Cleft lip/palate (CL/P) is the most prevalent facial abnormality in the world. CL/P is more frequent in males with a 2:1 ratio, although isolated cleft lip is seen equally in both sexes. Left-sided cleft defects are more common than right-sided cleft defects, and the ratio is 9:4.5:1 in the order of unilateral left/unilateral right/bilateral. The overall incidence is around 1:750 and ranges from 0.2 to 2.3 per 1000 depending on race. Cleft lip/palate is significantly more common in patients of Asian and Native American descent (1 in 450) and is least common in Afro-Caribbeans (1 in 2000). The incidence among white individuals is 1:1000. An incidence of 1:350 is more in keeping with hypospadias rather than cleft lip/palate. Associated syndromes are seen in around 10% of patients with cleft lip/palate (not 33%).1–5
oris? The normal lip comprises a number of key anatomic components that are relevant not only to cleft lip, but more generally to the management of lip injuries and in the context of lip reconstruction. The lips comprise of mucosa, muscle, subcutaneous tissues, and skin. The orbicularis oris muscles form a concentric ring of muscle around the mouth and superiorly, in the midline, the superficial fibers insert into the dermis of the lip to create the paired philtral columns. The philtral dimple is the concavity between the columns created by a relative paucity of muscle fibers. In a cleft lip, the orbicularis muscle abnormally inserts into the nasal alar and the philtral column is abnormally formed. Reconstruction of the philtral column is one of the key aims of cleft lip repair. The white roll is the prominent ridge between the skin of the lip and the vermilion which is the red mucosal portion. The vermilion itself is divided into wet and dry components, with the wet being within the mouth (nonkeratinized) and the dry being externally located (keratinized). The wet and dry vermilion are separated by the red line. Cupid’s bow is the curvature of the central white roll and has two lateral peaks. There is fullness to the vermilion at the central inferior apex of Cupid’s bow called the tubercle.1–3
3. Millard DR. The Unilateral Deformity. Vol. 1. Boston, MA: Little Brown; 1976 A cleft lip results in the projection and outward rotation of the premaxilla, with retropositioning of the lateral maxillary segment. The vertical lip height is reduced, and the philtrum is short. The vermilion width is decreased on the medial side of the cleft and increased laterally. The superficial part of orbicularis oris inserts into the alar base on the cleft side. The deep portion is interrupted but does not abnormally insert. Neither the prolabium in a bilateral cleft lip nor the Simonart’s band contains muscle. The continuity of muscle is disrupted in a microform cleft.1–4
4. Marcus JR, Allori AC, Santiago PE. Principles of cleft lip repair: conventions, commonalities, and controversies. Plast Reconstr Surg 2017;139(3):764e–780e The cleft nasal deformity comprises a collection of specific abnormalities. They can be remembered by considering anatomical subgroups as shown in Box 29.1 BOX 29.1 ANATOMIC CHARACTERISTICS OF CLEFT NASAL DEFORMITY Cartilaginous Deformities The caudal septum is deviated to the noncleft side. The posterior septum is convex on the cleft side and can block the cleft-side airway. Lower lateral cartilages are attenuated and displaced caudally on the cleft side. The medial crura are separated from one another at the dome. The angle between medial and lateral crura is more obtuse on the cleft side. Soft Tissue and Bone The nasal tip and columellar base deviate to the noncleft side. The cleft-side alar base is displaced laterally, posteriorly, and caudally. The maxilla is hypoplastic with a posteriorly displaced piriform margin on the cleft side. The nostril sill is deficient, and the nostril is widened on the cleft side. The cleft alar-facial angle is flattened. The vestibular lining is deficient on the cleft side. The columella is shorter on the cleft side. Flattening of the alar facial angle is a key finding on the cleft side. There is a more obtuse angle between the medial and lateral crura (not acute). There is widening of the cleft nostril with a deficiency of the sill. The nasal tip deviates away from (not toward) the cleft side. Columellar length is reduced on the cleft side (Fig. 29.1).1–3 Fig. 29.1 Unilateral cleft nasal deformity. A, Nasal tip deviated. B, Alar cartilage displaced caudally. C, Angle between medial and lateral crura more obtuse. D, Buckling in the lateral crura. E, Flattened alar facial angle. F, Widened nostril floor. G, Columella and anterior caudal septal border deviated. Not shown: Deficiency in bony development, and posterior septum convex on cleft side causing varying degrees of obstruction. (Adapted from Spira M, Hardy SB, Gerow FJ. Correction of nasal deformities accompanying unilateral cleft lip. Cleft Palate J 7:112, 1970.) E D C B F A G
