History of the ProcedureChinese physicians were the first to describe the technique of repairing cleft lip. The early techniques involved simply excising the cleft margins and suturing the segments together. The evolution of surgical techniques during the mid-17th century resulted in the use of local flaps for cleft lip repair. These early descriptions of local flaps for the treatment of cleft lip form the foundation of surgical principles used today
Tennison introduced the triangular flap technique of unilateral cleft liprepair, which preserved the Cupids bow in 1952. The geometry of thetriangular flap was described by Randall, who popularized this method of liprepair. Millard described the technique of rotating the medial segment andadvancing the lateral flap; thus, preserving the Cupids bow with thephiltrum. This technique has resulted in improved outcomes in cleft liprepair
Epidemiology and geneticsA. Incidence of cleft lip and of cleft lip and palate1. The overall incidence is 1 in 1,000 live births.2. White ancestry: 1 in 750 live births.3. Asian ancestry: 1 in 500 live births.4. African ancestry: 1 in 2,000 live births.
B. Demographics1. Male-to-female ratio of 2:1.2. The ratio of left (L) to right (R) to bilateral (B) clefts (L:R:B): 6:3:1.3. The ratio of CLP to CL is 2:1.4. Three percent are syndromic.
5. Risk factorsa. Medications:a.Phenytoin, methylprednisolone (Solu-Medrol), steroids, phenobarbital, diazepam, and isotretinoin.b. Smoking.c. Parental age, especially fathers age, or both mother and father over 30years old.
d. Family history.If parents have one child with CLP: 4%.If parents have two children with CLP: 9%.If one child and one parent have CLP: 14% to 17%. N.B. Most cases are sporadic (and multifactorial), but may be X-linked, autosomaldominant (Van der Woudes syndrome) or familial
AnatomyA. Normal lip anatomy1. Topographic landmarksa. Nasal alae.b. Columella.c. Philtral columns.d. White roll: Well-defined mucocutaneous or vermilion-cutaneous border.e. Vermilion: Red portion of lip.f. Tubercle.g. Cupids bow.h. Wet-dry border: The vermilion-mucosa junction is the border between keratinized and nonkeratinized mucosa.
2. Musculature.a. Orbicularis oris.(1) Fibers cross (decussate) in the midline and create the opposite philtral columns.(2) Functions as a sphincter (deep fibers) and for speech (superficial fibers).b. Levator labii superioris.(1) Inserts into the dermis at the vermilion border and the lower edge of the philtral columns.(2) Elevates the upper lip.c. Nasalis or depressor septi nasi muscle: The fibers run from the alveolar bone into the medial crural footplates, skin of the columella and the tip of the nose, and into the opposite philtral columns.
3. Normal measurements.a. Vertical length (height) of the upper lip. (1) Newborn: 10 mm. (2) Age 3 months: 13 mm. (3) Adult: 17 mm.b. The distance between the peaks of Cupids bow: Approximately 3 mm at 3 months.4. Arterial blood supply: The labial artery, bilaterally.5. Sensory innervation: The trigeminal nerve, cranial nerve (CN) V, maxillary division (V2).6. Motor innervation: The facial nerve, CN VII, zygomatic and buccal branches.
B. Cleft lip anatomy.1. Alterations in the orbicularis oris, levator labii, and nasalis result in disruption of continuity, orientation, and quality of the muscles.a. Fibers are disoriented and run parallel to the cleft margin.b. Fibers insert into the alar base on the cleft (lateral) segment and into the columella in the noncleft (medial) segment, as well as intradermally.c. Incomplete clefts.(1) Simonarts band consists of a skin bridge across the nasal sill. It does not usually contain any significant muscle mass.(2) Some fibers may cross the cleft, if the cleft is less than two-thirds of lip height.
d. Bilateral complete clefts: No muscle tissue is present in the prolabium.2. Vertical lip length is decreased: Cupids bow and the lip are rotated on both thelateral, cleft side as well as the medial side.3. Disrupted Cupids bow.4. The alveolus and nostril floor are open in a complete cleft lip.5. The premaxilla is rotated and protruding, especially in bilateral cleft lip, often withcollapse of the lateral segment of the cleft side(s).
6. Associated cleft lip nasal abnormalities .a. Hypoplastic, flattened alar dome on the affected side.b. Lack of upper lateral cartilage overlap of lower lateral cartilage.c. Subluxed lower lateral cartilage with alar base displaced posteriorly.d. Hypoplastic bony foundation (maxilla).e . Flattening of the nasal bones.f . Shortened columella, especially in bilateral cases.
ClassificationA. Extent of the cleft: Complete versus incompleteComplete cleft lip .1a. Complete disruption of the soft tissues to the nasal floor.b. Tends to be wider than incomplete clefts, with greaternasal deformities.
