CC
As a member of the multidisciplinary cleft and craniofacial anomalies team, you are asked to evaluate a male infant born with a right unilateral complete cleft lip and palate (CLP) Veau class III.
Cleft lip is a unilateral or bilateral gap in the upper lip that forms during the 3rd through 7th weeks of embryonic development. It develops from failure of fusion of the medial nasal process and the maxillary process. Clefting of the lip is generally described as either complete or incomplete. A complete cleft lip is a cleft of the entire lip with discontinuity of the orbicularis oris and usually the alveolar arch or premaxilla; an incomplete cleft lip involves only the lip to varying degrees and often spares the soft tissue of the associated alar base of the nose. A cleft palate is a gap in the hard or soft palate (or both) that forms during the 5th through 12th weeks of development. Cleft palate forms as a result of failure of attachment and alignment of the levator veli, tensor veli palatini, uvular, palatopharyngeus, and palatoglossus muscles.
There are both ethnic and racial variations in the incidence of CLP. It is most common in Asians (3.2 in 1000) followed by Whites (1.4 in 1000), and individuals of African descent (0.43 in 1000). CLP occurs more often in males and on the left side. Isolated cleft palate is a different genetic entity with no racial predilection. It is more common in females and when present is significantly associated with underlying sequence or syndromic genetic alterations or further midline developmental anomalies These may include Pierre Robin sequence, Stickler syndrome, van der Woude syndrome, or 22q deletion.
HPI
The CLP was known prenatally. Using high-resolution ultrasonography, cleft lip can be diagnosed reliably as early as 14 weeks of gestation, though a diagnosis of cleft palate may be more difficult until closer to 20 to 21 weeks of gestation. The infant was born at a community hospital with no obstetric complications. His presentation is consistent with a nonsyndromic CLP. The pregnancy was uncomplicated. Parents report no known environmental exposures, and there is no family history of cleft lip or palate. Potential environmental contributors to the development of CLP may include maternal alcohol or tobacco use, vitamin deficiencies, or exposure to medications such as corticosteroids and antiseizure treatments. If a single parent has CLP, the likelihood of an infant having CLP is 4% to 6%. If neither parent has a history of CLP and one child has CLP, the likelihood of a second child having CLP is 2% to 8%.
PMHX/PSHX/medications/allergies/SH/FH
Except for the CLP, the child has no other medical problems. He was born with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. There are no facial or systemic anomalies characteristic of any known syndromes (see Discussion later in this section), including any associated cardiac, respiratory, renal, ophthalmologic, or musculoskeletal abnormalities. There is no family history of CLP or isolated cleft palate.
Examination
General. Male infant is in the 25th percentile for weight and height (potentially because of difficulty with feeding).
Maxillofacial. The cleft lip (cleft lip) is complete, penetrating the entire thickness of the lip, alveolus, nasal tip cartilages, and floor of the nose ( Fig. 92.1 ). The cleft is unilateral, right of midline, and continuous with the palate (CLP is most commonly expressed unilaterally, with a 2:1 predilection for the left side).
Intraoral. The cleft continues through the hard and soft palates. (Structures anterior to the incisive foramen form the primary palate and posterior to the incisive foramen form the secondary palate.) Throughout the cleft, the nasal cavity, nasal conchae, and posterior pharyngeal wall are readily visible. The nasal mucosa appears inflamed and ulcerated (because of irritation of the fragile tissue from feeding). Bidigital palpation identifies solid supportive bone along the palatal shelves bordering the cleft site. This presentation would be consistent with a Veau class III cleft of the palate involving the complete primary, secondary, and soft palate areas. The Veau classification is commonly used, in which Veau class I represents soft palate clefting only, Veau class II the soft and hard palate but not the alveolus area, and Veau class IV representing complete bilateral clefting of all oral structures. It is important to palpate the hard palate of any infant. Even when an obvious defect is not readily visible, palpation may detect a notch at the posterior border of the hard palate, which together with a zona pellucida along the mid soft palate and notching of the uvula creates a clinical triad indicating submucous cleft palate.
Imaging
No imaging studies are indicated for the diagnosis and management of routine isolated CLP. Depending on the physical examination findings, further workup may be indicated. For example, if ear abnormalities exist, a screening renal ultrasound examination may be indicated. When midline defects such as isolated cleft palate are present, examination of other structures such as the heart with an echocardiogram are important. When orbital or cranial bony anomalies exist, computed tomography is indicated.