If one parent is affected, the risk of cleft lip/palate in the first child is 3–5%. If an affected parent already has an affected child the risk increases to 17% for the second child. If unaffected parents have one affected child, the risk of having subsequent affected children is 4%. When unaffected parents have two affected children, the risk of having other affected children is 9%. It is important to have an idea of relative risk for cleft lip and palate development as patients and families are likely to ask about this during consultations (Table 29.1).1–3 Table 29.1 Risk of Familial Recurrence in Cleft Lip with or without Cleft Palate Risk of Familial Recurrence Percent One affected parent 3–5 One affected child Two affected children Affected parent and affected child Monozygotic twins 40–50 Dizygotic twins Affected niece or nephew Affected cousin 0.5
The best method of feeding a baby with an isolated cleft lip is either directly from the breast or with a regular bottle. The need for specialized bottles or nipples is only for infants with a cleft palate. Three types of bottles used to feed cleft palate infants include: the Mead-Johnson cleft palate nurser, the Haberman feeder, and the Pigeon nipple. The Mead-Johnson device is a soft squeezable bottle with a long cross-cut nipple. The Haberman feeder has a one-way valve and does not require a squeezable bottle. The Pigeon nipple works by compression and can be used with normal or squeezable bottles and has a faster flow than other feeding systems.1
Presurgical approaches are used in cleft patients to help align and approximate maxillary segments, correct malposition of the nasal cartilages, and elongate the columella prior to definitive lip or palate repair. Lip adhesion is a surgical procedure used for wide cleft lips in which preliminary closure of the lip is performed around 2 months of age. The principal aim of this technique is to convert a complete cleft into an incomplete one with the hope that later definitive closure is facilitated and that the lip and nasal deformity are reduced by molding of the maxillary segments. The downsides are that an additional operation is required within the first 2 months of life and it risks damage to the tissues required for later definitive repair. To minimize this, the incisions are placed within tissue that will be discarded at final repair. In this procedure, the lateral lip and alar base are released from their attachments to the maxilla. A retention suture is placed to encircle the orbicularis muscle and a silicone nasal conformer is placed for columellar lengthening and to reshape the lower lateral cartilages. Presurgical orthopedic devices may be used alternatively to narrow the cleft margin and improve arch alignment before surgery. They can be either active or passive. The Latham device is an active appliance that is placed and secured with pins while the patient is under general anesthetic. Screw activation will expand the plate and retract the premaxilla, but this process can adversely affect maxillary growth and should be reserved for severe deformities. An additional anesthetic will be required for its removal. Nasoalveolar molding (NAM) is a nonsurgical way to reshape the gums, lip, and nostrils with a plastic plate before cleft lip and palate surgery. Both Latham and NAM treatment will require multiple outpatient clinic visits which are usually weekly or biweekly for monitoring and adjustment and hence will not decrease the outpatient burden.1–5
Primary repair of a cleft lip is performed around the age of 3 months. During this surgery the primary cleft nasal repair and gingivoperiosteoplasty are usually performed. A nasal repair involves release and repositioning of the cleft nasal components and alar cartilages (tip rhinoplasty). Tajima or McComb sutures may be used to support the lower lateral cartilage in primary cleft rhinoplasty. A gingivoperiosteoplasty involves the primary closure of an alveolar cleft using mucoperiosteal flaps. Palatoplasty is typically performed at 9–12 months of age. Alveolar bone grafting involves harvesting cancellous bone from the iliac crest and is most successful between the ages of 8 and 12 years because this coincides with eruption of the canine teeth. Orthognathic surgery and secondary or full rhinoplasty are usually performed once facial growth and secondary dentition is complete (Table 29.2).1,2 Table 29.2 Timings for Cleft Lip Repair and Associated Procedures Procedure Age Presurgical orthopedics Cleft lip repair 1 week–3 months 3–6 months Tip rhinoplasty Tympanostomy tubes Palatoplasty 9–18 months T-tube placement Speech evaluation 3–4 years Velopharyngeal insufficiency workup and surgery 4–6 years Tip rhinoplasty Phase I orthodontics Alveolar bone grafting 6–9 years 6–9 years 6–9 years Nasal reconstruction Phase II orthodontics 12–18 years 14–18 years Orthognathic surgery Final touchup surgery Completion of mandibular growth (14–18 years) Early adulthood
