Cleft lip with or without cleft palate occurs in 1/940 live births [1].

Cleft palate without cleft lip occurs in 1 in 1574 live births [1].

Native Americans have the highest incidence at 1/500 live births [2].

African Americans have the lowest incidence at 1/2500 live births [2].

Cleft lip occurs more often in males (on the left side), and cleft palate occurs more in females. This is more likely to occur on the left side as there is a delay in the rotation of the palatal process of the maxillae on the left side.

The incidence of isolated cleft palate is higher in females (3:2); 50% of isolated cleft palates are associated with a sequence or syndrome (e.g., Pierre Robin, Stickler, van der Woude, 22q deletion anomalies). Cleft lip with or without a cleft palate occurs with a 15% syndromic association [3].

The primary palate denotes the anatomy that is anterior to incisive foramen including the incisors, alveolus, and nasal spine. The secondary palate denotes all structures posterior to the incisive foramen including the soft/hard palates and the uvula.

Complete cleft lips extend into the nares and the alveolus.

Complete cleft palate involves the soft and hard palate and associated musculature.

A submucosal palatal cleft is an incomplete cleft resulting from failure of the submucosal levator muscle to fuse completely in the midline (patients may clinically have a bifid uvula, zona pellucida, or absent posterior nasal spine).

Factors associated with cleft include family history, maternal smoking and alcohol use, maternal zinc deficiency, advanced parental age, folate deficiency in the periconception period, exposure to alcohol, and certain medications (e.g., retinoids, corticosteroids, anticonvulsants including phenytoin and valproic acid).

The Veau system has been proposed for classification of palatal cleft. Group 1 includes soft palate only. Group 2 includes cleft of the soft and hard palate. Group 3 represents complete unilateral cleft lip and palate. Group 4 includes complete bilateral cleft lip and palate (Fig. 10.1).

The striped Y of Kernahan and Stark classification is a symbolic representation of the untreated cleft lip and palatal deformity with the incisive foramen as the dividing point. Right and left are distinguished for clefts of the lip, alveolus, and premaxilla.

Boxes 1 and 4 reflect clefts of the right and left lips.

Boxes 2 and 5 represent clefts of the right and left alveolus.

Boxes 3 and 6 represent clefts of the right and left premaxilla. The circle at the center reflects the incisive foramen.

Box 7 reflects an isolated hard palate cleft.

Box 8 represents a soft palate cleft.

Box 9 represents a submucous cleft. It does not describe the degree of cleft (e.g., complete or incomplete cleft lip) or its functional impact (e.g., presence of velopharyngeal insufficiency) (Fig. 10.2).

A team approach at specialized cleft centers is the standard of care for patients with clefts to provide coordinated and continuous care through the child’s growth. Members include surgeons (oral and maxillofacial, ENT, or plastic), pediatric dentists, orthodontists, speech pathologists, geneticists, audiologists, social workers, psychologists, and pediatricians.

Ultrasound can identify clefting as early as 13–14 weeks of gestation. The sensitivity in detecting CL ± CP prior to 18–20 weeks is much lower [4]. This allows for anticipatory guidance and referral to a cleft team for interdisciplinary management.

At birth, appropriate diagnostic testing includes echocardiogram and chromosomal array for isolated cleft palate patients in light of associated syndromes.

At birth, a lactation consultation and/or speech therapy consultation is indicated. Bottles are available with customized nipples (e.g., pigeon bottle, Haberman with a one-way valve and reservoir spaces, Dr. Brown’s cleft palate bottle) to reduce the child’s work in feeding. Additionally, a Mead Johnson bottle is compressible to aid in forward flow of fluids.

Try feeding promptly after birth. Keep child upright with frequent breaks. Feeds over 30–35 minutes will often fatigue the child and expend more calories than consumed.