Labs
Baseline hemoglobin and hematocrit levels are indicated before surgical correction of any cleft. In general, a hemoglobin level of 10 mg/dL often correlates with sufficient growth and development of the infant patient to facilitate safer administration of general anesthesia. Any family history of bleeding diatheses should be thoroughly investigated before surgery.
Assessment
Newborn male infant with nonsyndromic right unilateral complete CLP Veau class III.
Treatment
Preoperative considerations
There is no consensus regarding the timing and techniques used for CLP surgery. Individual cleft and craniofacial centers and surgeons follow various protocols according to their own experience, rationale, and preferences. The functional needs, esthetic concerns, and ongoing growth of affected individuals all create specific concerns that complicate the treatment process. All agree, though, that feeding, growth, and development are of primary importance. It is essential for such patients to be treated in a multidisciplinary team manner to allow for continued proper growth and development, particularly patients with clefting of the palate. Patients who have orofacial clefts that involve the palate cannot generate the appropriate suction seal with their mouths to feed properly. This leads to excessive effort when feeding and can lead to an imbalance between the nutrition an infant is able to consume versus the energy expended by the body in consuming it. Although specialized feeding bottles help tremendously in this endeavor, the input of multiple specialists is necessary for infants with CLP to grow appropriately.
Presurgical dentofacial orthopedics are increasingly used to optimize primary CLP repair. These techniques are commonly known as nasoalveolar molding (NAM). As the name implies, such dentofacial orthopedics can be used to improve lip soft tissue, nasal tissue and projection (particularly in bilateral CLP), and the palatal shelves. Patient growth and socioeconomic concerns must always remain critical factors when deciding whether to use NAM because the devices may require frequent clinic visits for alterations.
Timing considerations
Table 92.1 outlines the sequence of management of patients with CLP. Cleft lip repair is usually addressed at 10 to 14 weeks of age. It is essential to take into account if a patient was born prematurely because the necessary growth preoperatively may require waiting longer before performing primary cleft repairs. An additional advantage of waiting until this age is to allow for a thorough medical evaluation to determine whether the infant has any congenital defects or additional medical considerations. The surgical procedure is generally easier to perform when the child is slightly larger because anatomic landmarks are more prominent and well defined. It has historically been accepted that the safest anesthesia time period for infants is based on the “rule of 10s”—surgery can be performed when the child is at least 10 weeks of age, weighs at least 10 lb, and has a minimum hemoglobin value of 10 mg/dL. (However, there is no current scientific rationale to support this rule.) With modern intraoperative pediatric monitoring techniques, general anesthesia can be performed safely at an earlier age as needed, although there is little literature to support performing cleft lip repair before 3 months of age because severe growth restriction of the maxilla may occur. Excessive scarring and inferior esthetic results have also been documented when surgery is performed earlier than age 3 months.
| Procedure | Age or Timing | Comments |
|---|---|---|
| Dentofacial orthopedics | First few weeks of life | Improves tension-free lip closure |
| Lip adhesion (two-stage repair) | After dentofacial orthopedics and before definitive nasolabial repair | Some centers prefer one-stage closure and do not perform lip adhesions |
| Definitive nasolabial repair |
|
Timing may vary based on cleft type |
| Cleft palate repair |
|
|
| Correction of VPI (pharyngeal flap or sphincter pharyngoplasty) | Speech assessment begins at 1.5–2 years | VPI may occur after maxillary advancement and can be corrected 6–12 months later |
| Nasolabial revisions | Before 3 years | — |
| Phase 1 orthodontics | Before alveolar cleft bone grafting | Differentially expands the anterior maxilla |
| Alveolar bone grafting | At 8–12 years (when maxillary canine root is half to two-thirds formed) | Bone graft from anterior ilium is usually preferred |
| Phase 2 orthodontics | Permanent dentition phase | — |
| Correction of maxillary hypoplasia (orthognathic surgery, distraction osteogenesis, or both) | After completion of growth | Distraction osteogenesis should be considered when maxillary advancement is >10 mm |
| Rhinoplasty | At 6–12 months after maxillary advancement | — |
Cleft palate repair is usually performed between 9 and 18 months of age. It is intended to coincide with the progression of natural speech development and growth. In deciding on the timing of repair, the surgeon must consider the delicate balance between facial growth restriction after early surgery and early speech development, which requires an intact palate. It is important to avoid compensatory misarticulations that may result if speech development continues without an intact palate. Most children require an intact palate to produce certain sounds by 18 months of age. If developmental delay is present and speech is not anticipated to develop until later, cleft palate repair can be delayed. A high percentage of patients with CLP have severe and recurrent enough middle ear infections that if there exists middle ear disease at the time of palatal repair, myringotomy tubes are typically placed as well. There is alteration of the eustachian tube anatomy in patients with CLP, and in addition to a flatter angle relative to the skull base as pediatric patients develop, inadequate middle drainage is common.