A number of different techniques are available for cleft lip repair. The Millard repair is one of the most commonly used techniques and involves a superiorly placed Z-plasty. This is the one that is traditionally learned for clinical examinations in plastic surgery. The Randall-Tennison repair also uses a Z-plasty, but at the vermilion border, and can result in excessive vertical lip height. LeMesurier modified a quadrangular flap repair first described by Hagedorn. Techniques such as the Rose-Thompson technique involve a straight line and are no longer commonly used because they have been replaced with techniques involving Z-plasties to improve lip height. Bilateral cleft lip can be repaired using Millard or modified Manchester techniques. In the Millard technique the central skin of the prolabium is used to reconstruct the philtral dimple, but the white roll and vermilion are discarded. The modified Manchester technique preserves the white roll and vermilion from the prolabium to reconstruct Cupid’s bow and tubercle.1–9
The markings and steps of the Millard rotation-advancement repair are shown in Fig. 29.2, A through F. The key flaps used in this repair are as follows: The C flap is known as the columellar flap and is a triangle of skin that begins inferiorly at the high point of Cupid’s bow. It is used to close the nasal sill or lengthen the columella. The L flap is known as the lateral flap and is a superiorly based flap from the lateral lip that is used to line the lateral nasal vestibule. The M flap is known as the medial or mucosal flap and is a rectangular flap of mucosa from the medial lip. It is used to line the gingivobuccal sulcus. The R flap is the rotation flap based on the noncleft side (medial) lip that rotates into place during cleft lip repair to join with the cleft side lip (lateral). The A flap is the advancement flap based on the cleft side lip. It advances to join the R flap from the noncleft side.1,2
The Noordhoff flap is a triangular flap of dry vermilion taken from the lateral lip element and rotated into an incision along the wet–dry junction of the medial lip element to even out heights of the medial and lateral labial elements and help to prevent notching and development of a whistle deformity. It is incorporated into both the Millard and Fisher techniques. The Millard repair has been the most popular approach to repair of cleft lips for a number of years. It involves rotation of the medial lip element downward and fills the resulting defect with the lateral lip. In doing so it places the scar along the proposed philtral column. Although it is well recognized and is associated with high-quality outcomes, some criticisms exist including the technical difficulty and challenges in dealing with wide clefts, wide soft tissue undermining, and tension across the nasal sill following closure. The Fisher subunit repair is a geometric style technique which serves to address two perceived shortcomings in the rotation-advancement techniques. One issue is the complex scars created beneath the nose which may disrupt continuity of the columellar-labial crease and make future open rhinoplasty difficult. The other is the horizontal length of the lateral lip which is often shortened due to the Noordhoff point lying too laterally. In order to address these, the Fisher repair places the scars in the interfaces of anatomic subunits and incorporates a cutaneous triangle above the white roll to adjust the height of the medial labial element. The key differences in rotation- advancement (Millard) versus geometric style (Fisher) are therefore mostly related to the skin incisions and effect on symmetry. Both techniques allow for correction of mucosal, gingival, muscular, and nasal deformities. A Tajima suture is a type of suture used in primary cleft rhinoplasty. The suture is placed through the caudal cleft side of the lower lateral cartilage to the contralateral upper lateral cartilage to provide support to the lower lateral cartilage. It is not specifically part of either the Fisher or Millard lip repair techniques.1–3
The whistling or whistler deformity is normally secondary to a deficiency in the central part of the vermilion often in conjunction with abnormal orbicularis alignment. A B C Fig. 29.2 A, Flaps are marked using the alar base, columella, lip margins, philtral columns, and Cupid’s bow. B and C, The R, C, M, L, and A flaps are incised and elevated. D, The M and L flaps are sutured into place. E and F, Rotation-advancement is completed with interposition of C flap above A flap. D E F Various methods have been described for the surgical management of this condition and technique selection will depend on the precise nature and extent of the deformity. For example, if the deficiency is mild, the upper lip itself can be used for reconstruction, such as Z-plasty, V-Y advancement, double pendulum flaps, bilateral lateral vermilion border transposition flaps, and bilobed mucosal flaps. If the deformity is more severe then adjacent tissue such as the tongue and lower lip (Abbe flap) may be required. More novel approaches include fat grafting, palmaris longus tendon grafting, and dermofat grafts.1