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Vermilion notching, scarlike line or depression, lip
shortening
± Nasal deformity, usually mild
Surgery may or may not be indicated based on severity Incomplete CL (Fig. 13-1B)
Intact nasal sill (termed “Simonart band”—skin bridge
that contains no muscle)
Intact alveolar ridge Complete CL (Fig. 13-1C)
Clefting of the lip, nostril sill, and alveolus
Wider than incomplete clefts with greater cleft nasal
deformity
Complete CLP
CL deformity is same as above
Includes CP (posterior to incisive foramen)
RELEVANT LIP ANATOMY
Normal lip anatomy (see Chapter 26: Lip and Cheek Reconstruction, Fig. 26-1)
Central philtrum demarcated laterally by philtral columns and inferiorly by Cupid’s bow and tubercle Above the junction of vermilion-cutaneous border is mucocutaneous ridge (“white roll”) Within red vermilion, noticeable junction demarcating dry and wet vermilion (“wet-dry border”) Vertical height of upper lip = peak of Cupid’s bow to nasal sill
Newborn—10 mm
3 months—13 mm
Adult—17 mm
Musculature
Orbicularis oris—primary muscle of lip, has two well-defined components (CN VII)
Deep (internal)
Fibers circumferentially from modiolus to modiolus Functions as the primary sphincter for feeding
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Superficial (external)
Fibers run obliquely, decussate in the midline, and insert into the skin lateral to the opposite philtral groove forming the philtral columns. Philtral dimple is depressed centrally because no muscle fibers directly insert into the dermis in the midline. Provides subtle shades of expression and precise
movements of lip for speech. Pars marginalis—portion of orbicularis along the vermilion forming the tubercle of the lip with eversion of the muscle
Levator labii superioris
Fibers arise from medial aspect of infraorbital rim, insert near vermilion-cutaneous junction helping to define the lower philtral column and peak of Cupid’s bow. Functions to elevate the upper lip.
Blood supply
Superior labial arteries, bilaterally; columellar branch centrally
Branches of bilateral facial arteries
Sensory innervation: upper lip, maxillary division of trigeminal nerve (CN V2)
Motor innervation: zygomatic and buccal branches of the facial nerve (CN VII)
CLEFT ANATOMY
Unilateral CL
Muscles
Pathological insertion of orbicularis oris
Runs parallel along the edge of cleft and inserts on
alar base (cleft side), base of columella (noncleft
side)
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Responsible for nasal distortion and widening of
cleft with smiling
In incomplete cleft lips, some superficial orbicularis
fibers may traverse superior lip across the cleft Hypoplasia and disorientation of pars marginalis associated with disappearance of vermilion-cutaneous ridge (white roll) at cleft margin
Vertical lip height is decreased on noncleft side
Nasal abnormalities (Fig. 13-2)
Figure 13-2 The cleft nasal deformity. The lower lateral cartilage
on the cleft side is abnormally shaped and improperly positioned. Numbers 1 and 7, hypoplastic, flattened alar dome on the affected side; 2, lack of upper lateral cartilage overlap of lower lateral
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cartilage; 3, subluxed lower lateral cartilage with alar base displaced cephalad and posteriorly; 4, hypoplastic bony foundation (maxilla); 5, the caudal septum is pulled toward the non-cleft side; and 6, flattening of the nasal bones.
Hypoplastic, flattened alar dome on the affected side Lack of upper lateral cartilage overlap of lower lateral cartilage Subluxed lower lateral cartilage with alar base displaced cephalad and posteriorly Hypoplastic bony foundation (maxilla) Caudal septum is pulled toward the noncleft side by aberrant insertion of orbicularis oris Flattening of the nasal bones Shortened columella
Bilateral CL
Two lateral components (lesser segments) of lip-alveolus­palate with an intervening prolabium and premaxilla that varies in its degree of protrusion (mild vs “flyaway”)
Total absence of orbicularis muscle in prolabial segment results in the absence of philtral dimple/columns/white ridge and median tubercle Aberrant dry and wet vermilion on prolabial segment Absence of normal labial-gingival sulcus in premaxillary segment
Absence of Cupid’s bow Nasal abnormalities
Widened alar bases, with laterally flared alar domes and malpositioned cartilages Shortened columella Obtuse nasolabial angle
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EVALUATION
Often diagnosed prenatally on ultrasound Evaluate for associated anomalies (especially with isolated CP) Consultations
Genetics Social work Feeding/nutrition
Monitor for appropriate weight gain May require Haberman bottle or cross-cut nipple to reduce the work of feeding, especially with CP
Otolaryngology
Eustachian tube dysfunction (see Chapter 14: Cleft Palate) often requires myringotomy tubes. Repeat otitis media affects hearing and speech development.