2. Incomplete cleft lipa. Disruption of the soft tissues to varying degrees.b. The alveolus is usually intact, with less of a tendency for thepremaxilla to protrude.c. Forme fruste: A very mild cleft.(1) May be difficult to detect.(2) May appear as vermilion notching or a scarlike line ordepression.
B. Location of the cleft: Unilateral versus bilateral1. Unilateral cleft lip2. Bilateral cleft lipa. May have a complete or incomplete cleft on both sides, or a combination.b. More likely to be complete clefts and are often wide.c. In bilateral complete clefts, the prolabium lacks muscle tissue, andtherefore lacks philtral columns.C. Alveolar segments1. Narrow versus wide cleft2. Collapse versus no collapse
Staging of interventionA. Initial evaluation1. Reassure the parents and family that they are not to blame.2. Explain the stages and operations that should be expected throughout the childs lifetime.3. Evaluate for associated anomalies.4. Consultations a. Genetics, for evaluation and possible counseling b. Social work
c. Feeding/nutrition (1) The child may need special nipples or bottles (e.g., cross-cut nipple). (2) Monitor for appropriate weight gain. d. Otolaryngology: Children with cleft lip and palate have a high incidence ofeustachian tube dysfunction, and otitis media, requiring close follow-up. (1) The child may need myringotomy tubes. (2) If untreated, repeat otitis may affect hearing and speech development.
B. Wide clefts (>1 cm)1. Goal: Bring the segments closer together to facilitate a tension-free repair.a. Has not been shown to change skeletal development in the anteroposterior direction.2. Passive: Preoperative tapinga. Steri-Strip tapes applied across both segments of the lip.b. Requires reliable parents who can reapply the tape and keep it on .
3. Passive: Lip adhesion operationa. Suturing the edges of the cleft together is performed underanesthesia.b. The lip repair is performed once the segments have moved closertogether.c. Variable success.
C. Repair1. Timing (controversial)a. Repair at 3 months is generally accepted.b. Some argue for earlier repair in order to produce better scars.2. Rule of tens: For increased anesthetic safety, an infant shoulda. Be 10 weeks old.b. Weigh 10 pounds.c. Have a hemoglobin level of at least 10 mg/dL.
3. Cleft palate repair and secondary alveolar grafting .4. May also choose to address the cleft nasal deformity at time of liprepair
Goals of repairThe basic goal of primary cleft lip repair is toreconstitute oral competence and a dynamic muscularsphincter with the orbicularis oris muscle. Equallyimportant is the achievement of cosmeticreconstruction of the lip appearance. The focus is on1)correct alignment of Cupid’s bow.2)symmetric reconstruction of the vermilion.3)and accurate construction of the philtral column.
Additionally, the goals of primary cleft rhinoplastyperformed at the time of initial lip repair are important toachieve.1)nasal function is optimized by closing the nasal floorand nostril sill. Establishing a correct position for the alarbase is beneficial in overcoming the nasal asymmetry.2)improve the position and contour of the lower lateralcartilages. This enhances nasal aesthetics in the short termand sets the stage for future nasal reconstruction. Ideally these goals are achieved without causing excessivescar tissue formation, wound breakdown, or restriction ofmaxillofacial growth.
Types of repairA. Straight-line repair 1. Historically, the first cleft lip repairs relied on freshening the edges of the cleft and suturing them together. These have been largely replaced by various Z-plasty-based techniques. 2. Rose-Thompson repaira. Modified straight-line repair that can be used for minor clefts with lip length nearly equal on both sides of cleft (e.g., forme fruste).b. Fusiform excision with straight-line closure.
B. Quadrangular flap 1. Proposed by LeMesurier and Hagedorn. 2. Cupids bow is derived from the lateral lip. 3. 90-degree Z-plasty. 4. Violates Cupids bow and philtral dimple. 5. Has a tendency to produce a long lip.
C. Triangular flapThe triangular flap repair was initially described in 1952 by Tennison, In 1959, Randall described a mathematical approach to the triangular flap that was on the basis of precise measurements.This repair technique is conceptually similar to the rotation advancement repair. The primary difference is that the rotation back- cut in the noncleft segment is performed more inferiorly, closer to the vermilion border. Similarly the advancement segment on the cleft side is designed to occur inferiorly near the vermilion cutaneous border.
Markings for unilateral cleft lip repair with the triangular flap technique. Marked reference points indicate the following:1. Columellar base noncleft side2. Columellar base cleft side3. Alar base noncleft side4. Alar base cleft side5. Light scroll mark noncleft side6. Light scroll mark cleft side. This also becomes the medial base of the equilateral triangle flap7. Peak of Cupid’s bow noncleft side.8. Low point of Cupid’s bow .9. Apex of the equilateral triangle flap10. Lateral base of equilateral triangle flap .11. Marks the length of back cut in noncleft side.
