Jeffrey C. Posnick, DMD, MD

Facial Growth Implications of Cleft Palate Repair in the Infant with Bilateral Cleft Lip and Palate Deformity

Ross confirmed that individuals who are born with cleft lip and palate have intrinsic deficiencies in the midfacial skeleton that are made significantly worse by operations.¹¹⁸ Mulliken and colleagues from Boston Children’s Hospital found that 76.5% of teenagers with repaired bilateral cleft lip palate (BCLP) required maxillary advancement.⁴³ They showed that the need for orthognathic surgery is dependent on the severity of the cleft type as well as the number and extent of previous operative procedures. Results from The Hospital for Sick Children in Toronto, Canada, showed that 65.1% of their patients with BCLP required or underwent orthognathic surgery, whereas 70% of children with BCLP who were referred to their team some time after cleft lip repair were found to require orthognathic surgery.²³ David and colleagues from the cleft craniofacial unit in Adelaide, Australia, followed a consecutive group of patients with BCLP from birth to maturity and determined the need for orthognathic surgery.²⁴ Each patient was followed according to treatment protocol and then at 18 years of age assessed for dentofacial deformity. A skeletal Class III malocclusion that required orthognathic correction was present in 17 of the 19 patients (89.5%), whereas the remaining 2 patients were found to have a malocclusion that was manageable with orthodontics alone. Fourteen underwent orthognathic surgery, and the remaining three declined the offer for correction.

Saperstein and colleagues discussed facial growth in children with complete clefting of the primary palate (i.e., the lip through the incisal foramen) but with an intact secondary palate (i.e., the incisal foramen through the uvula).¹²⁰ This was a retrospective, cross-sectional analysis of non-syndromal patients with bilateral complete clefting of the primary palate (n = 7) as compared with those with bilateral complete clefting of both the primary and secondary palates (n = 27). Angular and linear measurements of the midfacial region were made on lateral cephalograms. The research documented that individuals with a bilateral cleft of the primary palate who underwent lip repair during infancy most frequently have a normal or even slightly forward maxillary position as compared with age-matched controls. This was in contrast with children with a bilateral cleft of both the primary and secondary palates who underwent lip repair followed by palate repair before they were 1 year old, who demonstrated a high incidence of maxillary deficiency. The study clarified that cleft palate repair carried out before the patient is 1 year old—and not the cleft lip repair itself—is responsible for the high incidence of midface hypoplasia.

It is now clear that orthognathic surgery is a necessary component of the treatment of a majority of individuals who are born with BCLP. Unfortunately, the necessary jaw reconstruction is often considered “too risky” and therefore postponed, delayed, ignored, or refused. If so, the patient is likely to be treated with orthodontic and prosthetic camouflage techniques. Adding further difficulty to successful rehabilitation is controversy about the indications and confusion regarding the exact techniques, timing, and extent of surgery needed to achieve reliable and predictable results. The occlusal, speech, upper airway, and aesthetic objectives in the adolescent patient with BCLP must first be clarified. A surgical, orthodontic, and dental treatment plan can then be coordinated to achieve improved head and neck function, facial enhancement, and dental rehabilitation.

Coordinated Team Approach

The care of the patient with BCLP is best delivered by an integrated group of dental and medical specialists who evaluate and then provide coordinated definitive treatment.8,77,92 It is no longer acceptable for individual surgeons, orthodontists, restorative dentists, speech pathologists, and otolaryngologists to carry out extended treatment without considering all aspects of the patient’s care and communicating effectively with the patient, the family, and the collaborating clinicians. The Eurocleft study found no association between high-intensity treatments and favorable results. In other words, the more operations and years of orthodontic appliances (i.e., heavy burden of care), the worse the outcome.⁷⁹

Treatment Protocol

Adolescents and adults with BCLP who also have jaw deformities should be seen at a minimum by an orthodontist, an orthognathic surgeon, an otolaryngologist, and a speech pathologist. Additional consultations with other dental (e.g. a prosthodontist, a pediatric or general dentist, a periodontist) and medical (e.g., sleep specialist, medical geneticist) specialists are obtained, if necessary. For the initial evaluation and eventual treatment of the cleft dentofacial deformity, records and tests should include the following: medical-quality photographs with views of the face and the occlusion; cephalometric and dental radiographs; dental models; direct facial measurements; speech assessment; and thorough evaluation of the upper airway.