MANAGEMENT
EARLY INTERVENTIONS
Preoperative molding may be used to bring cleft segments together to minimize tension during repair
Taping
Applied across both segments of the lip Requires compliant and reliable parents
Nasoalveolar molding (NAM)
Custom fabricated oral appliance with nasal stents adjusted weekly Positions nasal cartilages and alveolar processes to facilitate closure
Lengthens deficient columella
Takes advantage of increased plasticity of neonatal cartilage
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Active presurgical infant orthopedics
Orthodontic appliance (Latham device) rigidly fixed to palatal segments Parents adjust daily to bring alveolar segments into alignment Removed at the time of definitive lip repair
Lip adhesion: suturing cleft margins together
Incisions should be made in region that will be discarded at subsequent operation (mark key landmarks) Goal: turn a complete CL into an incomplete CL Definitive lip repair performed several weeks to months later
TIMING OF REPAIR
3 months of age, generally accepted “Rule of Tens” (historical criteria) for suitability for surgery
10 weeks old 10 lb Hemoglobin 10 mg/dL
May delay in syndromic patients with systemic concerns
GOALS OF REPAIR
Goals of unilateral repair Lengthen medial lip element Reconstitute orbicularis oris Restore Cupid’s bow, aligning white roll and wet-dry vermilion Correct nasal deformity (primary rhinoplasty)
Upright caudal septum Narrow alar base on the cleft side Establish convexity of lower lateral cartilage on the cleft side May use nasal conformers to maintain shape
Goals for bilateral repair
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Reconstitute orbicularis oris across premaxilla Achieve proper prolabial size and shape
~10 mm height
~3 mm from midline to each Cupid’s bow peak Formation of median tubercle from lateral lip vermillion-mucosa (see “Techniques” later) Correct nasal deformity (primary rhinoplasty) Narrowing alar base is key difference in emphasis from unilateral technique. Nasal conformers also used. Symmetry
KEY LANDMARKS (FIG. 13-3)
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Figure 13-3 Key landmarks in a cleft lip.
sbal, subalare; sn, subnasale; cphs, crista philtra superior; cphi, crista philtra inferior (aka Cupid’s bow peak), ls, labiale superius; ch, cheilion. (From Chung KC, Disa JJ, Gosain A, Lee G, Mehara B, Thorne CH, van Aalst J. Operative Techniques in Plastic Surgery. Wolters Kluwer; 2020. Tech Figure 8.3.1A.)
Alar bases (ie, subalare: alar insertion point onto upper lip) Columellar base midpoint
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Commissure bilaterally White roll
At the peak of Cupid’s bow on non-CL
At the trough of Cupid’s bow (tubercle) on non-CL
Additional mark equidistant from the trough to the peak on
medial cleft element
Define peak of Cupid’s bow for CL segment Peaks are ~3 mm from the trough on each side
*Noordhoff point: vanishing point of white roll on the CL segment
Most critical and difficult point to identify
Should correspond to region of thickest vermillion and
robust white roll
In bilateral cases, this is marked on each cleft segment
SURGICAL TECHNIQUES
UNILATERAL CL REPAIR
Multiple variations of techniques that have evolved over time Most are Z-plasty–based reconstructions Straight-line repair (Rose-Thompson): excision of cleft margin and primary straight-line closure
Incorporates Z-plasty to establish normal vertical height of
the lip Quadrangular flap (Le Mesurier): uses a back-cut above Cupid’s bow and a laterally based inferior rectangular flap to fill in the rotational defect of the medial lip element
Triangular flap (Randall-Tennison, Trauner, and Skoog):
incorporates Z-plasty to restore vertical height
May result in excess vertical length
Places scar in the center of lip with oblique portion crossing
philtrum
Rotation advancement: most common type of repair
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*Millard: see (Fig. 13-4) for step by step details
Figure 13-4 Unilateral cleft lip deformity, with preoperative markings for (Millard) repair. For
clarity, descriptive anatomy is used in place of numbers. First mark the peak of Cupid’s bow on the noncleft side. Then mark the nadir of Cupid’s bow. Extrapolate the proposed peak of Cupid’s bow in the cleft equidistant from the nadir of Cupid’s bow. Next, mark the wet-dry junction on the noncleft side. Then mark the superior point of the philtral column on the noncleft side: this is the height of the
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