There are 3 main advantages of the triangular flaprepair technique.First, it is readily used to close wide clefts without having to perform lipadhesion or presurgical tissue manipulation.Second, the operation is done on strictly geometric methods ofmathematical principles and measurements, leaving not much room forerrors in judgment when compared to the “cut as you go” techniques.Therefore, many experts consider the triangular flap techniqueto be well suited for less experienced surgeons.A third possible advantage of this technique is that the zigzag scar preventsscar contracture and lip shortening leading to a vermilion notch that canbe sometimes observed in the rotation advancement technique.
The main disadvantage of the triangular flaprepair technique is that the philtrum on the cleft side is violated by the triangular flap. Some authors believe this leaves a more noticeable scar.Another potential disadvantage is the difficulty in modifying the repair or performing secondary revision at a later stage due to the zigzag scars.
2. Skoog repaira. Consists of two Z- plasties.b. Violates Cupids bow and the philtral dimple.
D. Rotation advancement.The rotation advancement repair of the unilateral cleft lipdeformity as described by Millard is the most commonlyused method of repair at present in the USA.The main advantage of this technique is its flexibility and application. The rotation advancement technique relies on a “cut as you go” strategy that allows continuous modifications during the design and execution of the repair. It does not adhere to strict geometrical principles or measurements.Another advantage is that the suture line approximates a new philtral column. The aesthetic philtral subunit is not violated, and this tends to create a scar that is more camouflaged.Minimal tissue is discarded during the rotation advancement technique, and this tends to put less tension on the closure.
Furthermore, the rotation advancement technique allows easy access to the alarcartilages for primary rhinoplasty to be performed at the time of lip repair. Thisearly repair of the nasal deformity can be successful in achieving a moresymmetric nasal appearance and possibly avoiding the intermediate rhinoplastystep for many of these children.The primary disadvantage of the rotation advancement technique is thatexperience and artistry are required to achieve optimal results. The operationrelies on the surgeon’s spatial awareness and judgment.Additionally, the vertical scar that occupies the philtral column can be subject towound contracture. Such contracture can lead to shortening of the lip on the cleftside with resultant vermilion notching and whistle deformity.Finally the surgeon needs to be cautious when using the rotation advancementtechnique to avoid excessive narrowing of the nostril sill on the cleft side. This canlead to nasal vestibular stenosis as the wound matures.
Markings for unilateral cleft lip repair with the rotation advancement technique.1. Center (low point) of Cupid’s bow2. Peak of Cupid’s bow lateral, noncleft side3. Peak of Cupid’s bow, medial, noncleft side4. Alar base, noncleft side.5. Columellar base noncleft side; X. Back cut point, noncleft side.6. Oral c ommissure noncleft side.7. Oral commissure cleft side; 8. Light scroll, cleft side.9. Medial tip of advancement flap, cleft side.10.Midpoint of alar base cleft side.11. Lateral alar base, cleft side.12.Lateral alar base, extent of alotomy.
E. Bilateral cleft lip repair1. The premaxillary segment is often agreater problem than in a unilateral cleftlip.2. Consider taping, lip adhesion, orpresurgical orthodontics (see above).3. Most common techniquesa. Dissect the prolabium to maintain acentral skin flap to resemble thephiltrum.b. Deepithelialize the remainder of theprolabium.
c. Use the prolabial vermilion to create a labial sulcus, not for the final lip vermilion. The final lip vermilion is composed only of vermilion from the lateral lip segments, not from the prolabium. d. Columellar lengthening may be performed at the time of lip repair or as a secondary procedure.
Postoperative careA. Orders 1. Arm restraints for 3 weeks to prevent disruption ofrepair. 2. Specialized nipple/bottle to decrease sucking effortwhen bottle-feeding. 3. Breast-feeding is controversial; based on surgeonpreference.B. Leave Steri-Strips in place over the incision forreinforcement.C. Follow up in 1 week for suture removal if nonabsorbableskin sutures were used.
Complications of cleft lip repair1) inadequate reapproximation of the orbicularis oris muscle with a failure to reconstitute a competent oral sphincter. This can result in a visible muscle bulge that is readily apparent under the skin of the repaired lip on dynamic motion.2) inaccurate alignment of the vermilion-cutaneous junction leaving a small step-off deformity that is readily noticeable even to the untrained eye.
3) vertical scar contracture or inadequate rotation can cause shortening of the lip segment leading to a notch in the vermilion and a whistle deformity.4) scar contracture causing a narrow nostril sill with vestibular stenosis.5) wound healing complications such as dehiscence and scar widening