use oral RAE intubation and midline taping that does not alter
the lower lip (tape mesentery on chin).
Approach
■ Determination of commissure position is most critical.
■ This can be mirrored from the contralateral lip in unilateral cases.
■ In bilateral cases, there are several considerations:
• The cleft demarcation can be fairly obvious—normally demarcates with
clefted tissue.
• The normal position of the commissure lies below a vertical line that is
dropped from the medial limbus. This can be marked.
• Normative measures have been proposed for this use. However, these
measures are highly variable and are not suitable for individual patient use.
FIG 2 • Securing of the endotracheal tube in order to
avoid lip and commissure distortion. Tegaderm is used
to keep tape secure in a wet (Betadine) environment.
(Note the commonly associated preauricular skin tags.)
T E C H N I Q U E S
■ Vermilion-Shifted Commissuroplasty With
Optional Cutaneous Z-Plasty (Kaplan Technique)
■ The senior author’s (SPB) preferred technique for macrostomia repair was
inspired by the work of Ernest Kaplan4 and expanded upon by John
Mulliken.5
■ This repair uses mucosalized flaps to avoid scars directly within the
commissure.
Markings
■ Markings are performed using methylene blue ink and a pen point
instrument.
■ Important landmarks include the following:
■ Normal commissure (if present)
■ Peak of right and left Cupid’s bow
■ Lower lip cutaneous midline
■ Vertical registration marks are also drawn above and below the normal and
proposed commissures as reference (TECH FIG 1).
■ In unilateral cases, the distance from the Cupid’s bow peak to the normal
commissure is reflected onto the cleft-side white roll of the upper lip.
■ The distance from the lower lip midline to the oral commissure is then
reflected onto the white roll of the lower lip at the cleft commissure.
■ Because it is difficult to measure these distances around the curvature of
the lip, it is often easier to create a vertical registration marking on the
unaffected side and to then reflect that measurement to the affected side
(see TECH FIG 1).
TECH FIG 1 • Landmarks for unilateral macrostomia
repair: (c) normal commissure, (c’) upper and lower lip
cleft commissure, (cb) peak of Cupid’s bow on each
side of the upper lip, (LLM) lower lip midline. The
distance from c to the ipsilateral cb and to LLM is
measured. These measurements are then reflected to
the macrostomic side. Vertical registration marks can
allow for more accurate measurements and design.
■ In bilateral cases, the medial limbus is taken as the landmark for which to
position the commissures upon repair.
■ Vertical registration lines are drawn down from the medial limbus.
Design (see TECH FIG 2)
■ The commissure points are decided upon and tattooed with methylene blue
at the upper and lower lip white roll.
■ A vermilion flap is designed from either the upper or lower lip. The flap
begins medial to the commissure (c) point on the chosen lip. It is rectangular
in shape.
■ This flap will become the new mucosal commissure and importantly keep
suture line outside of the commissure.
■ The cutaneous margin of the cleft is marked—this tissue represents
cleft/mucosal tissue that will be reflected initially into the mouth. Some tissue
will be used for oral closure, and some will be discarded.
■ On the lip (upper or lower) that will receive the vermilion flap, the incision
at the cutaneous vermilion junction will also end medial to the commissure
(c) point.
■ The area of dissection is conservatively infiltrated with epinephrine-
containing solution.
Dissection and Orbicularis Repair
■ The markings are incised.
■ The vermilion flap is incised and raised.
■ The cleft mucosal tissue is freed and reflected intraorally with dissection to
include the deep surface of the orbicularis muscles.
TECH FIG 2 • Repair design. A vermilion flap is
designed that will bridge the new commissure in order
to keep a scar line out of the repair. The cleft margin
tissue shown with a dotted line is elevated and raised
intraorally. The vermilion flap begins medial to the
commissure point and will be inset medial to the
commissure point on the recipient lip.
■ The mal-positioned fibers of the orbicularis oris of the upper and lower lip
are dissected free.