Preoperative orthopedics – During the first few weeks of life, lip and/or nasal taping or naso-alveolar molding appliances may be utilized to prepare the labial-naso-alveolar complex for surgery with a regularly adjusted appliance that helps guide growth and improve tension-free lip closure. The goal is to approximate the segments to within 5 mm. The need for frequent adjustments increases the risks of irritation and ulceration which are drawbacks of this therapy. Latham proposed appliances with screw activation and pin retention for better control of molding. Risk of growth restriction and the need for anesthesia are detractors from this therapy, as well as the baby having to undergo additional procedures.

Lip adhesion procedure is a separate soft tissue closure used as part of a staged unilateral or bilateral cleft repair with the goal of narrowing the defect at the time of definitive closure. Normally carried out at 3–4 months of age. The major disadvantages is the need for an additional surgery with potential increase risk of scarring and questionable impact on the definitive repair.

Primary lip repair (cheilorrhaphy) is usually performed around 10 weeks of age to reduce risks of anesthesia (rule of 10’s). The timing of primary lip repair weighs the benefits of functional improvement (reestablishing muscular anatomy and function) with the risks of early surgery from anesthesia and growth limitation of surgical scarring.

“Rule of 10’s”: 10 weeks old, 10 pounds in weight, and 10 mg/dL of hemoglobin.

Audiology screening/ENT evaluation. Should be conducted before 6 months. Children with cleft palate have difficulty controlling middle ear pressure due to eustachian tube dysfunction as a result of abnormal insertions of the levator veli palatini and tensor veli palatini. With impaired ability to equalize middle ear pressure, infants with clefts usually have fluid in the middle ear space which can result in chronic otitis media, and conductive hearing loss. If left unmanaged, this can lead to permanent hearing loss. Treatment consists of myringotomy tubes or fluid evacuation.

Primary cleft palate repair (palatoplasty in one or multiple stages) is generally performed between 9 and 18 months and timed with speech development to avoid compensatory misarticulations. Staged-soft palate includes soft palate closure at 6–10 months followed by hard palate at 1–3 years. Early palate repair (before 9 months) increases the risk of maxillary growth restriction.

Correction of velopharyngeal insufficiency (including pharyngeal flap, sphincter pharyngoplasty, or mucosal augmentation procedures such as filler or fat grafting) is completed at 3–5 years following nasoendoscopy demonstrating poor adaptation of the soft palate to the posterior pharyngeal wall.

Nasolabial revision is delayed until completion of nasal growth with anticipation that a definitive rhinoplasty may still be required. It is preferable to delay at least until the time of alveolar bone grafting so that concomitant treatment can be performed.

Alveolar grafting is generally completed at 6–9 years, based on development of the dentition. Grafting is timed with formation of canine root (1/2 to 2/3 formation) to allow emergence into the cleft site. Some surgeons also time grafting to coincide with the development of the central or lateral incisor.

Early orthodontics may be needed in the mixed dentition stage with the goal of maintaining transverse dimension, encouraging maxillary growth, and/or preparing for alveolar bone grafting. A second phase of orthodontics is often required with the permanent dentition.

Orthognathic surgery (if required) is planned at 14 to 16 years of age in females, 16 to 18 years in males to address the maxillary hypoplasia, mandibular skeletal disharmony, and sleep-disordered breathing that is common in cleft patients.

Rhinoplasty is often delayed at least 6 to 12 months following maxillary surgery due to changes in septum and tip support with movement of the anterior nasal spine.

Cleft scar revision can be completed any time after 5 years of age but is ideally performed at the time of alveolar bone grafting.

Millard proposed the rotation advancement flap. The goal of the Millard repair is the development of a three-layered closure following excision of hypoplastic tissue at the cleft margins. Orbicularis oris muscle continuity is reestablished and incision lines are designed to fall within the natural philtral ridges of the lip to promote symmetry. The repair also allows for columellar lengthening. Bilateral repair is complicated by lack of quality tissue, a short columella, and upward rotation of the premaxillary segment. The goal of the bilateral repair is reconstruction of the nasal floor, orbicularis continuity, and the maxillary vestibule.