There is very little evidence to support cleft palate repair before 9 months of age. Surgical repairs before this time are associated with a higher incidence of maxillary hypoplasia later in life and show no improvement in speech. However, this pertains to repairs involving the hard palate. At some centers, the soft palate is repaired first to facilitate appropriate speech development and the hard palate repaired later to allow less effect on maxillary growth. Most centers, though, repair the hard and soft palate defects at the same time. After initial cleft palate repair, 15% to 20% of children develop inadequate closure of the velopharyngeal mechanism (velopharyngeal insufficiency [VPI]). This is usually diagnosed at 3 to 5 years of age, when a more detailed speech examination can be performed because of patient participation. It is important to have both objective (endoscopic nasendoscopy or video fluoroscopy) as well as subjective (speech-language pathology evaluation) assessments of a patient with VPI when considering the most appropriate intervention. Surgery may be performed to correct an anatomic defect at the velopharynx with the goal of improving closure between the palatal and nasopharyngeal tissues to reduce nasal air escape during the production of certain sounds. Multiple types of VPI surgery exist, and not all patients require surgical intervention when speech therapy can treat the primary issue if the diagnosis is accurate from the start.
Approximately 75% of patients with any type of cleft present with clefting of the maxilla and alveolus. Bone graft reconstruction of the alveolus is performed during the mixed dentition before eruption of the permanent canine or permanent lateral incisor. The timing of this procedure is based on dental development and not chronologic age. Reconstruction of the alveolus before the mixed dentition stage has been associated with a high degree of maxillary growth restriction and potential need for additional bone graft reconstructions at a later age. Autogenous bone grafted from the iliac crest has provided the best results for reconstruction of alveolar or maxillary cleft defects. Most commonly in the United States, the alveolar grafting is completed when the associated canine root is two-thirds formed, that is, typically at 9 to 11 years of age. Before grafting, orthodontic expansion of the maxilla is indicated to maximize the amount of graft placed in the alveolus and to facilitate elevation of soft tissues for nasal and oral reconstruction. Considerable literature now exists for the use of allogeneic bone graft materials as well as adjuncts such as bone morphogenetic protein, which may be considerations in older patients or those requiring repeat operations.
Orthognathic reconstruction of maxillary and mandibular discrepancies is generally performed between 14 to 18 years of age based on individual growth characteristics. A combined approach with both orthodontics and surgery is necessary for optimal patient outcomes. Orthognathic surgery before this time frame is performed only for severe cases of dysmorphology and for certain severe psychosocial concerns.
Lip and nasal revisions are best completed after orthognathic surgery is finished if planned and usually at least 1 year after jaw surgery. Correction of the skeletal facial discrepancy allows for the appropriate foundation for cleft rhinoplasty or otherwise.
Surgical techniques for cleft lip repair
Many techniques for cleft lip repair exist, each with multiple modifications. The technique most commonly used for unilateral cleft lip repair is the Millard rotation-advancement technique. A three-layer closure of skin, muscle, and mucosa is performed to reposition the superiorly inserted orbicularis oris muscle into its normal circular orientation. The medial cleft lip is rotated inferiorly as well, and the lateral lip segment is advanced to meet it. A separate C-flap along the medial margin of the cleft aids in closure and creation of the nasal sill. The small flap can also be positioned medially in part to lengthen the rotation side of the reconstruction. A critique of this technique is the potential for shortening of the repair or the need for longer backcutting incisions with larger advancements. Hypoplastic tissue along the cleft margins is excised and discarded. The Mohler repair is similar to the Millard repair but may be more useful for more vertically oriented unilateral cleft lips. The medial incision is carried more vertically at the base of the columella to gain vertical length as the small curved superior aspect becomes straight.