■ The muscles are repaired in an overlapping fashion if enough muscle bulk
exists for such a repair. Otherwise, they can be repaired end to end (TECH
FIG 3).
■ Repair is done with 3-0 or 4-0 Vicryl suture.
Commissuroplasty and Skin Closure
■ The tattooed (c) points are united with a buried dermal suture.
■ The vermilion flap is then inset with 4-0 chromic recreating the commissure.
TECH FIG 3 • Orbicularis repair in macrostomia. A.
Overlapping. B. end to end.
■ Excess mucosa intraorally may be trimmed, and intraoral closure is
completed with 4-0 chromic.
■ Deep fascia is closed in the lateral skin wound.
■ A small midincision Z-plasty is designed for transverse lengthening if
needed.
■ Skin closure is performed with buried deep dermal 5-0 Monocryl or with
simple fast-absorbing gut sutures.
■ Dermabond is used for the cutaneous incision with one Steri-Strip placed
along the nasolabial fold.
■ Franco et al. applied a similar concept, carrying the vermilion and orbicularis
as a unit across the cleft to recreate the commissure (TECH FIG 4).6
TECH FIG 4 • A. Vermilion flap inset after repair. B.
Repaired macrostomia. The use of Z-plasty for
cutaneous closure is an intraoperative decision.
■ Modifications of the Cutaneous Scar (TECH FIG 5)
■ Linear scar closure with no modification7
■ Z-plasty closure along the nasolabial fold4
■ W-plasty modification of the cutaneous scar for aggressive nonlinearization8
TECH FIG 5 • Cutaneous closure of macrostomia. A.
Straight line. B. Z-plasty. C. W-plasty.
PEARLS AND PITFALLS
Endotracheal
tube
■ If using an oral tube, the taping/securing must be closely guided by
the surgeon. This ensures minimal commissure distortion and a
secure tube throughout the case.
Local
anesthesia
■ It is important to judiciously use epinephrine-containing local
anesthetic. It helps keep the field relatively bloodless, but one must
allow sufficient time for the effect to occur (ie, 5+ minutes). It is
also important to not tumesce and thus distort the tissues.
Medial
limbus
■ A critical landmark for commissure location in bilateral
macrostomia cases.
Scar
hypertrophy
■ Preoperative counseling, a proactive postoperative scar
management program, and an established timeline for scar
interventions are critical.
Lateralization
and rounding
■ Many macrostomia repairs will result in lateral migration of the
commissuroplasty and a nonanatomic commissure.
POSTOPERATIVE CARE
■ For 3 to 4 weeks postoperatively, the scar is treated with massage and silicone
gel.
■ Hypertrophic scar may be treated with steroid injection at 3 to 6 months.
■ Scar revision may be necessary 1 year or more postoperatively.
OUTCOMES
■ Excellent, symmetric results can be achieved. Rogers and Mulliken (2007)
reported on 13 patients5:
■ Age at surgery 11 months, follow-up 10.3 years
■ Commissure position averaged 1 mm difference
■ No lateral commissure migration
COMPLICATIONS
■ Dehiscence
■ Scar contracture with lateral commissure migration
■ Scar hypertrophy
REFERENCES
1. Verheyden C. Anatomical considerations in the repair of macrostomia. Ann
Plast Surg. 1988;20(4):374-380.
2. Woods R, Varma S, David D. Tessier no. 7 cleft: a new subclassification and
management protocol. Plast Reconstr Surg. 2008;122(3):898-905.
3. Buonocore S, Broer P, Walker M. Macrostomia: a spectrum of deformity. Ann
Plast Surg. 2014;72(3):363-368.
4. Kaplan EN. Commissuroplasty and myoplasty for macrostomia. Ann Plast
Surg. 1981;7:136-144.
5. Rogers G, Mulliken J. Repair of transverse facial cleft in hemifacial
microsomia: long-term anthropometric evaluation of commissural symmetry.
Plast Reconstr Surg. 2007;120(3):728-737.
6. Franco D, Franco T, da Dilva Freitas R, et al. Commissuroplasty for
macrostomia. J Craniofac Surg. 2007;18(3):691-694.
7. Kawai T, Kurita K, Echiverre N, et al. Modified technique in surgical
correction of macrostomia. Int J Oral Maxillofac Surg. 1998;27(3):178-180.
8. Bauer BS, Wilkes G, Kernahan DA. Incorporation of the W-plasty in repair of
macrostomia. Plast Reconstr Surg. 1982;70(6):752-757.
18
CHAPTER
Section V: Cutaneous
Lesions
Tissue Expansion for
Congenital Nevi
Lee W. T. Alkureishi and Bruce S. Bauer
DEFINITION
■ Congenital melanocytic nevi (CMN) are the most common congenital nevi.
■ They are usually present at birth, but “tardive” CMN may arise within the first
2 years of life.
■ CMN range from millimeter size to giant, covering the majority of the body.
■ Implications include risk of melanoma, CNS involvement, and disfigurement.
PATHOGENESIS
■ CMN result in a disturbance of neural crest cell migration between the 5th and
24th weeks of gestation, producing ectopic rests of immature melanocytes.1
■ This is thought to be related to mutations in NRAS and, to a lesser extent,
BRAF oncogenes.2
■ Nevus cells can extend deep into the subcutaneous tissue, fascia, muscle,
and/or periosteum (rare).
■ Involvement of the leptomeninges or brain parenchyma is known as
neurocutaneous melanosis (NCM).
■ Histologic findings specific to CMN include cell clustering, a round shape,
and location within eccrine ducts, in follicular epithelium or blood vessels, and
in deeper tissues.
NATURAL HISTORY
Malignancy Risk
■ Malignant melanoma may arise within the nevus or in extracutaneous
locations, most commonly the CNS.
■ Population heterogeneity, lack of standardized descriptors, and multiple
treatment modalities lead to difficulty ascertaining the true melanoma risk in
CMN.
■ Earlier studies reported lifetime risk of up to 40%.
■ More recent studies suggest much lower lifetime risk: less than 5% for all
patients with large CMN and under 2% for smaller lesions.3
■ Factors associated with increased melanoma risk include larger nevus size,
truncal location, and multiple satellite lesions. The greatest risk may be
extracutaneous.
■ Risk of melanoma arising within a small CMN prior to puberty is extremely
low.
■ The same is not true for large (LCMN) and giant (GCMN) lesions; 50% of
those developing malignancy do so within the first 3 years of life.4
■ To date, there has not been a confirmed case of melanoma arising within a
satellite lesion.
■ Other tumors associated with CMN include lipomas, schwannomas, sarcomas,
malignant cellular blue nevus, and undifferentiated spindle cell neoplasms.
■ Reduction of the risk of melanoma is still viewed as a valid medical indication
for excision.
NEUROCUTANEOUS MELANOSIS AND IMAGING
■ The presence of nevus cells within the leptomeninges, brain, or spinal column
is NCM.
■ Symptoms can range from none to progressive, severe neurologic impairment,
hydrocephalus, seizures, and death.
■ Problems arise from either benign proliferation of nevus cells blocking
cerebrospinal fluid flow or malignant degeneration.
■ Exact incidence is unknown but thought to be around 5%.
■ Approximately 5% of these patients will be symptomatic, the vast majority
developing symptoms before age 3.
■ Prognosis of symptomatic NCM is poor, with over 90% dying within 3 years
of diagnosis.
■ The highest risk of NCM is thought to be in patients with LCMN of the
posterior midline or greater than 20 satellite nevi. These patients should be
strongly considered for MRI screening.5
■ MRI demonstrates shortening of the T1 relaxation time and, less commonly,
T2 relaxation time.
■ For best sensitivity, MRI is recommended prior to 4 months of age due to the
lack of myelinization in the newborn.
