Residency · Residency · Plastic Surgery
Cleft Lip: Evaluation and Primary Repair
Introduction
Cleft lip is one of the most common congenital anomalies, occurring in approximately 1 in 700 live births. Cleft lip with or without cleft palate (CL/P) is embryologically and genetically distinct from isolated cleft palate (CP). Male predominance for CL/P (2:1); female predominance for isolated CP. Ethnic variation: highest in Asian and Native American populations (~1:500), intermediate in Caucasians (~1:700), lowest in African descent (~1:2500). Left-sided clefts are most common (left:right:bilateral = 6:3:1).
Embryology and Pathogenesis
Cleft lip results from failure of fusion between the medial nasal process and the maxillary prominence during weeks 5-7 of gestation. Primary palate (premaxilla) is involved in complete cleft lip. Multifactorial inheritance: genetic susceptibility + environmental factors. Associated genes: IRF6, MSX1, TBX22, FGFR, TGFA, TGFB3.
Environmental risk factors: maternal smoking, alcohol, folate deficiency, anticonvulsants (phenytoin, valproic acid), retinoic acid. Syndromic associations in ~30% of cases: van der Woude syndrome (IRF6 mutation, lip pits), velocardiofacial syndrome, Stickler syndrome.
Classification
Unilateral Cleft Lip
Incomplete (forme fruste to wide): intact nasal sill/floor; Simonart band may be present. Complete: cleft extends through the entire lip, nasal sill, and into the nostril; associated with alveolar cleft and typically cleft palate. Microform: minor vermilion notch, minor muscle diastasis, subtle nasal deformity.
Bilateral Cleft Lip
Complete bilateral: premaxilla/prolabium is separated from both lateral lip elements; severe nasal deformity. Incomplete bilateral: one or both sides have a Simonart band. Asymmetric: complete on one side, incomplete on the other.
Veau Classification (for cleft palate)
Group I: soft palate only. Group II: hard and soft palate (secondary palate). Group III: unilateral complete (lip, alveolus, and palate). Group IV: bilateral complete.
Anatomy of the Unilateral Cleft Lip Deformity
Lip
Orbicularis oris muscle fibers insert abnormally into the alar base and columella base rather than crossing the midline. Lateral lip element: deficient in vertical height, rotated superolaterally. Medial lip element: shortened vertical height, philtral column absent or effaced on cleft side. Vermilion: deficient on medial side near cleft; white roll discontinuous. Cupid's bow: rotated superiorly on cleft side.
Nose (Cleft Nasal Deformity)
Lower lateral cartilage displaced inferiorly, posteriorly, and laterally on cleft side. Alar dome is flattened and widened. Columella is shortened and deviated to the non-cleft side. Nasal floor is absent on cleft side.
Alar base is displaced laterally and posteriorly. Nasal septum deviates to the non-cleft side (caudal septum). Nasal tip: asymmetric, under-projected.
Maxilla/Alveolus
Alveolar cleft: gap in the dental arch at the site of the lateral incisor. Greater (lateral) and lesser (medial/premaxillary) segments may be collapsed or expanded. Premaxillary deviation varies with cleft severity.
<image>Anatomical illustration comparing normal lip-nose anatomy (left side) with unilateral complete cleft lip anatomy (right side), shown in anterior view. The normal side shows intact orbicularis oris muscle crossing the midline, continuous white roll, symmetric Cupid's bow, and normal nasal cartilage position. The cleft side demonstrates the abnormal insertion of orbicularis oris into the alar base and columella base, the disrupted white roll and vermilion deficiency, the rotated Cupid's bow, the inferiorly displaced lower lateral cartilage with flattened alar dome, deviated columella, absent nasal floor, and the laterally displaced alar base. The alveolar cleft and maxillary segment separation are visible. All structures are clearly labeled with anatomical annotations.</image>
Preoperative Management
Presurgical Orthopedics
Nasoalveolar Molding (NAM): Acrylic intraoral molding plate with nasal stent. Begun at 1-2 weeks of age; weekly adjustments. Goals: approximate alveolar segments, elongate columella (bilateral), improve nasal cartilage position.
Reduces cleft width and may simplify surgical repair. Controversial: some centers report equivalent long-term outcomes without NAM. Lip taping/adhesion: approximates lip segments, guides alveolar alignment. Latham device: pin-retained active appliance for premaxillary repositioning (largely abandoned due to growth concerns).
Presurgical Evaluation
Feeding assessment: specialized bottles (Haberman, Dr. Brown's Specialty), upright positioning. Audiologic screening (high incidence of otitis media). Genetic evaluation for syndromic features. Multidisciplinary cleft team: plastic surgeon, orthodontist, speech pathologist, ENT, geneticist, social worker, psychologist.
Timing of Repair
Rule of 10s: 10 weeks of age, 10 pounds, hemoglobin 10 g/dL (traditional guideline). Most centers repair at 3-6 months of age. Earlier repair may offer psychological benefit to parents. Lip adhesion at 1-3 months may be used as a staged approach for wide clefts (controversial, less common today).
Surgical Techniques: Unilateral Cleft Lip
Millard Rotation-Advancement
Most widely used technique historically. Principle: rotation of the medial lip element downward; advancement of the lateral lip element into the gap. Medial lip: rotation incision along the base of the columella frees the Cupid's bow to rotate inferiorly to the correct position. Lateral lip: advancement flap fills the gap created by rotation.
Advantages: scar falls along the philtral column; flexible intraoperative design ("cut as you go"). Disadvantages: tendency to short lip on cleft side, straight-line scar, learning curve.