The reason the technique is called a “rotational-advancement” flap is because the non-cleft side is cut in such a way that the tissue rotates (A) to create a longer vertical width and the cleft side advances (B) horizontally. This allows the scar to be hidden in a normal philtral line. This adds tissue to the deficient non-cleft side of the lip and the cleft side of the nasal columella. The premise for this and all techniques include a three-layered closure (skin, orbicularis oris, and mucosa); the excision of hypoplastic tissue from the cleft margins; and re-approximation of anatomic structures.

This technique is often used for bilateral cleft lips. Similar to the Millard technique, in that the cleft side advances while the non-cleft side rotates.

In the case of a bilateral cleft, the technique is slightly modified. Both lateral sides are treated the same way, in that the design is the same and the muscles are dissected. The only difference is the prolabium, which is often proclined anteriorly. The prolabium is dissected with the following flap design seen in Fig. 10.4.

The difference lies in the treatment of the nose, as the nasal tissue is gently dissected to create a laxity that allows for reapproximation in a symmetrical position. With the incisions open after the initial dissection of the Millard technique, a pair of tenotomy scissors are used to dissect over the lower lateral cartilages (still beneath the skin, cartilages not exposed) to free this tissue from its abnormal insertions. After the nasal tissue is dissected, the muscle and skin are reapproximated with additional sutures placed in the area of the nasal floor to create symmetry between the nostrils.

A nasal bolster is often placed to maintain the nasal shape postoperatively.

A Z-plasty technique that some feel is best used in wider clefts or if more vertical repositioning of Cupid’s bow is needed. Proponents of this technique argue that the triangular design avoids wound contracture and lip length shortening seen with the rotation and advancement flap operation. A major disadvantage of this technique is the scarring that crosses the philtral column which may contribute to the asymmetry.

Tensor veli palatini – trigeminal nerve – tenses and depresses soft palate.

Levator veli palatini – pharyngeal nerve – elevates the palate.

Musculus uvulae – pharyngeal nerve – draws uvula upward and forward.

Palatoglossal – pharyngeal nerve – draws palate down and narrows pharynx.

Palatopharyngeal – pharyngeal nerve – draws palate down and narrows pharynx.

Cleft palate repair has two goals, namely, the watertight closure of the oronasal communication and the anatomic repair of the musculature within the soft palate, which is critical for normal creation of speech.

In the cleft patient, the tensor and levator veli palatini, as well as the palatoglossal and palatopharyngeal muscles, abnormally insert into the posterior hard palate in an AP orientation and have to be reoriented into their normal transverse alignment.

In this procedure, two full thickness flaps are created and mobilized with layered dissection of the soft palatal tissues. The anterior and posterior portion remain connected to periosteum to increase circulation. Having an anterior attachment can decrease the visualization for nasal mucosa closure, and there is usually an area laterally that is allowed to granulate in. This healing through secondary intention can increase pain and create scar formation that restricts growth. For these reasons, it has fallen out of favor.

The most commonly used procedure, two flaps based off of the greater palatine artery are raised, dissecting either two layers (hard palate – nasal and oral mucosa) or three layers (soft palate – nasal mucosa, muscle, and oral mucosa). Dissecting the muscle off the posterior edge of the soft palate allows closure to reapproximate a more physiologic orientation.

The Furlow Z-plasty was created to allow for lengthening of the hard palate by reorienting the muscles in a more physiologic reapproximation. One side is myomucosal and the other side is mucosal. This method is more technically difficult and has a higher rate of oronasal fistula formation.

Sometimes described as a modification of the von Langenbeck technique with anterior pedicle release, this technique involves the release of the muscles of the soft palate from the posterior edge of the hard palate and from the periosteum on the nasal side, allowing the creation of a more physiologic muscle sling and soft palate. There is an anterior two-layer closure and posterior three-layer closure, but still has an area that heals by secondary intention, which leads to severe growth restriction.

A major advantage is maximization of palatal length to decrease postoperative VPI.