Cleft lip and palate are the fourth most common congenital birth defect. Management requires multidisciplinary care owing to the complexity of these clefts on midface growth, dentition, Eustachian tube function, and lip and nasal cosmesis. Repair requires planning, but can be performed systematically to reduce variability of outcomes. The use of primary rhinoplasty at the time of cleft lip repair can improve nose symmetry and reduce nasal deformity. Use of nasoalveolar molding ranging from lip taping to the use of preoperative infant orthopedics has played an important role in improving functional and cosmetic results of cleft lip repair.
Key points
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The cleft lip design should be measured carefully and executed to reduce variability.
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Primary rhinoplasty at the time of the lip repair repositions the ala improves the stigmata of the cleft lip nasal deformity.
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Specialized orthodontists can be very effective using nasoalveolar molding to simplify the lip repair and ultimate outcome.
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Specialized orthodontia is a labor-intensive therapy that requires parental compliance and motivation.
Video content accompanies this article at http://www.facialplastic.theclinics.com .
Introduction
Orofacial clefts occur in a spectrum that include cleft lip–cleft palate and are the most common craniofacial birth defect. Cleft lip repair is just the beginning of sequential, interdisciplinary care that this patient population requires. Presurgical care can be optimized by partnering with specialized orthodontists and the use of nasoalveolar molding (NAM) with presurgical infant orthopedics (PSIO). This therapy can enhance the surgical repair. The cleft lip and its corresponding nasal deformity should be considered a complex dentofacial problem in most cases. Often, the residual cleft nasal deformity results in permanent cleft stigmata. An interdisciplinary cleft team can effectively identify and guide treatment in dentition, speech, swallowing, hearing, and psychosocial issues. The objectives of this manuscript are to describe an evidenced-based review of presurgical care (eg, lip taping and NAM), as well as preferred techniques for lip repair and primary rhinoplasty.
Introduction
Orofacial clefts occur in a spectrum that include cleft lip–cleft palate and are the most common craniofacial birth defect. Cleft lip repair is just the beginning of sequential, interdisciplinary care that this patient population requires. Presurgical care can be optimized by partnering with specialized orthodontists and the use of nasoalveolar molding (NAM) with presurgical infant orthopedics (PSIO). This therapy can enhance the surgical repair. The cleft lip and its corresponding nasal deformity should be considered a complex dentofacial problem in most cases. Often, the residual cleft nasal deformity results in permanent cleft stigmata. An interdisciplinary cleft team can effectively identify and guide treatment in dentition, speech, swallowing, hearing, and psychosocial issues. The objectives of this manuscript are to describe an evidenced-based review of presurgical care (eg, lip taping and NAM), as well as preferred techniques for lip repair and primary rhinoplasty.
Epidemiology
Orofacial clefting is the fourth most common birth defect after congenital heart deformities, spina bifida, and limb deformities. The incidence of cleft lip–cleft palate in the United States is between 1 in 600 and 1 in 750 live births, with some ethnic variability. A higher incidence in Native American and Asian populations is noted, and the lowest incidence is in African Americans and Africans. Isolated cleft palate is considered separate from a cleft lip occurring with or without cleft palate. Approximately two-thirds of orofacial clefts are cleft lip with or without cleft palate, whereas one-third are isolated cleft palate. The majority of cleft lip with or without cleft palate cases are unilateral and are more commonly left sided. Isolated cleft palate is more common in females, whereas cleft lip with or without cleft palate is more common in males.
The classification of an orofacial cleft based on laterality of the cleft lip (unilateral or bilateral), severity, and involvement of lip, alveolus, and/or palate. A complete cleft lip extends through the lip and nasal sill, whereas an incomplete cleft involves diastasis of the orbicularis oris and skin, but remains intact for at least three-quarters of the lip length. The microform, and less described nanoform, cleft is characterized by a philtral skin groove, minor nasal alar hooding and alar base asymmetry, furrowing of the orbicularis oris muscle, and a notch at the vermilion–cutaneous junction. A microform cleft lip, also called a form fruste, does not extend to more than one-quarter of the labial height, measured from the normal peak of Cupid’s bow to the nasal sill. The cleft alveolus can be complete or notched. Independent of the cleft lip type, a cleft palate can be unilateral (1 palatal shelf is attached to the nasal septum) or bilateral, and include the primary palate, portions of the hard and soft palate, or soft palate only.