■ In patients with symptomatic NCM, aggressive management of the cutaneous
lesion needs to be decided on a case-by-case basis and is generally not
recommended. This does not hold true for asymptomatic patients, in which the
prognosis is more favorable.
DIFFERENTIAL DIAGNOSIS
■ Blue nevus
■ Mongolian spot
■ Nevus of Ota/Ito
■ Café au lait spot
■ Nevus spilus
■ Sebaceous nevus
■ Spitz nevus
Classification
■ Several classification schemes have been proposed, most of which use the
projected adult size (PAS) of the nevus as the main feature.
■ PAS is calculated by multiplying the largest diameter of the nevus in infancy
by a factor that which varies by anatomical region (Table 1).
■ Large CMN are considered as any nevus with a PAS of greater than 20 cm or
greater than 2% of total body surface area.
Table 1 Projected Adult Size (PAS) of Congenital Nevi in Infancy (Data from
Krengel et al., 2012)
Body Area
Projected Adult Size (PAS)
Multiplication Factor
Head
1.7×
Neck, trunk, buttocks, arms
2.8×
Legs
3.3×
■ The most inclusive classification system is that recently described by Krengel
et al.6 (Table 2).
Nevus Distribution
■ CMN may arise anywhere on the body.
■ Large and giant CMN often occur in recognizable patterns or distributions.
■ Size and extent of involvement vary considerably between patients.
■ Extensive nevi often involve multiple adjacent areas and/or cross the midline.
■ Commonly encountered distributions include:
■ Scalp, forehead, root of nose, and upper eyelid
■ Face: cheek, nose, and lower eyelid
■ Trunk (posterior, anterior, or circumferential “bathing trunk” nevus)
■ Upper extremity (sleevelike distribution)
■ Lower extremity (as part of bathing trunk or isolated lower extremity)
FIG 1 • Facial nevus, involving the unilateral cheek and
abutting the lower eyelid.
■ The surgical management of these patients is tailored to the location of nevus,
extent of involvement, and presence of adjacent normal skin.
■ However, we describe common techniques and principles, many of which
may be applicable for the management of nevi in any location.
■ The example provided in this chapter focuses on a nevus involving the
unilateral cheek and lower eyelid (FIG 1).
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Patients are often seen in infancy.
■ Any changes in appearance of the lesion over time should be noted.
Table 2 Proposed New Classification of Congenital Melanocytic Nevi
CMN parameter
Terminology
Definition
CMN projected adult
size
“Small CMN”
“Medium CMN”
“M1”
“M2”
“Large CMN”
“L1”
“L2”
“Giant CMN”
“G1”
“G2”
“Multiple medium CMN”
<1.5
1.5–10 cm
10–20 cm
>20–30 cm
>30–40 cm
>40–60 cm
>60 cm
≥3 medium CMN without
a single, predominant
CMN
CMN localizationa
CMN of the head
CMN of the trunk
CMN of the
extremities
No. of satellite nevib
Additional
morphologic
characteristics
“Face,” “scalp”
“Neck,” “shoulder,” “upper
back,” “middle
back,” “lower back,”
“breast/chest,”
“abdomen,”
“flank,” “gluteal region,”
“genital region”
“Upper arm,” “forearm,”
“hand,” “thigh,” “lower
leg,” “foot”
“S0”
“S1”
“S2”
“S3”
“C0,” “C1,” “C2”
“R0,” “R1,” “R2”
“N0,” “N1,” “N2”
“H0,” “H1,” “H2”
No satellites
50 satellites
20–50 satellites
None, moderate, marked
color heterogeneity
None, moderate, marked
surface rugosity
None, scattered,
extensive dermal or
subcutaneous nodules
None, notable, marked
hypertrichosis
(“hairiness”)
aOne or more of these locations should be used to describe preponderant area of
involvement.
bRefers to number of satellites within the first year of life; in case this number is
not available, actual number should be mentioned.
CMN, Congenital melanocytic nevi.
Reprinted from 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, with permission from Elsevier.
■ Nevus size and location and the presence of multiple satellite nevi should
prompt screening for NCM.
■ A family history of melanoma should be elicited.
■ Potential psychological problems associated with this diagnosis should not be
underestimated, especially in older children and adolescents.
■ Emotional, behavioral, or social problems are present in 30%.
■ On initial examination, the extent of the nevus should be noted, including
involvement of critical structures and any areas of adjacent normal skin for
treatment planning.
■ Serial examinations every 3 to 6 months are recommended.
■ Darker pigmented areas or raised nodules may represent neural nevus rather
than malignancy.
■ Selective biopsy can be used to exclude malignancy for any suspicious areas
but is rarely indicated if excision and reconstruction are already contemplated.
NONOPERATIVE MANAGEMENT
■ Treatment of CMN is most often surgical, with excision and reconstruction.
Other modalities include dermabrasion, curettage, and laser therapy. Some
cases are best approached with observation alone.
■ Some giant nevi are so extensive as to preclude excision, and regular follow-
up by a pediatric dermatologist is recommended.
■ Although many LCMN are amenable to surgical excision and reconstruction,
the surgical experience needed for successful surgical management is not
readily available in many areas of the world.
Dermabrasion and Curettage
■ Dermabrasion and curettage are used to reduce, but not eliminate, the number
of nevus cells within a lesion.
■ Dermabrasion is used to abrade away the surface cells within the epidermis
and upper reticular dermis.
■ Curettage separates the cells at a natural cleavage plane between superficial
and deep dermis.
■ Both techniques are best performed within the first 15 days of life.
■ Lightening of the nevus can be successful initially, but this is variable and the
lesion may tend to redarken over time. Follow-up may also show marked
hypertrichosis.7
■ These techniques are theoretically less effective at reducing malignancy risk,
due to persistence of nevus cells at deeper levels. Resultant scarring may also
complicate surveillance of the lesion. Cases have been reported of melanoma
arising within the field of previous dermabrasion.8
Laser
■ Laser remains an attractive option, potentially reducing the level of
pigmentation without scarring.
■ Lasers used in the treatment of CMN include ruby, Q-switched, Er:YAG, and
carbon dioxide, either alone or in combination with Nd:YAG or Q-switched
ruby lasers.
■ Serial treatments are required, and scarring can occur with aggressive
treatment or improper settings.
■ Hypo- and hyperpigmentation may also occur and may be transient or
permanent.
■ The disadvantages of laser treatment are similar to those of
dermabrasion/curettage and include continued malignancy risk, scarring,
difficulties with surveillance, and redarkening of the lesion over time.
■ Despite their current limitations, nonoperative modalities do offer some
benefits in certain situations.
■ For example, lightening a thin lesion on the eyelid
■ Improvements in laser technology will likely continue to improve outcomes
with this modality, expanding its usefulness in the future.
SURGICAL MANAGEMENT
■ Surgical excision allows complete removal of nevus cells in the involved skin
and subcutaneous tissue.
■ Excision generally includes all tissue above the level of the deep fascia.
■ Although skin grafts have been used for resurfacing, the mainstay of
reconstruction is with tissue expansion.
■ Replacement of the excised tissue with similar thickness normal tissue (skin
and subcutaneous fat) provides the most functional and aesthetic outcome.
■ Early excision is recommended.
■ Procedures can be safely carried out starting as early as 6 months of age.
■ Those parents with concerns about multiple procedures under the age of 1 can
delay initial surgery until after the first birthday (though in expander patients
started at 6 months, the maximum additional surgeries under 1 year are only
two).
FIRST STAGE: PLACEMENT OF TISSUE EXPANDER(S)
Preoperative Planning
■ Treatment begins with the evaluation of the nevus extent and adjacent areas of
unaffected skin amenable to placement of tissue expander(s).