The Randall-Tennison technique is a Z-plasty technique used by some surgeons for unilateral cleft lip repair. This technique allows for less scar contracture because of its Z-plasty design but places the incision in a potentially less cosmetic location than other repairs.
Increasing in use is the geometric subunit repair created by Fisher. This repair uses aspects of a straight-line repair as well as triangular insertions to lengthen the lip in a very specific manner in which the lengths of each repair segment are precisely measured and marked. This is in contrast to the rotation-advancement technique in which modifications can be easily created as a repair proceeds if more length is needed. The repair avoids less cosmetic backcuts around the lateral crural footplate but requires discarding a more significant amount of soft tissue.
Primary nasal reconstruction is often performed at the time of lip repair to reposition the displaced lower lateral cartilages and alar tissues. Various techniques have been advocated, each with considerable variation. The repair essentially involves releasing the alar base, augmenting the area with allogenic subdermal grafts, or proceeding with open rhinoplasty with minimal dissection to avoid scar formation.
Bilateral cleft lip repair is a very challenging technical procedure, primarily because of the lack of quality tissue present and the manner of separation of the tissues caused by the clefting. The typically shortened columella and rotation of the premaxillary segment make achieving acceptable aesthetic results difficult, and preoperative NAM can be helpful. Most bilateral cleft lip repairs create orbicularis oris continuity across the prolabium (Millard, Mulliken, cutting) or simply attach the lateral lip segments to the prolabium medially without creating orbicularis oris direct continuity (Manchester).
Variations to surgical approaches range from aggressive lengthening of the columella with preservation of hypoplastic tissue to conservative primary nasal reconstruction as performed with McComb’s unilateral cleft lip technique. McComb’s technique involves release and repositioning of the lower lateral cartilages and alar base on both sides without aggressive degloving of the entire nasal complex. Aggressive corrective techniques often produce initial results that are very good. Long-term results, however, are not so favorable because of the progression of natural growth processes. Excessive angulations and lengthened structures provide a less-than-optimal esthetic effect. Revision of these deformities is usually very difficult and sometimes impossible. In general, if hypoplastic tissue is excised and incisions within the medial nasal base and columella are avoided, long-term esthetic results are excellent.
Surgical techniques for cleft palate repair
Successful cleft palate repair during infancy depends on two objectives. The first involves watertight closure of the oronasal communication involving the hard and soft palate except for the area of the alveolus. The second involves anatomic repair of the musculature within the soft palate, which is critical for the creation of normal speech. The soft palate functions in coupling and decoupling of the oral and nasal cavities in the production of speech. The tensor and levator veli palatini and uvularis muscles, which usually join at the midline to form a continuous sling, are separated and insert along the posterior edge of the hard palate. The velum, or soft palate, must be closed before the development of speech. If repair occurs after this time, compensatory speech articulations may result.
An approach used to address the speech issues with growth-related concerns involves staging the closure of the secondary palate with two procedures. This involves repair of the soft palate early in life followed by closure of the hard palate later during infancy. The intent of this approach is to accomplish timely repair of the soft palate, which is critical for speech, while delaying hard palate repair until further growth has occurred. This technique offers the advantage of less growth restriction, easier repair of larger clefts, and less chance for fistula formation.
The basic premise of cleft palate repair involves mobilization of multilayered flaps to close the defect created caused by the failed fusion of the palatal shelves. The nasal mucosa is first closed followed by repositioning and reconstruction of the levator veli palatini and palatopharyngeus muscles. Closure of the oral mucosa completes the repair.
Many techniques have been devised for cleft palate repair. The Bardach technique involves creation of two large, full-thickness flaps on each palatal shelf, which are brought to the midline for closure ( Fig. 92.2 A and B). This technique allows for preservation of the palatal neurovascular bundle, which is contained within the pedicle of each flap. The Von Langenbeck technique is similar to the Bardach technique, but it preserves an anterior pedicle (attachment to the anterior maxilla) for increased blood supply to the flaps. It also involves elevation of large mucoperiosteal flaps from the palate with midline approximation of the cleft margins. Long lateral releasing incisions are made at the border of the palatal and alveolar bone for both repairs to allow mobilization. The levator muscles are detached from their abnormal insertion along the hard palate, and the palatopharyngeus is intimately associated.