Fisher Anatomical Subunit Repair
Gaining widespread adoption as the preferred technique. Principle: precise anthropometric analysis with designed points placed at key landmarks. Key features: Scar placed along the philtral column using geometric planning.
Small triangular flap at the base creates the desired lip length. Preserves Cupid's bow anatomy more predictably. Less reliance on intraoperative improvisation. Advantages: reproducible, predictable lip length, minimal tissue discarding. Disadvantages: more rigid design; less flexibility for wide clefts.
Mohler Modification
Extends the Millard rotation incision into the columella. Provides additional length on the medial lip element. Scar extends onto the columella base.
Common Principles Across Techniques
Accurate identification and marking of key landmarks: Cupid's bow peaks (bilateral). Commissures. Alar bases.
Columella base. White roll at cleft edges. Orbicularis oris muscle repair: detach from abnormal insertions, reapproximate across cleft in anatomic position. Nasal floor reconstruction: closure of the nasal sill. Primary rhinoplasty: repositioning of the lower lateral cartilage, alar base repositioning.
Primary Rhinoplasty with Lip Repair
Widely accepted as standard of care at the time of lip repair. McComb or Tajima techniques: transcutaneous or rim incision sutures to reshape the lower lateral cartilage. Goals: improve alar dome projection and symmetry, reposition alar base, lengthen columella. Does not preclude secondary rhinoplasty at skeletal maturity. Overcorrection to some degree is appropriate given expected relapse.
Bilateral Cleft Lip Repair
Unique Challenges
Prolabium is small, devoid of muscle, and has deficient vermilion. Premaxilla may be severely protruding. Columella is extremely short. No functioning orbicularis oris in the prolabium.
Technique
Millard bilateral repair: banked fork flaps for columella lengthening at a later stage. Mulliken single-stage repair: preferred modern approach. Prolabial flap provides skin for philtral reconstruction. Vermilion and muscle are derived entirely from lateral lip elements.
Muscle is repaired across midline beneath prolabial skin. Primary nasal repair with open tip approach. Columella lengthening through V-Y or modified techniques. NAM is particularly valuable in bilateral clefts for columella lengthening and premaxillary alignment.
<image>Side-by-side surgical planning illustration for unilateral cleft lip repair comparing the Millard rotation-advancement technique (left panel) and the Fisher anatomical subunit technique (right panel). Both panels show an anterior view of the cleft lip with key anatomical landmarks marked with numbered points: Cupid's bow peaks, columella base, alar base, and white roll at cleft margins. The Millard panel shows the rotation incision curving along the columella base on the medial element and the advancement flap on the lateral element with arrows indicating the direction of tissue movement. The Fisher panel shows the geometric planned incisions along the philtral subunit with the small triangular flap at the lip base for length correction. Final scar lines are shown in dotted lines on each panel.</image>
Postoperative Care
Arm restraints (No-No splints) to prevent wound disruption (debated necessity). Resume feeding immediately (breast or bottle). Wound care: gentle cleansing, antibiotic ointment. Suture removal at 5-7 days (or absorbable sutures).
Scar management: silicone tape or sheeting starting at 2-4 weeks, sun protection. Nasal conformer/retainer after primary rhinoplasty (6-12 weeks).
Complications
Wound dehiscence (rare, <1%). Vermilion notching (whistle deformity). Scar hypertrophy or widening. Lip length discrepancy (short lip on cleft side).
Orbicularis oris muscle deficiency (visible depression). Nasal asymmetry persistence. Need for secondary revision (majority of patients).
Long-Term Management
Alveolar bone grafting: 8-11 years of age (mixed dentition, before canine eruption). Secondary rhinoplasty: after skeletal maturity (age 16-18). Orthodontic treatment throughout growth. Orthognathic surgery if maxillary hypoplasia develops (Le Fort I advancement). Speech therapy as needed. Psychosocial support throughout childhood.
Clinical Pearls
Accurate preoperative marking is the most critical step; even 1 mm of error at the vermilion-cutaneous junction (white roll) produces a visible deformity. The Fisher repair has gained favor because it provides more predictable lip length reconstruction compared to the Millard technique. Always repair the orbicularis oris muscle as a separate layer; muscle continuity is essential for dynamic lip function and long-term aesthetics.
In bilateral clefts, the prolabium lacks muscle and has poor vermilion; resist the temptation to use prolabial vermilion, which produces a "dead" central lip — use lateral lip vermilion instead. Primary rhinoplasty at the time of lip repair has been shown to improve nasal symmetry without adverse effects on growth; it should be considered standard practice. Wide clefts benefit from NAM to reduce the surgical challenge, though long-term outcome advantages remain debated in the literature.
References
- Fisher DM. Unilateral cleft lip repair: an anatomical subunit approximation technique. Plast Reconstr Surg. 2005;116(1):61-71.
- Millard DR Jr. Cleft Craft: The Evolution of Its Surgery. Little, Brown; 1976.
- Mulliken JB. Repair of bilateral complete cleft lip and nasal deformity — state of the art. Cleft Palate Craniofac J. 2003;40(1):2-12.
- Sitzman TJ, Girotto JA, Marcus JR. Current surgical practices in cleft care: unilateral cleft lip repair. Plast Reconstr Surg. 2008;121(5):261e-270e.
- Grayson BH, Cutting CB. Presurgical nasoalveolar molding in primary correction of the nose, lip, and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J. 2001;38(3):193-198.
- McComb H. Primary correction of unilateral cleft lip nasal deformity: a 10-year review. Plast Reconstr Surg. 1985;75(6):791-799.