■ For lesions affecting the cheek and lower eyelid, a single expander placed in
the lower cheek/neck is often appropriate.9
■ The size of expander chosen is dependent on the size of lesion, availability of
adjacent unaffected tissue, and anticipated size of flap required.
■ For many infants, a 70- to 90-cc expander is the largest that can be placed.
■ With adequate expansion, the resultant flap can be designed to place
incisions to lie less conspicuously at the junction of adjacent aesthetic
subunits.
■ For more extensive nevi involving the temporal forehead, scalp, or upper
eyelid, an additional expander may be required in the forehead region.
■ For nevus involving large portions of the nose, an expanded forehead flap
based on the supraorbital or supratrochlear artery is the best option and must be
done as the first expansion.
■ The expanded forehead flap places the final scar along the brow rather than
midforehead.
■ For extensive forehead and nasal lesions, subsequent expansion and flaps can
be safely done without risk to the prior nasal flap.
■ Prior to surgery, the family undergoes extensive teaching regarding the
procedures, postoperative care, technique of tissue expansion, and anticipated
timing of the next stage.
■ In the majority of cases requiring multiple rounds of expansion, each round is
separated by at least 4 months.
Positioning
■ The patient is placed supine on the operating table.
■ A roll is placed under the upper back and shoulders, ensuring adequate neck
extension.
■ This is especially important, as it is frequently necessary to dissect the pocket
for the expander past the level of the mandible and into the neck.
■ General anesthesia is administered via an oral RAE endotracheal tube.
■ Care is taken to ensure this is secured exactly in the midline, avoiding
asymmetric distortion of the facial anatomy.
■ If the nevus is hypertrichotic, a clipper is used to ensure the margins are
clearly visible for incision planning.
■ The face, scalp, neck, and shoulders are cleaned with surgical soap and saline,
and drapes are applied.
Approach
■ Considerations in planning the incision:
■ The length of the incision should be adequate to facilitate dissection of the
pocket and insertion of the expander, but no longer than necessary.
■ The incision is generally placed within the nevus, at the inferior margin. By
staying within the nevus, all healthy uninvolved skin is preserved.
• In selected cases with a narrow inferior “finger” of nevus flanked by
adjacent normal skin, it may be preferable to place the incision farther into
the substance of the nevus. Although this will result in the nevus being
partially expanded, it achieves maximal expansion of the normal skin.
■ In patients with fine hairs along the border of the nevus, it may be preferable
to place the incision just beyond the margin of the nevus, as those hairs may
complicate cleaning the incision and increase the likelihood of secondary
infection.
■ The dimensions of the pocket for the expander are measured and marked on
the skin.
■ The pocket measures 1 cm greater than the expander in both dimensions.
T E C H N I Q U E S
■ The incision line is infiltrated with lidocaine 1% and epinephrine 1:200 000.
■ Incision is made with a 15c blade and deepened to the level of the
subcutaneous fat.
■ Care is taken to stay perpendicular, avoiding unnecessary undermining of
the nevus.
■ With Iris scissors, the supra-SMAS plane is carefully developed under direct
vision.
■ Once the plane is established, the skin flap may be laid flat, and the majority
of the remaining pocket can be dissected using the scissors without direct
visualization.
■ The scissor blades can be seen on the surface of the skin, and the depth of
the dissection can be accurately gauged by paying close attention to the
tips.
■ This approach helps to minimize trauma to the thin skin flap.
■ Particular care must be taken in the region just anterior to the mandibular
angle, where the marginal mandibular nerve may be encountered. In addition,
the facial artery and vein can potentially cause troublesome bleeding in this
region. The depth of dissection is critical in this region, to avoid injury to the
nerve below while maintaining a robust skin flap of sufficient thickness
above. Of note is that the marginal mandibular nerve lies deep to the
platysma, and if dissection remains superficial to the SMAS plane, the
marginal mandibular nerve is spared.
■ The dissection is carried over the mandible into the neck, to the extent of the
pocket previously marked.