Timing of interventions
Interdisciplinary cleft team management of a child with a cleft lip–cleft palate follows a typical timeline. The cleft lip is typically repaired at 3 to 5 months of age, but may be later if NAM is chosen. Those with cleft palate have a higher incidence of Eustachian tube dysfunction, which is managed with bilateral tympanostomy tube placement based on tympanogram and otomicroscopy. We use a selective tube placement and obtain a behavioral audiogram around 8 months of age. Routine speech assessment and therapy begins in the first 2 years with routine 6-month follow-up. This leads to velopharyngeal dysfunction assessment and potential secondary speech surgery. Alveolar bone grafting usually needs orthodontic preparation at around 7 to 10 years old, with definitive orthognathic surgery reserved for those with dentofacial malocclusion after full skeletal growth. This may be followed by a cleft septorhinoplasty.
Bilateral cleft lip
The bilateral cleft lip presents a more involved defect of both sides of the premaxilla/prolabium, but obtaining the general symmetry is inherently easier than in the grossly asymmetric unilateral cleft lip deformity. The greatest challenges of the bilateral cleft lip repair are dealing with the short columella and upper lip, protruding premaxilla, and persistent nasal deformities, including hooding of nostrils and lack of tip projection and definition. Some surgeons choose a 2-stage repair with primary lip repair in infancy and a secondary columellar lengthening between 1 to 5 years of age. More commonly, a 1-stage Mulliken or Millard approach is performed with PSIO and/or NAM used for the more complex, wide cases.
Bilateral cleft lip
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Premaxilla is not attached to the lateral palatal shelves.
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Forward projected premaxilla.
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Absent or small anterior nasal spine.
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Posteriorly displaced lateral piriform apertures.
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Widely splayed lower lateral cartilages.
A wide bilateral cleft lip ( Fig. 1 ) may have a protruding premaxilla and excessive tension on the lip segments with the pinch test to allow a primary 1-staged repair. In these situations, we prefer to partner with our cleft team orthodontist team using NAM (see NAM section) to set the premaxilla back, establish the maxillary arch and increase columellar length with nasal prongs ( Fig. 2 ). In cases where NAM is not possible, the repair is delayed and lip taping is applied ( Fig. 3 ).
A staged cleft lip repair (lip adhesion) in a child with any of the following: (1) too old to start NAM (owing to rigidity of maxillary segments), (2) grossly asymmetric cleft, or (3) diminutive prolabium (<6 mm in height) A delayed definitive cleft lip repair is completed months later. In rare cases, premaxillary repositioning with vomer osteotomy is an option. Caution is advised owing to the risks of premaxilla devascularization and potential growth inhibition.
Surgical Technique: Bilateral Cleft Lip Repair with Primary Rhinoplasty
No matter what technique is chosen, precise planning and markings are of utmost importance. When one can see the landmarks well enough to create the correct markings, the lip repair becomes a calculated method with less trial and error. Cupid’s peak, the vermillion–cutaneous junction, and the junction of the columella and lip are examples of landmarks that should not be distorted by local anesthesia injections.
Measurements begin with design of a lozenge-shaped philtrum from the prolabial skin and soft tissues (there is no muscle in the prolabium in a complete bilateral cleft lip). Keys to this repair, based on Mulliken’s techniques, are to use back cuts on the lateral lip vermilion, which create flaps that will be sewn under the prolabium. Infraorbital nerve block and vasoconstriction of the superior labial arteries, buccal sulcus, and nose (columella and ala) is completed with gentle injection of bupivacaine 0.25% with 1:200,000 epinephrine injections.
Design of Prolabial Flaps
Initial the markings are made with a temporary tattoo of methylene blue using a 27- or 30-gauge needle and include the following ( Fig. 4 ):
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Subnasale— midline prolabium at the junction of the lip and columella;
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Alare— lateral-most aspects of bilateral nasal ala;
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Cupid’s bow peaks (4–5 mm apart) at the vermilion–cutaneous junction; and
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The columella–nasal sill junction.
Next the philtral heights (6–8 mm) and distance from subnasale to alare are measured. The philtrum is designed to be in the shape of a lozenge or standard necktie. Perpendicular lines from the columella–nasal sill junction extend below the nasal sill (these will receive the lateral lip flaps). The neophiltral flap is incised centrally and the lateral prolabial flaps are deepithelialized.
Design of the Lateral Lip Segments
Markings in the lateral lip include the following (see Fig. 4 ):
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Noordhoff’s point— vermilion–cutaneous junction where the cutaneous roll and vermillion (dry lip) fade as you trace superiomedially.
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Noordhoff’s red line— the (wet–dry) vermillion–mucosal junction.
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High point of flaps are chosen to include dermal hairs but not vibrissae of nose.
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Vermillion flap back cut marks approximately 3 to 4 mm inferior lip flap mark (dry vermillion).
