Cleft Lip and Palate

Abstract

This chapter will review unilateral and bilateral cleft lip, and cleft palate, with an emphasis on surgical markings. The reader will be able to categorize the defect and identify appropriate preoperative workup, and propose surgical plans and postoperative protocols. The reader will also be able to address postoperative events including dehiscence, fistulas, and velopharyngeal dysfunction.

Keywords: cleft lip, cleft palate, cleft lip and pala

Six Key Points

• The most important initial assessment is the airway.

• Cleft repair occurs along a standardized timeline.

• It is critical to understand and be able to draw cleft markings.

• The protuberant premaxilla should be controlled.

• Small lip dehiscences can be repaired primarily, while larger ones are left and revised after a year.

• Operative intervention for velopharyngeal dysfunction depends on the pattern of posterior and lateral wall movement.

Questions

1. What is your initial assessment of a child with cleft lip and/or palate?

Prenatal ultrasound detects over 90% of cleft lips. Many centers now have multidisciplinary cleft team evaluation of all infants with cleft lip with or without cleft palate, and presurgical assessment of an infant with cleft lip with or without cleft palate is threefold: airway, associated anomalies, and feeding and nutrition.

The most important initial assessment is of the airway—either at birth, or in the context of an airway history if the patient is being seen in clinic. If the airway is unstable, it needs to be stabilized with positioning, nasopharyngeal tubes, intubation, tongue–lip adhesion, or tracheotomy.

If the airway is stable, the infant should be evaluated for other anomalies, specifically cardiac and renal anomalies.

Finally, the infant needs a nutritional assessment. Some infants with cleft lip have difficulty latching onto a breast, although a nursing mother can often help create a seal over the cleft lip. Infants with cleft palate will not be able to create an oronasal seal, and will require a special nipple to obtain adequate nutrition. A pigeon nipple has a firm surface to abut the cleft, and a flexible surface for the tongue. The nipple is fitted with a one-way valve, which allows the infant to control and obtain fluid. Failure to thrive is a risk in children with clefts, and should be monitored.

2. What do you tell the parents?

The parents should be informed of the possible associated anomalies and feeding strategies, as well as the timing of repair.

3. What is the timing of surgery?

With cleft lip, if there is an alveolar cleft with a deformity of the alveolar arch, a lip adhesion with maxillary orthopaedics is performed at 6 weeks of age, followed by a definitive repair at 6 months. A single-stage palatoplasty is performed at 10 to 18 months. For cleft lip and palate, timing of surgery is around 10 weeks for the lip, and 10 to 18 months for the palate (Fig. 21.1, Table 21.1).

4. The family wants to wait until after the child is 18 months of age to repair the palate. What do you tell them?

The reason that cleft palate repair is performed before 18 months of age is because if the child cannot properly pronounce words, both speech and language acquisition will become disordered.

5. How do you control the premaxilla in bilateral cleft lip?

A protuberant premaxilla will need to be controlled. One can use preoperative orthodontics with passive presurgical molding or active molding, or bilateral lip adhesion. Passive molding is performed with nasoalveolar molding, which aligns the premaxilla with alveolar segments in bilateral clefts and aligns the alveolar segments in unilateral clefts. In addition, nasoalveolar molding uses nasal stents to mold the columella and nasal cartilage.

Fig. 21.1 Timeline of cleft care.

Table 21.1 Diagnosis, procedure associated with the diagnosis, and timing of the intervention.

Diagnosis	Procedure	Timing
Cleft lip and/or palate	Airway, associated anomalies, nutrition assessment	Birth
Alveolar cleft	Lip adhesion	6 wk
Cleft lip	Definitive cheiloplasty	6 mo
Cleft palate	Definitive cleft repair	10–18 mo

A Latham appliance is a device that is surgically inserted with pins, and is used to align the alveolus to make surgical repair easier. It is used for approximately 6 to 8 weeks.

6. When and how do you do a bilateral lip adhesion?

A bilateral lip adhesion can be performed at approximately 6 weeks of age. Bilateral lip adhesion is performed by raising 4 × 2 mm flaps bilaterally and suturing them together.

7. What is a maneuver you can use if the lip adhesion is tight?

You can put gentle pressure with your thumb on the segment to help reduce it. The risk with this maneuver is an infracture.

8. Draw the repair of a unilateral cleft lip.

The drawing can be made in the following steps (Fig. 21.2):

• Step 1. Mark the bilateral commissures, Cupid’s bow peak on the noncleft side at the vermillion cutaneous border and its mirror image, along with its match on the lateral lip element. The base of the nostrils on the medial and lateral edge, the subnasale, and the midpoint of the Cupid’s bow are also marked.

• Step 2. Once the landmarks have been marked, draw the rotation flap on the medial lip element. This is drawn from the mirror image of Cupid’s bow peak arching toward the midpoint of the columella, and then making a sharp downward turn at the midpoint of the columella.

• Step 3. The advancement flap is on the lateral lip element by drawing a line from Cupid’s bow peak match, along the cleft, and turning at the nasal sill.

• Step 4. The columellar flap is then made from Cupid’s bow peak on the medial cleft element to the lateral columella.

• Step 5. The mucosal incisions are marked as extensions of the rotation and advancement flaps.

9. Draw the repair of a bilateral cleft lip.

The drawing is as in Fig. 21.3.

10. Draw the repair of a cleft palate.

The drawing is as in Fig. 21.4.

11. How do you manage the nose?

The degree of nasal deformity is assessed preoperatively. The length of the columella on each side is assessed, as the columella may be shorter on the cleft side. The cleft-side dome is flattened, with an obtuse angle, and the medial crus is shorter. The alar base is posterior and inferior. It is important to note that the alar cartilage is not simply malpositioned; it is also more atrophic.

The nose is exposed through the dissection. The alar base incision is extended to the inferior turbinates, and the alar cartilage is released from its lining and muscular attachments. The dissection continues to the noncleft side, and once the cartilage is free, the alar cartilage is shaped by placing polypropylene suture on Keith needles and Dacron pledgets through the dome to act as a handle. As this is lifted and the dome is shaped, a base stitch is placed through the orbicularis muscle, and an alar base suture is placed.¹