■ The pocket is packed lightly with gauze for hemostasis, and attention is then
turned to the expander.
■ The location for the expander port is chosen and the length of tubing
adjusted as needed.
■ A remote internal port is used in all cases.
■ The port should be placed over firm underlying structures to facilitate
palpation.
■ The port should be placed through a limited tunnel to minimize risk of
displacement or flipping.
■ For lower face expanders, the temporal scalp is a typical location for the
port.
■ A narrow tunnel for the port is created using scissor dissection, a malleable
retractor of similar width to the port, a urethral sound, or a combination of
all.
■ The width of the tunnel should be limited to the diameter of the port, to
prevent migration of the expander along the tunnel.
■ A narrow gauge suction drain is placed into the expander pocket through a
separate stab incision (19-G butterfly IV, reconfigured as a drain).
■ The expander is filled with a volume of injectable saline dependent on size
and location of the expander, filling enough to ensure a smooth contour.
■ The port is generally inserted into the pocket first and guided into position
with finger pressure or using a malleable retractor.
■ The expander is rolled lengthwise, forming a thin tube with the edges rolled
underneath, which is then easily inserted into the pocket.
■ Once the majority of the expander is within the pocket, it can be unfurled
to lie flat.
■ Care should be taken to ensure that there are no folds present on the top
surface of the expander, particularly in the face where the overlying skin
flap can be very thin.
■ The skin is closed in layers with interrupted 5-0 clear nylon for the dermal
layer and a running 5-0 Prolene over this.
■ The expander port is then accessed, and an additional small volume of saline
may be injected.
■ The skin is observed and palpated during injection, to avoid overfilling.
■ In general, a total of approximately 15 cc is well tolerated at this stage.
■ The site is cleaned and dressed with bacitracin, Xeroform gauze, gauze
fluffs, and a loose SurgiNet dressing.
■ A tight dressing should be avoided over the expander, as this can easily
compromise the skin flap.
PEARLS AND PITFALLS
Physical
findings
■ The appearance of CMN is widely variable, ranging from
relatively inconspicuous flat, pale hairless nevi to dark or variably
pigmented, highly rugous/nodular nevi with hypertrichosis.
Technique
■ The use of Bovie electrocautery is limited, so as not to cause
thermal injury in the dissection of thin, potentially tenuous skin
flaps for the expander pocket.
■ A 19-gauge “butterfly needle” makes for an excellent drain. The
end of the tubing is perforated and placed into the pocket through a
separate stab incision. Standard blood draw vacutainers can be
used to provide suction.
■ Avoiding folds and points on the superficial surface of the
expander is key to minimizing the risk of skin breakdown and
subsequent exposure of the expander.
Postoperative
■ The key to successful expansion is to pay close attention to the
skin’s response to the fill. Aiming for a specified volume with each
expansion will inevitably lead to problems.
■ The first postplacement expander fill is typically 8–10 days
postoperatively, and expansion then continues weekly. This can
increase to q4-5 days in select cases if the overlying skin relaxes
sufficiently.
POSTOPERATIVE CARE
■ Patients can generally go home on the day of surgery, depending on parent
comfort.
■ Dressings are changed twice daily, keeping the incision and skin flap
moisturized with Aquaphor or similar dressing.
■ Drains are usually removed within 3 to 7 days, after which the dressing may
be minimal.
■ Postoperative antibiotics are continued until the drains are removed.
■ Expansion is begun at days 8 to 10.
■ The first expansion is performed under supervision in the clinic.
■ Subsequent expansions may be carried out at home or in the clinic depending
on parents’ preference.
■ Parents are advised not to focus on a target volume for each fill but to assess
the skin’s response to the additional volume.
■ Skin turgor
■ Blanching indicates excessive fill, and some volume should be removed.
■ Expansion should not be painful; discomfort indicates excessive fill.
■ Expansion is usually carried out weekly, unless the child has symptoms of
upper respiratory infection or fever of unknown origin.