In addition to performing lip, palate, and velopharyngeal (VP) repairs, the primary cleft surgeon generally plays a role in advising the patient and the family about the patient’s further care. 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 patients’ reconstruction should go forward. The cleft jaw surgeon should have a fundamental understanding of the patient’s dental, speech, upper airway, and aesthetic needs. The surgeon should request consultation with appropriate specialists, evaluate the clinical information, and be prepared to go forward with coordinated and timely up-to-date orthognathic procedures.

The orthodontist provides interceptive treatment during the mixed dentition in coordination with bone grafting; and carries out definitive orthodontics in conjunction with orthognathic surgery, when indicated. From the mixed-dentition phase, the orthodontist should recognize the patient with BCLP who will likely be in need of orthognathic surgery.20,21,25,36,52,53,64,80,90,114,117,121,132,145 Instituting extensive camouflage (dental compensatory) treatment is likely to jeopardize periodontal health and lead to late dental relapse of the occlusion. Proceeding with a compromised orthodontic (camouflage) approach should only be entered into with full disclosure to the family and other treating clinicians.

Before orthognathic surgery, a speech pathologist performs an evaluation to characterize VP function and to identify articulation errors that result from the cleft palate, the jaw deformity, and the dental malocclusion (see Chapter 8). Such evaluation is important, because VP function may deteriorate after maxillary advancement. A nasoendoscopic guided speech assessment is preferred to obtain maximum objective data. VP closure that is adequate before surgery may become borderline afterward, and VP closure that is borderline may become inadequate. The successful orthodontic, surgical, and dental correction of crossbites, open bite, cleft–dental gaps, negative overjet, and residual oronasal fistula represents the most effective way to correct the identified articulation distortions (see Chapter 8).

A thorough evaluation of the upper airway is conducted to assess for any areas of obstruction (see Chapter 10). A formal sleep study (polysomnogram) is completed if there is a suspicion of obstructive sleep apnea (see Chapter 26). If indicated, simultaneous intranasal procedures to open the airway should be carried out at the time of orthognathic surgery (see Chapters 10 and 15).

Discussions with the treating medical and dental consultants as well as the patient and his or her family clarify the need for and the extent of orthognathic procedures (e.g., Le Fort I osteotomy, sagittal ramus osteotomies, osseous genioplasty) and intranasal procedures (e.g., septoplasty, inferior turbinate reduction, recontouring of the nasal floor and rims). The overall plan for speech, jaw, upper airway, and dental rehabilitation and the enhancement of facial aesthetics is agreed to before the initiation of treatment.

Timing of Orthognathic Surgery

Correction of the cleft jaw deformity is best carried out with a consideration of skeletal maturity and before the patient finishes high school.66,69 Maxillofacial growth is generally complete between the ages of 14 and 16 years in girls and the ages of 16 and 18 years in boys. However, skeletal growth is variable, and it may be further clarified by an analysis of sequential cephalometric radiographs taken at intervals (see Chapter 17). Patient and family preferences for the timing of reconstruction on the basis of the patient’s psychosocial and functional needs (i.e., speech, swallowing, chewing, and breathing) should also be taken into account.

As early as 1986, investigators showed that, if Le Fort I advancement is completed during the mixed dentition in a patient with a cleft palate, then another orthognathic procedure will be necessary after skeletal maturity is reached.140–143 All research to date indicates that a Le Fort I osteotomy carried out at an early age, with the use of either standard or distraction osteogenesis (DO) techniques, results in no further horizontal maxillary growth.^{16,34,35,46,48,59,95}

Residual Deformities in the Adolescent with Bilateral Cleft Lip and Palate

The adolescent patient with BCLP often has several residual deformities that can be challenging to manage. The central deformity is maxillary hypoplasia, which is often combined with residual oronasal fistula, bone defects, intranasal obstruction, soft-tissue scarring, and occasionally VP dysfunction (Figs. 33-1 through 33-17). In addition, the maxillary lateral incisors are usually congenitally absent or hypoplastic (93% of the time), and the patients often have cleft–dental gaps.¹³ Secondary deformities of the nose, the mandible, and the chin region are also common. The prevalence of these residual deformities in mature patients with BCLP varies widely, depending on the treating clinician’s philosophy, available expertise, the individual’s intrinsic biologic growth potential, and patient and family interests. Published clinical surveys indicate that, despite a cleft team’s best efforts, a significant number of children with BCLP will not make themselves available for mixed-dentition grafting (i.e., before the eruption of the maxillary canines).^33,137 In addition, a percentage of those who do will undergo unsuccessful grafting procedures and require other means of reconstruction and dental rehabilitation. For all of these reasons, a subgroup of individuals with BCLP continues to present at or after adolescence with multiple cleft-related challenges that may include the following: