Summary
Clefts of the lip and palate require medical support from birth to adulthood. Attention should be given to multiple concerns such as breathing, feeding, speech, hearing, aesthetic, and orthodontic issues, as well as caregivers’ burden, anxiety, and stress. All of these issues can be properly addressed by using a multidisciplinary team.
Clefts can be minor with little affect or can be complete bilateral or with additional craniofacial deformities that might require early and prolonged medical attention.
Clefts of the primary palate have mainly aesthetic, dental, and orthodontic consequences.
Clefts of the secondary palate have mainly feeding, speech, ears, and orthodontic consequences.
Even though for some cleft patients the road is long and sometimes bumpy until completion of medical treatment, proper and dedicated treatment should bring the great majority of patients to normal aesthetic and function.
39 Cleft Lip and Palate
39.1 Introduction
Clefts of the lip and palate result from incomplete closure process of the palate and lip components between weeks 7 and 10 of pregnancy. The palate is divided according to embryological origins to primary palate (originating from the nasofrontal process) that includes the prolabium (premaxilla and median upper lip) and the secondary palate (originating from the maxillary shelves) that includes the palate posterior to the foramen incisivum, both hard and soft. During embryonic development the primary palate starts to fuse with the maxillary shelves from the foramen incisivum forward. Secondary palate is created by midline fusion of the bilateral maxillary shelves from the foramen incisivum backward. If fusion process of the primary and/or secondary palates is interrupted, complete or incomplete clefts occur.
Clefts of the primary palate (include lip) have mainly cosmetic, dental, and orthodontic concerns. Cleft of the secondary palate have mainly speech, feeding, swallowing, ears, and sometimes orthodontic concerns.
Treatment of cleft patient starts at birth and usually continues to late adolescence. The patients and caregivers are guided and supported through this prolong process by a dedicated multidisciplinary team. The team includes medical and paramedical members: experts in otolaryngology, maxillofacial surgery, plastic surgery, orthodontic, pediatric dentistry, genetics, speech and language pathology, occupational therapy, pediatric dietitian, audiology, nurse, social worker, and patients and team coordinator.
Primary and secondary clefts require different surgeries and medical care. General timeline for potential surgeries and medical care:
1 week of age: Presurgical orthodontic—Nasoalveolar molding (NAM) adjust and fixation (primary palate).
10 to 12 weeks: Cleft lip repair (primary palate).
10 to 12 months: Cleft palate repair and myringotomy tubes placement (secondary with/without primary palate).
2 to 6 years: Second set of myringotomy tubes in about 50% of secondary cleft palate patients.
4 years: Pharyngeal flap (in case of velopharyngeal insufficiency) (secondary palate).
7 to 8 years: Orthodontics (primary and/or secondary palate).
9 to 10 years: Alveolar ridge bone graft (primary palate).
17 years: Rhinoplasty for cleft lip nasal deformity (CLND) (primary palate).
Early adulthood: Orthognathic surgery—bimaxillary advancement (mainly secondary palate).
39.2 Unilateral Cleft Lip Repair
39.2.1 Introduction
Cleft lip comprises a range of deformities:
Microform: Slight depression in the vermilion or the column of the philtrum.
Incomplete cleft lip: A cleft of the lip tissue—vermilion and skin, but not including the floor of the nose.
Complete cleft lip: A cleft of the whole lip and anterior nasal floor. The cleft includes all layers of the lip—skin, muscle, and mucosa.
Closure addresses three main issues: lip cosmesis, orbicularis oris muscle continuity and function, and nasal deformity (cleft lip nasal deformity—CLND).
The cleft gap is closed by advancing and rotating adjacent tissues.
39.2.2 Preoperative Evaluation and Anesthesia
Soon after birth and prior to surgery, presurgical orthopedics is applied. Nasoalveolar molding (NAM) device is customized to the patient in order to improve nostril symmetry, as well as premaxilla and maxillary shelf position.
Repair usually takes place at 3 to 4 months of age. The patient should be evaluated by the pediatric anesthesiologist to ensure that the infant gained enough weight and is generally healthy in order to make the anesthesia and surgery at optimal safety, taking into consideration that the surgery is elective and mainly cosmetic.
39.2.3 Surgical Technique
Surgery is done under general inhalational anesthesia with an oral endotracheal RAE tube secured to the mandible but without distorting the lower lip. A shoulder roll is placed under the supine patient for a Rose position. The surgeon is positioned in front of the head of the bed, either sitting or standing.
Markings
The upper lip landmarks are carefully marked with an extra-fine marker and calipers.
The Millard rotation-advancement technique is widely used and is depicted in ▶ Fig. 39.1:
Point 1: Non-cleft side (NCS) commissure.
Point 2: NCS Cupid’s bow peak.
Point 3: Cupid’s bow nadir (midline).
Point 4: Cleft-side (CS) Cupid’s bow medial peak.
Distance 2 to 3 equals 3 to 4.
Point 5: CS base of columella.
Point 6: NCS base of columella.
Point 7: CS alar base.
Point 8: CS Cupid’s bow lateral peak.
Point 9: CS commissure.
Distance 1 to 2 equals 8 to 9.
Point 10: Base of rotation flap should not cross midline of columella.
