Cleft–Orthognathic Surgery

The Bilateral Cleft Lip and Palate Deformity

Jeffrey C. Posnick, DMD, MD

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

• Coordinated Team Approach

• Treatment Protocol

• Timing of Orthognathic Surgery

• Residual Deformities in the Adolescent with Bilateral Cleft Lip and Palate

• Orthodontic Considerations in the Patient with Bilateral Cleft Lip and Palate with Jaw Deformity

• Immediate Presurgical Assessment

• Orthognathic Approach for Bilateral Cleft Lip and Palate Deformities

• Avoiding Pitfalls

• Clinical Management after Initial Surgical Healing

• Orthognathic Surgery for Bilateral Cleft Lip and Palate: Review of Study

• Controversies and Unresolved Issues

• Conclusions

The reconstruction of bilateral cleft lip and palate is inextricably connected with orthodontic care and skeletal surgery. The successful management of these individuals is likely to present a formidable clinical challenge for the orthognathic surgeon, the orthodontist, and the dental team.

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:

Figure 33-1 A child who was born with bilateral cleft lip and palate underwent lip and palate repair during early childhood. During the mixed dentition, she presented to this surgeon with a mobile premaxilla, alveolar and palatal defects, and residual (bilateral) labial and palatal fistulas. She then underwent a short phase of orthodontic treatment to expand the maxillary arch width. This was followed by iliac grafting of the skeletal defects and fistula closure. She was fortunate to have adequately developed lateral incisors. A, Facial views during infancy and early childhood. B, Facial and occlusal views during the mixed dentition with interceptive orthodontic treatment in progress and before bone grafting. C, Illustration of incisions for the elevation of flaps, fistula closure, and grafting during the mixed dentition in a child with BCLP. Maintenance of the labial mucosa to the premaxilla is essential. D, Illustration of flap and wound closure after grafting for fistula closure. The occlusal splint is secured to the maxillary segments to immobilize the premaxilla during initial bone healing. Parts C, D are modified from an original illustration by Bill Winn. E, Frontal and occlusal views at 14 years of age after successful grafting, fistula closure, and stabilization of the premaxilla, with definitive orthodontic alignment in progress. F, Views of the healed skin incision used to harvest the iliac graft. G, Palatal and radiographic views before and after successful bone grafting and orthodontic alignment.

Figure 33-2 A child who was born with bilateral cleft lip and palate and who had intact nasal sills. She was referred to this surgeon and underwent lip and palate repair during infancy. During the mixed dentition, she underwent successful bone grafting with stabilization of the premaxilla. The canines erupted through the grafted cleft sites and established a normal alveolar ridge. The lateral incisors were essentially normal and maintained within the arch form. With significant maxillary hypoplasia, the patient underwent a comprehensive orthodontic and orthognathic approach. Her procedures included Le Fort I osteotomy in three segments (correction of arch width and curve of Spee, horizontal advancement, vertical lengthening) with interpositional grafting; bilateral sagittal split ramus osteotomies (clockwise rotation); and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, Frontal facial views before lip repair, after primary lip and palate repair, and at 8 years of age just before mixed dentition grafting. B, Frontal views in repose as a teenager before and after reconstruction. C, Frontal views with smile before and after reconstruction. D, Oblique facial views before and after reconstruction. E, Profile views before and after reconstruction. F, Occlusal views during the mixed dentition, with orthodontics in progress before orthognathic surgery, and then after the completion of treatment. G, Articulated dental casts indicate analytic model planning. H, Lateral cephalometric radiographs before and after reconstruction.

Figure 33-3 A child who was born with van der Woude syndrome that included bilateral cleft lip and palate and lower lip pits. He was referred to this surgeon and underwent lip and palate repair as well as lower lip pit excisions during childhood. He underwent successful mixed-dentition grafting and fistula closure. As a teenager, he demonstrated maxillary hypoplasia with secondary deformities of the mandible and the nasal cavity. He then underwent a comprehensive orthodontic and orthognathic/intranasal surgical approach. The patient’s procedures included standard Le Fort I osteotomy (vertical lengthening, horizontal advancement, and clockwise rotation) with interpositional grafting; bilateral sagittal split ramus osteotomies (clockwise rotation); osseous genioplasty (horizontal advancement); and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, The patient is shown at the time of birth and then just before mixed-dentition grafting. B, Frontal views in repose before and after reconstruction. C, Frontal views with smile before and after reconstruction. D, Oblique facial views before and after reconstruction. E, Profile views before and after reconstruction. F, Occlusal views are shown before mixed-dentition bone grafting and fistula closure, with orthodontics in preparation for orthognathic surgery, and after the completion of reconstruction. A dental implant is planned for the congenitally missing right maxillary bicuspid when the patient is 18 years old. G, Articulated dental casts indicate analytic model planning. H, Cephalometric radiographs before and after reconstruction.