The vertical philtral lip height was measured on the prolabium and is juxtaposed to advancement flap height (∼8 mm). Similar markings are made on the opposite side.
Flap Mobilization and Muscle Dissection
A 15-C blade or ophthalmologic cornea knife is used for the cutaneous incisions, which are made with a tourniquet squeeze of the lip using thumb and index fingers. The vermillion flaps are incised about 3 mm proximal to most inferior advancement flap point. Mucosal incisions using a cut setting on cautery are followed by blunt dissection of the lateral lip from the supraperiosteal plane of the maxilla. In wider clefts, cautery dissection is continued down to the insertion of the inferior turbinate to facilitate digital release of the alar base from the piriform aperture. This is repeated on the contralateral lip. The lateral lip segments are retracted medially to test the tension.
The prolabial incisions are made second to avoid dependent bleeding onto the lateral lip markings. The philtral columns are incised just through the dermis to maximize blood supply. The lateral circular prolabial incisions are made down to but not including the sulcus mucosa, which is preserved to cover the anterior face of the premaxilla (to prevent adherence to the reconstructed orbicularis oris muscle). Bilateral septal mucoperichondrial flaps are raised with a Freer elevator for nasal floor closure.
The orbicularis oris muscle layer is dissected free from the “sandwich” of overlying dermis and underlying mucosa just deep to the minor salivary glands. Adequate muscle release is when muscle approximation is tension free. Countertraction with a small double prong retractor and forceps facilitates muscle release.
Primary Rhinoplasty
Bilateral partial marginal incisions are created to expose the nasal tip fat pad, which is freed and passed superiorly. The lower lateral cartilages are sewn together in the midline in a lateral crural steal maneuver. The cephalic borders of the lower lateral cartilages are secured cephalad onto the upper lateral cartilages, similar to Skoog. When there is excessive hooding, an elliptical excision of the soft tissue triangle hooding is completed (Tajima’s reverse U).
Closure
The prolabium mucosa is laid superiorly and sewn to the premaxillary periosteum to create the gingivobuccal sulcus, which the orbicularis oris muscle will glide across. The nasal floor is closed with 5-0 chromic. An alar base cinching suture is placed using a “key” suture of 4-0 Vicryl or polydiaxone. The alare–alare distance is typically set as narrow as possible (∼25 mm in infants).
The intraoral lip mucosa is joined in the midline with 4-0 absorbable sutures. Simple and vertical mattress sutures (3-0 or 4-0 monocryl or polydiaxone) are used to join the lateral lip muscle flaps. The superior-most orbicularis oris is sewn to the periosteum of the nasal spine to accentuate the nasolabial angle.
A discrepancy in prolabium height to the lateral lip height is addressed with a standing cone repair at the alar base (minimal alotomy). A dermal suture from the philtrum to the deep muscle can create a philtral dimple. Deep 6-0 monocryl subcuticular sutures approximate the vermillion and philtrum. Lip closure creates tension on the columella and nasal tip flattening. The 2 dry vermilion flaps are sewn together, and the dart of the philtral column is inset. Cyanoacrylate surgical glue (Dermabond) in thin layers is used for final skin closure, and the lip is completed with 5-0 chromic. Nasal conformers are secured to the caudal septum. These stent open the collapsed nostrils, supporting the primary rhinoplasty maneuvers for up to 6 weeks ( Fig. 5 ).
Unilateral cleft lip
Cleft lip repair can be as simple as approximating the medial and lateral lip elements with preservation of natural lip landmarks. More severe clefts can involve the lip, alveolus, nasal sill, and extend back to the palate. Presurgical treatment for wider clefts can decrease wound tension and untoward results. Each surgeon will have a slightly different opinion about their favorite technique, but the general principles will align a concentric orbicularis oris muscle to establish symmetry and proportionality of the perioral and nasal landmarks.
Unilateral cleft lip
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Septal displacement out of the vomerine groove to the noncleft nostril.
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Shortened columella on the cleft side.
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Hypoplastic and malformed alar cartilage with short medial crus and elongated lateral crus.
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Inferior and posterior displacement of the lateral crus.
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Widened and asymmetric nasal tip owing to medial crus deformity and columella.
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Nostril asymmetry with widened nostril on the cleft side.
The design of the unilateral cleft lip repair can be categorized into 3 general schools: (1) straight-line closure, (2) geometric, and (3) rotation–advancement techniques. The rotation–advancement technique is the most common in the United States, which included the original Millard, and the Noordhoff, and the Mohler modifications ( Fig. 6 ). The Fisher subunit approach is a geometric approach that is increasingly more popular. The senior author uses a hybrid of this technique and describes it in detail.
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