Length of curved line 4 to 10 equals 7 to 8.
At points 4 and 8 the white roll usually attenuates and the vermilion is starting to narrow superiorly.
After marking the landmarks and the curved 4 to 10 rotation flap line on the NCS, lidocaine–adrenaline mixture is injected to the lip slowly in order not to get the tissue too swollen and distorted, and is rubbed in. This should reduce bleeding allowing clear and accurate incisions.
Incisions
Incisions are done as described in ▶ Fig. 39.2:
From point 4 vertically in the vermilion up to red line of wet mucosa.
From point 4 along the vermilion border up to its insertion to the columella at point 5.
From point 4 along the curved line up to point 10; a small back cut can be made if necessary.
From point 8 vertically in the vermilion.
From point 8 along the vermilion border up to its insertion to the ala nasi at point 7.
Short horizontal incision from point 7 laterally and curving around the ala nasi base to create advancement flap (marked green). Length of incision as required for tension free release of CS lip.
The vermilion mucosa is peeled posteriorly. Later excessive mucosa can be discarded ▶ Fig. 39.3.
The distorted edges of the orbicularis oris muscle are exposed and then its insertion to the columellar base and ala nasi base should be released ▶ Fig. 39.4. By holding the edge with a forceps or a hook and pooling it to its appropriate place, it is easy to appreciate if the muscle was released enough from the base of the nose. A good bulk of muscle should easily fill the vermilion without being tethered to the nasal base. Excessive or hypotrophic edges of the muscle can be cut and discarded to get fresh and bulky muscle edges. Free edges can be created by undermining the muscle from the mucosa. Small horizontal incisions can be done in order to create muscle digits that will be later used for interdigitation suturing. In order to reduce bleeding from the labial arteries, pinching the lip lateral to the incision could be done.
The skin C-flap is raised (marked purple).
At this point the landmarks are approximated to make sure that they are readily juxtaposed without tension and with good symmetry:
Orbicularis oris muscle edges.
Points 4 and 8.
Corner of C-flap and 7.
Superior corner of advancement flap and point 10.
Sutures
Suturing starts at the posterior wet vermilion mucosa (▶ Fig. 39.5), then muscle edges (▶ Fig. 39.6), then lip skin, and finally nasal sill which is created by the C-flap (▶ Fig. 39.7). Mucosa can be sutured by dissolvable suture, such as vicryl, muscle by long-term dissolving sutures, and skin by either 7–0 nondissolving monofilament sutures that should be later removed, or by 6–0 rapidly dissolving sutures.
Good tensionless eversion of the philtrum column skin edges would promise the best esthetic result.
39.2.4 Postoperative Care
Before the patient emerges from anesthesia, arm restraints are placed in order to prevent the infant from touching the surgical wound. Feeding is continued by syringe with attached soft rubber tube for 2 weeks. Breastfeeding can be continued after surgery, but it should be taken into consideration that some suckling infants would be uncomfortable with suckling during the few days after surgery and might not want to go back to breastfeeding after the postoperative recovery period.
If nondissolving sutures were used, the patient is brought back to the operating room or sedation suite after 1 week to remove the skin sutures under general anesthesia or sedation.
Prevention from the scar being exposed to sun is recommended and silicon gel (such as Kelo-cote or Mederma) can be recommended as well.
Antibiotic treatment is not routinely prescribed.
39.3 Bilateral Cleft Lip Repair
39.3.1 Introduction
Bilateral cleft lip deformity is characterized by extrusion of the premaxilla, symmetric deformed nostrils, and short columella. Degree of anterior deviation of the premaxilla significantly influences the perceived deformity and ease of repair. Suckling might be affected. Cleft lip is bilateral in about 25% of cases and more common in males.
39.3.2 Preoperative Evaluation and Anesthesia
Soon after birth and prior to surgery, presurgical orthopedics is applied. Nasoalveolar molding (NAM) device is customized to the patient in order to improve nostril shape, as well as premaxilla and maxillary shelf position.
Repair usually takes place at 3 to 4 months of age. The patient should be evaluated by the pediatric anesthesiologist to ensure that the infant gained enough weight and is generally healthy in order to make the anesthesia and surgery at optimal safety, taking into consideration that the surgery is elective and mainly cosmetic.
39.3.3 Surgical Technique
Surgery is done under general inhalational anesthesia with an oral endotracheal RAE tube secured to the mandible but without distorting the lower lip. A shoulder roll is placed under the supine patient for a Rose position. The surgeon is positioned in front of the head of the bed, either sitting or standing.
Markings
The upper lip landmarks are carefully marked with an extra-fine marker and calipers (▶ Fig. 39.8).
Prolabium
Point 1: Midpoint of the vermilion-cutaneous junction.
Point 2: On the vermilion-cutaneous junction, 2 to 2.5 mm lateral to point 1 to the right.
Point 3: On the vermilion-cutaneous junction, 2 to 2.5 mm lateral to point 1 to the left.
The distance from 1 to 2 is equal to 1 to 3 (for a symmetric cupid bow).
Point 4: Right prolabium columella junction.
Point 5: Left prolabium columella junction.
The distance from 2 to 4 is equal to 3 to 5 (for symmetric philtral columns).
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