Figure 33-4 A child who was born with bilateral cleft lip and palate underwent lip and palate repair during infancy and early childhood by another surgeon. During the mixed dentition, she was referred to this surgeon with a mobile premaxilla, alveolar and palatal defects, and residual labial and palatal fistulas. She had the congenital absence of several permanent teeth. She underwent a short phase of orthodontic expansion and then successful iliac grafting and fistula closure during the mixed dentition. As a teenager, she underwent a comprehensive orthodontic and orthognathic/intranasal surgical approach. The patient’s procedures included standard Le Fort I osteotomy (horizontal advancement and vertical shortening); bilateral sagittal split ramus osteotomies (clockwise rotation); osseous genioplasty (horizontal advancement); and septoplasty, inferior turbinate reduction, and nasal floor recontouring. A, The patient is shown at the time of birth. B, Facial and lateral cephalometric radiographic views during the adult dentition before and then after maxillary extractions with orthodontic retraction. C, Articulated dental casts that indicate analytic model planning. D, Occlusal view during the adult dentition before extractions. E, Occlusal view after extractions and before orthodontic decompensation. F, Occlusal view after orthodontic retraction and before surgery. G, Occlusal view early after surgical correction. H, Frontal views with smile before and after reconstruction. I, Oblique facial views before and after reconstruction. J, Profile views before and after reconstruction. K, Occlusal views before and after reconstruction.

Figure 33-5 A 17-year-old girl who was born with bilateral cleft lip and palate. She underwent lip repair by this surgeon during childhood. This was followed at another institution by cleft palate repair; a columella lengthening of the nasal soft tissues when she was 5 years old; a pharyngeal flap for the management of velopharyngeal insufficiency when she was 7 years old; and then bone grafting to the alveolar clefts when she was 8 years old. She was referred back to this surgeon when she was 17 years old and found to have cleft nasal deformities, chronic obstructed nasal breathing, severe maxillary hypoplasia, secondary deformities of the mandible, and retained wisdom teeth. The maxilla had been successfully grafted with orthodontic closure of the cleft dental gaps. The patient then underwent reconstruction by this surgeon, including maxillary Le Fort I osteotomy in two segments with differential repositioning (horizontal advancement +14 mm, vertical lengthening +5 mm, cant correction, and transverse narrowing) with interpositional grafting; bilateral sagittal split ramus osteotomies (asymmetry correction); osseous genioplasty (horizontal advancement); septoplasty, inferior turbinate reduction, and nasal base recontouring; and the removal of retained wisdom teeth. Before surgery, she underwent a nasoendoscopic speech assessment that demonstrated the satisfactory closure of the left lateral port with incomplete closure of the right lateral port (i.e., small air leakage). She maintained adequate velopharyngeal closure after surgery. Orthodontic maintenance and detailing continued and were followed by a removable maxillary retainer and a fixed lower anterior retaining wire. One year later, the patient underwent cleft rhinoplasty that included nasal osteotomies, the modification of the lower lateral cartilages, and a rib cartilage (caudal strut) graft. Note that ideal nasal aesthetics were compromised after a columella lengthening procedure that caused scarring of the soft tissues of the columella and distortions of the alar rims and tip. A, Frontal views in repose before and after reconstruction. B, Frontal views with smile before and after reconstruction. C, Oblique facial views before and after reconstruction. D, Profile views before and after reconstruction. E, Occlusal views with orthodontic appliances in place and then after reconstruction. F and G, Articulated dental casts that indicate analytic model planning. H, Lateral cephalometric radiographs before and after reconstruction.

Figure 33-6 Three young adults who were born with bilateral cleft lip and palate are shown. Each had undergone bone grafting during the mixed dentition at another institution. Facial, occlusal, and computed tomography scan views indicate successful grafting at one cleft site but not at the other. It is important to make this determination when orthognathic surgery is required. If successful grafting was accomplished at one cleft site, then a circumvestibular incision can be made with full down-fracture, as one would for an ungrafted unilateral cleft alveolus. If successful grafting was not accomplished on either side, then a modified Le Fort I osteotomy in three segments would be required to maintain circulation to each of the three segments (i.e., the premaxilla, the left posterior segment, and the right posterior segment). This is done to avoid the complication of aseptic necrosis of the premaxilla (see Fig. 33-17). A, A teenager who was born with van der Woude syndrome and bilateral cleft lip and palate. He underwent successful grafting of one cleft site but not the other at another institution. He presented to this surgeon for orthognathic surgery. Facial, occlusal, and computed tomography scan views are shown. B, A teenager who was born with bilateral cleft lip and palate underwent successful alveolar grafting on the left side but not the right at another institution. He presented to this surgeon for orthognathic surgery. Facial, occlusal, and computed tomography scan views are shown and confirm these facts. C, A teenage girl who was born with bilateral cleft lip and palate underwent successful alveolar grafting on the right side but not the left. She then presented to this surgeon for orthognathic surgery. Facial and occlusal views when the previous patient was 7 years old, before grafting. D, Computed tomography scans of the same girl when she was 16 years old confirm successful grafting on right side but continued alveolar clefting on left side. E, Illustrations of incisions for a modified Le Fort I osteotomy in two segments either for a patient with unilateral cleft lip and palate and an open cleft or a patient born with BCLP and a successful graft on just one side. Part E modified from an original illustration by Bill Winn. F, Illustrations of the incisions required to maintain circulation to each of three segments in an unsuccessfully grafted case of bilateral cleft lip and palate.