■ The frequency may be increased to q4-5 days in some cases.
■ Total expansion time may range from 8 to 12 weeks.
■ Expansion may continue up until a few days before surgery (FIG 2).
SECOND STAGE: EXCISION OF NEVUS WITH EXPANDED
FLAP RECONSTRUCTION
Preoperative Planning
■ The planning of the second stage procedure begins prior to the first stage.
■ Position of the expander should take into account the anticipated flap design.
■ When advancing tissue from the neck to the cheek area, a transposition
design is optimal.
■ This allows greater flap movement and helps align scars optimally along the
junction between aesthetic subunits.
■ The surgeon must decide whether to base the transposition flap laterally (most
common) or medially.
■ The size and location of the lesion help to determine this.
■ Where early experience involved use of medially based flaps, the
inferolaterally based flaps used today allow better reconstruction of the
aesthetic unit with less risk of downward traction on the lower eyelid.
■ Smaller lateral based cheek lesions may be reconstructed with expanded
inferomedially based flaps.
■ Even with expansion, the size of nevus that can be excised laterally without
undue traction on the eyelid or oral commissure is limited.
Positioning
■ Positioning, prepping, and draping are the same as for stage I.
FIG 2 • A. Fully expanded lower cheek/neck tissue
expander (B) ready for nevus excision and expanded
flap reconstruction.
Approach
■ The initial incision is planned to facilitate removal of the expander.
■ The incision is marked around the inferior extent of the nevus, as before, but
outside the nevus margin (FIG 3).
■ If the nevus margins are very light or indistinct, it is better to err on the side
of preserving potentially uninvolved skin. Any areas that subsequently darken
may be removed at the time of later scar revision or satellite removal.
■ In cases where the nevus margin is clear, a surgical margin of 1 to 2 mm is
adequate.
■ Similarly, if only partial excision is planned/possible, then it is often better to
keep as much of the scars within nevus tissue that will later be excised. In
these cases, placing the incision within the nevus margin is advisable.
FIG 3 • Marking the incision for expander removal. The
extent of the inferior extension of the incision is
determined after expander removal.
■ For laterally based flaps, the incision is often carried inferiorly in the line of
the nasolabial fold, to facilitate flap transposition. However, the extent of this
inferior incision is determined later, after expander removal.
T E C H N I Q U E S
■ Incision
■ Incision is made with a 15c blade through the skin and deepened through the
subcutaneous fat with electrocautery to the level of the expander capsule.
■ The expander pocket is entered through the capsule.
■ The capsule is extremely important for the vascular supply of the flap. As
such, it is important not to undercut the flap at this stage, as excessive
removal of capsule can jeopardize the vascularity of the flap’s extremes.
■ The incision and deeper dissection are continued inferiorly for a short
distance in the line of the nasolabial fold and marionette line, and once a
large enough window has been created, the expander is removed.
■ The tubing connecting the expander to its port is cut, and the port is left in
situ for now. Removal is often easier later in the procedure.
■ The expander pocket is now fully accessible. The skin flap can be placed
under moderate tension in the direction of the planned transposition, and
limited release of the capsule with electrocautery facilitates greater
transposition without creating undue tension on the closure (TECH FIG 1A).
■ Care must be taken not to perform too much capsular release, as the
importance of its vascular contribution cannot be overemphasized.
■ The extent of the dissection is a delicate balance between facilitating
movement and preserving the viability of the flap.
■ With the capsule is released, the skin becomes the limiting factor in
achieving the desired transposition.
■ Options for skin release include extending the incision further down the
nasolabial fold/melomental fold, backcutting into the flap, or both (TECH
FIG 1B).
■ The type of extended incision or backcuts used is dependent on the extent
and location of the nevus and the extent and location of uninvolved
adjacent skin.
■ Small triangles of uninvolved skin adjacent to the flap margins can often be
used to fill in the defects created by backcuts.