Jeffrey C. Posnick, DMD, MD

Facial Growth Implications of Cleft Palate Repair in the Infant with Isolated Cleft Palate

Ross documented that at least 20% of Caucasian individuals with ICP who undergo repair during infancy will experience maxillary hypoplasia that results in malocclusion that is not responsive to either traditional or compensatory orthodontic maneuvers alone.⁸⁶ Chen and colleagues reported on horizontal maxillary growth in both children and adults with Eastern Chinese ethnic backgrounds and with unoperated and operated ICP.¹⁴ There were two study groups: individuals in Group 1 had non-syndromal and unoperated ICP that was evaluated during the mixed dentition (n = 16); individuals in Group 2 had non-syndromal and unoperated ICP that was evaluated during the permanent dentition (n = 25). The control groups included patients with ICP who underwent repair during childhood as well as non-cleft individuals with Class I occlusion that was evaluated during both the mixed and permanent dentitions. Lateral cephalograms of all subjects were analyzed. The results of the mixed dentition groups showed almost normal sagittal growth in unoperated patients except for the reduced anterior-posterior maxilla length. In contrast, the operated patients showed reduced length of both the maxilla and mandible, as well as a clockwise rotation of the mandible. The analysis of the permanent dentition groups showed that in both the unoperated and operated ICP patients, there were reductions in maxilla length, mandibular protrusion, and maxillary retrusion. Interestingly, there appeared to be no correlation between maxillary growth restriction and the extent of the congenital clefting of the secondary palate (i.e., soft palate only versus hard and soft palate). The authors concluded that, for individuals born with ICP, the etiology of the maxillomandibular deficiency likely results from a combination of factors, including the intrinsic primary (cleft) defect; secondary hypoplasia as a result of the surgical repair during infancy (iatrogenic); and functional (environmental) factors (e.g., the effects of muscles of mastication, respiratory patterns, mandibular rest posture; see Chapter 4). These results were similar to those of Yoshida and colleagues, who found that individuals with ICP frequently end up with maxillary horizontal deficiency and clockwise rotation of the maxillomandibular complex that tend to become worse as the child reaches adulthood.⁹⁹

A significant percentage of adolescent patients who were born with ICP will present with a jaw deformity that requires, at minimum, a maxillary Le Fort I (down-fracture) osteotomy to align the jaws.5,85 When indicated, this is combined with mandibular and chin osteotomies to achieve improved facial proportions and with intranasal procedures (i.e., septoplasty and inferior turbinate reduction) to improve nasal breathing.

Coordinated Team Approach

The facial reconstructive and dental rehabilitation of an individual with ICP is best delivered via collaborative care provided by appropriate medical and dental specialists.6,62,71 It is no longer justified for individual practitioners—whether they are surgeons, orthodontists, restorative dentists, speech pathologists, or otolaryngologists—to carry out extensive treatment without informing the patient and his or her family of available options and considering clinical input from the other specialists who are caring for the patient.

Treatment Protocol

Patients who are referred for possible orthognathic surgery are seen by an orthodontist, an orthognathic surgeon, a speech pathologist, and an otolaryngologist. Consultation with other medical (e.g., sleep specialist, geneticist) and dental (e.g., periodontist, restorative dentist) specialists may also be indicated. Initial records and tests include, at minimum, medical-quality photographs, including views of the face and the occlusion; cephalometric and Panorex radiographs; dental models with centric bite registration; speech assessment with instrumentation (ex. naso-endoscopy); and a thorough evaluation of the upper airway. Discussions with the medical and dental consultants, the family, and the patient are needed to set the clinical objectives. Decisions about the timing and extent of the orthodontic, surgical, and dental treatments are made in an effort to reach the chosen objectives.

The primary cleft surgeon completes the patient’s palate repair during infancy, and this is followed by pharyngeal procedures later during the patient’s childhood if they are required to improve velopharyngeal function. The surgeon is also likely to play an important role in directing the patient’s care throughout adolescence and into adulthood. If the cleft surgeon is not trained in skeletal procedures, then a timely and seamless transition to the maxillofacial surgeon who will continue on with the patient’s reconstruction is necessary. The orthodontist identifies early abnormal facial growth patterns and may carry out interceptive treatment.6,10,11,18,19,27,34,51,55,64,65,87,90,91 Definitive orthodontic treatment is coordinated with orthognathic surgery, when indicated.³³ Extensive compensatory orthodontic treatment is likely to jeopardize periodontal health and to lead to dental relapse with recurrent malocclusion. Camouflage orthodontics should be avoided for these reasons and only be entered into with full disclosure to the family and other treating clinicians.

Before orthognathic surgery is performed, the speech pathologist performs an evaluation to assess the patient’s velopharyngeal function; this will ideally include nasoendoscopic instrumentation. Such evaluation is important, because velopharyngeal function may deteriorate after maxillary Le Fort I osteotomy with advancement (see Chapter 8). Velopharyngeal closure that was adequate before surgery may become borderline afterward, and closure that was borderline may become inadequate. In the past, investigators have speculated that, if the distraction (DO) technique is used to advance the Le Fort I osteotomy, then velopharyngeal function will not deteriorate. However, clinical studies have now documented that velopharyngeal function will deteriorate in a similar fashion after Le Fort I advancement when either DO or standard techniques are used.^{12,13,35,37,50,54,58,61,72,89,94} Articulatory distortions that result from the jaw discrepancy and malocclusion also are identified by the speech pathologist, and cause-and-effect relationships are determined. It is known that the successful orthodontic and surgical correction of crossbites, open bite, residual palatal fistulas, and negative overjet will generally correct the presenting articulation errors (see Chapter 8).

The otolaryngologist plays a role in the assessment of upper airway and middle ear function. Chronic nasal obstruction and sinusitis are frequent in the patient with a cleft palate. Mucous trapping may also be a problem after a pharyngeal flap procedure. If sleep apnea is suspected, an attended polysomnogram should be carried out (see Chapter 26). If intranasal procedures (i.e., septoplasty and inferior turbinate reduction) are needed to improve breathing, they should be simultaneously carried out in conjunction with orthognathic surgery (see Chapter 10).

Timing of Orthognathic Surgery

The correction of the jaw deformity associated with ICP is best carried out when the skeleton is mature and before the patient completes high school, if feasible. Maxillofacial growth is generally complete between the ages of 14 and 16 years in girls and between the ages of 16 and 18 years in boys. However, skeletal growth is variable, and it may be further clarified via the analysis of sequential cephalometric radiographs (see Chapter 17). Input from the patient and the family regarding the timing of surgery and the patient’s functional needs (e.g., breathing, speech, chewing, swallowing, body image, self-esteem) are also important considerations.

As early as 1986, clinicians clarified through preliminary studies that, if jaw surgery is undertaken in the growing patient with a cleft palate patient, revision orthognathic surgery will likely be required after skeletal maturity is reached.95–98 More recently, other investigators have tested this theory by proceeding with Le Fort I osteotomy involving DO methods during the mixed dentition. Studies to date indicate that a Le Fort I osteotomy carried out during the mixed dentition, whether with a standard or a DO technique, results in limited or no further horizontal maxillary growth.^{17,36,46,73,74} As the mandible continues to grow, an Angle Class III malocclusion recurs with the need for either additional Le Fort I advancement or mandibular set-back.

Residual Deformities in the Adolescent with Isolated Cleft Palate

The adolescent or adult patient with ICP who is referred for orthognathic evaluation will have an intact alveolar ridge and generally will have a full complement of teeth. However, these individuals may present with one or more of the following residual cleft-related problems (Figs. 34-1 through 34-9):